Absence of Right Pulmonary Artery With Pulmonary Valve Stenosis and Partial Connection of Descending Aorta：A Case Report

Background: Unilateral absence of pulmonary artery is a rare congenital disorder that can remain asymptomatic until adulthood. Absence of right pulmonary artery (ARPA) has been reported in two-thirds of this patient population.It may be isolated, but it is more often associated with other congenital cardiac defects such as aortic coarctation, tetralogy of Fallot, atrial septal defect and patent ductus arteriosus. Case presentation: We are the first to introduce an adult case of ARPA with pulmonary valve stenosis（PVS） and associated with partial connection of descending aorta.We investigated the condition by right heart catheterization,right ventricle angiography, pulmonary arteriography and aorta angiography. According to the above results，we suggest that surgical treatment should be positively considered Conclusions: : The case remind that echocardiography combined with detailed imaging examination is very important for the diagnosis of complex congenital heart disease and provide the basis for the selection of the best treatment.