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Considerations for prenatal counselling of patients with cardiac rhabdomyomas based on their cardiac and neurologic outcomes.
Cardiol Young. 2010 Feb; 20(1):18-24.CY

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.

Authors+Show Affiliations

Department of OBGYN and Divisions of Pediatric Cardiology and Neurology, University Hospital of Lausanne, Switzerland.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Multicenter Study

Language

eng

PubMed ID

20092673

Citation

Degueldre, Sabrina C., et al. "Considerations for Prenatal Counselling of Patients With Cardiac Rhabdomyomas Based On Their Cardiac and Neurologic Outcomes." Cardiology in the Young, vol. 20, no. 1, 2010, pp. 18-24.
Degueldre SC, Chockalingam P, Mivelaz Y, et al. Considerations for prenatal counselling of patients with cardiac rhabdomyomas based on their cardiac and neurologic outcomes. Cardiol Young. 2010;20(1):18-24.
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. (2010). Considerations for prenatal counselling of patients with cardiac rhabdomyomas based on their cardiac and neurologic outcomes. Cardiology in the Young, 20(1), 18-24. https://doi.org/10.1017/S1047951109992046
Degueldre SC, et al. Considerations for Prenatal Counselling of Patients With Cardiac Rhabdomyomas Based On Their Cardiac and Neurologic Outcomes. Cardiol Young. 2010;20(1):18-24. PubMed PMID: 20092673.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Considerations for prenatal counselling of patients with cardiac rhabdomyomas based on their cardiac and neurologic outcomes. AU - Degueldre,Sabrina C, AU - Chockalingam,Priya, AU - Mivelaz,Yvan, AU - Di Bernardo,Stefano, AU - Pfammatter,Jean-Pierre, AU - Barrea,Catherine, AU - Sekarski,Nicole, AU - Jeannet,Pierre-Yves, AU - Fouron,Jean-Claude, AU - Vial,Yvan, AU - Meijboom,Erik J, Y1 - 2010/01/22/ PY - 2010/1/23/entrez PY - 2010/1/23/pubmed PY - 2010/7/16/medline SP - 18 EP - 24 JF - Cardiology in the young JO - Cardiol Young VL - 20 IS - 1 N2 - 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. SN - 1467-1107 UR - https://www.unboundmedicine.com/medline/citation/20092673/Considerations_for_prenatal_counselling_of_patients_with_cardiac_rhabdomyomas_based_on_their_cardiac_and_neurologic_outcomes_ L2 - https://www.cambridge.org/core/product/identifier/S1047951109992046/type/journal_article DB - PRIME DP - Unbound Medicine ER -