Alternative approach for selected severe pulmonary hypertension of congenital heart defect without initial correction — Palliative surgical treatment

Objectives

Uncorrected congenital heart defects (CHD) with severe pulmonary hypertension (sPH, systolic pulmonary artery > 70% of systolic pressure) are usually considered inoperable. We are curious to know if some selected patients might benefit from palliative operation for those sPH with uncorrected CHD.

Methods

Adults or adolescents with sPH associated with ventricular septal defect (VSD) with/without great artery anomalies were selected for pulmonary artery banding (PAB) to reduce sPH. The target pulmonary pressure was less than half of the systolic blood pressure after arch or great arteries reconstruction. Repeated catheterization was performed to evaluate the feasibility of defect closure.

Results

Consecutively, 8 patients (age 26 ± 9 years) received PAB as a palliative procedure in the past 8 years without mortality. The pre-PAB systolic pulmonary pressure was 119 ± 9 mmHg. Additional PAB had been applied in 4 of them. All patients showed significant improvement in function class (III to I or II). The mean post-PAB pulmonary pressure decreased significantly (77.5 ± 9.2 mmHg to 42.0 ± 9.0 mmHg) and 6-minute walk test was also found to have great improvement (270 ± 86 m to 414 ± 49 m), but the saturation at rest did not show a difference. Three of them received corrective surgery to close defects over 3-5 years.

Conclusion

For some selected adult sPH with uncorrected CHD, PAB can work as a palliative procedure to improve their functional class and even provide a chance of total repair.