scholarly journals Cardiomyopathy Associated With Tertiary Adrenal Insufficiency Manifesting as Refractory Heart Failure, Shock, and Sudden Cardiac Death: A Case Report

2021 ◽  
Vol 8 ◽  
Author(s):  
Xuefeng Wang ◽  
Yong Luo ◽  
Jian Feng
Keyword(s):  
Heart Failure ◽  
Sudden Cardiac Death ◽  
Dilated Cardiomyopathy ◽  
Adrenal Insufficiency ◽  
Cardiac Death ◽  
Clinical Manifestations ◽  
Underlying Disease ◽  
Pituitary Hormones ◽  
Idiopathic Dilated Cardiomyopathy ◽  
Cardiac Resynchronization

Dilated cardiomyopathy is an etiologically heterogeneous disorder. Early diagnosis and prompt treatment of the underlying disease are of great significance. Primary and secondary adrenal insufficiency are considered quite rare causes of dilated cardiomyopathy. However, to the best of our knowledge, no case of cardiomyopathy associated with tertiary adrenal insufficiency has been reported. Herein, we described a 68-year-old woman with a 15-year history of seasonal dermatitis presented with frequent heart failure and shock. At first, she was diagnosed with idiopathic dilated cardiomyopathy, but standard heart failure and antishock treatment failed. Given her long-term use of dexamethasone for treating seasonal dermatitis, and clinical manifestations consistent with adrenal insufficiency, we tested her basal plasma cortisol, simultaneous corticotropin, and other pituitary hormones, confirming that she had tertiary adrenal insufficiency. Additionally, abdominal enhanced computed tomography revealed atrophic bilateral adrenal glands, indicating long-standing and severe adrenal insufficiency. Then hydrocortisone replacement therapy was initiated, and she recovered rapidly. During the next 2 years of follow-up, she never experienced any episodes of heart failure and shock. Unfortunately, she refused the implantation of defibrillator with cardiac resynchronization therapy (CRT-D) and died of sudden cardiac death 2 years later. Although we could not exclude the coincidence of idiopathic dilated cardiomyopathy with tertiary adrenal insufficiency with 100% certainty, her unique clinical course strongly indicated that her cardiomyopathy resulted from tertiary adrenal insufficiency. This case demonstrates that patients on corticosteroids are at risk for tertiary adrenal insufficiency, which may result in refractory cardiomyopathy and even sudden cardiac death.

scholarly journals Risk stratification and subclinical phenotyping of dilated and/or arrhythmogenic cardiomyopathy mutation-positive relatives: CVON eDETECT consortium

2021 ◽  
Author(s):  
R. W. Roudijk ◽  
K. Taha ◽  
M. Bourfiss ◽  
P. Loh ◽  
L. van den Heuvel ◽  
...  
Keyword(s):  
Heart Failure ◽  
Sudden Cardiac Death ◽  
Early Detection ◽  
Risk Stratification ◽  
Dilated Cardiomyopathy ◽  
Cardiac Death ◽  
Diagnostic Techniques ◽  
Arrhythmogenic Cardiomyopathy ◽  
Early Detection Of Disease ◽  
Non Invasive

AbstractIn relatives of index patients with dilated cardiomyopathy and arrhythmogenic cardiomyopathy, early detection of disease onset is essential to prevent sudden cardiac death and facilitate early treatment of heart failure. However, the optimal screening interval and combination of diagnostic techniques are unknown. The clinical course of disease in index patients and their relatives is variable due to incomplete and age-dependent penetrance. Several biomarkers, electrocardiographic and imaging (echocardiographic deformation imaging and cardiac magnetic resonance imaging) techniques are promising non-invasive methods for detection of subclinical cardiomyopathy. However, these techniques need optimisation and integration into clinical practice. Furthermore, determining the optimal interval and intensity of cascade screening may require a personalised approach. To address this, the CVON-eDETECT (early detection of disease in cardiomyopathy mutation carriers) consortium aims to integrate electronic health record data from long-term follow-up, diagnostic data sets, tissue and plasma samples in a multidisciplinary biobank environment to provide personalised risk stratification for heart failure and sudden cardiac death. Adequate risk stratification may lead to personalised screening, treatment and optimal timing of implantable cardioverter defibrillator implantation. In this article, we describe non-invasive diagnostic techniques used for detection of subclinical disease in relatives of index patients with dilated cardiomyopathy and arrhythmogenic cardiomyopathy.

