Biventricular Performance in Patients with Marfan Syndrome without Significant Valvular Disease: Comparison to Normal Subjects and Longitudinal Follow-Up

Department of Cardiology, Leiden University Medical Center, Leiden, The Netherlands.
Journal of the American Society of Echocardiography: official publication of the American Society of Echocardiography (Impact Factor: 4.06). 12/2011; 24(12):1392-1399.e1. DOI: 10.1016/j.echo.2011.09.004
Source: PubMed


The presence and progressive nature of primary myocardial involvement in Marfan syndrome are debated. The aim of this study was to evaluate the clinical relevance of left ventricular (LV) and right ventricular (RV) strain in adult patients with Marfan syndrome without significant valvular disease.
Adult patients with Marfan syndrome (n = 50; mean age, 35.2 ± 12.9 years) were followed prospectively. Echocardiography was performed annually and consisted of comprehensive assessment of ventricular and valvular function. Using speckle-tracking imaging, the baseline strain values of the Marfan population were calculated and compared with the values of normal controls. The follow-up evaluations were used to assess changes in ventricular strain. The association between the incidence of adverse events (heart failure, [supra]ventricular arrhythmias, and proximal aorta surgery) and baseline strain values was investigated.
Compared with controls, patients with Marfan syndrome had significantly lower peak longitudinal LV strain (-18.9 ± 2.3% vs -20.1 ± 1.9%, P < .01) and RV strain (±26.9 ± 5.2% vs ±29.3 ± 4.25%, P < .01). The absolute changes in LV longitudinal, radial, and circumferential strain and RV longitudinal strain during a median 4 years of follow-up were 0.1 ± 2.8%, 1.12 ± 7.6%, 0.3 ± 3.7%, and 0.9 ± 5.5%, respectively, which was not statistically significant. Cox regression demonstrated that reduced LV or RV strain was not associated with adverse outcome (supraventricular arrhythmias, n = 3; proximal aorta surgery, n = 4).
This study suggests that patients with Marfan syndrome show lower ventricular strain and strain rate values compared with healthy controls. However, no relevant changes in LV and RV function occurred during midterm follow-up in patients with Marfan syndrome without valvular disease at baseline. Although ventricular strain and strain rate were mildly reduced in patients with Marfan syndrome, this did not affect outcomes negatively in the present study.

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    • "Progressive dilatation of the aorta is the main cardiovascular irregularity of MFS individuals, and acute aortic dissection originating from dilation of the ascending aorta is the leading cause of premature death in untreated MFS individuals [2]. Other manifestations, including spontaneous pneumothorax, apical blebs, striae atrophicae, and lumbosacral dural ectasia, can also be observed in MFS patients [3,4]. "
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