Acute right heart failure and achalasia-like syndrome in a patient with limited cutaneous systemic sclerosis and primary biliary cirrhosis

Abstract

We report a case of a 63-year-old woman who developed acute right heart failure and an achalasia-like syndrome with limited cutaneous systemic sclerosis (lcSSc) and primary biliary cirrhosis. Intravenous administration of diuretics improved her acute heart failure. Anti-centromere antibodies and anti-mitochondria antibodies were present. A coronary angiogram and a Swan-Ganz catheter revealed no abnormalities. Thallium- 201 scan at rest demonstrated mild perfusion defects in both the apex and the anteroseptal and the inferior myocardium. A cine-esophagram revealed an achalasia-like syndrome. Though rare, physicians should be aware that some patients with lcSSc may develop acute right heart failure or achalasia-like syndrome. © 2010 The Japanese Society of Internal Medicine.