A 33-year-old woman with cirrhosis and right ventricular failure

Article Abstract:

A33-year-old women died from cirrhosis of the liver and pulmonary hypertension. The patient was admitted to the hospital with liver and respiratory failure to be evaluated for liver and lung transplantation. Seven years earlier, her liver became enlarged, and she was diagnosed with sarcoidosis. She began experiencing weakness and shortness of breath five months before admission, and had two fainting episodes two months before admission. She drank one glass of alcohol per week, and had no history of hepatitis. Tests for hepatitis and HIV infection were negative. A computed tomographic (CT) scan of the abdomen revealed liver nodules and an enlarged spleen. A CT scan of the chest showed large amounts of fluid in the lungs, and lung function was abnormal. Six days after admission, she experienced extreme respiratory distress, and died after cardiac arrest. An autopsy revealed cirrhosis of the liver and severe pulmonary hypertension caused by the cirrhosis.

Liver cirrhosis

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A 46-year-old woman with dermatomyositis, increasing pulmonary insufficiency, and terminal right ventricular failure

Article Abstract:

A 46-year-old woman was admitted to the hospital with elevated blood pressure in her lungs, a condition called pulmonary hypertension. Sixteen months earlier she had developed muscle weakness and pain, a rash, and shortness of breath. X-rays revealed that she had congestive heart failure. Her condition gradually deteriorated. Oxygen and medications did not improve her condition. A lung transplant was considered. However, her kidneys failed, and she died. An autopsy was performed and tissues were analyzed. Although the woman had symptoms of autoimmune disease, the mixture of symptoms precluded diagnosis with any single one, so she was diagnosed with mixed connective tissue disease. Microscopic analysis of lung tissue was consistent with this diagnosis. Few patients with mixed connective tissue disease die of adult respiratory distress syndrome despite having pulmonary hypertension.

Connective tissue diseases

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A 38-year-old woman with increasing pulmonary hypertension after delivery

Article Abstract:

A 38-year-old pregnant woman was admitted to a hospital with left-sided chest pain and difficulty breathing. She had a history of systemic lupus erythematosus and was in premature labor. Her baby was delivered and she subsequently became severely ill. She had seizures, respiratory failure, and heart failure, and died on the 10th hospital day. An autopsy confirmed that she had a connective tissue disease called scleroderma overlap syndrome. This condition damaged her heart and lungs, but high levels of estrogen during pregnancy protected her. Once her pregnancy was over, her heart and lungs failed despite aggressive treatment.

Scleroderma (Disease)

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