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Sudden perioperative death post aortic valve replacement with autopsy showing hypertrophic cardiomyopathy in elderly
Zubair Abdul Razak1, Joseph Westaby2, Mary N Sheppard3
1Department of Forensic Medicine, Hospital Sultan Ismail, Johor Bahru, Malaysia.
Insights
A sudden perioperative death occurred after aortic valve replacement in a patient with previously undiagnosed hypertrophic cardiomyopathy (HCM). Autopsy confirmed HCM, emphasizing the need for family screening to prevent future sudden cardiac deaths.
Area of Science:
- Cardiology
- Cardiac Surgery
- Pathology
Background:
- Perioperative death is a significant concern for patients and surgical teams.
- Autopsies are crucial for understanding unexpected perioperative fatalities.
- This case involves a sudden death following aortic valve replacement in an elderly female with undiagnosed hypertrophic cardiomyopathy (HCM).
Purpose of the Study:
- To report a rare case of sudden perioperative death due to undiagnosed hypertrophic cardiomyopathy (HCM) after aortic valve replacement (AVR).
- To highlight the importance of autopsy in explaining unexpected post-operative deaths.
- To underscore the need for genetic screening in families affected by HCM.
Main Methods:
- A 73-year-old female underwent elective aortic valve replacement (AVR) for severe aortic valve stenosis (AS).
- Despite successful surgery, the patient experienced persistent poor left ventricular function and died intraoperatively.
- Post-mortem examination, including heart weight, left ventricular septal hypertrophy measurement, and left ventricular histology, was performed.
Main Results:
- The patient died on the operating table due to unrecoverable poor left ventricular function post-AVR.
- Histological examination revealed significant septal hypertrophy (20 mm) and myocyte disarray, confirming hypertrophic cardiomyopathy (HCM).
- Aortic valve stenosis (AS) is a common indication for AVR, but sudden death can occur post-procedure.
Conclusions:
- Undiagnosed hypertrophic cardiomyopathy (HCM) can lead to sudden unexpected death even after successful aortic valve replacement (AVR).
- Autopsy plays a vital role in diagnosing conditions like HCM and providing closure for families.
- Screening of family members is essential for inherited conditions like HCM to prevent further sudden cardiac deaths.
Introduction:
Perioperative death is a catastrophic event for the family and the surgical team. It is in the interest of both that an autopsy is carried out to explain the death. We report a sudden perioperative death post aortic valve replacement in an undiagnosed hypertrophic cardiomyopathy (HCM) in an elderly female.
Case Report:
A 73-year-old female underwent an elective aortic valve replacement (AVR) due to severe aortic valve stenosis (AS). The operation went smoothly. However, left ventricular function was poor despite maximal inotropic treatment. The left ventricular function never recovered and she died on the operating table. The heart weight was normal but the left ventricle shows septal hypertrophy (20 mm). Histology of the left ventricle showed florid myocyte disarray indicating hypertrophic cardiomyopathy.
Discussion:
AS is the most common Valvular Heart Disease, and most patients undergo AVR. Nevertheless, sudden unexpected death remains a common cause of late mortality after successful valve replacement. Surprisingly this lady died just after the operative procedure. Histological examination confirmed HCM. HCM is an inherited cardiac condition and it is important for the family to be screened to prevent future sudden cardiac death.
Conclusion:
This case highlights the importance of autopsy in a post-operative death case. It can be of great value to the surgical team and family members.
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