ROLE OF LEFT VENTRICULAR CIRCUMFERENTIAL STRAIN IN THE PREDICTION OF CLINICAL OUTCOME IN PATIENTS WITH END-STAGE PHASE OF HYPERTROPHIC CARDIOMYOPATHY

Introduction: Left ventricular non-compaction (LVNC) is characterized by extensively trabeculaed myocardium adjacent to normal compacted myocardium of the left ventricle (LV). Hypertrophic cardiomyopathy (HCM) typically appears as diffuse or segmental LV hypertrophy, with or without outflow tract obstruction. Cardiac sarcomere mutations are present in most HCM cases and have also been identified in LVNC. Whether or not there is clinically significant phenotypic overlap between the two diseases is less well understood. We present a case of known HCM that met criteria for both LVNC and HCM by cardiac MRI. Case: A 49-year old man with HCM due to a c.3742_3759dup variant in MYBPC3 presented to clinic after an episode of syncope and ICD firing. In clinic, the device was interrogated and he was found to have had ventricular flutter which was successfully treated with one shock and a new, high (>20%) burden of premature ventricular beats. An echocardiogram showed a stable ejection fraction at 42%, mild concentric LV hypertrophy without obstruction and a newly dilated LV with an end diastolic diameter of 7.1cm (previously 6.2cm). A cardiac MRI was performed ( Figure ) and showed LV noncompaction and diffuse transmural and mid myocardial hyperenhancement/fibrosis of the septum, basilar lateral wall, anterior wall, and distal right ventricle consistent with patient's long-standing history of hypertrophic cardiomyopathy. Discussion: This case highlights the phenotypic overlap between HCM and LVNC by cardiac MRI. Had this patient not already carried a genetic diagnosis of HCM, he would likely have been diagnosed with LVNC based on this cardiac MRI. The phenotypic overlap in these diseases raises questions about ICD guidelines, the role of anticoagulation and prognosis.

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Abstract 15253: A Paradoxical Rise in Serum Copeptin After Non-Pulsatile LVAD: a New SIADH?

Circulation ◽

10.1161/circ.130.suppl_2.15253 ◽

2014 ◽

Vol 130 (suppl_2) ◽

Author(s):

Cara M Statz ◽

Aleksandra M Ras ◽

Kevin D Ballard ◽

Jason A Gluck ◽

Detlef Wencker

Keyword(s):

Surrogate Marker ◽

Left Ventricular ◽

Serum Samples ◽

Ventricular Assist ◽

Bridge To Transplant ◽

Left Ventricular Assist ◽

End Stage ◽

A Prospective Study

Introduction: Hyponatremia (Na< 136 mmol/L) is prevalent (~28%) among hospitalized heart failure (HF) patients and a marker of advanced/end-stage HF(AHF) with increased mortality. Treatment of hyponatremia has no survival benefit in this population. In a prospective study of AHF patients, we sought to define the prevalence, pathophysiology and role of V2 vasoreceptor activity in the development of hyponatremia. Serum copeptin (S-COP), a surrogate marker for AVP activity, was assessed during AHF therapies (AHFT) which included axial-non-pulsatile left ventricular assist device (LVAD) and/or heart transplant (HTx). Methods: Serum samples were collected from AHF patients pre and post AHFT and compared with normal controls. S-COP levels were assessed using an enzyme linked immunosorbant assay and correlated to clinical variables, serum Sodium (S-Na) and glomerular filtration rate (eGFR). Results: Among 89 consecutive (mean age 56.2±15.35; M=69) patients awaiting AHFT, 54 (60%) were hyponatremic. Preop S-COP was elevated compared to controls (0.68±0.50 vs 0.53±0.13 ng/ml, P=.02) and inversely correlated to S-Na and eGFR (r=-0.23, P<.05, r=-0.23, P<.05; N=81); conversely, eGFR and S-Na were uncorrelated. AHFT (n=42) normalized S-Na (133.2±4.1 vs 136.1±3.5 mmol/l; P=.001) and improved eGFR (47.7±13.6 vs 52.7±9.7 mL/min/1.73sqm; P=.001); however, post LVAD (n=34) S-COP rose (0.67±0.21 vs 1.84±0.76 ng/ml; P<.0001) with incomplete normalization of S-NA (132.9±4.3 vs 135.9±3.8 mmol/l; P<.01). In contrast, post HTx (n=9) S-COP was unchanged to pre-AHFT (0.59±0.32 vs 0.65±0.16 ng/ml; P>.1) and was lower compared to post LVAD (0.65±0.16 vs 1.84±0.76 ng/ml; P<.0001). Elevated S-COP with LVAD as bridge to transplant (n=3) showed a marked decrease post HTx (2.7±0.50 vs 1.0±0.29 ng/ml; P<.01). Conclusions: In AHF, the prevalence of hyponatremia is double compared to acute hospitalized HF and associated with S-COP surge prior to AHFT. Unexpectedly, LVAD but not HTx was associated with rising S-COP and incomplete S-Na recovery despite clinical improvement, suggesting inappropriate antidiuretic hormone release. The association of S-COP rise with non-pulsatile LVAD and potential benefit of long-term AVP inhibition post LVAD merits further studies.

