Long-term outcomes of percutaneous closure of patent ductus arteriosus associated with unilateral absence of a pulmonary artery.

OBJECTIVE: This study aimed to evaluate the long-term outcomes of patients with patent ductus arteriosus (PDA) associated with unilateral absence of a pulmonary artery (UAPA). METHODS: Patients diagnosed with PDA associated with UAPA between January 2005 and June 2019 were retrospectively enrolled in this study. Demographic and clinical characteristics, treatments, and follow-up information were evaluated. RESULTS: A total of 11 patients were diagnosed with PDA associated with UAPA. Percutaneous closure was successfully conducted in nine patients. The mean diameters of the PDA measured by aortogram and occluders were 5.3 ± 1.8 mm and 11.5 ± 3.9 mm, respectively. The median pulmonary systemic flow ratio (Qp:Qs) in five patients was 1.41, and the median total lung resistance was 12 Wood Units. The mean systolic pulmonary artery (PA) pressure was 68.3 ± 19.1 mmHg. In five patients with pre- and postprocedure catheter data, the systolic pulmonary arterial pressure decreased significantly after closure (from 77.0 ± 20.2 to 58.8 ± 17.5 mmHg; p = .024), as did the mean pulmonary arterial pressure (from 58.2 ± 14.6 to 39.0 ± 14.1 mmHg; p = .18). The PA pressure and heart size gradually decreased to normal levels in eight patients, and their quality of life was significantly improved. The ratio of lung to systemic circulation pressure was less than 0.75. CONCLUSIONS: In appropriate patients with PDA associated with UAPA, transcatheter closure of PDA has the potential to improve PA hypertension. A ratio of lung to systemic circulation pressure less than 0.75 may be an important reference index for predicting whether PA pressure can be reduced to a normal level after occlusion.

Hazards of ventricular pre-excitation to left ventricular systolic function and ventricular wall motion in children: analysis of 25 cases.

AimThe aim was to attach importance to the hazards of ventricular pre-excitation on left ventricular systolic function and size. METHOD: We analysed the clinical, electrophysiological, and echocardiographic characteristics of the 25 cases with abnormal ventricular wall motion, left ventricular systolic dysfunction, or dilation with co-existing right-sided overt accessary pathways before and after ablation or medication during March 2011 and June 2017. Moreover, we compared the therapy effect between patients with ventricular pre-excitation-induced dilated cardiomyopathy and idiopathic dilated cardiomyopathy without ventricular pre-excitation.ResultAbnormal ventricular wall motion was demonstrated using M-mode echocardiography in 23 cases. The basal segments of the interventricular septum became thin and moved similarly to an aneurysm with typical bulging during end-systole, which was observed in 16 cases. Dilated cardiomyopathy was diagnosed in 14 cases. A total of 23 patients underwent successful ablations and received medications, and the other two patients received only oral medications because of young age. The prognosis of pre-excitation-induced dilated cardiomyopathy is better than idiopathic dilated cardiomyopathy. All the cases with abnormal ventricular wall motion demonstrated recovery of normal left ventricular ejection fraction and decreased left ventricular end-diastolic diameter through ablation. CONCLUSION: Ventricular pre-excitation caused by right-sided accessory pathways may result in abnormal ventricular wall motion, left ventricular systolic dysfunction, dilation, and even dilated cardiomyopathy. In some cases with dilated cardiomyopathy, ventricular pre-excitation may not be the cause of disease but a harmful factor which hampered the recovering of left ventricular systolic function. These conditions are indications for ablation with good prognosis.

The effect of ventricular pre-excitation on ventricular wall motion and left ventricular systolic function.

