Faculty Opinions recommendation of Echocardiographic serial changes of hypertensive cardiomyopathy with severely reduced ejection fraction: comparison with idiopathic dilated cardiomyopathy.

Abstract Background: Cushing syndrome (CS) is well known to be associated with metabolic syndrome, venous thromboembolism, hypertension, left ventricular hypertrophy, and rarely dilated cardiomyopathy. The pathophysiological effects of hypercortisolism on the myocardium results in cardiomyocyte hypertrophy, myofibrillolysis, myocardial fibrosis, global longitudinal and circumferential strain. Our patient presents an example of CS in the setting of chronic heart failure with reduced ejection fraction, multiple thromboembolic events, and diabetes mellitus in a young adult. Clinical Case: A 34-year male with a past medical history of dilated cardiomyopathy with biventricular failure and left ventricle ejection fraction of 10%, pulmonary embolism, diabetes mellitus, and gout presented with shortness of breath. On physical exam he presented with symptoms of CS: moon facies, supraclavicular fat pads, dorsal fat pads, purple striae of abdominal skin, truncal obesity, ecchymosis, and skin atrophy. Labs showed elevated morning cortisol after overnight 1 mg dexamethasone suppression test (cortisol 8.6 mcg/dl, n< 5 mcg/dl), repeat morning cortisol after overnight 1 mg dexamethasone suppression test (cortisol 5.7 mcg/dl, n< 5 mcg/dl), and ACTH-concentrations <5 pg/ml. CT-scan was insignificant for adrenal hyperplasia. He was admitted and treated for acute congestive heart failure exacerbation with plans for definitive treatment of CS outpatient with further imaging studies. Conclusion: The occurrence of CS induced heart failure results in increased mortality. There have been numerous accounts of resolution of cardiomyopathy after surgical treatment for CS secondary to adrenal adenoma. Our patient had a 10-year history of chronic dilated cardiomyopathy prior to cushingoid symptoms and confirmatory endocrinological data. The definitive treatment for CS syndrome in our patient would eventually be surgery targeting the source of hypercortisolism, however his cardiovascular risk factors would make him a poor surgical candidate. Severely reduced ejection fraction is a contraindication for generalized anesthesia needed for surgery. When surgery is contraindicated in CS medical management is recommended according to guidelines that target pituitary-directed medical treatments for Cushing’s disease and targeted therapies to treat ectopic ACTH syndrome. To our knowledge there have been few studies that demonstrate the effects of CS treatment on chronic conditions such as dilated cardiomyopathy. Studies have shown that surgical treatment for CS have reversed cardiomyopathy caused by CS but it still remains to be answered whether this same effect is achieved to some degree in chronic dilated cardiomyopathy.

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The Effect of Sacubitril/Valsartan on Device Detected Arrhythmias and Electrical Parameters among Dilated Cardiomyopathy Patients with Reduced Ejection Fraction and Implantable Cardioverter Defibrillator

Journal of Clinical Medicine ◽

10.3390/jcm9041111 ◽

2020 ◽

Vol 9 (4) ◽

pp. 1111 ◽

Cited By ~ 1

Author(s):

Vincenzo Russo ◽

Roberta Bottino ◽

Anna Rago ◽

Andrea Antonio Papa ◽

Biagio Liccardo ◽

...

Keyword(s):

