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.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.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.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.