Diseases title

Tako-Tsubo cardiomyopathy )

Orpha Number: 66529
Ampulla cardiomyopathy, Apical ballooning syndrome, Ballooning cardiomyopathy, Stress cardiomyopathy, Tako-tsubo syndrome, Takotsubo cardiomyopathy, Takotsubo syndrome, Transient left ventricular apical ballooning syndrome

Takotsubo cardiomyopathy (TC) is a recently described acute cardiac syndrome that mimics acute myocardial infarction and is characterized by ischemic chest symptoms, an elevated ST segment on electrocardiogram, and elevated levels of cardiac disease markers. The syndrome was initially recognized in the Japanese population, but has recently been reported in the USA and Europe. It occurs most commonly in postmenopausal women between the ages of 55-75, with an estimated incidence in the general population of 1 in 36,000. Patients commonly present with ischemic-like chest pain or dyspnea, elevation of the ST segments and prolongation of the QT interval on electrocardiography, minor-to-moderate elevation of cardiac enzymes and biomarker levels, and transient 'ballooning' of the left ventricular apex and mid-ventricle. In contrast to the acute coronary arterial syndromes (ACS), patients with TC have no angiographically detectable or nonobstructive coronary arterial disease. The most common complications described are cardiogenic shock and obstruction of the left ventricular outflow tract, stroke, and formation of apical thrombus. Although the exact cause remains unknown, the syndrome is typically precipitated by acute emotional or physical stress, including a number of non-cardiac illnesses and surgery. Diagnosis is made by coronary arteriography, left ventriculography, and echocardiography. Differential diagnosis includes ACS, and other types of transient ventricular dysfunction. Patients should be monitored and treated in the hospital for atrial and ventricular arrhythmias, cardiac failure, and mechanical complications. The management consists of supportive and symptomatic therapy, such as administration of beta-blockers, angiotensin-converting enzyme inhibitors, aspirin, and diuretics. The short-term outcome is characterized by an excellent clinical recovery within a few weeks, when appropriate measures are taken during the acute phase of the illness. The evolution, although mainly uneventful, can be complicated by left ventricular rupture, thus making the syndrome a newly recognized cause of sudden death.
source: Orphanet