Pediatric Cardiology

Transfemoral device occlusion and minimally invasive surgical repair are performed for doubly committed subarterial ventricular septal defect (dcVSD) to reduce the invasiveness of the conventional surgical repair through a median sternotomy. However, few studies have compared them in terms of effectiveness and cost. Inpatients with isolated dcVSD who had undergone transfemoral device occlusion or minimally invasive surgical repair from January 2011 to June 2014 were reviewed for a comparative investigation between the two procedures. Procedure success was achieved in 36 transfemoral (75 %) and in 36 surgical (100 %) procedures (p = 0.001). Transfemoral patients were older, with a VSD size similar to that of surgical patients (14.5 ± 11.7 vs 4.4 ± 2.9 years, p < 0.001; 4.5 ± 1.5 vs 4.4 ± 1.3 mm, p = 0.577, respectively). No significant difference was observed in complication rates between the two treatment groups (p = 1). No large residual shunt was observed. Small residual shunt was noted in two transfemoral patients and four surgical patients (p = 0.674). All these small residual shunts closed spontaneously during follow-up. The surgical repair costs 26 % less than the device occlusion (Yuan 22063.2 ± 343.9 vs Yuan 29970.1 ± 1335.2, p < 0.001), where most of the cost was attributed to the occluder in the amount of Yuan 19,500. Compared with device occlusion, minimally invasive surgical repair can provide superior efficacy and comparable complication rates. In addition, it is 26 % cheaper than device occlusion. In low-income countries where healthcare resources are limited, medical resources must be judiciously allocated to the treatment that allows for effective treatment of the largest number of patients.

Despite aggressive immunosuppressive therapy, pediatric orthotopic heart transplant (OHT) candidates with elevated pre-transplant panel reactive antibody (PRA) carry an increased risk of rejection and early graft failure following transplantation. This study has aimed to more specifically evaluate the outcomes of transplant candidates stratified by PRA values. Records of pediatric patients listed for OHT between April 2004 and July 2008 were reviewed (n = 101). Survival analysis was performed comparing patients with PRA < 25 to those with PRA > 25, as well as patients with PRA < 80 and PRA > 80. Patients with PRA > 25 had decreased survival compared with those with PRA < 25 after listing (P = 0.004). There was an even greater difference in survival between patients with PRA > 80 and those with PRA < 80 (P = 0.002). Similar analyses for the patients who underwent successful transplantation showed no significant difference in post-transplant survival between patients with a pre-transplant PRA > 25 and those with PRA < 25 (P = 0.23). A difference approaching significance was noted for patients with PRA > 80 compared with PRA < 80 (P = 0.066). Patients with significantly elevated pre-transplant PRAs at the time of listing have a significantly worse outcome compared to those with moderately increased PRA values or non-sensitized patients. Further study is necessary to guide physician and family treatment decisions at the time of listing.

Sự xuất hiện bất thường của động mạch vành trái trước từ động mạch phổi là một biến thể hiếm gặp của sự xuất hiện bất thường của động mạch vành chính trái từ động mạch phổi. Chúng tôi báo cáo một bệnh nhân dường như không có triệu chứng với ALADCAPA và một khiếm khuyết vách ngăn tâm thất cơ nhỏ có tính chất hạn chế được chẩn đoán qua siêu âm tim trong giai đoạn sơ sinh. Bệnh nhân của chúng tôi đã trải qua phẫu thuật sửa chữa có kế hoạch khi được 3,5 tháng tuổi, sau đó khả năng ăn uống và tăng trưởng đã cải thiện đáng kể. Hình ảnh đa mô thức hữu ích để xác nhận biến thể hiếm này; tuy nhiên, các dấu hiệu siêu âm tim bao gồm việc thiếu nhánh động mạch vành trái hoặc một diễn biến động mạch vành bất thường nên làm tăng nghi ngờ về ALADCAPA. Trường hợp này cung cấp hỗ trợ cho việc sửa chữa sớm ở trẻ em với phát hiện tình cờ về dị tật này, vì thiếu máu cục bộ dưới lâm sàng có thể không được nhận diện qua các xét nghiệm hiện có nhưng có thể dẫn đến triệu chứng sau này trong cuộc sống.

We report a teenager with Marfan syndrome who presented to Cincinnati Children’s Hospital Medical Center as part of a preoperative evaluation for an orthopedic procedure after asymptomatic arrhythmia was recognized. Continuous cardiac monitoring showed frequent premature ventricular contractions and nonsustained runs of ventricular tachycardia. Cardiac magnetic resonance imaging showed left ventricular noncompaction (LVNC), prompting insertion of an implantable cardiac defibrillator. Although Marfan syndrome is associated with cardiac lesions, it has not previously been described with LVNC. Likewise LVNC has been seen in association with other cardiac lesions; however, this report represents the first reference of LVNC in the context of Marfan syndrome.

