Observational Study
Copyright ©The Author(s) 2016. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Pediatr. Aug 8, 2016; 5(3): 319-324
Published online Aug 8, 2016. doi: 10.5409/wjcp.v5.i3.319
Single institution experience with the Ladd’s procedure in patients with heterotaxy and stage I palliated single-ventricle
Kurt D Piggott, Grace George, Harun Fakioglu, Carlos Blanco, Sukumar Saguna Narasimhulu, Kamal Pourmoghadam, Hamish Munroe, William Decampli
Kurt D Piggott, Grace George, Harun Fakioglu, Carlos Blanco, Sukumar Saguna Narasimhulu, Kamal Pourmoghadam, Hamish Munroe, William Decampli, the Heart Center at Arnold Palmer Hospital for Children, Pediatric Cardiac Intensive Care Medicine, Orlando, FL 32806, United States
Author contributions: Piggott KD, George G, Fakioglu H, Blanco C, Pourmoghadam K, Munroe H, Decampli W contributed to study conception and design; Piggott KD and George G contributed to data analysis and interpretation and writing of article; Narasimhulu SS, Munroe H and Decampli W contributed to editing reviewing and final approval of article.
Institutional review board statement: This study was approved by the Arnold Palmer Medical Center institutional review board.
Informed consent statement: We obtained IRB approval including waiver of informed consent prior to commencing this paper.
Conflict-of-interest statement: All authors have no conflict of interest to report.
Data sharing statement: All authors consent to sharing and downloading of all data present in this paper.
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Correspondence to: Kurt D Piggott, MD, the Heart Center at Arnold Palmer Hospital for Children, Pediatric Cardiac Intensive Care Medicine, 92 W. Miller Street, MP307, Orlando, FL 32806, United States. kurt.piggott@orlandohealth.com
Telephone: +1-407-7662948 Fax: +1-321-8414260
Received: January 20, 2016
Peer-review started: January 20, 2016
First decision: March 24, 2016
Revised: April 11, 2016
Accepted: June 1, 2016
Article in press: June 3, 2016
Published online: August 8, 2016

AIM: To investigate and describe our current institutional management protocol for single-ventricle patients who must undergo a Ladd’s procedure.

METHODS: We retrospectively reviewed the charts of all patients from January 2005 to March 2014 who were diagnosed with heterotaxy syndrome and an associated intestinal rotation anomaly who carried a cardiac diagnosis of functional single ventricle and were status post stage I palliation. A total of 8 patients with a history of stage I single-ventricle palliation underwent Ladd’s procedure during this time period. We reviewed each patients chart to determine if significant intraoperative or post-operative morbidity or mortality occurred. We also described our protocolized management of these patients in the cardiac intensive care unit, which included pre-operative labs, echocardiography, milrinone infusion, as well as protocolized fluid administration and anticoagulation regimines. We also reviewed the literature to determine the reported morbidity and mortality associated with the Ladd’s procedure in this particular cardiac physiology and if other institutions have reported protocolized care of these patients.

RESULTS: A total of 8 patients were identified to have heterotaxy with an intestinal rotation anomaly and single-ventricle heart disease that was status post single ventricle palliation. Six of these patients were palliated with a Blaylock-Taussig shunt, one of whom underwent a Norwood procedure. The two other patients were palliated with a stent, which was placed in the ductus arteriosus. These eight patients all underwent elective Ladd’s procedure at the time of gastrostomy tube placement. Per our protocol, all patients remained on aspirin prior to surgery and had no period where they were without anticoagulation. All patients remained on milrinone during and after the procedure and received fluid administration upon arrival to the cardiac intensive care unit to account for losses. All 8 patients experienced no intraoperative or post-operative complications. All patients survived to discharge. One patient presented to the emergency room two months after discharge in cardiac arrest and died due to bowel obstruction and perforation.

CONCLUSION: Protocolized intensive care management may have contributed to favorable outcomes following Ladd’s procedure at our institution.

Keywords: Congenital heart disease, Heterotaxy, Single-ventricle, Pediatrics, Ladd’s procedure, Congenital heart disease

Core tip: Babies born with congenital heart disease consisting of a functional single-ventricle present a complicated subset of patients to care for. When these patients also have heterotaxy and an intestinal rotational anomaly combined with their heart defect, determining when and how to safely perform a Ladd’s procedure is challenging for clinicians involved in their care. Having experienced practitioners involved and using protocolized care may help reduce surgical morbidity and mortality in these patients.