Aims: The relationship between ventricular pre-excitation and left ventricular dysfunction has been described in the absence of sustained supraventricular tachycardia in a series of case reports. There have been no systematic studies about the effect of ventricular pre-excitation with different accessory pathway locations on ventricular wall motion and left ventricular (LV) systolic function. Methods and results: Thirty patients were selected for each of 4 groups, including those with right septal pathways (Group 1), right free-wall pathways (Group 2), left free-wall pathways (Group 3), and non-pre-excited patients undergoing electrophysiological evaluation for supraventricular tachycardia. We analysed the influence of the location of the accessory pathway on ventricular wall motion, systolic function, ventricular synchronism, and LV size. Right-sided accessory pathways were associated with abnormal motion of the interventricular septum, LV dyssynchrony, decreased LV systolic function, and increased LV diameter. Eighteen of 60 cases (30.0%) with right-sided accessory pathways had LV dyssynchrony, and these patients had lower LV ejection fraction and higher LV end-diastolic diameter. Conclusion: Right-sided accessory pathways may impair ventricular wall motion and LV systolic function, resulting in decreased LV ejection fraction and increased LV end-diastolic diameter. These effects occurred in patients with LV dyssynchrony. These effects, including LV dyssynchrony, resolved after radiofrequency ablation. A right-sided free-wall accessory pathway may have more detrimental effects than a septal accessory pathway. Left ventricular dyssynchrony and abnormal interventricular septal motion appeared to be responsible for the pathogenesis of LV dysfunction and remodelling.

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.

Common Genotypes of Long QT Syndrome in China and the Role of ECG Prediction.

OBJECTIVES: Genetic testing, a gold standard for long QT syndrome (LQTS) diagnosis, is time-consuming and costly when all the 15 candidate genes are screened. Since genotype-specific ECG patterns are present in most LQT1-3 mutation carriers, we tested the utility of ECG-guided genotyping in a large cohort of Chinese LQTS patients. METHODS AND RESULTS: We enrolled 230 patients (26 ± 17 years, 66% female) with a clinical diagnosis of LQTS. Genotypes were predicted as LQT1-3 based on the presence of ECG patterns typical for each genotype in 200 patients (85 LQT1, 110 LQT2 and 5 LQT3). Family-based genotype prediction was also conducted if gene-specific ECG patterns were found in other affected family members. Mutational screening identified 104 mutations (44% novel), i.e. 46 KCNQ1, 54 KCNH2 and 4 SCN5A mutations. The overall predictive accuracy of ECG-guided genotyping was 79% (157/200) and 79% (67/85), 78% (86/110) and 80% (4/5) for LQT1, LQT2 and LQT3, respectively. The predictive accuracy was 98% (42/43) when family-based ECG assessment was performed. CONCLUSIONS: From this large-scale genotyping study, we found that LQT2 is the most common genotype among the Chinese. Family-based ECG-guided genotyping is highly accurate. ECG-guided genotyping is time- and cost-effective. We therefore recommend it as an optimal approach for the genetic diagnosis of LQTS.

A Retrospective Study of 1,526 Cases of Transcatheter Occlusion of Patent Ductus Arteriosus.

BACKGROUND: Patent ductus arteriosus (PDA) is one of the most common congenital heart diseases and began to get treated by transcatheter occlusion since 1997 in China. Since then, several devices have been invented for occluding PDA. This study aimed to evaluate the technical feasibility, safety, and efficacy of transcatheter occlusion of PDA with different devices. METHODS: One thousand five hundred and twenty-six patients (537 boys, 989 girls) with PDA from January 1997 to September 2014 underwent descending aortogram and transcatheter occlusion procedure. We retrospectively analyzed data of these patients, including gender, age, weight, size and morphology of PDA, and devices used in transcatheter occlusion, outcomes, and postoperational complications. RESULTS: Median age and median weight were 4.0 years (range: 0.3-52.0 years old) and 15.3 kg (range: 4.5-91.0 kg), respectively. Mean ductal diameter, aortic ductal diameter, ductal length, and pulmonary artery pressure were 3.50 ± 2.15 mm, 10.08 ± 2.46 mm, 7.49 ± 3.02 mm, and 30.21 ± 17.28 mmHg, respectively. Morphology of PDA assessed by descending aortogram was of type A in 1428 patients, type B in 6 patients, type C in 79 patients, type D in 4 patients, and type E in 9 patients according to the classification of Krichenko. Of all the 1526 patients, 1497 patients underwent transcatheter PDA closure, among which 1492 were successful. Devices used were Amplatzer duct occluder I (ADO I, 1280, 85.8%), Cook detachable coils (116, 7.8%), ADO II (ADO II, 68, 4.6%), muscular VSD occluder (12, 0.8%), and Amplatzer vascular plug (16, 1.0%). CONCLUSIONS: Excellent occlusion rates with low complication rates were achieved with all devices regardless of PDA types. With transcatheter occlusion technique and devices developing, more patients with PDA can be treated with transcatheter closure both safely and efficiently.

