Considerations for prenatal counselling of patients with cardiac rhabdomyomas based on their cardiac and neurologic outcomes

Degueldre, Sabrina C; Chockalingam, Priya; Mivelaz, Yvan; Di Bernardo, Stefano; Pfammatter, Jean-Pierre; Barrea, Catherine; Sekarski, Nicole; Jeannet, Pierre-Yves; Fouron, Jean-Claude; Vial, Yvan; Meijboom, Erik J (2010). Considerations for prenatal counselling of patients with cardiac rhabdomyomas based on their cardiac and neurologic outcomes. Cardiology in the young, 20(1), pp. 18-24. Cambridge: Cambridge University Press 10.1017/S1047951109992046

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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.

Item Type:

Journal Article (Original Article)

Division/Institute:

04 Faculty of Medicine > Department of Gynaecology, Paediatrics and Endocrinology (DFKE) > Clinic of Paediatric Medicine

UniBE Contributor:

Pfammatter, Jean-Pierre

ISSN:

1047-9511

Publisher:

Cambridge University Press

Language:

English

Submitter:

Factscience Import

Date Deposited:

04 Oct 2013 14:07

Last Modified:

27 Apr 2018 08:49

Publisher DOI:

10.1017/S1047951109992046

PubMed ID:

20092673

Web of Science ID:

000277330600003

BORIS DOI:

10.7892/boris.179

URI:

https://boris.unibe.ch/id/eprint/179 (FactScience: 196620)

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