Rhabdomyoma association with atrioventricular septal defect in an infant: a rare coincidence
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CASE REPORT
Rhabdomyoma association with atrioventricular septal defect in an infant: a rare coincidence Ilker Mercan 1
&
Muhammet Akyüz 1
&
Onur Isık 1
Received: 17 May 2020 / Revised: 21 June 2020 / Accepted: 6 July 2020 # Indian Association of Cardiovascular-Thoracic Surgeons 2020
Abstract Cardiac rhabdomyoma is the most common primary heart tumor in childhood. This tumor, which is frequently associated with tuberous sclerosis complex, mostly disappears in childhood with spontaneous regression. Surgical resection is required in case of outflow obstruction and arrhythmia and when protruding to disrupt the filling of the heart cavities. There are very few case series in the literature about rhabdomyoma, whose relationship with other congenital heart defects has not been clearly verified. In this study, we report our approach to the tumor during the corrective surgery of the infant, who was diagnosed with an atrioventricular septal defect and patent ductus arteriosus, and rhabdomyoma accompanying these malformations. We treated this asymptomatic rhabdomyoma with everolimus in line with the current literature, without excision. Keywords Rhabdomyoma . Infant . Atrioventricular septal defect
Introduction Cardiac rhabdomyoma is the most common primary heart tumor detected in infancy and childhood. The clinical course is generally benign. In reported studies, spontaneous regression was observed in the majority of cases. The relationship of this tumor, which was first described in 1862, with tuberous sclerosis (TS) is well established [1]. Although it has a good clinical course, surgical resection is inevitable when it causes ventricular outflow/inflow obstruction, myocardial dysfunction, or malignant arrhythmias. Despite many studies, very few congenital heart diseases have been reported to be associated with cardiac rhabdomyoma. There are studies that argue that rhabdomyoma may lead to any defect by causing a disruption in the embryological development steps of the heart. However, it is still controversial whether these two cardiac pathologies emerge as different pathologies [2]. In this case study, we report a rare case of TS with cerebral,
* Ilker Mercan [email protected] 1
Department of Pediatric Heart Surgery, Izmir University Of Health Sciences Tepecik Training And Research Hospital, Sanayi Caddesi No:7 Bornova, İzmir, Turkey
dermatological, and renal lesions associated with cardiac rhabdomyoma and atrioventricular septal defect (AVSD).
Case report A female child was referred to our clinic with symptoms of congestive heart failure, mild pulmonary hypertension (pulmonary artery pressure, 30 mmHg), and resistant to medical treatment. The patient was 3 months old and with 3.800 g of weight. During the examination, S1 hard, S2 double, grade 3/ 6 pansystolic murmur was heard in the lower left sternal region. The electrocardiogram was a sinus rhythm at 150 beats/ min with left axis deviation. The patient had growth retardation and tachypnea with an oxygen saturation of 95% on room air. On chest x-ray
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