Syndrome of Reversible Cardiogenic Shock and Left Ventricular Ballooning in Obstructive Hypertrophic Cardiomyopathy
Background Cardiogenic shock from most causes has unfavorable prognosis. Hypertrophic cardiomyopathy (HCM) can uncommonly present with apical ballooning and shock in association with sudden development of severe and unrelenting left ventricular (LV) outflow obstruction. Typical HCM phenotypic featur...
| Main Authors: | , , , , , , , , , , , , , |
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| Format: | Article |
| Language: | English |
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Wiley
2021-10-01
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| Series: | Journal of the American Heart Association: Cardiovascular and Cerebrovascular Disease |
| Subjects: | |
| Online Access: | https://www.ahajournals.org/doi/10.1161/JAHA.121.021141 |
| _version_ | 1797607133026451456 |
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| author | Mark V. Sherrid Daniel G. Swistel Iacopo Olivotto Maurizio Pieroni Omar Wever‐Pinzon Katherine Riedy Richard G. Bach Mustafa Husaini Sharon Cresci Alex Reyentovich Daniele Massera Martin S. Maron Barry J. Maron Bette Kim |
| author_facet | Mark V. Sherrid Daniel G. Swistel Iacopo Olivotto Maurizio Pieroni Omar Wever‐Pinzon Katherine Riedy Richard G. Bach Mustafa Husaini Sharon Cresci Alex Reyentovich Daniele Massera Martin S. Maron Barry J. Maron Bette Kim |
| author_sort | Mark V. Sherrid |
| collection | DOAJ |
| description | Background Cardiogenic shock from most causes has unfavorable prognosis. Hypertrophic cardiomyopathy (HCM) can uncommonly present with apical ballooning and shock in association with sudden development of severe and unrelenting left ventricular (LV) outflow obstruction. Typical HCM phenotypic features of mild septal thickening, outflow gradients, and distinctive mitral abnormalities differentiate these patients from others with Takotsubo syndrome, who have normal mitral valves and no outflow obstruction. Methods and Results We analyzed 8 patients from our 4 HCM centers with obstructive HCM and abrupt presentation of cardiogenic shock with LV ballooning, and 6 cases reported in literature. Of 14 patients, 10 (71%) were women, aged 66±9 years, presenting with acute symptoms: LV ballooning; depressed ejection fraction (25±5%); refractory systemic hypotension; marked LV outflow tract obstruction (peak gradient, 94±28 mm Hg); and elevated troponin, but absence of atherosclerotic coronary disease. Shock was managed with intravenous administration of phenylephrine (n=6), norepinephrine (n=6), β‐blocker (n=7), and vasopressin (n=1). Mechanical circulatory support was required in 8, including intra‐aortic balloon pump (n=4), venoarterial extracorporeal membrane oxygenation (n=3), and Impella and Tandem Heart in 1 each. In refractory shock, urgent relief of obstruction by myectomy was performed in 5, and alcohol ablation in 1. All patients survived their critical illness, with full recovery of systolic function. Conclusions When cardiogenic shock and LV ballooning occur in obstructive HCM, they are marked by distinctive anatomic and physiologic features. Relief of obstruction with targeted pharmacotherapy, mechanical circulatory support, and myectomy, when necessary for refractory shock, may lead to survival and normalization of systolic function. |
| first_indexed | 2024-03-11T05:25:57Z |
| format | Article |
| id | doaj.art-8b946d68a414448790109dc012bea5ad |
| institution | Directory Open Access Journal |
| issn | 2047-9980 |
| language | English |
| last_indexed | 2024-03-11T05:25:57Z |
| publishDate | 2021-10-01 |
| publisher | Wiley |
| record_format | Article |
| series | Journal of the American Heart Association: Cardiovascular and Cerebrovascular Disease |
| spelling | doaj.art-8b946d68a414448790109dc012bea5ad2023-11-17T17:30:17ZengWileyJournal of the American Heart Association: Cardiovascular and Cerebrovascular Disease2047-99802021-10-01102010.1161/JAHA.121.021141Syndrome of Reversible Cardiogenic Shock and Left Ventricular Ballooning in Obstructive Hypertrophic CardiomyopathyMark V. Sherrid0Daniel G. Swistel1Iacopo Olivotto2Maurizio Pieroni3Omar Wever‐Pinzon4Katherine Riedy5Richard G. Bach6Mustafa Husaini7Sharon Cresci8Alex Reyentovich9Daniele Massera10Martin S. Maron11Barry J. Maron12Bette Kim13Hypertrophic Cardiomyopathy Program NYU Langone Health New York NYDepartment of Cardiothoracic Surgery NYU Langone Health New York