Annals of Pediatric Cardiology最新文献

Context and aim: Absent pulmonary valve syndrome (APVS) in association with tetralogy of Fallot (TOF) is a rare congenital heart disease characterized by the aneurysmal dilatation of pulmonary arteries and associated major airway anomalies. Postoperative outcomes have been suboptimal, especially concerning dependency on positive pressure ventilation and the need for repeated cardiac reinterventions. This study aims to describe the postoperative course of these patients and to identify the risk factors associated with poor outcomes.

Methods and results: A retrospective review of 38 patients who underwent surgery for TOF with APVS between 2001 and 2021 at a single cardiac center was conducted. There were 16 (42%) patients in the young infants group. They required longer postoperative mechanical ventilation, 77 (interquartile ranges [IQR] 22-306) versus 17 (IQR 6-67) hours for the older children (P-0.009); longer inotropic support, 127 (103-285) versus 60 (20-108) hours for older children (P-0.000); longer intensive care unit (ICU) stay, 12 (4-29) versus 4 (2-7) days (P-0.01), and longer hospital stay 23 (14-64) versus 15 (11-20) days (P-0.02). Seven (43%) of the younger infants' group required readmission, whereas 4 (18%) of the older children needed to be readmitted (P < 0.04). Bloodstream infections (BSIs) occurred more frequently in the infants' group (31%) compared to older children (4.5%) (P < 0.03). The need for redo surgeries and the incidence of hospital-acquired infections other than BSIs did not show differences between the groups.

Conclusions: Age <3 months or weight <4 kg is associated with a more difficult postoperative course, characterized by longer ventilation days, a higher inotropic score, longer ICU and hospital stays, and more readmissions.

Right atrial aneurysm (RAA) is a rare congenital heart malformation that can originate from the free wall of the atrium or the appendage. We report a case of prenatally diagnosed RAA imaged using three-dimensional/four-dimensional spatiotemporal image correlation fetal echocardiography and its postnatal follow-up.

Background: Despite improvement in the outcome of the Norwood procedure, the prognosis of hypoplastic left heart syndrome (HLHS) remains problematic. The aim of this study was to assess the impact of pre- and perioperative factors and catheterization data on outcomes after the Norwood procedure.

Subjects and methods: This case-control study included all patients who underwent the Norwood procedure for HLHS at Fukuoka Children's Hospital between January 2000 and October 2011. Subjects (n = 66) were divided into two outcome groups: survivors (n = 41) and nonsurvivors (n = 25). First, we compared the pre- and perioperative data. Second, we compared the catheterization data after the Norwood procedure.

Results: The mean follow-up period was 40 months (range: 1.4-141). Overall, there was a 38% mortality, including early death. The pre- and perioperative data, as well as PaO₂ in room air at catheterization, were not significantly different. However, univariate analysis revealed that the incidence of home oxygen therapy to maintain oxygen saturation >80% was significantly higher in the survivors (P < 0.001). The right ventricular ejection fraction was higher in the survivors (48.7% ± 1.9%, mean ± standard error) than in the nonsurvivors (41.4% ± 2.8%, P = 0.041). The severity of tricuspid regurgitation (TR) and the pulmonary blood flow/systemic blood flow ratio were lower in the survivors than in the nonsurvivors. Multivariate logistic regression analysis identified severe TR as the only significant prognostic marker of mortality (P = 0.041).

Conclusions: The severity of TR was associated with the prognosis of HLHS after the Norwood procedure.

Grown-up with congenital heart disease (GUCH) represents an emerging but under-recognized public health challenge in India. With an estimated 3.5 million adults living with congenital heart disease (CHD), the country has one of the world's largest GUCH populations. Despite improving pediatric cardiac surgery outcomes, adult CHD care remains fragmented, with critical gaps in infrastructure, training, transition protocols, and national policy. This editorial explores the epidemiological burden, systemic challenges, and recent progress, including the growth of pediatric cardiac programs, the emergence of GUCH clinics, and increased use of transcatheter interventions for the Indian GUCH patients. We need a structured, level-based care model, development of specialized centers, dedicated GUCH training, and the creation of national registries and India-specific guidelines. Bridging the divide between pediatric and adult cardiology is essential to ensure lifelong, coordinated care for this vulnerable population.

