Search

In patients with pulmonary artery hypertension (PAH) associated with atrial septal defect (ASD), closure of ASD may carry significant risks. We aimed to evaluate the safety and efficacy of transcatheter closure of ASD in selected patients with PAH using a fenestrated device followed by pulmonary vasodilator therapy. During the 14.8-year period, 51 ASD patients (10 males, age 46 ± 18 years) with a mean pulmonary artery pressure (PAP) ≥ 35 mm Hg and/or systolic PAP ≥ 60 mm Hg, underwent closure with a fenestrated device. Of them, mean Qp/Qs ratio, systolic PAP and mean PAP were 2.6 ± 1.2, 73 ± 14 mm Hg, and 44 ± 8 mm Hg, respectively. A total of 35 patients received pulmonary vasodilator therapy. The New York Heart Association (NYHA) functional class improved at 3 to 6 months follow-up. (p < 0.001) Nineteen patients underwent repeated catheterization. A comparison of the hemodynamic parameters between baseline and repeated catheterization revealed significant decreases in both systolic and mean PAP (77 ± 11 vs 55 ± 14 mm Hg, p < 0.0001 & 48 ± 7 vs 37 ± 8 mm Hg, p = 0.001, respectively), pulmonary vascular resistance (PVR) (5.1 ± 2.3 vs 4.0 ± 1.7 WU, p = 0.011) and PVRi (7.7 ± 3.3 vs 6.2 ± 2.4 WU*m2, p = 0.024). After a follow-up period of 84 ± 45 months, 6 mortalities were noted in which 2 were due to cardiac causes. In conclusion, catheter closure of ASD in patients with PAH using a fenestrated device followed by vasodilator therapy is safe and effective., (Copyright © 2021. Published by Elsevier Inc.)

Patients with coarctation of the aorta (CoA) may develop hypertension and cerebrovascular accident (CVA) late after intervention, but the risk is still unclear. Therefore, we studied the epidemiologic profile from a general population aged <60 years to assess the relative risk. Our study population consisted of 126,943 patients with congenital heart disease (CHD) derived from Taiwan's National Health Insurance database from 2000 to 2010 (population 22,765,535). There were 2,295 patients with CoA (50.3% men) (prevalence: 0.116/1000). Associated cardiac lesions in 44% of the patients with CoA included ventricular septal defect (VSD; 23.3%), patent ductus arteriosus (7.5%), atrial septal defect (9.0%), and so forth. Hypertension occurred in 190 patients (8.3%) and was related to age, without associated VSD (odds ratio [OR] 5.90, 95% confidence interval [CI] 2.12 to 16.39, p = 0.001) and male gender (OR 1.59, 95% CI 1.09 to 2.30, p = 0.015). The incidence of hypertension increased rapidly in the young adulthood and was 1.36% in pediatric age group and 28.9% and 45.7% in age group 20 to 29 and 30 to 39, respectively. CVA occurred in 29 patients (1.67%), and the coexisting hypertension was the single predictor (OR 3.19, 95% CI 1.13 to 9.00, p = 0.029). Furthermore, in all patients with CHD, CoA was an independent risk factor for hypertension (OR 15.65, 95% CI 12.44 to 19.68, p <0.001) and CVA (OR 6.55, 95% CI 4.41 to 9.74, p <0.001). Comparing with non-CoA CHD patients, patients with CoA, particularly men, adult, and patients without VSD, have high risk of hypertension, which would further increase the risk of CVA. Early recognition and timely intervention for the hypertension in patients with CoA is mandatory., (Copyright © 2015 Elsevier Inc. All rights reserved.)