NYCardiomyopathy Unit Division of Cardiology Careggi University Hospital Florence ItalyCardiovascular Department San Donato Hospital Arezzo ItalyUniversity of Utah Health Salt Lake City UTHypertrophic Cardiomyopathy Program NYU Langone Health New York NYWashington University Medical Center St. Louis MOWashington University Medical Center St. Louis MOWashington University Medical Center St. Louis MOLeon Charney Division of Cardiology Heart Failure Advanced Care Center NYU Langone Health New York NYHypertrophic Cardiomyopathy Program NYU Langone Health New York NYTufts Medical Center Boston MATufts Medical Center Boston MAMount Sinai West New York NYBackground Cardiogenic shock from most causes has unfavorable prognosis. Hypertrophic cardiomyopathy (HCM) can uncommonly present with apical ballooning and shock in association with sudden development of severe and unrelenting left ventricular (LV) outflow obstruction. Typical HCM phenotypic features of mild septal thickening, outflow gradients, and distinctive mitral abnormalities differentiate these patients from others with Takotsubo syndrome, who have normal mitral valves and no outflow obstruction. Methods and Results We analyzed 8 patients from our 4 HCM centers with obstructive HCM and abrupt presentation of cardiogenic shock with LV ballooning, and 6 cases reported in literature. Of 14 patients, 10 (71%) were women, aged 66±9 years, presenting with acute symptoms: LV ballooning; depressed ejection fraction (25±5%); refractory systemic hypotension; marked LV outflow tract obstruction (peak gradient, 94±28 mm Hg); and elevated troponin, but absence of atherosclerotic coronary disease. Shock was managed with intravenous administration of phenylephrine (n=6), norepinephrine (n=6), β‐blocker (n=7), and vasopressin (n=1). Mechanical circulatory support was required in 8, including intra‐aortic balloon pump (n=4), venoarterial extracorporeal membrane oxygenation (n=3), and Impella and Tandem Heart in 1 each. In refractory shock, urgent relief of obstruction by myectomy was performed in 5, and alcohol ablation in 1. All patients survived their critical illness, with full recovery of systolic function. Conclusions When cardiogenic shock and LV ballooning occur in obstructive HCM, they are marked by distinctive anatomic and physiologic features. Relief of obstruction with targeted pharmacotherapy, mechanical circulatory support, and myectomy, when necessary for refractory shock, may lead to survival and normalization of systolic function.https://www.ahajournals.org/doi/10.1161/JAHA.121.021141cardiogenic shockhypertrophic cardiomyopathyhypertrophic obstructive cardiomyopathyleft ventricular ballooningleft ventricular outflow tract obstructionsupply‐demand ischemia |
| spellingShingle | Mark V. Sherrid Daniel G. Swistel Iacopo Olivotto Maurizio Pieroni Omar Wever‐Pinzon Katherine Riedy Richard G. Bach Mustafa Husaini Sharon Cresci Alex Reyentovich Daniele Massera Martin S. Maron Barry J. Maron Bette Kim Syndrome of Reversible Cardiogenic Shock and Left Ventricular Ballooning in Obstructive Hypertrophic Cardiomyopathy Journal of the American Heart Association: Cardiovascular and Cerebrovascular Disease cardiogenic shock hypertrophic cardiomyopathy hypertrophic obstructive cardiomyopathy left ventricular ballooning left ventricular outflow tract obstruction supply‐demand ischemia |
| title | Syndrome of Reversible Cardiogenic Shock and Left Ventricular Ballooning in Obstructive Hypertrophic Cardiomyopathy |
| title_full | Syndrome of Reversible Cardiogenic Shock and Left Ventricular Ballooning in Obstructive Hypertrophic Cardiomyopathy |
| title_fullStr | Syndrome of Reversible Cardiogenic Shock and Left Ventricular Ballooning in Obstructive Hypertrophic Cardiomyopathy |
| title_full_unstemmed | Syndrome of Reversible Cardiogenic Shock and Left Ventricular Ballooning in Obstructive Hypertrophic Cardiomyopathy |
| title_short | Syndrome of Reversible Cardiogenic Shock and Left Ventricular Ballooning in Obstructive Hypertrophic Cardiomyopathy |
| title_sort | syndrome of reversible cardiogenic shock and left ventricular ballooning in obstructive hypertrophic cardiomyopathy |
| topic | cardiogenic shock hypertrophic cardiomyopathy hypertrophic obstructive cardiomyopathy left ventricular ballooning left ventricular outflow tract obstruction supply‐demand ischemia |
| url | https://www.ahajournals.org/doi/10.1161/JAHA.121.021141 |
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