[Acute atrio-ventricular block in sickle cell anemia]. / Bloc auriculo-ventriculaire transitoire aigu dans la drépanocytose. Première série.

BACKGROUND: Even though sickle cell disease has a high prevalence amongst the black race and despite its well known potential of micro infarction, there have been only a few reports regarding the acute myocardial damage during vaso-occlusive crisis. The risk of atrio ventricular block during these crises has never been described in a large survey. PATIENTS AND RESULTS: Ten patients (six men and four women, mean age 39 years old) were hospitalized for an acute atrio ventricular block. The patients were all African or Caribbean natives. Three patients were found with a heterozygous phenotype for hemoglobin S (sickle trait) and seven were found with a homozygous phenotype. The most common symptoms were asthenia (10 cases), shortness of breath (8 cases) and acute coronary syndrome (1 case) (syncope was not reported). Four patients had a second degree atrio ventricular block and six patients had third degree block. The treatment involved bed rest, intravenous hydration, and pain relief with opiates. All the cases of atrio ventricular block were only transitory and none of the patients underwent a pacemaker implantation. CONCLUSION: This report is the largest survey regarding transitory acute atrio ventricular block in patients with sickle cell disease. A local ischemic event affecting the AV node and Hiss bundle area can explain the conduction abnormalities. Sickle cell disease must be ruled out in black patients with an AV block.

[Transitory acute atrioventricular block in an African patient: consider sickle cell anemia]. / Bloc auriculoventriculaire complet transitoire chez un africain: la drépanocytose comme cause.

This case report shows a rare cardiac complication of sickle cell anemia in a young African patient which was an acute paroxysmal atrio-ventricular block. Acute paroxysmal atrioventricular block is a rare complication of polymerization of hemoglobin S during sickle cell disease. Hence, sickle cell anemia should be considered as a cause of auriculoventricular block in black African patients. Cardiac complications of sickle cell anemia are presented in this article.

[Cardiac manifestations of sickle cell anemia]. / Manifestations cardiaques de la drépanocytose.

HEMOGLOBINS S AND C: Drepanocytosis, the occurrence of sickle cells (drepanocytes) in the blood, is an inherited condition. Electrophoresis demonstrates hemoglobin SS in homozygous subjects who present the typical clinical features of severe hemolytic sickle-cell anemia. Heterozygous subjects have sickle-cell anemia trait, an asymptomatic condition associated with a 50% hemoglobin S and 50% hemoglobin C at electrophoresis. Hemoglobin S and C are transmitted by Mendelian inheritance. CARDIAC DISORDERS: Well-known, cardiac disorders occur in more than 82% of homozygous subjects while only 2% of heterozygous subjects are affected. Heart murmur, radiological cardiomegaly, or eletrocardiographic anomalies are often the only signs. There is a risk of fatal heart failure in children and neonates. Acute rheumatic fever or infectious endocarditis, particularly due to pneumococcal or Haemophilus influenzae infection, may trigger heart failure. CARDIAC ANOMALIES: Patients with sickle-cell anemia can develop an "anemic heart" expressed by an elevated cardiac output and systemic ejection volume at rest and a fall in arteriolar peripheral resistance. Patients who develop cor pulmonae have an elevated pulmonary pressure at exercise and experience venous occlusive events with a progressive reduction in the pulmonary vascular bed and development of a left-right shunt. Myocardiopathy leads to left ventricular dysfunction contrasting with the dilated right heart seen at echocardiography and rare cases of transmural infarction.

[Subannular pseudoaneurysm of the left ventricle complicating mitral valve replacement; apropos of a case]. / Pseudo-anévrysme sous-annulaire du ventricule gauche compliquant un remplacement valvulaire mitral; à propos d'un cas.

The authors report the case of a 72 year old woman with a Carpentier-Edwards bioprosthesis implanted in March 1981 for severe mitral stenosis, asymptomatic until 1986. The progression of effort dyspnoea with prosthetic valve dysfunction despite digitalo-diuretic therapy and severe ventricular subannular pseudoaneurysm. Doppler echocardiography and cardiac catheterisation and angiography confirmed the mitral subannular pseudoaneurysm and led to surgical cure of this fibrous structure and implantation of a mechanical mitral valve prosthesis. This complication of mitral valve replacement is a cause of pseudoaneurysm which should not be overlooked.