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Yan J, Yang H, Xiao H, Li C. Congenital intestinal malrotation, duodenal obstruction combined with dextrocardia: a rare case report. Front Pediatr 2025; 13:1554891. [PMID: 40264466 PMCID: PMC12011855 DOI: 10.3389/fped.2025.1554891] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/03/2025] [Accepted: 03/17/2025] [Indexed: 04/24/2025] Open
Abstract
Background Both intestinal malrotation and situs inversus are usually asymptomatic and extremely rare. We reported a case of congenital intestinal malrotation, duodenal obstruction, dextrocardia, and pancreatic and splenic hypoplasia in a newborn. Case presentation The patient was a 17-day-old premature infant who had experienced recurrent vomiting for 5 days. Upper gastrointestinal and small intestine contrast imaging indicated intestinal malrotation and duodenal obstruction. Abdominal contrast-enhanced CT showed a small, underdeveloped pancreas, multiple nodular soft tissue densities in the area anterior to the left kidney and posterior to the stomach, and unclear splenic venous and arterial structures. Cardiac CT revealed dextrocardia with an atrial septal defect (secundum type). The laparoscopic Ladd's procedure was performed. The postoperative course was uneventful, and the patient recovered well during a 4-month follow-up. Conclusions This patient was very young and presented with multiple abnormalities. This case highlights the importance of early diagnosis, timely referral, and management of such rare congenital anomalies to achieve favorable outcomes.
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Affiliation(s)
| | | | | | - Chuanxin Li
- Department of General Surgery, Kunming Children’s Hospital, Kunming, China
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Hwang MS, Kuo CC, Wang CJ, Su WJ, Chu JJ, Chung HT, Hsiao HJ, Chang YJ. Clinical implications of dextrocardia based on four visceroatrial situs studies. Pediatr Neonatol 2024; 65:566-570. [PMID: 38692948 DOI: 10.1016/j.pedneo.2023.10.011] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/28/2023] [Revised: 09/26/2023] [Accepted: 10/30/2023] [Indexed: 05/03/2024] Open
Abstract
BACKGROUND Previous studies on congenital heart diseases (CHD) associated with dextrocardia were based on selective patient databases and did not reflect the full spectrum of dextrocardia in the general population. Additionally, these studies had complex classification and presentation. Nor did these studies elaborate on the distribution of the associated CHD's complexity, the various segmental connections, and associated CHD among the four visceroatrial situs. METHODS We retrospectively reviewed the medical records of 211 children with primary dextrocardia. We used a segmental approach to diagnose CHD. We then analyzed and compared the distribution of the above-mentioned issues among the four visceroatrial situs. RESULTS Dextrocardia occurred most commonly with situs inversus (52.6%), followed by situs solitus (28.4%), asplenia (17.1%), and polysplenia (1.9%). Although some patients had a structurally normal heart (22.7%) or they were associated with simple CHD (17.5%), most patients had complex CHD (59.7%) consisting of a single ventricle (34.6%) or conotruncal anomaly (25.1%) (double-outlet right ventricle [7.6%], corrected transposition of the great arteries [6.2%], complete transposition of the great arteries [5.7%], tetralogy of Fallot [4.7%], etc.). Situs inversus or polysplenia had a higher prevalence of a structurally normal heart or associated with simple CHD, two patent atrioventricular (AV) valves connections, and biventricular AV connections. Situs solitus or asplenia had a higher prevalence of associated complex CHD, common AV valve connection, univentricular AV connection, pulmonary outflow tract obstruction, and anomalous pulmonary venous drainage. CONCLUSION Our study finds that situs inversus is the most common visceroatrial situs in dextrocardia. Although some patients had a structurally normal heart or were associated with simple CHD, most patients have associated complex CHD consisting of a single ventricle or conotruncal anomaly. Dextrocardia is associated with a higher incidence of complex CHD in situs solitus and asplenia groups than in situs inversus and polysplenia groups.
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Affiliation(s)
- Mao-Sheng Hwang
- Department of Pediatrics, Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Taoyuan, Taiwan.
| | - Ching-Chia Kuo
- Department of Pediatrics, Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Taoyuan, Taiwan
| | - Chao-Jan Wang
- Department of Radiology, Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Taoyuan, Taiwan
| | - Wen-Jen Su
- Department of Pediatrics, Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Taoyuan, Taiwan
| | - Jaw-Ji Chu
- Department of Surgery, New Taipei Municipal Tucheng Hospital, Chang Gung Memorial Hospital, Tucheng 236, Taiwan; Chang Gung University College of Medicine, Taoyuan, 333, Taiwan
| | - Hung-Tao Chung
- Department of Pediatrics, Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Taoyuan, Taiwan
| | - Hsiang-Ju Hsiao
- Department of Pediatrics, Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Taoyuan, Taiwan
| | - Yi-Jung Chang
- Department of Pediatrics, Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Taoyuan, Taiwan.
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Yang M, Pu SL, Li L, Ma Y, Qin Q, Wang YX, Huang WL, Hu HY, Zhu MF, Li CZ. Hypoparathyroidism with situs inversus totalis: A case report. World J Radiol 2024; 16:561-568. [PMID: 39494145 PMCID: PMC11525822 DOI: 10.4329/wjr.v16.i10.561] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/05/2024] [Revised: 08/31/2024] [Accepted: 09/19/2024] [Indexed: 10/28/2024] Open
Abstract
BACKGROUND Hypoparathyroidism (HP) is a rare endocrine disorder, while situs inversus totalis (SIT) is a rare condition in which the internal organs are positioned in a mirrored pattern compared to their usual positions. This case illustrates some potential shared mechanisms between HP and SIT, highlighting the importance of accurate identification and prompt first emergency, offering insights for future research. CASE SUMMARY This report discusses a case of a middle-aged patient with adolescent-onset HP with concurrent SIT. The patient experienced recurrent episodes of increased neuromuscular excitability (manifesting as spasms in the hands and feet and laryngospasms) and even periods of unconsciousness. Initially, these symptoms led to a misdiagnosis of epilepsy. Nevertheless, upon thorough examination and treatment in the general medicine ward, the correct diagnosis was established. Corresponding treatment resulted in improved management of the patient's symptoms. CONCLUSION Co-occurrence of HP and SIT may be associated with genetic mutations, chromosomal anomalies, or hereditary factors, as may other similar conditions.
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Affiliation(s)
- Mao Yang
- Department of General Medicine, First People’s Hospital of Zunyi (Third Affiliated Hospital of Zunyi Medical University), Zunyi 563000, Guizhou Province, China
| | - Sheng-Lan Pu
- Department of General Medicine, First People’s Hospital of Zunyi (Third Affiliated Hospital of Zunyi Medical University), Zunyi 563000, Guizhou Province, China
| | - Ling Li
- Department of General Medicine, First People’s Hospital of Zunyi (Third Affiliated Hospital of Zunyi Medical University), Zunyi 563000, Guizhou Province, China
| | - Yu Ma
- Department of Nutrition, First People’s Hospital of Zunyi (Third Affiliated Hospital of Zunyi Medical University), Zunyi 563000, Guizhou Province, China
| | - Qin Qin
- Department of General Medicine, First People’s Hospital of Zunyi (Third Affiliated Hospital of Zunyi Medical University), Zunyi 563000, Guizhou Province, China
| | - Yan-Xia Wang
- Department of General Medicine, First People’s Hospital of Zunyi (Third Affiliated Hospital of Zunyi Medical University), Zunyi 563000, Guizhou Province, China
| | - Wen-Long Huang
- Department of General Medicine, First People’s Hospital of Zunyi (Third Affiliated Hospital of Zunyi Medical University), Zunyi 563000, Guizhou Province, China
| | - Hong-Ya Hu
- Department of General Medicine, First People’s Hospital of Zunyi (Third Affiliated Hospital of Zunyi Medical University), Zunyi 563000, Guizhou Province, China
| | - Mei-Feng Zhu
- Department of General Medicine, First People’s Hospital of Zunyi (Third Affiliated Hospital of Zunyi Medical University), Zunyi 563000, Guizhou Province, China
| | - Chun-Zhu Li
- Department of General Medicine, First People’s Hospital of Zunyi (Third Affiliated Hospital of Zunyi Medical University), Zunyi 563000, Guizhou Province, China
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Khan ZS, Saini SK, Chua WJ, (Jacky) Liao HT, Manikkam S. Kartagener syndrome with pectus excavatum and upper lobar bronchiectasis. Radiol Case Rep 2024; 19:3952-3958. [PMID: 39050650 PMCID: PMC11266874 DOI: 10.1016/j.radcr.2024.06.007] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/10/2024] [Revised: 05/28/2024] [Accepted: 06/03/2024] [Indexed: 07/27/2024] Open
Abstract
Primary Ciliary Dyskinesia (PCD) is a rare autosomal recessive disorder caused by impaired ciliary function. The incidence of PCD is 1 in 20,000 births. Kartagener's syndrome (KS), a subtype of PCD, is distinguished by the presence of situs inversus. KS occurs in about 1 in 32,000 to 40,000 births. Characterized by a triad of situs inversus totalis, sinusitis, and typically lower lobe bronchiectasis, Kartagener's syndrome presents with distinct radiological features, which are explored in this case study. We report on an adolescent male with Kartagener's syndrome, manifesting atypical bronchiectasis in the left upper lobe, leading to a bilateral lung transplant, and severe pectus excavatum requiring surgical correction. This case documents a male patient with concurrent Kartagener's syndrome and pectus excavatum, supporting a previously explored, albeit theoretical association between these conditions.