Epicardial adipose tissue (EAT) contains ganglionated plexuses and adipocytes that can affect the pathogenesis of atrial fibrillation (AF). The aim of this study was to quantify the EAT surrounding the left atrium (LA) and correlate it with occurrence of AF and outcome after catheter ablation. EAT was evaluated using 64-slice multidetector computed tomography in 68 patients with AF and 34 controls. EAT volume was acquired by semiautomatically tracing axial images from the pulmonary artery to the coronary sinus. Topographic distribution of EAT was assessed by dividing the periatrial space into 8 equal regions. EAT volume significantly increased in patients with AF than in controls (29.9 ± 12.1 vs 20.2 ± 6.5 cm(3), p <0.001). Most EAT was located in regions (1) within the superior vena cava, right pulmonary artery, and right-sided roof of the LA (29.8%), (2) within the aortic root, pulmonary trunk, and left atrial appendage (26.5%), and (3) between the left inferior pulmonary vein and left atrioventricular groove (18.1%). Baseline variables were analyzed in patients with (n = 24) and without (n = 44) AF recurrence after ablation. The recurrent group showed significantly increased EAT (35.2 ± 12.5 vs 26.8 ± 11.1 cm(3), p = 0.007). Multivariate analysis revealed that EAT was an independent predictor of AF recurrence after ablation (p = 0.038). In conclusion, EAT of LA was increased in patients with AF. Large clusters of EAT were observed adjacent to the anterior roof, left atrial appendage, and lateral mitral isthmus. Abundance of EAT was independently related to AF recurrence after ablation., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Late cardiovascular complications after intervention for coarctation of the aorta (CoA) might be common. Such data, especially in Asian populations, are still limited. A total of 169 patients with CoA who survived balloon (n = 41) or surgical (n = 128) intervention from 1986 to 2004 were enrolled. The total follow-up was 1,776 patient-years, and the patient age at last follow-up was 14.7 +/- 8.5 years. Reintervention for recoarctation, systemic hypertension, and left ventricular outflow tract obstruction (LVOTO) was subsequently noted in 57 (34%), 36 (21.3%), and 24 (14.2%) patients, respectively, without significant differences between the balloon and surgical groups. The 20-year freedom from reintervention, systemic hypertension, and LVOTO rate was 52%, 78.6%, and 80.8% in the balloon group and 59%, 51%, and 81.9% in the surgical group, respectively. The age at CoA intervention and the aortic geometry after CoA intervention of gothic type were common risk factors for reintervention for recoarctation and late systemic hypertension. In contrast, the presence of bicuspid aortic valves and gothic arch geometry after CoA intervention increased the risk of LVOTO. Plasma renin/aldosterone activities were not elevated in those with or without systemic hypertension. In conclusion, the profile of reintervention for recoarctation and late systemic hypertension after CoA interventions in our Asian cohort with CoA was similar to that observed in white patients. Asian patients might have a lower risk of LVOTO.

We explored the role of QRS prolongation (>or=120 ms) and its relation to mechanical dyssynchrony and outcomes in childhood idiopathic dilated cardiomyopathy (IDC). A total of 89 patients 32.6 ms was used to define intra-LV dyssynchrony. The 1- and 5-year survivals were 70% and 53%, respectively. Requirement of intravenous inotropes at follow-up (hazard ratio 3.10) and initial LV ejection fraction (hazard ratio 0.95) were major prognostic factors. QRS prolongation, primarily left bundle branch block, was identified in 16 patients (18%) and tended to increase the risk of requiring inotropes. Moreover, none of those with QRS prolongation regained normal cardiac function at follow-up. Two patients with QRS prolongation showed marked improvement in cardiac function after cardiac resynchronization therapy. Mechanical dyssynchrony was noted in all patients with QRS prolongation and in 8% (interventricular) or 38% (intra-LV) of those without. In conclusion, QRS prolongation was common in childhood IDC and was possibly associated with persistent LV dysfunction and worse cardiac outcome. Mechanical (inter- and intraventricular) dyssynchrony was highly prevalent in those with QRS prolongation and was still often observed in those without.

This study was undertaken to delineate tracheobronchial anomalies associated with congenital heart disease. From June 1995 to December 2000, 1,245 children with congenital heart disease underwent cardiac electron beam computed tomography with 3-dimensional reconstruction on an independent workstation. Tracheobronchial anomalies are strongly associated with congenital heart disease and accompanying tracheal stenosis is not uncommon. With 3-dimensional reconstruction, electron beam computed tomography provided excellent anatomic definition of the central tracheobronchial abnormalities.

Three main patterns of aortic sinus rotation were defined on 517 lateral angiograms of tetralogy of Fallot with 14 cases of anomalous coronary artery pattern occurring only in patients with a right anterior or right lateral aorta. The significant dependence of coronary artery types on the aortic sinus pattern made it possible to predict the predisposing coronary artery pattern in tetralogy of Fallot based on the aortic sinus pattern.

