Cardiomyopathy, Myocarditis and Pericarditis Flashcards
<p>What are the different types of cardiomyopathy?</p>
<p>Hypertrophic</p>
<p>Dilated</p>
<p>Restrictive</p>
<p>Myocarditis</p>
<p></p>
<p>What are thedifferent types of pericardial disease?</p>
<p>Pericarditis and effusion (with or without tamponade)</p>
<p>What is dilated cardiomyopathy?</p>
<p>A condition when the heart becomes enlarged and cannot pump blood efficiently, affects other body systems such as the lungs, liver.</p>
<p>Why does dilated cardiomyopathy result in ventricular remodelling?</p>
<p>Left or right systolic pump is impairedleading to progressiveheart enlargementviaventricular hypertrophyandventricular dilation,</p>
<p>What is dilated cardiomyopathy often a cause of?</p>
<p>Congestive heart failure</p>
<p>Thrombosis in chambers is not uncommon</p>
<p>What are the possible genetic aetiological backgrounds for dilated cardiomyopathy?</p>
<p>Mutations in the SCN5A gene (a heart sodium channel gene)</p>
<p>Muscular dystrophy</p>
<p>What are the possible aetiological backgrounds for dilated cardiomyopathy? (besides genetic causes)</p>
<p>Pregnancy (post partum)</p>
<p>Chagas disease</p>
<p>Toxic insults, including drugs, chemicals and hormones (doxorubicin(Adriamycin), andcobalt are included)</p>
<p>Injury</p>
<p>Inflammatory response</p>
<p>Infection</p>
<p>Autoimmune disease</p>
<p>Chronic severe ventricular extrasystole</p>
<p>Fibrous change of the myocardium from a previousmyocardial infarction</p>
<p></p>
<p>Which chambers are affected in dilated cardiomyopathy?</p>
<p>Can be one but more often all chambers dilated and functionally impaired</p>
<p>What is the prognosis for dilated cardiomyopathy?</p>
<p>Some causes are specifically sought as they are reversible/part reversible but most are progressive and irreversible</p>
<p>What are the symptoms of dilated cardiomyopathy?</p>
<p>•Progressive, slow onset,</p>
<p>Dyspnoea</p>
<p>Fatigue</p>
<p>Orthopnoea</p>
<p>PND (paroxysmal nocturnal dyspnoea)</p>
<p>Ankle swelling</p>
<p>Weight gain of fluid overload</p>
<p>Cough.</p>
<p>What is a typical past medical history for someone with dilated cardiomyopathy?</p>
<p>Systemic illness</p>
<p>Travel</p>
<p>Hypertension</p>
<p>Vascular disease</p>
<p>Thyroid</p>
<p>Neuromuscular disease</p>
<p>What would you find on examination of someone with dilated cardiomyopathy?</p>
<p>Poor superficial perfusion</p>
<p>Thready pulse</p>
<p>Irregular pulse if in atrial fibrillation</p>
<p>SOB at rest</p>
<p>Narrow pulse pressure</p>
<p>JVP elevated</p>
<p>Displaced apex beat</p>
<p>S3 and S4 heart sounds</p>
<p>MR murmur</p>
<p>oedema (pulmonary, ankle and sacral)</p>
<p>Pleural effusions</p>
<p>Acites</p>
<p>Hepatomegaly</p>
<p>What are the relevant investigations for dilated cardiomyopathy?</p>
<p>Repeated <strong>ECG</strong> noting LBBB if present</p>
<p><strong>CXR</strong></p>
<p><strong>Basic bloods</strong></p>
<p>N terminal pro <strong>Brain Natriuretic peptide</strong></p>
<p>Basic bloods <strong>FBC</strong>, <strong>U + E</strong></p>
<p><strong>Echo</strong></p>
<p><strong>CMRI</strong> (best imaging modality)</p>
<p><strong>Coronary angiogram</strong></p>
<p>Sometimes <strong>biopsy</strong> depending on time course of cardiomyopathy</p>
<p>What is general measures in the treatmentof dilated cardiomyopathy?</p>
<p>•Correct anaemia</p>
<p>• Remove exacerbating drugs eg NSAIDs</p>
<p>• Correct any endocrine disturbance</p>
<p>• Advise on fluid and salt intake, reduce it</p>
<p>• Advise on managing weight to identify fluid overload</p>
<p>• HF nurse referral</p>
<p>What are the more specific measures in the treatment of dialted cardiomyopathy?</p>
<p><strong>ACEi</strong></p>
<p><strong>Angiotensin receptor blockers</strong></p>
<p><strong>Diuretics</strong></p>
<p><strong>Beta Blockers</strong></p>
<p><strong>Spironolactone</strong></p>
<p><strong>Anticoagulants as required</strong></p>
