Resumo Fundamento Apesar dos relatos de redução da aptidão física em crianças com cardiopatia congênita (CC), não foram realizadas avaliações específicas de desempenho para atividades de vida diária. Objetivos O objetivo foi comparar as atividades de vida diária, qualidade de vida, postura, aptidão física e níveis de atividade física entre crianças com CC e controles saudáveis (CS). Métodos O estudo incluiu 30 crianças, de 6 a 14 anos, com diagnóstico de CC moderada ou grave e 30 consideradas CS pareadas [...] Resumo Fundamento Apesar dos relatos de redução da aptidão física em crianças com cardiopatia congênita (CC), não foram realizadas avaliações específicas de desempenho para atividades de vida diária. Objetivos O objetivo foi comparar as atividades de vida diária, qualidade de vida, postura, aptidão física e níveis de atividade física entre crianças com CC e controles saudáveis (CS). Métodos O estudo incluiu 30 crianças, de 6 a 14 anos, com diagnóstico de CC moderada ou grave e 30 consideradas CS pareadas

Objectives: In the neonatal period, 25% of cases with critical congenital heart disease (CCHD) require surgical or interventional palliative and corrective procedures. Prenatal diagnosis and timely intervention can positively impact neonatal mortality and morbidity. This study evaluated the effects of perinatal follow-up on the management of CCHD. Methods: The study was conducted on term neonates diagnosed with CCHD, who were monitored in the neonatology and pediatric cardiac intensive care unit between 1 January 2023 and 1 January 2024. The cases were categorized into CCHD with prenatal follow-up (Group I), CCHD born without follow-up at our hospital (Group II), and CCHD accepted from external centers (Group III). Neonatal mortality and morbidity outcomes of these cases that underwent surgical or interventional procedures were statistically evaluated. Results: During the study period, there were 280 neonatal cases (50% male). Among these cases, 30% were in Group I (n = 84), 20% in Group II (n = 56), and 50% in Group III (n = 140). The cesarean section rate was higher in Group I compared to the other groups (80% vs. 52% vs. 45%), and the preoperative lactate levels were lower (0.9 vs. 1.7 vs. 2.1). The anatomical diagnoses, ventricular physiology, operation time, and interventional procedure time were similar. After interventional or surgical procedures, morbidity (22% vs. 25% vs. 36%) and mortality rates (6% vs. 9% vs. 18%) were lower in Group I and Group II compared to Group III. Conclusions: All infants diagnosed with CCHD before birth should be delivered in a tertiary heart center, which positively contributes to neonatal mortality and morbidity. More effort is needed to improve prenatal screening programs.

Fulminant myocarditis is a life-threatening event that can present as cardiogenic shock. Human metapneumovirus (hMPV)–associated myocarditis is exceptionally uncommon, particularly in the pediatric population. Treatment may require mechanical ventilation, inotropic agents, vasopressors, and advanced life support systems. In this article, we report an 18-month-old previously healthy infant who presented with severe metabolic acidosis, elevated lactate, and profound biventricular systolic dysfunction secondary to hMPV infection. Despite mechanical ventilation, inotropic support, and initial immunomodulatory therapy with intravenous immunoglobulin, high-dose methylprednisolone, and anakinra, the patient’s clinical condition deteriorated rapidly, requiring venoarterial extracorporeal membrane oxygenation (VA-ECMO) within the first 12 hours of admission. Given the absence of a patent foramen ovale and significant ventricular distention risk, surgical left ventricular decompression via a cannula inserted through the right upper pulmonary vein was performed. Hemoadsorption was additionally incorporated to mitigate hyperinflammation. Laboratory findings fulfilled macrophage activation syndrome criteria, and interferon-gamma blockade with emapalumab was initiated due to refractory cytokine storm physiology. Antioxidant therapy (nicotinamide adenine dinucleotide, coenzyme Q10, quercetin) was used as supportive treatment. Progressive improvement in ventricular function was observed under this comprehensive life support–based regimen. By day 12 of ECMO support, biventricular systolic function had normalized, and the patient was successfully weaned and discharged with full recovery. This case underscores the importance of early recognition, advanced immunomodulation, and effective ventricular unloading in managing fulminant hMPV myocarditis in children.

Background: Double-outlet right ventricle is a complex congenital cardiac anomaly in which both great arteries arise predominantly from the right ventricle. Accurate anatomical evaluation is critical for surgical planning. While transthoracic echocardiography is commonly used for intracardiac assessment, its limitations in visualising extracardiac structures highlight the need for additional imaging modalities like CT angiography. Objective: To compare the diagnostic accuracy and clinical utility of transthoracic echocardiography and CT angiography in the pre-operative evaluation and surgical planning of infants with double-outlet right ventricle. Methods: This retrospective, single-centre study included 78 infants diagnosed with double-outlet right ventricle. All patients underwent both transthoracic echocardiography and CT angiography before surgical intervention. Imaging findings were compared with intraoperative surgical results to assess sensitivity, specificity, positive predictive value, negative predictive value, and overall accuracy for each modality. Results: CT angiography was significantly more effective than transthoracic echocardiography in identifying extracardiac anomalies such as coronary artery anomalies ( p = 0.014), aortic arch hypoplasia ( p = 0.024), aortopulmonary collateral vessels ( p = 0.039), anomalous pulmonary venous connections ( p = 0.001), and persistent left superior vena cava ( p = 0.001). In contrast, transthoracic echocardiography was more sensitive in detecting small ventricular septal defects ( p = 0.035). Conclusion: Transthoracic echocardiography remains the first-line modality for intracardiac evaluation in double-outlet right ventricle, but CT angiography provides superior visualisation of extracardiac structures. The combined use of both imaging techniques improves diagnostic accuracy and surgical planning, supporting better outcomes in the management of CHD.

Ventriculoarterial connection is one of the important points of the segmental approach to congenital cardiac malformations. Double outlet of both ventricles is a rare form where both great arterial roots override the interventricular septum. In this article, we aimed to draw attention to this very rare form of ventriculoarterial connection by presenting an infant case diagnosed using echocardiography, CT angiography, and 3-dimensional modelling.