The results of transcatheter valvotomy in pulmonary atresia with intact ventricular septum (PA-IVS) patients are presented with an attempt to identify the predictive factors for pulmonary valvotomy alone as definitive treatment. Between June 1995 and December 1997, 14 PA-IVS neonates with tripartite right ventricle underwent an attempted pulmonary valvotomy. For perforation of the pulmonary valve, a guidewire was used in 4, and a radiofrequency guidewire in 10 patients. Two outcome groups were identified. Group I included those in whom transcatheter treatment achieved a definitive success; group II patients required surgery despite an initial successful valvotomy. The attempt failed in 3 patients, 1 of whom had pericardial effusion. Perforation of the pulmonary valve was achieved in 11 patients: 2 with a guidewire and 9 with a radiofrequency guidewire. A subsequent balloon valvuloplasty was performed in these 11 patients. After valvuloplasty, mean right ventricular pressure decreased from 124 +/- 24 to 60 +/- 15 mm Hg (p <0.01). One died of heart failure and infection 10 days later, despite successful weaning from prostaglandin E1. Group I patients (n = 6) were treated with transcatheter valvotomy alone. Group II patients (n = 4) required right ventricular outflow patch. Significant differences between the 2 groups (group I vs II) were identified in tricuspid valve Z value (0.52 +/- 0.37 vs -1.25 +/- 0.48, p <0.05), pulmonary valve Z value (-3.47 +/- 0.59 vs -5.43 +/- 0.94, p <0.05), and ratio of right-to-left ventricular area on the apical 4-chamber view (0.73 +/- 0.06 vs 0.49 +/- 0.03, p <0.05). There were no significant differences in hemodynamic characteristics between the 2 groups. After a follow-up period ranging from 7 to 35 months (mean 18 +/- 10.3), the most recent echocardiograms in the 10 patients showed a mean pressure gradient across the pulmonary valve of 17 +/- 15 mm Hg. All 10 patients had an oxygen saturation of >92%. Transcatheter valvotomy using a radiofrequency guidewire is a safe and effective treatment in selected patients with PA-IVS. Transcatheter valvotomy can be a definitive treatment in PA-IVS patients with a tricuspid valve Z value > or = -0.1, pulmonary valve Z value > or = -4.1 and ratio of right-to-left ventricular area > or = 0.65.

Children with heterotaxy syndrome frequently have complex cardiac and noncardiac malformations requiring detailed diagnostic evaluation for management planning. Direct delineation of these structures by electron beam computed tomography (EBCT) is validated as a means of diagnosis. From July 1995 to March 1997, 32 patients (16 girls, 16 boys) with clinically impressed heterotaxy syndrome were enrolled in this study. After evaluation by echocardiography, EBCT studies were performed. Interpretation of cardiac anomalies was performed by sequential analysis based on these cross-sectional images. The diagnoses were subsequently confirmed by angiocardiography and surgical findings. Twenty-eight patients had bilateral trifurcated bronchi, and most of these (24 of 28) did not have a spleen. Four patients had bilateral bifurcated bronchi, 2 patients had polysplenia, and the other 2 patients had a lobulated single spleen. We found that laterality could be identified by EBCT in all patients. Comparison of diagnostic yield between echocardiography, catheterization, and EBCT showed that EBCT is superior to echocardiography and catheterization in demonstration of pulmonary venous anatomy and presence of a very small rudimentary ventricle. In addition, associated visceral, bronchopulmonary, mediastinal, and intracardiac anomalies could all be clearly delineated by EBCT at the same time. Thus, EBCT is a promising complementary modality for an overall understanding of heterotaxy syndrome.

Double-chambered right ventricle (DCRV) is commonly associated with ventricular septal defect (VSD). In this study, an assessment was made of the relevance of a malalignment-type VSD to hemodynamic and morphologic features in DCRV. During an 8.5-year period, 53 patients with DCRV were enrolled after study with echocardiography, catheterization, and angiography. Patients were divided into 2 groups: group I included 40 patients, aged 3.7 +/- 3.2 years, with a malalignment-type VSD; group II consisted of 13 patients, aged 8.6 +/- 2.7 years, without a malalignment-type VSD. History of congestive heart failure in infancy was present in 21 group I and 2 group II patients (53% vs 15%, respectively, p <0.05). The mean pulmonary-to-systemic flow ratio was significantly higher in group I than in group II (1.89 +/- 0.74 vs 1.14 +/- 0.21, respectively, p <0.05). The mean pressure gradient across the right ventricular outflow tract was lower in group I than in group II (41 +/- 16 vs, 73 +/- 33 mm Hg, respectively, p <0.05). Among 42 patients who had a series of echocardiograms recorded, progression of pressure gradient was evident in 35: 28 in group I and 7 in group II. A subaortic ridge was present exclusively in 29 group I patients (73%). Prolapse of the aortic valve was present in 26 (49%): 20 group I (50%) and 6 group II (46%) patients. Aneurysm formation of the septal defect was found in 17 (43%) and 7 (54%) group I and II patients, respectively. It can be concluded that a history of congestive heart failure was more common in DCRV patients with a malalignment-type VSD. Malalignment-type VSD is significantly associated with a larger pulmonary-to-systemic flow ratio and subaortic ridge.