Ejection Fraction ◽

Dilated Cardiomyopathy ◽

Implantable Cardioverter Defibrillator ◽

Reverse Remodeling ◽

Electrical Parameters ◽

Pacing Threshold ◽

Reduced Ejection Fraction ◽

Cardioverter Defibrillator ◽

Echocardiographic Parameters

Sacubitril/valsartan therapy reduces sudden cardiac death (SCD) among patients with reduced ejection fraction (HFrEF) when compared to guidelines recommended doses of enalapril, however the mechanism is still not clear. There are few, contrasting results about the effect of sacubitril/valsartan on arrhythmias in the clinical context of dilated cardiomyopathy (DCM) and there are no clinical data about its effect on measured implantable cardioverter defibrillator (ICD) electrical parameters, such as atrial/ventricular electrograms sensing and pacing threshold. We conducted a 12 month follow-up observational study in 167 ischemic and nonischemic DCM patients (mean age 68.1 ± 11.6 years; 85% male), with dual-chamber ICD on sacubitril/valsartan treatment, to evaluate the incidence of device detected tachyarrhythmia events, both atrial and ventricular, and the change in measured ICD electrical parameters. We collected data on clinical, electrocardiographic and echocardiographic parameters to find a possible electro-mechanical correlation within results. Our results show that DCM patients with reduced ejection fraction and ICD on sacubitril/valsartan treatment experienced a reduction in both atrial and ventricular arrhythmias incidence and an improvement in ICD electrical atrial parameters. The findings might be explained by the electro-mechanical cardiac reverse remodeling induced by sacubitril/valsartan therapy.

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Abstract 14182: Myocardial Fibrosis and Reverse Remodelling in Nonischaemic Dilated Cardiomyopathy

Circulation ◽

10.1161/circ.132.suppl_3.14182 ◽

2015 ◽

Vol 132 (suppl_3) ◽

Author(s):

Andrea Barison ◽

Alessandro Ortalda ◽

Giancarlo Todiere ◽

Giuseppe Vergaro ◽

Gianluca Mirizzi ◽

...

Keyword(s):

Ejection Fraction ◽

Dilated Cardiomyopathy ◽

Myocardial Fibrosis ◽

Left Ventricular ◽

Idiopathic Dilated Cardiomyopathy ◽

Post Contrast ◽

Reverse Remodelling ◽

Lv Volumes ◽

Lv Ejection Fraction

Introduction: In nonischaemic dilated cardiomyopathy (NICM), myocardial fibrosis can be detected by cardiovascular magnetic resonance (CMR) as late gadolinium enhancement (LGE) and is associated with worse prognosis. Hypothesis: Absence of myocardial fibrosis is associated with left ventricular reverse remodelling (LV-RR). Methods: One-hundred-and-twenty-five NICM patients (age 51±16 years, 82 male) were enrolled and underwent baseline CMR; patients with ischaemic, valvular, congenital heart disease, other cardiomyopathies or contraindications to CMR were excluded. After a 24-month follow-up on optimal medical therapy, all patients underwent a second CMR; patients who died, underwent device implantation or declined a second CMR, were also excluded from the study. LGE was quantified on post-contrast CMR images. LV-RR was defined as an increase in LV ejection fraction ≥10 U or decrease in LV end-diastolic volume ≥10% at follow-up. Results: Mean LV ejection fraction was 41±11% at baseline, 47±12% at follow-up: LV-RR was observed in 59 patients (47%), with no age or gender difference (p=NS) . LGE was present in 69 (54%) patients at baseline (mean extent 12±6 g), without significant differences at follow-up (mean extent 13±7 g). Patients experiencing LV-RR during follow-up presented a baseline worse LV ejection fraction (36±12%) than no-LV-RR patients ( 45±9%, p<0.01), greater LV volumes (123±38 vs. 110±22 ml/m2, p=0.02) and worse right ventricular ejection fraction (54±12% vs. 59±10%, p=0.02) . Nevertheless, only 17 (29%) LV-RR patients presented LGE compared to 31 (47%, p=0.04) no-LV-RR patients. Moreover, among LGE-positive patients (n=48), only 17 (35%) developed LV-RR, while among LGE-negative patients (n=77), 42 (55%) developed LV-RR (p=0.04). Multivariate regression analysis showed that the absence of LGE at baseline CMR was a strong predictor of LV-RR (p=0.02), even after correction for age, New York Heart Association class, LV volumes and systolic function. Conclusions: In patients with idiopathic dilated cardiomyopathy, absence of LGE was a strong independent predictor of LV-RR at 2-year follow-up, irrespective of the initial clinical status and the severity of ventricular dilatation and dysfunction.

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