Hypertrophic cardiomyopathy with dynamic obstruction and high left ventricular outflow gradients associated with paradoxical apical ballooning
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]
Search for more papers by this authorKatherine Riedy MD
Hypertrophic Cardiomyopathy Program, Division of Cardiology, New York University Langone Health, New York University School of Medicine, New York City, New York
Search for more papers by this authorBarry Rosenzweig MD
Hypertrophic Cardiomyopathy Program, Division of Cardiology, New York University Langone Health, New York University School of Medicine, New York City, New York
Search for more papers by this authorMonica Ahluwalia MD
Hypertrophic Cardiomyopathy Program, Division of Cardiology, New York University Langone Health, New York University School of Medicine, New York City, New York
Search for more papers by this authorMilla Arabadjian NP
Hypertrophic Cardiomyopathy Program, Division of Cardiology, New York University Langone Health, New York University School of Medicine, New York City, New York
Search for more papers by this authorMuhamed 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
Search for more papers by this authorSandhya Balaram MD
Mount Sinai St. Luke’s, Icahn School of Medicine at Mount Sinai, New York City, New York
Search for more papers by this authorDaniel 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
Search for more papers by this authorHarmony 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
Search for more papers by this authorBette Kim MD
Mount Sinai West, Icahn School of Medicine at Mount Sinai, New York City, New York
Search for more papers by this authorCorresponding 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]
Search for more papers by this authorKatherine Riedy MD
Hypertrophic Cardiomyopathy Program, Division of Cardiology, New York University Langone Health, New York University School of Medicine, New York City, New York
Search for more papers by this authorBarry Rosenzweig MD
Hypertrophic Cardiomyopathy Program, Division of Cardiology, New York University Langone Health, New York University School of Medicine, New York City, New York
Search for more papers by this authorMonica Ahluwalia MD
Hypertrophic Cardiomyopathy Program, Division of Cardiology, New York University Langone Health, New York University School of Medicine, New York City, New York
Search for more papers by this authorMilla Arabadjian NP
Hypertrophic Cardiomyopathy Program, Division of Cardiology, New York University Langone Health, New York University School of Medicine, New York City, New York
Search for more papers by this authorMuhamed 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
Search for more papers by this authorSandhya Balaram MD
Mount Sinai St. Luke’s, Icahn School of Medicine at Mount Sinai, New York City, New York
Search for more papers by this authorDaniel 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
Search for more papers by this authorHarmony 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
Search for more papers by this authorBette Kim MD
Mount Sinai West, Icahn School of Medicine at Mount Sinai, New York City, New York
Search for more papers by this authorAbstract
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.
Supporting Information
Filename | Description |
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echo14212-sup-0001-VideoS1.mp4MPEG-4 video, 1.9 MB | Movie S1. A 70 year old patient with HCM and latent LVOT obstruction presented 10 years after his initial diagnosis with near syncope, heart failure and hypotension evolving to refractory cardiogenic shock. The first 2 movies are soon after admission with the ballooning event. The 3rd movie was done postoperatively. Parasternal long-axis echocardiogram showing a mild septal bulge, SAM, and mitral-septal contact. There is ballooning of the apical and mid-LV segments with akinesia there. Systolic LVOT gradient was 90 mm Hg. Orange arrow shows the SAM. White arrowheads indicate the akinetic septum and posterior wall. |
echo14212-sup-0002-VideoS2.mp4MPEG-4 video, 2.8 MB | Movie S2. Apical 3-chamber view in the same patient. There is a very long anterior mitral valve leaflet 39 mm long, 19.5 mm/m2(elongated >16 mm/m2). There is ballooning of the apical and mid-LV segments. The anterior septal and posterior wall are akinetic while the apex was severely hypokinetic. The video demonstrates the very long anterior mitral valve leaflet (orange arrow), the akinetic septum and posterior wall (small white arrowheads), and the septal bulge (red arrow). |
echo14212-sup-0003-VideoS3.mp4MPEG-4 video, 1.6 MB | Movie S3. Postoperative long-axis echocardiogram performed 4 days after surgery to relieve LVOT obstruction with a mitral valve replacement and limited myectomy. Yellow arrow points to the bioprosthetic mitral valve. Paradoxical septal motion is present due to the LBBB from the myectomy. Nine years after surgery to relieve his severe LVOT obstruction and shock he is well, and NYHA I. He has paroxysmal atrial fibrillation. Mitral valve replacement was explicitly selected in the two cardiogenic shock patients because of the only modest septal thickening and to assure that only one pump run would be necessary. |
echo14212-sup-0004-VideoS4.mp4MPEG-4 video, 8.1 MB | Movie S4. A 73-year-old female was diagnosed with HCM and latent LVOT gradients with symptoms of dizziness and episodic dyspnea. Three and a half years later she developed severe orthostatic hypotension and heart failure symptoms with mild exercise. When acute dyspnea at rest occurred, she was found to have apical ballooning on TTE. Video loops from her TEE in the operating room are shown, performed before cannulation, before surgical septal myectomy. Yellow arrowheads in lower loops point to the apical ballooning. Red arrow points to mitral-septal contact. Note the poor mid-LV systolic function on the short axis loop. |
echo14212-sup-0005-VideoS5.mp4MPEG-4 video, 3.9 MB | Movie S5. Same patient as Movie S4. TEE video loops performed immediately after myectomy and completion of cardiopulmonary bypass showing resolution of the severe LV systolic dysfunction. |
echo14212-sup-0006-caption.docxWord document, 11.5 KB |
Please note: The publisher is not responsible for the content or functionality of any supporting information supplied by the authors. Any queries (other than missing content) should be directed to the corresponding author for the article.
