There is limited knowledge on titration, optimal dosing, and efficacy of angiotensin-converting enzyme inhibitors in paediatric patients following cardiac surgery. Patients after cardiac surgery to repair ventricular septal defect or coarctation of the aorta from 01/2017 to 12/2019 were eligible for a retrospective single-centre study. Medical records were reviewed for patient characteristics and outcomes. Mean arterial pressure response and angiotensin-converting enzyme inhibitor dosage were collected. Controls were patients not receiving angiotensin-converting enzyme inhibitor postoperatively. Appropriate statistics were used for analysis. Among a total of 286 patients [n = 188 (66%) ventricular septal defect; n = 98 (34%) coarctation of the aorta], 170 (59%) received angiotensin-converting enzyme inhibitor on any postoperative day 1 to 5. The median age was 4.9 months (IQR 1.2-14.4) and weight 5.5 kg (IQR 3.7-9.2). The most common angiotensin-converting enzyme inhibitor was captopril on day 1 [n = 117 (69%)] and lisinopril at discharge [n = 86 (51%)]. Patients in treatment group were shown to have higher median mean arterial pressure at baseline and at time 1, compared with controls (mean difference 3.57 (95% CI: 1.85, 5.35) and 3.46 (95% CI: 1.41, 5.50), respectively. Median mean arterial pressure among controls significantly increased over time with a slope of 0.97 (95% CI: 0.2, 1.74), while median mean arterial pressure among treatment group decreased with a slope of -0.31 (-0.93,0.31). Patients who received high and medium doses of angiotensin-converting enzyme inhibitor showed significantly decreasing median mean arterial pressure over time with a slope of -2.85 (-5.14, -0.56) and -1.25 (-2.4, -0.11), respectively. High and medium dose angiotensin-converting enzyme inhibitor therapy had a greater effect in decreasing mean arterial pressure when compared to low dose.

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.

Dexmedetomidine is frequently used in paediatric anaesthesia. This includes use in patients with CHD, but detailed analysis of haemodynamics after administration in these patients has not yet been published. We performed a systematic review and meta-analyses examining haemodynamic changes immediately after dexmedetomidine administration in patients with CHD. We conducted a systematic review of PubMed, Embase, and Medline from inception until May 31, 2024. Inclusion criteria were studies that contained children with CHD who received dexmedetomidine for a cardiac procedure and reported at least one haemodynamic variable before and after administration of dexmedetomidine. Exclusion criteria were studies of noncardiac procedures. We performed a meta-analysis on each haemodynamic variable that was reported by at least four studies. We screened 5383 abstracts. We included 85 studies for review, and 16 studies were accepted for four meta-analyses (heart rate, 16 studies, n = 408; systolic blood pressure, 11 studies, n = 280; diastolic blood pressure, 10 studies, n = 276; mean arterial pressure, 5 studies, n = 130). Analysis of heart rate, systolic blood pressure, and diastolic blood pressure showed a statistically significant reduction (p < 0.001), while there was no significant change in mean arterial pressure. The clinical difference was minimal with a decrease in heart rate of 11.3 beats per minute, and a decrease in systolic blood pressure/diastolic blood pressure of 5.9 and 6.2 mmHg, respectively. Heterogeneity was high in all analyses. Dexmedetomidine is associated with small changes in heart rate, systolic blood pressure, and diastolic blood pressure in children with CHD. Further study is warranted.

This case describes a 32-year-old adult male who was incidentally diagnosed with isolated dextrocardia, Double inlet left ventricle (l-looped), l-transposed great arteries with subpulmonary stenosis during evaluation for an orbital abscess. This case highlights protective factors that enabled this patient's survival into adulthood without cardiac surgeries or medications despite single ventricle physiology, namely his "self-banded" pulmonary flow.

Although first performed several years ago, percutaneous total cavopulmonary connection has not yet become a widely adopted procedure in patients with univentricular circulation. We describe a case involving a novel, modified technique for the percutaneous completion of the Fontan circulation using covered stents in a 12 kg patient. The transcatheter Fontan completion was successfully performed. Minimal surgical preparation included banding of the inferior vena cava during the preceding superior cavopulmonary connection. Using needle puncture to create the anastomosis and implanting covered stents, a total cavopulmonary connection with an intra-atrial tunnel was established. The patient recovered uneventfully. Percutaneous total cavopulmonary connection is feasible and may represent a less invasive alternative for selected patients. However, data on this approach are currently very limited, and further studies are urgently needed.

Superior vena cava obstruction following paediatric cardiac surgery is a rare yet serious complication. After arterial switch operations, four neonates diagnosed with acute superior vena cava thrombosis were treated using transcatheter interventions. The importance of early recognition and implementation of transcatheter intervention for a successful outcome is emphasised.

