The Journal of Thoracic and Cardiovascular Surgery
Congenital: Pulmonary ValvePulmonary atresia with intact ventricular septum: Intended strategies
Graphical abstract
Section snippets
Patients and Data Collection
A retrospective chart review was performed on patients with PAIVS born between January 1, 1995, and December 31, 2018, who underwent initial treatment at the Children's Hospital of Philadelphia. Exclusion criteria included patients who had any degree of antegrade pulmonary blood flow, TV anomalies, including Ebstein anomaly or tricuspid atresia, or any patient who underwent initial palliation at an outside hospital. Although written consent was not required, verbal consent for participation and
Overall
A total of 119 patients with PAIVS underwent initial intervention at our center between January 1, 1995, and December 31, 2018. Median follow-up duration was 6.2 years (IQR, 1.6-14.9 years) in total and 10.2 years (IQR, 4.4-16.1 years) for patients alive at last follow-up. The cohort was 52% boys and 13 out of 119 patients (11%) had a chromosomal abnormality. Twenty-three (19%) patients were considered to have had low birth weight and 21 (18%) were premature. Median TV z score was –3.47 (IQR,
Discussion
This retrospective study evaluated long-term outcomes in patients who underwent initial palliation for PAIVS at our institution over the past several decades. Just more than half of the patients enrolled underwent RV decompression, portending a BiV repair. Of the 64 patients intended for BiV repair, 56% achieved a BiV repair, whereas 11% either died, underwent transplant, or were converted to SV. A small percentage (6%) became a 1.5V repair. It is important to recognize that a large portion of
Conclusions
PAIVS remains a highly lethal lesion, with overall survival of 80% in this series. Mortality remains highest in patients with SV physiology awaiting Fontan completion, likely due to the presence of RVDCC. As such, these patients may benefit from consideration for early transplant referral. Careful consideration should be given to creating intentional 1.5V end states in an effort to minimize mortality and morbidity for those patients who require SV or marginal BiV repairs. Development of a
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