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Affiliation(s)
- Zain Saleem Khan
- Gold Coast University Hospital, 1 Hospital Blvd, Southport Queensland 4215, Australia
- Griffith University, 1 Parklands Dr, Southport Queensland 4215, Australia
| | - Saransh Kumar Saini
- Gold Coast University Hospital, 1 Hospital Blvd, Southport Queensland 4215, Australia
- Griffith University, 1 Parklands Dr, Southport Queensland 4215, Australia
| | - Weng Joe Chua
- Royal North Shore Hospital, Reserve Rd, St Leonards NSW 2065, Australia
| | - Hao Ting (Jacky) Liao
- Gold Coast University Hospital, 1 Hospital Blvd, Southport Queensland 4215, Australia
- Griffith University, 1 Parklands Dr, Southport Queensland 4215, Australia
| | - Samuel Manikkam
- Gold Coast University Hospital, 1 Hospital Blvd, Southport Queensland 4215, Australia
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Tadesse KD, Birhan YA. Mirror Image Dextrocardia with Severe Rheumatic Mitral Stenosis Undergone Successful Percutaneous Transvenous Commissurotomy: The First Case in East Africa. Int Med Case Rep J 2024; 17:677-682. [PMID: 39050699 PMCID: PMC11268842 DOI: 10.2147/imcrj.s439610] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/18/2023] [Accepted: 07/05/2024] [Indexed: 07/27/2024] Open
Abstract
Percutaneous transvenous mitral commissurotomy is an established treatment for rheumatic mitral stenosis. Dextrocardic heart can be affected by rheumatic pathology just like the normal heart. However, it is technically demanding to percutaneous interventions especially the septal puncture which should be done in a mirror image of the normal. Here we are reporting a 45 year old woman with severe rheumatic mitral stenosis with dextrocardia with situs inversus totalis. She has undergone a successful percutaneous transvenous mitral commissurotomy.
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Xue H, Yu A, Chen L, Guo Q, Zhang L, Lin N, Chen X, Xu L, Huang H. Prenatal genetic diagnosis of fetuses with dextrocardia using whole exome sequencing in a tertiary center. Sci Rep 2024; 14:16266. [PMID: 39009665 PMCID: PMC11251054 DOI: 10.1038/s41598-024-67164-w] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/31/2024] [Accepted: 07/09/2024] [Indexed: 07/17/2024] Open
Abstract
To evaluate the genetic etiology of fetal dextrocardia, associated ultrasound anomalies, and perinatal outcomes, we investigated the utility of whole exome sequencing (WES) for prenatal diagnosis of dextrocardia. Fetuses with dextrocardia were prospectively collected between January 2016 and December 2022. Trio-WES was performed on fetuses with dextrocardia, following normal karyotyping and/or chromosomal microarray analysis (CMA) results. A total of 29 fetuses with dextrocardia were collected, including 27 (93.1%) diagnosed with situs inversus totalis and 2 (6.9%) with situs inversus partialis. Cardiac malformations were present in nine cases, extra-cardiac anomalies were found in seven cases, and both cardiac and extra-cardiac malformations were identified in one case. The fetal karyotypes and CMA results of 29 cases were normal. Of the 29 cases with dextrocardia, 15 underwent WES, and the other 14 cases refused. Of the 15 cases that underwent WES, clinically relevant variants were identified in 5/15 (33.3%) cases, including the diagnostic variants DNAH5, DNAH11, LRRC56, PEX10, and ZIC3, which were verified by Sanger sequencing. Of the 10 cases with non-diagnostic results via WES, eight (80%) chose to continue the pregnancies. Of the 29 fetuses with dextrocardia, 10 were terminated during pregnancy, and 19 were live born. Fetal dextrocardia is often accompanied by cardiac and extra-cardiac anomalies, and fetal dextrocardia accompanied by situs inversus is associated with a high risk of primary ciliary dyskinesia. Trio-WES is recommended following normal karyotyping and CMA results because it can improve the diagnostic utility of genetic variants of fetal dextrocardia, accurately predict fetal prognosis, and guide perinatal management and the reproductive decisions of affected families.
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Affiliation(s)
- Huili Xue
- Medical Genetic Diagnosis and Therapy Center, Fujian Key Laboratory for Prenatal Diagnosis and Birth Defect, Fujian Maternity and Child Health Hospital College of Clinical Medicine for Obstetrics and Gynecology and Pediatrics, Fujian Medical University, No. 18 Daoshan Road, Gulou District, Fuzhou City, 350001, Fujian Province, China.
| | - Aili Yu
- Reproductive Medicine Center, Fujian Maternity and Child Health Hospital College of Clinical Medicine for Obstetrics and Gynecology and Pediatrics, Fujian Medical University, No. 18 Daoshan Road, Gulou District, Fuzhou City, 350001, Fujian Province, China
| | - Lingji Chen
- Medical Genetic Diagnosis and Therapy Center, Fujian Key Laboratory for Prenatal Diagnosis and Birth Defect, Fujian Maternity and Child Health Hospital College of Clinical Medicine for Obstetrics and Gynecology and Pediatrics, Fujian Medical University, No. 18 Daoshan Road, Gulou District, Fuzhou City, 350001, Fujian Province, China
| | - Qun Guo
- Medical Genetic Diagnosis and Therapy Center, Fujian Key Laboratory for Prenatal Diagnosis and Birth Defect, Fujian Maternity and Child Health Hospital College of Clinical Medicine for Obstetrics and Gynecology and Pediatrics, Fujian Medical University, No. 18 Daoshan Road, Gulou District, Fuzhou City, 350001, Fujian Province, China
| | - Lin Zhang
- Fujian Medical University, No. 88 Jiaotong Road, Cangshan District, Fuzhou City, 350001, Fujian Province, China
| | - Na Lin
- Medical Genetic Diagnosis and Therapy Center, Fujian Key Laboratory for Prenatal Diagnosis and Birth Defect, Fujian Maternity and Child Health Hospital College of Clinical Medicine for Obstetrics and Gynecology and Pediatrics, Fujian Medical University, No. 18 Daoshan Road, Gulou District, Fuzhou City, 350001, Fujian Province, China
| | - Xuemei Chen
- Medical Genetic Diagnosis and Therapy Center, Fujian Key Laboratory for Prenatal Diagnosis and Birth Defect, Fujian Maternity and Child Health Hospital College of Clinical Medicine for Obstetrics and Gynecology and Pediatrics, Fujian Medical University, No. 18 Daoshan Road, Gulou District, Fuzhou City, 350001, Fujian Province, China
| | - Liangpu Xu
- Medical Genetic Diagnosis and Therapy Center, Fujian Key Laboratory for Prenatal Diagnosis and Birth Defect, Fujian Maternity and Child Health Hospital College of Clinical Medicine for Obstetrics and Gynecology and Pediatrics, Fujian Medical University, No. 18 Daoshan Road, Gulou District, Fuzhou City, 350001, Fujian Province, China.
| | - Hailong Huang
- Medical Genetic Diagnosis and Therapy Center, Fujian Key Laboratory for Prenatal Diagnosis and Birth Defect, Fujian Maternity and Child Health Hospital College of Clinical Medicine for Obstetrics and Gynecology and Pediatrics, Fujian Medical University, No. 18 Daoshan Road, Gulou District, Fuzhou City, 350001, Fujian Province, China.
