Unilateral Absent Pulmonary Artery in Pregnancy: A Case Report and Systematic Literature Review

Abstract

Objective Unilateral absence of a pulmonary artery (UAPA) is a rare congenital malformation associated with hemoptysis, pulmonary hypertension, and infection. Little is known about the impact on pregnancy outcomes. We sought to synthesize the existing literature on pregnancy outcomes in patients with maternal UAPA.

Study Design We report a case of maternal UAPA and performed a systematic review of the existing literature. Articles in English reporting pregnancy outcomes among women with unilateral absence or hypoplasia of the pulmonary artery were included. Articles were reviewed at the abstract level and, if eligible, at the full-text level by two independent reviewers with disagreements adjudicated by a third reviewer. Data were abstracted by two independent reviewers. Outcomes of interest were mode of delivery, gestational age at delivery, intensive care admission, maternal death, and length of stay. Summary statistics for each outcome are presented.

Results We identified 14 studies, including the presented case, reporting outcomes in 22 pregnancies impacted by maternal UAPA. Median age at diagnosis was 21 years. Concurrent cardiac comorbidities were reported in 6/13 (46.2%) with pulmonary hypertension in 5/20 (25%) of cases where this information was reported. We observed high rates of preterm birth (4/12, 33.3%), cesarean delivery (10/15, 66.7%), and operative vaginal delivery (2/5, 40.0%). There was one maternal death occurring in the immediate postpartum period for a mortality rate of 4.5%.

Conclusion Our study provides a comprehensive review of existing literature on maternal UAPA. Our findings suggest increased rates of adverse outcomes and underscore the importance of early diagnosis, identification of pulmonary hypertension, and multidisciplinary care.

Key Points

• There may be increased adverse outcomes in maternal UAPA.

• Concurrent cardiac abnormalities are common in maternal UAPA.

• Early diagnosis, identification of pulmonary hypertension, and multidisciplinary care are important.

Keywords

pulmonary artery aplasia - pregnancy - maternal mortality - pulmonary hypertension - hemoptysis

Authors' Contributions

S.D., J.Q-N., M.E.L., L.M.K., and B.L. were responsible for study conception and design. L.M.K., S.D., J.Q-N., and J.L.C. were responsible for search strategy, data collection and synthesis. J.Q-N. and L.M.K. were responsible for data analysis. L.M.K., S.D., and J.Q-N. primarily drafted the manuscript and all authors (S.D., J.Q-N., M.E.L., L.M.K., B.L., and J.L.C.) provided critical review and revision.

Publication History

Received: 18 November 2023

Accepted: 21 November 2023

Accepted Manuscript online:
23 November 2023

Article published online:
21 December 2023

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