[Percutaneous mitral commissurotomy associated to sinus venosus atrial septal defect and partially anomalous pulmonary venous connection: a case report]. / Commissurotomie mitrale percutanée associée à une communication interauriculaire type sinus venosus et un retour veineux anormal partiel: à propos d'un cas.

Lutembacher's syndrome refers to the rare combination of congenital atrial septal defect and acquired mitral stenosis. It is rarely associated to partial anomalous pulmonary venous connection. This condition is treated surgically by mitral commissurotomy or mitral valve operation with concomitant closure of the atrial septal defect with correction of the abnormal pulmonary venous connection. Percutaneous mitral commissurotomy before surgery can be a therapeutic alternative when mitral valve stenosis is severe and valve anatomy is favourable. The authors bring back the case of a 24 years old man having mitral stenosis in sinus rhythm associated to sinus venosus septal defect and partial anomalous pulmonary venous connection. The diagnosis was made for the age of 17 years old on the occasion of dyspnea. He benefited in February 2003 of rescue percutaneous mitral commissurotomy because of pulmonary oedema. Mitral valve area increased from 0.7 cm(2) to 1.6 cm(2). The patient was clinically approved, so that he refused surgery and was lost sight. Seven years later (August 2010) he was taken back for a second rescue percutaneous mitral commissurotomy because of a very severe mitral stenosis (mitral valve area was 0.8cm(2)), in pulmonary oedema with echocardiographic evaluated pulmonary hypertension at 68mmHg. The trans-septal complicated of a false road from the right atrium, towards the pericardic cavity. The patient was operated as the matter of urgency, and benefited from a mitral valve replacement by mechanical prosthesis, of closure of sinus venosus septal defect by PTFE patch and correction of abnormal pulmonary venous connection. Operating suites were simple, and the postoperative echocardiography concludes to a good prosthesis profile, the absence of residual shunt and a decrease of pulmonary artery blood pressure from 68 to 40mmHg. In conclusion, percutaneous mitral commissurotomy may be a waiting procedure for surgery of this disease or emergency treatment of it's valve anomaly, with regular monitoring while awaiting surgery faster and in better conditions.

HLA class II polymorphisms in Tunisian patients with dilated cardiomyopathy.

Cardiomyopathies (CMs) are primary disorders of cardiac muscle. They are a major cause of morbidity and mortality for all ages and, like acquired forms of cardiovascular disease, often result in heart failure. Molecular genetic studies have made remarkable progress in defining the pathogenesis of CM. The present study was the first report to evaluate the relationship between class II major histocompatibility complex (MHC) genes (HLA-DRB1 and HLA-DQB1) and the genetic susceptibility to primary dilated cardiomyopathy (DCM) in Tunisian patients. The human leukocyte antigen (HLA)-DRB1 and -DQB1 alleles were analyzed in 76 patients with primary DCM and 111 ethnically matched healthy controls using polymerase chain reaction-sequence specific primers technique. An increased frequencies of HLA-DRB1*0401 (OR = 2.67, P < 0.001), HLA-DQB1*0302 (OR = 3.28, P = 0.001) and HLA-DQB1*0401 (OR = 6.26, P = 0.005) alleles were found in the patients with primary DCM compared with healthy controls. Individuals with HLA-DRB1*1301 (OR = 0.24, P < 0.001) and HLA-DQB1*0201 (OR = 0.49, P = 0.002) alleles have a protective effect against primary DCM. Two haplotypes were associated with increased risk of primary DCM: DRB1*0401/DQB1*0302 (OR = 4.53, P = 0.002) and DRB1*0401/DQB1*0401 (OR = 9.42, P = 0.004). In conclusion, our data suggest that the variation in class II HLA alleles could be a genetic factor involved in the susceptibility to primary DCM in the Tunisian population.