Cardiology in the Young最新文献

A 15-year-old male presented with vasovagal syncope and troponin leak 4 days after his second COVID-19 vaccine. Based on initial diagnostic work-up, he was thought to have COVID-19 vaccine-associated myocarditis. His cardiac dysfunction persisted and further work-up including genetic evaluation and serial MRI studies later confirmed a diagnosis of arrhythmogenic cardiomyopathy. This is a unique case of an incorrect diagnosis based on timing and context of vaccine-related myocarditis. Reports of mild and self-limited myocarditis post-COVID-19 vaccination may cause vaccine hesitancy among the public, and so case reports such as this one show the importance of discerning underlying conditions amongst rare COVID-19 vaccination complications.

Background: Glenn procedure carries low morbidity and mortality within stages of single-ventricle palliation. However, some patients with Glenn failure need a stage reversal, while others require unanticipated surgical interventions. Our understanding of perioperative factors and outcomes associated with such unexpected interventions is extremely limited.

Methods: Patients who underwent unexpected surgery after the Glenn procedure between January 2010 and December 2019 within the Society of Thoracic Surgeons Congenital Heart Surgery Database (STS-CHSD) were identified with a subgroup analysis of those reverting to stage I physiology. Patient's requiring reversal to stage I palliation were matched 1:5 with controls. Multivariable logistic regression analysis was performed to evaluate risk factors for reintervention.

Results: A total of 16,913 patients underwent Glenn procedure with 1221 (7.2%) requiring unexpected cardiac surgical intervention and 95 (0.56%) patients required takedown to a stage I. Significant clinical and operative risk factors were identified for such unexpected interventions.The overall mortality after Glenn procedure was 1.2%, while mortality after unexpected reintervention was 6.6% at 30 days and after Glenn takedown was 27.5% at last follow-up. Unexpected surgical intervention and right ventricular dominance were significant risk factors for mortality.

Conclusion: Unexpected reinterventions, including need for takedown after the Glenn procedure, are associated with significantly higher mortality. Further studies should focus on improving our patient selection, understanding the risk factors mechanistically, including impact of the right ventricle as systemic ventricle in order to avoid need for unexpected surgical interventions.

Not only has Wolff-Parkinson-White syndrome been associated with congenital cardiac abnormalities and cardiomyopathies, but familial clustering of Wolff-Parkinson-White syndrome has also been reported. Despite these well-known associations, direct genetic aetiology is rarely implicated in patients with Wolff-Parkinson-White syndrome. We report a 17-year-old girl with Wolff-Parkinson-White syndrome and left ventricular non-compaction cardiomyopathy due to a rare genetic variant in PR-domain containing protein 16. The report is supplemented by a comprehensive review of literature on association between PRDM16, left ventricular non-compaction and Wolff-Parkinson-White syndrome.

Objective: To assess the feasibility of transcatheter closure in patients with dextrocardia and isolated atrial septal defect or ventricular septal defect.

Methods: A retrospective analysis was performed on the clinical data of 10 patients with dextrocardia and atrial septal defect or ventricular septal defect from June 2013 to January 2023 and successfully underwent transcatheter closure. Patient data were meticulously collected.

Results: The study cohort comprised 10 patients, with three males, aged between 3 and 38 years. Intraoperative right heart catheterisation revealed the following measurements: the mean pulmonary arterial pressure of 20.5(18,24,3) mmHg, and a pulmonary-to-systemic flow ratio of 1.80(1.58,2.15). There were five atrial septal defects, with defect diameters of 26(20,30) mm, comprising three dextroversion and two mirror-image dextrocardia. Intraoperative echocardiography confirmed the absence of any residual shunt. Among them, a patient with mirror-image dextrocardia, atrial septal defects, and interrupted inferior vena cava required an alternative approach due to femoral vein limitations. The right internal jugular vein was punctured, and a 22 mm atrial septal occluder was successfully deployed via this route. The study identified five ventricular septal defects, with defect diameters of 4(3.5,5.5) mm, including two dextroversion and three mirror-image dextrocardia. Upon post-operative repeat left ventriculography, no residual shunt was detected in all but one case, which exhibited a minimal residual shunt. Throughout the perioperative period and subsequent post-operative follow-up, no severe complications were observed.

Conclusion: For patients with dextrocardia accompanied by simple CHD, transcatheter closure is a viable option when interventional treatment is indicated. Although transcatheter closure of atrial septal defects or ventricular septal defect in patients with dextrocardia presents unique challenges, it is safe and effective when the anatomical nuances of dextrocardia are thoroughly understood.

Objectives: The sutureless repair technique has been favoured due to its purported reduction in post-operative pulmonary venous obstruction rates. This study aims to compare the outcomes of conventional versus sutureless repair techniques in Total Anomalous Pulmonary Venous Drainage.

Methods: In this retrospective single-centre analysis (2012-2022), we evaluated children who underwent conventional or sutureless repair for isolated total anomalous pulmonary venous drainage, excluding complex cardiac anomalies and incomplete data. Patients were categorised into conventional (Group C, n = 58) and sutureless (Group S, n = 41) groups. Primary outcomes included mortality, morbidity, and post-operative complications. Statistical analysis included Mann-Whitney U, chi-square, and Fisher's exact tests where appropriate.

Results: Supracardiac type predominated in both groups (53.4% in Group C and 70.7% in Group S), with higher cardiac type frequency in Group C (24.1% versus 2.4%, p = 0.016). Early complications occurred in 58.5% versus 53.4% of cases in Groups S and C, respectively (p = 0.767). The mortality rate (17.2% versus 14.6%, p = 0.944) and post-operative pulmonary venous obstruction (21.2% versus 19.0%, p = 0.809) were higher in Group C, though not significantly. Mean cardiopulmonary bypass times were comparable between groups (105 versus 89 minutes, p = 0.424).