Antenatal diagnosis of right heart enlargement has a wide spectrum of differential diagnosis from maternal, placental and fetal causes, and outcomes of all are not known. Coarctation of the aorta is in the differential diagnosis of right heart enlargement. In our study, we focused to measure multiple cardiac dimensions in fetuses with right heart enlargement to identify the fetus with coarctation of the aorta utilizing echocardiographic measurements. Ten cardiovascular dimensions were measured from fetal studies between 20- and 34-week gestation, and six were measured on postnatal echocardiograms. Z-scores for the cardiac dimensions were calculated, and each variable for fetuses and infants was tested using a two-sample t test between patients with and without coarctation. We excluded fetuses with TAPVR, Shone complex, interrupted aortic arch, Ebstein anomaly or HLHS. Of the 31 fetuses with in utero right heart enlargement, 11 had coarctation postnatally and 20 did not have coarctation. We compared the fetal and newborn cardiac dimensions between the groups. The mean fetal carotid-subclavian index (CS Index) was 0.7 mm with coarctation compared with 1.1 mm without coarctation (p < 0.0001). The mean difference in diameter z-scores for fetal aortic isthmus (p < 0.0001), mitral valve (<0.001) and aortic valve (p < 0.009) was also significantly different. Similar significant differences were noted postnatally in the diameters of the cardiac dimensions between the coarctation and no-coarctation group: CS index (p < 0.0001), aortic isthmus (p < 0.0002) and aortic valve annulus (p < 0.007). A spectrum of diagnoses was found postnatally in fetuses with right heart enlargement, including a normal heart. The likelihood of identifying fetuses with coarctation of the aorta and planning for postnatal management can be refined by noninvasive screening measurements. A smaller CS index and smaller diameters of the aortic isthmus, mitral valve and aortic valve were significantly associated prenatally (p < 0.05) with coarctation of the aorta versus without coarctation and might be useful in prenatally diagnosing coarctation of the aorta. Postnatally, these measurements are reproducible. This is the first study utilizing these specific measurements to diagnose coarctation prenatally.

Cardiopulmonary exercise testing (CPET) provides assessment of the integrative responses involving the pulmonary, cardiovascular, and skeletal muscle systems. Application of exercise testing remains limited to children who are able to understand and cooperate with the exercise protocol. Near-infrared spectroscopy (NIRS) provides a noninvasive, continuous method to monitor regional tissue oxygenation (rSO2). Our specific aim was to predict anaerobic threshold (AT) during CPET noninvasively using two-site NIRS monitoring. Achievement of a practical noninvasive technology for estimating AT will increase the compatibility of CPET. Patients without structural or acquired heart disease were eligible for inclusion if they were ordered to undergo CPET by a cardiologist. Data from 51 subjects was analyzed. The ventilatory anaerobic threshold (VAT) was computed on $$ {\text{V}}_{{{\text{CO}}_{2} }} $$ and respiratory quotient post hoc using the standard V-slope method. The inflection points of the regional rSO2 time-series were identified as the noninvasive regional NIRS AT for each of the two monitored regions (cerebral and kidney). AT calculation made using an average of kidney and brain NIRS matched the calculation made by VAT for the same patient. Two-site NIRS monitoring of visceral organs is a predictor of AT.

The purpose of the study is to examine (1) nationally representative incidence rates of Emergency Department (ED) visits due to sudden cardiac arrest (SCA) in pediatric and young adult populations, (2) basic characteristics of the ED visits with SCA, and (3) patient and hospital factors associated with survival after SCA. We used the Nationwide Emergency Department Sample from 2006 to 2013. ICD-9-CM diagnostic codes identified ED visits due to SCA for patients ≤ 30 years old. Outcomes included yearly incidence of ED visits for SCA, and survival to hospital discharge. Predictors of interest were age groups, sex, and SCA case volume. A logistic regression model adjusted by patient- and hospital-level variables was used. Stratified analyses of age by (< 12 and ≥ 12 years old) were performed to explore the effect of pubertal development on SCA. With 71,881 ED visits due to SCA, the total incidence rate was 6.9 per 100,000 population, with a mortality rate of 89.6% and male/female ratio of 1.7. With the adjusted regression models, there were no differences in survival rate by sex; however, when stratified at 12 years old, males were less likely to survive than females above 12 years old (odds ratio [OR] 0.71, P < 0.01), but not under 12 years old. No statistically significant differences in survival rates between low- and high-SCA volume EDs were detected (OR 1.03, P = 0.77). Data showed no benefit of regionalized care for post-SCA in ≤ 30-year-old populations. With further examination of the differences between sexes, new management strategies for SCA cases can be developed.