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Edward Malec
Gerd Juchem
Ingo Kaczmarek
Ralf Sodian
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Right arrow Congenital - cyanotic
Right arrow Transplantation - heart

J Thorac Cardiovasc Surg 2009;138:738-743
© 2009 The American Association for Thoracic Surgery

Cardiothoracic Transplantation

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

Katarzyna Januszewska, MD, PhD*, Edward Malec, MD, PhD, Gerd Juchem, MD, PhD, Ingo Kaczmarek, MD, PhD, Ralf Sodian, MD, PhD, Peter Uberfuhr, MD, PhD, Bruno Reichart, MD, PhD

Department of Cardiac Surgery, Klinikum Grosshadern, Ludwig Maximilians University, Munich, Germany

Received for publication September 12, 2008; revisions received November 18, 2008; accepted for publication December 25, 2008.

* Address for reprints: Katarzyna Januszewska, MD, PhD, Department of Cardiac Surgery, Klinikum Grosshadern, Marchioninistr. 15, 81377 Munich, Germany. (Email: mijanusz{at}cyf-kr.edu.pl).

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






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