REFERENCES
- 1Maron MS, Olivotto I, Zenovich AG, et al. Hypertrophic cardiomyopathy is predominantly a disease of left ventricular outflow tract obstruction. Circulation. 2006; 114: 2232–2239.
- 2Joshi S, Patel UK, Yao SS, et al. Standing and exercise Doppler echocardiography in obstructive hypertrophic cardiomyopathy: the range of gradients with upright activity. J Am Soc Echocardiogr. 2011; 24: 75–82.
- 3Cannon RO 3rd, Schenke WH, Maron BJ, et al. Differences in coronary flow and myocardial metabolism at rest and during pacing between patients with obstructive and patients with nonobstructive hypertrophic cardiomyopathy. J Am Coll Cardiol. 1987; 10: 53–62.
- 4Ormerod JO, Frenneaux MP, Sherrid MV. Myocardial energy depletion and dynamic systolic dysfunction in hypertrophic cardiomyopathy. Nat Rev Cardiol. 2016; 13: 677–687.
- 5Ashrafian H, McKenna WJ, Watkins H. Disease pathways and novel therapeutic targets in hypertrophic cardiomyopathy. Circ Res. 2011; 109: 86–96.
- 6Crilley JG, Boehm EA, Blair E, et al. Hypertrophic cardiomyopathy due to sarcomeric gene mutations is characterized by impaired energy metabolism irrespective of the degree of hypertrophy. J Am Coll Cardiol. 2003; 41: 1776–1782.
- 7Mahmod M, Francis JM, Pal N, et al. Myocardial perfusion and oxygenation are impaired during stress in severe aortic stenosis and correlate with impaired energetics and subclinical left ventricular dysfunction. J Cardiovasc Magn Reson. 2014; 16: 29.
- 8Sherrid MV, Gunsburg DZ, Pearle G. Mid-systolic drop in left ventricular ejection velocity in obstructive hypertrophic cardiomyopathy–the lobster claw abnormality. J Am Soc Echocardiogr. 1997; 10: 707–712.
- 9Conklin HM, Huang X, Davies CH, et al. Biphasic left ventricular outflow and its mechanism in hypertrophic obstructive cardiomyopathy. J Am Soc Echocardiogr. 2004; 17: 375–383.
- 10Breithardt OA, Beer G, Stolle B, et al. Mid systolic septal deceleration in hypertrophic cardiomyopathy: clinical value and insights into the pathophysiology of outflow tract obstruction by tissue Doppler echocardiography. Heart. 2005; 91: 379–380.
- 11Barac I, Upadya S, Pilchik R, et al. Effect of obstruction on longitudinal left ventricular shortening in hypertrophic cardiomyopathy. J Am Coll Cardiol. 2007; 49: 1203–1211.
- 12Sherrid MV, Balaran SK, Korzeniecki E, et al. Reversal of acute systolic dysfunction and cardiogenic shock in hypertrophic cardiomyopathy by surgical relief of obstruction. Echocardiography. 2011; 28: E174–E179.
- 13Sherrid MV, Wever-Pinzon O, Shah A, et al. Reflections of inflections in hypertrophic cardiomyopathy. J Am Coll Cardiol. 2009; 54: 212–219.
- 14Spirito P, Maron BJ, Bonow RO, et al. Occurrence and significance of progressive left ventricular wall thinning and relative cavity dilatation in hypertrophic cardiomyopathy. Am J Cardiol. 1987; 60: 123–129.
