Down Syndrome and congenital heart diseases with severe pulmonary hypertension surgical results

Abstract

Severe pulmonary hypertension, as many textbooks and papers have stated, is a well known risk factor in the surgical management of patients with congenital heart diseases. The objective of this paper is to try to get more information about the outcome of patients with Down Syndrome with severe pulmonary hypertension when submitted to correctice cardiac surgery. Material and Methods.-We reviewed the clinical records of all the patients (38 patients in total) with non cyanotic congenital heart diseases and severe pulmonary hypertension already submitted to corrective surgery during the period of october 1986 to december 2002 all of which had an adequate catheterization study,a useful result of a pulmonary biopsy and a follow-up period of the living patients of no less than two years and selected those with this genetic disorder. Results: The incidence of patients with Down Syndrome was 26 %, the congenital anomalies found among them were Atrioventricular Septal defects (AVSD) with an incidence of 60% and Ventricular Septal Defects (VSD) was 40%, average surgical age was more than one year and respiratory and cardiovascular complications were very frequent; mortality was 40%, all among the patients with AVSD. Outcome of survivors was good with clinical and echocardiographic regression of the high pulmonary pressures at two or more years of surgery, except in three patients, two of which were the cases with AVSD, who required medical treatment for a residual discrete pulmonary hypertension. Conclusions: We did not detect any influence in the the presence of this genetic disorder in the outcome of patients, neither in relation with preoperative nutritional status or extracorporeal circulation time, but there were diferences in the surgical results according to the type of anomaly, with mortality and presence of some residual pulmonary hypertension related to patients with Atrioventricular Septal Defects.These results show that severe pulmonary hypertension is not synonimous of inoperability, being more probable the coexistence of an Atrioventricular Septal Defect and the advanced age of surgery as risk factors for a non satisfactory outcome.