Diagnostic approach of valvular myocardial and pericardial disease

1. Diagnostic approach of valvular myocardial and pericardial disease AmirrezaSajjadieh Interventional Cardiologist 1392/11/13

2. Myocardial and Pericardial Diseases

3. Outlines: • Introducation • Myocardial diseases: • - Myocarditis • - Cardiomyopathy : 4 major types : 1. Dilated. 2. Hypertrophic. 3. Restrictive. 4. Arrhythmogenic right venticular. • Pericardial diseas: • acute pericarditis • pericardial effusion and cardiac tamponade • constrictive pericarditis.

4. Myocardial Diseases

5. Introduction Of Myocardial Diseases: • Although the myocardium is involved in most types of heart disease, the term myocarditis and cardiomyopathy are usually reserved for conditions that primerly affect the heart muscle. • So we are discussing today Myocardial diseases that is not due to ischaemic, valvular or hypertensive heart disease or a known infiltrative, metabolic/toxic or neuromuscular disorder

6. Myocardial diseases may be caused by: 1. an acute or chronic inflammatory pathology (myocarditis) 2. idiopathic myocardial disease (cardiomyopathy).

8. Myocarditis • Acute inflammation of the myocardium. • It has many causes: • Idiopathic • Infective • Viral: Coxsackievirus, adenovirus, CMV, echovirus, influenza, polio, hepatitis, HIV. • Parasitic: Trypanosoma cruzi, Toxoplasma gondii (a cause of myocarditis in the newborn or immunocompromised) • Bacterial: Streptococcus (most commonly rheumatic carditis), diphtheria (toxin-mediated heart block common)

9. Cont.causes: • Spirochaetal: Lyme disease (heart block common),leptospirosis. • Fungal. • Rickettsial. • Toxic. • Drugs: Causing hypersensitivity reactions, e.g. methyldopa, penicillin, sulphonamides, antituberculous,modafinil. • Radiation: May cause myocarditis but pericarditis more common. • Autoimmune:An autoimmune form with autoactivated T cells and organ specific antibodies may occur.

10. Pathology: • In the acute phase myocarditic hearts are flabby with focal haemorrhages; in chronic cases they are enlarged and hypertrophied. • Histologically an inflammatory infiltrate is present. • lymphocytes predominating in viral causes. • Polymorphonuclear cells in bacterial causes. • eosinophilsin allergic and hypersensitivity causes.

11. Clinical features: • Myocarditis may be an acute or chronic process. • its clinical presentations range from: • an asymptomatic state associated with limited and focal inflammation. • fatigue, palpitations, chest pain, dyspnoeaand fulminant congestive cardiac failure due to diffuse myocardial involvement.

12. Physical examination: • includes soft heart sounds. • a prominent third sound. • often a tachycardia. • A pericardial friction rub may be heard.

13. Investigations: ■ Chest X-ray may show some cardiac enlargement. ■ ECG demonstrates ST- and T wave abnormalities and arrhythmias. ■ Cardiac enzymes are elevated. ■ Viral antibody titres may be increased. However, since enteroviralinfection is common in the general population, the diagnosis depends on the demonstration of acutely rising titres. ■ Endomyocardial biopsy may show acute inflammation but false negatives are common by conventional criteria. Biopsy is of limited value outside specialized units. ■ Viral RNA can be measured from biopsy material using polymerase chain reaction (PCR). Specific diagnosis requires demonstration of active viral replication within myocardial tissue.

14. CARDIOMYOPATHY • Cardiomyopathies are a group of diseases of the myocardium that affect the mechanical or electrical function of the heart. • They are not secondary to coronary artery diseases, hypertension, or congenital, valvular or pericardial abnormalities. • They are frequently genetic and may produce inappropriate ventricular hypertrophy or dilatation and can be primarily a cardiac disorder or part of a multi-system disease.

