Birth Control Online - Pulmonary Vascular Occlusion and Fibrosing Mediastinftis: Case Reports
Patients with fibrosing and granulomatous medi-astinitis commonly present a diagnostic challenge because of the variability of involvement of the different structures of the mediastinum. The spectrum of the disease varies from a benign condition with minor symptoms to severe pulmonary hypertension resulting in death. We have recently encountered three patients who have further broadened this spectrum. All were referred to us as potential candidates for pulmonary thromboendarterectomy with a presumptive diagnosis of pulmonary hypertension due to chronic thrombotic obstruction of major pulmonary arteries. The findings in these three patients did, in fact, remarkably mimic those in patients with chronic, large vessel thrombotic obstruction. The history and physical findings, the results of perfusion and ventilation scanning, as well as pulmonary angiographic and certain findings on right heart catheterization all paralleled those which characterize chronic, major vessel thrombotic obstruction. However, all of the findings were explained by pulmonary arterial and/or venous obstruction due to fibrosing mediastinitis. Because an attempted thromboendarterectomy in such patients would have had no positive—indeed, likely, quite adverse—effects, we feel it is important to describe this experience and to emphasize the differential diagnostic features between chronic thrombotic obstruction and this form of fibrosing mediastinitis. Case Reports Case 1 A 28-year-old white woman first noticed dyspnea on exertion in May 1982. Throughout the following months, she also noted mild intermittent cough productive of blood streaked sputum on several occasions. By May 1983, her dyspnea had progressed so that she was short of breath after one block of level walking. Her medical history was significant only for her use of birth control pills - https://mybirthcontrolonline.com/birth-control-online-pills-after-sexual-intercourse.html. She denied any symptoms of deep venous thrombosis. She was a nonsmoker. She had lived in Tennessee for 23 years prior to moving to California. In May 1983, she was evaluated at another hospital. Pulmonary ventilation and perfusion scans and right heart catheterization led to the diagnosis of probable chronic thrombotic obstruction of major pulmonary arteries. She was treated with coumadin and referred to UCSD Medical Center for possible thromboendarterectomy. Physical examination showed unlabored respiration at rest with a rate of 16. The lungs were clear. Cardiac examination showed a sustained left parasternal lift, a split S2 that widened with inspiration, and an increased Pt. There were no signs of right ventricular failure. Chest x-ray films showed cardiomegaly with a right ventricular configuration, fullness of the left hilum, right pleural thickening, and possible Kerley B lines in both lower lobes. An area of possible subcarinal calcification was seen. A perfusion scan showed near total absence of perfusion to the right lung, with only minimal perfusion to the right apex; on the left, there were several basilar subsegmental defects. Ventilation scan was normal (Fig 1). Echocardiogram revealed an enlarged right atrium and ventricle, and a normal mitral valve and left ventricle. Impedance plethysmography was normal bilaterally. At initial right heart catheterization, the pulmonary artery pressure was 87/45 (mean 60 mg Hg). The (Fick) cardiac output was 6.8 L/min. Hie pulmonary vascular resistance was elevated at 649 dynes/s/cm’. The wedge pressure was not measured for technical reasons. Pulmonary angiography showed abrupt cut-offs of the right lower and middle lobe arteries, and the right pulmonary artery was narrowed by 70 to 80 percent. On the left, there were questionable filling defects in the left lower lobe segmental vessels (Fig 2). The CT scan of the chest demonstrated a large, calcified subcarinal mass displacing the left atrium and right pulmonary artery anteriorly (Fig 3). Calcified granuloma were seen in the liver and spleen. A histoplasmin skin test was positive; PPD, negative. Results of serologic tests for histoplasmosis, blastomycosis, and coccidiodomy-cosis were negative. At repeat right heart catheterization, wedge pressures were measured in several areas: 26 mm Hg in the right lower lobe, 19 mm Hg in the right upper lobe, 26 mm Hg in the left lower lobe, and 21 mm Hg in the left upper lobe. Selective pulmonary angiography on the right showed normal vasculature distal to the previously noted “cut-offs” (Fig 4). Left heart catheterization disclosed a mean left atrial pressure of 7 mm Hg, no mitral gradient, and normal left ventricular pressures. Selective injection of pulmonary veins clearly demonstrated obstruction near the entrance to the left atrium (Fig 5). Because of severe cough, she was treated with a course of prednisone. Her cough markedly improved but the shortness of breath did not. Case 2 A 28-year-old woman was referred to UCSD Medical Center for pulmonary thromboendarterectomy. She was well until two years before when she had an episode of right pleuritic chest pain associated with shortness of breath and hemoptysis. She was treated as an outpatient with antibiotics. Three months later, she had similar symptoms and was again treated for pneumonia. After the second episode, dyspnea, a severe nonproductive cough, and wheezing persisted, despite treatment with bronchodilators. Over the subsequent two years, dyspnea on exertion slowly increased. A third episode similar to the first two resulted in admission to another hospital in April 1983. Physical examination was remarkable for a right ventricular heave without evidence of right ventricular failure. Perfusion scan showed no flow to the right lung and defects in the left upper lobe. The angiogram reportedly showed stenotic main, right upper lobe and right lower