Chronic cor pulmonale in pulmonary sarcoidosis.

Abstract

pulmonale in pulmonary sarcoidosis. Right-heart overload happens relatively rarely in sarcoidosis, even with fibroemphysematous changes. Of 21 cases that we studied, six (28%) had clinical and/or electrocardiographic features of cor pulmonale. The cause of cor pulmonale often evoked is an invasion of the walls of pulmonary vessels by sarcoid granulomas or their compression by the fibrotic process. Pathological studies in one patient showed compression of large pulmonary arteries associated with specific sarcoid lesions in small and medium-sized arteries.The development of chronic cor pulmonale due to pulmonary hypertension has rarely been described in pulmonary sarcoidosis, even in chronic fibroemphysematous forms. The mechanisms often evoked to explain the occurrence of pulmonary hypertension are based on histological findings, the perivascular occurrence of granulomas with the fibrosis which sometimes results, and the possibility of thrombosis or invasion of arterial and vein walls by granulomas. Material and methodsTwenty-one cases of chronic sarcoidosis were studied.The diagnosis was made using clinical, radiological, pathological (bronchial biopsy), and immunological (Kveim test, tuberculin anergy) findings. Such a diagnosis was confirmed in life by these methods even in the two cases (2 and 5) where no definite evidence of sarcoidosis was found at necropsy many years later.The radiographic classification was based on postero-anterior and lateral views, occasionally confirmed by tomography.Evidence of right-sided heart involvement was based upon clinical examination and six electrocardiographic criteria (Schaub, 1966): 1 QRS vector angle in the frontal plane (angle a)>+900.2 Negative T wave in S2 and S3 or S, and ca. +900.3 S wave in V, and V >07 mV.4 R wave in V,+S wave in V5>1 mV.5 QRS direction to the right and posterior:rS of V1 to V4, sometimes rS or Qs of V1 to V6 with positive or flattened T waves.6 Incomplete right bundle-branch block: rS R' in V1 with R' in V1>10 mV. Radiological measurement of the size of the right heart chambers and the diameter of the pulmonary arteries was in most cases impossible because of the perihilar pulmonary opacities which obscured the cardiac and arterial contours. Thus radiological criteria were not taken into account.

Sarcoidosis-Associated Fibrosing Mediastinitis with Resultant Pulmonary Hypertension: A Case Report and Review of the Literature

“…While fibrosing mediastinitis is not common with sarcoidosis, pulmonary hypertension occurs in up to 28% of patients [1,2,3,4,5,14]. However, because this estimate reflects the prevalence at referral centers, it may be biased [1,6].…”

“…Etiologies of pulmonary hypertension with sarcoidosis include fibrosis with chronic hypoxia or destruction of small vessels [1,2,5,14], extrinsic compression of pulmonary arteries by granulomatous disease [8,16] or lymphadenopathy [7], and granulomatous vasculitis [9,11,13] which occasionally mimics pulmonary veno-occlusive disease [7,12,17]. These etiologies were unlikely in our patient as he had no radiographic evidence of parenchymal fibrosis, normal resting oxygen saturation, no arterial compression by MRA and no perfusion abnormalities by CTA.…”

Technetium-99m Sestamibi/Tetrofosmin Myocardial Perfusion Scanning in Cardiac and Noncardiac Sarcoidosis

“…RV asynergy on well-conducted transthoracic echocardiography and the high signal on magnetic resonance image are considered possible diagnostic tools [16]. However, the complexity of the RV configuration and the wide spectrum of myocardial involvement and hypertrophy in sarcoidosis [1, 3, 24]makes the diagnosis very difficult. Recently, several authors have reported that SPECT using 99m Tc is useful for confirmation of right myocardial involvement in RV infarction and myocardial hypertrophy [17, 18, 19, 20, 21, 22].…”

“…Primary cardiac involvements such as myocardial, pericardial, valvular, or microvascular [1, 2]and secondary cor pulmonale [24]must be firmly examined not only for diagnostic interest but also to determine high-risk individuals. In the present study, we demonstrated that the detection of perfusion abnormalities by SPECT using technetium-99m is useful for confirming myocardial involvement in both ventricles of patients with cardiac sarcoidosis.…”

Successful Treatment of Sarcoidosis-Associated Pulmonary Hypertension with Bosentan

“…This has been observed to occur in the absence of significant lung parenchymal involvement. Pulmonary hypertension related to compression of the central pulmonary vasculature by enlarged mediastinal lymph nodes and leading to severe PH with right heart failure has also been reported [3]. Additionally, PH can occur in the setting of left heart disease.…”