Predictors of operability in children with severe pulmonary hypertension associated with congenital heart disease / 中华医学杂志(英文版)

BACKGROUND@#Pulmonary artery hypertension associated with congenital heart disease (PAH-CHD) occurs predominantly among patients with uncorrected CHD. Treatment of severe pediatric PAH-CHD remains a major intractability. This study evaluated the predictors and prognoses of children with PAH-CHD who underwent surgical correction.@*METHODS@#The data for 59 children with severe PAH-CHD who underwent surgical correction, with or without postoperative medication, between May 2011 and June 2015 at the Guangdong Provincial People's Hospital were analyzed retrospectively. A regression analysis, receiver-operating characteristic (ROC) curves, and Kaplan-Meier curves were used for survival analysis.@*RESULTS@#Fifty-nine children with severe PAH-CHD underwent heart catheterization and correction, with or without specific anti-PAH drugs postoperatively, were included in this study. The pulmonary pressure, heart function, and ending events were observed and median observation period was 49 ± 20 months. Twenty-eight patients (50%) received at least one additional anti-PAH drug after correction. The survival rate after 2 years was 91.5% (54/59); two patients were in a critical condition, and three were lost to follow-up. Twelve patients (29%) still received over one additional PAH-specific therapy at follow-up, whereas 42 (75%) had successfully stopped drug treatment. Two patients (3.5%) died and one underwent a second thoracotomy to remove the ventricular septal defect patch. Acute vasoreactivity test (AVT) criteria had limited efficacy in predicting pediatric PAH-CHD, whereas pulmonary vascular resistance (PVR) ≤ 6.65 Wood units (WU)/m or PVR/systemic vascular resistance (SVR) ≤ 0.39 during AVT indicated a good prognosis after surgical correction with an AUC of 98.3% (95% confidence interval [CI]: 96.0-100%), 98.4% (95% CI: 96.0-100%) sensitivity of 100%, 100% and specificity of 82.1%, 92.9%, respectively.@*CONCLUSIONS@#Although the criteria for positive AVT currently used are unsuitable for pediatric patients with PAH-CHD, PVR and PVR/SVR during AVT are excellent predictors of outcome in pediatric PAH-CHD. Surgery aided by anti-PAH drugs is an effective strategy and should be recommended for severe pediatric PAH-CHD with PVR ≤ 6.65 WU/m and PVR/SVR ≤ 0.39 after iloprost aerosol inhalation.

Peg-IFNa-2a/RBV antiviral efficacy in cirrhotic hepatitis C patients after splenectomy or partial splenic embolization / 中华肝脏病杂志

To investigate the antiviral efficacy of combination therapy with pegylated-interferon alpha (peg-IFNa)-2a and ribavirin (RBV) in hepatitis C patients with liver cirrhosis after splenectomy or partial splenic embolization. Forty-nine hepatitis C patients with liver cirrhosis who were unable to use antiviral therapy because of hypersplenism were recruited for study and treated with splenectomy or partial splenic embolization. Three months later, a regimen of antiviral combination therapy was initiated with peg-IFNa-2a (once-weekly subcutaneous injection: 135 μg or 180 μg) and RBV (daily oral: 800 to 1200 mg), and was maintained for 48 weeks. The patients were followed up at treatment weeks 1, 2, 4, 6, 8, and 12. Thereafter, follow-up was conducted every four weeks. The patients were observed until 24 weeks after treatment discontinuation. Follow-up testing included liver function, blood chemistry, renal function, and HCV RNA level. Any adverse reactions were recorded. Liver cirrhosis patients complicated by hypersplenism can be treated effectively with peg-IFNa-2a/RBV combination antiviral therapy after splenectomy or partial splenic embolization. The antiviral-induced sustained viral response rates was 65.00% in cirrhotic/hypersplenic hepatitis C patients receiving splenectomy and 58.62% in those receiving partial splenic embolization. Hypersplenism patients with hepatitis C-related cirrhosis achieved a good antiviral therapeutic effect with peg-IFNa-2a/RBV combination therapy following splenectomy or partial splenic embolization. This sequence of treatment may help to decrease incidences of chronic hepatitis C-induced liver failure and liver cancer in these patients.

