Subaortic and midventricular obstructive hypertrophic cardiomyopathy with extreme segmental hypertrophy

BACKGROUND: Subaortic and midventricular hypertrophic cardiomyopathy in a patient with extreme segmental hypertrophy exceeding the usual maximum wall thickness reported in the literature is a rare phenomenon. CASE PRESENTATION: A 19-year-old man with recently diagnosed hypertrophic cardiomyopathy (HCM) was referred for sudden death risk assessment. The patient had mild exertional dyspnea (New York Heart Association functional class II), but without syncope or chest pain. There was no family history of HCM or sudden death. A two dimensional echocardiogram revealed an asymmetric type of LV hypertrophy; anterior ventricular septum = 49 mm; posterior ventricular septum = 20 mm; anterolateral free wall = 12 mm; and posterior free wall = 6 mm. The patient had 2 types of obstruction; a LV outflow obstruction due to systolic anterior motion of both mitral leaflets (Doppler-estimated 38 mm Hg gradient at rest); and a midventricular obstruction (Doppler-estimated 43 mm Hg gradient), but without apical aneurysm or dyskinesia. The patient had a normal blood pressure response on exercise test and no episodes of non-sustained ventricular tachycardia in 24-h ECG recording. Cardiac MRI showed a gross late enhancement at the hypertrophied septum. Based on the extreme degree of LV hypertrophy and the myocardial hyperenhancement, an implantation of a cardioverter-defibrillator was recommended prophylactically for primary prevention of sudden death. CONCLUSION: Midventricular HCM is an infrequent phenotype, but may be associated with an apical aneurysm and progression to systolic dysfunction (end-stage HCM)

Risk factors for sudden cardiac death in hypertrophic cardiomyopathy

Aim of this study was the evaluation of six non invasive clinical indices as risk factors for sudden death (SD)in hypertrophic cardiomyopathy (HCM). Previous syncope, family history of SD, non sustained ventricular tachycardia, abnormalblood pressure response during exercise, excessive hypertrophy ≥3 cm and left ventricular outflow tract obstructionwith a peak gradient ≥30 mmHg were evaluated in a cohort of 166 patients(112 males, 51.8 ± 15.6 years), followed up for amedian of 32.4 months (range 1 to 209 months). During follow up 13 patients reached study’s endpoints: SD, cardiac arrest,documented sustained ventricular tachycardia and/or Implantable Cardioverter Defibrillator (ICD)-discharge. Patients havingexperienced syncope or presenting with a Maximum Wall Thickness ≥3cm in echocardiography were more sensitive to SDemergence since they had a 13.07 (95%CI: 4.00-46.95, p < 0.0001) and a 10.07 (95%CI: 2.92-34.79, p = 0.003) greater relativerisk respectively. In our cohort of patients only two of the six ‘recognised’ potential risk factors for SD were found sensitive,a result causing scepticism about the validity of criteria used for ICD implantation in HCM patients for SD prevention

Identification of high risk patients with hypertrophic cardiomyopathy in a northern Greek population

This is an Open Access article distributed under the terms of the Creative Commons Attribution Licens

Hypertrophic cardiomyopathy with midventricular obstruction and apical aneurysm formation in a single family: case report

<p>Abstract</p> <p>Background</p> <p>Hypertrophic cardiomyopathy (HCM) is an extremely heterogeneous disease. An under recognized and very often missed subgroup within this broad spectrum concerns patients with left ventricular (LV) apical aneurysms in the absence of coronary artery disease.</p> <p>Case presentation</p> <p>We describe a case of HCM with midventricular obstruction and apical aneurysm formation in 3 patients coming from a single family. This HCM pattern was detected by 2D-echocardiography and confirmed by cardiac magnetic resonance imaging. A cardioverter defibrillator was implanted in one of the patients because of non-sustained ventricular tachycardia detected in 24-h Holter monitoring and an abrupt drop in systolic blood pressure during maximal exercise test. The defibrillator activated 8 months after implantation by suppression of a ventricular tachycardia providing anti-tachycardia pacing. The patient died due to refractory heart failure 2 years after initial evaluation. The rest of the patients are stable after a 2.5-y follow-up period.</p> <p>Conclusion</p> <p>The detection of apical aneurysm by echocardiography in HCM patients may be complicated. Ventricular tachycardia arising from the scarred aneurysm wall may often occur predisposing to sudden death.</p