Apical hypertrophic cardiomyopathy (AHCM) is a subtype of hypertrophic cardiomyopathy (HCM). The expression level of high-sensitive cardiac troponin T (hs-cTNT) and N-terminal pro-BNP (NT-proBNP) in AHCM patients, and these relationships between echocardiography parameters were still unclear.

We retrospectively screened AHCM patients between January 2019 and December 2021 in Zhongshan Hospital Fudan University. The relationship between the level of hs-cTNT, NT-proBNP and echocardiography parameters were analyzed. The risk factors for elevated hs-cTNT and NT-proBNP level were investigated with linear regression analysis.

A total of 267 AHCM patients were enrolled. They were divided into hs-cTNT normal (129, 48.3%) and abnormal (138, 51.7%) group. Compared with hs-cTNT normal group, hs-cTNT abnormal group were elder (68.3 ± 11.6 vs. 63.8 ± 10.6, P = 0.001); with higher rate of atrial fibrillation (AF) (41.3% vs. 17.8%, P < 0.001) and higher level of NT-proBNP concentration (752.0 [343.8-1345.5] vs. 249.0 [104.0-541.0], P < 0.001). For echocardiography parameters, hs-cTNT abnormal patients have thicker interventricular septum (IVS) (11.6 ± 2.0 vs. 11.0 ± 1.7, P = 0.02), thicker left ventricular apical (LVA) (16.9 ± 3.0 vs. 14.9 ± 2.3, P < 0.001) and larger left atrium diameter (LAD) (45.9 ± 6.6 vs. 42.4 ± 5.1, P < 0.001). LVA was independently correlated with both the level of hs-cTNT and NT-proBNP (hs-cTNT r = 0.224, P = 0.143; NT-proBNP r = 0.370, P < 0.001). Linear regression analysis revealed that LVA was independent risk factor of both the elevated hs-cTNT and NT-proBNP level.

More than half of AHCM patients had abnormal hs-cTNT level. LVA was positively and independently correlated with the level of hs-cTNT and NT-proBNP.

Despite the progress made in risk stratification, sudden cardiac death and heart failure remain dreaded complications for hypertrophic cardiomyopathy (HCM) patients. Myocardial ischaemia is widely acknowledged as a contributor to cardiovascular events, but the assessment of ischaemia is not yet included in HCM clinical guidelines. This review aims to evaluate the HCM-specific pro-ischaemic mechanisms and the potential prognostic value of imaging for myocardial ischaemia in HCM. A literature review was performed using PubMed to identify studies with non-invasive imaging of ischaemia (cardiovascular magnetic resonance, echocardiography, and nuclear imaging) in HCM, prioritising studies published after the last major review in 2009. Other studies, including invasive ischaemia assessment and post-mortem histology, were also considered for mechanistic or prognostic relevance. Pro-ischaemic mechanisms in HCM reviewed included the effects of sarcomeric mutations, microvascular remodelling, hypertrophy, extravascular compressive forces and left ventricular outflow tract obstruction. The relationship between ischaemia and fibrosis was re-appraised by considering segment-wise analyses in multimodal imaging studies. The prognostic significance of myocardial ischaemia in HCM was evaluated using longitudinal studies with composite endpoints, and reports of ischaemia-arrhythmia associations were further considered. The high prevalence of ischaemia in HCM is explained by several micro- and macrostructural pathological features, alongside mutation-associated energetic impairment. Ischaemia on imaging identifies a subgroup of HCM patients at higher risk of adverse cardiovascular outcomes. Ischaemic HCM phenotypes are a high-risk subgroup associated with more advanced left ventricular remodelling, but further studies are required to evaluate the independent prognostic value of non-invasive imaging for ischaemia.

