Case

Primary Pulmonary Hypertension: Right Ventricular Failure

At the time of her death, Celia Lukas was a 38-year-old homemaker and mother of three children, 15, 14, and 12 years of age. She had an associates degree in computer programming from a community college, but had not worked outside the home since the birth of her first child. Keeping house and driving the children to activities kept her very busy. To stay in shape, she took aerobics classes at the local community center. The first sign that Celia was ill was vague: she fatigued easily. However, within 6 months, Celia was short of breath (dyspnea), both at rest and when she exercised, and she had swelling in her legs and feet. She made an appointment to see her physician. On physical examination, Celias jugular veins were distended, her liver was enlarged (hepatomegaly), and she had ascites in her peritoneal cavity and edema in her legs. A fourth heart sound was audible over her right ventricle. The physician was very concerned and immediately scheduled Celia for a chest x-ray, an ECG, and a cardiac catheterization. The chest x-ray showed enlargement of the right ventricle and prominent pulmonary arteries. The ECG findings were consistent with right ventricular hypertrophy. The results of cardiac catheterization are shown in Table 24.

Consulting physicians in cardiology and pulmonology concluded that Celia had primary pulmonary hypertension, a rare type of pulmonary hypertension that is caused by diffuse pathologic changes in the pulmonary arteries. These abnormalities lead to increased pulmonary vascular resistance and pulmonary hypertension, which causes right ventricular failure (cor pulmonale). Celia was treated with vasodilator drugs, but they were not effective. Her name was added to a list of patients awaiting a heartlung transplant. However, she died of right heart failure before a transplant could be performed.