Balloon Angioplasty as a Modality to Treat Children with Pulmonary Stenosis Secondary to Complex Congenital Heart Diseases

Background： Pulmonary stenosis is common in children with complex congenital heart diseases. Proper management of this problem, especially postoperatively, is still controversial. This study was designed to assess the rate and determinants of success or failure of balloon angioplasty for such lesions. Methods： Clinical and hemodynamic data from 40 pediatric patients （24 boys and 16 girls） with complex congenital heart diseases who underwent balloon angioplasty were reviewed retrospectively from January 2012 to December 2016. Patients were divided into four groups according to the site of stenosis, which included pulmonary valve stenosis （PVS）, valved conduit stenosis, pulmonary artery stenosis （PAS）, and supravalvular pulmonary stenosis （SVPS）. Success rates were calculated according to defined criteria for initial success and favorable clinical impacts, and comparison between the successful subgroup and the unsuccessful subgroups was analyzed. Results： Grouped by the site of stenosis, initial success rates varied from 40.0% to 52.4% with the greatest success being seen in the PVS group, followed by the PAS group and SVPS group. In the PVS group and the PAS group, there was no statistical difference among age at dilation, postoperative interval, balloon/stenosis ratio, or pressure gradient predilation between the successful and the unsuccessful subgroups. Favorable clinical impacts included success rates of balloon angioplasty in the SVPS group, which was best （100%）, followed by the PVS group （90.9%） and the PAS group （85.7%）. There were a total of two transient complications （5.0%）. Conclusions： Balloon angioplasty was proven to be a safe and useful modality in children with complex congenital heart diseases and postoperative pulmonary stenosis, which should be the initial therapeutic modality in selected patients.

Assessment of Intracardiac and Extracardiac Deformities in Patients with Various Types of Pulmonary Atresia by Dual-Source Computed Tomography

Background:Pulmonary atresia(PA)is a group of heterogeneous complex congenital heart disease.Only one study modality might not get a correct diagnosis.This study aims to investigate the diagnostic power of dualsource computed tomography(DSCT)for all intracardiac and extracardiac deformities in patients with PA compared with transthoracic echocardiography(TTE).Materials and Methods:This retrospective study enrolled 79 patients and divided them into three groups according to their main diagnosis.All associated malformations and clinical information,including treatments,were recorded and compared among the three groups.The diagnostic power of DSCT and TTE on all associated malformations were compared.The surgical index(McGoon ratio,pulmonary arterials index(PAI),and total neopulmonary arterial index)and radiation dose were calculated on the basis of DSCT.Results:Of the patients,32,30,and 17 were divided into the groups of PA with ventricular septal defect(VSD),PA with VSD and major aortopulmonary collateral arteries,and PA with other major malformations,respectively.Consequently,182,162,and 13 intracardiac,extracardiac,and other major malformations were diagnosed,respectively.Moreover,DSCT showed a better diagnostic performance in extracardiac deformities(154 vs.117,p<0.001),whereas TTE could diagnose intracardiac deformities better(159 vs.139,p=0.001).The McGoon ratio,PAI,and treatment methods were significantly different among the three groups(p=0.014,p=0.008,and p=0.018,respectively).Conclusion:More than one imaging modality should be used to make a correct diagnosis when clinically suspecting PA.DSCT is superior to TTE in diagnosing extracardiac deformities and could be used to roughly calculate surgical indices to optimize treatment strategy.

The application of aortopulmonary shunt in cyanotic congenital heart disease with severe pulmonary hypoplasia in adolescents and adults

Background Pulmonary arterial hypoplasia is a common complication in complex cyanotic congenital heart disease. For extreme stenosis of the branch pulmonary arteries,which is not feasible for complete repair,palliative systemic-pulmonary shunt is needed to increase pulmonary blood flow and pulmonary tree growth as early as beyond 2 years old. Unfortunately,due to poor medical setting in local hospitals,there are still a small number of patient with severe hypoplastic pulmonary arteries in developing area who have failed to undergo appropriate surgical intervention till to teenagers even adults. In order to explore the resolution of the dilemma for these notable cyanotic patients,hence we began to utilize three-dimensional computed tomography(CT)to reconstruct pulmonary artery anatomy and to simulate virtual palliative systemic pulmonary shunt conduit module,to facilitate and improve the intraoperative aortopulmonary shunt performance. Methods FromApril 2011 to August 2018,13 consecutive patients undergoing aortopulmonary shunt with older age(13-35 years old)who missed the optimal timing for surgery were identified from 196 cases involving palliative systemic pulmonary shunt. An individually pre-designed prosthetic expanded poly tetra fluoroethylene(ePTFE)conduit was utilized based on the threedimensional enhanced computed tomography reconstruction and simulation. The post-operative recovery courses and complications were documented. Blood gas analysis,electrocardiogram,echocardiography were performed routinely prior to discharge and compared with the preoperative data. Re-evaluation of finger pulse saturation(SpO2),echocardiography and electrocardiogram was performed in clinical follow-up in 1 st month,6 th month,12 th month and every year postoperatively. A retrospective analysis of operative data,postoperative outcomes and complications were performed. Results All the 13 consecutive patients underwent successfully non-cardiopulmonary bypass systemic-pulmonary shunt with ePTFE conduits via median sternotomy. Postoperative thirtyday mortality was 1 in 13 due to pulmonary hemorrhage. During the follow-up,no more mortality was documented but a re-intervention for ePTFE conduit revision. For the 12 survivors,the postoperative SpO2,and arterial partial oxygen pressure(PaO2)on room air significantly increased from 68.0±2.42% to 88.46±4.67%(P<0.01),and from 42.61±3.94 mmhg to 49.62±1.76 mmhg(P<0.01),respectively. While the postoperative hematocrit and hemoglobin significantly decreased from 72.01±3.12% to 61.03±3.21%(P<0.01),and from 196.77±10.56 g/dL to 171.76±6.52 g/dL(P<0.01),respectively. Conclusions Appropriate systemic-pulmonary shunt based on threedimensional reconstruction and simulation can significantly alleviate the hypoxia with elevated oxygen saturation for severe cyanotic adolescent or adult patient with extreme pulmonary hypoplasia and unrepairable complex congenital heart disease,so as to improve their clinical symptoms and life quality,although it cannot promote secondary pulmonary artery development directly.