Myocardial Disease and Ventricular Arrhythmia in Marfan Syndrome – a prospective study

Autor: Katya De Groote, Anthony Demolder, Hans De Wilde, Daniel Devos, Danilo Babin, Luc Jordaens, Daniël De Wolf, Laura Muiño-Mosquera, Julie De Backer
Přispěvatelé: Pediatrics, Heartrhythmmanagement
Rok vydání: 2020
Předmět:
Male
0301 basic medicine
Cardiomyopathy
lcsh:Medicine
030204 cardiovascular system & hematology
Ventricular tachycardia
RECOMMENDATIONS
Sudden cardiac death
0302 clinical medicine
Medicine and Health Sciences
Mitral valve prolapse
Pharmacology (medical)
Prospective Studies
skin and connective tissue diseases
MUTATION
Genetics (clinical)
Aortic dissection
Ejection fraction
General Medicine
Cardiac surgery
Cardiology
Female
Cardiomyopathies
ECHOCARDIOGRAPHY
Arrhythmia
Adult
musculoskeletal diseases
medicine.medical_specialty
congenital
hereditary
and neonatal diseases and abnormalities
HEART-TRANSPLANTATION
Heart failure
macromolecular substances
03 medical and health sciences
AORTIC DISSECTION
Internal medicine
medicine
Humans
cardiovascular diseases
CARDIOMYOPATHY
business.industry
Research
lcsh:R
Arrhythmias
Cardiac
ADULTS
medicine.disease
Myocardial disease
Marfan syndrome
030104 developmental biology
Case-Control Studies
business
Zdroj: ORPHANET JOURNAL OF RARE DISEASES
Orphanet Journal of Rare Diseases, Vol 15, Iss 1, Pp 1-11 (2020)
Orphanet Journal of Rare Diseases
ISSN: 1750-1172
DOI: 10.21203/rs.3.rs-37970/v2
Popis: Background Aortic root dilatation and—dissection and mitral valve prolapse are established cardiovascular manifestations in Marfan syndrome (MFS). Heart failure and arrhythmic sudden cardiac death have emerged as additional causes of morbidity and mortality. Methods To characterize myocardial dysfunction and arrhythmia in MFS we conducted a prospective longitudinal case–control study including 86 patients with MFS (55.8% women, mean age 36.3 yr—range 13–70 yr–) and 40 age—and sex-matched healthy controls. Cardiac ultrasound, resting and ambulatory ECG (AECG) and NT-proBNP measurements were performed in all subjects at baseline. Additionally, patients with MFS underwent 2 extra evaluations during 30 ± 7 months follow-up. To study primary versus secondary myocardial involvement, patients with MFS were divided in 2 groups: without previous surgery and normal/mild valvular function (MFS-1; N = 55) and with previous surgery or valvular dysfunction (MFS-2; N = 31). Results Compared to controls, patients in MFS-1 showed mild myocardial disease reflected in a larger left ventricular end-diastolic diameter (LVEDD), lower TAPSE and higher amount of (supra) ventricular extrasystoles [(S)VES]. Patients in MFS-2 were more severely affected. Seven patients (five in MFS-2) presented decreased LV ejection fraction. Twenty patients (twelve in MFS-2) had non-sustained ventricular tachycardia (NSVT) in at least one AECG. Larger LVEDD and higher amount of VES were independently associated with NSVT. Conclusion Our study shows mild but significant myocardial involvement in patients with MFS. Patients with previous surgery or valvular dysfunction are more severely affected. Evaluation of myocardial function with echocardiography and AECG should be considered in all patients with MFS, especially in those with valvular disease and a history of cardiac surgery.
Databáze: OpenAIRE
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