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

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Serval ID
serval:BIB_B3B7160AA3EF
Type
Article: article from journal or magazin.
Collection
Publications
Institution
Title
Considerations for prenatal counselling of patients with cardiac rhabdomyomas based on their cardiac and neurologic outcomes.
Journal
Cardiology In the Young
Author(s)
Degueldre S.C., Chockalingam P., Mivelaz Y., Di Bernardo S., Pfammatter J.P., Barrea C., Sekarski N., Jeannet P.Y., Fouron J.C., Vial Y., Meijboom E.J.
ISSN
1467-1107 (Electronic)
ISSN-L
1047-9511
Publication state
Published
Issued date
2010
Volume
20
Number
1
Pages
18-24
Language
english
Notes
Publication types: Journal Article ; Multicenter Study
Publication Status: ppublish
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.
Keywords
Child, Cohort Studies, Developmental Disabilities/diagnosis, Developmental Disabilities/epidemiology, Directive Counseling/methods, Disease Progression, Echocardiography/methods, Female, Fetal Diseases/ultrasonography, Follow-Up Studies, Gestational Age, Heart Neoplasms/complications, Heart Neoplasms/embryology, Humans, Infant, Infant, Newborn, Neuropsychological Tests, Pregnancy, Prenatal Care, Prenatal Diagnosis/methods, Retrospective Studies, Rhabdomyoma/complications, Rhabdomyoma/embryology, Tuberous Sclerosis/complications, Tuberous Sclerosis/embryology, Ultrasonography, Prenatal
Pubmed
Web of science
Open Access
Yes
Create date
28/05/2010 10:42
Last modification date
01/10/2019 6:19
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