- 15Klues HG, Maron BJ, Dollar AL, et al. Diversity of structural mitral valve alterations in hypertrophic cardiomyopathy. Circulation. 1992; 85: 1651–1660.
- 16Sherrid MV, Balaram S, Kim B, et al. The mitral valve in obstructive hypertrophic cardiomyopathy: a test in context. J Am Coll Cardiol. 2016; 67: 1846–1858.
- 17Balaram SK, Ross RE, Sherrid MV, et al. Role of mitral valve plication in the surgical management of hypertrophic cardiomyopathy. Ann Thorac Surg. 2012; 94: 1997. discussion 1997–8
- 18Cavalcante JL, Barboza JS, Lever HM. Diversity of mitral valve abnormalities in obstructive hypertrophic cardiomyopathy. Prog Cardiovasc Dis. 2012; 54: 517–522.
- 19Maron MS, Olivotto I, Harrigan C, et al. Mitral valve abnormalities identified by cardiovascular magnetic resonance represent a primary phenotypic expression of hypertrophic cardiomyopathy. Circulation. 2011; 124: 40–47.
- 20Balaram SK, Tyrie L, Sherrid MV, et al. Resection-plication-release for hypertrophic cardiomyopathy: clinical and echocardiographic follow-up. Ann Thorac Surg. 2008; 86: 1539–1544. discussion 1544–5
- 21Jiang L, Levine RA, King M, et al. An integrated mechanism for systolic anterior motion of the mitral valve in hypertrophic cardiomyopathy based on echocardiographic observations. Am Heart J. 1987; 113: 633–644.
- 22Messmer BJ. Extended myectomy for hypertrophic obstructive cardiomyopathy. Ann Thorac Surg. 1994; 58: 575–577.
- 23Alhaj EK, Kim B, Cantales D, et al. Symptomatic exercise-induced left ventricular outflow tract obstruction without left ventricular hypertrophy. J Am Soc Echocardiogr. 2013; 26: 556–565.
- 24Halpern DG, Swistel DG, Po JR, et al. Echocardiography before and after resect-plicate-release surgical myectomy for obstructive hypertrophic cardiomyopathy. J Am Soc Echocardiogr. 2015; 28: 1318–1328.
- 25Ro R, Halpern D, Sahn DJ, et al. Vector flow mapping in obstructive hypertrophic cardiomyopathy to assess the relationship of early systolic left ventricular flow and the mitral valve. J Am Coll Cardiol. 2014; 64: 1984–1995.
- 26Ferrazzi P, Spirito P, Iacovoni A, et al. Transaortic chordal cutting: mitral valve repair for obstructive hypertrophic cardiomyopathy with mild septal hypertrophy. J Am Coll Cardiol. 2015; 66: 1687–1696.
- 27Feiner E, Arabadjian M, Winson G, et al. Post-prandial upright exercise echocardiography in hypertrophic cardiomyopathy. J Am Coll Cardiol. 2013; 61: 2487–2488.
- 28Maron BJ, Gottdiener JS, Arce J, et al. Dynamic subaortic obstruction in hypertrophic cardiomyopathy: analysis by pulsed Doppler echocardiography. J Am Coll Cardiol. 1985; 6: 1–18.
- 29Shah A, Duncan K, Winson G, et al. Severe symptoms in mid and apical hypertrophic cardiomyopathy. Echocardiography. 2009; 26: 922–933.
- 30Po JR, Kim B, Aslam F, et al. Doppler systolic signal void in hypertrophic cardiomyopathy: apical aneurysm and severe obstruction without elevated intraventricular velocities. J Am Soc Echocardiogr. 2015; 28: 1462–1473.
- 31Rowin EJ, Maron BJ, Haas TS, et al. Hypertrophic cardiomyopathy with left ventricular apical aneurysm: implications for risk stratification and management. J Am Coll Cardiol. 2017; 69: 761–773.
- 32Olivotto I, Cecchi F, Gistri R, et al. Relevance of coronary microvascular flow impairment to long-term remodeling and systolic dysfunction in hypertrophic cardiomyopathy. J Am Coll Cardiol. 2006; 47: 1043–1048.
- 33Jaber WA, Wright SR, Murphy J. A patient with hypertrophic obstructive cardiomyopathy presenting with left ventricular apical ballooning syndrome. J Invasive Cardiol. 2006; 18: 510–512.
- 34Singh NK, Rehman A, Hansalia SJ. Transient apical ballooning in hypertrophic obstructive cardiomyopathy. Tex Heart Inst J. 2008; 35: 483–484.
