dilated cardiomyopathy.pptx

Dcm

1. Dilated cardiomyopathy Presenter:- Dr Abdirisaq Jacda IMR1 moderator :- Dr A.Hakin (internist ) AU-HRU Department of Internal Medicine 7 August 2023 1 Dr Abdirisaq Jacda

2. Outline Introduction Definition Epidemiology etiology and risk factors Approach Management plan Prognosis References 7 August 2023 2 Dr Abdirisaq Jacda

3. cardiomyopathy  1980, WHO) defined cardiomyopathies as "heart muscle diseases of unknown cause" to distinguish cardiomyopathy from cardiac dysfunction due to known cardiovascular entities such as hypertension, ischemic heart disease, or valvular disease 1995 WHO/International Society and Federation of Cardiology (ISFC) all diseases affecting heart muscle and to take into consideration etiology as well as the dominant pathophysiology A 2006 AHA "Cardiomyopathies are a heterogeneous group of diseases of the myocardium associated with mechanical and/or electrical dysfunction that usually (but not invariably) exhibit inappropriate ventricular hypertrophy or dilation and are due to a variety of causes that frequently are genetic. Cardiomyopathies either are confined to the heart or are a part of generalized systemic disorders, often leading to cardiovascular death or progressive heart failure-related disability." 7 August 2023 3 Dr Abdirisaq Jacda

4. Latest AHA 2007 and UpToDate definition A myocardial disorder in which the heart muscle is structurally and functionally abnormal in the absence CAD HTN VHD CHD sufficient to explain the observed myocardial abnormality 7 August 2023 4 Dr Abdirisaq Jacda

5. Epidemiology Cardiomyopathy is disease of the heart muscle. It is estimated that cardiomyopathy accounts for 5–10% of the 5–6 million patients already diagnosed with heart failure in the United States This term is intended to exclude cardiac dysfunction that results from other structural heart disease, such as coronary artery disease, primary valve disease, or severe hypertension 7 August 2023 Dr Abdirisaq Jacda 5

6. They were classified according to anatomy and physiology into the following types, each of which has multiple different causes: Dilated cardiomyopathy (DCM) Hypertrophic cardiomyopathy (HCM) Restrictive cardiomyopathy (RCM) Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) Unclassified cardiomyopathies 6 CLASIFICATION 7 August 2023 Dr Abdirisaq Jacda 6

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9. Dilated cardiomyopathy (DCM) 7 August 2023 Dr Abdirisaq Jacda 9

10. Dilated cardiomyopathies (DCM) DCM is characterized by dilation and impaired contraction of one or both ventricles. The dilation often becomes severe and is invariably accompanied by an increase in total cardiac mass (hypertrophy). Affected patients have impaired systolic function and clinical presentation is usually with features of HF. 10 7 August 2023 Dr Abdirisaq Jacda

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12. Dilated cardiomyopath y (DCM) The wall becomes thin and stretched, compromising cardiac contractility thus poor left ventricular function(systolic dysfunction). Usually all chambers are enlarged Most common cardiomyopathy in the world Genetic causes and acquired Childhood /fourth and fifth decades of life. 12 7 August 2023 Dr Abdirisaq Jacda

13. DCM Progressive heart failure and Decline in LV contractile function, ventricular supraventricular arrhythmias, conduction system abnormalities, thromboembolism, sudden or heart failure–related death. 7 August 2023 Dr Abdirisaq Jacda 13

14. 14 Epidemiology One of the most common causes of heart failure and the most common indication for heart transplantation worldwide . The incidence of DCM has been estimated to be 5-8 per 100,000 population, with a prevalence of 36 per 100,000. Evaluation with ECG and echocardiogram of 767 relatives of 189 probands with DCM revealed unrecognized disease in 4.6 percent of relatives. 7 August 2023 Dr Abdirisaq Jacda

15. prognosis Most common Couse of heart transplant 1/3 spontaneous recovery Many with chronic DCM improve drastically with medication to near normal LV function 7 August 2023 Dr Abdirisaq Jacda 15

16. Pathophysiology of DCM 7 August 2023 Dr Abdirisaq Jacda 16

17. Etiology of Dilat e d Cardiomyopathy 17 7 August 2023 Dr Abdirisaq Jacda

18. Etiology Can be primary or secondary Secondary Inflammatory Toxins Metabolic Structural Familial and hereditary Idiopathic Miscellaneous 7 August 2023 Dr Abdirisaq Jacda 18

19. Common causes special points Familial account for 1/3 of the causes of DCM Extensive family-based studies have shown that if clinical screening with an electrocardiogram (ECG) and/or echocardiogram is conducted in the first-degree family members of patients with DCM, evidence of DCM will be found in at least 20% to 35% of them , thereby establishing a diagnosis of familial DCM. Recent studies in families with familial DCM suggest that a genetic cause can be identified in at least 30%-35% of cases and some recent suggest as high as 40% to 50% as extrapolated from studies of individual genes or small numbers of genes in gene discovery publications but not published yet due to small scale of those studies 7 August 2023 Dr Abdirisaq Jacda 19

