Apical Ballooning and Cardiogenic Shock in Obstructive Hypertrophic Cardiomyopathy

Acute Left Ventricular Ballooning: Tools to Differentiate Hypertrophic Cardiomyopathy with Outflow Obstruction from Neurohumoral Takotsubo Syndrome

“…Our literature search yielded case series [ 7 , 8 , 9 , 12 , 13 , 14 ], case reports [ 15 , 16 , 17 , 18 , 19 , 20 , 21 , 22 , 23 , 24 , 25 , 26 , 27 , 28 , 29 , 30 , 31 , 32 , 33 , 34 , 35 , 36 , 37 , 38 ], one prospective cohort study [ 39 ], and one systematic review [ 40 ] that described cases of obstructive HCM with LV ballooning overlapping with neurohumoral Takotsubo syndrome (TTS). We propose key features to differentiate these diagnoses, as shown in Fig.…”

“…Multiple case reports of LV apical ballooning in patients with HCM describe how prompt treatment of septal hypertrophy, predominantly medically but notably also after emergency myectomy or alcohol septal ablation, can lead to resolution of LVOT obstruction and rapid clinical improvement [ 19 , 22 , 23 , 24 ]. While many of these patients were diagnosed with overlapping TTS and HCM, the almost immediate clinical improvement of shock and ballooning after surgical relief of septal hypertrophy and LVOT obstruction provides striking evidence that acute apical ballooning is in many cases a direct complication of severe obstructive HCM [ 8 , 15 , 20 , 45 ].…”

“…A retrospective study of two HCM treatment programs found that 0.9% of HCM patients experienced acute LV ballooning associated with dynamic LVOT obstruction and mean gradients of 92 37 mmHg [ 7 ]. It is hypothesized that severe LVOT obstruction (usually 50 mmHg) independently leads to acute apical ballooning through afterload mismatch and supply-demand ischemia [ 15 , 17 ]. A graphic demonstration of the corrosive effect of LV outflow obstruction by afterload mismatch is the Doppler finding of a mid-systolic drop in LV ejection velocities, the so-called “lobster claw abnormality” (Fig.…”

“…Intra-aortic balloon pump counter-pulsation, by decreasing afterload, and Impella support, by decreasing preload, may exacerbate LVOT obstruction [ 8 ]. Among the available mechanical circulatory support devices, VA-ECMO may be the favored option as it can offer full circulatory support while mildly increasing afterload, which could therefore benefit patients with obstructive HCM physiology [ 15 ]. Moreover, VA-ECMO can act as a bridge to patient recovery or to septal reduction therapy [ 8 , 15 ].…”

“…Among the available mechanical circulatory support devices, VA-ECMO may be the favored option as it can offer full circulatory support while mildly increasing afterload, which could therefore benefit patients with obstructive HCM physiology [ 15 ]. Moreover, VA-ECMO can act as a bridge to patient recovery or to septal reduction therapy [ 8 , 15 ]. These management practices are guided by observational studies and case series, and have been extrapolated from the current knowledge base in patients with HCM without acute apical ballooning.…”