alexa Multiple Cardiac Rhabdomyomas Causing Left Ventricular Outflow Obstruction | Open Access Journals
ISSN: 2572-0775
Clinical Pediatrics: Open Access
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Multiple Cardiac Rhabdomyomas Causing Left Ventricular Outflow Obstruction

Soham Dasgupta1*and Ashraf M Aly2

1Department of Pediatrics, University of Texas Medical Branch, USA

2Department of Pediatric Cardiology, University of Texas Medical Branch, USA

*Corresponding Author:
Soham Dasgupta
Department of Pediatrics
University of Texas Medical Branch, USA
Tel: 832-693-1917
E-mail: [email protected]

Received Date: September 15, 2016; Accepted Date: September 20, 2016; Published Date: September 27, 2016

Citation: Dasgupta S, Aly AM, Clin Pediatr (2016) Multiple Cardiac Rhabdomyomas Causing Left Ventricular Outflow Obstruction 1: I104. doi: 10.4172/2572-0775.1000i104

Copyright: © 2016 Dasgupta S, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution and reproduction in any medium, provided the original author and source are credited.

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Clinical Image

A term female infant was born via an urgent C-section for fetal arrhythmia. Postnatally, the heart rhythm was irregular and an EKG showed a normal sinus rhythm and frequent premature atrial complexes. There was cardiomegaly noted on a chest X-ray. A loud systolic murmur was appreciated. An echocardiogram showed multiple myocardial tubers (Figure 1). One tuber was attached to the septal leaflet of the tricuspid valve and another was located right below the pulmonary valve. However, the most significant one was located just below the aortic valve and measured 12 × 7 mm causing a moderate to severe left ventricular outflow tract obstruction (LVOTO) (50-60 mmHg peak gradient) (Figure 2). A T1 weighted brain MRI showed sub-ependymal hematomas (Figure 3) and cortical tubers consistent with tuberous sclerosis. This was further confirmed by gene sequencing showing a mutation in the TSC-2 gene. The patient is being monitored closely as an outpatient for the possible deterioration of the LVOTO or the development of arrhythmias.

clinical-multiple-myocardial-tubers

Figure 1: An echocardiogram showed multiple myocardial tubers.

clinical-parasternal-long-axis

Figure 2: A parasternal long axis view.

clinical-sub-ependymal-hematomas

Figure 3: MRI showing sub-ependymal hematomas.

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