Multiple complex venous and intracardiac anomalies are frequently seen in patients with right atrial isomerism, and complete anatomic information may not always be obtained from combined echocardiography and angiography. Twenty patients with right atrial isomerism were prospectively studied with use of magnetic resonance imaging (MRI); satisfactory imaging was obtained in 18 patients, aged 6 days to 12 years (median 3 months). All patients had undergone echocardiography, cardiac catheterization, and angiocardiography. The purpose of this study was to assess the usefulness of MRI in the evaluation of venoatrial connections, atrial morphology, bronchial situs, and additional anomalies. Bilateral superior venae cavae were documented in 12 patients and anomalous hepatic venous drainage in 11. The pulmonary venous system was imaged in all 18 patients, of whom 12 had total anomalous pulmonary venous connection: 9 supracardiac, 2 infracardiac, and 1 mixed type. Some obstruction to pulmonary venous return was present in 9 of the 12 patients, and the site of obstruction was demonstrated by MRI in 6. Bilateral morphologic right atrial appendages were imaged in 7 patients. Bilateral morphologic right bronchi were identified in 17 patients, asplenia was confirmed in 17 of 18 patients by the absence of splenic tissue on imaging, and hiatal hernia was imaged in 4. Compared with angiography and echocardiography, MRI more clearly defined the pulmonary venous connections in 10 patients with pulmonary venous obstruction. Anomalous hepatic venous connections were better defined by MRI in 2. Therefore, MRI provided valuable supplementary information on the cardiovascular and visceral anomalies in these patients.

Defects on the membranous ventricular septum (perimembranous ventricular septal defect [VSD]) may spontaneously close or diminish in size by "aneurysmal transformation" (i.e., adherence of the tricuspid valve or adjacent tissue onto the VSD). It was recently shown that the appearance of aneurysmal transformation may be associated with subaortic ridges, as well as left ventricular (LV)-to-right atrial (RA) shunts. In all, 877 consecutive patients with isolated perimembranous VSD were retrospectively studied. Results of actuarial analysis showed that patients with a perimembranous VSD had a 98% probability of aneurysmal transformation by the age of 240 months. Of these patients, the expected probabilities of developing LV-RA shunt, spontaneous closure and subaortic ridge were 45, 35 and 6%, respectively. Patients with LV-RA shunts had higher probabilities of developing infective endocarditis (p = 0.002) and persistent left-to-right shunts (p < 0.001). The presence of a subaortic ridge may be accompanied by LV outflow tract obstruction (10 of 25 patients), infundibular pulmonary stenosis (n = 3), or aortic valve deformity or aortic regurgitation (n = 5), and none of those with a subaortic ridge closed spontaneously. It was confirmed that a substantial proportion of aneurysmal transformation of isolated perimembranous VSD is associated with LV-RA shunts and subaortic ridges.

This study was performed to elucidate the anatomic features of double-inlet ventricle in the Chinese population. The echocardiograms of 60 Chinese patients diagnosed as having double-inlet ventricle were reviewed and analyzed. Both atria were connected to a dominant right ventricle in 36 patients (60%), to a dominant left ventricle in 17 (28%) and to an indeterminate ventricle in 7 (12%). Right atrial isomerism was present in 30 patients (83%) with double-inlet right ventricle, in 5 (29%) with double-inlet left ventricle, and in 6 (86%) with double-inlet indeterminate ventricle. Right atrial isomerism was more frequently associated with double-inlet right and indeterminate ventricles (p < 0.01; chi-square). Common atrioventricular valve was frequently present in both double-inlet right and indeterminate ventricles. Double-outlet main chamber was the predominant type of ventriculoarterial connection in double-inlet right and indeterminate ventricles. Total anomalous pulmonary venous connection was found exclusively in 9 patients with right atrial isomerism (5 with double inlet right and 4 with double-inlet indeterminate ventricles). In contrast to other reports, this series found double-inlet right ventricle to be the predominant type of double-inlet ventricle. This discrepancy may be due to a greater prevalence of right atrial isomerism that is usually associated with double-inlet right ventricle in Chinese patients.