title: Isolated left ventricular noncompaction in sub-Saharan Africa: a clinical and echocardiographic perspective.
abstract: BACKGROUND
  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.
  
  
  RESULTS
  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.
  
  
  CONCLUSIONS
  ILVNC in patients of African descent can be characterized by biventricular abnormality and pulmonary hypertension, in addition to isolated left-sided abnormality.
authors.affiliation: Department of Cardiology, Chris Hani Baragwanath Hospital, University of the Witwatersrand, Johannesburg, South Africa.
authors.forename: Ferande
authors.initials: F
authors.lastname: Peters
authors.forename: Bijoy K
authors.initials: BK
authors.lastname: Khandheria
authors.forename: Claudia
authors.initials: C
authors.lastname: dos Santos
authors.forename: Hiral
authors.initials: H
authors.lastname: Matioda
authors.forename: Nirvarthi
authors.initials: N
authors.lastname: Maharaj
authors.forename: Elena
authors.initials: E
authors.lastname: Libhaber
authors.forename: Farouk
authors.initials: F
authors.lastname: Mamdoo
authors.forename: Mohammed R
authors.initials: MR
authors.lastname: Essop
mesh_terms: D000328:Adult; D000704:Analysis of Variance; D044383:Black People / Q000706:statistics & numerical data; D016022:Case-Control Studies; D016009:Chi-Square Distribution; D005260:Female; D006333:Heart Failure / Q000000981:diagnostic imaging / Q000208:ethnology / Q000503:physiopathology; D006801:Humans; D006976:Hypertension, Pulmonary / Q000000981:diagnostic imaging / Q000208:ethnology / Q000503:physiopathology; D017380:Hypertrophy, Right Ventricular / Q000000981:diagnostic imaging / Q000208:ethnology / Q000503:physiopathology; D056830:Isolated Noncompaction of the Ventricular Myocardium / Q000000981:diagnostic imaging* / Q000208:ethnology / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D010210:Papillary Muscles / Q000002:abnormalities / Q000000981:diagnostic imaging; D010641:Phenotype; D011237:Predictive Value of Tests; D011379:Prognosis; D011446:Prospective Studies; D012042:Registries; D013019:South Africa / Q000453:epidemiology; D013599:Systole; D014262:Tricuspid Valve Insufficiency / Q000000981:diagnostic imaging / Q000208:ethnology / Q000503:physiopathology; D018615:Ultrasonography, Doppler, Color; D018487:Ventricular Dysfunction, Left / Q000000981:diagnostic imaging / Q000208:ethnology / Q000503:physiopathology; D016277:Ventricular Function, Left; D016278:Ventricular Function, Right