Key result
Pectus excavatum in adults with Marfan syndrome was associated with a higher frequency of mitral valve prolapse (57% vs 27%, P<0.001) and mitral annular disjunction (58% vs 37%, P=0.003).
Why the study?
Pectus excavatum is common in Marfan syndrome, but its association with cardiovascular manifestations, biventricular size, and function was not fully evaluated.
Does pectus excavatum associate with worse ventricular remodeling and mitral valve abnormalities in adults with Marfan syndrome?
Observational (n=212)
Does pectus excavatum associate with worse ventricular remodeling and mitral valve abnormalities in adults with Marfan syndrome?
Absolute Event Rate: 57% vs 27%
p-value: p=<.001
In adults with Marfan syndrome, the presence of pectus excavatum is associated with a higher frequency of mitral valve abnormalities, increased right ventricular volumes, and lower right ventricular ejection fraction.
Pectus excavatum may flag higher mitral abnormality risk in Marfan syndrome; leaves open need for targeted imaging and outcome studies.
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.
No takes yet. Share an insight, caveat, or question.
Hashem et al. (2022) conducted an observational in Marfan syndrome (n=212). Pectus excavatum (PEX) vs. Without pectus excavatum (PEX-) was evaluated on Mitral valve prolapse (MVP) (p=<.001). Pectus excavatum in adults with Marfan syndrome was associated with a higher frequency of mitral valve prolapse (57% vs 27%, P<0.001) and mitral annular disjunction (58% vs 37%, P=0.003).
Synapse has enriched 5 closely related papers on similar clinical questions. Consider them for comparative context: