A 56-Year-Old Woman With Worsening Dyspnea and Sarcoidosis

Kennedy O. Omonuwa, MD; Arunabh Talwar, MD; Lindsay Goodman, MD

Disclosures

October 31, 2019

The development of pulmonary hypertension in patients with pulmonary sarcoidosis is typically insidious and is associated with minimal symptoms and signs in earlier stages. Dyspnea, especially on exertion, is the most common symptom associated with pulmonary hypertension. Because exertional dyspnea is also a symptom in patients with pulmonary sarcoidosis, the presence of coexisting pulmonary hypertension could easily be overlooked in such patients.

Advanced stages of pulmonary hypertension (especially in patients with concomitant significant tricuspid valve regurgitation) are easier to recognize and are associated with such symptoms as leg edema, abdominal bloating and distention, angina, and syncope (symptoms that are indicative of right-sided heart failure). In cases of early pulmonary hypertension in which the tricuspid valve remains competent, abnormal physical findings may not be present. Patients with severe or advanced disease may exhibit such signs as a loud pulmonic valve sound (P2), a systolic murmur of tricuspid regurgitation, or a right ventricular gallop and jugular venous distention resulting from right ventricular failure.[1,2]

Because the signs and symptoms of pulmonary sarcoidosis and pulmonary hypertension are nonspecific, the occurrence of progressive dyspnea in an adult with pulmonary sarcoidosis should not be attributed to the underlying disease until other causes have been excluded. Dyspnea is a common symptom, with many differential diagnoses. Most cases of dyspnea are caused by cardiac or pulmonary disease. In patients with a pulmonary disease, consider cardiac causes, such as left-sided heart failure, myocardial infarction, valvular dysfunction, and cardiomyopathy. The clinician should also, in the appropriate clinical setting, consider mixed cardiac and pulmonary disorders, such as interstitial lung disease with pulmonary hypertension, cor pulmonale, and pulmonary emboli.[1,2]

The progression of pulmonary sarcoidosis to include pulmonary hypertension may be poorly recognized because the clinical presentation tends to be nonspecific, particularly in the presence of underlying pulmonary parenchymal disease. In patients with pulmonary hypertension, the ECG commonly reveals abnormalities of right ventricular hypertrophy, right-axis deviation, right bundle branch block, and right atrial enlargement. An R wave amplitude in lead V1 of more than 1.2 mV is reportedly 94% sensitive for detecting a systolic mean pulmonary arterial pressure of more than 90 mm Hg. Furthermore, a QRS axis of more than 100° has been found to be highly predictive of right ventricular enlargement, and a pulmonary vascular resistance of more than 5 Wood units is indicative of pulmonary hypertension.[2,3]

Chest radiography may reveal signs suggestive of pulmonary hypertension, such as enlarged pulmonary arteries, attenuation of the peripheral pulmonary vasculature, and right ventricular enlargement. These abnormalities may also be appreciated on high-resolution CT scanning, which is commonly required for the evaluation of underlying parenchymal lung disease and mediastinal disorders in patients with pulmonary sarcoidosis.

PFTs and assessment of the arterial blood oxygenation are necessary to evaluate the degree of pulmonary functional impairment. Transthoracic echocardiography is an essential, noninvasive test for patients with suspected pulmonary hypertension. Assessment of the velocity of the tricuspid regurgitation jet by Doppler echocardiography can help estimate the peak systolic pulmonary arterial pressure, and 2D echocardiography can assess the presence of associated abnormalities, such as right atrial enlargement, right ventricular enlargement or right or left ventricular dysfunction, valvular disease, and pericardial effusion.[1]

Right-heart catheterization is the criterion standard for the diagnosis of pulmonary hypertension. In patients with suspected pulmonary hypertension, right-heart catheterization is required to confirm the presence of pulmonary hypertension by measuring the mean pulmonary arterial pressure, pulmonary vascular resistance, right atrial pressure, cardiac output, and the index. Right-heart catheterization may also determine the severity of pulmonary hypertension, assess the prognosis, and guide initial therapy by vasoreactivity testing.

The clinician should consider other causes of pulmonary hypertension and not necessarily assume a causal relationship between pulmonary sarcoidosis and suspected pulmonary hypertension. With that in mind, the clinician should perform other tests as appropriate, such as ventilation/perfusion scanning to evaluate for chronic thromboembolic disease, autoantibody tests for collagen vascular disease, and HIV testing.

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