INSTRUCTION Examine this patient's cardiovascular system. SALIENT FEATURES HistoryPast history of hypertension, ischaemic heart disease or cardiomyopathy.Examination · Signs of fluid retention: raised jugular venous pressure, lung crepitations, pitting leg oedema, tender hepatomegaly. · Signs of impaired perfusion: cold clammy skin, low blood pressure. · Signs of ventricular dysfunction: displaced left ventricular apex, right ventricular heave, third or fourth heart sound, functional mitral or tricuspid regurgitation, tachycardia. Look for the aetiology: · Valvular disease. · Atherosclerotic vascular disease. · Severe hypertension. · Severe anaemia or volume overload, e.g. arteriovenous shunt. · Pathological arrhythmia. · Evidence of generalized myopathy or poisoning. DIAGNOSIS This patient has congestive cardiac failure due to hypertension and is severely limited with NYHA class IV dyspnoea. QUESTIONS How would you investigate this patient? · Chest radiography, echocardiogram, cardiopulmonary exercise testing to confirm diagnosis. · Exercise testing is useful to identify ischaemic heart disease. · Cardiopulmonary exercise testing is useful to determine functional capacity betore cardiac rehabilitation and to determine eligibility for cardiac transplantation. · ECG to look for underlying cause, e.g. ischaemia or infarction, left ventricular hypertrophy, arrhythmia, other causes of pathological Q waves. · Echocardiogram to detect valvular disease; determines whether left ventricle function is globally impaired (e.g. idiopathic dilated cardiomyopathy) or whether there are segmental wall motion abnormalities (e.g. in ischaemic heart disease). Ejection fraction can be estimated and usually treatment is initiated when ejection fraction is ?<40. Doppler echocardiography allows determination of diastolic dysfunction. · Blood tests for associated disease: renal, liver and electrolyte disturbances com-mon; for metabolic causes, e.g. haemochromatosis, hypocalcaemic cardiomyo-pathy, thyroid heart disease, anaemia, heavy metal poisons, amyloid (serum electrophoresis, rectal biopsy), sarcoid (serum angiotensin converting enzyme). · Coronary angiography to identify ischaemic heart disease. · Ventricular biopsy for specific myocarditis, especially viral, and to exclude infiltrative diseases such as cardiac sarcoidosis and amyloidosis. · Radionuclide ventriculography or echocardiography: to quantitate severity of systolic dysfunction (ejection fraction). · 24-Holter ECG monitoring: for ventricular arrhythmias. What is the pharmacological treatment of left ventricular systolic dysfunction ? · Diuretics for symptomatic patients to maintain appropriate fluid balance. · For patients with systolic dysfunction (EF <40%) who have no contraindications: -ACE inhibitors for all patients. - Beta-blockers for all patients except those who are haemodynamically unstable or who are intolerant. - Spironolactone for patients with rest dyspnoea or with a history of rest dypnoea. -Digoxin both for patients who remain symptomatic despite diuretics, ACE inhibitors and beta-blockers and patients with rest dyspnoea or who have a history of rest dyspnoea. ADVANCED-LEVEL QUESTIONS What is diastolic dysfunction? It is excessive stiffness of the heart resulting in an inability of the heart to fill properly (Eur Heart J 1998; 19: 990-1003). This is in contrast to systolic dys-function, where contractility is impaired. Patients have clinical features of left heart failure but normal systolic function by echocardiography or radionuclide ven-triculography. It is a feature of hypertrophic cardiomyopathy, severe left ventricular hypertrophy (e.g. aortic stenosis or hypertension) and restrictive cardiomyopathy (e.g. amyloidosis). Treatment is directed towards the underlying cause. What are the indications for heart transplantation? When patients are refractory to treatment, both medical and surgical (such as valve replacement), and are in New York Heart Association class IV, then they are unlikely to survive for I year and should be considered for heart transplantation. The survival rate is about 69c)~ at 5 years, although most patients have one episode of rejection and 25% have multiple episodes. They are also prone to accelerated coronary atherosclerosis. In 1967, Christiaan Barnard, a South African surgeon, was the first to perform cardiac transplantation in humans. Sir Magdi Yacoub, contemporary Professor of Cardiology at University of London and Royal Brompton Hospital and Harefield Hospital is an Egyptian-born surgeon who performed several pioneering cardiac operations. R. Sanders Williams, MD, Professor of Medicine and contemporary Dean, Duke University Medical School and Vice Chancellor, Duke University, has worked successively at Duke, Harvard, Oxford, and UT Southwestern in Dallas. He has made major contributions to the understanding of molecular mechanisms of cardiac function.