Abstract | December 20, 2022

Mitral Valve Prolapse presenting as Sudden Cardiac Death

Presenting Author: Riaz Mahmood, DO, MS, Chief Resident Internal Medicine PGY-4, Department of Internal Medicine, Graduate Medical Education, Northeast Georgia Medical Center, Gainesville, Georgia

Coauthors: Joon Ahn, MD, Electrophysiologist, Georgia Heart Institute, Gainesville, GA

Learning Objectives

  1. Discuss the mechanism of sudden cardiac death from mitral valve prolapse
  2. Identify lead oversensing as a complication of ICD placement

Introduction: Sudden cardiac death (SCD) can have devastating consequences, especially in younger patients. The presence of mitral valve prolapse (MVP) may increase the risk of sudden death due to ventricular arrhythmias. S-ICD is an ideal choice for younger patients who require ICD therapy. The pitfalls of S-ICD are related to over-sensing, which may lead to inappropriate shocks.

 

Case Presentation: A 25-year-old female presented with cardiac arrest due to ventricular fibrillation. ROSC was achieved after CPR and three shocks within 15 minutes. ECG post-arrest showed atrial fibrillation with a prolonged QT and no acute ischemic change. She was admitted to the ICU in shock, requiring high-dose vasopressor support, and started on TTM protocol. The initial transthoracic echocardiogram revealed an EF < 20%. Heart catheterization showed cardiogenic shock with normal coronary anatomy and no coronary artery disease. An Impella device was inserted, and inotropic therapy was initiated. Trans-esophageal echocardiogram showed prolapse of the anterior and posterior mitral valve leaflets with mild to moderate mitral regurgitation. Cardiac MRI showed a normalized EF of 54% and an area of patchy delayed hyper-enhancement of the mid inferolateral wall. Mitral annular disjunction was present. Her rhythm spontaneously converted to sinus rhythm and her QT normalized. Her cardiac arrest was attributed to arrhythmic mitral valve prolapse. She underwent an implant of a subcutaneous ICD (S-ICD) for secondary prevention of sudden death. The patient was discharged home.

 

Three months later, she presented with an inappropriate ICD shock due to noise artifact while washing her hair. She underwent an explant of her lead and re-implant of a new lead to a deeper subcutaneous location. That evening she experienced another inappropriate shock due to over-sensing of noise. She had severely diminished sensing in all vectors. Subcutaneous air around the electrode was the suspected cause. 

 

Final Diagnosis: Sudden Cardiac Death due to ventricular arrhythmia from mitral valve prolapse

 

Management: She underwent an explant of her S-ICD with an implant of a transvenous single-chamber ICD. A follow-up five months later showed normal device function with no ventricular arrhythmias. Analysis of the returned electrodes and generator showed no abnormalities.

 

References:

  1. Epstein AE, et al. ACC/AHA/HRS 2008 Guidelines for Device-Based Therapy of Cardiac Rhythm Abnormalities: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Revise the ACC/AHA/NASPE 2002 Guideline Update for Implantation of Cardiac Pacemakers and Antiarrhythmia Devices): developed in collaboration with the American Association for Thoracic Surgery and Society of Thoracic Surgeons. Circulation. 2008 May 27;117(21):e350-408. doi: 10.1161/CIRCUALTIONAHA.108.189742. Epub 2008 May 15. Erratum in: Circulation.2009 Aug 4; 120(5):e34-5. PMID: 18483207.
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