Pulmonary atresia with intact ventricular septum: Intended strategies

Amrita Sukhavasi, Sara McHugh-Grant, Andrew C. Glatz, Antara Mondal, Heather Griffis, Nancy Burnham, Jonathan M. Chen, Christopher E. Mascio, J. William Gaynor, Thomas L. Spray, Stephanie M. Fuller

Research output: Contribution to journalArticlepeer-review

2 Scopus citations

Abstract

Objective: Neonatal interventional strategies for pulmonary atresia with intact ventricular septum are based on tricuspid valve hypoplasia and right ventricle-dependent coronary circulation. We sought to evaluate long-term outcomes comparing biventricular (BiV) versus single-ventricle (SV) strategies. Methods: Retrospective review was performed of 119 patients diagnosed with pulmonary atresia with intact ventricular septum from 1995 to 2018. Descriptive statistics summarized patient characteristics and a multivariable Cox survival model was used to compare treatment strategies. Results: Of 119 patients, 62 (52.1%) were male and 13 (10.9%) had a chromosomal abnormality. BiV was pursued in 53.8% (64 out of 119) and SV in 46.2% (55 out of 119) with median tricuspid valve z scores of −1.59 (interquartile range, −3.03 to 0.21) and −5.12 (interquartile range, −5.60 to −4.06), respectively. The median follow-up was 6 years (interquartile range, 2-15 years). Overall survival at 1, 3, and 10 years was 82.4% (98 out of 119), 80.6% (96 out of 119) and 79.8% (95 out of 119), respectively. End states include 36 (30.3%) BiV, 33 (27.7%) SV, 22 (18.5%) alive without definitive end state, 21 (17.6%) death before end state, 4 (3.4%) 1-and-a-half ventricle, and 3 (2.5%) transplants. No SV were converted to BiV, whereas 4 out of 64 (6.3%) BiV were converted to SV. After adjusting for gender, chromosomal abnormalities, gestational age, and birth weight, SV patients had a significantly higher hazard of mortality (hazard ratio, 9.0; 95% CI, 2.65-30.69; P < .001). Mortality was higher in those with right ventricle-dependent coronary circulation (41.9% [13 out of 31]) compared with those without right ventricle-dependent coronary circulation (7.3% [6 out of 82]) (P < .001). Conclusions: Pulmonary atresia with intact ventricular septum remains a challenging lesion for those patients on the SV pathway, particularly with right ventricle-dependent coronary circulation.

Original languageEnglish
Pages (from-to)1277-1288
Number of pages12
JournalJournal of Thoracic and Cardiovascular Surgery
Volume164
Issue number5
DOIs
StatePublished - Nov 2022

Keywords

  • congenital heart disease
  • congenital heart surgery
  • pulmonary atresia
  • pulmonary atresia with intact ventricular septum
  • pulmonary valve

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