Unilateral absence of the pulmonary artery(UAPA) that normally arises from the sixth aortic arch is a very rare congenital malformation with a prevalence of 0005%。 UAPA is more common on the right side1.UAPA may present in infancy with respiratory distress, pulmonary hypertension and congestive heart failure1. When severe pulmonary hypertension does not develop in infancy, the condition may remain asymptomatic until adulthood. This report presents a rare case of UAPA with PVS and partial connection of descending aorta and underscores the role of imaging modalities in identifying this condition and providing evidence for further treatment.
This case involves a 52-year-old female with presenting symptom of worsening shortness of breath after activity 1year ago. No history of hypertension, diabetes or smoking.Clinical workup reveaed for Right heart failure.Echocardiography indicates congenital heart disease, moderate and severe pulmonary valve stenosis and regurgitation(Fig. 1).In order to explore the disease and choose the appropriate treatment,Our team performed right heart catheterization, right ventricle angiography ,pulmonary arteriography and ascending aorta angiography.Right heart catheterization examination indicated right ventricular pressure (SBP 142mmHg, diastolic pressure − 8mmHg, mean pressure 42mmHg), right atrium pressure (SBP 20mmHg, diastolic pressure 6mmHg, mean pressure 10mmHg), pulmonary artery pressure (SBP 104mmHg, diastolic pressure 27mmHg, Mean pressure 52mmHg), PCWP 6mmHg, total pulmonary resistance 10.64 wood and minor pulmonary resistance 9.41 wood, and pulmonary hypertension was considered.Right ventricular angiography showed muscular trabecular hypertrophy, no stenosis of right ventricular outflow tract, pulmonary valve hypertrophy, limited opening, "vault sign" and "spray column sign"(Fig. 2,video1). Pulmonary angiography indicated that the right pulmonary artery was not developed, and the left pulmonary artery and its branches were dilated. ARPA was considered(Fig. 2,video1). Aortography suggested that multiple lateral branches of the descending aorta supplied the right lung field(Fig. 2,video1).Based on the above results, the clinical diagnosis is as follows: congenital heart disease, PVS, ARPA, pulmonary hypertension, collateral circulation of descending aorta supplying right lung.Combined cardiac surgery and thoracic surgery are recommended.