Outcomes of total anomalous pulmonary venous drainage repair in neonates and the impact of pulmonary hypertension on survivalCentral MessagePerspective
Antonia Schulz, MD,
Damien M. Wu, MD,
Shuta Ishigami, MD, PhD,
Edward Buratto, MBBS, PhD, FRACS,
Duncan MacGregor, MBBS, PhD, FRCPA,
Matthew S. Yong, MBBS, PhD, FRACS,
Yaroslav Ivanov, MD, PhD,
Roberto Chiletti, MD, CICM,
Christian P. Brizard, MD,
Igor E. Konstantinov, MD, PhD, FRACS
Affiliations
Antonia Schulz, MD
Department of Cardiothoracic Surgery, Royal Children's Hospital, Melbourne, Australia
Damien M. Wu, MD
Department of Cardiothoracic Surgery, Royal Children's Hospital, Melbourne, Australia
Shuta Ishigami, MD, PhD
Department of Cardiothoracic Surgery, Royal Children's Hospital, Melbourne, Australia
Edward Buratto, MBBS, PhD, FRACS
Department of Cardiothoracic Surgery, Royal Children's Hospital, Melbourne, Australia; Department of Paediatrics, University of Melbourne, Melbourne, Australia; Heart Research Group, Murdoch Children's Research Institute, Melbourne, Australia
Duncan MacGregor, MBBS, PhD, FRCPA
Department of Anatomical Pathology, Royal Children's Hospital, Melbourne, Australia
Matthew S. Yong, MBBS, PhD, FRACS
Department of Cardiothoracic Surgery, Royal Children's Hospital, Melbourne, Australia
Yaroslav Ivanov, MD, PhD
Department of Cardiothoracic Surgery, Royal Children's Hospital, Melbourne, Australia
Roberto Chiletti, MD, CICM
Department of Paediatric Intensive Care, Royal Children's Hospital, Melbourne, Australia
Christian P. Brizard, MD
Department of Cardiothoracic Surgery, Royal Children's Hospital, Melbourne, Australia; Department of Paediatrics, University of Melbourne, Melbourne, Australia; Heart Research Group, Murdoch Children's Research Institute, Melbourne, Australia
Igor E. Konstantinov, MD, PhD, FRACS
Department of Cardiothoracic Surgery, Royal Children's Hospital, Melbourne, Australia; Department of Paediatrics, University of Melbourne, Melbourne, Australia; Heart Research Group, Murdoch Children's Research Institute, Melbourne, Australia; Address for reprints: Igor E. Konstantinov, MD, PhD, FRACS, Royal Children's Hospital, Flemington Rd, Parkville, Victoria 3052, Australia.
Background: Mortality after repair of total anomalous pulmonary venous drainage (TAPVD) in neonates has remained high. Analysis of risk factors may help identify therapeutic targets to improve survival. Methods: Retrospective analysis of all neonates who underwent simple TAPVD repair. Results: Between 1973 and 2021, 175 neonates underwent TAPVD repair, at a median age of 6 days (interquartile range, 2-15 days) and a mean weight of 3.2 ± 0.6 kg. TAPVD was supracardiac in 42.3% of the patients (74 of 175), cardiac in 14.3% (25 of 175), infracardiac in 40% (70 of 175), and mixed type in 3.4% (6 of 175), with obstruction in 65.7% (115 of 175). Pulmonary hypertension (PHT) crisis occurred in 12% (21 of 175). Early mortality was 9.7% (17 of 175) and late mortality was 5.1% (8 of 158), with most deaths occurring within 1 year (75%; 6 of 8). Survival was 86.5% (95% CI, 80.3%-90.8%) at 1 year and 85.8% (95% CI, 79.6%-90.3%) at 5, 10, 15, and 20 years. Survival was lower in patients with obstructed TAPVD, patients with emergent surgery, and those with PHT crisis. PHT crisis (hazard ratio [HR], 4.93; 95% CI, 1.95-12.51; P = .001), urgency of surgery (HR, 2.51; 95% CI, 1.11-5.68; P = .027), and higher pulmonary artery pressure–to–systemic blood pressure percentage ratio (HR, 1.06; 95% CI, 1.01-1.11; P = .026) were identified as risk factors for mortality. Histopathological analysis of 17 patients (9.7%; 17 of 175) showed signs of pulmonary arterial hypertension with media hypertrophy in 58.8% (10 of 17). Conclusions: Mortality after TAPVD repair occurred mainly within the first year of life. Urgency of surgery and persistent PHT appears to be risk factors for mortality. Lung biopsy might be useful for identifying patients at risk and guiding newer treatment modalities.
