Treatment Options for Pulmonary Atresia/VSD/MAPCAs
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Purpose of review
This review provides an up to date overview of the treatment options for newborns with pulmonary atresia/VSD/MAPCAs.
It answers the question of how best to diagnose and formulate a surgical plan for this diverse and heterogeneous patient population.
The surgical repair of patients with pulmonary atresia/VSD/MAPCAs can be performed safely with excellent results as a single stage procedure and in the neonatal period in specific circumstances.
Pulmonary atresia/VSD/MAPCAs is a complex and varied lesion. Its clinical presentation and management vary between patients and is mainly dictated by the anatomic characteristics of the major aortopulmonary collaterals which constitute the extracardiac sources of pulmonary blood flow. A rigorous evaluation of the patient’s anatomy is mandatory. Precise surgical planning is a prerequisite and exact surgical execution is required for a successful patient outcome.
KeywordsPulmonary atresia MAPCAs Major aortopulmonary collaterals Unifocalization
Thank you to the UCSF and Stanford groups for their dedicated and exhaustive efforts leading the effort in the management of patients with pulmonary atresia/VSD/MAPCAs.
Financial support and sponsorship
This work was self-funded and non-sponsored.
Compliance with ethical standards
Conflict of interest
The authors declare no conflict of interest.
Human and animal rights and informed consent
This article does not contain any studies with human or animal subjects performed by any of the authors.
References and Recommended Reading
Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance
- 6.Reddy VM, Hanley F. Surgical treatment of pulmonary atresia with ventricular Septal defect. In: Mavroudis C, Backer C, editors. Pediatric cardiac surgery. Oxford: Wiley-Blackwell; 2013.Google Scholar
- 8.Carrillo SA, Mainwaring RD, Patrick WL, Bauser-Heaton HD, Peng L, Reddy VM, et al. Surgical repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals with absent intrapericardial pulmonary arteries. Ann Thorac Surg. 2015 Aug 1;100(2):606–14.CrossRefGoogle Scholar
- 9.•Koth A, Sidell D, Bauser-Heaton H, Wise-Faberowski L, Hanley FL, McElhinney DB, Asija R. Deletion of 22q11 chromosome is associated with postoperative morbidity after unifocalisation surgery. Cardiology in the young. 2019 Jan;29(1):19–22. This study retrospectively reviewed all patients with pulmonary atresia/VSD/MAPCAs who had DiGeorge Syndrome, or 22q11 deletion. The report is from investigators at Stanford University and the group found longer ICU and hospital length of stays secondary to prolonged mechanical ventilation when compared to the group who did not have chromosomal abnormalities. The entire cohort was 180 individuals of whom 41% had 22q11 deletion.Google Scholar
- 12.Barron DJ, Botha P. Approaches to pulmonary atresia with major aortopulmonary collateral arteries. In seminars in thoracic and cardiovascular surgery: pediatric cardiac surgery annual 2018 mar 1 (Vol. 21, pp. 64-74). WB Saunders.Google Scholar
- 14.Puga FJ, Leoni FE, Julsrud PR, Mair DD. Complete repair of pulmonary atresia, ventricular septal defect, and severe peripheral arborization abnormalities of the central pulmonary arteries. Experience with preliminary unifocalization procedures in 38 patients. J Thorac Cardiovasc Surg. 1989 Dec;98(6):1018–28.PubMedGoogle Scholar
- 15.••Zhu J, Meza J, Kato A, Saedi A, Chetan D, Parker R, Caldarone CA, McCrindle BW, Van Arsdell GS, Honjo O. Pulmonary flow study predicts survival in pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries. The Journal of thoracic and cardiovascular surgery. 2016 Dec 1;152(6):1494-503. This study is one of the few which reports the use of the intraoperative flow study as first described by Reddy and colleagues in 1997. Over a 13-year period, 50 patients at the Hospital for Sick Children underwent repair of pulmonary atresia/VSD/MAPCAs. 40 patients underwent the intraoperative flow study and based on those results, 34 (85.5%) were able to undergo concominant VSD closure. Just as important, the group found that the estimated RV pressure elucidated from the intraoperative flow study was associated with medium-term survival. Cox regression analysis identified a mean PAP > 25 mm hg as the sole predictor for death (P =.037).Google Scholar
- 18.Reddy VM, McElhinney DB, Amin Z, Moore P, Parry AJ, Teitel DF, et al. Early and intermediate outcomes after repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries: experience with 85 patients. Circulation. 2000 Apr 18;101(15):1826–32.CrossRefGoogle Scholar
- 20.Alfieri O, Blackstone EH, Parenzan L. Growth of the pulmonary anulus and pulmonary arteries after the Waterston anastomosis. J Thorac Cardiovasc Surg. 1979 Sep;78(3):440–4.Google Scholar