title: [Family form of isolated left ventricular noncompaction; case of a mother and her son observed in Gabon].
abstract: We describe a case report of a young Gabonese lady who presented an acute pulmonary oedema and we suspected a paripartum cardiomyopathy. Subsequent investigations showed isolated left ventricular noncompaction. A few months later, the same disease was disclosed at her 9 year-old son who presented a cardiac insufficiency. Therefore, we suspect a family form of left ventricular noncompaction. And it is the first description in subsaharan Africa. The hereditary character of this new form of cardiomyopathy linked to a genetic mutation on the X chromosome is well known. This disease is associated with heart failure, high incidence of systemic thromboembolism complications or ventricular arrhythmia. The echocardiography and the cardiac magnetic resonance imaging has been reported to be tools for diagnosis. In Africa, access to these techniques remains a privilege. So the discovery of illness is often late and the family screening are special. In our area, the therapeutic management is the medical treatment of heart failure. Implatable cardioverter defibrillator or heart transplantation are not available. So long-term prognosis of our patients with congestive heart failure stays poor. With best equipment in our hospitals and good training of African cardiologists, we should improve the management of our patients.
authors.affiliation: Service de cardiologie, centre hospitalier de Libreville, B.P. 6392 Libreville, Gabon. mipinda@yahoo.fr
authors.forename: J B
authors.initials: JB
authors.lastname: Mipinda
authors.forename: J
authors.initials: J
authors.lastname: Ibaba
authors.forename: D D
authors.initials: DD
authors.lastname: Nkoghe
authors.forename: P A
authors.initials: PA
authors.lastname: Kombila
mesh_terms: D000328:Adult; D056889:Barth Syndrome / Q000175:diagnosis / Q000188:drug therapy / Q000235:genetics*; D044383:Black People; D002317:Cardiovascular Agents / Q000627:therapeutic use; D002648:Child; D003906:Developing Countries; D004359:Drug Therapy, Combination; D004452:Echocardiography; D005260:Female; D005681:Gabon; D005820:Genetic Testing; D006297:Health Services Accessibility; D006333:Heart Failure / Q000175:diagnosis / Q000188:drug therapy / Q000235:genetics; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D011204:Poverty Areas; D011379:Prognosis; D011644:Puerperal Disorders / Q000175:diagnosis / Q000188:drug therapy / Q000235:genetics; D011654:Pulmonary Edema / Q000175:diagnosis / Q000188:drug therapy / Q000235:genetics; D011787:Quality of Health Care
chemical_list: D002317:Cardiovascular Agents