Huge Pericardial Rhabdomyoma with Hypoplastic Left Ventricle and Lung

ABSTRACT

We describe the clinical course and autopsy findings of a female fetus with hydrops fetalis due to a huge pericardial rhabdomyoma. Fetal echocardiography at 21 weeks gestation demonstrated a huge tumor in the left ventricle. The fetus died of hydrops fetalis due to cardiac dysfunction at 24 weeks gestation. Autopsy demonstrated that the tumor protruded from the epicardial region of the apex into the pericardial cavity and induced a hypoplastic left ventricle and lung. Microscopically, the cardiac tumor showed characteristics of rhabdomyoma. This localization of cardiac rhabdomyoma is rare, but we remain aware of the possibility of an unusual and rapid progression of cardiac rhabdomyoma.

KEYWORDS

Pericardial rhabdomyoma - hypoplastic left ventricle - fetal death

REFERENCES

• 1 Issacs J R. Cardiac tumors. In: Gilbert-Barness E Potter's Pathology of the Fetus and Infant. St. Louis; Mosby 1997: 1319-1323
• 2 Holley D G, Martin G R, Brenner J I et al.. Diagnosis and management of fetal cardiac tumors: a multicenter experience and review of published reports.  J Am Coll Cardiol. 1995;  26 516-520
• 3 Bader R S, Chitayat D, Kelly E et al.. Fetal rhabdomyoma: prenatal diagnosis, clinical outcome, and incidence of associated tuberous sclerosis complex.  J Pediatr. 2003;  143 620-624
• 4 Sallee D, Spector M L, van Heeckeren D W, Patel C R. Primary pediatric cardiac tumors: a 17 year experience.  Cardiol Young. 1999;  9 155-162
• 5 Nir A, Ekstein S, Nazari M, Raas-Rothschild A, Rein AJJT. Rhabdomyoma in the fetus: illustration of tumor growth during the second half of gestation.  Pediatr Cardiol. 2001;  22 515-518
• 6 Soltan M H, Keohane C. Hydrops fetalis due to congenital cardiac rhabdomyoma: case report.  Br J Obstet Gynaecol. 1981;  88 771-773
• 7 Tworetzky W, McElhinney D B, Margossian R et al.. Association between cardiac tumors and tuberous sclerosis in the fetus and neonate.  Am J Cardiol. 2003;  92 487-489
• 8 Fesslova V, Villa L, Rizzuti T, Mastrangelo M, Mosca F. Natural history and long-term outcome of cardiac rhabdomyomas detected prenatally.  Prenat Diagn. 2004;  24 241-248
• 9 Calhoun B C, Watson P T, Hegge F. Ultrasound diagnosis of an obstructive cardiac rhabdomyoma with severe hydrops and hypoplastic lungs: a case report.  J Reprod Med. 1991;  36 317-319
• 10 Russell G A, Dhasmana J P, Berry P J, Gilbert-Barness E F. Coexistent cardiac tumors and malformations of the heart.  Int J Cardiol. 1989;  22 89-98
• 11 Watanabe T, Hojo Y, Kozaki T, Nagashima M, Ando M. Hypoplastic left heart syndrome with rhabdomyoma of the left ventricle.  Pediatr Cardiol. 1991;  12 121-122
• 12 Fouron J-C. Fetal cardiovascular physiology. In: Allan L, Hornberger L, Sarland G Textbook of Fetal Cardiology. London; Greenwich Medical Media 2000: 29-53
• 13 Roach E S, Smith M, Huttenlocher P, Bhat M, Alcorn D, Hawley L. Diagnostic criteria: tuberous sclerosis complex. Report of the Diagnostic Criteria Committee of the National Tuberous Sclerosis Association.  J Child Neurol. 1992;  7 221-224
• 14 Bosi G, Lintermans J P, Pellegrino P A, Svaluto-Morelo G, Viers V. The natural history of cardiac rhabdomyoma with and without tuberous sclerosis.  Acta Paediatr. 1996;  85 928-931