Annals of Pediatric Cardiology最新文献

Anomalous left coronary artery origin from pulmonary artery causes heart failure in infancy from ischemia and secondary mitral regurgitation. Rich intramyocardial collateralization may permit survival to adult age, where coronaries become tortuous and aneurysmally dilated. Surgery in adults involves left coronary ligation and providing a bypass graft to the left system, unlike coronary translocation adopted in infants. Unfavorable coronary remodeling in operated adults may lead to late coronary thrombotic occlusions. Two adults with markedly dilated tortuous coronary arteries showed variable remodeling after corrective intervention that impacted outcomes on follow-up. We stress the need for lifelong angiographic surveillance in older patients.

Total anomalous pulmonary venous connection (TAPVC) is a critical congenital heart disease which is often missed on prenatal echocardiography because of the decreased pulmonary blood flow in fetal life. Improvement in technology has resulted in increasing prenatal diagnosis of this condition. We report a foetus with infra cardiac TAPVC in whom prenatal diagnosis was facilitated by the use of STIC technology.

Background: The ratio of triglycerides-to-high-density lipoprotein cholesterol (TG/HDL-C) is considered a robust biomarker of metabolic syndrome (MetS) and is associated with several diseases, including type 2 diabetes mellitus (T2DM) and hypertension (HTN). This study examined the independent association of adiposity and fitness with the TG/HDL-C ratio.

Materials and methods: This is a cross-sectional study comprising 403 (201 girls) Nigerian adolescents aged 11-19 years. Participants were evaluated for body mass index, cardiorespiratory fitness, and TG/HDL-C. Regression models adjusting for age and biological maturity were used to evaluate the association of adiposity and fitness with TG/HDL-C ratio. The TG/HDL-C ratios of 1.0 mmol/L and 1.1 mmol/L were used to stratify female and male participants into low- and high-risk groups, respectively.

Results: Of the 135 high-risk adolescents, 21.1%, 5.5%, 12.7%, 14.9%, and 0.5% were at risk of T2DM, systolic HTN, diastolic HTN, abdominal obesity, and MetS, respectively. The independent variables were significantly associated with TG/HDL-C ratio only in boys but not girls. In boys, high adiposity (β = 0.193; P = 0.025) and low fitness (β = -0.169; P = 0.048) were independently associated with the dependent variable. Unfit boys were 3.9 (95% confidence interval [CI] =1.37-10.94, P = 0.011) times more likely to develop elevated TG/HDL-C ratio than their fit peers. The likelihood of girls at risk of MetS developing a high TG/HDL-C ratio was 13.7 (95% CI = 3.89-48.32, P < 0.001) times compared to their counterparts without MetS.

Conclusions: Adiposity and fitness were independently associated with TG/HDL-C in boys but not in girls. Health promotion intervention focusing on lowering TG/HDL-C ratio among Nigerian adolescents should include an emphasis on healthy diet and endurance activity programs.

Background: Polytetrafluoroethylene (PTFE) patch is commonly used during surgical closure for atrial septal defect (ASD) and ventricular septal defect (VSD). However, this patch has several limitations such as its inability to grow or remodel, especially in children and young adults. To tackle these limitations, we have tried to use fibronectin and human adipose-derived mesenchymal stem cells (hAMSCs) in the PTFE patch.

Objective: To understand the impact of fibronectin to enhance hAMSCs cell-to-cell adherence and cell-to-patch surface attachment into PTFE patches used in the surgical closure of ASD or VSD.

Materials and methods: The hAMSCs were plated and fixated with 15 mL methanol and cluster of differentiation (CD) 90+, CD105+, and CD45 - antibodies were labeled with fluorescein isothiocyanate, rinsed with phosphate-buffered saline, and analyzed under a fluorescence microscope. Fibronectin solution (0.1%) was used to soak patch scaffolds for approximately 2-h duration and then dried for 20 min in the treatment group. The samples were examined with a scanning electron microscope (SEM).

Results: SEM examination showed incomplete attachment of the cells even after 10 days in the control group at 1.14 ± 1.13. In contrast, the treatment group showed more cells attached to the patch surface at 31.25 ± 13.28 (P ≤ 0.0001). The observation at 5 days was 17.67 ± 20.21, at 7 days was 12.11 ± 10.94, and at 10 days was 18.83 ± 23.25. There was no significant statistical difference in mean cell per view among each treatment group (P = 0.802).

