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

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.

Congenital pulmonary vein atresia / 中华实用儿科临床杂志

Congenital pulmonary vein atresia (PVA) is a rare anomaly in clinic.Patients with congenital PVA are usually symptomatic and present with recurrent episodes of pneumonia or haemoptysis in infancy or childhood.Because PVA often occurs in the setting of other congenital heart lesions,so repeated infection of lung might be considered to associate with left-to-right intracardiac shunt with increased pulmonary blood,but ignore further examination of more rare cause.Diagnosis of PVA is based primarily on imaging test,chest radiograph,lung CT and fiberoptic bronchoscopy might reveal some indirect characteristic changes of the disease.Three dimensional contrast enhanced cardiac CT can make a definite diagnosis.The disease is difficult to cure at present,the aim of the treatment is to preserve lung function.The prognosis is associated with the number of involved pulmonary veins.

Individual Pulmonary Vein Atresia in Adults: Report of Two Cases

We present two cases of individual pulmonary vein atresia without vestige of an involved pulmonary vein. On CT, we noted the absence or interruption of normal pulmonary venous structures, and the presence of abnormal vascular structures that represented collaterals for the involved lung parenchyma. On angiography, the atretic pulmonary vein was found to drain into the other ipsilateral pulmonary veins through the collaterals.

A Case of Congenital Unilateral Pulmonary Vein Atresia

Congenital unilateral pulmonary vein atresia is a very rare cardiac malformation. In this case, the patient was brought to the hospital for recurrent upper respiratory infection without exertional dyspnea or hemoptysis, and cardiac murmurs were heard on physical examination. The volume of affected lung was lost on simple chest x-ray. Lung perfusion scan revealed no visualization of affected lung. Cardiac catheterization revealed mild pulmonary artery hypertension. Pulmonary angiography showed a small right pulmonary artery, stasis of contrast material and venous phase revealed nonvisualization of draining right pulmonary veins. In this case, the patient did not undergo any surgical treatment for no symptoms other than recurrent upper respiratory infection were reported. We report a case of congenital right pulmonary vein atresia.

Unilateral Pulmonary Vein Atresia with Absent Right Superior Vena Cava

A case of unilateral pulmonary vein atresia with absent right superior vena cava in 9 years-old boy is described with a discussion. Clinical findings were similar to other reports and consisted of hemoptysis, dyspnea and anemia. A chest X-rary revealed irregular vascular marking on left lung fiedld and increased interstitial marking on right upper lung field. The diagnosis could be confirmed with radionuclide perfusion study, MRI scan of heart and great vessels and angiography which demonstrated a small right pulmonary artery, pruned its peipheral branches, stasis of contrast material and nonvisualization of draining righ pulmonary veins. Pneumonectomy, patch angioplasty of diaphargm excision will be necessary.