Faculty Bibliography

Clinical spectrum of hypertrophic cardiomyopathy (HC) has been expanded to include patients with mild or no thickening of the left ventricle (LV), who nevertheless have outflow tract obstruction at rest or after exercise, due to systolic anterior motion (SAM) and ventricular septal contact, with mitral valve elongation and papillary muscles anomalies. Apical ballooning mimicking a takotsubo syndrome (TS) wall motion pattern can occur in HC with mild septal thickening when latent obstruction becomes unrelenting. To define the prevalence of anatomic abnormalities characteristic of HC in patients diagnosed with TS, we analyzed echocardiograms of 44 unselected TS patients, age 67±12 years, 95% women including studies performed before the event (n = 11, median 515 days) and after recovery of left ventricular function (n = 33, median 92 days, interquartile range = 29 to 327) and compared the findings to 60 age and sexed matched controls. Analysis of echocardiograms was blinded to event timing, and patient vs. control status. During the ballooning event, 13 patients (30%) had SAM including 9 with LV outflow obstruction, peak gradients 71±40 mmHg, as well as: ventricular septal thickening (16 ± 4 mm), elongated anterior leaflets (30 ± 3mm), and increased mitral coaptation to posterior wall distance (17 ± 5 mm), consistent with diagnosis of the HC phenotype. Compared to 31 TS patients without SAM, study patients with SAM had longer anterior leaflets (30 ± 3 vs 26 ± 4 mm, p = 0.006), thicker septum (16 ± 4 vs 12 ± 3 mm), increased coaptation to posterior wall distance (17 ± 5 vs 14 ± 4 mm, p < 0.04) and reduced distance from coaptation to septum (19 ± 5 vs 27 ± 5, p < 0.001). In the 13 patients with SAM, morphologic characteristics of HC persisted after normalization of LV function. In conclusion, a subset of patients experiencing TS events demonstrates a constellation of morphologic abnormalities characteristic of HC that persist after recovery of LV wall motion. These findings suggest that dynamic outflow obstruction may cause apical ballooning in susceptible patients.

Background Recently the clinical spectrum of hypertrophic cardiomyopathy (HCM) has been expanded to include patients with mild or no thickening of the left ventricle (LV) yet who have outflow tract obstruction at rest or after exercise, principally due to characteristic HCM anterior mitral leaflet (AML) elongation and papillary muscle anomalies. Apical ballooning mimicking a takotsubo syndrome (TTS) wall motion pattern can occur in mild-septal-thickening HCM when latent obstruction becomes unrelenting. The objective of this study is to define the prevalence of anatomic abnormalities characteristic of HCM in an unselected population of patients diagnosed clinically with TTS. Methods We analyzed echocardiograms of 44 admitted TTS patients including studies performed during admission, before the event (n=11, median 515 days before) and after recovery of left ventricular function (n=33, median 92 days, IQR=29-327) and compared them to 60 controls, age-matched normal women. Analysis of 148 echocardiograms was blinded to timing, and patient vs. control status. Results Age was 67+/-12 years, 42 female (95%). During the ballooning event, 13 (30%) had SAM and 9 patients (20%) had LV outflow tract obstruction (LVOTO), gradients 71+/-40 mmHg. Compared to TTS patients without SAM, those with SAM had longer AML (30 vs. 26mm), and thicker septum (16 vs. 12 mm) and less distance from septum to coaptation (19 vs. 27mm), all p <=0.006. Eleven of the SAM patients had >=2 anatomic abnormalities predisposing to obstruction (defined as > 2 SD above normal), and/or an anomalous papillary muscle/chordae. In the 44 TTS patients each parameter differed from controls before, during and after the TTS event. Eight (18%) had abnormal right ventricular wall motion, none of whom were obstructed. Conclusion Thirty percent of unselected TTS patients have SAM and 20% have significant LVOT gradients. This subset had AML abnormalities and septal thickening typical of obstructive HCM and known to predispose to LVOT obstruction. They are phenotypically identical to patients with documented HCM with mild septal thickening and LVOT obstruction, who have experienced episodes of ballooning.

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/UNASSIGNED: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.

BACKGROUND:Midwall fibrosis and low stroke volume are independent predictors of mortality in severe aortic stenosis (AS) with preserved LV ejection fraction (LVEF). The role of speckle tracking echocardiography (STE) to identify latent myopathy pre- and post- aortic valve replacement (AVR) in high risk AS patients with normal LVEF is limited. METHODS:. RESULTS:; P = .01) improved post-AVR. Pre-AVR mid-segments showed a similar myopathy as the basal segments (-9.5 ± 4.3% vs -9.0 ± 4.2%;P = .3). The 16 (43%) LF patients in this study had lower pre- and post-AVR strain compared to NF patients (GLS Pre-AVR:LF vs NF: -5.1 ± 4.1% vs -8.4 ± 4.9% (P = .04) and GLS Post-AVR:LF vs NF: -9.2 ± 3.7% vs -12.5 ± 3.9% (P = .01)). However, there was no difference in absolute and %change improvement in GLS post-AVR (LF vs NF:∆ -4.2 ± 3.5% vs ∆-4.1 ± 5.3% (P = .90) and 193 ± 214% vs 143 ± 230% change (P = .5)). The lowest GLS was seen in LF/HG AS followed by LF/LG, NF/LG and NF/HG AS; P = .03. CONCLUSIONS:Latent myopathy is more pronounced in LF AS both pre- and post-AVR. Our study provides evidence of improvement in myopathy in LF AS despite a persistent worse myopathy compared to NF patients post-AVR.

Transesophageal echocardiography is essential in guiding the surgical approach for patients with obstructive hypertrophic cardiomyopathy. Septal hypertrophy, elongated mitral valve leaflets, and abnormalities of the subvalvular apparatus are prominent features, all of which may contribute to left ventricular outflow tract obstruction. Surgery aims to alleviate the obstruction via an extended myectomy, often with an intervention on the mitral valve and subvalvular apparatus. The goal of intraoperative echocardiography is to assess the anatomic pathology and pathophysiology in order to achieve a safe intraoperative course and a successful repair. This guide summarizes the systematic evaluation of these patients to determine the best surgical plan.

BACKGROUND:It is not clear whether radial artery (RA), right internal thoracic artery (RITA), or saphenous vein (SV) is the preferred second bypass graft during coronary artery bypass graft surgery using the left internal thoracic artery (LITA) in patients aged less or greater than 70 years. METHODS:Late survival data were collected for 13,324 consecutive, isolated, primary coronary artery bypass graft surgery patients from three hospitals. Cox regression analysis was performed on all patients grouped by age. RESULTS:Adjusted Cox regression showed overall better RA versus SV survival (hazard ratio [HR] 0.82, p < 0.001) and no difference in RITA versus SV survival (HR 0.95, p = 0.35). However, the survival benefit of RA versus SV was seen only in patients aged less than 70 years (HR 0.77, p < 0.001); and RITA patients aged less than 70 years also had a survival benefit compared with SV (HR 0.86, p = 0.03). There was no difference in survival for RA versus RITA across all ages. CONCLUSIONS:For patients aged less than 70 years, the optimal grafting strategy is using either RA or RITA as the second preferred graft. In patients aged 70 years or more, RA and RITA grafting should be used selectively. Multiple arterial grafting using either RA or RITA should be more widely utilized during coronary artery bypass graft surgery for patients less than 70 years of age.