Prospective risk stratification of sudden cardiac death in Marfan's syndrome

Hoffmann, Boris; Rybczynski, Meike; Rostock, Thomas; Servatius, Helge; Drewitz, Imke; Steven, Daniel; Aydin, Ali; Sheikhzadeh, Sara; Darko, Vivien; Kodolitsch, Yskert Von; Willems, Stephan

doi:10.1016/j.ijcard.2012.06.036

Cited by 52 publications

(79 citation statements)

References 39 publications

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“…All these processes, to some extent, take place in the organism of patients and HRV decrease reflects the degree of metabolic derangements and heart tissue damage. The growing interest in HRV as the prognostic and risk stratification marker for cardiovascular diseases also appears to be important for MS [12]. Moreover, monitoring of HRV for a follow-up observation of disease progression could serve as a sensitive indicator reflecting dynamics of treatment efficiency [15].…”

Section: Discussionmentioning

confidence: 99%

“…The other study from this group includes HRV data from 24 h monitoring. However, this paper focused on stratification of sudden cardiac death in MS, and the HRV was compared between two subgroups of patients without indication if it was high or low compared to healthy subjects [12]. There are also two papers reporting increased fatigue and orthostatic intolerance in MS patients.…”

Section: Introductionmentioning

confidence: 99%

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A marked decrease in heart rate variability in Marfan syndrome patients with confirmed FBN1 mutations

Cherkas

Zhuraev

2016

Cardiol J.

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Results: Heart rates in MS patients with the FBN1 mutation were increased in both the supine position and orthostatic test (p < 0.001). Most of the time-domain (standard deviation, pNN50) and frequency-domain (total power, very low, low, and high frequency) parameters of HRV were significantly reduced in the MS patients (p < 0.001).Conclusions: A marked decrease in HRV, documented in the study, may be an important clinical feature in MS patients with confirmed FBN1 gene mutations. (Cardiol J 2016; 23, 1: 23-33)

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

A marked decrease in heart rate variability in Marfan syndrome patients with confirmed FBN1 mutations

Cherkas

Zhuraev

2016

Cardiol J.

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“…Their left ventricular volume in both systolic and diastolic phase was larger than in controls, and ejection fraction was reduced [ 21 ]. This was thought to be due to increased left ventricular afterload caused by aortic root stiffness [ 22 ]. Extracellular matrix remodelling and abnormal TGF-β due to fi brillin defi ciency also contribute to impaired left ventricular function [ 22 ].…”

Section: Left Ventricular Dilatation and Dysfunctionmentioning

confidence: 99%

“…This was thought to be due to increased left ventricular afterload caused by aortic root stiffness [ 22 ]. Extracellular matrix remodelling and abnormal TGF-β due to fi brillin defi ciency also contribute to impaired left ventricular function [ 22 ].…”

Section: Left Ventricular Dilatation and Dysfunctionmentioning

confidence: 99%

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Cardiac Management

Isekame¹,

Gati²

2016

Diagnosis and Management of Marfan Syndrome

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Association of Mitral Annular Disjunction With Cardiovascular Outcomes Among Patients With Marfan Syndrome

et al. 2021

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IMPORTANCE Mitral annular disjunction (MAD) has received particular interest in patients with mitral valve prolapse, ventricular tachycardia, and sudden cardiac death. The clinical significance of MAD for patients with Marfan syndrome (MFS) remains largely unexplored. OBJECTIVE To define the prevalence of MAD and examine its association with cardiovascular outcomes and arrhythmia among patients with MFS. DESIGN, SETTING, AND PARTICIPANTS This retrospective, single-center cohort study included 142 patients with a diagnosis of MFS based on the revised Ghent criteria and a confirmed (likely) pathogenic variant in the FBN1 gene who underwent regular follow-up between January 1, 2004, and December 31, 2019. MAIN OUTCOMES AND MEASURES The presence of MAD was assessed by echocardiography, and the extent of MAD was categorized in tertiles. Patients also underwent resting electrocardiography and 24-hour Holter monitoring. Outcomes included aortic events (aortic dissection or prophylactic aortic surgery), arrhythmic events (defined as sustained ventricular tachycardia or sudden cardiac death), and mitral valve surgery. RESULTS A total of 142 patients (72 female patients [51%]; median age at first examination, 25 years [range, 2-64 years]) were evaluated. Forty-eight patients (34%) had MAD. Patients with MAD had larger aortic root z scores than patients without MAD (4.1 [interquartile range, 2.8-5.7] vs 3.0 [interquartile range, 1.8-4.0]; P < .001) and more often had mitral valve prolapse (34 of 48 [71%] vs 14 of 94 [15%]; P < .001), ventricular ectopy (14 of 33 [42%] vs 15 of 70 [21%]; P = .03), and nonsustained ventricular tachycardia (13 of 33 [39%] vs 12 of 70 [17%]; P = .01). During follow-up, aortic events occurred at similar rates among patients with vs without MAD (15 of 43 [35%] vs 21 of 84 [25%]; P = .24), but patients in the upper MAD tertile (>10 mm) showed a higher occurrence of aortic events compared with patients with MAD of 10 mm or smaller (9 of 15 [60%] vs 6 of 28 [21%]; P = .01). Patients with arrhythmic events (n = 5) and patients requiring mitral valve surgery (n = 7) were observed exclusively in the group displaying MAD.CONCLUSIONS AND RELEVANCE This study suggests that MAD among patients with MFS is associated with the occurrence of arrhythmic events, a higher need for mitral valve intervention, and, among patients with extensive MAD, more aortic events. Cardiac imaging for patients with MFS should consider the assessment of MAD as a potential marker for adverse outcomes.

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Prospective risk stratification of sudden cardiac death in Marfan's syndrome

Cited by 52 publications

References 39 publications

A marked decrease in heart rate variability in Marfan syndrome patients with confirmed FBN1 mutations

A marked decrease in heart rate variability in Marfan syndrome patients with confirmed FBN1 mutations

Cardiac Management

Association of Mitral Annular Disjunction With Cardiovascular Outcomes Among Patients With Marfan Syndrome

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