Congenital unilateral absence of right pulmonary artery with VSD and wide aortopulmonary window in an adult patient: a case report

Abstract

Introduction and background: Unilateral absence of pulmonary artery (UAPA) is a very rare condition, with an estimated prevalence of 1 in 200,000 population, which is commonly associated with various cardiovascular anomalies or can occur in an isolated manner. Isolated cases survive to adulthood and remain asymptomatic, but they may frequently experience hemoptysis, repeated infections, or symptoms like dyspnea and chest pain. Due to the rarity of the disorder and its ambiguous appearance, diagnosis can be very challenging. Case presentation: We present a case of a 28-year-old male who visited our center with the diagnosis of ventricular septal defect with Eisenmenger syndrome elsewhere for further evaluation and was found to have right-sided UAPA with ipsilateral pulmonary hypoplasia and some associated cardiac anomalies. Clinical discussion: Discussions are held regarding typical chest radiograph findings, diagnostic methods, and possible therapies. Conclusion: Physicians should be aware of UAPA, which might go undiagnosed for several years despite regular medical care and can show up later in life, causing chronic respiratory symptoms along with Eisenmenger syndrome and ventricular septal defect like in our case.

CT Assessment of Aortopulmonary Septal Defect: How to Approach It?

“…Moreover, in patients with pulmonary atresia and ventricular septal defect, an APW may be the sole source of pulmonary blood flow in the absence of PDA and major aortopulmonary collateral arteries [30]. A congenital unilateral absence of the right pulmonary artery with a VSD and a wide aortopulmonary window has also been described [38]. of tetralogy of Fallot with membranous pulmonary atresia [37].…”

Embolization treatment of right pulmonary artery agenisis with patent ductus arteriosus causing pulmonary hypertension and hemoptysis: a case report and literature review

“…Diagnosis typically occurs during childhood for most patients and common complications include recurrent pulmonary infections, hemoptysis, chest pain and Eisenmenger syndrome. Clinical indicators facilitating early diagnosis comprise features like pulmonary hypertension and congestive heart failure [ 5 ]. The mortality rate for individuals with right PAA experiencing pulmonary hemorrhage ranges from 27 to 48% [ 2 , 6 ], further increasing when accompanied by congenital heart disease.…”