A 22-year-old man was admitted with chief complaint of dyspnea on exertion. The patient had a history of operation 10 years ago for coarctation of the aorta. Transthoracic echocardiography showed bicuspid aortic valve, aortic regurgitation, left ventricular (LV) enlargement with ejection fraction of 45%, and a deeply trabeculated LV with deep intertrabecular recesses communicating with left ventricular cavity as demonstrated by color Doppler flow, the characteristic of the left ventricular noncompaction. Medical therapy and aortic valve replacement was performed. In the follow up, he was free of symptoms 12 months later.

A 36-year-old man, who had a history of aortic valve replacement 8 years previously because of severe aortic stenosis and bicuspid aortic valve, presented to the emergency department with a hemodynamically unstable ventricular tachycardia. Echocardiography showed an asymmetrical left ventricular hypertrophy and a normal functioning prosthetic valve with a negligible transvalvular gradient and no evidence of patient-prosthetic mismatch. Cardiac magnetic resonance imaging revealed left ventricular hypertrophy with an apical aneurysm, diffuse late gadolinium enhancement, and a midcavitary obstruction, all in favor of hypertrophic cardiomyopathy. Ventricular tachycardia ablation was done via the trans-septal approach, and an implantable cardioverter-defibrillator was inserted.

Background: The aim of the present study was to investigate the effects of the left ventricular outflow gradient on tissue doppler imaging (TDI) values in patients with hypertrophic cardiomyopathy (HCM).Methods: This prospective observational study was conducted in a tertiary referral heart hospital (Tabriz, Iran) from March 2004 to March 2008. Fifty two patients (28 men and 24 women) with definite diagnoses of HCM were selected for the study. 2D, M-mode echocardiography and TDI were performed for all patients. In TDI, the velocities of myocardium was measured at lateral and septal corner of the mitral valve annulus in peak early diastolic (Ea), peak late diastolic (Aa) and peak systolic (Sa). Then, the TDI velocities were compared in patients with left ventricle outflow gradient less and more than 30 mmHg.Results: The mean age of the patients was 42.13 ± 12.22 years. All patients were in New York Heart Association functional class I or II. The mean EF was 55 ± 7%. It was found that TDI velocities are reduced in patients with HCM, compared to normal vaues and Ea in septal corners of the mitral annulus was reduced more in patients with left ventricle outflow gradient > 30 mmHg (5.3±1.6 vs 7.1±1.2, P=0.01).Conclusion: TDI findings specially peak early diastolic (Ea) mitral annular velocity are good parameters in HCM patients assessment.

Left ventricular non-compaction cardiomyopathy is a rare congenital cardiomyopathy that affects both children and adults.Since the clinical manifestations are not sufficient to establish diagnosis, echocardiography is the diagnostic tool that makes it possible to document ventricular non-compaction and establish prognostic factors. We report a 47-year-old woman with a history of dilated cardiomyopathy with unknown etiology. Echocardiography showed mild left ventricular enlargement with severe systolic dysfunction (EF=20-25%). According to cardiac magnetic resonance imaging findings non-compaction left ventricle with hypertrophic cardiomyopathy was considered, and right ventricular septal biopsy was recommended.Right ventricular end omyocardial biopsy showed moderate hypertrophy of cardiac myocytes with foci of myocytolysis and moderate interstitial fibrosis. No evidence of infiltrative deposition was seen.