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

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

Disclosures

October 31, 2019

Discussion

An eventual diagnosis of pulmonary hypertension was made in this patient with pulmonary sarcoidosis who presented with persistent dyspnea that was refractory to steroid therapy. The loud P2 detected on the cardiac examination, together with the abnormal ECG findings of right ventricular hypertrophy (tall R wave > 6 mm in V1), right-axis deviation, and a right atrial abnormality (P wave > 2.5 mm in lead II and > 1.5 mm in V1) suggestive of right-heart dysfunction were useful clues to the suspicion of complicating pulmonary hypertension in this patient with a known cause (sarcoidosis) of pulmonary hypertension.

These discoveries correlated with the 2D echocardiogram findings, which showed a high estimated peak pulmonary artery systolic pressure of 86 mm Hg (normal range, 18-25 mm Hg at rest). The subsequent right-heart catheterization confirmed the diagnosis of pulmonary hypertension (with an elevated mean pulmonary arterial pressure of 55 mm Hg and elevated pulmonary vascular resistance of 11.13 Wood units). Because the PFT results remained unchanged after 6 months, the progressive dyspnea is less likely to be secondary to worsening pulmonary sarcoidosis itself.

Sarcoidosis is a multiorgan disorder of unknown cause characterized by the presence of noncaseating granulomas, most commonly involving the lungs. Sarcoid patients with advanced pulmonary disease, especially end-stage pulmonary fibrosis, are at risk for developing sarcoidosis-associated pulmonary hypertension (SAPH). Among end-stage sarcoid patients awaiting lung transplantation, pulmonary hypertension is documented in approximately 75% of cases, which is predictive of increased mortality. Pulmonary hypertension has also been reported as an early manifestation of sarcoidosis and in patients with sarcoidosis who are not considered candidates for transplant.[1,2]

Pulmonary hypertension is defined as a mean pulmonary arterial pressure greater than 25 mm Hg at rest or 30 mm Hg with exercise. Patients with pulmonary hypertension and pulmonary sarcoidosis have a worse prognosis.

SAPH is placed in the miscellaneous (Group V) category of the World Health Organization classification of pulmonary hypertension because the mechanism of pulmonary hypertension in sarcoidosis patients is multifactorial. The pathogenesis of SAPH includes fibrotic destruction of the lung vasculature, granulomatous pulmonary angiitis, or extrinsic compression of major pulmonary vessels by mediastinal or hilar adenopathy. In addition to its direct effects on the pulmonary vasculature, left ventricular myocardial infiltration may cause diastolic dysfunction or diminished systolic function and lead to secondary pulmonary hypertension.[1,2]

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