Cardiology in the Young

Original Articles

Considerations for Prenatal Counselling of Patients with Cardiac Rhabdomyomas based on their Cardiac and Neurologic Outcomes

Sabrina C. Degueldrea1, Priya Chockalingama1, Yvan Mivelaza1, Stefano Di Bernardoa1, Jean-Pierre Pfammattera2, Catherine Barreaa3, Nicole Sekarskia1, Pierre-Yves Jeanneta1, Jean-Claude Fourona4, Yvan Viala1 and Erik J. Meijbooma1 c1

a1 Department of OBGYN and Divisions of Pediatric Cardiology and Neurology, University Hospital of Lausanne, Switzerland

a2 Division of Pediatric Cardiology, University Hospital of Bern, Switzerland

a3 Divison of Pediatric Cardiology, University Hospital of Saint-Luc, Bruxelles, Belgium

a4 Division of Pediatric Cardiology, Centre Hospitalier Universitaire de Sainte-Justine, Montreal, Canada

Abstract

Cardiac rhabdomyomas are benign cardiac tumours with few cardiac complications, but with a known association to tuberous sclerosis that affects the neurologic outcome of the patients. We have analysed the long-term cardiac and neurological outcomes of patients with cardiac rhabdomyomas in order to allow comprehensive prenatal counselling, basing our findings on the records of all patients seen prenatally and postnatally with an echocardiographic diagnosis of cardiac rhabdomyoma encountered from August, 1982, to September, 2007. We analysed factors such as the number and the location of the tumours to establish their association with a diagnosis of tuberous sclerosis, predicting the cardiac and neurologic outcomes for the patients.

Cardiac complications include arrhythmias, obstruction of the ventricular outflow tracts, and secondary cardiogenic shock. Arrhythmias were encountered most often during the neonatal period, with supraventricular tachycardia being the commonest rhythm disturbance identified. No specific dimension or location of the cardiac rhabdomyomas predicted the disturbances of rhythm.

The importance of the diagnosis of tuberous sclerosis is exemplified by the neurodevelopmental complications, with four-fifths of the patients showing epilepsy, and two-thirds having delayed development. The presence of multiple cardiac tumours suggested a higher risk of being affected by tuberous sclerosis. The tumours generally regress after birth, and cardiac-related problems are rare after the perinatal period. Tuberous sclerosis and the associated neurodevelopmental complications dominate the clinical picture, and should form an important aspect of the prenatal counselling of parents.

(Received February 12 2009)

(Accepted July 17 2009)

(Online publication January 22 2010)

Correspondence:

c1 Correspondence to: Erik J. Meijboom, MD, PhD, FACC, Division Pediatric Cardiology, Centre Hospitalier Universitaire Vaudois (CHUV), BH 11, 626, Rue du Bugnon 46, 1011, Lausanne, Switzerland. Tel: +41 21 314 3553; Fax: +41 21 314 3665; E-mail: erik.meijboom@chuv.ch