Malignant bileaflet mitral valve prolapse syndrome in patients with otherwise idiopathic out-of-hospital cardiac arrest

Chenni S. Sriram, Faisal F. Syed, M. Eric Ferguson, Jonathan N. Johnson, Maurice E Sarano, Frank Cetta, Bryan C. Cannon, Samuel J Asirvatham, Michael John Ackerman

Research output: Contribution to journalArticle

84 Citations (Scopus)

Abstract

Objectives The aim of this study was to investigate the prevalence of mitral valve prolapse (MVP) and its association with ventricular arrhythmias in a cohort with "unexplained" out-of-hospital cardiac arrest. Background Ventricular arrhythmias are an important cause of sudden unexpected death in the young. The role of MVP in sudden unexpected death remains controversial. Methods Of 1,200 patients evaluated between July 2000 and December 2009 in the Mayo Clinic's Long QT Syndrome/Genetic Heart Rhythm Clinic, all 24 (16 women, median age 33.5 years) with idiopathic out-of-hospital cardiac arrest (i.e., negative for ischemia, cardiomyopathy, and channelopathy) were reviewed. Results All 24 patients had implantable cardioverter-defibrillators (ICDs). Out-of-hospital cardiac arrest was the sentinel event in 22 (92%). Bileaflet MVP was found in 10 (42%). Compared with patients with normal mitral valves, patients with bileaflet MVP: 1) were over-represented by women (9 of 10 [90%] vs. 7 of 14 [50%], p = 0.04); 2) had a higher prevalence of biphasic or inverted T waves (7 of 9 [77.8%] vs. 4 of 14 [29%], p = 0.04); and 3) on Holter interrogation had higher prevalence of ventricular bigeminy (9 of 9 [100%] vs. 1 of 10 [10%], p < 0.0001), ventricular tachycardia (7 of 9 [78%] vs. 1 of 10 [10%], p = 0.006), and premature ventricular contractions originating from the outflow tract alternating with the papillary muscle or fascicular region (7 of 9 [78%] vs. 2 of 10 [20%], p = 0.02). Over a median 1.8 years (range: 0.1 to 11.9 years) from ICD placement, 13 of 24 patients (54%) received appropriate ventricular fibrillation-terminating ICD shocks. Only bileaflet MVP was associated with ventricular fibrillation recurrences requiring ICD therapy on follow-up (logistic regression odds ratio: 7.2; 95% confidence interval: 1.1 to 48; p = 0.028). Conclusions The authors describe a "malignant" subset of patients with MVP who experienced life-threatening ventricular arrhythmias. This phenotype is characterized by bileaflet MVP, female sex, and frequent complex ventricular ectopic activity, including premature ventricular contractions of the outflow tract alternating with papillary muscle or fascicular origin.

Original languageEnglish (US)
Pages (from-to)222-230
Number of pages9
JournalJournal of the American College of Cardiology
Volume62
Issue number3
DOIs
StatePublished - Jul 16 2013

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Out-of-Hospital Cardiac Arrest
Mitral Valve Prolapse
Implantable Defibrillators
Cardiac Arrhythmias
Ventricular Premature Complexes
Papillary Muscles
Ventricular Fibrillation
Sudden Death
Channelopathies
Long QT Syndrome
Ventricular Tachycardia
Cardiomyopathies
Mitral Valve
Shock
Ischemia
Logistic Models
Odds Ratio
Confidence Intervals
Phenotype
Recurrence

Keywords

  • mitral valve prolapse
  • out-of-hospital cardiac arrest
  • premature ventricular contraction
  • sudden unexpected death
  • ventricular ectopic activity

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine

Cite this

Malignant bileaflet mitral valve prolapse syndrome in patients with otherwise idiopathic out-of-hospital cardiac arrest. / Sriram, Chenni S.; Syed, Faisal F.; Ferguson, M. Eric; Johnson, Jonathan N.; Sarano, Maurice E; Cetta, Frank; Cannon, Bryan C.; Asirvatham, Samuel J; Ackerman, Michael John.

In: Journal of the American College of Cardiology, Vol. 62, No. 3, 16.07.2013, p. 222-230.

