To evaluate the feasibility and efficacy of transcatheter closure of perimembranous ventricular septal defects (pmVSD) with aneurysmatic formation and muscular ventricular septal defects (mVSD) with Amplatzer duct occluder II.This retrospective analysis included 48 cases received transcatheter closure of pmVSD aneurysmatic formation or mVSD from February 2011 to March 2012 in our hospital (42 pmVSD with aneurysmatic formation and 6 mVSD). Median age was 5.2 years (range: 1.8 - 15 years), and median weight was 20.2 kg (range: 12 - 44 kg). Amplatzer duct occluder II was selected depending on the condition of ventricular septal defect. The device was implanted by antegrade or retrograde approach. Complications such as residual shunt, valvular regurgitation and arrhythmia were evaluated by echocardiography or angiography. Median follow-up was 9.5 months (range: 1 - 13 months).The mean ratio of pulmonary (Qp) to systemic (Qs) blood flow was 1.35 ± 0.15 before transcatheter closure. The diameter of exit hole of ventricular septal defects was (2.46 ± 0.53) mm measured by transthoracic echocardiography, and (2.35 ± 0.40) mm by angiography. Successful implantation of the device was achieved in 46 patients (96%) and unsuccessful in two cases due to acute aortic insufficiency. Forty-two (92%) patients were closed successfully, and trivial residual leak was evidenced in four patients and remained unchanged during follow-up. One patient with mVSD still had trivial residual shunt at 6 months post procedure. New trivial tricuspid insufficiency was observed in 1 patient (2.1%) during follow-up. Two patients developed procedural related left anterior fascicular block and remained unchanged during follow-up.pmVSD with aneurysm and mVSD could be successfully treated with Amplatzer duct occluder II. However, the long waist and large disc of the device could interfere with tricuspid valve function and cause tricuspid insufficiency.
Postoperative pulmonary artery stenosis is one of the popular complications.The disappointing results of surgical treatment for such lesions led to the introduction of interventional procedures(balloon angioplasty and stent implantation). Balloon angioplasty is applicable for branch pulmonary artery stenosis, however, balloon dilation alone rarely been effective in the long-term for these lesions.Since pulmonary artery stenting was introduced in 1991, indications and use of stents for dealing with pulmonary artery stenosis have played a leading role in recent times.Intravascular stents are effective for stenotic artery dilation, can reduce right ventricle pressure and improve heart function.Excellent mid-and long-term results after stenting of postoperative pulmonary artery stenosis are demonstrated by numerous researches, with the new developments in stent design, indications for pulmonary artery stent implantation are likely to widen in the future.
Key words:
Branch pulmonary artery; Stenosis; Stent; Child
Objective
To explore the etiology, clinical features and outcomes predictors of pediatric dilated cardiomyopathy(DCM).
Methods
The clinical findings and follow-up data of 183 pediatric DCM patients who were diagnosed during Jan.2008 to Dec.2012 were analyzed.
Results
In the 183 patients, the cause of 24 cases(13.1%)was known; but the etiology of 132 follow-up cases remain unknown, 85 were male(64.0%) and 47 were female(36.0%); 33 cases(25.0%) recovered, 62 cases(47.0%)died, and 37 cases(28.0%) remained the same.The median age of onset was 8 months(ranged from 1 day to 15 years). The onset age of the patients who had recovered was younger than the dead(P<0.001) and the unchanged patients(P=0.008). For the dead cases, the heart function on diagnosis was worse than the cured(P<0.001) and the unchanged patients(P=0.015); the left ventricular ejection fraction(LVEF) and the left ventricular fractional shortening(LVFS) detected by ultrasonic cardiogram were significantly lower than those of the cured(P=0.006, 0.009) and the unchanged (P=0.013, 0.006); the cases with mode-rate and serious mitral regurgitation(MR) were also more than the other groups(P=0.003, 0.004); The cases in the unchanged group were less than those of the cured group and the dead group whose serum concentration of cardiac troponin I was≥0.10 μg/L(P=0.002, 0.004). For the 159 cases with the unknown cause, 1-year survival rate was 67% and 5-year survival rate was 46%.By multivariable analysis, LVFS[hazard ratios(HR)0.890, 95% confidence interval (CI)0.836-0.948, P<0.001], moderate and serious MR[HR 3.580, 95%CI 2.030-6.315, P<0.001], malignant ventricular arrhythmia[HR 2.062, 95%CI 1.009-4.213, P=0.047], abnormal Q wave[HR 4.044, 95%CI 1.851-8.832, P<0.001]were associated with increased risk of death.