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Supplementary role of left ventricular global longitudinal strain for predicting sudden cardiac death in hypertrophic cardiomyopathy

European Heart Journal - Cardiovascular Imaging ◽

10.1093/ehjci/jeab187 ◽

2021 ◽

Author(s):

Hyun-Jung Lee ◽

Hyung-Kwan Kim ◽

Sang Chol Lee ◽

Jihoon Kim ◽

Jun-Bean Park ◽

...

Keyword(s):

Hypertrophic Cardiomyopathy ◽

Sudden Cardiac Death ◽

Risk Score ◽

Primary Endpoint ◽

Cardiac Death ◽

Longitudinal Strain ◽

Global Longitudinal Strain ◽

Left Ventricular ◽

C Statistic

Abstract Aims We investigated the prognostic role of left ventricular global longitudinal strain (LV-GLS) and its incremental value to established risk models for predicting sudden cardiac death (SCD) in patients with hypertrophic cardiomyopathy (HCM). Methods and results LV-GLS was measured with vendor-independent software at a core laboratory in a cohort of 835 patients with HCM (aged 56.3 ± 12.2 years) followed-up for a median of 6.4 years. The primary endpoint was SCD events, including appropriate defibrillator therapy, within 5 years after the initial evaluation. The secondary endpoint was a composite of SCD events, heart failure admission, heart transplantation, and all-cause mortality. Twenty (2.4%) and 85 (10.2%) patients experienced the primary and secondary endpoints, respectively. Lower absolute LV-GLS quartiles, especially those worse than the median (−15.0%), were associated with progressively higher SCD event rates (P = 0.004). LV-GLS was associated with an increased risk for the primary endpoint, independent of the LV ejection fraction, apical aneurysm, and 2014 European Society of Cardiology (ESC) risk score [adjusted hazard ratio (aHR) 1.14, 95% confidence interval (CI) 1.02–1.28] or 2011 American College of Cardiology/American Heart Association (ACC/AHA) risk factors (aHR 1.18, 95% CI 1.05–1.32). LV-GLS was also associated with a higher risk for the composite secondary endpoint (aHR 1.06, 95% CI 1.01–1.12). The addition of LV-GLS enhanced the performance of the ESC risk score (C-statistic 0.756 vs. 0.842, P = 0.007) and the 2011 ACC/AHA risk factor strategy (C-statistic 0.743 vs. 0.814, P = 0.007) for predicting SCD. Conclusion LV-GLS is an important prognosticator in patients with HCM and provides additional information to established risk stratification strategies for predicting SCD.

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Hypertrophic cardiomyopathy

The ESC Textbook of Cardiovascular Imaging ◽

10.1093/med/9780198849353.003.0043 ◽

2021 ◽

pp. 629-644

Author(s):

Nuno Cardim ◽

Alexandra Toste ◽

Robin Nijveldt

Keyword(s):

Hypertrophic Cardiomyopathy ◽

Multimodality Imaging ◽

Imaging Techniques ◽

Genetic Diagnosis ◽

Left Ventricular ◽

Morphological Data ◽

Clinical Settings ◽

Functional Information ◽

Clinical Questions

Imaging plays a major role in the evaluation of hypertrophic cardiomyopathy (HCM) patients, offering answers to clinical questions. Imaging techniques provide a broad spectrum of information, including morphological data, functional information, and ischaemia assessment, useful in many clinical settings of HCM. The clinical diagnosis of HCM is based on unexplained left ventricular hypertrophy (LVH) by imaging, though the role of genetic diagnosis has increased. A multimodality imaging (MMI) approach is encouraged in HCM. Each technique must be selected to provide solutions to the specific problems, avoiding duplicated data, and taking into account its technical characteristics, availability, benefits, risks, and costs.

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Effect of hypokintic transformation on the clinical phenotype and functional capacity in hypertrophic cardiomyopathy

European Heart Journal ◽

10.1093/ehjci/ehaa946.2091 ◽

2020 ◽

Vol 41 (Supplement_2) ◽

Author(s):

Y Wasserstrum ◽

E Itelman ◽

R Barriales-Villa ◽

X Fernandez-Fernandez ◽

Y Adler ◽

...

