Annals of Pediatric Cardiology最新文献

Aortic arch branching anomalies are rare incidental findings in angiographies performed for cardiovascular and cerebrovascular causes. Recognizing them and candid reporting are crucial for treatment planning, interventional access sites for catheterization, catheter selection, and deciding the need for further imaging. We report a rare case of truncus bicaroticus with the aberrant origin of the right subclavian artery in a patient with an atrioventricular septal defect diagnosed on computerized tomography angiography and highlight its clinical implications.

Viral myocarditis is the inflammation of heart myocytes resulting from viral infection. Incidence in the pediatric population could reach 2 per 100,000 per year, and COVID-19 infection is a significant risk factor, which increases the possibility of having an infection by 40 times. Early detection results in catching the disease early and consequently improves outcomes. Clinical presentation of viral myocarditis in children could vary from mild prodromal symptoms to severe heart failure. Clinical examination, electrocardiogram, and chest X-ray may give clues for physiological and structural signs usually associated with the disease. However, they are inconclusive as they lack both accuracy and specificity. Biomarkers used to track the disease usually lack sensitivity and specificity. Cardiac magnetic resonance (CMR) is the imaging of choice to diagnose viral myocarditis by showing edema and late gadolinium enhancement. Point-of-care ultrasound has been approved as a good imaging method for early detection. It can be used as an effective screening tool for high-risk patients. Positron emission tomography scan is very sensitive in detecting disease early in its acute phase, especially if combined with CMR. All imaging studies are prone to interpretation bias, leading to a misdiagnosis. Endomyocardial biopsy is the gold standard method for diagnosis. However, it is time-consuming and ineffective as an early detection tool. Artificial intelligence (AI) helps with interpretation, decreasing bias, improving accuracy, and saving time and manpower. With more research and evidence, adopting AI-based methods to diagnose myocarditis in pediatrics could offer early detection, reduce costs, and save time for early intervention. Genetics helps identify inflammatory pathways involved in vulnerable patients, and genetic therapy may suppress disease progression by mitigating these pathways. Research focused on children is highly encouraged, and collaboration between healthcare institutions to develop telemedicine-based programs is influential.

RASopathies are the most common underlying etiology in infants with hypertrophic cardiomyopathy. An improved understanding of the downstream molecular mechanisms of the disease has enabled us to target therapy for genetic abnormalities in human cancers with somatic mutations in the RASopathy genes. This therapy is now being extended to RASopathies, which are due to germline mutations in the same genes. We report an infant with Noonan syndrome with multiple lentigines who presented with symptomatic heart failure in infancy. Everolimus therapy over 1 year resulted in symptomatic improvement with no significant adverse clinical events.

Background: Aortic coarctation (CoA) accounts for 5%-8% of congenital heart defects, and patients' symptoms can range from neonatal shock to hypertension in adolescence or even adulthood. While surgical repair is the gold standard, catheter-based therapies are increasingly utilized. Despite advancements, complications, and recurrence rates necessitating re-intervention remain concerns.

Patients and methods: We analyzed the postoperative outcomes and long-term intervention rates for pediatric patients undergoing extended end-to-end CoA repair without cardiopulmonary bypass between October 2002 and January 2024 at the Leipzig Heart Center. Data were prospectively collected and retrospectively analyzed.

Results: Among 168 patients, the median age at surgery was 11 days (interquartile range [IQR] 6-26). There was no early mortality. Median intensive care unit stay was 4 days (IQR 3-5), and hospital stay was 9 days (IQR 7-12). Early re-intervention during the same hospital stay was required in 3% due to re-coarctation. Median follow-up was 33 months (IQR 7 months-8 years). Long-term survival at 1, 5, 10, and 14 years was 100%, 98.9%, 98.9%, and 98.9%, respectively. Freedom from catheter-based intervention was 74.3%, 70.1%, 67.9%, and 64.8% at the same intervals.

Conclusion: Extended end-to-end anastomosis for CoA repair in children yields excellent survival and acceptable long-term outcomes, though re-intervention remains a consideration.

Lung agenesis associated with congenital heart disease is a rare and serious malformation that requires immediate medical attention. If not addressed with preplanned surgical and medical support, it can often lead to mortality. This report describes an unusual case of a 2-month-old male infant with left lung agenesis and obstructed infracardiac total anomalous pulmonary venous connection. The infant presented with congestive heart failure, dominated by symptoms of obstruction and pulmonary hypertension. We detail the successful surgical intervention and postoperative outcomes for this rare association.

