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Objective Investigate the clinical features,diagnosis and treatments of the scimitar syndrome,and different forms of treatment to alleviate pulmonary hypertension.Methods A retrospective analysis of clinical data of 14 children with scimitar syndrome from 2013 to 2017,including clinical symptoms and signs,chest X ray,echocardiography,chest CT and cardiac catheterization,treatment outcome and follow-up.Assess embolization of systemic pulmonary collateral and pulmonary venous drainage correction surgery,which is better for lowering pulmonary blood flow.Results 14 patients with scimitar syndrome were diagnosed from 2013 to 2017.There were 5 boys and 9 girls;3 cases ＜7 kg in weight.Scimitar syndrome was suspected because of extroversion,and diagnosed by color Doppler echocardiography and 13 of them confirmed by cadiac CT scan when ascimitar vein was detected entering the inferior vena cava.11 patiens had right lung dysplasia and 4 had horseshoe lung.Three patients had severe pulmonary arterial hypertension,3 had moderate to severe pulmonary arterial hypertension,and 2 had moderate pulmonary arterial hypertension,the left had slight pulmonary arterial hypertension.4 patients had pulmonary venous drainage correction surgery,after that 2 of them had systemic pulmonary collateral embolism.6 patients systemic pulmonary collateral embolism first,then 4 of them had surgical repair,1 case of 13 years old asymptomatic child without surgery.1 patient with heart failure,severe pulmonary hypertension,pulmonary infection,died before surgery,while another died after surgical repair.At last 1 patient was lost for follow-up visits.Systemic pulmonary collateral embolism and pulmonary venous drainage correction surgery could all reduce blood flow of pulmonary.After systemic pulmonary collateral embolism,patients had slight pulmonary arterial hypertension just need follow-up visits.Conclusion Clinically,found children with heart of dextrocardia position,growth retardation,recurrent lung infections,unexplained right heart failure,pulmonary hypertension,should consider the possibility scimitar syndrome.Whether pulmonary vascular embolization or surgical repair,can significantly reduce pulmonary artery's blood flow and alleviate pulmonary hypertension to protect pulmonary,even reduced the incidence of pneumonia and mortality.So we suppose ealy pulmonary hypertension in scimitar syndrome patients maybe dynamic pulmonary hypertension.
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Objective To summarize the clinical effects of one stop hybrid approach for treating the tetralogy of fallot complicated with aorta pulmonary collateral arteries. Methods From Janu 2008 to June 2013, 79 cases of tetralogy of fallot were complicated with aorta pulmonary collateral arteries with mean age (5.4±3.9) years and weight (18.2±5.7) kg. All patients were diagnosed by echocardiography examination and accepted 64-row CT for demonstration of no dysplasia in pulmonary ressels, McGoon index was (1.7±0.6) and Nakata index was (176.7±7.3). 76 cases had aorta pulmonary collateral arteries for preliminary screening, and accepted cardiovascular angiography before operation. 3 cases were misdiagnosed before surgery and were confirmed by cardiovascular angiography after operation. Results There were 4 deceased cases with an operative mortality of 5.1%. 1 case died of lung infection and 3 cases died of refractory heart failure. In all aorta pulmonary collateral arteries, the smallest diameter was 2.5 mm, the largest was 9.4 mm, average (5.3±2.1) mm. 2 to 21 coils were implanted in respective case with an average of 10.2 coils per case. The maximum number of coils implanted in 1 single ressel was 9. The average number of ressels occluded was 3.7 (range from 1 to 11 ressels) in each case. One case received second closure due to re-open of collateral arteries on neo-vasculanigation. 14 cases had lung infection and 3 cases had pulmonary edema post operation. 75 cases were followed up for 3-72 months. 4 cases with post operative heart function of NYHA gradeⅢtoⅣand all other cases with gradeⅡon higher. Conclusions Peri-operative hybrid management for aorta-pulmonary collateral arteries can increase the operative success rates and reduce complications.
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PURPOSE: The purpose of this study is to help determine the optimal time and method for operation of pulmonary atresia(PA) with ventricular septal defect(VSD). METHODS: Seventy patients who were diagnosed as PA with VSD in the Pediatric Department of Cardiology, Severance Hospital between May, 1991 and April, 1995, were included in our study. Subjects were divided into two groups depending on the presence of major aorto-pulmonary collateral arteries(MAPCAs). In patients with MAPCAs, the distribution of MAPCAs concerning its relationship with central PA were analyzed, while in patients without them, morphologic changes of PA after performing conventional modified Blalock-Taussig(B-T) shunt were studied. RESULTS: After performing modified B-T shunts, the number of cases of PA stenosis and interruption increased from 26 to 37. Fifteen cases of advanced stenosis and six cases of interrupted PA were also observed. After performing modified B-T shunt, CSAI of both PA was increased from 197+/-101 to 311+/-138, more remarkably in cases without progression of PA stenosis. Among the patients with MAPCAs, 12 cases showed PDA, while 16 cases showed confluent PA. On average, MAPCAs were present in 2.5 cases. Concerning the types of MAPCAs, 14 cases were unifocal, while 36 cases were multifocal and most of them originated from descending aorta. CONCLUSION: In cases where pulmonary flow is maintained by PDA, stenosis of central PA is common, and can result in retarded pulmonary arterial development or increased stricture after modified B-T shunt, so careful attention is needed during follow-up. In cases with dominant MAPCAs, confluent PA is common, and multifocal pulmonary supply renders surgery difficult, so careful analysis of pulmonary supplies to each pulmonary segments, their shapes and relationships, are indicated preoperatively.