Solitary accessory and papillary muscle hypertrophy manifested as dynamic mid-wall obstruction and symptomatic heart failure: diagnostic feasibility by multi-modality imaging

BMC Cardiovasc Disord. 2014 Mar 10:14:34. doi: 10.1186/1471-2261-14-34.

Abstract

Background: Solitary papillary muscle (PM) hypertrophy is an unique type of hypertrophic cardiomyopathy (HCM), which is characterized by predominant papillary muscle hypertrophy sparing the rest of other left ventricular segments. It has recently drawn our attention about the mechanism of left ventricular mid-cavity obstruction and the influence of pressure gradient in the left ventricular outflow tract (LVOT), thus carries clinical importance.

Case presentation: We reported a symptomatic, 83-year-old woman who presented with dynamic, high resting left ventricle (LV) mid-wall gradient without obvious septal hypertrophy or systolic anterior motion (SAM). Subsequent real-time (RT) three-dimensional echocardiography (3DE) and cardiac magnetic resonance imaging (MRI) demonstrated large, hypertrophic accessory papillary muscles squeezing mid-cavity of left ventricle producing dynamic pressure gradient during systole in the absence of left ventricular wall anomalies.

Conclusion: We proposed that combined use of echocardiography particularly RT-3DE and cardiac magnetic resonance imaging (MRI) can accurately identify this specific type of hypertrophic cardiomyopathy without remarkable traditional features.

Publication types

  • Case Reports
  • Video-Audio Media

MeSH terms

  • Aged, 80 and over
  • Cardiomyopathy, Hypertrophic / complications
  • Cardiomyopathy, Hypertrophic / diagnosis*
  • Cardiomyopathy, Hypertrophic / physiopathology
  • Echocardiography, Doppler, Color
  • Echocardiography, Three-Dimensional
  • Electrocardiography
  • Female
  • Heart Failure / diagnosis*
  • Heart Failure / etiology
  • Heart Failure / physiopathology
  • Humans
  • Magnetic Resonance Imaging
  • Multimodal Imaging* / methods
  • Papillary Muscles* / diagnostic imaging
  • Papillary Muscles* / pathology
  • Papillary Muscles* / physiopathology
  • Predictive Value of Tests
  • Ventricular Function, Left
  • Ventricular Pressure