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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.

Acute Right Heart Failure and Achalasia-like Syndrome in a Patient with Limited Cutaneous Systemic Sclerosis and Primary Biliary Cirrhosis