Neonatal rhabdomyoma causing right ventricular inflow obstruction with duct-dependent pulmonary blood flow: successful stenting of PDA.
We describe an infant with the antenatal diagnosis of a large intracardiac rhabdomyoma causing tricuspid inflow obstruction. Postnatal cyanosis and arterial hypoxemia prompted commencement of intravenous prostaglandin E1 with immediate improvement in saturations. Clinical and genetic testing confirmed diagnosis of tuberous sclerosis. To secure ductal patency, two tandem coronary stents were implanted via an antegrade approach. Several episodes of long RP atrio-ventricular re-entrant tachycardia were observed. At 7(1/2) months of age, the tumor was no longer obstructive to tricuspid inflow, ductal flow was highly restrictive and there was no recurrence of supraventricular tachycardia.
Ilina
Maria V
MV
Division of Cardiology, Department of Pediatrics, The Hospital for Sick Children, University of Toronto School of Medicine, Toronto, Ontario, Canada.
Jaeggi
Edgar T
ET
Lee
Kyong-Jin
KJ
D000328:Adult; D006328:Cardiac Catheterization; D017023:Coronary Angiography; D004374:Ductus Arteriosus, Patent / Q000150:complications / Q000175:diagnosis / Q000503:physiopathology / Q000628:therapy*; D018618:Echocardiography, Doppler, Color; D004562:Electrocardiography; D005260:Female; D005820:Genetic Testing; D006338:Heart Neoplasms / Q000150:complications* / Q000175:diagnosis / Q000235:genetics / Q000503:physiopathology; D006801:Humans; D007231:Infant, Newborn; D050498:Live Birth; D011296:Prenatal Diagnosis; D011652:Pulmonary Circulation; D015641:Radiography, Interventional; D012207:Rhabdomyoma / Q000150:complications* / Q000175:diagnosis / Q000235:genetics / Q000503:physiopathology; D015607:Stents; D016896:Treatment Outcome; D014402:Tuberous Sclerosis / Q000150:complications* / Q000175:diagnosis / Q000235:genetics / Q000503:physiopathology; D014694:Ventricular Outflow Obstruction / Q000209:etiology* / Q000503:physiopathology