We sought to recognize the blood flow velocity (BFV) through the left anterior descending (LAD) coronary artery and its small intramyocardial (IM) branches by transthoracic Doppler-echocardiography in patients with aortic stenosis (AS). Sixty-two patients, aged 74.0 ± 9.6 years, 37 women, with preserved left ventricular (LV) function, apparently free of active ischemic disease, were enrolled and classified into 3 groups according to the mean gradient (MG) across the aortic valve: 13 patients (21%) entered the group A (MG ≤ 20 mmHg), 29 (48%) group B (MG 21-40 mmHg) and 20 (31%) group C (MG > 40 mmHg). Peak and mean coronary BFVs were demonstrated to gradually increase according to AV gradient, especially through the IM arteries. Peak IM-BFV was 58.9 cm/s (95% CI 46.4-71.4) in group A, 73.2 cm/s (95% CI 64.8-81.6) in group B, and 96.4 cm/s (95% CI 86.3-106.5) in group C (p < 0.001), whereas peak LAD-BFV was 38.1 cm/s (95% CI 32.8-43.3), 44.4 cm/s (95% CI 40.9-47.9) and 47.3 cm/s (95% CI 43.1-52.5), respectively (p = 0.03). Also, 34 patients complaining with unspecific symptoms showed much higher IM-BFV than those who were not. High values were also recognized in patients with LV ejection fraction/velocity ratio (EFVR) ≤ 0.90 (IM-BFV 91 ± 26 cm/s vs. 72 ± 24 cm/s in those with EFVR > 0.90, p = 0.001). In conclusion, AS patients in the present study showed gradually higher coronary BFVs according to AS gradient, especially through the IM vessels, and both peak and mean velocities were discriminating specific patient subsets. Pathophysiological mechanisms and potential clinical implications are discussed.

Apical hypertrophic cardiomyopathy (AHCM) is a relatively rare morphologic variant of HCM in which the hypertrophy of myocardium is localized to the left ventricular apex. Symptoms of AHCM might vary from none to others mimic coronary artery disease including acute coronary syndrome, thus resulting in inappropriate hospitalization. Transthoracic echocardiography is the first-line imaging technique for the diagnosis of hypertrophic cardiomyopathies. However, when the hypertrophy of the myocardium is localized in the ventricular apex might results in missed diagnosis. Aim of this paper is to review the different imaging techniques used for the diagnosis of AHCM and their role in the detection and comprehension of this uncommon disease.

Hypertrophic cardiomyopathy is a hereditary, not uncommon cardiac disease, which is associated with asymmetric thickening and hypertrophy of the interventricular septum unrelated to hemodynamic reasons. Despite hypertrophic cardiomyopathy is considered to be a disorder of the heart muscle, several associated vascular alterations have been described. The aim of the present review is to summarize vascular functional alterations in hypertrophic cardiomyopathy.

The aim of the present study was to analyze the parameters related to baseline coronary flow velocity (CFV) and coronary flow reserve (CFR) using Doppler transthoracic echocardiography (TTE), and to assess their associations with components of the Framingham risk score (FRS), which estimates 10-year risk of coronary heart disease, in subjects with chest pain and a normal coronary angiogram.

A total of 354 individuals (mean age: 55+/-11 years, M:F ratio =186:168) with angina or angina-like chest pain and a normal coronary arteriogram were enrolled. CFR, using TTE and adenosine or dipyridamole, was measured within 2 weeks after coronary angiogram. The clinical, electrocardiographic, echocardiographic and laboratory parameters related to baseline CVF and CFR were analyzed, and CFR was compared with FRS. There was an inverse correlation between baseline CFV and CFR (r=-0.374, P<0.001). On multivariate analysis the fulfilling of left ventricular hypertrophy criteria on electrocardiography was an independent predictor of baseline CFV for the upper 75% quartile (23.2> or =cm/s; odds ratio (OR) = 2.840, 95% confidence interval (CI) =1.155-6.983, P=0.023). On multivariate analysis hemoglobin A(1c) level was independently related to a CFR <2.0 (OR = 2.195, 95%CI = 0.920-1.005, P=0.013). CFR had an inverse correlation with FRS (r=-0.222, P<0.001). On multiple regression analysis among the components of the FRS system (FRSS), independent factors related to a CFR <2.0 included age (OR =1.033, 95%CI =1.000-1.067, P=0.041), high-density lipoprotein-cholesterol level (OR = 0.961, 95%CI = 0.933-0.991, P=0.012) and smoking status (OR = 2.461, 95%CI =1.078-5.618, P=0.033), respectively.

