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Background <p>Unilateral absence of the pulmonary artery (UAPA) is a rare congenital anomaly that may remain clinically silent until adulthood. Pregnancy and the postpartum period may unmask previously compensated congenital heart disease and pulmonary vascular disease, creating diagnostic challenges when patients present with heart failure symptoms.</p> Case report <p>A 22-year-old woman presented three months postpartum with rapidly progressive dyspnea, orthopnea, paroxysmal nocturnal dyspnea, and generalized edema after an uncomplicated vaginal delivery. Peripartum cardiomyopathy was initially suspected; however, cyanosis, digital clubbing, elevated jugular venous pressure, right ventricular heave, and loud pulmonary component of the second heart sound suggested chronic right-sided pressure overload. Echocardiography showed right atrial and ventricular dilatation, reduced right ventricular function, severe tricuspid regurgitation, severe pulmonary hypertension, bidirectional shunting across a ventricular septal defect and patent ductus arteriosus, and non-visualization of the right pulmonary artery. Computed tomography confirmed unilateral absence of the right pulmonary artery, a large subaortic ventricular septal defect extending into the membranous septum, persistent patent ductus arteriosus, and systemic collateral supply to the right lung. These findings were most consistent with complex cyanotic congenital heart disease with severe pulmonary hypertension and clinically suspected Eisenmenger-like physiology unmasked postpartum. She improved with diuresis, oxygen therapy, anticoagulation, and pulmonary vasodilator therapy.</p> Conclusion <p>Postpartum heart failure with cyanosis, clubbing, or predominant right-sided findings should prompt evaluation for previously unrecognized congenital heart disease and pulmonary vascular disease. Right pulmonary artery agenesis may be an important associated finding, but pulmonary hypertension should be interpreted according to the complete congenital anatomy and shunt physiology.</p>

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Postpartum heart failure unmasking unilateral absence of the right pulmonary artery: a case report

  • 期刊论文

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  • 发表日期：

    2026年6月24日

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  • Damasi F. Bayo,
  • Furaha S. Lyamuya,
  • Eliasa K. Ndale,
  • Meshack R. Mwantake,
  • James R. Mpagama,
  • Prosper Mbise,
  • Alex F. Lwiza,
  • Simon C. Peter,
  • Vailet A. Mteta,
  • Adnan M. Sadiq,
  • Abid M. Sadiq

摘要

Background

Unilateral absence of the pulmonary artery (UAPA) is a rare congenital anomaly that may remain clinically silent until adulthood. Pregnancy and the postpartum period may unmask previously compensated congenital heart disease and pulmonary vascular disease, creating diagnostic challenges when patients present with heart failure symptoms.

Case report

A 22-year-old woman presented three months postpartum with rapidly progressive dyspnea, orthopnea, paroxysmal nocturnal dyspnea, and generalized edema after an uncomplicated vaginal delivery. Peripartum cardiomyopathy was initially suspected; however, cyanosis, digital clubbing, elevated jugular venous pressure, right ventricular heave, and loud pulmonary component of the second heart sound suggested chronic right-sided pressure overload. Echocardiography showed right atrial and ventricular dilatation, reduced right ventricular function, severe tricuspid regurgitation, severe pulmonary hypertension, bidirectional shunting across a ventricular septal defect and patent ductus arteriosus, and non-visualization of the right pulmonary artery. Computed tomography confirmed unilateral absence of the right pulmonary artery, a large subaortic ventricular septal defect extending into the membranous septum, persistent patent ductus arteriosus, and systemic collateral supply to the right lung. These findings were most consistent with complex cyanotic congenital heart disease with severe pulmonary hypertension and clinically suspected Eisenmenger-like physiology unmasked postpartum. She improved with diuresis, oxygen therapy, anticoagulation, and pulmonary vasodilator therapy.

Conclusion

Postpartum heart failure with cyanosis, clubbing, or predominant right-sided findings should prompt evaluation for previously unrecognized congenital heart disease and pulmonary vascular disease. Right pulmonary artery agenesis may be an important associated finding, but pulmonary hypertension should be interpreted according to the complete congenital anatomy and shunt physiology.