A 36-year-old woman comes to the office due to dyspnea on exertion. She has no other medical problems. She does not use tobacco, alcohol, or drugs. Her father has prostate cancer and her mother had a stroke. She takes no medication and has no known drug allergies. Her temperature is 36.7 C (98 F), blood pressure is 140/90 mm Hg, pulse is 84/min, and respirations are 22/min. Examination shows clear lung fields. Her chest x-ray shows prominent pulmonary arteries and an enlarged right heart border. ECG shows right axis deviation. Which of the following is the most likely diagnosis?

Question

A 36-year-old woman comes to the office due to dyspnea on exertion.  She has no other medical problems.  She does not use tobacco, alcohol, or drugs.  Her father has prostate cancer and her mother had a stroke.  She takes no medication and has no known drug allergies.  Her temperature is 36.7 C (98 F), blood pressure is 140/90 mm Hg, pulse is 84/min, and respirations are 22/min.  Examination shows clear lung fields.  Her chest x-ray shows prominent pulmonary arteries and an enlarged right heart border.  ECG shows right axis deviation.  Which of the following is the most likely diagnosis?

Quizplus · Accepted Answer

Explanation: The physical findings in this patient are suggestive of pulmonary hypertension. Pulmonary hypertension is defined as a mean pulmonary arterial pressure ≥25 mm Hg at rest. It is seen following the narrowing of the precapillary vessels, the loss of the pulmonary capillary bed, or passive backpressure from the post capillary vessels from any cause. It can be classified as follows: Group 1 - Pulmonary arterial hypertension (PAH), which can be idiopathic (primary PAH) or due to drugs, HIV, or connective tissue diseases Group 2 - Pulmonary hypertension due to left heart disease Group 3 - Pulmonary hypertension due to chronic lung disease or hypoxemia Group 4 - Pulmonary hypertension due to chronic thromboembolic disease Group 5 - Pulmonary hypertension due to other causes (eg, hematologic, systemic disorders) The clinical features of pulmonary hypertension remain the same regardless of the underlying cause. Dyspnea, weakness, and fatigue are early complaints, which may be followed by chest pain, hemoptysis, syncope, or hoarseness (due to compression of the recurrent laryngeal nerve). Right ventricular failure develops late in the disease and manifests with right ventricular heave, jugular venous distension, tender hepatomegaly, ascites, edema, etc. Chest x-ray would show enlargement of the pulmonary arteries with rapid tapering of the distal vessels (pruning) and enlargement of the right ventricle. An ECG may show right axis deviation, which is due to right ventricular strain and hypertrophy due to pulmonary hypertension. Untreated pulmonary hypertension would eventually lead to cor pulmonale. This patient most likely has idiopathic (primary) PAH. (Choices A, B, and C) All these options can cause secondary pulmonary hypertension; however, signs and symptoms of pulmonary venous congestion (eg, cephalization) would be seen. (Choices D, E, and I) Emphysema and chronic bronchitis are unlikely in this patient who is young and a nonsmoker. X-ray findings in chronic bronchitis are nonspecific but can include increased bronchovascular markings; findings in emphysema can include hyperinflation and bullae. Viral pneumonia is unlikely to be associated with clear lung fields and would not cause right axis deviation. (Choices F and G) Pulmonary fibrosis is a possibility but it is typically associated with some physical findings (eg, crackles) and chest x-ray abnormalities. Scleroderma would cause pulmonary fibrosis, which eventually leads to secondary pulmonary hypertension. Educational objective: Primary pulmonary hypertension can be seen in middle-aged patients, and it presents with exertional breathlessness. Lungs are generally clear to auscultation. Chest x-ray would show enlargement of the pulmonary arteries with rapid tapering of the distal vessels (pruning) and enlargement of the right ventricle.

A 36-Year-Old Woman Comes to the Office Due to Dyspnea