ABSTRACT
Noonan syndrome (NS) is a pleomorphic genetic disorder. Up to 50-80% of individuals have associated congenital heart disease. The scope of cardiac disease in NS is quite variable depending on the gene mutation. The most common forms of cardiac defects include pulmonary stenosis, hypertrophic cardiomyopathy (HCM), atrial septal defect and left-sided lesions. Amongst the rare vascular abnormalities few case reports have been mentioned about coronary artery lesions apart from sinus of Valsalva aneurysm, aortic dissection, intracranial aneurysm. This is a case report a rare case of asymptomatic coronary artery aneurysm in a young male with NS. There is lack of unified protocol for the screening, diagnosis, treatment, and follow-up of coronary artery disease in patients with NS. We conclude, echocardiography is sufficient in most cases in children. But a CT scan is appropriate in adults or when other lesions are suspected.
ABSTRACT
In response to the survey among early career pediatric cardiologists from India and the accompanying editorial, we invited comments and suggestions from thought leaders and senior functionaries in the field. We have summarized the thoughts and suggestions as a mini-symposium.
ABSTRACT
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.
ABSTRACT
This article reviews the transcatheter interventions for ruptured sinus of Valsalva, coronary arteriovenous fistulas, and pulmonary arteriovenous fistulas. The current day indications, the required hardware, and divergent techniques are described at length. A detail account of published literature on transcatheter interventions of these lesions is given. We shall review in this article the catheter interventions for these rare miscellaneous lesions in congenital heart disease (CHD)-rupture of sinus of Valsalva, coronary arteriovenous (AV) fistulas, and pulmonary AV fistulas. The indication and recommendation for the transcatheter intervention, the necessary hardware and technique, and the review of published data in this regard will be dealt in details.