pulmonary arteries, as well as cutoffs in the left upper lobe. There was an episode of greater than 100 ml of hemoptysis after angiography. Treatment with coumadin was initiated and a Greenfield filter was placed in the inferior vena cava. In July 1983, there was another episode of severe hemoptysis that spontaneously resolved. Bronchoscopy revealed friability and increased vascularity of the mucosa of the major bronchi on the right Her other medical history was significant for a history of birth control pill use. At physical examination at UCSD, the jugular venous pressure was 8. There was a left parasternal lift. The Ps was markedly increased and split An S3, S4 and a 2/6 systolic murmur were present at the lower left sternal border. Chest x-ray film revealed a small right lung and pulmonary artery. There were increased right interstitial markings. Old films showed the right lung and pulmonary artery were normal in 1980. Right heart catheterization showed pulmonary artery pressures of 70/30 mm Hg with a mean of 45 mm Hg. The pulmonary artery wedge was 25 in LUL and LLL (cardiac output was 5.9 L/min; PVR 816 dynes s cm"). Angiography of the left lung revealed a large left PA with attenuated peripheral branches. The left upper lobe pulmonary veins appeared stenotic near their entry into the left atrium. The right PA could not be selectively catheterized, and angioscopy revealed no visible lumen (of the right PA). The CT scan of the chest showed large calcified nodes involving the subcarinal and pretracheal regions and compromising the right pulmonary artery. The histoplasmin skin test was positive. After discharge, she moved to sea level with substantial resolution of signs and symptoms of pulmonary hypertension. During this time, she was also treated with a tapering course of prednisone. Case 3 This 68-year-old woman was admitted to University Hospital on Jan 2, 1985, for evaluation for possible pulmonary thromboendarterectomy. History disclosed recurrent respiratory illness dating to her childhood. She had “always” been mildly short of breath and could not keep up with her peers by adolescence. At least one episode of “pneumonia” required two weeks of hospitalization in 1942. In 1957, she had an excisional biopsy of cervical lymph nodes. No diagnosis was made. In the mid 1960s, she was found to have a positive PPD and abnormal findings on chest x-ray film. She was treated for an unknown period for tuberculosis. In 1971, she was presented with chest pain and shortness of breath and was presumed to have a pulmonary embolus. Neither perfusion scan or angiography was done. She was anticoagulated for six months. Apparently, several ECGs were suggestive of an acute myocardial infarction. Over the next ten years, there was a slow increase in dyspnea on exertion. Recurrent episodes of cough and pleuritic chest pain continued. She also described exertional chest pain relieved by nitroglycerin and rest. Two months before admission, evaluation elsewhere led to coronary angiography, which showed triple vessel disease, and pulmonary angiography, which disclosed total obstruction of the right pulmonary artery. Chronic pulmonary thromboembolic disease was diagnosed. She denied any history of leg swelling or tenderness. She had lived in St. Louis until 1942 when she moved to San Diego. Family members were thought to have had histoplasmosis. On physical examination, she was an afebrile elderly woman coughing frequently. She was not dyspneic at rest. Chest examination revealed bilateral lower lung field crackles more prominent on the right. The jugular venous pressure was 8 to 9 cm. Cardiac examination was normal. Chest x-ray film showed biventricular cardiac enlargement, right lung volume loss, right pleural thickening, subtle bilateral Kerley B lines, and a right lower lung field infiltrate. Hie ABC showed a pH of 7.46, Pa02 of 65, and a PaC02 of 36 on room air. Impedance plethysmography was normal in both legs. Skin tests for histoplasmosis and PPD were positive. A perfusion lung scan showed no perfusion to the right lung with scattered subsegmental defects in the left lung. The ventilation scan was normal. A CT scan of the chest disclosed a large calcified subcarinal mass, right of the midline. The mass was seen to compress the right lower pulmonary vein and the right pulmonary artery. Repeat cardiac catheterization revealed RA pressure of 8 to 10, RV pressure of 70/8, and PA pressure of70/24. The mean wedge pressure was 20 in both the LUL and LLL. The P(A-a) 02 difference was 5.1 volumes percent; the 02 consumption 130 ml/min/m2. The cardiac index (Fick) was 2.6 L/min/m2. The calculated pulmonary vascular resistance was 408 dynes-s•cm. The mean left atrial pressure was 12. The LA injection showed no filling of the right pulmonary veins. As in case 1, cough was a major problem. Prednisone was instituted with substantial relief. Figure 1A (left). Perfusion scan (posterior view) shows that only the apex of the right lung is perfused. Subsegmental cuts are possibly present in the left lung. Ventilation scan (B, right) is normal. Figure 2A (left). Injection of the right PA shows a narrow right pulmonary artery with multiple “cutoffs.” (B, right) Pulmonary angiography shows a large left pulmonary artery and a question of defects in the lobar artery to the lower lobe. Figure 3. CT scan as shown is a cut just below the carina. Note the large subcarinal mass and the generous main and left pulmonary arteries. (A) Calcified subcarinal mass; (B) Ascending aorta; (C) Right mainstem bronchus; (D) Left mainstem bronchus; (£) Descending aorta; (F) Left pulmonary artery; and (C) Large main pulmonary artery. Figure 4. Selective injection of the right pulmonary artery from a more peripheral spot demonstrates that vessels are patent in areas where cutoffs had apparently been present. Figure 5. Catheterization of left atrium shows a pulmonary vein (arrow) with an abrupt “cutoff.”
