title: Hypoplastic left heart syndrome secondary to intrauterine rhabdomyoma necessitating single ventricle palliation.
abstract: Rhabdomyoma, a benign hamartomatous tumor of the cardiac embryonic myocyte, is the most common intrauterine cardiac tumor and accounts for 0.12% of prenatal fetal studies. Fetal cardiac rhabdomyomas increase in size during second and early third trimester and spontaneously regress postnatally. The clinical presentation is usually benign, however, compromise of the ventricular outflow tract leading to decreased cardiac output and fetal death have been reported. We present a case of large cardiac rhabdomyoma in a fetus that might have caused complete left ventricular outflow tract obstruction and development of hypoplastic left heart syndrome (HLHS) necessitating postnatal single ventricle palliation therapy. The clinical course and outcomes of prenatally diagnosed cardiac rhabdomyoma are reviewed and theories of the development of hypoplastic left heart syndrome are explored.
authors.affiliation: Department of Pediatrics, Division of Cardiology, Oklahoma University Children Hospital, Oklahoma University Health Sciences Center, Oklahoma City, Oklahoma, USA.
authors.forename: Arshid
authors.initials: A
authors.lastname: Mir
authors.affiliation: Department of Pediatrics, Division of Cardiology, University of Texas Southwestern Medical Center, Children Medical Center Dallas, Dallas, Texas, USA.
authors.forename: Catherine Minor
authors.initials: CM
authors.lastname: Ikemba
authors.affiliation: Department of Pediatrics, Division of Cardiology, University of Texas Southwestern Medical Center, Children Medical Center Dallas, Dallas, Texas, USA.
authors.forename: Surendranath R
authors.initials: SR
authors.lastname: Veeram Reddy
keywords: Cardiac tumor; hypoplastic left heart syndrome (HLHS); rhabdomyoma