Objective: The objective of this study was to assess the presence of aortopathy on follow-up in operated and unoperated adolescent and adult patients with Tetralogy of Fallot (TOF) and to identify the various associated factors.

Methods: This was a single-center observational study conducted at a tertiary care institute in India. All consecutive patients with a diagnosis of TOF, TOF with pulmonary atresia, or TOF with absent pulmonary valve, either repaired, palliated, or unoperated, >10 years of age who attended our institute between August 2021 and June 2023 were considered for enrolment. Primary outcome (aortopathy) was defined as sinus of Valsalva (SOV)/root diameter of ≥40 mm (for age ≥18 years), SOV/root Z-score ≥+2 (for age <18 years), or ≥ moderate aortic regurgitation by echocardiogram.

Results: The study enrolled 193 patients. Their mean age was 25.0 ± 11.87 years; for the operated patients, the mean duration of follow-up after surgery was 17.42 ± 7.9 years. Mean aortic annulus, SOV, sinotubular junction, and ascending aortic dimension were 22.8 ± 4.26 mm, 33 ± 5.8 mm, 26.9 ± 5.08 mm, and 29.4 ± 5.46 mm, respectively. Aortopathy was noted in 69 (35.8%) out of a total of 193 subjects. TOF with pulmonary atresia, unrepaired TOF, those who had univentricular palliation, and non-confluent pulmonary arteries were associated with the primary outcome. The mean increase in the ascending aortic dimension was 0.68 ± 0.6 mm/year for the cohort.

Conclusion: Aortopathy was noted in 35.8% of TOF patients on follow-up. Pulmonary atresia, absence of definite repair, and non-confluent pulmonary arteries are factors associated with aortopathy in TOF.

Late-onset atrioventricular (AV) block is an infrequent but life-threatening complication after surgical repair of congenital heart disease. We present a rare case of late-onset AV block in an adolescent who developed high-grade AV block long after undergoing an arterial switch operation and ventricular septal defect (VSD) patch closure. The patient had a significant past medical history of ventricular ectopy detected two and a half years before her presentation with AV block, for which an ablation of a slow ventricular tachycardia was performed close to the superior border of the VSD patch. On follow-up, a few years later, she was noted to have a high-grade AV block during stress testing and an electrophysiology study and underwent a pacemaker implantation. AV heart block is a life-threatening complication that needs to be monitored for in all patients with a history of a VSD repair.

We describe a 7-day-old newborn who presented with arterial desaturation and respiratory distress. The evaluation showed a duct with a continuous right-to-left shunt and an anomalous origin of the right pulmonary artery from the aorta. We discuss the hemodynamics leading to continuous right-to-left ductal flow and the subsequent intraoperative evaluation that resulted in a successful single-stage surgical repair of this interesting case.

Patients with single-ventricle physiology who received enalapril in the Pediatric Heart Network Infant Single-ventricle Trial displayed no differences in heart failure outcomes compared to those who received a placebo. The objective of this study was to use noninvasive pressure-volume loop analysis to assess for differences in cardiac mechanics between the enalapril and placebo groups. Core-lab echocardiogram measures prior to superior cavopulmonary connection (SCPC) and post-SCPC at 14 months of age were obtained. End-systolic elastance (Ees), a measure of contractility, and arterial elastance (Ea), a measure of afterload, were calculated. Ventriculo-arterial coupling was expressed as Ea/Ees. Data from 150 patients were analyzed. There was no difference in Ees, Ea, or Ea/Ees between study groups at either time point. The failure of enalapril to improve heart failure outcomes in this cohort may have some association with its failure to improve cardiac mechanics in single-ventricle physiology.

A 13-year-old girl developed infective endocarditis (IE) on the native mitral valve after hospitalization for osteomyelitis. The clinical course was complicated by intramyocardial abscess and severe mitral regurgitation. Blood cultures were positive for Stenotrophomonas maltophilia, a rare cause of IE, which is known to be resistant to the first-line agents used for the treatment of IE, and has a propensity to cause a myocardial abscess. Hence, an increased awareness regarding endocarditis due to S. maltophilia is warranted.