Value of the Ratio of Occluder Versus Atrial Septal Length for Predicting Arrhythmia Occurrence after Transcatheter Closure in Children with Ostium Secundum Atrial Septal Defect.

BACKGROUND: Transcatheter occlusion has been applied to treat ostium secundum atrial septal defect (OS ASD) since 1997. During the clinical practice, several postoperative complications including arrhythmia have been reported. This study aimed to evaluate the value of the ratio of atrial septal occluder (ASO) versus atrial septal length (ASL) for predicting arrhythmia occurrence after transcatheter closure in children with OS ASD. METHODS: Six hundred and fifty-one children diagnosed with OS ASD underwent occlusion procedures after completing routine examinations. The onsets and types of arrhythmia both during and after the occlusion procedures were monitored. Treatments were given based on the individual types of arrhythmia. The binary logistic regression analysis and receiver-operating characteristic (ROC) curve were used in the analysis of value of the ratio of ASO/ASL for predicting postoperative arrhythmia occurrence. RESULTS: Transcather occlusions were conducted in 651 children, among whom 7 children had different types and degrees of arrhythmia, with an incidence of 1.1%. The types of arrhythmia included sinus bradycardia, atrial premature beats, bundle branch block, and different degrees of atrioventricular block. Normal electrocardiograph findings were resumed in these 7 patients following active therapies such as corticoids, nutrition, and surgeries. The binary logistic regression and ROC analysis suggested that the ratio of ASO/ASL exhibited an intermediate predictive value for predicting arrhythmia occurrence after occlusion procedures. A cut-off value of 0.576 in the ratio provided a sensitivity of 87.5% and a specificity of 76.2% with an area under the ROC curve of 0.791 (95% confidence intervals, 0.655-0.926; P < 0.05) in predicting arrhythmia occurrence after the closure procedures. CONCLUSIONS: The ratio of ASO/ASL might be a useful index for predicting arrhythmia occurrence after closure procedures in children with OS ASD.

[Adverse effects of type B ventricular pre-excitation on ventricular wall motion and left ventricular function: clinical analysis of 9 cases].

OBJECTIVE: To analyze the adverse effects of type B ventricular pre-excitation on ventricular wall motion and left ventricular function and its clinical characteristic. METHOD: The clinical, electrophysiological and echocardiographic characteristics of the 9 cases with type B ventricular pre-excitation before and after ablation seen between March 2011 and March 2013 were analyzed. The patients aged from 3 to 16 years. Five of them were female. RESULT: Dyschronous left ventricular contraction was demonstrated by M-Mode echocardiography in all of the cases. The basal segments of the interventricular septum turned thin and moved in a manner similar to that of an aneurysm, with typical bulging during end-systole, which was observed in six cases. All patients received successful RFCAs. The locations of the accessory pathways (APs) were the right-sided anteroseptum (n = 2) and the free wall (n = 7). Their physical activities and growth improved greatly in the four cases with coexisting dilated cardiomyopathy (DCM). The echocardiographic data demonstrated that their LV contraction recovered to synchrony shortly after the ablation, LVEF recovered to normal and LVED decreased to almost normal gradually during the follow-up. CONCLUSION: Overt right-sided APs may have adverse effects on ventricular wall motion and left ventricular function. They can even result in DCM. Dyssynchronous ventricular contraction induced by right-sided overt accessory pathway may be the vital mechanism. Such kinds of cases are indication for ablation with good prognosis.