Conclusion: Our work demonstrates that fibronectin has a positive impact on hAMSC attachment seeded onto the PTFE patch. These properties, in combination with their developmental plasticity, have generated tremendous interest in regenerative medicine.

Ascending aortic aneurysm is very rare in children, and is usually seen in patients with underlying connective tissue disorders such as Marfans and Ehler-Danlos syndrome. Loeys-Dietz syndrome (LDS) is less commonly seen as a cause of ascending aortic aneurysms in children. In this case report, we describe pediatric Bentall procedure, which we successfully performed to a child with LDS (Type I) with giant ascending aortic enlargement and significant aortic regurgitation.

Background: Budd - Chiari syndrome (BCS) due to hepatic venous outflow obstruction is a rare cause of liver disease with dismal outcome, often amenable to catheter intervention.

Materials and methods: This retrospective single-center study analyzed the clinical profile and medium-term outcome of interventional treatment with balloon angioplasty ± stenting in all pediatric BCS over a 10-year period. Clinical, laboratory, imaging, and interventional data were retrieved. Transhepatic (TH) access was utilized in the recent 3 years.

Results: We included a total of 27 patients. Acute and subacute BCS comprised 93% of subjects. Ascites was the most common symptom. COVID-19 infection and Takayasu arteritis were two novel etiologies in our study. There was isolated hepatic vein (HV) narrowing in 11 (41%), isolated inferior vena cava obstruction in 4, and combined occlusion in 12 (44%). Intervention was successful in 22 (82%) patients. Stenting was required in 14 (64%) patients and the rest underwent balloon angioplasty. The immediate outcome was better with stenting than balloon (91% vs. 64%). Transhepatic access in 6 patients allowed HV cannulation in all and achieved patency in five patients. Two patients from the balloon group (25%) and 9 from the stent group (64%) are alive with patent veins at a median follow-up of 60 months, indicating a high attrition rate.

Conclusion: Catheter interventions restored physiological blood flow in pediatric BCS. TH route improved cannulation of occluded HV compared to other accesses. Immediate and medium-term outcomes were better after stenting with lower rates of reinterventions than balloon angioplasty. Life-long surveillance is required as mortality is high on follow-up.

Transcatheter correction of superior sinus venosus defects using covered stent is increasingly reported in the literature and provides an alternative option to surgery in appropriately selected adults. Being a new intervention, meticulous attention to procedural techniques and precise surveillance imaging modalities are vital to detect and avoid potential early and late complications. This report highlights the occurrence of a residual interatrial communication following covered stent placement and large subclinical asymptomatic nonocclusive thrombus formation at the right atrial end of the stent. The management of both these complications is also highlighted in this report.

Background: Repair of aortic coarctation through left thoracotomy is the standard treatment when anatomically feasible. Long-term outcomes are well studied, including the need for reintervention. However, the timely variation in residual gradients across the repaired segment is ill-defined. The aim of this work was to study the progressive changes of estimated peak gradient (ePG) acquired by transthoracic continuous-wave Doppler echocardiography across the aortic arch after repair and to assess the role of timing of assessment and values of ePG in prediction of reintervention.

Materials and methods: All eligible patients for this study who underwent aortic coarctation repair through left thoracotomy from 2001 to 2017 were reviewed. Details of the aortic arch dimensions and associated lesions were obtained by transthoracic echocardiography (TTE). The primary outcome was the ePG across the aortic arch after repair. Longitudinal data analyses with mixed effect modeling were used to determine independent predictors for ePGs.

Results: A total of 312 patients were included. Median age and weight were 30 days and 4 kg, respectively. Associated lesions included ventricular septal defect (VSD) (53%), bicuspid aortic valve (53%) and mitral stenosis (25%). Over 15-years follow-up the freedom from reintervention was 92.3%, while 24 out of the 312 patients underwent reintervention (7.7%). Longitudinal data analyses of serial 2566 TTE studies were done. The graphical display showed that the ePG across coarctation area in the first postoperative TTE was the most notable difference between those who underwent reintervention and those who did not. Further testing with proportional hazard and logistic regression modeling confirmed this finding. The area under receiver operating curve statistics showed that an ePG of 25 mmHg is an optimal cutoff value for the prediction of the reintervention.

Conclusions: The ePG acquired in the first postoperative TTE is the most important predictor for reinterventions. The presence of VSD is associated with decreased ePGs. We propose that an ePG in the first postoperative TTE of 25 mmHg or more is a strong predictor for the need of reintervention.