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Carvalho TD, Freitas OGAD, Chalela WA, Hossri CAC, Milani M, Buglia S, Precoma DB, Falcão AMGM, Mastrocola LE, Castro I, Albuquerque PFD, Coutinho RQ, Brito FSD, Alves JDC, Serra SM, Santos MAD, Colombo CSSDS, Stein R, Herdy AH, Silveira ADD, Castro CLBD, Silva MMFD, Meneghello RS, Ritt LEF, Malafaia FL, Marinucci LFB, Pena JLB, Almeida AEMD, Vieira MLC, Stier Júnior AL. Brazilian Guideline for Exercise Test in the Adult Population - 2024. Arq Bras Cardiol 2024; 121:e20240110. [PMID: 38896581 PMCID: PMC11656589 DOI: 10.36660/abc.20240110] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 06/21/2024] Open
Affiliation(s)
- Tales de Carvalho
- Clínica de Prevenção e Reabilitação Cardiosport, Florianópolis, SC - Brasil
- Universidade do Estado de Santa Catarina, Florianópolis, SC - Brasil
| | | | - William Azem Chalela
- Instituto do Coração do Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo (InCor-HCFMUSP), São Paulo, SP - Brasil
| | | | - Mauricio Milani
- Universidade de Brasília (UnB), Brasília, DF, Brasil
- Hasselt University, Hasselt - Bélgica
- Jessa Ziekenhuis, Hasselt - Bélgica
| | - Susimeire Buglia
- Instituto Dante Pazzanese de Cardiologia, São Paulo, SP - Brasil
| | | | - Andréa Maria Gomes Marinho Falcão
- Instituto do Coração do Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo (InCor-HCFMUSP), São Paulo, SP - Brasil
| | | | - Iran Castro
- Instituto de Cardiologia do Rio Grande do Sul, Porto Alegre, RS - Brasil
| | | | | | | | | | - Salvador Manoel Serra
- Instituto Estadual de Cardiologia Aloysio de Castro (IECAC), Rio de Janeiro, RJ - Brasil
| | - Mauro Augusto Dos Santos
- Instituto Nacional de Cardiologia do Rio de Janeiro, Rio de Janeiro, RJ - Brasil
- Linkcare Saúde, Rio de Janeiro, RJ - Brasil
| | | | - Ricardo Stein
- Universidade Federal do Rio Grande do Sul (UFRGS), Porto Alegre, RS - Brasil
| | - Artur Haddad Herdy
- Clínica de Prevenção e Reabilitação Cardiosport, Florianópolis, SC - Brasil
| | - Anderson Donelli da Silveira
- Universidade Federal do Rio Grande do Sul (UFRGS), Porto Alegre, RS - Brasil
- Hospital de Clínicas de Porto Alegre, Porto Alegre, RS - Brasil
- Hospital Moinhos de Vento, Porto Alegre, RS - Brasil
| | - Claudia Lucia Barros de Castro
- Universidade Federal do Rio de Janeiro (UFRJ), Rio de Janeiro, RJ - Brasil
- CLINIMEX - Clínica de Medicina de Exercício, Rio de Janeiro, RJ - Brasil
| | | | | | - Luiz Eduardo Fonteles Ritt
- Escola Bahiana de Medicina e Saúde Pública, Salvador, BA - Brasil
- Instituto D'Or de Pesquisa e Ensino, Salvador, BA - Brasil
- Hospital Cárdio Pulmonar, Salvador, BA - Brasil
| | - Felipe Lopes Malafaia
- Hospital Samaritano Paulista, São Paulo, SP - Brasil
- UnitedHealth Group Brasil, São Paulo, SP - Brasil
| | - Leonardo Filipe Benedeti Marinucci
- Instituto do Coração do Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo (InCor-HCFMUSP), São Paulo, SP - Brasil
| | - José Luiz Barros Pena
- Faculdade Ciências Médicas de Minas Gerais, Belo Horizonte, MG - Brasil
- Hospital Felício Rocho, Belo Horizonte, MG - Brasil
| | | | - Marcelo Luiz Campos Vieira
- Instituto do Coração do Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo (InCor-HCFMUSP), São Paulo, SP - Brasil
- Hospital Israelita Albert Einstein, São Paulo, SP - Brasil
| | - Arnaldo Laffitte Stier Júnior
- Universidade Federal do Paraná (UFPR), Curitiba, PR - Brasil
- Secretaria Municipal de Saúde Curitiba, Curitiba, PR - Brasil
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Kim JH, Jung JY, Lee S, Hwang S, Park JW, Lee EJ, Lee HN, Kim DK, Kwak YH. Ideal chest compression site for cardiopulmonary resuscitation in fontan circulation patients with dextrocardia. BMC Cardiovasc Disord 2024; 24:22. [PMID: 38172727 PMCID: PMC10765782 DOI: 10.1186/s12872-023-03691-0] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/06/2023] [Accepted: 12/24/2023] [Indexed: 01/05/2024] Open
Abstract
BACKGROUND We aimed to identify the ideal chest compression site for cardiopulmonary resuscitation (CPR) in patients with a single ventricle with dextrocardia corrected by Fontan surgery. METHODS The most recent stored chest computed tomography images of all patients with a single ventricle who underwent Fontan surgery were retrospectively analysed. We reported that the ideal chest compression site is the largest part of the compressed single ventricle. To identify the ideal chest compression site, we measured the distance from the midline of the sternum to the point of the maximum sagittal area of the single ventricle as a deviation and calculated the area fraction of the compressed structures. RESULTS 58 patients (67.2% male) were analysed. The mean right deviation from the midline of the sternum to the ideal compression site was similar to the mean sternum width (32.85 ± 15.61 vs. 31.05 ± 6.75 mm). When chest compression was performed at the ideal site, the area fraction of the single ventricle significantly increased by 7%, which was greater than that of conventional compression (0.15 ± 0.10 vs. 0.22 ± 0.11, P < 0.05). CONCLUSIONS When performing CPR on a patient with Fontan circulation with dextrocardia, right-sided chest compression may be better than the conventional location.
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Affiliation(s)
- Jin Hee Kim
- Department of Emergency Medicine, Seoul National University Hospital, 101 Daehak-ro, Jongno-gu, Seoul, 03080, Republic of Korea
| | - Jae Yun Jung
- Department of Emergency Medicine, Seoul National University Hospital, 101 Daehak-ro, Jongno-gu, Seoul, 03080, Republic of Korea.
| | - Sangyun Lee
- Department of Paediatrics, Seoul National University Children's Hospital, 101 Daehak-ro, Jongno-gu, Seoul, 03080, Republic of Korea
| | - Soyun Hwang
- Department of Paediatrics, Severance Hospital, 50-1, Yonsei-ro, Seodaemun-gu, Seoul, Republic of Korea
| | - Joong Wan Park
- Department of Emergency Medicine, Seoul National University Hospital, 101 Daehak-ro, Jongno-gu, Seoul, 03080, Republic of Korea
| | - Eui Jun Lee
- Department of Emergency Medicine, Seoul National University Hospital, 101 Daehak-ro, Jongno-gu, Seoul, 03080, Republic of Korea
| | - Ha Ni Lee
- Department of Emergency Medicine, Seoul National University Hospital, 101 Daehak-ro, Jongno-gu, Seoul, 03080, Republic of Korea
| | - Do Kyun Kim
- Department of Emergency Medicine, Seoul National University Hospital, 101 Daehak-ro, Jongno-gu, Seoul, 03080, Republic of Korea
| | - Young Ho Kwak
- Department of Emergency Medicine, Seoul National University Hospital, 101 Daehak-ro, Jongno-gu, Seoul, 03080, Republic of Korea
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Messmore M, Kassab AJ, Prather RO, Arceo DAC, DeCampli W. Cilia and Nodal Flow in Asymmetry: An Engineering Perspective. Crit Rev Biomed Eng 2024; 52:63-82. [PMID: 38523441 DOI: 10.1615/critrevbiomedeng.2024051678] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 03/26/2024]
Abstract
Over the past several years, cilia in the primitive node have become recognized more and more for their contribution to development, and more specifically, for their role in axis determination. Although many of the mechanisms behind their influence remain undocumented, it is known that their presence and motion in the primitive node of developing embryos is the determinant of the left-right axis. Studies on cilial mechanics and nodal fluid dynamics have provided clues as to how this asymmetry mechanism works, and more importantly, have shown that direct manipulation of the flow field in the node can directly influence physiology. Although relatively uncommon, cilial disorders have been shown to have a variety of impacts on individuals from chronic respiratory infections to infertility, as well as situs inversus which is linked to congenital heart disease. After first providing background information pertinent to understanding nodal flow and information on why this discussion is important, this paper aims to give a review of the history of nodal cilia investigations, an overview of cilia mechanics and nodal flow dynamics, as well as a review of research studies current and past that sought to understand the mechanisms behind nodal cilia's involvement in symmetry-breaking pathways through a biomedical engineering perspective. This discussion has the additional intention to compile interdisciplinary knowledge on asymmetry and development such that it may encourage more collaborative efforts between the sciences on this topic, as well as provide insight on potential paths forward in the field.
