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Investigating the Pathogenesis of MYH7 Mutation Gly823Glu in Familial Hypertrophic Cardiomyopathy using a Mouse Model
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Hypertrophic cardiomyopathy with dynamic obstruction and high left ventricular outflow gradients associated with

Mark V Sherrid1, Katherine Riedy1, Barry Rosenzweig1

  • 1Hypertrophic Cardiomyopathy Program, Division of Cardiology, New York University Langone Health, New York University School of Medicine, New York City, New York.

Echocardiography (Mount Kisco, N.Y.)
|December 15, 2018
PubMed

Insights

Acute left ventricular ballooning in obstructive hypertrophic cardiomyopathy is rare but can cause severe instability. Early recognition and treatment, including surgery in some cases, can lead to full recovery.

Area of Science:

  • Cardiology
  • Cardiovascular Medicine
  • Medical Diagnostics

Background:

  • Acute left ventricular (LV) apical ballooning with normal coronary angiography is a rare presentation in obstructive hypertrophic cardiomyopathy (OHCM).
  • This condition can be associated with severe hemodynamic instability and profound LV decompensation.

Purpose of the Study:

  • To investigate the occurrence and characteristics of acute LV apical ballooning in patients with OHCM.
  • To understand the clinical course, management, and outcomes of this specific complication.

Main Methods:

  • A retrospective search was conducted in two HCM treatment program databases for cases of acute LV ballooning with apical hypokinesia/akinesia.
  • Diagnosis of OHCM was based on conventional criteria, and patient data including LV hypertrophy, outflow tract (LVOT) gradients, and echocardiographic findings were analyzed.

Main Results:

  • Of 1519 patients, 13 (0.9%) presented with acute LV ballooning, characterized by high LVOT gradients (92 ± 37 mm Hg).
  • The majority were female (77%), with mild septal hypertrophy and specific anatomical features like elongated anterior mitral leaflets (77%).
  • Complications included cardiogenic shock and heart failure in 5 patients; 3 required urgent surgery for LVOT obstruction, resulting in immediate improvement and sustained normal LV function post-operatively.

Conclusions:

  • Acute LV apical ballooning associated with high dynamic LVOT gradients can occur in obstructive HCM.
  • This syndrome requires prompt recognition on echocardiography due to its association with severe, yet potentially reversible, LV decompensation.
Abstract

Keywords:
hypertrophic cardiomyopathyleft ventricular outflow obstructionleft ventricular systolic dysfunction

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