Interrupted aortic arch is a rare congenital heart anomaly characterised by a complete discontinuity between the ascending and descending aorta, accounting for approximately 1-1.5% of CHDs. Its incidence in live births ranges from 3 to 20 per million. Survival into adulthood is only possible with the presence of a well-developed collateral circulation. In this case, a 49-year-old male patient presented with acute anterior myocardial infarction, and during emergency coronary angiography, discontinuity of the aortic arch was noticed. The procedure was successfully completed via radial access after femoral access failed. Subsequent thoracic computed tomography angiography confirmed the diagnosis of Type A interrupted aortic arch and revealed widespread collateral arterial circulation. No blood pressure difference was detected between the right-left arms and lower extremities, and the patient remained asymptomatic. This case demonstrates that this rare anomaly in adults can be incidentally detected during emergency procedures and that radial access is an effective alternative in overcoming anatomical obstacles.

Double aortic arch is a congenital vascular ring with tracheal and oesophageal compression, potentially causing stridor and dysphagia. While some recommend early surgery, others favour observation. We present a 7-week-old female with mild symptoms and prenatally diagnosed double aortic arch who suffered cardiac arrest, highlighting the need for early surgery in patients with vascular rings and airway or oesophageal compression.

The anomalous origin of the right pulmonary artery from the ascending aorta is a rare congenital anomaly usually diagnosed in infancy. When diagnosed later during adolescence, it presents diagnostic and treatment challenges, especially in determining whether pulmonary vascular disease can be reversed. We report the case of an 11-year-old girl who experienced increasing fatigue and was diagnosed with anomalous origin of the right pulmonary artery from the ascending aorta through echocardiography, CT angiography, and cardiac catheterisation. Segmental pulmonary hypertension was noted, but the operability was uncertain. A new non-invasive test combining 2D phase-contrast cardiac MRI with inhaled iloprost was performed. Flow measurements revealed a 42% rise in right pulmonary artery flow, indicating preserved vasoreactivity. Lung biopsy confirmed pulmonary vascular changes consistent with Heath-Edwards Stage II-III. Based on these findings, surgical reimplantation of the anomalous artery into the main pulmonary artery was performed. The postoperative recovery after surgery was uneventful, and follow-up catheterisation demonstrated normalised haemodynamics without residual stenosis. This case highlights the potential value of cardiac MRI-based vasoreactivity testing with inhaled iloprost as an additional tool alongside standard diagnostics for evaluating operability in late-presenting anomalous origin of the right pulmonary artery from the ascending aorta. Combining imaging, histopathology, and haemodynamic data allowed a personalised and safe surgical approach.

Hypoplastic left heart syndrome is considered one of the most severe CHDs and occurs in approximately 2-3% of all CHD cases. Hybrid procedures have been introduced as an alternative to traditional surgical techniques, such as the Norwood procedure, particularly for neonates at high risk. Although hybrid approaches claim to reduce surgical risks and improve recovery, little is known regarding their comparative effectiveness and safety. This review aims to describe the contribution of hybrid procedures in hypoplastic left heart syndrome management regarding survival rates, postoperative complications, and quality of life, through recovery outcomes and long-term results, in light of conventional surgical techniques. A systematic review was carried out following the PRISMA guidelines. Data were retrieved from PubMed, Scopus, Web of Science, and Cochrane Library. The search articles were published from January 2014 to November 2024. Inclusion was focused on paediatric patients diagnosed with hypoplastic left heart syndrome and studies comparing hybrid procedures versus traditional surgical methods. All screening, data extraction, and quality assessment were done by two independent reviewers. This review analysed data from 11 studies comprising 934 neonates with hypoplastic left heart syndrome, of whom 371underwent hybrid procedures (HP) and 563 received the Norwood procedure. The findingsdemonstrated that both approaches achieved comparable survival rates at 1, 3, and 5years. Hybrid procedures were linked to slightly earlier interventions (standardised mean difference=-0.10, 95% confidence interval: -0.61 to 0.41, p = 0.77) and were favored for high-risk neonates due to reduced invasiveness. However, hybrid procedures showed a higher rate of interstage events (risk ratio = 0.81), 95% confidence interval: -0.62 to 2.25, p = 0.09) and higher rates of pulmonary artery stenosis requiring reinterventions (30% vs. 18% for Norwood). Norwood procedures were associated with fewer reinterventions and lower interstage event rates, highlighting their effectiveness for stable patients. Hybrid procedures offer a practical alternative to the Norwood procedure, especially for high-risk neonates. Although both approaches show similar long-term survival rates, hybrid procedures are associated with a higher risk of complications, including increased interstage mortality. These challenges highlight the need for continued advancements to refine hybrid techniques and to improve long-term outcomes. This review emphasises the critical role of tailored patient selection and calls for further research to enhance hybrid procedure protocols and optimise their effectiveness for specific patient populations.