Impact of long lasting pulmonary arterial hypertension on the surgery of congenital heart defect

Background

Little is known about the management of this high-risk subgroup of patients because only a few small retrospective studies were available in the literature.

Objectives

This study was designed to identify the determinants of post operative mortality and to assess during 10 years follow-up functional and hemodynamic variables for children presenting at surgery with pulmonary vascular disease associated with congenital heart defect.

Methods

This is a prospective study that enrolled children with pulmonary arterial hypertension associated with congenital heart disease and presenting beyond the optimal time frame for surgery. Inclusion criteria were late presentation for surgery and severe pulmonary arterial hypertension, defined as mean pulmonary arterial pressure > 50 mmhg. An univariable analysis, receiver operating characteristic curves, and Kaplan-Meier curves were used for survival analysis.

Results

75 patients underwent repair of their cardiac lesions. The operative mortality rate was 16%(12 patients); 43 patients (57 %) had right heart catheterization, and all of them were positive for acute vasodilatatory challenge (NO, oxygen). In the postoperative period, 32 % of patients had pulmonary hypertensive crisis, and 72 % had right heart failure. During follow-up, pulmonary arterial hypertension subclass 4 was present in 5% of our patients.

Conclusion

Our study indicated that for operable patients but beyond the optimal time frame the surgical mortality remained high despite using iNO. However, for those who survived they achieved good results in functional exercise, their PAP dropped to normal level and small proportion had persistent pulmonary arterial hypertension encouraging surgical closure of the defect.