CFR can be a comprehensive indicator of cardiovascular risk factors, including parameters of the FRSS, in subjects with chest pain and a normal coronary angiogram.

Reduction in coronary flow velocity reserve (CFR) is a recognized feature in hypertrophic cardiomyopathy (HCM). We sought to assess the long-term prognostic value of CFR by pulsed-wave Doppler transesophageal echocardiography (TEE) in HCM patients. The study comprised 20 patients with typical features of HCM. The patients were enrolled in 1999. All patients underwent a standard transthoracic echo-Doppler study to evaluate left ventricular function and a stress vasodilator TEE study to evaluate CFR. The success rate of follow-up was 18 out of 20 (90%). During a mean follow-up of 90 +/- 24 months, four patients suffered cardiovascular death (2 sudden cardiac deaths and 2 strokes). The other seven patients underwent invasive procedures (coronary angiography, implantable cardioverter defibrillator implantation, percutaneous transluminal septal myocardial ablation) or showed cerebrovascular events. Using receiver operator characteristic analysis, CFR < 2.35 was a significant predictor for cardiovascular event-free survival (sensitivity 91%, specificity 71%, area under the curve 74%, P = 0.05). Multivariable regression analysis showed that only CFR (hazard ratio (HR) 4.21, P < 0.05) was an independent predictor of cardiovascular event-free survival. Despite the relatively small number of patients involved in the study, results could suggest that CFR should be considered as an independent predictor for future cardiovascular events in HCM patients. However, further studies with larger HCM patient populations are warranted.

16-slice multidetector CT (MDCT) cardiac findings of a middle-aged man with known apical hypertrophic cardiomyopathy (AHC) and recent atypical chest pain are presented. MDCT enabled comprehensive evaluation of the coronary arteries, diagnosing myocardial bridging of the left anterior descending (LAD) and first diagonal arteries. It also enabled dynamic evaluation of myocardial thickness and left ventricular global and regional function. This case illustrates the full capabilities of MDCT in the evaluation of AHC.

The purpose of this study was to compare coronary flow reserve (CFR) capacity of penetrating intramyocardial coronary artery (PICA) using transthoracic Doppler echocardiography and biochemical marker of myocardial fibrosis in hypertension (HTN).

In 58 patients (male:female ratio = 31:27; mean age 47 +/- 9 years) with chest pain and normal coronary angiogram findings, the width of color Doppler signal and peak diastolic velocity of PICA flow were measured in the myocardium just beneath the apical impulse window using transthoracic Doppler echocardiography. PICA-CFR and PICA-width ratio were calculated as the ratio of hyperemic to baseline peak diastolic velocity and as the ratio of hyperemic to baseline width after the adenosine infusion (140 mug/kg/min), respectively. Serum carboxy-terminal propeptide of procollagen type I, as a biochemical marker, was measured and patients were divided into 3 groups: 19 with HTN and PICA-CFR less than 2.0 (group A); 23 with HTN and PICA-CFR of 2.0 or more (group B); and 16 who were normotensive with PICA-CFR of 2.0 or more (group C).

Baseline peak diastolic velocity for group A was higher than the other two groups (P < .005 vs groups B and C). PICA-width ratio was higher than the other two groups (P < .005 vs groups B and C). Serum propeptide of type I was 137.1 +/- 16.6 ng/mL in group A, 96.2 +/- 13.7 ng/mL in group B, and 78.8 +/- 11.2 ng/mL in group C (P < .0001 vs group B and group C). PICA-CFR was closely related to serum propeptide of type I (P < .001, r = -0.723).

The impaired PICA-CFR is related to myocardial fibrosis in patients with HTN, chest pain, and normal coronary angiogram results.