Unilateral pulmonary vein atresia of children:4 cases report and literature review / 中华实用儿科临床杂志

ABSTRACT

Objective To summarize the clinical characteristics,imaging characteristics,treatment and progno-sis of unilateral pulmonary vein atresia (UPVA)in children and to improve the clinician′ s understanding of this disease. Methods The clinical data of 4 cases of UPVA from January 2014 to December 2016 in Department of Cardiology,Guangzhou Women and Children′s Medical Center were retrospectively analyzed,and 50 cases from reviews of PubMed,OVID and Elsevier in the international medical literature database and 4 cases in Wanfang database for the domestic report were reviewed. The clinical characteristics,diagnosis,treatment and prognosis of total of 58 cases were analyzed. Results Four patients,with an average age of 1. 8 years (1. 5 - 2. 7 years),showing congenital UPVA in 3 patients and secondary UPVA in 1 patient. There was 1 case of left upper pulmonary vein atresia,2 cases of left pulmo-nary vein atresia and 1 case of right pulmonary vein atresia. Three cases showed recurrent hemoptysis and recurrent cough occurred in 1 case. Three cases were complicated with congenital heart disease. There was one case underwent bronchial artery embolization,1 case received pulmonary vein left atrium connection,and 2 cases received conservative treatment. All patients had been followed up for 1 - 3 years so far. The patient receiving pulmonary vein left atrium had been completely cured,and the patient receiving bronchial artery embolization showed no occurrence of hemoptysis but still showed recurrent cough hemoptysis. The patient with secondary UPVA had no obvious clinical symptoms,the other 1 case who rejected operation and received conservative treatment still had recurrent pulmonary infection,intermittent hemoptysis. The average age of 54 cases(40 cases with age ≤18 years old)from the literature reports was 13. 76 years (8 days - 43 years)in which 52 cases were diagnosed as congenital UPVA,while 2 cases were secondary UPVA. Twenty - seven cases were right pulmonary vein atresia,22 cases were left pulmonary vein atresia,and 5 cases were other types. There were 94. 4％(51 / 54 cases)of the patients having recurrent cough,pulmonary infection,92. 6％(50 /54 cases)of the patients with exertional dyspnea and polypnea,68. 5％(37 / 54 cases)of the patients with hemoptysis and hematemesis. There were 50. 0％(27 / 54 cases)of UPVA patients who were complicated with heart malformation. Different degrees of pulmonary hypertension were observed in 75. 9％(41 / 54 cases)of children,and 35. 2％(19 / 54 cases)of patients had pulmonary lymphatic dilatation. Pulmonary resection was performed in 25 cases,pulmonary vein left atrium connection was performed in 11 cases,bronchial artery embolization was performed in 7 cases,and conserva-tive treatment was performed in 11 cases. After operation,most of the patients had good prognosis without obvious clini-cal symptoms or mild symptoms. Conclusions In clinical practice,if unexplained hemoptysis,recurrent lower respira-tory tract infection,pulmonary consolidation,pulmonary dysplasia or pulmonary hypertension present,the possibility of UPVA should be considered. Early diagnosis and early bronchial artery embolization,reconstruction of the pulmonary vein and atrial connection and repair of the defect of heart,can improve the symptoms or cure the children and reduce the morta-lity significantly.