Cardiology in the Young最新文献

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.

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.

The initial and updated Society of Thoracic Surgeons-European Association for Cardiothoracic Surgery (STAT and STAT 2020) and Risk Adjusted Classification for Congenital Heart Surgery-1 and Risk Adjusted Classification for Congenital Heart Surgery-2 scoring systems are validated to predict early postoperative mortality following congenital heart surgery in children; however, their ability to predict long-term mortality has not been examined. We performed a retrospective cohort study using data from the Pediatric Cardiac Care Consortium, a US-based registry of cardiac interventions in 47 participating centres between 1982 and 2011. Patients included in this cohort analysis had select congenital heart surgery representing the spectrum of severity as determined by STAT and Risk Adjusted Classification for Congenital Heart Surgery-1 and were less than 21 years of age. We applied STAT, STAT 2020, Risk Adjusted Classification for Congenital Heart Surgery-1, and Risk Adjusted Classification for Congenital Heart Surgery-2 for prediction of early mortality and long-term postoperative survival probability by surgical risk category. Long-term outcomes were obtained by matching Pediatric Cardiac Care Consortium patients with deaths reported in the National Death Index through 2021. Of 20,753 eligible patients, 18,755 survived the postoperative period and 2,058 deaths occurred over a median follow up of 24.4 years (Interquartile Range: 21-28.4). Each scoring system performed well for predicting early postoperative mortality with the following c-statistics: STAT: 0.7872, Risk Adjusted Classification for Congenital Heart Surgery-1: 0.7872, STAT 2020: 0.7724 and Risk Adjusted Classification for Congenital Heart Surgery-2: 0.7668. The predictive ability for long-term risk of death was as follows: STAT: 0.6995, Risk Adjusted Classification for Congenital Heart Surgery-1 c = 0.6741, Risk Adjusted Classification for Congenital Heart Surgery-2: 0.7156 and STAT 2020: c = 0.7156. Risk-adjusted score systems for congenital heart surgery maintain adequate but diminishing discriminative power to predict long-term mortality. Future efforts are warranted to develop a tool with improved long-term survival prediction.

We describe a 12-year-old boy with agenesis of the intrahepatic segment of the inferior vena cava (a rare congenital anomaly) in whom an electrified coronary guidewire with electrocautery was used percutaneously to perforate and open the atretic segment, followed by stent implantation. This technique may be a safe and feasible option for paediatric patients, offering a therapeutic alternative for complex cardiac anatomies.

Biallelic pathogenic variants in the inorganic pyrophosphatase 2 (PPA2) gene are a rare but established cause of sudden infant death, which may be precipitated by a pyrexial or viral illness. It has also been associated with sudden death secondary to alcohol ingestion in young adults. We describe the case of a thirteen-month-old female who presented following out-of- hospital cardiac arrest and was subsequently diagnosed with compound, heterozygous pathogenic variants of PPA2. She survived this episode and made excellent neurological recovery despite her prolonged out-of-hospital resuscitation. This case describes her initial presentation, consideration of PPA2-related cardiac arrest in the differential diagnosis, and the subsequent secondary preventative strategies. These included insertion of an implantable cardiac defibrillator, avoidance of alcohol in her medication preparations and administration of childhood vaccinations in a hospital environment due to risk of pyrexia-induced arrhythmias.

Introduction: Extubation failure after neonatal cardiac surgery is associated with increased intensive care unit length of stay, morbidity, and mortality. We performed a quality improvement project to create and implement a peri-extubation bundle, including extubation readiness testing, spontaneous breathing trial, and high-risk criteria identification, using best practices at high-performing centers to decrease neonatal and infant extubation failure by 20% from a baseline of 15.7% to 12.6% over a 2-year period.

Methods: Utilising the transparency of the Pediatric Cardiac Critical Care Consortium database, five centres were identified as high performers, having better-than-expected neonatal extubation success rates with the balancing metric of as-expected or better-than-expected mechanical ventilation duration. Structured interviews were conducted with cardiac intensive care unit physician leadership at the identified centers to determine centre-specific extubation practices. Data from those interviews underwent qualitative content analysis which was used to develop a peri-extubation bundle. The bundle was implemented at a single-centre 17-bed cardiac intensive care unit. Extubation failure, defined as reintubation within 48 hours of extubation for anything other than a procedure, ventilator days and bundle compliance was tracked.

Results: There was a 41.4% decrease in extubation failure following bundle implementation (12 failures of 76 extubations pre-implantation; 6 failures of 65 extubations post-implementation). Bundle compliance was 95.4%. There was no difference in ventilator days (p = 0.079) between groups.

Conclusion: Implementation of a peri-extubation bundle created from best practices at high-performing centres reduced extubation failure by 41.4% in neonates and infants undergoing congenital heart surgery.

Objective: This research seeks to ascertain the prevalence and determinants of mirror-image dextrocardia in fetuses.

Study design: With December 2022 as the reference point, we compiled colleted data on pregnant women who carried fetuses with mirror-image dextrocardia in Xi'an, Shaanxi Province: September-October 2022, November 2022, and December 2022-January 2023. An online questionnaire was distributed to 209 pregnant across China who had contracted COVID-19. The case group comprised women whose final menstrual cycle occurred in November 2022 and who had a fetus with mirror-image dextrocardia. Women with a November 2022 final menstrual period and a fetus without this condition made up the control group. To identify the risk factors associated with fetal mirror-image dextrocardia, both univariate and multivariate logistic regression analyses were employed.

Results: A significant difference was noted in the gestational age at COVID-19 infection women with a September to October 2022 and December 2022 to January 2023 final menstrual period who did not bear a fetus with mirror-image dextrocardia, and those with a November 2022 final menstrual period whose fetus exhibited this condition. The univariate and multivariate analyses conducted on pregnant women with a final menstrual period in November 2022 who had contracted COVID-19 revealed significant differences in the presence and duration of fever between those bearing fetuses with mirror-image dextrocardia and those without (P = 0.000).

Conclusion: The findings suggest two critical factors to the increased prevalence of fetal mirror-image dextrocardia: 1) the infection timing which occurs between the 4th and 6th week of pregnancy and 2) the presence of fever and its prolonged duration.