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Affiliation(s)
| | - Alain J Kassab
- Department of Mechanical and Aerospace Engineering, University of Central Florida, 4000 Central Florida Blvd, Orlando, Florida, USA
| | - Ray O Prather
- Embry-Riddle Aeronautical University, Daytona Beach, FL, 32114, USA; University of Central Florida, Orlando, FL 32816, USA; The Heart Center at Orlando Health Arnold Palmer Hospital for Children, Orlando, FL 32806, USA
| | - David A Castillo Arceo
- College of Engineering and Computer Science (CECS), University of Central Florida, Orlando, FL, USA
| | - William DeCampli
- University of Central Florida, Orlando, FL, 32816, USA; The Heart Center, Arnold Palmer Hospital for Children, Orlando, FL, 32806, USA
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Hayashi LC, Acharya R. Situs inversus totalis in an asymptomatic adolescent - importance of patient education: A case report. World J Clin Pediatr 2023; 12:359-364. [PMID: 38178936 PMCID: PMC10762603 DOI: 10.5409/wjcp.v12.i5.359] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/06/2023] [Revised: 09/09/2023] [Accepted: 09/26/2023] [Indexed: 12/08/2023] Open
Abstract
BACKGROUND Situs inversus totalis (SIT) may be an incidental finding in asymptomatic children. Patients may not understand the implications of this condition and the importance of relaying the diagnosis to their healthcare providers. CASE SUMMARY We report an asymptomatic seventeen-year-old adolescent with previously-diagnosed SIT who presented for a routine well-child visit. During history taking, he denied any past medical conditions, including cardiovascular conditions. Only when physical exam revealed point of maximal impulse and heart sounds on the right side, did he convey that he had been diagnosed with SIT incidentally at age of 12 years. He was not aware of associated conditions or the potential implications of his diagnosis, nor did he realize it is pertinent medical history to be relayed to healthcare providers. Chest X-ray confirmed dextrocardia and abdominal X-ray showed right-sided stomach. Abdomen sonogram showed left-sided liver and right-sided spleen. Echocardiogram showed normal valvular structure and function. A comprehensive discussion was provided to address the patient's lack of understanding that SIT is a medical diagnosis with potential implications. CONCLUSION While SIT is rare and mostly asymptomatic, affected patients may not comprehend the importance of the diagnosis and its potential ramifications. Recognition of the patient's lack of awareness allows the healthcare provider to educate the patient and hopefully can prevent potential medical and surgical complications.
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Affiliation(s)
- Lauren C. Hayashi
- Department of Pediatrics, University of Florida College of Medicine, Gainesville, FL 32608, United States
| | - Ratna Acharya
- Division of General Pediatrics, Department of Pediatrics, University of Florida, Gainesville, FL 32608, United States
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Nerune SM, Bidri SR, Sandilya U, Das SK. Role of autopsy in diagnosing asplenia and right bilobed lung in a fetus with dextrocardia. BMJ Case Rep 2023; 16:e256209. [PMID: 37775274 PMCID: PMC10546108 DOI: 10.1136/bcr-2023-256209] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/01/2023] Open
Abstract
We present a unique case of a fetus with dextrocardia, asplenia and a right bilobed lung in a primigravida woman in her 20s at 21 weeks' gestation. Prenatal ultrasound examination revealed dextrocardia and other anomalies such as atrioventricular septal defect and situs ambiguous with the gallbladder on the left, leading to termination of the pregnancy. Fetal autopsy confirmed the diagnosis, detected additional findings such as asplenia and right bilobed lung missed on ultrasound and highlighted the importance of autopsy in prenatal diagnosis. This rare case emphasises the value of a comprehensive prenatal assessment, fetal autopsy and a multidisciplinary approach in diagnosing, managing and counselling families affected by congenital anomalies. Timely detection and appropriate genetic counselling can guide affected families in making informed decisions regarding future pregnancies while providing closure and support in their grieving process.
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Affiliation(s)
- Savitri M Nerune
- Department of Pathology, Shri B.M. Patil Medical College, Hospital and Research Centre, BLDE (Deemed to be University), Vijayapura, Karantaka, India
| | - Shailaja R Bidri
- Department of Obstetrics and Gynecology, Shri B.M. Patil Medical College Hospital and Research Centre, BLDE (Deemed to be University), Vijayapura, Karnataka, India
| | - Upasana Sandilya
- Department of Pathology, Shri B.M. Patil Medical College, Hospital and Research Centre, BLDE (Deemed to be University), Vijayapura, Karantaka, India
| | - Sayandeep K Das
- Department of Pathology, Shri B.M. Patil Medical College, Hospital and Research Centre, BLDE (Deemed to be University), Vijayapura, Karantaka, India
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12
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Sidrak MMA, De Feo MS, Gorica J, Corica F, Conte M, Filippi L, De Vincentis G, Frantellizzi V. Medication and ECG Patterns That May Hinder SPECT Myocardial Perfusion Scans. Pharmaceuticals (Basel) 2023; 16:854. [PMID: 37375801 DOI: 10.3390/ph16060854] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/28/2023] [Revised: 05/24/2023] [Accepted: 06/02/2023] [Indexed: 06/29/2023] Open
Abstract
Coronary artery disease (CAD) is the leading cause of death followed by cancer, in men and women. With risk factors being endemic and the increasing costs of healthcare for management and treatment, myocardial perfusion imaging (MPI) finds a central role in risk stratification and prognosis for CAD patients, but it comes with its limitations in that the referring clinician and managing team must be aware of and use at their advantage. This narrative review examines the utility of myocardial perfusion scans in the diagnosis and management of patients with ECG alterations such as atrioventricular block (AVB), and medications including calcium channel blockers (CCB), beta blockers (BB), and nitroglycerin which may impact the interpretation of the exam. The review analyzes the current evidence and provides insights into the limitations, delving into the reasons behind some of the contraindications to MPI.
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Affiliation(s)
- Marko Magdi Abdou Sidrak
- Department of Radiological Sciences, Oncology and Anatomo-Pathology, Sapienza, University of Rome, 00161 Rome, Italy
| | - Maria Silvia De Feo
- Department of Radiological Sciences, Oncology and Anatomo-Pathology, Sapienza, University of Rome, 00161 Rome, Italy
| | - Joana Gorica
- Department of Radiological Sciences, Oncology and Anatomo-Pathology, Sapienza, University of Rome, 00161 Rome, Italy
| | - Ferdinando Corica
- Department of Radiological Sciences, Oncology and Anatomo-Pathology, Sapienza, University of Rome, 00161 Rome, Italy
| | - Miriam Conte
- Department of Radiological Sciences, Oncology and Anatomo-Pathology, Sapienza, University of Rome, 00161 Rome, Italy
| | - Luca Filippi
- Department of Nuclear Medicine, Santa Maria Goretti Hospital, 04100 Latina, Italy
| | - Giuseppe De Vincentis
- Department of Radiological Sciences, Oncology and Anatomo-Pathology, Sapienza, University of Rome, 00161 Rome, Italy
| | - Viviana Frantellizzi
- Department of Radiological Sciences, Oncology and Anatomo-Pathology, Sapienza, University of Rome, 00161 Rome, Italy
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13
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Dinato FJ, de Almeida Brandão CM, Veronese ET, Pomerantzeff PMA, Jatene FB. Redo aortic valve surgery in a case of dextrocardia with situs inversus totalis. Int J Surg Case Rep 2022; 98:107531. [PMID: 36027831 PMCID: PMC9424602 DOI: 10.1016/j.ijscr.2022.107531] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/10/2022] [Revised: 08/15/2022] [Accepted: 08/18/2022] [Indexed: 11/29/2022] Open
Abstract
Introduction Dextrocardia is a rare congenital cardiac anomaly where the base-apex axis of the heart is directed to the right side. It may be associated with situs solitus, situs inversus, or situs ambiguous. Such cases are technically challenging when heart surgery is needed. Presentation of case We presented a case of a patient with dextrocardia and situs inversus totalis, who had severe aortic bioprosthetic valve degeneration needing a redo aortic valve replacement. Our operative strategy was to change the position of the main surgeon to the left side of the patient to perform most surgical steps. Discussion A meticulous pre-operative surgical plan involving the whole team was very important for a smooth intra-operative course and a favourable outcome. Regarding the position of the main surgeon standing on the left side of the patient, we believed this surgical team arrangement added much to the technical ease for the procedure, since the anatomy was opposite to what our minds are used to. Also, computed tomography scan played a crucial role for a proper preoperative anatomical evaluation and surgical planning. Conclusion Our approach with the surgeon on the left side provided excellent exposure for redo aortic valve replacement in dextrocardia with situs inversus totalis. This surgical management played an important role for the good result in this technically challenging scenario.
Dextrocardia with situs inversus totalis is technically challenging when needs surgery. The main surgeon stood on the left side of the patient to perform most surgical steps. Computed tomography scan played a crucial role for surgical planning. Surgical team arrangement added technical ease for the surgery.
