[Total anomalous pulmonary venous connection. Experience with 70 cases]. / Drenagem anômala total de veias pulmonares. Experiência com 70 casos.

PURPOSE: To evaluate the clinical and surgical experience with consecutive patients with isolated total anomalous pulmonary venous connection admitted to the Heart Institute, São Paulo, Brasil. METHODS: Hospital records of 70 consecutive patients (January 1985 to December 1993) were retrospectively reviewed. There were 28 girls and 42 boys whose ages ranged from two days to four years (mean 20.4 +/- 29 weeks). The following variables were selected for analysis: anatomic type, age at admission, clinical status, the presence of infection, restrictive atrial septal defect, and the presence of pulmonary venous obstruction. Univariate and multivariate analysis were used to identify possible risk factors for death. RESULTS: The abnormal anatomical connection was supracardiac in 63%, cardiac in 17%, infracardiac in 8.5% and mixed in 11.5% of the cases. Eighteen (25.7%) patients were admitted in poor clinical condition. Infection, mainly pulmonar, was present in 31 (44%) patients. Pulmonary venous obstruction was present in 21% of the patients. Atrial septostomy was performed in 38 patients, 13 of them admitted in a poor clinical status. The preoperative mortality was 15.7% and the operative, 17%. Statistical analysis demonstrated that infection, poor clinical status and age were risk factors for death. CONCLUSION: Surgical correction of total anomalous pulmonary venous drainage in infancy can be performed at low risk with good results. However early referral to a cardiac care center, agressive preoperative stabilization and postoperative care are important.

[Dissection in an adult with dilatation of the ascendent aorta diagnosed in childhood]. / Dissecção no adulto em dilatação de aorta ascendente diagnosticada na infância.

Dilatation of the ascending aorta is relatively infrequent during childhood. Besides the Marfan syndrome, the congenital origin should be considered. We report a patient with dilatation of the ascending aorta diagnosed at the age of 10 who presented acute aortic dissection and rupture after a 13-year period of follow-up. Several aspects of the proper diagnosis are discussed.

Coexistence of divided left atrium (cor triatriatum) and tetralogy of Fallot.

The association of a partitioned left atrium (cor triatriatum) and tetralogy of Fallot has been reported a few times in the literature. In all of these cases, there was no clinical evidence of an obstructive lesion in the left heart, raising difficulties in establishing the proper diagnosis. We call attention to this rare combination of anomalies and to the particular morphological presentation of the dividing atrial shelf, which was partially formed by the wall of the left superior caval vein.

[Transluminal angioplasty in total anomalous drainage of obstructed pulmonary veins. A case report]. / Angioplastia transluminal na drenagem anômala total de veias pulmonares na forma obstrutiva. Relato de caso.

Transluminal balloon angioplasty has been successfully used in the treatment of various congenital heart diseases, specially in pulmonary valve stenosis. Dilation angioplasty has also been used in venous obstructions in children, but the results have been contradictory. This report describes the case of a three-month-old female infant with obstructed total anomalous pulmonary venous return into the right superior vena cava. Percutaneous angioplasty was performed with two 4 mm diameter balloon catheters (Simpson Ultra Low Profile), with success. Right ventricular systolic pressure decreased from 68 to 49 mmHg and mean pulmonary vein pressure decreased from 41 to 17 mmHg. There was significant clinical improvement. Although it would be an unique experience in our Department, authors suggest that transluminal balloon angioplasty could be an initial therapeutic alternative in infants with obstructive total anomalous pulmonary venous return, a high-risk group for total surgical repair.