Abstract:

Objective To analyze the clinical characteristics and prognosis of PAPVC-IAS in order to improve the understanding of the disease. Methods A total of 16 children with PAPVC-IAS confirmed by echocardiography, cardiac CTA or surgery from June 2014 to June 2024 were collected. Their clinical and imaging features, as well as prognosis were analyzed. Results Among the 16 cases, there were 7males and 9 females. The age of the first diagnosis was from 4 to 101months. The most common type of ectopic drainage was the supracardiac type, including 7 cases (43.7%) of left superior pulmonary vein draining into left innominate vein through vertical vein, 3 cases (18.8%) of ectopic drainage of the right upper pulmonary vein into the superior vena cava; followed by infracardiac type, 4 cases (25.0%) of ectopic drainage of right inferior pulmonary vein to inferior vena cava; and the most rare is the intracardiac type, 2 cases (12.5%) of pulmonary vein directly flowing into right atrium. It was reported that the right atrium and right ventricle were slightly enlarged, and 31.2% (5/16) of the patients were associated with mild pulmonary hypertension. Surgical treatment can be given to children with surgical indications. PAPVC repair is a relatively safe operation with good outcomes and few complications. Conclusions PAPVC-IAS is easy to be missed in the early stage because of mild clinical symptoms. If unexplained cardiac enlargement or unexplained pulmonary hypertension is found, the diagnosis of the disease should be considered. The prognosis of the disease is good after surgical treatment.

Key words: intact atrial septum, partial anomalous pulmonary venous connection, cardiovascular abnormalities