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14
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Motaganahalli AR, Wang SK, Sawchuk AP. Endovascular Repair of an Intrathoracic Subclavian Artery Aneurysm in a Patient With Dextrocardia. Vasc Endovascular Surg 2022; 57:69-74. [PMID: 35675973 DOI: 10.1177/15385744221108045] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/16/2022]
Abstract
This manuscript describes an endovascular repair of a symptomatic, large proximal left subclavian artery aneurysm in a patient with dextrocardia and right-sided aortic arch and absent bilateral internal carotid arteries. The patient had surgical reconstruction as an infant for congenital heart disease with Ventricular Septal Defect, bifid sternum . Given her previous surgical history, we declined an open operation and performed an endovascular repair with stent grafts to successfully repair the subclavian artery aneurysm. The patient had an uneventful postoperative course and follow-up ultrasonography demonstrated successful repair with preservation of flow through the left subclavian and vertebral arteries with resolution of her symptoms.
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Affiliation(s)
| | - S Keisin Wang
- 12339University of Texas Health Sciences Center, Houston, TX, USA
| | - Alan P Sawchuk
- Department of Surgery, Indiana University School of Medicine, Indianapolis, IN, USA
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15
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Zhang W, Xing W, Zhong X, Zhu M, He J. Emergency thrombectomy for cerebrovascular occlusion in a patient with mirror-image dextrocardia: a case report. J Int Med Res 2022; 50:3000605211072785. [PMID: 35023389 PMCID: PMC8793582 DOI: 10.1177/03000605211072785] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/29/2021] [Accepted: 12/16/2021] [Indexed: 11/15/2022] Open
Abstract
Cases of patients complicated with dextrocardia who suffer from acute cerebral infarction with large vessel occlusion and receive emergency thrombectomy are particularly rare and have not been widely reported. This article aimed to increase the awareness and knowledge of these cases. We report the case of a patient with mirror-image dextrocardia who suffered from cerebral infarction with large vessel occlusion and received emergency thrombectomy. A male patient in his early 60s with dextrocardia had acute cerebral infarction with posterior circulation large vessel occlusion and underwent emergency thrombectomy. During the operation, the rapid confirmation of dextrocardia and use of flexible interventional instruments helped establish a pathway for blood flow. We used an intracranial thrombectomy stent and intracranial balloon dilation catheter to restore the cerebral blood supply. The Modified Rankin Scale score was 0 at 3 months after thrombectomy, indicating a good prognosis of the patient. Acute cerebral infarction with large vessel occlusion in patients with dextrocardia is extremely rare. Emergency thrombectomy is feasible to recanalize cerebral blood flow and give patients a chance to recover.
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Affiliation(s)
- Wensheng Zhang
- Department of Neurology, Heyuan People’s Hospital, Guangdong Provincial People’s Hospital Heyuan Hospital, Guangdong Province, China
| | - Weifang Xing
- Department of Neurology, Heyuan People’s Hospital, Guangdong Provincial People’s Hospital Heyuan Hospital, Guangdong Province, China
| | - Xiaojing Zhong
- Department of Neurology, Heyuan People’s Hospital, Guangdong Provincial People’s Hospital Heyuan Hospital, Guangdong Province, China
| | - Minzhen Zhu
- Department of Neurology, Heyuan People’s Hospital, Guangdong Provincial People’s Hospital Heyuan Hospital, Guangdong Province, China
| | - Jinzhao He
- Department of Neurology, Heyuan People’s Hospital, Guangdong Provincial People’s Hospital Heyuan Hospital, Guangdong Province, China
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16
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Chinawa AT, Chinawa JM. Compendium of cardiac diseases among children presenting in tertiary institutions in southern Nigeria: a rising trend. Libyan J Med 2021; 16:1966217. [PMID: 34382500 PMCID: PMC8366613 DOI: 10.1080/19932820.2021.1966217] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/21/2021] [Accepted: 08/06/2021] [Indexed: 10/26/2022] Open
Abstract
The burdenofchildren with cardiovascular disease is on a rising trend. These rising trends are enhanced by the development of new technology in the diagnosis of cardiac lesions.This article is aimed at documenting the current trends in the occurrence of cardiac disease among children attending tertiary hospitals in Sothern Nigeria.This is a descriptive study which involved four hundred and thirty-nine (439) children who had cardiac disease confirmed with echocardiography from four health institutions, namely; Niger Delta University, Bayelsa, Nigeria; University of Nigeria Teaching Hospital, Enugu, Nigeria; Triple care hospital, Enugu, Nigeria and; Blessed children hospital, Enugu, Nigeria over a -five -year period from July 2016- July 2020.The prevalence of congenital heart disease was 83.6% and only 16.4% being acquired heart disease. Many of the children with congenital cardiac defects have associated features of Down syndrome (trisomy 21) and the frequency was related to the type of defect; 6.4% (28/439). These features of Down syndrome are seen mainly in children with AV canal defect 33.3% (14/42) and least in children with TOF 2.0% (1/51). Zero-point two nine percent (1/439) had dextro-cardia and 0.2% (1/340) had VACTERAL (ano-rectal malformation) association, 0.2 % (1/439) had cleft lip, 1.8% (8/439) had dysmorphism other than down syndrome. Five 62.5% (5/8) of such dysmorphic features were associated with VSD.The majority of the children with cardiac disease were from the middle class 63.6% followed by the higher class 21.9% and the least is from the lower class 14.5%. The prevalence values of heart disease were quite higher than that documented in previous studiesThe is a rising prevalence of congenital heart disease (83.6%) and acquired heart disease (16.4%) among children with cardiac disease in southern Nigeria. Extracardiac anomalies were also associated with these trends.
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Affiliation(s)
- Awoere T. Chinawa
- Department Of Community Medicine, Enugu State University Teaching Hospital, Enugu State, Nigeria
| | - Josephat M. Chinawa
- Deprtment of paediatrics, University of Nigeria/University of Nigeria Teaching Hospital (UNTH), Ituku- Ozalla, Enugu State, Nigeria
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17
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Sayuti KA, Shamsuddin AM, Mamat AZ, Zain MRM, Ramli N, Mokhtar AM, Ali S. Right-sided patent ductus arteriosus with bovine aortic arch, tetralogy of Fallot, dextrocardia and situs inversus. J Surg Case Rep 2021; 2021:rjab307. [PMID: 34316346 PMCID: PMC8302079 DOI: 10.1093/jscr/rjab307] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/11/2021] [Accepted: 06/22/2021] [Indexed: 12/05/2022] Open
Abstract
A neonate with cyanosis at birth was found to have a rare type of tetralogy of Fallot. Echocardiography showed dextrocardia, left aortic arch with constricting and tortuous patent ductus arteriosus (PDA). Computed tomography angiography thorax revealed visceroatrial situs inversus, mirror image dextrocardia, tetralogy anatomy and tortuous right PDA arising from bovine brachiocephalic artery. In view of severe cyanosis, emergency division of PDA and modified right Blalock-Taussig shunt through median sternotomy were performed under cardiopulmonary bypass. Post-operatively, the sternum was left open and he developed pulmonary overcirculation requiring prolonged ICU stay. He was discharged well at Day 26 post surgery. This case highlights a rare association tetralogy of Fallot, dextrocardia and situs inversus, with concomitant unilateral right PDA and bovine aortic arch.
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Affiliation(s)
- Khairil Amir Sayuti
- Department of Radiology, School of Medical Sciences, Health Campus, Universiti Sains Malaysia Kubang Kerian, Kelantan, Malaysia
| | | | - Ahmad Zuhdi Mamat
- Department of Surgery, School of Medical Sciences, Health Campus, Universiti Sains Malaysia Kubang Kerian, Kelantan, Malaysia
| | - Mohd Rizal Mohd Zain
- Department of Pediatrics, School of Medical Sciences, Health Campus, Universiti Sains Malaysia Kubang Kerian, Kelantan, Malaysia
| | - Noraida Ramli
- Department of Pediatrics, School of Medical Sciences, Health Campus, Universiti Sains Malaysia Kubang Kerian, Kelantan, Malaysia
| | - Ariffin Marzuki Mokhtar
- Department of Anesthesia, School of Medical Sciences, Health Campus, Universiti Sains Malaysia Kubang Kerian, Kelantan, Malaysia
| | - Saedah Ali
- Department of Anesthesia, School of Medical Sciences, Health Campus, Universiti Sains Malaysia Kubang Kerian, Kelantan, Malaysia
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18
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Aggarwal P, Reddy R, Singh RS. Multivalvular rheumatic heart disease in a case of dextrocardia with situs inversus: an arduous surgical access. Indian J Thorac Cardiovasc Surg 2020; 35:584-586. [PMID: 33061056 DOI: 10.1007/s12055-019-00822-1] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/10/2019] [Revised: 03/16/2019] [Accepted: 03/19/2019] [Indexed: 11/26/2022] Open
Abstract
Dextrocardia is a rare congenital cardiac anomaly where the base-apex axis of the heart is directed to right side. Incidence of dextrocardia is 1 in 10,000 live births, and it may be associated with other congenital cardiac diseases. In cases of dextrocardia, the atrial situs can be situs solitus, situs inversus, or situs ambiguous of which dextrocardia with situs inversus (mirror image dextrocardia) is more common (40%). If all the visceral organs also get mirrored, then it is called dextrocardia with situs inversus totalis. Though dextrocardia can be associated with other congenital cardiac anomalies, its association with rheumatic heart disease is extremely rare. We report a case of dextrocardia with rheumatic heart disease involving the mitral and tricuspid valves.
