Published online August 1, 2005
PEDIATRICS Vol. 116 No. 2 August 2005, pp. 423-430 (doi:10.1542/peds.2004-2168)
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Preterm Infants With Congenital Heart Disease and Bronchopulmonary Dysplasia: Postoperative Course and Outcome After Cardiac Surgery

Colin J. McMahon, MRCPI*, Daniel J. Penny, MD, David P. Nelson, MD, PhD#, Anne M. Ades, MD**, Salim Al Maskary, MRCP, Michael Speer, MD{ddagger}, Julie Katkin, MD§, E. Dean McKenzie, MD||, Charles D. Fraser, Jr, MD|| and Anthony C. Chang, MD*

* Divisions of Pediatric Cardiology
{ddagger} Neonatology
§ Pulmonology
|| Congenital Heart Surgery, Texas Children's Hospital, Houston, Texas
Department of Pediatric Cardiology, Royal Children's Hospital, Melbourne, Australia
# Department of Pediatric Cardiology, Cincinnati Children's Hospital, Cincinnati, Ohio
** Division of Neonatology, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania

Objective. Success in treatment of premature infants has resulted in increased numbers of neonates who have bronchopulmonary dysplasia (BPD) and require surgical palliation or repair of congenital heart disease (CHD). We sought to investigate the impact of BPD on children with CHD after heart surgery.

Methods. This was a retrospective, multicenter study of patients who had BPD, defined as being oxygen dependent at 28 days of age with radiographic changes, and CHD and had cardiac surgery (excluding arterial duct ligation) between January 1991 and January 2002. Forty-three infants underwent a total of 52 cardiac operations. The median gestational age at birth was 28 weeks (range: 23–35 weeks), birth weight was 1460 g (range: 431–2500 g), and age at surgery was 2.7 months (range: 1.0–11.6 months). Diagnoses included left-to-right shunts (n = 15), conotruncal abnormalities (n = 13), arch obstruction (n = 6), univentricular hearts (n = 4), semilunar valve obstruction (n = 3), Shone syndrome (n = 1), and cor triatriatum (n = 1).

Results. Thirty-day survival was 84% with 6 early and 6 late postoperative deaths. Survival to hospital discharge was 68%. There was 50% mortality for patients with univentricular hearts and severe BPD. The median duration of preoperative ventilation was 76 days (range: 2–244 days) and of postoperative ventilation was 15 days (range: 1–141 days). The median duration of cardiac ICU stay was 7.5 days (range: 1–30 days) and of hospital stay was 115 days (range: 35–475 days). Current pulmonary status includes on room air (n = 14), O2 at home (n = 4), and ventilated at home (n = 4) or in hospital (n = 4), and 5 patients were lost to follow-up.

Conclusions. BPD has significant implications for children who have CHD and undergo cardiac surgery, leading to prolonged ICU and hospital stays, although most survivors are not O2 dependent. Postoperative mortality was highest among patients with univentricular hearts and severe BPD. Optimal timing of surgery and strategies to improve outcome remains to be delineated.

Key Words: bronchopulmonary dysplasia • congenital heart disease/defects • cardiac surgery • low birth weight

Abbreviations: BPD, bronchopulmonary dysplasia • HMD, hyaline membrane disease • NHLBI, National Heart, Lung, and Blood Institute • VLBW, very low birth weight • CHD, congenital heart disease • PCR, pulmonary vascular resistance • MOSF, multiorgan system failure • PA, pulmonary artery • VSD, ventricular septal defect • iNO, inhaled nitric oxide • NEC, necrotizing enterocolitis • BCPA, bicavopulmonary anastomosis • HLHS, hypoplastic left heart syndrome • CICU, cardiac ICU

Accepted Nov 23, 2004.