scholarly journals Familial Dilated Cardiomyopathy: Risk Stratification for Sudden Cardiac Death

2020 ◽  
Author(s):  
Gustav Mattsson ◽  
Peter Magnusson
Keyword(s):  
Heart Failure ◽  
Health Care ◽  
Sudden Cardiac Death ◽  
Risk Stratification ◽  
Dilated Cardiomyopathy ◽  
Health Care Providers ◽  
Cardiac Death ◽  
Reduced Ejection Fraction ◽  
Improve Risk Stratification ◽  
Familial Dilated Cardiomyopathy

Heart failure implies a considerable burden for patients and resources for the health care system. Dilated cardiomyopathy is defined as left ventricular dilation and reduced systolic function, not solely explained by ischemic heart disease or abnormal loading conditions. Numerous genes have been identified in familial cases of dilated cardiomyopathy. Heart failure with reduced ejection fraction increases the risk for sudden cardiac death. Implantable cardioverter defibrillator therapy can provide a means of preventing sudden cardiac death in those deemed to be at high risk. Health care providers are in need of better tools in order to improve risk stratification. This chapter aims to provide an overview of the current knowledge about risk of arrhythmia and sudden death in patients with familial dilated cardiomyopathy, in particular for those patients with a specific mutation.

scholarly journals SUDDEN CARDIAC DEATH IN PATIENTS WITH DILATED CARDIOMYOPATHY

2014 ◽  
Vol 5 (4) ◽  
pp. 41-48
Author(s):  
D V Duplyakov ◽  
I V Kondratyeva ◽  
S V Garkina
Keyword(s):  
High Risk ◽  
Sudden Cardiac Death ◽  
Risk Stratification ◽  
Dilated Cardiomyopathy ◽  
Cardiac Death ◽  
Clinical Manifestations ◽  
Scientific Data ◽  
National Guidelines ◽  
Causative Factors ◽  
Definition Of

The review discusses accumulated to date scientific data on dilated cardiomyopathy: causative factors, clinical manifestations, sudden cardiac death in patients with the disease and its risk-stratification in the light of National guidelines on the definition of risk and prevention of sudden cardiac death (2012). A case of a patient with dilated cardiomyopathy and high-risk of sudden cardiac death is presented.

Time trends in sudden cardiac death risk in heart failure patients with cardiac resynchronization therapy: a systematic review

2019 ◽  
Vol 41 (21) ◽  
pp. 1976-1986 ◽  
Author(s):  
Sérgio Barra ◽  
Rui Providência ◽  
Kumar Narayanan ◽  
Serge Boveda ◽  
Rudolf Duehmke ◽  
...  
Keyword(s):  
Heart Failure ◽  
Sudden Cardiac Death ◽  
Cardiac Resynchronization Therapy ◽  
Cardiac Death ◽  
Cardiac Resynchronization ◽  
Resynchronization Therapy ◽  
Heart Failure Patients ◽  
The Difference ◽  
Over Time

Abstract Aims While data from randomized trials suggest a declining incidence of sudden cardiac death (SCD) among heart failure patients, the extent to which such a trend is present among patients with cardiac resynchronization therapy (CRT) has not been evaluated. We therefore assessed changes in SCD incidence, and associated factors, in CRT recipients over the last 20 years. Methods and results Literature search from inception to 30 April 2018 for observational and randomized studies involving CRT patients, with or without defibrillator, providing specific cause-of-death data. Sudden cardiac death was the primary endpoint. For each study, rate of SCD per 1000 patient-years of follow-up was calculated. Trend line graphs were subsequently constructed to assess change in SCD rates over time, which were further analysed by device type, patient characteristics, and medical therapy. Fifty-three studies, comprising 22 351 patients with 60 879 patient-years of follow-up and a total of 585 SCD, were included. There was a gradual decrease in SCD rates since the early 2000s in both randomized and observational studies, with rates falling more than four-fold. The rate of decline in SCD was steeper than that of all-cause mortality, and accordingly, the proportion of deaths which were due to SCD declined over the years. The magnitude of absolute decline in SCD was more prominent among CRT-pacemaker (CRT-P) patients compared to those receiving CRT-defibrillator (CRT-D), with the difference in SCD rates between CRT-P and CRT-D decreasing considerably over time. There was a progressive increase in age, use of beta-blockers, and left ventricular ejection fraction, and conversely, a decrease in QRS duration and antiarrhythmic drug use. Conclusion Sudden cardiac death rates have progressively declined in the CRT heart failure population over time, with the difference between CRT-D vs. CRT-P recipients narrowing considerably.