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Affiliation(s)
- Pankaj Aggarwal
- Department of CTVS, Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh, 160012 India
| | - Revanth Reddy
- Department of CTVS, Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh, 160012 India
| | - Rana Sandeep Singh
- Department of CTVS, Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh, 160012 India
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19
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Shen L, Zhao J, Yuan T, Li M, Cheng Y. A case report of late-onset symptoms of erythrocytosis in univentricular dextrocardia. Medicine (Baltimore) 2020; 99:e21092. [PMID: 32629741 PMCID: PMC7337453 DOI: 10.1097/md.0000000000021092] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/25/2022] Open
Abstract
RATIONALE Univentricular dextrocardia is a rare congenital heart disease that usually presents cyanotic manifestations from childhood. Due to the sustained dysfunction of blood oxygenation, it is very difficult to keep an asymptomatic survival. Herein, we described an interesting case of univentricular dextrocardia who suffered from initial symptoms in his middle age. PATIENT CONCERNS A 54-year-old male patient with numbness and tingling of limbs was admitted to hospital due to the secondary manifestations of congenital heart disease. DIAGNOSIS The patient was diagnosed as univentricular dextrocardia with pulmonary hypertension and secondary erythrocytosis based on computed tomography (CT) scan, echocardiography, and laboratory examinations. INTERVENTIONS Intravenous hydration therapy with normal saline successfully eliminated his hyperviscosity associated symptoms. In view of socio-economic reasons, this patient refused surgical evaluation and further medical interventions. OUTCOMES During 18-month follow up, he received no drug except for regular water intake. Fortunately, his life quality was satisfactory, and no other symptoms emerged except for mild numbness of limbs. LESSONS In univentricular dextrocardia, it is possible to keep a long-term asymptomatic period due to the slow progress of pathophysiology. In this population, regular cardiac function evaluation and avoiding dehydration may help improve the quality of life.
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Affiliation(s)
- Lishui Shen
- National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing
| | | | | | | | - Yun Cheng
- Department of Ultrasound, Zhejiang Hospital, Hangzhou, China
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20
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Qutbi M. SPECT myocardial perfusion imaging in patients with Dextrocardia. J Nucl Cardiol 2019; 26:1197-1204. [PMID: 31062220 DOI: 10.1007/s12350-019-01732-w] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/18/2019] [Accepted: 04/15/2019] [Indexed: 12/29/2022]
Affiliation(s)
- Mohsen Qutbi
- Department of Nuclear Medicine, Taleghani Educational Hospital, School of Medicine, Shahid Beheshti University of Medical Sciences, Yaman St., Velenjak, Tehran, Iran.
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21
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Garnai SJ, Brinkmeier ML, Emery B, Aleman TS, Pyle LC, Veleva-Rotse B, Sisk RA, Rozsa FW, Ozel AB, Li JZ, Moroi SE, Archer SM, Lin CM, Sheskey S, Wiinikka-Buesser L, Eadie J, Urquhart JE, Black GC, Othman MI, Boehnke M, Sullivan SA, Skuta GL, Pawar HS, Katz AE, Huryn LA, Hufnagel RB, Camper SA, Richards JE, Prasov L. Variants in myelin regulatory factor (MYRF) cause autosomal dominant and syndromic nanophthalmos in humans and retinal degeneration in mice. PLoS Genet 2019; 15:e1008130. [PMID: 31048900 PMCID: PMC6527243 DOI: 10.1371/journal.pgen.1008130] [Citation(s) in RCA: 53] [Impact Index Per Article: 8.8] [Reference Citation Analysis] [Abstract] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/11/2018] [Revised: 05/20/2019] [Accepted: 04/09/2019] [Indexed: 01/11/2023] Open
Abstract
Nanophthalmos is a rare, potentially devastating eye condition characterized by small eyes with relatively normal anatomy, a high hyperopic refractive error, and frequent association with angle closure glaucoma and vision loss. The condition constitutes the extreme of hyperopia or farsightedness, a common refractive error that is associated with strabismus and amblyopia in children. NNO1 was the first mapped nanophthalmos locus. We used combined pooled exome sequencing and strong linkage data in the large family used to map this locus to identify a canonical splice site alteration upstream of the last exon of the gene encoding myelin regulatory factor (MYRF c.3376-1G>A), a membrane bound transcription factor that undergoes autoproteolytic cleavage for nuclear localization. This variant produced a stable RNA transcript, leading to a frameshift mutation p.Gly1126Valfs*31 in the C-terminus of the protein. In addition, we identified an early truncating MYRF frameshift mutation, c.769dupC (p.S264QfsX74), in a patient with extreme axial hyperopia and syndromic features. Myrf conditional knockout mice (CKO) developed depigmentation of the retinal pigment epithelium (RPE) and retinal degeneration supporting a role of this gene in retinal and RPE development. Furthermore, we demonstrated the reduced expression of Tmem98, another known nanophthalmos gene, in Myrf CKO mice, and the physical interaction of MYRF with TMEM98. Our study establishes MYRF as a nanophthalmos gene and uncovers a new pathway for eye growth and development.
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Affiliation(s)
- Sarah J. Garnai
- Department of Ophthalmology and Visual Sciences, W.K. Kellogg Eye Center, University of Michigan, Ann Arbor, MI, United States of America
- Harvard Medical School, Boston, MA, United States of America
| | - Michelle L. Brinkmeier
- Department of Ophthalmology and Visual Sciences, W.K. Kellogg Eye Center, University of Michigan, Ann Arbor, MI, United States of America
- Department of Human Genetics, University of Michigan, Ann Arbor, MI, United States of America
| | - Ben Emery
- Jungers Center for Neurosciences Research, Department of Neurology, Oregon Health & Science University, Portland, OR, United States of America
| | - Tomas S. Aleman
- The Children’s Hospital of Philadelphia, Philadelphia, PA, United States of America
- Scheie Eye Institute, Department of Ophthalmology, Philadelphia, PA, United States of America
| | - Louise C. Pyle
- Division of Human Genetics, Children’s Hospital of Philadelphia, Philadelphia, PA, United States of America
| | - Biliana Veleva-Rotse
- Jungers Center for Neurosciences Research, Department of Neurology, Oregon Health & Science University, Portland, OR, United States of America
| | - Robert A. Sisk
- Cincinnati Eye Institute, Cincinnati, Ohio, United States of America
| | - Frank W. Rozsa
- Department of Ophthalmology and Visual Sciences, W.K. Kellogg Eye Center, University of Michigan, Ann Arbor, MI, United States of America
- Molecular and Behavior Neuroscience Institute, University of Michigan, Ann Arbor, MI, United States of America
| | - Ayse Bilge Ozel
- Department of Human Genetics, University of Michigan, Ann Arbor, MI, United States of America
| | - Jun Z. Li
- Department of Human Genetics, University of Michigan, Ann Arbor, MI, United States of America
| | - Sayoko E. Moroi
- Department of Ophthalmology and Visual Sciences, W.K. Kellogg Eye Center, University of Michigan, Ann Arbor, MI, United States of America
| | - Steven M. Archer
- Department of Ophthalmology and Visual Sciences, W.K. Kellogg Eye Center, University of Michigan, Ann Arbor, MI, United States of America
| | - Cheng-mao Lin
- Department of Ophthalmology and Visual Sciences, W.K. Kellogg Eye Center, University of Michigan, Ann Arbor, MI, United States of America
| | - Sarah Sheskey
- Department of Ophthalmology and Visual Sciences, W.K. Kellogg Eye Center, University of Michigan, Ann Arbor, MI, United States of America
| | - Laurel Wiinikka-Buesser
- Department of Ophthalmology and Visual Sciences, W.K. Kellogg Eye Center, University of Michigan, Ann Arbor, MI, United States of America
| | - James Eadie
- Department of Ophthalmology and Visual Sciences, W.K. Kellogg Eye Center, University of Michigan, Ann Arbor, MI, United States of America
