Volume 36, Issue 1 pp. 47-60
ORIGINAL INVESTIGATION

Hypertrophic cardiomyopathy with dynamic obstruction and high left ventricular outflow gradients associated with paradoxical apical ballooning

Mark V. Sherrid MD

Corresponding Author

Mark V. Sherrid MD

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

Correspondence

Mark V. Sherrid, Hypertrophic Cardiomyopathy Program, Division of Cardiology of New York, New York University School of Medicine, New York City, NY.

Email: [email protected]

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Katherine Riedy MD

Katherine Riedy MD

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

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Barry Rosenzweig MD

Barry Rosenzweig MD

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

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Monica Ahluwalia MD

Monica Ahluwalia MD

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

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Milla Arabadjian NP

Milla Arabadjian NP

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

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Muhamed Saric MD, PhD

Muhamed Saric MD, PhD

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

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Sandhya Balaram MD

Sandhya Balaram MD

Mount Sinai St. Luke’s, Icahn School of Medicine at Mount Sinai, New York City, New York

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Daniel G. Swistel MD

Daniel G. Swistel MD

Hypertrophic Cardiomyopathy Program, Division of Cardiac Surgery, New York University Langone Health, New York University School of Medicine, New York City, New York

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Harmony R. Reynolds MD

Harmony R. Reynolds MD

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

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Bette Kim MD

Bette Kim MD

Mount Sinai West, Icahn School of Medicine at Mount Sinai, New York City, New York

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First published: 12 December 2018
Citations: 17

Abstract

Background

Acute left ventricular (LV) apical ballooning with normal coronary angiography occurs rarely in obstructive hypertrophic cardiomyopathy (OHCM); it may be associated with severe hemodynamic instability.

Methods, Results

We searched for acute LV ballooning with apical hypokinesia/akinesia in databases of two HCM treatment programs. Diagnosis of OHCM was made by conventional criteria of LV hypertrophy in the absence of a clinical cause for hypertrophy and mitral-septal contact. Among 1519 patients, we observed acute LV ballooning in 13 (0.9%), associated with dynamic left ventricular outflow tract (LVOT) obstruction and high gradients, 92 ± 37 mm Hg, 10 female (77%), age 64 ± 7 years, LVEF 31.6 ± 10%. Septal hypertrophy was mild compared to that of the rest of our HCM cohort, 15 vs 20 mm (P < 0.00001). An elongated anterior mitral leaflet or anteriorly displaced papillary muscles occurred in 77%. Course was complicated by cardiogenic shock and heart failure in 5, and refractory heart failure in 1. High-dose beta-blockade was the mainstay of therapy. Three patients required urgent surgical relief of LVOT obstruction, 2 for refractory cardiogenic shock, and one for refractory heart failure. In the three patients, surgery immediately normalized refractory severe LV dysfunction, and immediately reversed cardiogenic shock and heart failure. All have normal LV systolic function at 45-month follow-up, and all have survived.

Conclusions

Acute LV apical ballooning, associated with high dynamic LVOT gradients, may punctuate the course of obstructive HCM. The syndrome is important to recognize on echocardiography because it may be associated with profound reversible LV decompensation.

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