Congenital: Pulmonary Valve
Pulmonary atresia with intact ventricular septum: Intended strategies

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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.

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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