Annals of Pediatric Cardiology最新文献

Large vessel stenosis is currently successfully treated with percutaneous stent implantation. Zephyr stent is a cobalt-chromium peripheral stent specifically designed for large-vessel stenting. Its specific "S-" and "C-" shaped flexible polylinks prevent stent foreshortening and recoil. We report our experience of using Zephyr among the pediatric population and their medium-term outcome. The study included a total of 19 patients treated with 21 Zephyr stents. The indications for stenting included coarctation of the aorta (four patients), stenosed pulmonary artery (eight patients), stenosed right ventricular outflow tract (five patients) or conduit (one patient), and percutaneous Fontan (one patient). All the procedures were successful. None of the patients showed any stent-related complications. Stent fractures, foreshortening, or recoil were not seen during follow-up. Our medium-term (1-20 months) follow-up showed a favorable outcome. One patient developed restenosis. Zephyr stent is a good therapeutic option for large-vessel stenting. However, it needs large-scale studies for further confirmation.

Background: Transcatheter patent ductus arteriosus (PDA) closure with unusual morphology associated with multiple constrictions or unusual circumstances such as interruption of inferior vena cava (IVC) is still challenging. This study evaluates the use of KONAR-MF™ (Lifetech Scientific Co Ltd., Shenzhen) for transcatheter closure of PDA in such situations.

Materials and methods and results: Between January 2021 and October 2021, 24 patients from three different tertiary care centers underwent PDA device closure using the KONAR-MF™ device and are included in the study. Patient demographics, echocardiographic assessment, procedural details including the approach, device details, complications, and follow-up data were recorded. The median age and weight of the cohort were 2 years (0.5-41) and 10 kg (5-98), respectively. The indications for using KONAR-MF™ were (a) unusual morphology of PDA in 14, long tubular PDA in 8, and long tubular with multiple constrictions in 6 patients and (b) unusual circumstances such as interrupted IVC in 6 and inability to cross PDA antegradely in 4 due to dilated main pulmonary artery. The procedure was successful in all the patients and no procedure-related complications were noted in this cohort. Median follow-up of 9 months (range: 5-18) showed no residual shunt and only one patient had flow acceleration across the left pulmonary artery (peak Doppler gradient 16 mmHg).

Conclusions: KONAR-MF™ device can be used safely and effectively for transcatheter closure of PDA with unusual anatomy or in unusual circumstances.

Incontinentia pigmenti (IP) is a rare X-linked dominant neuroectodermal dysplasia affecting almost exclusively females. It is caused by loss-of-function mutations in the inhibitor of kappa light polypeptide gene enhancer in B cells, kinase gamma gene, formerly known as NF-κB essential modulator. The disorder is typically identified by peculiar skin findings that develop throughout the 1^st year of life. Approximately one-third of patients has ocular and neurologic abnormalities causing severe disability. Defects of hair, nails, and teeth can also occur. Among systemic complications, pulmonary arterial hypertension (PAH) is uncommon but potentially life-threatening. Only six cases have been described in the literature so far, and four of them died before reaching 1 year of age. Herein, we report the case of a 2-month-old girl with IP and severe PAH, successfully treated with pulmonary antihypertensive and anti-inflammatory therapy.

Arterial thoracic outlet syndrome (aTOS) is rarely diagnosed in children. A 15-year-old boy with recent-onset claudication was found to have aTOS on investigation. Bony abnormality other than the cervical rib might also cause aTOS. Enhanced awareness of aTOS as a cause of vascular symptoms in children is warranted.

Left main coronary artery obstruction due to a congenital supra-aortic membrane is a rare anomaly, but it can have serious consequences, including significant myocardial ischemia that may result in impaired ventricular function or even sudden death. Therefore, early diagnosis and treatment of this condition are crucial for improving patient outcomes. We present, in this report, a patient with intermittent obstruction of the left coronary arteries caused by a congenital ostial membrane diagnosed in adulthood that resulted in myocardial ischemia.

The images depict a rare case of Scimitar syndrome involving the left lower pulmonary vein.

Background: Changes in left ventricular (LV) systolic function have not been well described in premature neonates after transcatheter patent ductus arteriosus (PDA) closure.

Methods and results: We retrospectively identified all premature neonates < 3 kg who underwent a transcatheter PDA closure at our center between January 1 2015 and January 31, 2021. LV indices before and after closure were extracted and an analysis was performed. Overall, 23 neonates were included with a mean procedural weight of 1894 ± 622 g. At 24 h after closure, the median left ventricular ejection fraction (LVEF) (66% interquartile range [IQR] 12% vs. 61% IQR 12, P< 0.001) and median LV end-diastolic dimension z-score (3.3 IQR 1.8 vs. 1.4 IQR 2.6, P < 0.001) both decreased and 5 (22%) neonates had an LVEF <55%. Patients who had an LVEF <55% at 24 h had a higher preprocedure LV end-diastolic dimension z-score (4.2 IQR 1.2 vs. 2.8 IQR 1.6, P = 0.01), a higher preprocedure LV end-diastolic volume (19 mL IQR 4 mL vs. 11 mL IQR 11, P = 0.03), a higher birth weight (940 g IQR 100 g vs. 760 g IQR 140, P = 0.04), and were more likely to receive intravenous calcium during the procedure (60% vs. 11%, P = 0.04) compared to those with an LVEF ≥55% at 24 h after closure. Of those with LVEF <55% at 24 h, all normalized before discharge.

Conclusion: In preterm neonates who underwent successful transcatheter PDA closure, 23% developed abnormal LVEF after closure and those with significant LV dilation before the procedure were at increased risk for the development of LVEF <55% after closure.

The major challenges for pediatric cardiac nursing in India include inadequacies in their levels of education and lack of recognition of their vital role in the health-care system. The aim of this study was to understand the impact of the leadership educational initiatives on pediatric cardiac nursing taken by Children's HeartLink and the Pediatric Cardiac Society of India. Semi-structured interviews were conducted with eight pediatric cardiac nurse leaders from different Indian hospitals. A review of interviews from a previous study and an extensive literature review provided further foundational data on two major themes. The first theme on nursing education focused on nursing curriculum update, continuing education, and leadership development. The second theme focused on improving the value of nursing in public perception, value in hospitals, and their career promotions. The study identified the potential targets for improvement and provided a nursing career development ladder applicable to India.

A survey was conducted to ascertain the current situation of early-mid-career pediatric cardiologists (<20 years of experience) practicing in India. A formatted questionnaire was sent as a Google Form through email and WhatsApp link. Out of 275 eligible participants, 138 responses were received. Two pediatric cardiologists independently analyzed the data and responses are presented. Results showed pediatric cardiology is not considered a lucrative career option by most of the surveyed Indian pediatric cardiologists. Urgent remedial measures are needed to maintain the recently found momentum in the field in the country.