Aortic regurgitation in Marfan syndrome patients who underwent prophylactic surgery: A single-center experience

Abstract

Background: Marfan syndrome is an inherited connective tissue disease that causes aortic root dilatation and dissection and requires surgical intervention. Apart from emergent surgery for aortic dissection or aortic aneurysmal rupture, prophylactic surgical intervention can also be administered, depending on the severity of aortic root dilatation. The direct relationship between surgical intervention and aortic regurgitation was seldom mentioned in previous studies. Methods: A retrospective cohort study was designed to determine the clinical presentations of prophylactic surgery in patients with Marfan syndrome. Between January 2009 and May 2019, 112 patients, adolescents and young adults, treated in the Department of Pediatric Cardiology of Taipei Veterans General Hospital, were enrolled. All patients' sex, body measurements, echocardiography reports, and surgical notes were collected for statistical analysis. Results: Among the participants, nine patients (8%) underwent the Bentall procedure, and the other 103 did not receive surgical intervention. The operation group had a larger aortic root size (4.89 vs 2.86 cm, p < 0.001), more dilated left ventricle (4.81 vs 4.1 cm, p = 0.002), and higher prevalence of moderate and severe aortic regurgitation (66% vs 1%, p < 0.001) than the nonoperation group. Conclusion: Among adolescents and young adults with Marfan syndrome, echocardiographic presentation of aortic root dilatation, left ventricular dilatation, and significant aortic regurgitation was significantly associated with prophylactic surgical intervention. According to the study, significant aortic regurgitation should also be considered as an important indication for prophylactic surgery.

Frequency of Cardiac Valvulopathies in Patients With Marfan Syndrome: A Systematic Review and Meta-Analysis

“… De Backer et al [ 22 ], Fan et al [ 23 ], Brown et al [ 24 ], Roman et al [ 25 ], Gillinov et al [ 26 ], Bruno et al [ 27 ], Lacro et al [ 28 ], Attias et al [ 29 ], Wozniak-Mielczarek et al [ 30 ], Ozdemir et al [ 31 ], Gu et al [ 32 ], Rybczynski et al [ 33 ], Mueller et al [ 34 ], Seo et al [ 35 ], Marsalese et al [ 36 ], Tayel et al [ 37 ], Kunkala et al [ 38 ], Pyeritz et al [ 39 ], van Karnebeek et al [ 40 ], Veldhoen et al [ 41 ], Lopez et al [ 42 ], Geva et al [ 43 ], Sisk et al [ 44 ], Hirata et al [ 45 ], Figueiredo et al [ 46 ], Come et al [ 47 ], Freed et al [ 48 ], Pan et al [ 49 ], Yetman et al [ 50 ], Taub et al [ 51 ], Geva et al [ 52 ], Espınola-Zavaleta et al [ 21 ] …”

“… De Backer et al [ 22 ], Fan et al [ 23 ], Forteza et al [ 53 ], Svensson et al [ 54 ], Gillinov et al [ 26 ], Lacro et al [ 28 ], Attias et al [ 29 ], Wozniak-Mielczarek et al [ 30 ], Ozdemir et al [ 31 ], Gu et al [ 32 ], Rybczynski et al [ 33 ], Mueller et al [ 34 ], Seo et al [ 35 ], Tayel et al [ 37 ], Pyeritz et al [ 39 ], van Karnebeek et al [ 40 ], Veldhoen et al [ 41 ], Lopez et al [ 42 ], Sisk et al [ 44 ], Hirata et al [ 45 ], Davis et al [ 55 ], Yetman et al [ 50 ], Geva et al [ 52 ] …”

“…Overall, it has been noted that echocardiography improves the routine detection of underlying cardiovascular disease in patients with MFS when compared to physical examination (auscultation) [ 47 ]. Given that the valvular damage caused by MFS usually manifests silently in the early stages of childhood and is progressive throughout life, serial echocardiography follow-up can often facilitate elective surgery and is imperative [ 21 ], especially in the current treatment era where prophylactic surgery has shown to be crucial in preventing MFS-related valvular complications, with a resultant improvement in life expectancy [ 23 , 53 ].…”