Cardiology in the Young最新文献

Introduction: There is limited knowledge on titration, optimal dosing, and efficacy of angiotensin-converting enzyme inhibitors in paediatric patients following cardiac surgery.

Methods: 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.

Results: 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.

Conclusion: 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.

Background: Pulmonary artery sling with complete tracheal rings represents a rare and challenging congenital anomaly, particularly in premature infants. We present a case of successful repair in an extremely low-weight premature infant.

Case presentation: A male premature infant (34 weeks of gestation, birth weight 1820 g) was diagnosed prenatally with pulmonary artery sling, perimembranous ventricular septal defect, and patent ductus arteriosus. At one month of age, bronchoscopy revealed severe tracheal stenosis with complete tracheal rings (3.1 mm external diameter). Despite the high surgical risk due to low body weight, complete surgical repair was performed at 2.7 kg through median sternotomy under cardiopulmonary bypass. The procedure included pulmonary artery sling repair with autologous pericardial augmentation, slide tracheoplasty using interrupted everted 6-0 PDS sutures, ventricular septal defect closure, and patent ductus arteriosus ligation. Intraoperative bronchoscopy confirmed adequate airway patency.

Conclusion: This case demonstrates that successful complete repair of complex cardiac and airway anomalies can be achieved in premature, low-weight infants when conventional weight gain thresholds cannot be met. Key factors for success include meticulous surgical technique, precise cardiopulmonary bypass management, careful perioperative care optimisation, and a multidisciplinary approach. While body weight alone should not be an absolute contraindication for surgical intervention, careful patient selection and appropriate institutional expertise are essential.

Wolff-Parkinson-White syndrome, characterised by accessory pathways, is rarely seen with dextrocardia. We present a case of situs inversus-dextrocardia with Wolff-Parkinson-White syndrome successfully treated via catheter ablation using 3D mapping (EnSite Precision®). Adjustments included reversed electrocardiogram (ECG) electrode placement, EnSite patch positioning, and fluoroscopic views. Coronary sinus access required counterclockwise manoeuvers. Mapping identified a left-sided accessory pathway, necessitating transseptal puncture with mirror-image adjustments. Ablation at the optimal site resulted in success. This case highlights the feasibility of catheter ablation in dextrocardia with tailored procedural modifications.

The clinical case describes a 13-year-old boy with a history of transposition of the great arteries, ventricular septal defect, and pulmonary stenosis who underwent surgical correction at age 1 with the REV procedure and the Lecompte maneuver. At age 2, severe subaortic obstruction required reoperation for subaortic tunnel reconstruction, myectomy, and reimplantation. Due to severe right ventricular outflow tract dysfunction, a 19 mm No-React Injectable BioPulmonic prosthesis was implanted. At 12 years, the patient presented with reduced exercise tolerance. Echocardiography and cardiac catheterisation demonstrated severe stenosis and regurgitation of the pulmonary bioprosthesis, right ventricular dilatation and hypertrophy, and an increased right ventricular-pulmonary artery gradient with normal pulmonary artery pressures. The manuscript presents an in vitro test demonstrating that the No-React Injectable BioPulmonic prosthesis frame can be modified using high-pressure balloons to increase its true inner diameter. The patient subsequently underwent transcatheter valve-in-valve implantation of a 23 mm Sapien 3 following pre-dilation and frame modification of the 19 mm No-React Injectable BioPulmonic prosthesis with a 22 × 20 mm Atlas Gold balloon, achieving an favourable haemodynamic outcome. Post-implant pulmonary arteriography excluded any intra-perivalvular regurgitation. Post-procedure haemodynamic assessment showed a residual peak-to-peak gradient of 10 mmHg, with systolic right ventricular pressures below half of systemic pressure. At the 6-month follow-up after the procedure, the post-procedural peak echocardiographic gradient across the pulmonary prosthesis was measured at 28 mmHg, with no evidence of regurgitation. Short-term results have been optimal, which encourages the use of this strategy for the treatment of similar cases.

Purpose: Children with CHDs are exposed to ionising radiation during diagnostic and interventional cardiac catheterisation procedures. Data regarding radiation doses a child is exposed to during these procedures are scarce. We aimed to investigate the radiation dose and other related parameters for paediatric cardiac catheterisation and propose these as local reference levels.

