title: Heart-lung transplantation in patients with pulmonary atresia and ventricular septal defect.
abstract: 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.
authors.affiliation: Department of Cardiac Surgery, Klinikum Grosshadern, Ludwig Maximilians University, Munich, Germany. mijanusz@cyf-kr.edu.pl
authors.forename: Katarzyna
authors.initials: K
authors.lastname: Januszewska
authors.forename: Edward
authors.initials: E
authors.lastname: Malec
authors.forename: Gerd
authors.initials: G
authors.lastname: Juchem
authors.forename: Ingo
authors.initials: I
authors.lastname: Kaczmarek
authors.forename: Ralf
authors.initials: R
authors.lastname: Sodian
authors.forename: Peter
authors.initials: P
authors.lastname: Uberfuhr
authors.forename: Bruno
authors.initials: B
authors.lastname: Reichart
mesh_terms: D000293:Adolescent; D000328:Adult; D001014:Aortic Aneurysm / Q000209:etiology; D001989:Bronchiolitis Obliterans / Q000209:etiology; D002423:Cause of Death; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D006345:Heart Septal Defects, Ventricular / Q000150:complications / Q000601:surgery*; D016041:Heart-Lung Transplantation / Q000009:adverse effects / Q000379:methods* / Q000401:mortality; D006801:Humans; D007902:Length of Stay; D008297:Male; D008875:Middle Aged; D011115:Polyneuropathies / Q000209:etiology; D018633:Pulmonary Atresia / Q000150:complications / Q000601:surgery*; D051437:Renal Insufficiency / Q000209:etiology; D012189:Retrospective Studies; D015996:Survival Rate; D014135:Tracheal Stenosis / Q000209:etiology; D055815:Young Adult