CHAPTER 18 Myocardial Disease
Diseases of the myocardium are common and extend from primary forms (cardiomyopathies) to the secondary forms such as hypertensive heart disease, alcoholic cardiomyopathy, Takotsubo cardiomyopathy, and more rare forms of secondary myocardial disease such as muscular dystrophy cardiomyopathy or peripartum cardiomyopathy. The primary forms are typically genetically transmitted, whereas secondary myocardial diseases are mostly acquired. Secondary forms may also have a genetic background, which favours the development of secondary myocardial disease. A special form of myocardial disease represents inflammatory diseases of the myocardium such as myocarditis or viral cardiomyopathy.A variant of the whole spectrum represents hypertrophic cardiomyopathy, which is—in contrast to other cardiomyopathies—associated with normal or supranormal myocardial function. The haemodynamic characteristics of this form of cardiomyopathy are: 1) the presence of systolic obstruction of the outflow tract; and 2) the existence of diastolic dysfunction. Most other forms show a reduction in systolic ejection performance (= systolic dysfunction).
Management of primary myocardial disease includes heart failure therapy in dilated cardiomyopathy, alcohol ablation of the septum in hypertrophic cardiomyopathy, defibrillator therapy in arrhythmogenic cardiomyopathy, immunomodulatory treatment in myocarditis or viral cardiomyopathy, and ultimately heart transplantation in end-stage myocardial disease.
Management of secondary myocardial disease is mainly linked to an identifiable source of myocardial dysfunction such as hypertension, diabetes, or excess alcohol consumption. A new form of secondary myocardial disease is Takotsubo cardiomyopathy, which shows a good prognosis over the short and mid-term follow-up and is thought to be due to excessive catecholamine exposure with coronary artery vasospasms.
Chapter 28)