15. Four main types: 1. Dilated. 2. Hypertrophic. 3. Restrictive. 4. Arrhythmogenic right venticular.

16. Dilated cardiomyopathy (DCM): • is characterized by dilatation of the ventricular chambers and systolic dysfunction with preserved wall thickness. • In about 25% of the patients it is a familial disease. • Sporadic DCM can be caused by multiple conditions: ■ myocarditis – Coxsackie, adenoviruses, erythroviruses, HIV, bacteria, fungae, mycobacteria, parasitic (Chagas’ disease) ■ toxins – alcohol, chemotherapy, metals (cobalt, lead, mercury, arsenic) ■ autoimmune. ■ endocrine. ■ neuromuscular.

17. Clinical features: • DCM can present with: • heart failure. • cardiac arrhythmias. • conduction defects. • thromboembolism. • sudden death. • Increasingly, evaluation of relatives of DCM patients is allowing identification of early asymptomatic disease, prior to the onset of these complications. • Clinical evaluation should include a family history and construction of a pedigree where appropriate.

18. Investigations: • ■ Chest X-ray demonstrates generalized cardiac enlargement. • ■ ECG may demonstrate diffuse non-specific ST segment and T wave changes. Sinus tachycardia, conduction abnormalities and arrhythmias are also seen. • ■ Echocardiogram reveals dilatation of the left and/or right ventricle with poor global contraction function. • ■ Cardiac MR may demonstrate other aetiologies of left ventricular dysfunction (e.g. previous myocardial infarction) or demonstrate abnormal myocardial fibrosis. Cardiac MR is also useful for identifying myocardial thrombus . • ■ Coronary angiography should be performed to exclude coronary artery disease in all individuals at risk (generally patients > 40 years or younger if symptoms or risk factors are present). • ■ Biopsy is generally not indicated outside specialist care.

19. 2. Hypertrophic cardiomyopathy (HCM) • It is characterized by marked vantricular hypertrophy in the absence of an alternate cause (e.g. aortic stenosis or hypertension). • Usually with disproportionate involvment of the interventricular septum. • The hypertrophic non-compliant vantricles impair diastolic filling, so that stroke volume is reduced. • Most cases are familial, autosomal dominant and caused by mutation in genes coding for proteins that regulate conraction, e.gtroponin and B-myosin.

20. Clinical features: Symptoms: • ■ many are asymptomatic and are detected through family screening of an affected individual or following a routine ECG examination. • ■ chest pain, dyspnoea, syncope or pre-syncope (typically with exertion), cardiac arrhythmias and sudden death are seen. • ■ sudden death occurs at any age but the highest rates (up to 6% per annum) occur in adolescents or young adults. ■ dyspnoea occurs due to impaired relaxation of the heart muscle or the left ventricular outflow tract obstruction that occurs in some patients. ■ If a patient develops atrialfibrillation there is often a rapid deterioration in clinical status due to the loss of atrial contraction and the tachycardia – resulting in elevated left atrialpressure and acute pulmonary oedema.

21. Signs: • ■ double apical pulsation (forceful atrialcontraction producing a fourth heart sound). • jerky carotid pulse because of rapid ejection and sudden obstruction to left ventricular outflow during systole • ■ ejection systolic murmur due to left ventricular outflow obstruction late in systole. • ■ pan-systolic murmur due to mitraregurgitation. • ■ fourth heart sound (if not in AF).

22. Investigations: • ■ ECG abnormalities of HCM include left ventricular hypertrophy, ST and T wave changes, and abnormal Q waves especially in the infero-lateral leads. • ■ Echocardiography is usually diagnostic and in classical HCM there is asymmetric left ventricular hypertrophy (involving the septum more than the posterior wall), systolic anterior motion of the mitral valve, and a vigorously contracting ventricle. • ■ Cardiac MR can detect both the hypertrophy but also abnormal myocardial fibrosis. • ■Genetic analysis, where available, may confirm the diagnosis and provide prognostic information for the patient and relatives.

23. Treatment: • The management of HCM includes treatment of symptoms and the prevention of sudden cardiac death in the patient and relatives. • Risk factors for sudden death: • ■ massive left ventricular hypertrophy (> 30 mm on echocardiography). • ■ family history of sudden cardiac death (< 50 years old). • ■ non-sustained ventricular tachycardia on 24-hour Holter monitoring. • ■ prior unexplained syncope. • ■ abnormal blood pressure response on exercise (flat or hypotensiveresponse).