Dyssynchronous ventricular contraction in Wolff-Parkinson-White syndrome: a risk factor for the development of dilated cardiomyopathy.

UNLABELLED: Emerging evidence suggests that significant left ventricular dysfunction may arise in right-sided septal or paraseptal accessory pathways (APs) with Wolff-Parkinson-White syndrome, even in the absence of recurrent or incessant tachycardia. During 1 year and 9 months, we identified four consecutive female children with median age of 8 years diagnosed as having dilated cardiomyopathy (DCM) combined with overt right-sided APs several years ago. Incessant or recurrent tachycardia as the cause of DCM could be excluded. Anti-heart failure chemotherapy did not produce satisfactory effects. The patients underwent radiofrequency ablations (RFCAs). This report describes the clinical and echocardiographic characteristics of the cases before and after the ablation. Dyssynchronous ventricular contraction was observed in all patients. The locations of the APs were the right-sided anteroseptum and the free wall (n = 2 each). All patients received successful RFCAs. Their physical activities and growth improved greatly, and the echocardiographic data demonstrated that their left ventricular (LV) contraction recovered to synchrony shortly after the ablation and that their LV function recovered to normal gradually during the follow-up. CONCLUSIONS: A causal relationship between overt ventricular preexcitation and the development of DCM is supported by the complete recovery of LV function and reversed LV remodeling after the loss of ventricular preexcitation. Preexcitation-related dyssynchrony was probably the crucial mechanism. Not only right-sided septal or paraseptal but also free wall overt APs may induce LV dysfunction and even DCM. AP-induced DCM is an indication for ablation with a good prognosis.

[Inhaled iloprost during acute pulmonary vasodilator testing for preoperative assessment of surgical operability of congenital heart disease with severe pulmonary hypertension].

OBJECTIVE: To evaluate the efficacy of iloprost in acute vasodilatation test during cardiac catheterization and to explore a useful hemodynamic indication regarding operability in the patients with severe pulmonary hypertension (PH) related to congenital heart disease (CHD). METHODS: The clinical data of 46 patients [mean age (12 ± 9) years] with severe PH related to CHD from June 2006 to December 2008 was retrospectively analyzed. All patients underwent standard right and left cardiac catheterization and a trial of inhaled iloprost test during cardiac catheterization. The mean pulmonary arterial pressure was (80 ± 13) mm Hg (1 mm Hg = 0.133 kPa) and pulmonary vascular resistance index was (17 ± 10) wood.m². A positive response to inhaled iloprost was defined as a decrease of at least 20% in pulmonary vascular resistance index (PVRI) without changes on systemic artery pressure. Patients with positive response to iloprost underwent cardiac surgical repair. The pulmonary artery pressure and PVRI was monitored by Swan-Ganz catheter postoperatively. RESULTS: Of the 46 patients, 29 (63.1%) showed a positive response after iloprost inhalation, defined by a significant reduction in PVRI from (15 ± 6) wood.m(2) at baseline to (9 ± 4) wood.m² in response to iloprost inhalation therapy (P < 0.05). The ratio of pulmonary to systemic resistance (Rp/Rs) decreased from 0.7 ± 0.2 to 0.4 ± 0.2 (P < 0.05). Seventeen patients (36.9%) didn't respond to iloprost displayed only little changes in PVRI [from (21 ± 10) wood.m(2) to (19 ± 9) wood.m²] and Rp/Rs (from 1.0 ± 0.5 to 0.9 ± 0.5). Out of 29 positive patients, 21 (72%) underwent successful cardiac surgical repair with a reduction of mean pulmonary arterial pressure (mPAP) to an average of (27 ± 10) mm Hg after the operation. Only 2 patients out of the 17 patients from the negative group were referred to surgery. Their mPAP was greater than 45 mm Hg. CONCLUSIONS: A significant reduction in pulmonary artery pressure after cardiac surgery was observed in patients with positive response to inhaled iloprost. Inhaled iloprost may be a valuable tool in the preoperative evaluation of patients with severe PH related to CHD.