Research output: Contribution to journalArticle

Sriram, Chenni S. ; Syed, Faisal F. ; Ferguson, M. Eric ; Johnson, Jonathan N. ; Sarano, Maurice E ; Cetta, Frank ; Cannon, Bryan C. ; Asirvatham, Samuel J ; Ackerman, Michael John. / Malignant bileaflet mitral valve prolapse syndrome in patients with otherwise idiopathic out-of-hospital cardiac arrest. In: Journal of the American College of Cardiology. 2013 ; Vol. 62, No. 3. pp. 222-230.
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title = "Malignant bileaflet mitral valve prolapse syndrome in patients with otherwise idiopathic out-of-hospital cardiac arrest",
abstract = "Objectives The aim of this study was to investigate the prevalence of mitral valve prolapse (MVP) and its association with ventricular arrhythmias in a cohort with {"}unexplained{"} out-of-hospital cardiac arrest. Background Ventricular arrhythmias are an important cause of sudden unexpected death in the young. The role of MVP in sudden unexpected death remains controversial. Methods Of 1,200 patients evaluated between July 2000 and December 2009 in the Mayo Clinic's Long QT Syndrome/Genetic Heart Rhythm Clinic, all 24 (16 women, median age 33.5 years) with idiopathic out-of-hospital cardiac arrest (i.e., negative for ischemia, cardiomyopathy, and channelopathy) were reviewed. Results All 24 patients had implantable cardioverter-defibrillators (ICDs). Out-of-hospital cardiac arrest was the sentinel event in 22 (92{\%}). Bileaflet MVP was found in 10 (42{\%}). Compared with patients with normal mitral valves, patients with bileaflet MVP: 1) were over-represented by women (9 of 10 [90{\%}] vs. 7 of 14 [50{\%}], p = 0.04); 2) had a higher prevalence of biphasic or inverted T waves (7 of 9 [77.8{\%}] vs. 4 of 14 [29{\%}], p = 0.04); and 3) on Holter interrogation had higher prevalence of ventricular bigeminy (9 of 9 [100{\%}] vs. 1 of 10 [10{\%}], p < 0.0001), ventricular tachycardia (7 of 9 [78{\%}] vs. 1 of 10 [10{\%}], p = 0.006), and premature ventricular contractions originating from the outflow tract alternating with the papillary muscle or fascicular region (7 of 9 [78{\%}] vs. 2 of 10 [20{\%}], p = 0.02). Over a median 1.8 years (range: 0.1 to 11.9 years) from ICD placement, 13 of 24 patients (54{\%}) received appropriate ventricular fibrillation-terminating ICD shocks. Only bileaflet MVP was associated with ventricular fibrillation recurrences requiring ICD therapy on follow-up (logistic regression odds ratio: 7.2; 95{\%} confidence interval: 1.1 to 48; p = 0.028). Conclusions The authors describe a {"}malignant{"} subset of patients with MVP who experienced life-threatening ventricular arrhythmias. This phenotype is characterized by bileaflet MVP, female sex, and frequent complex ventricular ectopic activity, including premature ventricular contractions of the outflow tract alternating with papillary muscle or fascicular origin.",
keywords = "mitral valve prolapse, out-of-hospital cardiac arrest, premature ventricular contraction, sudden unexpected death, ventricular ectopic activity",
author = "Sriram, {Chenni S.} and Syed, {Faisal F.} and Ferguson, {M. Eric} and Johnson, {Jonathan N.} and Sarano, {Maurice E} and Frank Cetta and Cannon, {Bryan C.} and Asirvatham, {Samuel J} and Ackerman, {Michael John}",
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T1 - Malignant bileaflet mitral valve prolapse syndrome in patients with otherwise idiopathic out-of-hospital cardiac arrest

AU - Sriram, Chenni S.

AU - Syed, Faisal F.

AU - Ferguson, M. Eric

AU - Johnson, Jonathan N.

AU - Sarano, Maurice E

AU - Cetta, Frank

AU - Cannon, Bryan C.

