Abstract 12401: Hypertrophic Cardiomyopathy With Dynamic Obstruction and High Left Ventricular Outflow Gradients Associated With Paradoxical Apical Ballooning

• Published in: Circulation (New York, N.Y.) Vol. 138; no. Suppl_1 Suppl 1; p. A12401
• Main Authors: Riedy, Katherine N, Ahluwalia, Monica, Rosenzweig, Barry, Arabadjian, Milla, Saric, Muhamed, Swistel, Daniel, Reynolds, Harmony R, Owlia, Mina, Serrato, Stephanie, Sharma, Sonali, Troy, Aaron, Rivera, Elsy, Kim, Bette, Balaram, Sandhya, Frenneaux, Michael, Sherrid, Mark V
• Format: Journal Article
• Language: English
• Published: by the American College of Cardiology Foundation and the American Heart Association, Inc 06.11.2018
• ISSN: 0009-7322; 1524-4539

BackgroundAcute left ventricular (LV) apical ballooning with normal coronary angiography occurs rarely in obstructive hypertrophic cardiomyopathy (OHCM); it may be associated with severe hemodynamic instability.MethodsWe searched for acute LV ballooning with apical hypokinesia/akinesia in the 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.ResultsAmong 1,519 HCM patients we observed acute LV ballooning associated with dynamic outflow tract (LVOT) obstruction and high gradients in 13 (0.9%), 10 female (77%), age 64 ±7 years. At their ballooning event LVOT obstruction was severe with gradients 92 ±37mmHg. In 8 patients latent obstructive HCM had been diagnosed a median 25 months before their ballooning event. In 5 patients obstructive HCM was first diagnosed at the ballooning with high LVOT gradients at that time. In recovery in these 5 patients, gradients subsided to <30 mmHg and LV function normalized. However, 5 months later severe latent LVOT obstruction was definitively demonstrated by provocation of high gradients after Valsalva or exercise. Septal hypertrophy was mild compared to that of the rest of our HCM cohort, 15 vs. 20 mm (p<0.00001), and was limited to the basal anterior septum. An elongated anterior mitral leaflet or anteriorly displaced papillary muscles occurred in 77%. LV ejection fraction was 31.9 ±10%. Biomarker rise was modest for degree of wall motion abnormality. 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 3 patients, surgery immediately normalized refractory severe LV dysfunction and reversed cardiogenic shock and heart failure.ConclusionsAcute LV apical ballooning may punctuate the course of obstructive HCM associated with high dynamic left ventricular outflow gradients. The syndrome is important to recognize on echocardiography because it may be associated with profound reversible LV decompensation.