Right ventricular dilated cardiomyopathy associated with primary biliary cirrhosis.

A case of right ventricular dilated cardiomyopathy associated with primary biliary cirrhosis is described. The patient was a middle aged woman, who initially complained of fatigue and itching. The diagnosis of primary biliary cirrhosis was made based on clinical, biochemical and histological evidence of the disease. Seven years later severe right-sided heart failure developed. The diagnosis of right ventricular dilated cardiomyopathy was made based on echocardiographic and angiographic evidence of a globally dilated and poorly contracting right ventricle. Left ventricular function was within normal limits. Autoimmune serology screening at this time revealed the presence of organ-specific cardiac antibody (titre 1/20) and of antinuclear antibody (titre 1/80) by indirect immunofluorescence. There were no findings of mitochondrial antibody or other non-organ specific or organ-specific antibodies. Overall, this assessment demonstrates autoimmunity in both hepatic and heart muscle disease in a patient with primary biliary cirrhosis and right ventricular dilated cardiomyopathy.

Echocardiographically revealed collapse of the right ventricle and prolonged inversion of both atria without clinical cardiac tamponade.

In a patient with a large pericardial effusion without any clinical signs of cardiac tamponade, echocardiography revealed collapse of the right ventricle and prolonged (greater than 34%) inversion of the free wall of both atria, maximal in early systole. The presence of such findings in the absence of clinical signs of haemodynamic compromise is discussed.

Treadmill stress testing in the evaluation of patients with valvular heart disease. Possible role in the assessment of functional capacity and severity of the lesion.

A series of 123 patients with valvular heart disease underwent treadmill stress testing (Bruce's protocol). Neither mortality nor serious complications were observed. The incidence of exercise-induced ventricular ectopy was 35% and that of complex ventricular ectopy 19%. Patients with pure left ventricular volume overload had a significantly higher incidence of exercise-induced ventricular ectopy than did patients with other valvular diseases. A significant relation was found between functional class (NYHA) and either duration of exertion or functional aerobic impairment. The NYHA classification was found to coincide in 62% of the instances with another based on the results obtained from stress testing. In patients with mitral stenosis a significant although slight correlation was found to exist between mitral valve area and duration of exercise. The inability to reach stage III of Bruce's protocol was associated with a critical mitral stenosis. A hypotensive response to exercise was frequent both in mitral stenosis and aortic stenosis. But whereas in aortic stenosis it was characteristic of the most severe lesions, in mitral stenosis it bore no relation whatsoever to severity. Thus, treadmill stress testing in valvular heart disease is safe, can be helpful in quantifying functional disability and may provide a clue as to the severity of the lesion in specific subsets.