AU - Asirvatham, Samuel J

AU - Ackerman, Michael John

PY - 2013/7/16

Y1 - 2013/7/16

N2 - Objectives The aim of this study was to investigate the prevalence of mitral valve prolapse (MVP) and its association with ventricular arrhythmias in a cohort with "unexplained" out-of-hospital cardiac arrest. Background Ventricular arrhythmias are an important cause of sudden unexpected death in the young. The role of MVP in sudden unexpected death remains controversial. Methods Of 1,200 patients evaluated between July 2000 and December 2009 in the Mayo Clinic's Long QT Syndrome/Genetic Heart Rhythm Clinic, all 24 (16 women, median age 33.5 years) with idiopathic out-of-hospital cardiac arrest (i.e., negative for ischemia, cardiomyopathy, and channelopathy) were reviewed. Results All 24 patients had implantable cardioverter-defibrillators (ICDs). Out-of-hospital cardiac arrest was the sentinel event in 22 (92%). Bileaflet MVP was found in 10 (42%). Compared with patients with normal mitral valves, patients with bileaflet MVP: 1) were over-represented by women (9 of 10 [90%] vs. 7 of 14 [50%], p = 0.04); 2) had a higher prevalence of biphasic or inverted T waves (7 of 9 [77.8%] vs. 4 of 14 [29%], p = 0.04); and 3) on Holter interrogation had higher prevalence of ventricular bigeminy (9 of 9 [100%] vs. 1 of 10 [10%], p < 0.0001), ventricular tachycardia (7 of 9 [78%] vs. 1 of 10 [10%], p = 0.006), and premature ventricular contractions originating from the outflow tract alternating with the papillary muscle or fascicular region (7 of 9 [78%] vs. 2 of 10 [20%], p = 0.02). Over a median 1.8 years (range: 0.1 to 11.9 years) from ICD placement, 13 of 24 patients (54%) received appropriate ventricular fibrillation-terminating ICD shocks. Only bileaflet MVP was associated with ventricular fibrillation recurrences requiring ICD therapy on follow-up (logistic regression odds ratio: 7.2; 95% confidence interval: 1.1 to 48; p = 0.028). Conclusions The authors describe a "malignant" subset of patients with MVP who experienced life-threatening ventricular arrhythmias. This phenotype is characterized by bileaflet MVP, female sex, and frequent complex ventricular ectopic activity, including premature ventricular contractions of the outflow tract alternating with papillary muscle or fascicular origin.

AB - Objectives The aim of this study was to investigate the prevalence of mitral valve prolapse (MVP) and its association with ventricular arrhythmias in a cohort with "unexplained" out-of-hospital cardiac arrest. Background Ventricular arrhythmias are an important cause of sudden unexpected death in the young. The role of MVP in sudden unexpected death remains controversial. Methods Of 1,200 patients evaluated between July 2000 and December 2009 in the Mayo Clinic's Long QT Syndrome/Genetic Heart Rhythm Clinic, all 24 (16 women, median age 33.5 years) with idiopathic out-of-hospital cardiac arrest (i.e., negative for ischemia, cardiomyopathy, and channelopathy) were reviewed. Results All 24 patients had implantable cardioverter-defibrillators (ICDs). Out-of-hospital cardiac arrest was the sentinel event in 22 (92%). Bileaflet MVP was found in 10 (42%). Compared with patients with normal mitral valves, patients with bileaflet MVP: 1) were over-represented by women (9 of 10 [90%] vs. 7 of 14 [50%], p = 0.04); 2) had a higher prevalence of biphasic or inverted T waves (7 of 9 [77.8%] vs. 4 of 14 [29%], p = 0.04); and 3) on Holter interrogation had higher prevalence of ventricular bigeminy (9 of 9 [100%] vs. 1 of 10 [10%], p < 0.0001), ventricular tachycardia (7 of 9 [78%] vs. 1 of 10 [10%], p = 0.006), and premature ventricular contractions originating from the outflow tract alternating with the papillary muscle or fascicular region (7 of 9 [78%] vs. 2 of 10 [20%], p = 0.02). Over a median 1.8 years (range: 0.1 to 11.9 years) from ICD placement, 13 of 24 patients (54%) received appropriate ventricular fibrillation-terminating ICD shocks. Only bileaflet MVP was associated with ventricular fibrillation recurrences requiring ICD therapy on follow-up (logistic regression odds ratio: 7.2; 95% confidence interval: 1.1 to 48; p = 0.028). Conclusions The authors describe a "malignant" subset of patients with MVP who experienced life-threatening ventricular arrhythmias. This phenotype is characterized by bileaflet MVP, female sex, and frequent complex ventricular ectopic activity, including premature ventricular contractions of the outflow tract alternating with papillary muscle or fascicular origin.

KW - mitral valve prolapse

KW - out-of-hospital cardiac arrest

KW - premature ventricular contraction

KW - sudden unexpected death

KW - ventricular ectopic activity

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