Heart–lung transplantation in patients with pulmonary atresia and ventricular septal defect

Objective

Heart–lung transplantation for patients with pulmonary atresia and ventricular septal defect is challenging. The aim of the study was to present a single-center experience with heart–lung transplantation in this difficult group of patients.

Methods

A retrospective review identified 9 patients aged 4.1 to 45.6 years (median, 25.4 years) with pulmonary atresia and ventricular septal defect who underwent heart–lung transplantation. Four (44.4%) patients had previous heart operations: 3 of them had palliative procedures (systemic-to-pulmonary shunts), and 1 had multistage correction. A standard transplantation method was used, with the exception of 1 patient with heterotaxy syndrome who underwent a modified operation. Major aortopulmonary collateral arteries were controlled by using various techniques.

Results

Follow-up ranged between 2 days and 12.6 years (median, 1.2 years). The hospital mortality rate was 22.2% (n = 2). In the late postoperative period, 3 patients died. The survival curve was similar to that of patients with other diagnoses undergoing heart–lung transplantation. The median length of intensive care unit stay was 58 days (range, 22–82 days), and the median length of hospital stay was 83 days (range, 35–136 days). The most common early complication was bleeding requiring re-exploration. In all cases the bleeding was proved to be from collateral vessels.

Conclusions

Heart–lung transplantation in patients with pulmonary atresia and ventricular septal defect requires carefully planned and meticulously performed surgical intervention. This management should be taken into consideration as a future option if the specific anatomy is uncorrectable in early childhood, and the palliative procedures should be avoided.

Abbreviations and Acronyms: CMV, cytomegalovirus, CPB, cardiopulmonary bypass, HLTX, heart–lung transplantation, MAPCA, major aortopulmonary collateral artery, PA+VSD, pulmonary atresia with ventricular septal defect

CTSNet classification: 21, 34