Annals of Pediatric Cardiology最新文献

Background: Catecholaminergic polymorphic ventricular tachycardia (CPVT) is an inherited disorder characterized by ventricular arrhythmias induced by physical or emotional stress. Currently, there are limited data available on the long-term prognosis of CPVT.

Methods and results: In this study, which included both retrospective and prospective components, 12 patients with CPVT (7 males and 5 females) under 18 years old were enrolled to gather and evaluate demographic, clinical, and genetic data. The mean age at diagnosis onset was 7.0 ± 3.1 years. All patients experienced syncope. The mean follow-up duration was 20.1 years. During the follow-up period, all patients experienced at least one episode of supraventricular tachycardia (SVT). Despite beta-blocker therapy, nine patients experienced syncope (75%), and four patients were noncompliant with their treatment. An implantable cardiac defibrillator (ICD) implantation was performed in 10 patients (83%), and among those 5 (50%) experienced appropriate shocks. Inappropriate shocks were observed in all patients with an ICD. The left cardiac sympathetic denervation was performed in 6 patients (50%). One patient died during the follow-up period. Genetic testing was performed in eight patients, five of whom had RYR2 mutations, one patient had mutations in CASQ2, one in TECRL, and one was gene-elusive.

Conclusions: The prevalence of cardiac events, even after the initiation of beta-blocker therapy, was found to be distressingly high during long-term follow-up. SVT, such as atrial fibrillation, were found to be more common than previously thought. Combination therapy with a beta-blocker and an IC antiarrhythmic drug shows promise. An individualized approach to the selection of treatment strategies is essential.

Background: The mortality risks of children undergoing various cardiac surgeries for congenital heart disease (CHD) in India are not well defined. We conducted a systematic review and meta-analysis to estimate the inhospital mortality of various common CHD surgeries reported in India and compared it to representative data from established Western databases.

Methods and results: We searched four bibliographic databases for studies published in India over the last 25 years. In total, 135 studies met the inclusion criteria and included 30,587 patients aged from 1 day to 65 years. The pooled mortality rate of 43 Indian studies reporting multiple CHD surgical outcomes is 5.63% (95% confidence interval [CI]: 4.26-7.16; I ² = 93.9%), whereas the Western data showed a pooled mortality rate of 2.65% (P value for comparison <0.0001). The pooled mortality risk for ventricular septal defect closure and tetralogy of Fallot repair in Indian studies was 2.87% (95% CI: 0.76-5.91; I ² = 62.4%) and 4.61% (95% CI: 2.0-8.02; I ² = 87.4%), respectively. The estimated mortality risk was higher than the Western databases for all subcategories studied except for surgeries in the grown-ups with CHD population and coarctation repair.

Conclusions: The estimated mortality risks are higher among Indian patients undergoing cardiac surgery for CHD as compared to Western data. We need prospective multicentric data to document whether the observed excess mortality exists after adjusting for various high-risk features and comorbidities in Indian patients. We need systemic measures to improve the outcomes of CHD surgeries in India.

Arterial desaturation following surgical repair of tetralogy of Fallot (TOF) is rare. In most instances, it results from residual right ventricular outflow tract obstruction, causing right-to-left shunt across residual interatrial or interventricular communication. In this report, we present an unusual scenario of arterial desaturation due to a recanalized left cardinal vein in a child with repaired TOF. We also discuss stepwise evaluation that led to successful identification and occlusion of the abnormal venous channel by percutaneous device closure.

A 4.1 kg male neonate with a diagnosis of double-inlet left ventricle with dextro-transposition of the great arteries was intubated shortly after birth due to respiratory insufficiency. The initial management consisted of a successful Stage I hybrid procedure. Persistent respiratory insufficiency led to cross-sectional imaging and bronchoscopy that demonstrated severe airway compression from a dilated main pulmonary artery. A Norwood procedure with Blalock-Thomas-Taussig shunt was performed at 1 month of age to relieve the airway obstruction. The patient was discharged home on room air at 2 months of age. This case highlights a unique single-ventricle anatomic variant with airway compression, which was successfully managed with deferred Norwood palliation.

Introduction: Permanent pacemaker implantation (PPI) in neonates is challenging with respect to indications, device selection, implantation technique, and long-term outcomes. Complex anatomy, the need for long-term pacing with high rates, and a problematic postoperative period are the major problems.

Methods: We prospectively followed up 22 newborns who underwent PPI below 28 days of life at our institute.

Results: The median age at implantation was 2 days (interquartile range 1-9 days), and 9% were born preterm. The average heart rate before implantation was 46.4 ± 7.2 bpm. Maternal lupus antibodies were positive in 8 (36.4%) neonates, whereas 11 (50.0%) had associated congenital heart disease. Nineteen neonates underwent single chamber (VVI) and three underwent dual chamber (DDD) pacemaker implantation. Over a median follow-up of 46 months (range 2-123 months), the average ventricular pacing percentage was 87.5 ± 24.9%, with a stable pacing threshold. Seven children underwent pulse generator replacement due to battery depletion at a median age of 47 months. Pacing-induced ventricular dysfunction was seen in five children at a median age of 23.6 months, and two underwent upgradation to cardiac resynchronization therapy. Overall mortality was 13.6%, all due to tissue hypoperfusion and lactic acidosis in the postimplantation period.