<p>Risk of sudden cardiac death is reduced with <strong>implantable cardioversion devices</strong> (small defibrillators) or <strong>Cariac resynchronisation therapy defibrillators</strong> (these devicesresynchronizes the contractions of the heart’s ventricles by sending tiny electrical impulses to the heart muscle, which can help the heart pump blood throughout the body more efficiently, they also have the function of a defibrillator)</p>
<p><strong>Cardiac Transplant</strong></p>
<p>What is restrictive cardiomyopathy?</p>
<p>Whenthe walls of the heart are rigidand the heart is restricted from stretching and filling with blood properly.</p>
<p>What are the non infilatrative forms of restrictive cardiomyopathy?</p>
<p>Familial</p>
<p>Forms of hypertrophic cardiomyopathy</p>
<p>Scleroderma (a chronic hardening and contraction of the skin and connective tissue, either locally or throughout the body - autoimmune disease)</p>
<p>Diabetic pseudoxanthoma elasticum (degeneration of elastic fibres within the body)</p>
<p>What are the infiltrative forms of restrictive cardiomyopathy?</p>
<p>Amyloidosis and sarcoidosis</p>
<p>What are the storage disease associated with restrictiva cardiomyopathy?</p>
<p><strong>Haemachromatosis</strong> (a hereditary disorder in which iron salts are deposited in the tissues, leading to liver damage, diabetes mellitus, and bronze discoloration of the skin.)</p>
<p><strong>Fabry disease</strong> (a rare genetic lysosomal storagedisease - alpha-galactosidase A deficiency)</p>
<p>What are theEndomyocardial conditions asoociated withRestrictivecardiomyopathy?</p>
<p>Fibrosis, carcinoid, radiation, drug effects</p>
<p>Why is the process of relaxation of the ventricular walls described as active rather than passive?</p>
<p>It needs functioning intact myocytes</p>
<p>What are the relevant investigations for restrictive cardiomyopathy?</p>
<div>•Repeated ECG noting LBBB if present and other conduction defects</div>
<div>• CXR</div>
<div>• N termial pro Brain Natriuetic Peptide</div>
<div>• Basic bloods FBC, U+E, be on the look out for sarcoid and haemachromatosis</div>
<div>• Auto antibodies for sclerotic CT diseases</div>
<div>• Amyloid needs non cardiac biopsy to help establish the diagnosis</div>
<div>• Fabry; low plasma alpha galactosidase A activity</div>
<div>• Echo</div>
<div>• CMRI, probably best imaging modality</div>
<p>Biopsy more helpful but still has high false negative rate</p>
<p>What is meant by amyloidosis?</p>
<p>Amyloidosisis a rare and serious disease caused by accumulation ofproteinsin the form of abnormal, insoluble fibres, known asamyloidfibrils, within theextracellular spacein the tissues of the body</p>
<p>What are the general measures for restrictive cardiomyopathy?</p>
<p>•Limited diuretic use as low filling pressures will cause problems</p>
<p>• Beta blockers limited ACEI use</p>
<p>• Anticoagulants as required</p>
<p>• SCD risk assessment with ICD or CRT-D/P implant</p>
<p>• Cardiac transplant</p>
<p>If iron overload, specific forms of amyloid or Fabrys then specific treatments are available</p>
<p>Endomyocardial fibrosis has little specific treatment</p>
<p><u>Prognosis</u></p>
<p>Unless reversible then poor prognosis</p>
<p>Define hypertrophic cardiomyopathy</p>
<p>Hypertrophic cardiomyopathy(HCM) is a disease in which a portion of themyocardium(heart muscle) ishypertrophic(enlarged) without any obvious cause, creating functional impairment of the heart.</p>
<p>Theleft ventricle(one of your heart's four chambers) is almost always affected, and in some people the muscle of the right ventricle also thickens.</p>
<p>What gene is responsible for hypertrophic cardiomyopathy?</p>
<p>Sarcomere gene</p>
<p>What is the inheritance pattern for hypertrophic cardiomyopathy?</p>
<p>Autosomal dominant but with variable expression and incomplete penetrance</p>
<p></p>
<p>50% chance of inheriting the gene but how it is expressed is not known until time passes</p>