Methods and materials: We collected radiation exposure data for all diagnostic and interventional cardiac catheterisation cases (excluding electrophysiology procedures) utilising fluoroscopy at "Anonymized for review" between January 1, 2021 and November 30, 2021. Direct measures of radiation usage, i.e. fluoroscopy time, dose-area product value, and air kerma, were reported for each case by the INNOVA 2100 manufactured by Wipro GE Healthcare. The absorbed radiation dose was measured using a Bhabha Atomic Research Centre dosimeter, which was placed directly on the patients' chests. Differences in measures of radiation exposure across procedural and interventional types were tested using the normality of continuous data by Kolmogorov-Smirnov test. Spearman's correlation coefficient was calculated when the data violated normality.

Results: The overall median dose-area product, fluoroscopy time, air kerma, and absorbed dose measured using a Bhabha Atomic Research Centre dosimeter were 300 mg.m², 10.5 mins, 37 mGy, and 2.4 mSv, respectively. We have further categorised the median exposure parameters into 5 age groups and propose them as local dose reference levels. There was a strong correlation between fluoroscopy time with the absorbed radiation dose (Rho = 0.942). Fluoroscopy time showed fair correlation with air kerma (Rho = 0.65) and dose-area product (Rho = 0.59). Absorbed radiation dose showed fair correlation with dose-area product ( Rho = 0.56).

Conclusion: Fluoroscopy time alone is not a sufficient metric to monitor radiation exposure. Even when fluoroscopy times were comparable, air kerma and dose-area product levels were quite different. Therefore, the local dose reference levels proposed can be used as a benchmark for future studies aimed at process optimisation and further reducing the radiation exposure in paediatric patients.

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.

Background: Severe obesity in adolescents is a public health crisis of increasing concern. Cardiac maladaptation, such as left ventricular hypertrophy, increases the risk of future cardiovascular disease. This study aims to evaluate the impact of weight loss surgery on cardiac geometry in morbidly obese adolescents.

Methods: A single-centre, randomised controlled trial titled comparing laparoscopic adjustable gastric banding with multidisciplinary lifestyle intervention against a control group with multidisciplinary lifestyle intervention alone. The trial included severely obese adolescents aged 14-16 who had not responded to a minimum of 12 months of multidisciplinary lifestyle intervention. The primary outcome measured was the percentage change in total body weight one year after surgery. Left ventricular geometry was evaluated ultrasonographically before and one year after bariatric surgery.

Results: In the intervention and control group, a total of 10 and 8 patients received both the baseline and follow-up ultrasonographic examination. BMI significantly decreased in the intervention group compared to the control group (-6.75; 95% CI: -10.28 - -3.23). Moreover, left ventricular mass index (-8.21; 95% CI: -15.43 - -0.98) and relative left ventricular wall thickness (-0.04; 95% CI: -0.07 - 0.00) significantly decreased within the intervention group, reflecting amelioration of adverse remodelling, albeit without statistically significant intervention effect when compared to the control group.

Conclusions: Significant weight loss through bariatric surgery may improve left ventricular geometry in severely obese adolescents. These findings support the potential of bariatric surgery not only for reducing weight loss but also for improving cardiac health.

Slim et al.'s paper provided an insight into the differences between repaired tetralogy of Fallot and isolated pulmonary regurgitation in their strain. Repaired tetralogy of Fallot had higher right ventricular circumferential strain, while isolated pulmonary regurgitation relied on longitudinal strain more. This allowed the authors to infer that repaired tetralogy of Fallot can withstand more chronic regurgitation before valve replacement is necessary. We highlighted new findings relevant to this paper. Arrhythmia in repaired tetralogy of Fallot is associated with a reduced global circumferential strain of the right ventricle. Specifically, a value of below -14% was associated with a 6.3 times increase in the risk for an arrhythmic event. We believe this would be beneficial for patients when considered for valve replacements, suggesting modification of current valve replacement guidelines to include strain thresholds alongside current volumetric thresholds. However, the data for isolated pulmonary regurgitation remains scarce. Further investigation is needed to provide clearer timelines for valve replacement. We emphasised the importance of exploring the underlying architecture of repaired tetralogy of Fallot patients' hearts and why they could generate more global circumferential strain. We acknowledged the broader effect of this paper and its specific benefit in our country, Egypt. This paper provided insights useful for broader global health impact, especially in low-income countries.

Background/objectives: 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.

Methods: 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.

Results: This review analysed data from 11 studies comprising 934 neonates with hypoplastic left heart syndrome, of whom 371 underwent hybrid procedures (HP) and 563 received the Norwood procedure. The findings demonstrated that both approaches achieved comparable survival rates at 1, 3, and 5 years. 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.

Conclusions: 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.