Partial anomalous pulmonary venous connection (PAPVC) is a rare form of congenital heart disease. We describe an extremely rare case of PAPVC with triple drainage, involving the left upper pulmonary vein.

Atrioventricular septal defect (AVSD) with cor triatriatum sinister (CTS) is a rare congenital anomalous combination. Few cases of AVSD and CTS have been reported in the literature. However, an isolated case of CTS with partial AVSD, left superior vena cava, and unroofed coronary sinus in an adult has not been reported in the literature. A 20-year-old male, a known case of partial AVSD with CTS, presented with dyspnea on exertion. The patient was surgically managed after a thorough preoperative evaluation and had an uneventful postoperative course. Meticulous evaluation with multiple imaging in the preoperative workup is required in all cases of AVSD to look for associated lesions.

Background: Pulmonary arterial hypertension (PAH) is a chronic, progressive disease affecting the pulmonary vasculature, with a high mortality rate. Patients with PAH due to congenital heart disease (CHD) (World Health Organization Group 1) exhibit significantly reduced exercise tolerance compared to other PAH groups. Right ventricular (RV) dysfunction is a commonly used independent prognostic parameter in PAH. This study aimed to investigate an echocardiographic-derived parameter of RV-pulmonary artery (PA) coupling to exercise tolerance in PAH-CHD.

Subjects and methods: This cross-sectional study was performed on 51 consecutive patients with a confirmed diagnosis of PAH-CHD between February and March 2024. These patients underwent a 6-minute walk test (6MWT) and echocardiographic evaluation, including the measurement of tricuspid annular plane systolic excursion (TAPSE), pulmonary artery systolic pressure (PASP), TAPSE/PASP, and tricuspid regurgitation velocity (TRV) as parameters of RV function. The data were analyzed using the IBM SPSS Statistics software (version 26.0).

Results: Fourteen men and 37 women aged 36.0 ± 11.2 years participated in this study. The most common CHD was atrial septal defect (88.2%) with left-to-right shunt (51.0%). The mean of 6MWT was 366.8 ± 60.6 m. There was a significant association between 6MWT and TAPSE (r = 0.330, P < 0.05), PASP (r = -0.273, P < 0.05), TAPSE/PASP (r = 0.392, P < 0.05), and TRV (r = -0.354, P < 0.05).

Conclusion: The TAPSE/PASP is a simple echocardiographic parameter that correlates with RV function and exercise tolerance in patients with PAH-CHD.

Background: Tetralogy of Fallot (TOF), the most prevalent cyanotic congenital heart anomaly, impacts around 3.9 in 10,000 live births. Repair aims to address intracardiac shunting and right ventricular outflow tract obstruction. Despite successful historical surgeries, the optimal timing for repair remains debated.

Objective: The aim of the study was to provide an updated comparison of the TOF repair timing (neonatal vs. postneonatal periods) based on post- and perioperative outcomes.

Methods: A literature search was conducted across PubMed, Scopus, EBSCO, Science Direct, and Epistemonikos. Quality assessment was performed using the Risk of Bias in Non-Randomized Studies of Interventions, whereas the outcomes were analyzed using RevMan 5.4.

Results: Nineteen studies comprising 28,968 patients were included in the study. All studies were classified as high-quality. The neonatal repair exhibited longer intensive care unit (ICU) stays (standard mean differences [SMD] 1.58; 95% confidence interval [CI] 1.04-2.12; P < 0.00001) and hospital stays (SMD 5.18; 95% CI 3.54-6.82; P < 0.00001). Moreover, the analysis showed an overall result favoring the postneonatal repair, including mortality (odds ratio [OR] 1.68; 95% CI 1.47-1.92; P < 0.00001), delayed chest closure (OR 2.52; 95% CI 2.06-3.09; P < 0.00001), and pacemaker implantation (OR 3.68; 95% CI 2.89-4.70; P < 0.00001).

Conclusion: Complete repair during the post-neonatal period yielded better postoperative outcomes, shorter hospital stays, ICU stays, and ventilation time.PROSPERO registration: CRD42024503630.