Structural abnormalities in hypertrophic cardiomyopathy beyond left ventricular hypertrophy by multimodality imaging evaluation.

BACKGROUND: Echocardiography- and cardiovascular magnetic resonance (CMR)-based studies have revealed a wide range of phenotypic manifestations in hypertrophic cardiomyopathy (HCM) apart from hypertrophy. This study sought to comprehensively describe a number of structural abnormalities in HCM beyond hypertrophy, by multimodality imaging. METHODS: A total of 100 HCM patients were prospectively enrolled, undergoing standard and contrast echocardiography, and CMR. Morphological characteristics involving mitral valve leaflets (MVL), subvalvular apparatus, and left ventricular cavity and wall were investigated. Seventy healthy volunteers served as control population. RESULTS: As assessed by echo, MVLs were longer in HCM patients than in controls (anterior method 1: 24[22,28] vs 19[18,20] mm, P < 0.01; anterior method 2: 27[24, 29] vs 21[19, 23] mm, P < 0.01; posterior: 15[12,19] vs 14[13,15] mm, P < 0.01). Abnormal chordal attachment to anterior MVL, anterior papillary muscle displacement, and accessory apical-basal muscle bundle were present in 42 (42%), 61 (61%), and 35 (35%) patients, respectively (P values vs controls <0.01); direct papillary muscle insertion into MVL and hypertrabeculation were found in two and five patients, respectively. Contrast echocardiography (n = 94) detected myocardial crypts in 15 patients (16%). Overall, 83% of HCM subjects had at least one of these phenotypic manifestations. Echocardiography and CMR agreement for MVL length was poor, while for structural characteristics was moderate to substantial (Cohen's Kappa: 0.53-1.00). Except for posterior MVL length and hypertrabeculation, the phenotypic characteristics studied had acceptable reproducibility by echocardiography and CMR. CONCLUSIONS: Structural abnormalities in HCM beyond hypertrophy are significantly common. Multimodality imaging approach to these HCM facets by echocardiography and CMR is feasible and desirable.

Clinical and echocardiographic course in tako-tsubo cardiomyopathy: Long-term follow-up from a multicenter study.

OBJECTIVES: To jointly describe clinical characteristics, ECG and echocardiographic findings, and adverse cardiovascular events in patients with tako-tsubo cardiomyopathy (TC) in the long-term. METHODS: Longitudinal multicenter study including retrospective analysis of clinical and ECG data, and follow-up evaluation with clinical interview, electrocardiogram and echocardiogram. RESULTS: Data from 66 cases of TC were available for analysis of clinical and adverse cardiovascular events, and 56 of them completed the follow-up visit including electrocardiogram and echocardiogram. Most patients (97%) were asymptomatic or oligosymptomatic (NYHA I [58%] or II [39%], respectively) at follow-up (median time: 3.7 [1.8-6.6] years). The vast majority of individual QRS complex and repolarization abnormalities had disappeared (87% with no ECG abnormalities at follow-up). On echocardiography, left ventricular ejection fraction was ≥50% in all patients (mean: 63±6%). Wall motion abnormalities were observed in 4 patients (7%; 3 with apical wall motion abnormalities and 1 with mild global hypokinesia). Long-term outcomes were as follows: 4 deaths (6%), 2 cardiovascular and 2 non-cardiovascular; no atrial fibrillation development; no stroke events; 5 acute recurrence events of TC (8%). Globally, 57 patients (86%) had a clinical course free from adverse cardiovascular events. CONCLUSIONS: After a long period following the admission event, patients discharged from TC remain asymptomatic or minimally symptomatic, and feature a low prevalence of both ECG and left ventricular wall motion abnormalities; moreover, the latter lead to a very mild impairment of ejection fraction. Among cardiovascular adverse events, recurrence of the TC event appears to play the most significant role.