Syndrome of Reversible Cardiogenic Shock and Left Ventricular Ballooning in Obstructive Hypertrophic Cardiomyopathy

Abstract

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.

Obstructive Hypertrophic Cardiomyopathy and Takotsubo Syndrome: How to Deal With Left Ventricular Ballooning?

“…These patients completely resemble patients reported previously with mild‐septal‐thickness OHCM who have developed acute LV ballooning. 7 , 8 Based on these data, on previous observations of LV ballooning in OHCM, and on plausible pathophysiologic mechanisms, in a subset of patients with clinically diagnosed LV ballooning, mitral–septal contact is not a result of the wall motion abnormalities but instead is the cause of severe systolic dysfunction.…”

“… 9 Within 2 hours their severe LV dysfunction and their shock had improved, and LV function rapidly returned to normal. Subsequently, in a multicenter report, 14 other cases of cardiogenic shock in OHCM from LVOTO and ballooning have been reported, 8 with 8 requiring mechanical circulatory support and 5 reversible only by septal reduction. The reversal of ballooning and refractory cardiogenic shock, by relief of obstruction, provides compelling evidence that the obstruction was the cause of ballooning and shock.…”

“… 3 , 4 Others, however, have shown that a minority of patients with LV ballooning have obstructive hypertrophic cardiomyopathy (OHCM), with latent obstruction and the sudden development of unrelenting high‐outflow gradients as a precipitating cause. 5 , 6 , 7 , 8 Hickam's dictum would allow >1 cause for LV ballooning.…”

“…In this review, we will refer to this pathogenesis as LV ballooning from OHCM. 5 , 6 , 7 , 8 , 9 , 10 …”

Intra-Aortic Balloon Pump Exacerbates Left Ventricular Outflow Tract Obstruction in a Patient With Takotsubo and Hypertrophic Cardiomyopathy

“… 5 , 6 , 7 Sherrid et al . 8 described a case series of 14 patients with HCM who developed cardiogenic shock associated with apical ballooning and high LVOT gradients. Although mimicking classical takotsubo cardiomyopathy, the apical ballooning was thought to reflect the culmination of underlying septal hypertrophy, LVOT obstruction, and mitral valve abnormalities.…”