Hypertrophic cardiomyopathy with dynamic obstruction and high left ventricular outflow gradients associated with paradoxical apical ballooning

Abstract

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. K E Y W O R D S hypertrophic cardiomyopathy, left ventricular outflow obstruction, left ventricular systolic dysfunction

Left ventricle accessory antero-septal papillary muscle: an echocardiography and cardiac MRI case-series report

“…Two of the four echocardiography cases illustrated had moderate LV hypertrophy and three presented a prominent "sigmoid septum" at the level of the accessory muscle insertion [7]. While false LV cords with no apparent contraction and an anteriorly displaced papillary muscle inserting onto the base of the anterior mitral leaflet [1,8] or elongated anterior mitral valve leaflet have been previously described, no reports have looked at a contractile papillary muscle inserting into the antero-septal area [9]. At the same time, no reports to date have described a series of patients with an anteroseptal papillary muscle present both in patients with and without HOCMas seen by CMR, more often though detectable in HOCM patients.…”

Cardiogenic Shock in Obstructive Hypertrophic Cardiomyopathy Plus Apical Ballooning

“…In this case, it was also possible to attribute the apical ballooning to a Takotsubo syndrome, induced by the infection, or to an increase in the LVOTO due to fever and hypovolemia ( 4 ).…”

“…The combination of apical ballooning and obstructive HCM is rare, and similar cases were initially considered superimposition with a Takotsubo syndrome ( 3 ). A recent hypothesis is that the LVOTO, through afterload mismatch and supply/demand ischemia, is itself responsible for apical ballooning, as supported by the rapid recovery after LVOTO resolution ( 4 ).…”

“…Septal reduction therapy is recommended in patients in New York Heart Association functional classes III to IV with a LVOT gradient ≥50 mm Hg ( 1 ). We found 2 patients with LVOTO, apical ballooning, and CS treated with urgent septal myectomy ( 4 ) or alcohol ablation ( 7 ). In our case, initial urgent septal myectomy was not considered a feasible option due to severe hemodynamic instability and severe hypoxia; therefore, a 2-step approach with VA-ECMO support, followed by a surgical approach, was chosen.…”

Mechanical stress concealed force enhancement and ionic transient membrane potential and metabolism in akinesia of stress cardiomyopathy

“…The relaxation was interrupted. The intracellular Ca 2+ transient time was mostly prolonged in optimally lengthened cardiac muscle cells (H7 line cells); however, stretching beyond the optimum length significantly reduced this time (Figure 20) [106] .…”