Pulmonary atresia with a ventricular septal defect and left pulmonary artery discontinuity: a case report\xa0

Hyun-Hwa Cha,Hae Min Kim,Won Joon Seong

doi:10.1186/s13256-021-02750-4

Hyun-Hwa Cha, Hae Min Kim + Show 1 more

Open Access

https://doi.org/10.1186/s13256-021-02750-4

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Abstract

BackgroundUnilateral pulmonary artery discontinuity is a rare malformation that is associated with other intracardiac abnormalities. Cases accompanied by other cardiac abnormalities are often missed on prenatal echocardiography. The prenatal diagnosis of isolated unilateral pulmonary artery discontinuity can also be delayed. However, undiagnosed this malformation would have an effect on further prognosis. We report our case of a prenatal diagnosis of pulmonary atresia with ventricular septal defect and left pulmonary artery discontinuity.Case presentationA 33-year-old Asian woman visited our institution at 24 weeks of gestation because of suspected fetal congenital heart disease. Fetal echocardiography revealed a small atretic main pulmonary artery giving rise to the right pulmonary artery without bifurcation and the left pulmonary artery arising from the ductus arteriosus originating from the left subclavian artery. The neonate was delivered by cesarean section at 376/7 weeks of gestation. Postnatal echocardiography and multidetector computed tomography showed a right aortic arch, with the small right pulmonary artery originating from the atretic main pulmonary artery and the left pulmonary artery originating from the left subclavian artery. Patency of the ductus arteriosus from the left subclavian artery was maintained with prostaglandin E1. Right ventricular outflow tract reconstruction and pulmonary angioplasty with Gore-Tex graft patch was performed 25th day after birth. Unfortunately, the neonate died because of right heart failure 8 days postoperation.ConclusionThere is a possibility that both pulmonary arteries do not arise from the same great artery (main pulmonary artery or common arterial trunk). Therefore, clinicians should check the origin of both pulmonary arteries.

Highlights

Unilateral pulmonary artery discontinuity is a rare malformation that is associated with other intra‐ cardiac abnormalities
Unilateral pulmonary artery discontinuity (UPAD) is a rare form of congenital heart disease (CHD) that is usually accompanied by other cardiac anomalies, though it may occur as an isolated form [1]
We report a case of pulmonary atresia with ventricular septal defect (PAVSD) with left pulmonary artery (LPA) discontinuity

Summary

Background

Unilateral pulmonary artery discontinuity (UPAD) is a rare form of congenital heart disease (CHD) that is usually accompanied by other cardiac anomalies, though it may occur as an isolated form [1] When it is complicated with other CHDs, the precise prenatal diagnosis may be obscured. The subsequent fetal echocardiography, which we performed at 26 weeks of gestation, revealed a highly atretic MPA from the right ventricle (RV) giving rise to the right pulmonary artery (RPA), without bifurcation (Fig. 2a). Multidetector computed tomography (MDCT) revealed a right-sided aortic arch, with the left-sided ductus arteriosus (DA) originating from the LSA and MAPCA It revealed a narrow RPA (2.7 mm) connecting with the MPA (2.7 mm), without connection with the LPA (2.7 mm size).

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