Conclusions: In this comprehensive analysis of paediatric Total Anomalous Pulmonary Venous Drainage repair, both conventional and sutureless techniques demonstrated comparable safety profiles and clinical outcomes. These findings suggest that surgical approach selection should be individualised based on patient characteristics and surgeon expertise. Further prospective studies with larger cohorts are needed to validate these observations.

While the pathophysiology affecting patients after Fontan palliation versus those with orthostatic intolerance is quite different, a common therapeutic approach exists. Exercise training, specifically augmenting the lower extremity skeletal muscle pump, improves the suboptimal haemodynamics of "preload failure" and thus clinical outcomes for each patient group. In this review, we will describe the problematic physiology affecting these patients, examine the anatomy and haemodynamics of the skeletal muscle pump, and finally review how exercise benefits both groups of patients through augmentation of musculovenous force.

Introduction: Cases of Fontan failure with normal Fontan pressure have been reported. This study aimed to identify catheterisation-derived haemodynamic predictors of heart transplantation/death, other than Fontan pressure, in late post-Fontan patients.

Methods: This retrospective study evaluated post-Fontan patients who underwent cardiac catheterisation at age ≥10 years between 1993 and 2018. The predictive effect of cardiac index-systemic vascular resistance index plot and perfusion pressure on freedom from the primary outcome (heart transplantation/death) was evaluated. Patients were categorised into haemodynamic categories A (cardiac index ≥ 3, systemic vascular resistance index ≥ 13), B (cardiac index < 3, systemic vascular resistance index ≥ 13), C (cardiac index ≥ 3, systemic vascular resistance index < 13), and D (cardiac index < 3, systemic vascular resistance index < 13).

Results: In total, 79 patients (median age: 15.7 [range: 10.1-50.2] years) were included; of them, the primary endpoint occurred in 10 (13%; median follow-up: 1.9 [range: 0.1-18.8] years). Category C patients had significantly shorter freedom from the endpoint than categories A and B patients. Univariate analysis identified significant haemodynamic predictors, including Fontan pressure, pulmonary/systemic vascular resistance index, pulmonary/systemic flow, systemic arterial oxygen saturation, systemic venous oxygen saturation, systemic vascular resistance index, perfusion pressure, perfusion pressure < 53 mmHg, and category C. In multivariable analysis, perfusion pressure < 53 mmHg and category C emerged as predictors of heart transplantation/death alongside Fontan pressure.

Conclusion: Haemodynamic profiling of late post-Fontan patients using the cardiac index-systemic vascular resistance index plot can aid to comprehend the post-Fontan status and predict clinical prognosis.

Introduction: Acute rheumatic fever commonly causes carditis in children. It is critical to have biomarkers for diagnostic and prognostic purposes. We aimed to investigate the expressions of miR-101, miR-1183, and miR-1299 in children with acute rheumatic fever and rheumatic heart disease and determine their potential as diagnostic biomarkers.

Methods: This cross-sectional study was conducted on 69 paediatric acute rheumatic fever patients and 27 gender and age-matched controls. The clinical findings were noted from medical records, echocardiography was repeated, and plasma miR-101, miR-1183, and miR-1299 expressions were studied using RT-PCR.

Results: Out of 54 patients with carditis, 36 developed rheumatic heart disease with significant left-sided heart involvement. No significant demographic difference was present between rheumatic heart disease patients and controls (p > 0.05). miR-101 and miR-1183 expressions significantly reduced by 4.5-fold in acute rheumatic fever patients compared to controls (p : 0.029 and p : 0.024, respectively). Family history was found to be significantly associated with 9-fold reduced miR-1183 expression in acute rheumatic fever patients (p : 0.005). The expression of miR-1183 in acute rheumatic fever children with carditis in general and severe carditis was significantly reduced 4.5- and 9-fold, respectively (p : 0.024 and p : 0.007, respectively). In acute rheumatic fever cases with moderate-severe carditis and patients who did not develop rheumatic heart disease, miR-1183 expression significantly reduced 8.3-fold and 9-fold, respectively (p : 0.015 and p : 0.019, respectively). The ROC analysis revealed that miR-101 and miR-1183 were statistically significant for acute rheumatic fever diagnosis (AUC: 0.64 for both miRNAs).

Conclusions: Significantly lower plasma expressions of miR-101 and miR-1183 in acute rheumatic fever patients, especially in cases with moderate-severe carditis, might indicate the potential use of these miRNAs as diagnostic biomarkers.

Percutaneous interventions have become significant in the management of congenital heart diseases, with transcatheter procedures being increasingly used for valve dysfunction, particularly for cases requiring repetitive surgeries. This abstract presents a successful transcatheter valve-in-valve implantation in a 16-year-old patient with severe tricuspid regurgitation following a bioprosthetic tricuspid valve replacement. The procedure involved transcatheter tricuspid valve implantation using the Mammoth 25x40 mm balloon catheter and the 26 mm Myval transcatheter heart valve system (Meril Life Sciences Pvt. Ltd, Vapi, Gujarat, India), resulting in immediate improvement in right atrial pressure and regurgitation. The patient underwent an electrophysiological assessment as part of the follow-up and was discharged with a normal sinus rhythm. Tricuspid valve interventions, although less common, are essential in congenital heart diseases, which necessitate prosthetic heart valve implantation due to long-term complications. The valve-in-valve procedure offers a safe alternative, especially in paediatric patients, for reducing risks caused by repetitive surgeries, providing a valuable treatment option in experienced centres.