Conclusions: PPI in neonates has a favorable outcome with excellent lead survival. Overall mortality is 13.6%, which is predominantly in the postimplantation period and related to myocardial dysfunction.

Introduction: The study aims to assess the procedural outcomes and follow-up after transcatheter closure of ventricular septal defects (VSDs) in children utilizing the Konar-MF™ occluder (Lifetech Scientific, Shenzhen, PRC) device.

Materials and methods: Clinical features, demographic characteristics, and follow-up findings of children undergoing percutaneous VSD device closure were retrospectively analyzed from the medical records.

Results: Fifty-seven patients underwent VSD closure using the Konar-MF occluder between January 2019 and April 2023. Median age and body weight of patients were 36 (5-216) months and 12.5 (3.8-42) kg, respectively. The mean size of the defect on the left ventricular side was 6.5 ± 2.4 mm on echocardiography; the mean pulmonary artery pressure was 19.1 ± 9.7 mmHg. Three patients with severe pulmonary hypertension had successful device closure. The most used device size was 8 mm × 6 mm. The initially chosen device was upsized in 4 (7.01%) patients and downsized in 1 (1.7%) patient. Forty-five patients (78.9%) had device closure through the retrograde route. The procedure was successful in 53 (93.0%) patients. Immediate shunt occlusion was achieved in 86.8% of patients. Major complications, namely, embolization (1) and moderate aortic regurgitation (1) in two patients were successfully managed by surgery. One patient with severe tricuspid regurgitation has been on close follow-up. There was no mortality. Late complications such as valve regurgitation or rhythm disturbance were not identified on a median follow-up of 6 (1.5-47) months.

Conclusion: Transcatheter VSD closure using a Konar-MF occluder device is safe and effective, even in smaller children. The ability to deliver both anterogradely and retrogradely is a unique advantage.

Anatomical configurations where the viscero-atrial structures do not follow the usual arrangement or mirror-imaged arrangement is described conventionally as heterotaxy. Isomerism in the context of the congenitally malformed heart is a situation where some paired structures on opposite sides of the left-right axis of the body are, in morphologic terms, symmetrical mirror images of each other. It encompasses two separate entities, right and left isomerism, the former being usually associated with asplenia and the latter with polysplenia. We report herein a rare case of left isomerism that is associated with asplenia in a 4-year-old girl.

Background: This prospective, randomized, double-blind trial was done to compare intranasal dexmedetomidine and intranasal midazolam as premedication for sedation and ease of child-parent separation in pediatric patients of tetralogy of Fallot (TOF) undergoing corrective cardiac surgery.

Materials and methods: Forty children with TOF, between 1 and 10 years, undergoing corrective cardiac surgery were included in the study and, after randomization, were given intranasal midazolam (0.2 mg/kg) or intranasal dexmedetomidine (1 µg/kg), 30 min before shifting to the operation room (OR). Patients were assessed for sedation and child-parent separation, along with hemodynamic parameters, respiratory rate, and oxygen saturation (SpO2) 30 min after drug administration, at the time of shifting inside the OR, and at the time of induction of anesthesia.

Results: Both groups had comparable child-parent scores, hemodynamic parameters, SpO2, and respiratory rate. However, the dexmedetomidine group had significantly better sedation levels than the midazolam group patients at the time of shifting inside the OR (dexmedetomidine group: 3.55 ± 0.82 vs. midazolam group: 2.80 ± 0.83; P = 0.007) and at the time of induction of anesthesia (dexmedetomidine group: 3.40 ± 0.75 vs. midazolam group: 2.70 ± 0.86; P = 0.009).

Conclusion: Intranasal dexmedetomidine provides better sedation than midazolam, with similar child-parent separation scores and hemodynamic parameters, respiratory rate, and SpO2. No adverse events were observed in both groups. A study on a larger population will help in further establishing the safety and superiority of dexmedetomidine and will further its regular use as an intranasal premedication.

Transcatheter closure of sinus venosus defect (SVD) is an emerging intervention that utilizes a covered stent to redirect the right upper pulmonary vein to the left atrium. Preprocedural computed tomography analysis, as well as the interventional procedure, necessitates the use of radiographic contrast media. Contrast use is prohibited in patients with advanced kidney disease, who also carry high surgical risks of cardiopulmonary bypass. Transesophageal echocardiography-guided catheter intervention with zero contrast use is presented in this report, along with technical details about planning the procedure. Covered stent exclusion of SVD without contrast use has not been reported in the literature so far.