Modified Bentall procedure with mitral valve replacement in a patient with Marfan syndrome: a case report

Sanjay K. Raha; Sumit Barua; M. Kamrul Hasan; M. Shafiqul Islam; M. Jobaid Hasan; N. Faisal N. Majumder; Mahmodul H. Al-Faraby; Kaushik Roy; M. Sorower Hossain; Smriti K. Biswas; Hosne Jahan

doi:10.18203/2320-6012.ijrms20241913

Authors

Sanjay K. Raha Department of Cardiac Surgery, National Institute of Cardiovascular Diseases (NICVD), Dhaka, Bangladesh
Sumit Barua Department of Cardiac Surgery, Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka, Bangladesh
M. Kamrul Hasan Department of Cardiac Surgery, National Institute of Cardiovascular Diseases (NICVD), Dhaka, Bangladesh
M. Shafiqul Islam Department of Cardiac Surgery, National Institute of Cardiovascular Diseases (NICVD), Dhaka, Bangladesh
M. Jobaid Hasan Department of Cardiac Surgery, National Institute of Cardiovascular Diseases (NICVD), Dhaka, Bangladesh
N. Faisal N. Majumder Department of Cardiac Surgery, National Institute of Cardiovascular Diseases (NICVD), Dhaka, Bangladesh
Mahmodul H. Al-Faraby Department of Cardiac Surgery, National Institute of Cardiovascular Diseases (NICVD), Dhaka, Bangladesh
Kaushik Roy Department of Cardiac Surgery, National Institute of Cardiovascular Diseases (NICVD), Dhaka, Bangladesh
M. Sorower Hossain Department of Cardiac Surgery, National Institute of Cardiovascular Diseases (NICVD), Dhaka, Bangladesh
Smriti K. Biswas Department of Gynaecology and Obstetrics, Shaheed Suhrawardy Medical College Hospital, Dhaka, Bangladesh
Hosne Jahan Department of Microbiology, National Institute of Cardiovascular Diseases (NICVD), Dhaka, Bangladesh

DOI:

https://doi.org/10.18203/2320-6012.ijrms20241913

Keywords:

Marfan syndrome, Bentall procedure, Mitral valve replacement, Aortic root aneurysm

Abstract

Marfan syndrome (MFS) is an autosomal dominant connective tissue disorder characterized by fibrillin-1 protein abnormalities, predisposing individuals to cardiovascular complications such as aortic root dilation and mitral valve prolapse (MVP). Management often requires surgical intervention, with the modified Bentall procedure being a standard approach for aortic root replacement. However, managing concomitant mitral valve pathology poses additional challenges due to the complex anatomical relationship between the two valves and the prolonged nature of the procedure. We present a case report of a 35-year-old male with Marfan syndrome who underwent a successful simultaneous modified Bentall procedure and mitral valve replacement for concurrent aortic root aneurysm and mitral valve prolapse. Surgical interventions involved meticulous attention to detail to mitigate risks associated with malpositioned coronary ostial sutures, debridement of the severely calcified mitral valve, and achieving hemostasis. Following surgery, the patient exhibited favorable postoperative outcomes, with evidence of optimal valve function and improved systolic and diastolic parameters, indicative of a successful recovery.

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