24. patients with two or more risk factors should be assessed for implantable cardioverter- defibrillator (ICD). • In patients in whom the risk is less, amiodarone is an appropriate alternative. • Chest pain and dyspnoea are treated with B-blockers and verapamil. • Family members should be screened for evidence for evidence of disease by ECG and echocardiography.

25. Restrictive cardiomyopathy • In this rare condition, ventricular filling is impaired because the ventricles are 'stiff' . • This leads to  high atrial pressures with atrial hypertrophy  dilatation  later atrial fibrillation.

26. Restrictive cardiomyopathy Aetiology: • Amyloidosis is the most common cause of restrictive cardiomyopathy in the UK. • other forms of infiltration (e.g. glycogen storage diseases), idiopathic perimyocyte fibrosis .

27. Restrictive cardiomyopathy Clinical features : • Dyspnoea. • fatigue . • embolic symptoms are the presenting features. NB : Restriction to ventricular filling (especially right) results in persistently elevated venous pressures, consequent hepatic enlargement, ascites, and dependent oedema.

28. Restrictive cardiomyopathy Investigations: • Chest X-ray: may show pulmonary venous congestion. The heart can be normal or show cardiomegaly and/or atrial enlargement. • ECG usually has low-voltage and ST segment and T wave abnormalities. • Echocardiogram shows symmetrical myocardial thickening and often a normal systolic ejection fraction, but impaired ventricular filling. • Cardiac catheterization and haemodynamic studies help distinction from constrictive pericarditis. • Endomyocardial biopsy in contrast with other cardiomyopathies is often useful in this condition and may permit a specific diagnosis such as amyloidosis to be made.

29. PERICARDIAL DISEASES

30. Pericardial disease

31. The pericardium acts as a protective covering for the heart. It consists of two separate layers, the inner visceral pericardium and the outer parietal pericardium. The visceral pericardium reflects back upon it self at the level of the great vessels to join the parietal pericardium, thus forming a sac. The pericardial sac contains up to 50 mLof pericardial fluid in the normal heart, although this is a potential space for fluid to collect. The pericardium serves to lubricate the surface of the heart, prevents deformation and dislocation of the heart and acts as a barrier to the spread of infection.

32. Presentations of pericardial disease include: acute pericarditis. apericardial effusion and cardiac tamponade constrictive pericarditis.

33. 1-acute pericarditis This refers to inflammation of the pericardium. Classically, fibrinous material is deposited into the pericardial space and pericardial effusion often occurs. Acute pericarditis has numerous aetiologies Most commonly in the UK, it is due to viral infection and myocardial infarction, although in many cases the cause is unknown.

34. Type • Viral pericarditis:The most common viral causes are Coxsackie B virus and echovirus. Viral pericarditis is usually painful but has a short time course and rarely long-term effects. • Post-myocardial infarction pericarditis:occurs in about 20% of patients in the first few days following MI.. It may be difficult to differentiate this pain from recurrent angina when it occurs early (day 1-2 post- infarct) but a good history of the pain and serial ECG monitoring is helpful. • Pericarditis may also occur later on in the recovery phase after infarction.

35. Uraemic pericarditis:is due to irritation of the pericardium by accumulating toxins. It can occur in 6- 10% of patients with advanced renal failure if dialysis is delayed. • Bacterial pericarditis :may rarely occur with septicaemia or pneumonia or it may stem from an early postoperative infection after thoracic surgery or trauma or may complicate endocarditis. • Staphylococcus aureusis a frequent cause of purulent pericarditis in HIV patients. This form of pericarditis, especially staphylococcal, is fulminant and often fatal. • Other endemic infectious pericarditis includes: mycoplasmosis and Lyme pericarditis which are often effusive and require pericardial drainage. The diagnosis is based on serological tests of pericardial fluid and identification of organisms in pericardial or myocardial biopsies.

36. Tuberculous pericarditis:usually presents with chronic low-grade fever, parti cularly in the evening, associated with features of acute pericarditis, dyspnoea, malaise, night sweats and weight loss. • Fungal pericarditis:is a common complication of endemic fungal infections, such as histoplasmosis and coccidioidomycosis but may be also caused by Candida albicans, especially in immunocompromised patients, drug addicts or after cardiac surgery.