| | - Jill E. Urquhart
- Manchester Centre for Genomic Medicine, Manchester Academic Health Sciences Centre, Manchester University NHS Foundation Trust, St Mary’s Hospital, Manchester, United Kingdom
- Division of Evolution and Genomic Sciences, Faculty of Biology, Medicine and Health, University of Manchester, Manchester, United Kingdom
| | - Graeme C.M. Black
- Manchester Centre for Genomic Medicine, Manchester Academic Health Sciences Centre, Manchester University NHS Foundation Trust, St Mary’s Hospital, Manchester, United Kingdom
- Division of Evolution and Genomic Sciences, Faculty of Biology, Medicine and Health, University of Manchester, Manchester, United Kingdom
| | - Mohammad I. Othman
- Department of Ophthalmology and Visual Sciences, W.K. Kellogg Eye Center, University of Michigan, Ann Arbor, MI, United States of America
| | - Michael Boehnke
- Department of Biostatistics and Center for Statistical Genetics, University of Michigan, Ann Arbor, MI, United States of America
| | - Scot A. Sullivan
- Dean McGee Eye Institute, Department of Ophthalmology, University of Oklahoma, Oklahoma City, OK
| | - Gregory L. Skuta
- Dean McGee Eye Institute, Department of Ophthalmology, University of Oklahoma, Oklahoma City, OK
| | - Hemant S. Pawar
- Department of Ophthalmology and Visual Sciences, W.K. Kellogg Eye Center, University of Michigan, Ann Arbor, MI, United States of America
| | - Alexander E. Katz
- Medical Genomics and Metabolic Genetics Branch, National Human Genome Research Institute, National Institutes of Health, Bethesda, MD, United States of America
| | - Laryssa A. Huryn
- Ophthalmic Genetics and Visual Function Branch, National Eye Institute, National Institutes of Health, Bethesda, MD, United States of America
| | - Robert B. Hufnagel
- Ophthalmic Genetics and Visual Function Branch, National Eye Institute, National Institutes of Health, Bethesda, MD, United States of America
| | | | - Sally A. Camper
- Department of Human Genetics, University of Michigan, Ann Arbor, MI, United States of America
| | - Julia E. Richards
- Department of Ophthalmology and Visual Sciences, W.K. Kellogg Eye Center, University of Michigan, Ann Arbor, MI, United States of America
- Department of Epidemiology, University of Michigan, Ann Arbor, MI, United States of America
| | - Lev Prasov
- Department of Ophthalmology and Visual Sciences, W.K. Kellogg Eye Center, University of Michigan, Ann Arbor, MI, United States of America
- Ophthalmic Genetics and Visual Function Branch, National Eye Institute, National Institutes of Health, Bethesda, MD, United States of America
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22
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Yeo L, Luewan S, Markush D, Gill N, Romero R. Prenatal Diagnosis of Dextrocardia with Complex Congenital Heart Disease Using Fetal Intelligent Navigation Echocardiography (FINE) and a Literature Review. Fetal Diagn Ther 2017. [PMID: 28641300 DOI: 10.1159/000468929] [Citation(s) in RCA: 17] [Impact Index Per Article: 2.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/17/2022]
Abstract
Fetal dextrocardia is a type of cardiac malposition where the major axis from base to apex points to the right side. This condition is usually associated with a wide spectrum of complex cardiac defects. As a result, dextrocardia is conceptually difficult to understand and diagnose on prenatal ultrasound. The advantage of four-dimensional sonography with spatiotemporal image correlation (STIC) is that this modality can facilitate fetal cardiac examination. A novel method known as fetal intelligent navigation echocardiography (FINE) allows automatic generation of nine standard fetal echocardiography views in normal hearts by applying intelligent navigation technology to STIC volume datasets. In fetuses with congenital heart disease, FINE is also able to demonstrate abnormal cardiac anatomy and relationships when there is normal cardiac axis and position. However, this technology has never been applied to cases of cardiac malposition. We report herein for the first time, a case of fetal dextrocardia and situs solitus with complex congenital heart disease in which the FINE method was invaluable in diagnosing multiple abnormalities and defining complex anatomic relationships. We also review the literature on prenatal sonographic diagnosis of dextrocardia (with an emphasis on situs solitus), as well as tricuspid atresia with its associated cardiac features.
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Affiliation(s)
- Lami Yeo
- Perinatology Research Branch, Program for Perinatal Research and Obstetrics, Division of Intramural Research, Eunice Kennedy Shriver National Institute of Child Health and Human Development, NIH, Bethesda, MD, and Detroit, MI, USA
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Ausejo Ramos T, Ramón Trapero JL, Cuerda Santaren D. Reconocimiento electrocardiográfico de una dextrocardia. Aten Primaria 2017; 49:120-121. [PMID: 27423245 PMCID: PMC6876026 DOI: 10.1016/j.aprim.2016.03.009] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/09/2016] [Accepted: 03/15/2016] [Indexed: 11/26/2022] Open
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Guo G, Yang L, Wu J, Sun L. Implantation of VVI pacemaker in a patient with dextrocardia, persistent left superior vena cava, and sick sinus syndrome: A case report. Medicine (Baltimore) 2017; 96:e6028. [PMID: 28151908 PMCID: PMC5293471 DOI: 10.1097/md.0000000000006028] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/26/2022] Open
Abstract
BACKGROUND Dextrocardia, or right-lying heart, is an uncommon congenital heart disease in which the apex of the heart is located on the right side of chest. Persistent left superior vena cava (PLSVA) is a rare venous anomaly that is often associated with the abnormalities of cardiac transduction system. A case with combination of dextrocardia, persistent left superior vena cava, and sick sinus syndrome has not been reported. METHODS We used different techniques including cardiac color Doppler echocardiography, 24-hour Holter monitoring, and abdominal ultrasound to make a diagnosis and treated the patient by implanting a VVI pacemaker. RESULTS A 50-year-old woman was admitted with a syncope. Angiography of the right atrium and superior vena cava, echocardiography, electrocardiography, and abdominal ultrasound revealed the presence of the combination of mirror image dextrocardia, PLSVA, and sick sinus syndrome. The complex structural anomalies presented great technical challenges for interventional treatments. After thorough examination and understanding of the structural anatomy and anomalies of the superior and inferior vena cava and cardiac chambers, we successfully treated this patient by implanting a VVI pacemaker. CONCLUSION Physicians must be aware of the complexity of the morphological and anatomical structures of dextrocardia accompanying PLSVC. Given that the diagnosis of situs inversus was performed at a relatively advanced age, it is therefore important to make such a correct diagnosis followed by appropriate therapeutic intervention.
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Affiliation(s)
- Gongliang Guo
- Department of Cardiology, China-Japan Union Hospital, Jilin University
| | - Lili Yang
- Department of Gynecology and Obstetrics
| | - Jinyi Wu
- Department of Cardiology, China-Japan Union Hospital, Jilin University
| | - Liqun Sun
- Department of Pediatric, the First Hospital of Jilin University, Changchun, Jilin Province, china
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Fritschka M, Schlegl M, Borges A, Werner M, Gebker R, Pieske B, Kelle S. Unusual case of ATTR amyloidosis with cardiac manifestation and situs inversus totalis. Clin Res Cardiol 2017; 106:311-316. [PMID: 28116497 PMCID: PMC5360841 DOI: 10.1007/s00392-016-1067-9] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/27/2016] [Accepted: 12/16/2016] [Indexed: 01/15/2023]
Affiliation(s)
- Max Fritschka
- Klinik für Innere Medizin und Kardiologie, Deutsches Herzzentrum Berlin, Augustenburger Platz 1, 13353, Berlin, Germany. .,DZHK (German Centre for Cardiovascular Research), Partner Site Berlin, 652133, Berlin, 13357, Germany.
