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

• References

• 1 Reading DW, Oza U. Unilateral absence of a pulmonary artery: a rare disorder with variable presentation. Proc (Baylor Univ Med Cent) 2012; 25 (02) 115-118
• 2 Tian M, Zheng M. Unilateral absence of pulmonary artery analysis based on echocardiographic feature. Rev Cardiovasc Med 2021; 22 (02) 483-488
• 3 Bouros D, Pare P, Panagou P, Tsintiris K, Siafakas N. The varied manifestation of pulmonary artery agenesis in adulthood. Chest 1995; 108 (03) 670-676
• 4 Furuya T, Iida R, Konishi J, Kato J, Suzuki T. Anesthesia for cesarean section in a patient with isolated unilateral absence of a pulmonary artery. Braz J Anesthesiol 2017; 67 (01) 85-88
• 5 Alshibli N, Ashley M, Fuller M. et al. Pulmonary hypertension and pregnancy: a preliminary report of 25 pregnancies. Am J Obstet Gynecol 2021; 224 (02) S482
• 6 Brooke BS, Schwartz TA, Pawlik TM. MOOSE Reporting Guidelines for Meta-analyses of Observational Studies. JAMA Surg 2021; 156 (08) 787-788
• 7 Arya S, Kaji AH, Boermeester MA. PRISMA Reporting Guidelines for Meta-analyses and Systematic Reviews. JAMA Surg 2021; 156 (08) 789-790
• 8 Choi Y, Kang N, Im Y, Choe J, Kim TJ, Kim H. Unilateral pulmonary artery agenesis manifested by hemoptysis in pregnancy. Respir Med Case Rep 2018; 26: 14-16
• 9 Vohra N, Alvarez M, Abramson AF, Lockwood CJ. Hypoplastic pulmonary artery: an unusual entity mimicking pulmonary embolism during pregnancy. Obstet Gynecol 1992; 80 (3 Pt 2): 483-485
• 10 Moroi HTH, Yamada E, Mano Y, Sumigama S, Kontani T, Kikkawa F. Congenital unilateral absence of a pulmonary artery in a pregnant woman: a case report. J Matern Fetal Neonatal Med 2014; 27: 60
• 11 Ko T, Gatz MG, Reisz GR. Congenital unilateral absence of a pulmonary artery: a report of two adult cases. Am Rev Respir Dis 1990; 141 (03) 795-798
• 12 Ferrari M, Karrazi R, Lampronti G. et al. Effect of changing position on arterial oxygenation in a patient with agenesia of the left pulmonary artery. Respiration 1997; 64 (05) 371-374
• 13 Yoshihara K, Kurabayashi T, Tsuchiya M. et al. Pregnancy with congenital unilateral absence of a pulmonary artery. Acta Obstet Gynecol Scand 2006; 85 (06) 755-757
• 14 Kurokawa S, Hirooka K, Nagai M, Ozaki M, Nomura M. Anesthetic considerations for cesarean section in a parturient complicated by Scimitar syndrome-like pathophysiology. JA Clin Rep 2018; 4 (01) 78
• 15 Lal C, Barker J, Strange C. Unilateral pulmonary artery aplasia in a pregnant patient. Case Rep Med 2011; 2011: 806723
• 16 Koga H, Hidaka T, Miyako K, Suga N, Takahashi N. Age-related clinical characteristics of isolated congenital unilateral absence of a pulmonary artery. Pediatr Cardiol 2010; 31 (08) 1186-1190
• 17 Stiller RJ, Soberman S, Turetsky A, Lockwood C, Haddad R. Agenesis of the pulmonary artery: an unusual cause of dyspnea in pregnancy. Am J Obstet Gynecol 1988; 158 (01) 172-173
• 18 Spinnato JA, Kraynack BJ, Cooper MW. Eisenmenger's syndrome in pregnancy: epidural anesthesia for elective cesarean section. N Engl J Med 1981; 304 (20) 1215-1217
• 19 Kuroshima MBM, Ikuhashi Y, Takeda K, Suda S, Arai T, Ota T. Challenges in the management of pregnancy and delivery in two cases of women with heart disease. J Obstet Gynaecol Res 2019; 1756 (08) DOI: 10.1111//jog.14030.