37. Malignant pericarditis. :Carcinoma of the bronchus, • carcinoma of the breast and Hodgkin's lymphoma are the most common causes of malignant pericarditis. Leukaemia and malignant melanoma are also associated with pericarditis.

38. etiology Infectious pericarditis Viral (Coxsackievirus, echovirus, mumps, herpes, HIV) Bacterial (staphylococcus, streptococcus, pneumococcus, meningococcus, Haemophilu influenzae, mycoplasmosis, borreliosis, Chlamydia) Tuberculous Fungal (histoplasmosis, coccidioidomycosis, Candida) II. Post-myocardial infarction pericarditis Acute myocardial infarction (early) Dressler's syndrome (late) III. Malignant pericarditis Primary tumours of the heart (mesothelioma) Metastatic pericarditis (breast and lung carcinoma, lymphoma, leukaemia, melanoma)

39. IV. Uraemic pericarditis • V. Myxoedematous pericarditis • VI. Chylopericardium • VII. Autoimmune pericarditis Collagen-vascular (rheumatoid arthritis, rheumatic fever, systemic lupus erythematosus, scleroderma) Drug-induced (procainamide, hydralazine, isoniazid, doxorubicin, cyclophosphamides) • VIII. Post-radiation pericarditis • IX. Post-surgical pericarditis Postpericardiotomy syndrome • X. Post-traumatic pericarditis • XI. Familial and idiopathic pericarditis

40. Clinical features Pericardial inflammation produces sharp central chest pain exacerbated by movement, respiration and lyingdown. It is typically relieved by sitting forward. It may be referred to the neck or shoulders. The main differential diagnoses are angina and pleurisy.

41. The classical clinical sign is • a pericardial friction rub occurring in three phases corresponding to atrial systole, ventricular systole and ventricular diastole. It may also be heard as a biphasic 'to and fro' rub. • The rub is heard best with the diaphragm of the stethoscope at the lower left sternal edge at the end of expiration with the patient leaning forward. • There is usually a fever, leucocytosis or lymphocytosis when pericarditis is due to viral or bacterial infection, rheumatic fever or myocardial infarction. • Large pericardial effusion can compress adjacent bronchi and lung tissue and may cause dyspnoea.

42. Investigations • ECG is diagnostic. There is concave-upwards (saddle-shaped) • ST elevation .These changes evolve over time, with resolution of the ST elevation, T wave flattening/inversion and finally T wave normalization. • The early ECG changes must be differentiated from ST • elevation found in myocardial infarction . • Sinus tachycardia may result from fever or haemodynamic • embarrassment, and rhythm and conduction • abnormalities may be present if myocardium is involved. • Cardiac enzymes should be assayed as they may be • elevated if there is associated myocarditis • Chest X ray, echocardiograms and radionucleotide • scans are of little value in un complicated acute • pericarditis.

43. ECGs associated with pericarditis.(a) Acute pericarditis. Note the raised ST segment, concaveupwards (arrow), (b) Chronic phase of pericarditis :associated with a pericardial effusion. Note the T wave flattening and inversion and the alternation of the QRSamplitude (QRS alternans). (c) The same patient after evacuation of the pericardial fluid. Note that the QRS voltage has increased and the T waves have returned to normal.

44. 2-Pericardial effusion and cardiactamponade

45. A pericardial effusion is a collection of fluid within the potential space of the serous pericardial sac as in the figure • commonly accompanying an episode of acute pericarditis. • When a large volume collects in this space, ventricular filling is compromised leading to embarrassment of the circulation. This is known as cardiac tamponade.

46. Chest X-ray showing a pericardial effusion,the heart appears globular.

47. Chest X-ray showing a peric ardial calcification (arrow).

48. Valvular Heart Disease

49. Types • Mitral Stenosis • Mitral Regurgitation • Mitral Valve Prolapse • Aortic Stenosis • Aortic regurgitation • Tricuspid valve is affected infrequently • Tricuspid stenosis – causes Rt HF • Tricuspid regurgitation –causes venous overload

50. Rheumatic Heart Disease • Inflammatory process that may affect the myocardium, pericardium and or endocardium • Usually results in distortion and scarring of the valves