Mitral valve disease in patients with marfan syndrome undergoing aortic root replacement

Meghana R. Kunkala, Hartzell V Schaff, Zhuo Li, Irina Volguina, Harry C. Dietz, Scott A. Lemaire, Joseph S. Coselli, Heidi Connolly

Research output: Contribution to journalArticle

12 Citations (Scopus)

Abstract

BACKGROUND - Cardiac manifestations of Marfan syndrome include aortic root dilation and mitral valve prolapse (MVP). Only scant data exist describing MVP in patients with Marfan syndrome undergoing aortic root replacement. METHODS AND RESULTS - We retrospectively analyzed data from 166 MFS patients with MVP who were enrolled in a prospective multicenter registry of patients who underwent aortic root aneurysm repair. Of these 166 patients, 9% had mitral regurgitation (MR) grade >2, and 10% had MR grade 2. The severity of MVP and MR was evaluated by echocardiography preoperatively and ≤3 years postoperatively. Forty-one patients (25%) underwent composite graft aortic valve replacement, and 125 patients (75%) underwent aortic valve-sparing procedures; both groups had similar prevalences of MR grade >2 (P=0.7). Thirty-three patients (20%) underwent concomitant mitral valve (MV) intervention (repair, n=29; replacement, n=4), including all 15 patients with MR grade >2. Only 1 patient required MV reintervention during follow-up (mean clinical follow-up, 31±10 months). Echocardiography performed 21±13 months postoperatively revealed MR >2 in only 3 patients (2%). One early death and 2 late deaths occurred. CONCLUSIONS - Although the majority of patients with Marfan syndrome who undergo elective aortic root replacement have MVP, only 20% have concomitant MV procedures. These concomitant procedures do not seem to increase operative risk. In patients with MR grade ≤2 who do not undergo a concomitant MV procedure, the short-term incidence of progressive MR is low; however, more follow-up is needed to determine whether patients with MVP and MR grade ≤2 would benefit from prophylactic MV intervention.

Original languageEnglish (US)
JournalCirculation
Volume128
Issue numberSUPPL.1
DOIs
StatePublished - Sep 10 2013

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Marfan Syndrome
Mitral Valve
Mitral Valve Insufficiency
Mitral Valve Prolapse
Aortic Valve
Echocardiography
Aortic Aneurysm
Registries
Dilatation

Keywords

  • Marfan syndrome
  • Mitral valve
  • Thoracic aorta

ASJC Scopus subject areas

  • Physiology (medical)
  • Cardiology and Cardiovascular Medicine

Cite this

Kunkala, M. R., Schaff, H. V., Li, Z., Volguina, I., Dietz, H. C., Lemaire, S. A., ... Connolly, H. (2013). Mitral valve disease in patients with marfan syndrome undergoing aortic root replacement. Circulation, 128(SUPPL.1). https://doi.org/10.1161/CIRCULATIONAHA.112.000113

Mitral valve disease in patients with marfan syndrome undergoing aortic root replacement. / Kunkala, Meghana R.; Schaff, Hartzell V; Li, Zhuo; Volguina, Irina; Dietz, Harry C.; Lemaire, Scott A.; Coselli, Joseph S.; Connolly, Heidi.

In: Circulation, Vol. 128, No. SUPPL.1, 10.09.2013.

Research output: Contribution to journalArticle

Kunkala, MR, Schaff, HV, Li, Z, Volguina, I, Dietz, HC, Lemaire, SA, Coselli, JS & Connolly, H 2013, 'Mitral valve disease in patients with marfan syndrome undergoing aortic root replacement', Circulation, vol. 128, no. SUPPL.1. https://doi.org/10.1161/CIRCULATIONAHA.112.000113
Kunkala, Meghana R. ; Schaff, Hartzell V ; Li, Zhuo ; Volguina, Irina ; Dietz, Harry C. ; Lemaire, Scott A. ; Coselli, Joseph S. ; Connolly, Heidi. / Mitral valve disease in patients with marfan syndrome undergoing aortic root replacement. In: Circulation. 2013 ; Vol. 128, No. SUPPL.1.
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AU - Kunkala, Meghana R.

AU - Schaff, Hartzell V

AU - Li, Zhuo

AU - Volguina, Irina

AU - Dietz, Harry C.

AU - Lemaire, Scott A.

AU - Coselli, Joseph S.

AU - Connolly, Heidi

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N2 - BACKGROUND - Cardiac manifestations of Marfan syndrome include aortic root dilation and mitral valve prolapse (MVP). Only scant data exist describing MVP in patients with Marfan syndrome undergoing aortic root replacement. METHODS AND RESULTS - We retrospectively analyzed data from 166 MFS patients with MVP who were enrolled in a prospective multicenter registry of patients who underwent aortic root aneurysm repair. Of these 166 patients, 9% had mitral regurgitation (MR) grade >2, and 10% had MR grade 2. The severity of MVP and MR was evaluated by echocardiography preoperatively and ≤3 years postoperatively. Forty-one patients (25%) underwent composite graft aortic valve replacement, and 125 patients (75%) underwent aortic valve-sparing procedures; both groups had similar prevalences of MR grade >2 (P=0.7). Thirty-three patients (20%) underwent concomitant mitral valve (MV) intervention (repair, n=29; replacement, n=4), including all 15 patients with MR grade >2. Only 1 patient required MV reintervention during follow-up (mean clinical follow-up, 31±10 months). Echocardiography performed 21±13 months postoperatively revealed MR >2 in only 3 patients (2%). One early death and 2 late deaths occurred. CONCLUSIONS - Although the majority of patients with Marfan syndrome who undergo elective aortic root replacement have MVP, only 20% have concomitant MV procedures. These concomitant procedures do not seem to increase operative risk. In patients with MR grade ≤2 who do not undergo a concomitant MV procedure, the short-term incidence of progressive MR is low; however, more follow-up is needed to determine whether patients with MVP and MR grade ≤2 would benefit from prophylactic MV intervention.

AB - BACKGROUND - Cardiac manifestations of Marfan syndrome include aortic root dilation and mitral valve prolapse (MVP). Only scant data exist describing MVP in patients with Marfan syndrome undergoing aortic root replacement. METHODS AND RESULTS - We retrospectively analyzed data from 166 MFS patients with MVP who were enrolled in a prospective multicenter registry of patients who underwent aortic root aneurysm repair. Of these 166 patients, 9% had mitral regurgitation (MR) grade >2, and 10% had MR grade 2. The severity of MVP and MR was evaluated by echocardiography preoperatively and ≤3 years postoperatively. Forty-one patients (25%) underwent composite graft aortic valve replacement, and 125 patients (75%) underwent aortic valve-sparing procedures; both groups had similar prevalences of MR grade >2 (P=0.7). Thirty-three patients (20%) underwent concomitant mitral valve (MV) intervention (repair, n=29; replacement, n=4), including all 15 patients with MR grade >2. Only 1 patient required MV reintervention during follow-up (mean clinical follow-up, 31±10 months). Echocardiography performed 21±13 months postoperatively revealed MR >2 in only 3 patients (2%). One early death and 2 late deaths occurred. CONCLUSIONS - Although the majority of patients with Marfan syndrome who undergo elective aortic root replacement have MVP, only 20% have concomitant MV procedures. These concomitant procedures do not seem to increase operative risk. In patients with MR grade ≤2 who do not undergo a concomitant MV procedure, the short-term incidence of progressive MR is low; however, more follow-up is needed to determine whether patients with MVP and MR grade ≤2 would benefit from prophylactic MV intervention.

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KW - Mitral valve

KW - Thoracic aorta

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