20. Alcohol Alcohol is The most common toxin implicated in Chronic DCM, may contribute to more than 10% of total cases but more percentage in developed world Toxicity is attributed both to alcohol and to its primary metabolite acetaldehyde. The alcohol consumption necessary to produce cardiomyopathy in an otherwise normal heart has been estimated to be six drinks (about 4 ounces of pure ethanol) daily for 5–10 years Even with severe disease, marked improvement can occur within 3–6 months of abstinence. 7 August 2023 Dr Abdirisaq Jacda 20

21. Viral infections In humans, viruses are often suspected but rarely confirmed as the direct cause of myocarditis. Often implicated RNA viruses: Coxsackie, echovirus, poliovirus, Influenza viruses DNA viruses: adenovirus, variola (smallpox) and vaccinia (smallpox vaccine), and the herpesviruses (Varicella zoster, Cytomegalovirus, and Epstein-Barr virus) From genetic analyses of biopsy tissue, parvovirus B19, coxsackie, adenovirus, and Epstein-Barr virus are the agents most often implicated. Cases with HIV DCM increased last decade Other implicated vital illnesses are HCV 7 August 2023 Dr Abdirisaq Jacda 21

22. metabolic The most common endocrine courses and long standing uncontrolled diabetes Particularly genetically predisposed individuals Type 2 is more common than type 1 Other endocrine causes Thyroid cardiac disease Both hyperthyroid and hypothyroidism others acromegaly Pheochromocytoma Nutritional like thiamine deficiency causing wet or dry beriberi 7 August 2023 Dr Abdirisaq Jacda 22

23. autoimmune SLE Sarcoidosis RA Sjogren syndrome 7 August 2023 Dr Abdirisaq Jacda 23

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25. ABOUT _______________of new onset cardiomyopathy have spontaneous full recovery Half 1/3 2/3 Less than 5 percent 7 August 2023 Dr Abdirisaq Jacda 25

26. Approach Detailed history Typical case is 20-45 years old with heart failure symptoms Body edema SOB orthopnea PND Fever if preceded by viral or other infections Heart failure in cases with no ischemic heart disease HTN DM dislipedemia smoking Initially may presents with complications of DCM like arrythmias from wall stretching Palpitation SOB syncopy May present with thromboemboli , mesenteric ischemia, stroke, leg cloudication , subclavian syndrome Acquire FMhx sudden cardiac death before the age of 30 years. Ask about lcohol ingestion for how long how much 7 August 2023 Dr Abdirisaq Jacda 26

27. Physical examination Examination findings Displaced apical beat Biventricular heave Pansystolic murmur at apex Features of systolic heart failure Edema Ascites ↑JVP Rales 7 August 2023 Dr Abdirisaq Jacda 27

28. Basic work up Investigtion dependimg on presentation along symptoms of heart failure E.g. patients with hx painless chancre urethral discharge you should send VDRL or treponema specific antibody Patients with parotid swelling, orchitis pancreatitis you should evaluate for mumps And so on 7 August 2023 Dr Abdirisaq Jacda 28

29. Basic work up CBC ESR VIRAL MARKERS FOR COMMON VIRAL INFECTIONS DEPENDING ON SUSPECTING ETIOLOGY RF, ANA ANTI TA ANTI LA VDRL, PRL ECG ECHO CXR 7 August 2023 Dr Abdirisaq Jacda 29

30. Electrocardiography frequently reveals LV hypertrophy (LVH), nonspecific ST-T wave changes, or bundle branch block . Pathologic Q waves may be present, AND ALL CHAMPER ENLARMENT 7 August 2023 Dr Abdirisaq Jacda 30

31. ECHOCARDIOGRAPHY Echocardiography reveals biventricular dilation, which can range from mild to severe, as can LV systolic dysfunction LV wall thickness is usually within the normal range, Most commonly, global LV hypokinesis is present, regional wall motion abnormalities may also be seen, particularly septal dyskinesis in those with left bundle branch block. Disproportionate thinning of a dyskinetic wall should raise the possibility of coronary artery disease rather than primary cardiomyopathy. 7 August 2023 Dr Abdirisaq Jacda 31

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34. CXR-Cardiomegaly +other HF features Cephalization Pleural effusion\ Cardiomegaly Pulmonary edema CXR 34

35. Management Its proven to give OGDMT of HFrEF or systolic dysfunction 8 August 2023 Dr Abdirisaq Jacda 35

36. CARDIAC RESYNCHRONIZATION THERAPY Nonsynchronous contraction between the walls of the left ventricle ( intraventricular ) or between the ventricular chambers ( interventricular ) impairs systolic function, decreases mechanical efficiency of contraction, and adversely affects ventricular filling. Mechanical dyssynchrony results in an increase in wall stress and worsens functional mitral regurgitation. The single most important association of extent of dyssynchrony is a widened QRS interval on the surface ECG, particularly in the presence of a LBBB pattern. 36

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38. Treat specific cause if identified 8 August 2023 Dr Abdirisaq Jacda 38

39. REFERENCE Harrison 21th Edition UpToDate 2023 NATIONAL INSITUTE OF HEALTH WHO AHA/ACA 7 August 2023 39 Dr Abdirisaq Jacda