- 35Brabham WW, Lewis GF, Bonnema DD, et al. Takotsubo cardiomyopathy in a patient with previously undiagnosed hypertrophic cardiomyopathy with obstruction. Cardiovasc Revasc Med. 2011; 12: 70. e1–e5.
- 36Modi S, Ramsdale D. Tako-tsubo, hypertrophic obstructive cardiomyopathy & muscle bridging–separate disease entities or a single condition? Int J Cardiol. 2011; 147: 133–134.
- 37Nalluri N, Asti D, Anugu VR, et al. Cardiogenic shock secondary to takotsubo cardiomyopathy in a patient with preexisting hypertrophic obstructive cardiomyopathy. Cardiovasc Imaging Case Rep. 2018; 2: 78–81.
- 38Gersh BJ, Maron BJ, Bonow RO, et al. 2011 ACCF/AHA guideline for the diagnosis and treatment of hypertrophic cardiomyopathy: executive summary: a report of the American College of Cardiology Foundation/American Heart Association. J Am Coll Cardiol. 2011; 58: 2703–2738.
- 39 Authors/Task Force, Elliott PM, Anastasakis A, et al. 2014 ESC Guidelines on diagnosis and management of hypertrophic cardiomyopathy: the Task Force for the Diagnosis and Management of Hypertrophic Cardiomyopathy of the European Society of Cardiology (ESC). Eur Heart J. 2014; 35: 2733–2779.
- 40Sherrid MV, Pearle G, Gunsburg DZ. Mechanism of benefit of negative inotropes in obstructive hypertrophic cardiomyopathy. Circulation. 1998; 97: 41–47.
- 41Flores-Ramirez R, Lakkis NM, Middleton KJ, et al. Echocardiographic insights into the mechanisms of relief of left ventricular outflow tract obstruction after nonsurgical septal reduction therapy in patients with hypertrophic obstructive cardiomyopathy. J Am Coll Cardiol. 2001; 37: 208–214.
- 42Templin C, Ghadri JR, Diekmann J, et al. Clinical features and outcomes of Takotsubo (stress) cardiomyopathy. N Engl J Med. 2015; 373: 929–938.
- 43Norcliffe-Kaufmann L, Kaufmann H, Martinez J, et al. Autonomic findings in Takotsubo cardiomyopathy. Am J Cardiol. 2016; 117: 206–213.
- 44Sherrid MV, Gunsburg DZ, Moldenhauer S, et al. Systolic anterior motion begins at low left ventricular outflow tract velocity in obstructive hypertrophic cardiomyopathy. J Am Coll Cardiol. 2000; 36: 1344–1354.
- 45Maron BJ, Gottdiener JS, Goldstein RE, Epstein SE. Hypertrophic cardiomyopathy: the great masquerader. Clinical conference from the Cardiology Branch of the National Heart, Lung, and Blood Institute, Bethesda, Md. Chest. 1978; 74: 659–670.
- 46Hong JH, Schaff HV, Nishimura RA, et al. Mitral regurgitation in patients with hypertrophic obstructive cardiomyopathy: implications for concomitant valve procedures. J Am Coll Cardiol. 2016; 68: 1497–1504.
- 47El Mahmoud R, Mansencal N, Pilliére R, et al. Prevalence and characteristics of left ventricular outflow tract obstruction in Tako-Tsubo syndrome. Am Heart J. 2008; 156: 543–548.
- 48Yalta K, Yetkin E. Late-onset dynamic outflow tract gradient in the setting of tako-tsubo cardiomyopathy: an interesting phenomenon with potential implications? Indian Heart J. 2017; 69: 328–330.
- 49Yoshioka T, Hashimoto A, Tsuchihashi K, et al. Clinical implications of midventricular obstruction and intravenous propranolol use in transient left ventricular apical ballooning (Tako-tsubo cardiomyopathy). Am Heart J. 2008; 155(): 526. e1–e7
- 50Azzarelli S, Galassi AR, Amico F, et al. Intraventricular obstruction in a patient with tako-tsubo cardiomyopathy. Int J Cardiol. 2007; 121: e22–e24.
- 51Merli E, Sutcliffe S, Gori M, Sutherland GG. Tako-Tsubo cardiomyopathy: new insights into the possible underlying pathophysiology. Eur J Echocardiogr. 2006; 7: 53–61.
- 52Wittstein IS, Thiemann DR, Lima JAC, et al. Neurohumoral features of myocardial stunning due to sudden emotional stress. N Engl J Med. 2005; 352: 539–548.