Case 30--Pulmonary Hypertension in a 45-Year-Old Man

Clinical summary: A 45-y-old man underwent bilateral lung transplantation on 5/25/99 for pulmonary hypertension of 3 1/2 years' duration. Preoperative medications included epoprostanol (Flolan), digitalis, lasix, prednisone, and coumadin.

The diagnosis of pulmonary hypertension was made in 4/96 during an admission for diagnosis of the cause of progressive dyspnea. During the preceding 8 months, he had had increasing dyspnea attributed to asthma but not responsive to inhalers or prednisone. On admission, the blood pressure was 121/82 mmHg, heart rate 100/min, and respiratory rate 20/min. The lungs had a few diffuse crackles and wheezes. Jugular venous distension to the angle of the jaw, a right ventricular heave, a grade I murmur of tricuspid regurgitation, a very loud P2, and an S3 were present. He had 1+ edema of the lower extremities. The white blood cell count was 9.8 k/µl, hematocrit 56%, and platelet count 196 k/µl. A chest radiograph showed low lung volumes, a large heart, and prominent pulmonary arteries. Oxyhemoglobin saturation was in the mid 80s. An echocardiogram showed tricuspid regurgitation and a pulmonary artery pressure of 75 mmHg. Catheterization was consistent with precapillary pulmonary hypertension (right pulmonary artery pressure 98/42). Cardiac output was 3.8 L/min with a normal capillary wedge pressure of 8 mmHg. A ventilation/perfusion scan indicated a low probability for embolism, and a pulmonary angiogram was negative. Epoprostanol was begun at 5 ng/kg/min. He was discharged on oxygen, diltiazem, and coumadin. Subsequently, he had mild dyspnea at rest and could walk only a few steps.

He was a produce broker, who had had asthma since childhood, but had not required hospitalization in the past 10 years. He quit smoking cigarettes in 4/94 (20 pack-year history). He drank no alcohol since 4/96. Two sisters died of breast cancer, one with pulmonary hypertension. One brother died of pulmonary hypertension.

In 2/97 he was admitted for a febrile episode, which was treated with antibiotics. During that admission, epoprostanol was increased to 12.5 ng/kg/min. Oxyhemoglobin saturation was 84% on air and 93% on 6 L of nasal oxygen.

Radiology | Image 1 | Image 2 | Image 3 | Image 4 | Image 5 | Discussion

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