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Ann Cardiol Angeiol (Paris) ; 62(1): 56-9, 2013 Feb.
Artículo en Francés | MEDLINE | ID: mdl-21676376

RESUMEN

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.


Asunto(s)
Síndrome de Barth/genética , Población Negra , Países en Desarrollo , Adulto , Síndrome de Barth/diagnóstico , Síndrome de Barth/tratamiento farmacológico , Fármacos Cardiovasculares/uso terapéutico , Niño , Quimioterapia Combinada , Ecocardiografía , Femenino , Gabón , Pruebas Genéticas , Accesibilidad a los Servicios de Salud , Insuficiencia Cardíaca/diagnóstico , Insuficiencia Cardíaca/tratamiento farmacológico , Insuficiencia Cardíaca/genética , Humanos , Imagen por Resonancia Magnética , Masculino , Áreas de Pobreza , Pronóstico , Trastornos Puerperales/diagnóstico , Trastornos Puerperales/tratamiento farmacológico , Trastornos Puerperales/genética , Edema Pulmonar/diagnóstico , Edema Pulmonar/tratamiento farmacológico , Edema Pulmonar/genética , Calidad de la Atención de Salud
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