P994Association of late gadolinium enhancement with prognosis and sudden cardiac death risk in non-ischemic dilated cardiomyopathy

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
I Rodriguez Sanchez ◽  
J J Onaindia ◽  
S Velasco ◽  
U Aguirre ◽  
A Capelastegui ◽  
...  
Keyword(s):  
Heart Failure ◽  
Late Gadolinium Enhancement ◽  
Sudden Cardiac Death ◽  
Dilated Cardiomyopathy ◽  
Cardiovascular Mortality ◽  
Cardiac Death ◽  
Composite Endpoint ◽  
Heart Failure Hospitalization ◽  
Gadolinium Enhancement ◽  
Non Ischemic Dilated Cardiomyopathy

Abstract Objectives to evaluate the association between late gadolinium enhancement (LGE) on cardiac magnetic resonance imaging (cMRI) and prognosis in patients with non-ischemic dilated cardiomyopathy (NIDM). Background Risk stratification in NIDM needs to be improved. Methods A total of 210 patients with NIDM and cMRI from 2005 to 2018 were included in our population. Outcomes were retrospectively assessed by medical records. The pattern of LGE was classified as midwall, subepicardial, or both patterns. Primary endpoint was sudden cardiac death (SCD) and aborted SCD. Secondary endpoints were global mortality and a composite endpoint of cardiovascular mortality and heart failure hospitalization. Demographic and clinical parameters were also evaluated. Patients with LGE (LGE+) were more likely to be male (80,6% vs 66,7%, p=0,03). No significant differences were observed between LGE+ and LGE− patients in comorbidities, NYHA class, left ventricular ejection fraction (LVEF), left bundle branch block or neurohormonal treatment. Results Of 210 patients (71,4% men, median age 59,8 years) with a median follow up of 5,6 years (3,24–8,15), 72 patients (34,3%) had non ischemic LGE (LGE+). Mean LVEF was 34%. SCD or aborted SCD occurred in 11 patients (5,2%). 6 patients (9,5%) with LGE+ reached the primary endpoint vs 5 (4,07%) of LGE− patients (p=0,19). The adjusted OR for the presence of LGE in the composite endpoint (cardiovascular mortality and heart failure hospitalization) was 2,45, confidence interval (CI): 1,16–5,17, (p=0,02). LGE presence was not associated with global mortality. The subepicardial pattern of LGE was associated with SCD and aborted SCD: 3 out of 11 patients (27, 1%) with subepicardial pattern of LGE suffered from SCD or aborted SCD (p=0,02). Conclusions In our cohort of 210 patients with NIDM, LGE presence was not associated with SCD and aborted SCD, probably because of low event rate in a relatively small population. However, LGE presence was associated with the composite endpoint of cardiovascular mortality and heart failure hospitalization. The subepicardial pattern of LGE identified patients at high risk of SCD and aborted SCD.

scholarly journals Hypertrophic cardiomyopathy: Translating cellular cross talk into therapeutics

2012 ◽  
Vol 199 (3) ◽  
pp. 417-421 ◽  
Author(s):  
Polakit Teekakirikul ◽  
Robert F. Padera ◽  
J.G. Seidman ◽  
Christine E. Seidman
Keyword(s):  
Heart Failure ◽  
Heart Disease ◽  
Hypertrophic Cardiomyopathy ◽  
Sudden Cardiac Death ◽  
Mouse Models ◽  
Clinical Diagnosis ◽  
Cardiac Death ◽  
Clinical Manifestations ◽  
Biochemical Properties ◽  
Adverse Outcomes

Hypertrophic cardiomyopathy (HCM) is a common inherited heart disease with serious adverse outcomes, including heart failure, arrhythmias, and sudden cardiac death. The discovery that mutations in sarcomere protein genes cause HCM has enabled the development of mouse models that recapitulate clinical manifestations of disease. Studies in these models have provided unexpected insights into the biophysical and biochemical properties of mutated contractile proteins and may help to improve clinical diagnosis and management of patients with HCM.

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