Acute right heart failure and achalasia-like syndrome in a patient with limited cutaneous systemic sclerosis and primary biliary cirrhosis.
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.
Nomoto
Takahiko
T
Department of Internal Medicine, Takanoko Hospital, Matsuyama, Japan.
Kikuchi
Takanori
T
Kusunoki
Yukiko
Y
Akesaka
Kazuyuki
K
Yano
Kazuo
K
Fujii
Yasuhisa
Y
Makino
Hideichi
H
D000208:Acute Disease; D004562:Electrocardiography; D004931:Esophageal Achalasia / Q000150:complications / Q000175:diagnosis* / Q000503:physiopathology; D005260:Female; D006333:Heart Failure / Q000150:complications / Q000175:diagnosis* / Q000503:physiopathology; D006801:Humans; D008105:Liver Cirrhosis, Biliary / Q000150:complications / Q000175:diagnosis* / Q000503:physiopathology; D008875:Middle Aged; D045745:Scleroderma, Limited / Q000150:complications / Q000175:diagnosis* / Q000503:physiopathology