Predictors of Outcome of Mitral Valve Prolapse in Patients With the Marfan Syndrome

Rybczynski, Meike; Treede, Hendrik; Sheikhzadeh, Sara; Groene, Eike F.; Bernhardt, A.; Hillebrand, Mathias; Mir, Thomas S.; Kühne, K.; Koschyk, Dietmar; Robinson, Peter N.; Berger, Jürgen; Reichenspurner, Hermann; Meinertz, Thomas; Kodolitsch, Yskert Von

doi:10.1016/j.amjcard.2010.08.070

Cited by 40 publications

(23 citation statements)

References 29 publications

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“…Cardiovascular manifestations often do not become overt and diagnosed until the third decade of life [10]. By that time, progressive aortic dilatation and aneurysms often dictate aortic and mitral valve surgery [68,72]. As shown in a recent meta-analysis, the mean age of Marfan syndrome patients undergoing cardiac surgery lies somewhere in their early/mid-thirties [14].…”

Section: Discussionmentioning

confidence: 99%

The economic impact of Marfan syndrome: a non-experimental, retrospective, population-based matched cohort study

Achelrod

Blankart

Linder

et al. 2014

Orphanet J Rare Dis

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BackgroundMarfan syndrome is a rare disease of the connective tissues, affecting multiple organ systems. Elevated morbidity and mortality in these patients raises the issue of costs for sickness funds and society. To date, there has been no study analysing the costs of Marfan syndrome from a sickness fund and societal perspective.ObjectiveTo estimate excess health resource utilisation, direct (non-)medical and indirect costs attributable to Marfan syndrome from a healthcare payer and a societal perspective in Germany in 2008.MethodsA retrospective matched cohort study design is applied, using claims data. For isolating the causal effect of Marfan syndrome on excess costs, a genetic matching algorithm was used to reduce differences in observable characteristics between Marfan syndrome patients and the control group. 892 patients diagnosed with Marfan syndrome (ICD-10 Q87.4) were matched from a pool of 26,645 control individuals. After matching, we compared health resource utilisation and costs.ResultsFrom the sickness fund perspective, an average Marfan syndrome patient generates excess annual costs of €2496 compared with a control individual. From the societal perspective, excess annual costs amount to €15,728. For the sickness fund, the strongest cost drivers are inpatient treatment and care by non-physicians. From the sickness fund perspective, the third (25–41 years) and first (0–16 years) age quartiles reveal the greatest surplus in total costs. Marfan syndrome patients have 39% more physician contacts, a 153% longer average length of hospital stay, 119% more inpatient stays, 33% more prescriptions, 236% more medical imaging and 20% higher average prescription costs than control individuals. Depending on the prevalence, the economic impact from the sickness fund perspective ranges between €24.0 million and €61.4 million, whereas the societal economic impact extends from €151.3 million to €386.9 million.ConclusionsRelative to its low frequency, Marfan syndrome requires high healthcare expenditure. Not only the high costs of Marfan syndrome but also its burden on patients’ lives call for more awareness from policy-makers, physicians and clinical researchers. Consequently, the diagnosis and treatment of Marfan syndrome should begin as soon as possible in order to prevent disease complications, early mortality and substantial healthcare expenditure.

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Section: Discussionmentioning

confidence: 99%

The economic impact of Marfan syndrome: a non-experimental, retrospective, population-based matched cohort study

Achelrod

Blankart

Linder

et al. 2014

Orphanet J Rare Dis

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“…During ventricular contraction, the mitral valve has to withstand its force, and prolapse of posterior leafl et is more common [ 17 ]. In 2011, Rybczynski and Treede, reported the prevalence of MVP in MFS is 40 %, severe MR is 12 %, and infective endocarditis is 2.5 % [ 18 ]. The prevalence of more severe myxomatous mitral valve thickening with prolapse is 25 % in MFS patients.…”

Section: Mitral Valvementioning

confidence: 96%

“…Mitral valve (MV) infective endocarditis, heart failure, MV replacement and repair were seen in 28 %, predicted by fl oppy mitral valve and mild or moderate MR. Aortic dilatation, dural ectasia, and new mutation cause were not related to outcome. Neither blood pressure nor medication (Beta-blocker (BB) and Angiotensin Receptor Blocker (ARB)) had any effect on MV related outcome [ 18 ].…”

Section: Mitral Valvementioning

confidence: 99%

Cardiac Management

Isekame¹,

Gati²

2016

Diagnosis and Management of Marfan Syndrome

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“…In contrast to idiopathic MVP, for which the average age was 65 years and the frequency of MVP-related events was 13%, Rybczynski et al 13 found that patients with MaSy experienced clinical events almost 30 years earlier and at a frequency more than twice. Flail leaflet was an independent predictor of progression of MR and MV-related clinical events.…”

Section: Marfan Syndromementioning

confidence: 98%

Cardiac Valve Involvement in Systemic Diseases: A Review

Grimaldi

Vermi

Pappalardo

et al. 2013

Clinical Cardiology

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Increasing age and new trends of mixed populations have newly aroused interest in valvular heart disease in the developed countries still in need of new clinical insights. In the clinical setting of systemic diseases, the proper assessment of cardiovascular abnormalities may be challenging, and the characterization of valvular involvement might help to recognize the underlying disease and cardiac sequelae. Prompt identification of valvular lesions may, therefore, also be useful for differential diagnosis. This article reviews the cardiac involvement in systemic diseases from etiology and background definition to echocardiographic assessment and clinical interpretation.The authors have no funding, financial relationships, or conflicts of interest to disclose.

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Predictors of Outcome of Mitral Valve Prolapse in Patients With the Marfan Syndrome

Cited by 40 publications

References 29 publications

The economic impact of Marfan syndrome: a non-experimental, retrospective, population-based matched cohort study

The economic impact of Marfan syndrome: a non-experimental, retrospective, population-based matched cohort study

Cardiac Management

Cardiac Valve Involvement in Systemic Diseases: A Review

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