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Aspirin
5%
6/131
Bosentan
43%
56/131
Furosemide
31%
41/131
Hydroxychloroquine
6%
8/131
Propranolol
8%
10/131
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This patient who presents with hepatomegaly, pitting edema, and a right ventricular heave has right-sided heart failure most likely due to systemic sclerosis given the symptoms of intractable reflux, Raynaud phenomenon, and hard skin. Pulmonary hypertension in systemic sclerosis can be treated using endothelin inhibitors such as bosentan. Pulmonary hypertension is the result of increased in pulmonary vascular resistance, which can be caused by smooth muscle proliferation of the media and intima. These changes can occur idiopathically or be associated with a secondary case such as systemic sclerosis, schistosomiasis, chronic obstructive pulmonary disease, or cocaine abuse. Pulmonary hypertension can present initially with fatigue and dyspnea on exertion but will progress to right heart failure with signs of fluid overload such as hepatomegaly and pitting edema. The treatment of pulmonary hypertension includes prostacyclin I2, endothelin receptor antagonists such as bosentan, and phosphodiesterase inhibitors such as sildenafil. All of these drugs work through vasodilation of the pulmonary circulation. Incorrect Answers: Answer 1: Aspirin can be used in the treatment of coronary artery disease or be administered after stenting of blocked arteries. Though myocardial infarctions can lead to right-sided heart failure, the presence of other symptoms are more consistent with pulmonary hypertension secondary to systemic sclerosis. Answer 3: Furosemide can be used in acute decompensated heart failure, which can present with edema and hepatomegaly; however, this patient has no previous history of heart failure and no signs of left-sided heart failure such as orthopnea or paroxysmal nocturnal dyspnea. Answer 4: Hydroxychloroquine can be used in the treatment of systemic lupus erythematosus, which can be associated with renal dysfunction and pericardial inflammation; however, this patient's symptoms are more consistent with systemic sclerosis given the intractable reflux and skin tightening in the fingers. Answer 5: Propranolol can be used prophylactically to prevent bleeding from cirrhotic varices; however, this patient's hepatomegaly and loss of appetite are not accompanied by any other stigmata of liver failure such as palmar erythema or jaundice. Bullet Summary: Pulmonary hypertension can be treated with endothelin inhibitors such as bosentan and ambrisentan.
2.6
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