Ann Thorac Surg 2005;80:1529
© 2005 The Society of Thoracic Surgeons
Images in cardiothoracic surgery
Fetal Rhabdomyoma Causing Postnatal Critical Left Ventricular Outflow Tract Obstruction
Catherine M. Ikemba, MD
a
,
*
,
Benjamin W. Eidem, MD
a
,
V. Vivian Dimas, MD
a
,
Mary P. O'Day, MD
b
,
Charles D. Fraser, Jr, MD
c
a Division of Cardiology, Texas Children's Hospital/Baylor College of Medicine, Houston, Texas, USA
b Department of Obstetrics and Gynecology, Division of Maternal-Fetal Medicine, Baylor College of Medicine, Houston, Texas, USA
c Division of Congenital Heart Surgery, Michael E. DeBakey Department of Surgery, Texas Children's Hospital/Baylor College of Medicine, Houston, Texas
* Address reprint requests to Dr Ikemba, Division of Cardiology, Children's Medical Center of Dallas, 1935 Motor St, Dallas, TX 75235 (Email: ccikemba{at}yahoo.com).
Level II obstetric ultrasound performed at 32 weeks' gestation on a diabetic mother revealed an echogenic aortic and subaortic region with possible left ventricular outflow tract (LVOT) obstruction. Subsequent fetal echocardiography at 34 and 38 weeks' gestation revealed a moderately obstructive LVOT mass but no evidence of ventricular dysfunction, fetal hydrops, or hemodynamic compromise (Fig 1A).
After birth, the neonate was clinically asymptomatic. Echocardiography of the apical 4-chamber view (Fig 1B) and parasternal long-axis projection (Fig 1C) confirmed the presence of a large, homogenous, subaortic mass arising from the interventricular septum and obstructing the LVOT (LV = left ventricle; RA = right atrium; RV = right ventricle). A significant portion of the systemic output was supplied by the ductus arteriosus; therefore, intravenous prostaglandin E1 was begun. On postnatal day 4, transaortic resection of the obstructive mass was performed. Surgical inspection revealed 80% obstruction of the LVOT by a pedunculated mass surrounded by a dense fibrous capsule that originated from the leftward aspect of the interventricular septum and was adherent to the right aortic leaflet. The aortic valve leaflets appeared normal, and no additional intracardiac masses were noted. Pathology revealed a 15 x 13-mm bosselated, polypoid-like structure consistent with rhabdomyoma on microscopic examination. The infant's postoperative recovery was unremarkable. A postoperative echocardiogram revealed a minimal residual immobile, nonobstructive mass adherent to the interventricular septum. The infant was discharged home on postnatal day 15 in excellent condition.
The natural history of cardiac rhabdomyoma is spontaneous regression, with partial or complete regression in more than 50% of patients during a mean follow-up of 3 years [1]. Only 4 of 44 patients with cardiac rhabdomyoma exhibited outflow tract gradients greater than 40 mm Hg [1]. In contrast, fetal rhabdomyoma may grow during the second and third trimester, resulting in a hemodynamically significant obstructive lesion in the neonatal period after ductal closure. A review of fetal tumors described 5 of 19 causing some degree of obstruction, 1 of which led to fetal demise [2].
Our patient demonstrated an unusual presentation of a neonatal rhabdomyoma that caused critical LVOT obstruction. Early identification with fetal echocardiography enabled expeditious postnatal treatment with lifesaving surgical resection of the obstructive mass.
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References
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- Beghetti M, Gow RM, Haney I, Mawson J, Williams WG, Freedom RM. Pediatric primary benign cardiac tumorsa 15-year review. Am Heart J 1997;134:1107-1114.[Medline]
- Holley DG, Martin GR, Brenner JI, et al. Diagnosis and management of fetal cardiac tumorsa multicenter experience and review of published reports. J Am Coll Cardiol 1995;26:516-520.[Abstract]
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