[Total anomalous pulmonary venous connection: a 17-years experience of a single pediatric cardiology center].

OBJECTIVE

We reviewed our patients who underwent surgical repair for total anomalous pulmonary venous connection at our hospital.

MATERIAL AND METHODS

All patients with total anomalous pulmonary venous connection who underwent correction from February 1995 to October 2012 were included in this retrospective analysis. Clinical data was collected on presentation,morphology, associated cardiac lesions, echocardiography and cardiac cathetherization results, surgical repair and postoperative course.

RESULTS

There were 25 patients in the cohort, median age 19 days. The type of total anomalous venous connection was supracardiac in fifteen, cardiac in five, infracardiac in four, mixed-type in one patient. Six patients had single-ventricle anatomy with atrial isomerism in five. All patients with biventricular anatomy had cardiac defects associated: interauricular communication(17), patent arterial duct (9), tricuspid insufficiency (3), pulmonary stenosis (2), interventricular communication (1), mitral valve atresia (1), aortic arch hypoplasia (1). Nine patients (36%) presented with pulmonary venous obstruction. Respiratory distress was the most frequent symptom on presentation (80%), five patients were invasively ventilated. One patient was treated with extracorporeal membrane oxygenation prior to surgical correction. Twenty four cases were diagnosed with transthoracic echocardiography alone. Nine patients were evaluated with angiography that confirmed the echocardiographic diagnosis in eight patients; it allowed diagnosis of total anomalous pulmonary venous connection in one patient. Twenty four patients were submitted to surgery in the first six months of live (68% in neonatal period), one patient was operated at 27 years. Total anomalous pulmonary venous connection was corrected at first surgery in 22 patients. Two patients died after surgical correction,one with infracardiac total anomalous pulmonary venous connection with obstruction and another with supracardiac type with complex cardiac anomaly. There were no deaths in the last eleven years (14 patients). Postrepair pulmonary venous obstruction was diagnosed in one patient who was reoperated successfully.

CONCLUSION

Echocardiography provides adequate diagnostic data on total anomalous pulmonary venous connection so that surgery can be performed without angiography in most cases. Early surgical correction of total anomalous pulmonary venous connection has acceptable mortality and good long-term postrepair outcome.