Interventional catheterization management for patients with postoperative residual cardiovascular malformations / 中华心血管病杂志

<p><b>OBJECTIVE</b>To evaluate the efficacy of interventional therapy for complex congenital heart defects patients with un-repaired or postoperative residual lesions.</p><p><b>METHODS</b>Between March 1998 and April 2009, 42 patients (12 females), mean age 6 years (5 months to 30 years) received interventional therapy, 17 cases underwent occlusion of major aorto-pulmonary collateral arteries (MAPCAs), 15 underwent device closure of residual ventricular or atrial septal shunting, 12 underwent balloon angioplasty (n = 10) and stenting (n = 2) for stenosis of the anastomosis of vessels or branched pulmonary arteries.</p><p><b>RESULTS</b>Twenty-three MAPCAs were performed in 17 patients without residual shunting. One patient died of multiple organ failure after intervention therapy and the remaining patients discharged without complication, successful device closure was performed in 15 patients and there was minimal residual shunting in 1 patient. There were no severe arrhythmias such as complete atrio-ventricular block during and post procedure. Exercise capacities were significantly improved in 12 patients underwent balloon angioplasty or stenting. Pressure gradients were significantly decreased and there was no aneurysmal or thromboembolic formation post procedure.</p><p><b>CONCLUSIONS</b>Interventional therapy is a safe and effective therapy option for treating complex congenital heart defects patients with un-repaired or postoperative residual lesions.</p>

Effects of transcatheter closure in children with congenital coronary artery fistula / 中华心血管病杂志

<p><b>OBJECTIVE</b>To observe the effects of transcatheter closure method for treating congenital coronary artery fistula (CAF) in children.</p><p><b>METHODS</b>Twenty-three children with CAF received transcatheter closure. Under anesthesia, heart catheterization and selective coronary angiography were performed to show the CAF size and relationship with normal coronary artery. CAF with the narrowest inner diameter < 3 mm (n = 16) were occluded with coil device, and CAF with narrowest inner diameter > 3 mm (n = 7) were closed with Amplatzer duct or VSD occluder.</p><p><b>RESULTS</b>Transcatheter closure was successfully performed in 21 cases and failed in 2 cases (CAF is too tortuous in one case and right CAF outlet near the right coronary artery main stem in another case) and CAF were closed by surgery in these 2 patients. No residual shunt or other complications were observed during the 3 months to 3 years follow up.</p><p><b>CONCLUSION</b>Transcatheter closure was an effective and mini-traumatic method for CAF treatment in children.</p>

Applying interventional treatment for the atrial septal defect in 165 children under five years of age / 中华儿科杂志

<p><b>OBJECTIVE</b>To study the clinical technology of transcatheter closure of secundum atrial septal defects (ASD) with Amplatzer device in younger and lower body weight children.</p><p><b>METHODS</b>The transcatheter closure of ASD using Amplatzer septal occluder (ASO) was performed in 165 children under 5 years of age (75 boys and 90 girls) with secundum ASD from Aug 1998 to May 2004. The age of the cases ranged from 2 to 5 (mean 3.7 +/- 1.1) years. The body weight ranged from 9 to 18 (mean 12.6 +/- 2.3) kg. The ratio of pulmonary circulation quantity to the systemic circulation quantity (Qp/Qs) was 3.2 +/- 1.9. All the patients underwent clinical examination, X-ray, electrocardiography (ECG) and echocardiography (Echo) for diagnosis of secundum ASD. The transthoracic echocardiography (TTE) was used to detect and measure the defect of the patients and even trans-esophageal echocardiography (TEE) had to be used when it was necessary. With Echo and X-ray guidance, the measuring balloon was used in the body and outside the body to determine the balloon-stretch diameters of ASD, and proper occluders were selected accordingly for the patients for interventional treatment of ASD.</p><p><b>RESULTS</b>The devices were implanted successfully in 163 (98.8%) cases. One failure occurred in a case in whom the device moved into the left atrium after release, and the other failure was that the position of the device was uncertain because of temporary unavailability of a special transducer for TEE. Surgical operations were performed for these two cases. The stretch diameter of ASD was from (8 - 30) mm, (mean 18.3 +/- 5.1) mm. The size of device was selected according to the stretch diameter of ASD. The diameter of the occluders selected was from (8 - 30), (mean 18.6 +/- 5) mm in this series. The occlusion procedure was monitored by fluoroscopy and TTE and in 5 cases (3%) by TEE. The diameter of right ventricle was improved within 2 days after occlusion from (mean 16.4 +/- 4.9) mm to (mean 12.6 +/- 3.8) mm, (p < 0.01). One hundred and forty seven cases belonged to the simple secundum ASD(89%). Thirteen cases who were complicated with other cardiac deformity were treated successfully with different interventional procedure. Six cases had multiple openings and three of these cases had tumour-like changes of the atrial septum which were closed completely just by one occluder. In only one case small quantity of residual shunt remains. No other severe complication was found in this group. About 100 cases (60%) had large ASD, so the procedure was more difficult in those cases.</p><p><b>CONCLUSION</b>The clinical effectiveness of treatment of ASD in children under 5 years of age with Amplatzer occluders was satisfactory and therefore this therapeutic procedure is feasible for this age group of patients. Nevertheless, we do not recommend to use the technique for infants and children under 2 years of age. Strict selection of indications and proper size of occluder and good cardiologic and surgical settings are among the basic factors for successful interventional occlusion of ASD in young children.</p>

