Association of Pectus Excavatum With Ventricular Remodelling and Mitral Valve Abnormalities in Marfan Syndrome

Author:

Abdulmonem L Hashem Dalia1,Chan Victor Siang Hua1,Hanneman Kate1ORCID,Wald Rachel M.12,Thavendiranathan Paaladinesh12,Ouzounian Maral3,Oechslin Erwin2ORCID,Karur Gauri R.1ORCID

Affiliation:

1. Joint Department of Medical Imaging, Toronto General Hospital, University of Toronto, Toronto, ON, Canada

2. Division of Cardiology, Peter Munk Cardiac Centre, University Health Network, University of Toronto, Toronto, ON, Canada

3. Division of Cardiac Surgery, Department of Surgery, Peter Munk Cardiac Centre, University Health Network, University of Toronto, Toronto, ON, Canada

Abstract

Background: Marfan syndrome (MFS) is an inherited connective tissue disorder. Pectus excavatum (PEX) is common in MFS. The purpose was to evaluate the association of PEX with cardiovascular manifestations of MFS, biventricular size and function. Methods: MFS adults undergoing cardiac MRI were retrospectively evaluated. Exclusion criteria were incomplete cardiac MRI, significant artifacts, co-existent ischaemic or congenital heart disease. Haller Index (HI) ≥3.25 classified patients as PEX positive (PEX+) and PEX negative (PEX−). Cardiac MRI analysis included assessment of mitral valve prolapse (MVP), mitral annular disjunction (MAD), biventricular volumetry and aortic dimensions. Results: 212 MFS patients were included, 76 PEX+ and 136 PEX− (HI 8.3 ± 15.2 vs 2.3 ± 0.5, P < .001). PEX+ were younger (33.4 ± 12.0 vs 38.1 ± 14.3 years, P = .02) and similar in sex distribution (55% vs 63% male, P = .26) compared to PEX−. MVP and MAD were more frequent in PEX+ vs PEX− (43/76 [57%] vs 37/136 [27%], P < .001; 44/76 [58%] vs 50/136[37%], P = .003, respectively). PEX+ had higher right ventricular end-diastolic and end-systolic volumes (RVEDVi 92 ± 17mL/m2 vs 84 ± 22mL/m2, P = .04; RVESVi 44 ± 10 mL/m2 vs 39 ± 14 mL/m2, P = .02), lower RV ejection fraction (RVEF 52 ± 5% vs 55 ± 6%, P = .01) compared to PEX−. Left ventricular (LV) volumes, LVEF and aortic dimensions were similar. Conclusion: MFS adults with PEX have higher frequency of cardiac manifestations including MV abnormalities, increased RV volumes and lower RVEF compared to those without PEX. Awareness of this association is important for all radiologists who interpret aortic CT or MRI, where HI can be easily measured. PEX in MFS may suggest more severe disease expression necessitating careful screening for MV abnormalities and outcomes surveillance.

Publisher

SAGE Publications

Subject

Radiology, Nuclear Medicine and imaging,General Medicine

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