Conclusions
Twenty-five percent pediatric idiopathic DCM patients with the unknown cause recovered after proper treatment, and nearly 50.0% patients died; 1-year survival rate was 67.0% and 5-year survival rate was 46.0%.Three months after onset, the patients whose left ventricular function improved obviously had a better outcome.Risk factors for death include LVFS, moderate and serious MR, malignant ventricular arrhythmia, abnormal Q wave.
Key words:
Dilated cardiomyopathy; Etiology; Outcomes; Child
Objective
Patients with single-ventricle physiology pose a wide variety of therapeutic challenges. Pulmonary artery or anastomosis stenosis in single-ventricle physiology will have only subtle hemodynamic derangements due to the lack of the pumping chamber to force blood across the stenosis. This study aimed to evaluate the feasibility and usefulness of the stent implantation for treatment of branch pulmonary artery and anastomosis stenosis in children with univentricular hearts.
Methods
Retrospective analysis of 8 implanted stents between March 2014 and January 2015 in Shanghai Children's Medical Center. Transthoracic echocardiography, chest roentgenography and electrocardiography were carried out as follow-up studies at the second day after operation and 1, 3, 6, 12 months after the procedure.
Results
In all of eight patients( 6 males and 2 females), 5 cases were post-Glenn operation and 3 cases were post-Fontan operation. The median age was 6.6 years(range 4.0-8.5 years). The median weight was 19.5 kg(range 13.8-25.6 kg). 9 stents were implanted successfully in all patient, 6 in left pulmonary arteries, 2 in right pulmonary arteries and 1 in anastomosis. All stents were placed in the target lesion without any complication. The diameter of the narrowed segment improved from(3.63±2.06) mm to(7.89±1.62) mm(P<0.01). During follow-up no other complication occurred except thrombus in one patient because of discontinuation anticoagulation.
Conclusion
Branch pulmonary arterial stenosis in single-ventricle patients may often be underestimated due to the low pressure venous system and/or the development of venovenous collaterals bypassing and decompressing the pulmonary circuit. In these patients, even mild stenosis should be treated aggressively, especially in the presence of pleural effusions, pericardial effusions, protein-losing enteropathy and low-output states. Stent implantation is an effective method of treating branch pulmonary artery stenosis.
Key words:
Pulmonary artery; Stents; Child; Single ventricle
The main clinical manifestations of neonatal critical and complex congenital heart disease (CHD) are cardiac insufficiency and severe hypoxemia.Because of the rapid development of the CHD, most children will die early.Through the analysis of the successful interventional treatment of some severe CHDs in the neonatal, the indivi-dualized interventional treatment can be used according to different congenital malformations, and the interventional materials, which are different from the pediatric cardiology, are used reasonably.So many interventional therapies before or after surgery were used, and even, replace traditional surgical treatment to cure or palliate the CHD in neonates.
Key words:
Congenital heart disease; Interventional therapy; Infant, newborn
Background: Few data on paediatric hypertrophic cardiomyopathy (HCM) are available in developing countries. A multicentre, retrospective, cohort study was conducted to profile the clinical characteristics and survival of children with HCM in China.Methods: We collected longitudinal data on children with HCM aged 0–18 years at the participating institutions between January 1, 2010 and December 31, 2019. The primary outcomes were all-cause death or heart transplantation. The Kaplan–Meier method was used to estimate the survival rate of different groups.Findings: A total of 564 children were recruited, with a median age at diagnosis of 1.0 year (interquartile range: 0.4–8.0 years). The underlying aetiology was sarcomeric (382, 67.7%), inborn errors of metabolism (IEMs) (108, 19.2%), and RASopathies (74, 13.1%). The freedom from mortality was 71.1% (95% confidence interval [CI], 66.3%–75.3%) at 5 years. Patients with IEMs or those diagnosed during infancy had the poorest outcomes, with an estimated 5-year survival rate of 16.9% (95% CI, 7.7%–29.1%) and 56.0% (95% CI, 48.8%–62.5%), respectively. Heart failure was the leading cause of death in the cohort (90/149, 60.4%), while sudden cardiac death was the leading cause in patients with sarcomeric HCM (32/66, 48.5%).Interpretation: The distribution of paediatric HCM aetiology in China differs markedly from that in Western countries, with a higher proportion of IEMs and a lower proportion of neuromuscular disease. Overall, mortality remains high in China, especially in patients with IEMs and those diagnosed during infancy. Funding: Supported by the National Natural Science Fund of China (81770380, 81974029), China Project of Shanghai Municipal Science and Technology Commission (20MC1920400, 21Y31900301).Declaration of Interest: All the authors declare no competing interests.Ethical Approval: The study was conducted in accordance with the principles of the Declaration of Helsinki, and the study protocol was approved by the Ethics Committee of Shanghai Children’s Medical Center (SCMCIRB-W2020053). All participating centres obtained institutional review board approval for the study. Patient consent for inclusion was waived because of the retrospective nature of the study
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