The development of a new perimembranous ventricular septal defect occluder / 中华心血管病杂志

<p><b>OBJECTIVE</b>The aim of this study was to develop a new perimembranous VSD occluder and to evaluate it.</p><p><b>METHODS</b>The shape of VSD occluder was designed as fabric frame "I" shape that comprised two types: symmetric and asymmetric. The safety, efficacy, feasibility and complication were tested in 22 animal models and in 58 VSD patients in clinical trial. The device were compared with Amplatzer occluder in this study.</p><p><b>RESULTS</b>The new perimembranous VSD occluder was passed the national material test. In animal study, artificial VSD were all occluded by using the new devices with no complication in follow up except one pig expresented wound infection. In clinical trial, all 58 VSD cases were healing with the new device. One patient suffered with atria-ventricular block 5 days after procedure and was free from AV block with medicine therapy. Compared with Amplatzer perimembranous VSD occluder, the new devices had lower frequency of residual shunt.</p><p><b>CONCLUSION</b>The new perimembranous VSD occluder is a safe and effective perimembranous VSD interventional apparatus, and the effect of the new occluders seems not worse than that of the Amplatzer ones.</p>

Management of the arrhythmia around the procedure of transcatheter closure of ventricular septal defects in pediatric patients / 中华心血管病杂志

<p><b>OBJECTIVE</b>To discuss the treatment of arrhythmia occurs in the process of transcatheter closure of perimembranous ventricular septal defects (VSD) in pediatric patients.</p><p><b>METHODS</b>182 cases (mean age: 6.2 +/- 3.3 years) with membranous VSD underwent transcatheter occlusion procedure. Two different devices were used: the Amplatzer membranous VSD occluder in 81 patients and the domestic-made device in 101 patients. Electrocardiogram of all patients was recorded before and during closure and at one day after the procedure, and Holter monitoring was performed one week after the procedure.</p><p><b>RESULTS</b>Perioperation arrhythmia occurred in thirty-one patients (17%). Second- or third-degree atrioventricular bundle (AVB) was noted during the procedure in four patients. Normal AV conduction recovered spontaneously before the catheters were withdrawn in three cases and another patient underwent surgical repair. In the other twenty-seven patients, arrhythmia was first documented between one day and one week after the procedure. Third-degree AVB was found in three (1.6%) children after the procedure and underwent the temporary pacemaker (TPM) was implanted, two of them recovered to normal sinus rhythm within one week, another patient underwent elective surgery to remove the occluder and repair the defect. Other arrhythmias were: left bundle-branch block (n = 3), right bundle-branch block (n = 12), second-degree AVB (n = 2), sinus tachycardia (n = 6).</p><p><b>CONCLUSIONS</b>In properly selected cases of perimembranous VSD, the transcatheter closure is safe and effective by using appropriate devices. During and after the procedure, closure of VSD can be associated with some kinds of arrhythmia, such as A-V block, more intensive observation and follow-up were therefore needed.</p>