Keyword(s):

Hypertrophic Cardiomyopathy ◽

Nyha Class ◽

Left Ventricular ◽

Left Ventricular Ejection ◽

Ventricular Tachyarrhythmias ◽

Ventricular Ejection Fraction ◽

Icd Therapy ◽

Medical Centers ◽

Increased Risk

Abstract Background Advanced hypertrophic cardiomyopathy (HCM) may be complicated by a dilated hypokinetic transformation. Reduced left ventricular ejection fraction (HFrEF) has been described in terms of specific risks of morbidity and mortality, and specifically in terms of increased risk for fatal arrhythmias. Nevertheless, recent publications have casted doubt regarding the role of arrhythmia in non-ischemic HFrEF and questioned the role of primary prevention strategies in these cases. Methods We've reviewed clinical characteristics of 883 patients age ≥40, diagnosed with HCM who were evaluated in the cardiomyopathy clinic in two tertiary medical centers in Israel and Spain. Results Forty-five patients (5%) suffered from hypokinetic transformation. They were younger at diagnosis (median 32 [IQR 24–55] vs. 49 [35–60], p<0.001), had a lower body-mass index (28.4 [±4.7] vs. 26.0 [±3.9], p<0.001), and suffered more from strokes (19% vs 6%, p<0.001). They had lower had a lower NYHA class (p=0.001) and lower exercise capacity (7.3 [4.5–10.8] vs. 9.6 [6.7–12.0] METS, p<0.001). Patients with hypokinetic HCM had higher rates of pacemaker and implanted defibrillator (ICD) implantations (41% vs 11%, p<0.001) and (43% vs 13%, p<0.001) respectively. These patients had a higher incidence of sustained ventricular tachyarrhythmias (14% vs 2%, p<0.001). Among patients who had an ICD, patients suffering from hypokinetic transformation had received more appropriate ICD therapy (27% vs 12%, p<0.001). These patients received more heart transplantations (13% vs 1%, p<0.001), and had a trend for higher incidence rate of Sudden cardiac death (6% vs 2% p=0.06) and a higher 5-year mortality rates (21% vs. 5%, p<0.001). Conclusions HCM patients suffering from hypokinetic transformation have lower functional and exercise capacities, are more likely to suffer from ventricular tachyarrhythmias and experience appropriate ICD therapy, and undergo heart transplantation. They also have a significantly lower 5-year survival. Five-year survival Funding Acknowledgement Type of funding source: None

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Pathology of End-stage Remodeling in a Family of Cats with Hypertrophic Cardiomyopathy

Veterinary Pathology ◽

10.1354/vp.42-4-458 ◽

2005 ◽

Vol 42 (4) ◽

pp. 458-467 ◽

Cited By ~ 34

Author(s):

M. F. Cesta ◽

C. J. Baty ◽

B. W. Keene ◽

I. W. Smoak ◽

D. E. Malarkey

Keyword(s):

Heart Failure ◽

Hypertrophic Cardiomyopathy ◽

Inflammatory Cell ◽

Interventricular Septum ◽

Coronary Vessels ◽

Left Ventricular ◽

Coronary Vessel ◽

End Stage ◽

Mononuclear Inflammatory Cell ◽

Fractional Shortening

End-stage hypertrophic cardiomyopathy (ES-HCM), affecting 5-10% of human hypertrophic cardiomyopathy (HCM) patients, is characterized by relative thinning of the ventricular walls and septum with dilation of the ventricular lumen, decreased fractional shortening, and progression to heart failure. C. J. Baty and others recently documented similar progressive changes to ES-HCM in a family of four cats through serial echocardiograms. At the time of heart failure, these cats exhibited changes similar to those exhibited by human ES-HCM patients. Our objectives were to describe the pathologic alterations associated with ES-HCM and investigate the pathogenesis in three of the four cats. Grossly, there was left atrial dilation with relative thinning of the interventricular septum (IVS) and left ventricular free wall (LVFW). The left atrium contained large thrombi in two of the three cats, and all three cats died following thromboembolization of the aortic bifurcation. Histologically, all three cats had subendocardial and myocardial fibrosis, predominantly of the IVS and LVFW, and one cat had acute, multifocal, myocardial infarcts with mononuclear inflammatory cell infiltrates. The pathogenesis of ES-HCM is uncertain, but theories implicate occlusion of the coronary blood flow by thickening of the coronary vessels, coronary vascular thromboembolism or coronary vessel spasm, apoptosis of myocytes, and myocardial hypertrophy beyond the ability of the vasculature to supply blood. Apoptosis assays did not reveal any apoptotic myocytes. Considering the hypercoagulative state of these cats, coronary vascular thromboembolism could be a major contributing factor. We cannot exclude apoptosis or coronary vessel spasm on the basis of the data presented.

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