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      Isolated left ventricular noncompaction in sub-Saharan Africa: a clinical and echocardiographic perspective.

      Circulation. Cardiovascular Imaging
      Adult, African Continental Ancestry Group, statistics & numerical data, Analysis of Variance, Case-Control Studies, Chi-Square Distribution, Female, Heart Failure, ethnology, physiopathology, ultrasonography, Humans, Hypertension, Pulmonary, Hypertrophy, Right Ventricular, Isolated Noncompaction of the Ventricular Myocardium, Male, Middle Aged, Papillary Muscles, abnormalities, Phenotype, Predictive Value of Tests, Prognosis, Prospective Studies, Registries, South Africa, epidemiology, Systole, Tricuspid Valve Insufficiency, Ultrasonography, Doppler, Color, Ventricular Dysfunction, Left, Ventricular Function, Left, Ventricular Function, Right

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          Isolated left ventricular noncompaction (ILVNC) is a cardiomyopathy caused by intrauterine failure of the myocardium to compact. Common clinical complications are heart failure, arrhythmias, and cardioembolism. A paucity of data exists relating to clinical and echocardiographic features of ILVNC in Africans. This study is a single-center, prospective case-control study, whereby subjects attending a dedicated cardiomyopathy clinic were screened for and diagnosed with ILVNC, provided they had no other associated structural heart disease and fulfilled all the accompanying echocardiographic criteria: (1) end-systolic ratio of noncompacted layer to compacted layer >2, (2) presence of >3 prominent apical trabeculations, and (3) deep intertrabecular recesses that fill with blood from the ventricular cavity visualized using color Doppler ultrasound. Fifty-four subjects were identified, age 45.4±13.1 years (mean±SD), 95% confidence interval 3.6 to 10.2, 55.6% male, and 63.0% New York Health Association Class II, and prevalence of LVNC in our clinic was 6.9%, 95% confidence interval 3.6 to 10.2. Heart failure because of systolic dysfunction was the most common clinical presentation (53 subjects, 98.1%). Left ventricular end-diastolic diameter was 61.4±7.2 mm (mean±SD) and ejection fraction 26.7±11.9% (mean±SD). Common sites of noncompaction were the apical (100%), midinferior (74.1%), and midlateral (64.8%) walls. Right ventricular noncompaction occurred in 12 subjects (22.2%). Pulmonary hypertension was documented in 45 cases (83.3%). Right ventricular dilation was noted in 40 subjects (74.1%), while right ventricular function was depressed in 32 (59.3%). Tricuspid S' was 9.6±2.8 cm/s (mean±SD). No echocardiographic features suggestive of ILVNC were noted in a healthy control group of African descent. ILVNC in patients of African descent can be characterized by biventricular abnormality and pulmonary hypertension, in addition to isolated left-sided abnormality.

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