| | - Michael Schlegl
- Praxis Westend, Cardiology Outpatient Clinic, Spandauer Damm 130, 14050, Berlin, Germany
| | - Adrian Borges
- HELIOS Klinikum Emil von Behring, Abteilung für Innere Medizin I / Kardiologie, Walterhöferstraße 11, Berlin, 14165, Germany
| | - Mathias Werner
- HELIOS Klinikum Emil von Behring, Institut für Pathologie, Walterhöferstraße 11, Berlin, 14165, Germany
| | - Rolf Gebker
- Klinik für Innere Medizin und Kardiologie, Deutsches Herzzentrum Berlin, Augustenburger Platz 1, 13353, Berlin, Germany.,DZHK (German Centre for Cardiovascular Research), Partner Site Berlin, 652133, Berlin, 13357, Germany
| | - Burkert Pieske
- Klinik für Innere Medizin mit Schwerpunkt Kardiologie, Campus Virchow-Klinikum, Charité-Universitätsmedizin Berlin, Augustenburger Platz 1, Berlin, 13353, Germany.,Klinik für Innere Medizin und Kardiologie, Deutsches Herzzentrum Berlin, Augustenburger Platz 1, 13353, Berlin, Germany.,DZHK (German Centre for Cardiovascular Research), Partner Site Berlin, 652133, Berlin, 13357, Germany
| | - Sebastian Kelle
- Klinik für Innere Medizin und Kardiologie, Deutsches Herzzentrum Berlin, Augustenburger Platz 1, 13353, Berlin, Germany.,DZHK (German Centre for Cardiovascular Research), Partner Site Berlin, 652133, Berlin, 13357, Germany
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26
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Sequential segmental classification of feline congenital heart disease. J Vet Cardiol 2016; 17 Suppl 1:S10-52. [PMID: 26776571 DOI: 10.1016/j.jvc.2015.04.005] [Citation(s) in RCA: 26] [Impact Index Per Article: 2.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/31/2014] [Revised: 04/01/2015] [Accepted: 04/21/2015] [Indexed: 12/17/2022]
Abstract
Feline congenital heart disease is less commonly encountered in veterinary medicine than acquired feline heart diseases such as cardiomyopathy. Understanding the wide spectrum of congenital cardiovascular disease demands a familiarity with a variety of lesions, occurring both in isolation and in combination, along with an appreciation of complex nomenclature and variable classification schemes. This review begins with an overview of congenital heart disease in the cat, including proposed etiologies and prevalence, examination approaches, and principles of therapy. Specific congenital defects are presented and organized by a sequential segmental classification with respect to their morphologic lesions. Highlights of diagnosis, treatment options, and prognosis are offered. It is hoped that this review will provide a framework for approaching congenital heart disease in the cat, and more broadly in other animal species based on the sequential segmental approach, which represents an adaptation of the common methodology used in children and adults with congenital heart disease.
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27
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Franco A, Jo SY, Mehta AS, Pandya DJ, Yang CW. A Rare Triad of Giant Occipital Encephalocele with Lipomyelomeningocele, Tetralogy of Fallot, and Situs Inversus. J Radiol Case Rep 2016; 10:36-46. [PMID: 27200165 DOI: 10.3941/jrcr.v10i3.2718] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/23/2022] Open
Abstract
Giant encephalocele is an uncommon congenital anomaly with very few published reports available in the English literature. Tetralogy of Fallot associated with situs inversus is also infrequently reported. To our knowledge there are no published reports of an association between giant encephalocele and Tetralogy of Fallot. The additional finding of situs inversus results in a rare pathologic triad, not heretofore described.
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Affiliation(s)
- Arie Franco
- Department of Radiology, The University of Chicago Medical Center, Chicago USA
| | - Stephanie Y Jo
- Department of Radiology, The University of Chicago Medical Center, Chicago USA
| | - Amar S Mehta
- Department of Radiology, The University of Chicago Medical Center, Chicago USA
| | - Dave J Pandya
- Department of Cardiology, The University of Chicago Medical Center, Chicago USA
| | - Carina W Yang
- Department of Radiology, The University of Chicago Medical Center, Chicago USA
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28
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Evans WN, Acherman RJ, Restrepo H. Heterotaxy in southern Nevada: prenatal detection and epidemiology. Pediatr Cardiol 2015; 36:930-4. [PMID: 25586256 DOI: 10.1007/s00246-015-1099-3] [Citation(s) in RCA: 23] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/04/2014] [Accepted: 01/07/2015] [Indexed: 11/30/2022]
Abstract
We retrospectively analyzed a combination of prenatally detected and postnatally diagnosed patients with heterotaxic situs that included those with situs inversus, bilateral left-sidedness, and bilateral right-sidedness for the period between April 2002 and July 2014. We found a statistically higher prevalence in the Hispanic population of Southern Nevada of 2.7/10,000 live births (95 % confidence intervals of 1.7-3.9) versus the non-Hispanic population of 1.6/10,000 live births (95 % confidence intervals of 1.1-2.1), p = 0.04. Additionally, we noted a high prenatal detection rate of 68 % over the 12-year period of time, rising to 100 % over the last 2 years.
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Affiliation(s)
- William N Evans
- Children's Heart Center - Nevada, 3006 S. Maryland Pkwy Ste. 690, Las Vegas, NV, 89109, USA,
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Poh CL, Xu M, Galati JC, Iyengar AJ, Cheung M, Brizard CP, Konstantinov IE, d'Udekem Y. Surgical palliation in patients with a single ventricle and dextrocardia. J Thorac Cardiovasc Surg 2014; 148:1475-80. [DOI: 10.1016/j.jtcvs.2013.10.077] [Citation(s) in RCA: 11] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/21/2012] [Revised: 09/17/2013] [Accepted: 10/22/2013] [Indexed: 11/17/2022]
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30
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Szczechowicz M, Januszewska K, Jux C, Malec E. Hypoplastic left heart syndrome with dextrocardia and situs solitus. World J Pediatr Congenit Heart Surg 2014; 5:323-5. [PMID: 24668985 DOI: 10.1177/2150135113515277] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/16/2022]
Abstract
Hypoplastic left heart syndrome with dextrocardia and situs solitus is a very rare condition. We describe a 3.5-year-old male child with this constellation who underwent multistage repair and a successful extracardiac Fontan operation in our hospital.
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Pinheiro V, Pereira L, Lima A, Branco E. Dextrocardia with situs solitus and inversion apex-basis axis in lesser anteater (Tamandua tetradactyla): case report. ARQ BRAS MED VET ZOO 2014. [DOI: 10.1590/s0102-09352014000100017] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/22/2022] Open
Abstract
Dextrocardia is a rare cardiac anomaly where the heart is situated on the right antimeres of the thorax. This study had the objective of describing a case of dextrocardia with situs solitus and apex-basis axis inversion in a lesser anteater (Tamandua tetradactyla) between five evaluated animals, all from the area of Mine Bauxite - Paragominas - Para. The arterial system was filled with contrasted latex and the animals were fixed with 10% formaldehyde and a posterior dissection was done. The heart of an animal was found in right antimere with inversion of the base-apex axis. The right atrium was more developed then the left and the pulmonary veins arrived directly in the left ventricle. The main vases of the base were identified with some topographic alterations resulting in: aorta dorsal to the cava caudal vein, pulmonary artery dorsal and cranial to aorta, pulmonary veins ventral to the pulmonary artery, cava caudal vein in ventral plain and cava cranial vein in dorsal plan in relation to the other vessels. Internally there were four cardiac chambers, with absence of septal communication.
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Affiliation(s)
| | | | - A.R. Lima
- Universidade Federal Rural da Amazônia
| | - E. Branco
- Universidade Federal Rural da Amazônia
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Kashyap R, Abrar ML, Bhattacharya A, Rohit MK, Mittal BR. Myocardial perfusion scintigraphy in a case of dextrocardia: Doing it "right". Indian J Nucl Med 2013; 27:252-3. [PMID: 24019657 PMCID: PMC3759088 DOI: 10.4103/0972-3919.115398] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/25/2022] Open
Abstract
We present the challenges in performing the stress test and acquisition of images in myocardial scintigraphy in a 54-year-old female patient with dextrocardia. Dextrocardia and situs inversus were documented on prior investigations including a chest roentgenogram and sonography.
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Affiliation(s)
- Raghava Kashyap
- Department of Nuclear Medicine, Post Graduate Institute of Medical Education and Research, Chandigarh, India
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Altın H, Alp H, Şap F, Karataş Z, Baysal T, Karaaslan S. Double Aortic Arch with Mirror-Image Dextrocardia. ELECTRONIC JOURNAL OF GENERAL MEDICINE 2013. [DOI: 10.29333/ejgm/82314] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/21/2022]
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Furtado AD, Chikkatur R, Peer SM, Bhat S. A technique for repair of atrioventricular canal defect in dextrocardia. Eur J Cardiothorac Surg 2012; 42:173-4. [PMID: 22328627 DOI: 10.1093/ejcts/ezs019] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/14/2022] Open
Abstract
Congenital heart disease with dextrocardia, situs solitus, is not uncommonly seen in paediatric cardiac surgery. An approach through the right atrium for correction of associated cardiac anomalies is needed in most of these cases. We present a technique for operating on this subset of patients wherein the heart can be displaced into the left pleural space allowing for surgery in an anatomical orientation that is familiar to the surgeon and emphasize the precautions to be taken following surgery.
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Affiliation(s)
- Arul Dominic Furtado
- Department of Cardio-Thoracic Surgery, Sri Jayadeva Institute of Cardiovascular Sciences and Research, Bangalore, India.
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