{"id":7071,"date":"2013-01-22T21:31:50","date_gmt":"2013-01-23T02:31:50","guid":{"rendered":"http:\/\/insidesurgery.com\/?p=7071"},"modified":"2013-01-23T20:42:56","modified_gmt":"2013-01-24T01:42:56","slug":"pulmonary-hypertension","status":"publish","type":"post","link":"https:\/\/insidesurgery.com\/2013\/01\/pulmonary-hypertension\/","title":{"rendered":"Pulmonary Hypertension"},"content":{"rendered":"

Pathophysiology<\/strong><\/p>\n

1)<\/strong> increased arterial pressure in lungs 2)<\/strong> primary or idiopathic form occurs in absence of known precipitating causes 3)<\/strong> secondary form caused by – mitral stenosis, pulmonary venous occlusive disease, extreme obesity (Pickwickian syndrome), chronic hypoxemia, recurrent pulmonary embolism, kyphosis, infiltrative lung disease, chronic bronchitis, long periods at high altitude<\/p>\n

<\/p>\n

Signs and Symptoms<\/strong><\/p>\n

1)<\/strong> insidious onset of dyspnea idiopathic form 2)<\/strong> right heart failure with venous distention of neck 3)<\/strong> peripheral cyanosis late in disease 4)<\/strong> palpable right ventricular heave (owing to cor pulmonale) 5)<\/strong> fatigue 6)<\/strong> syncope 7)<\/strong> hepatomegaly 8)<\/strong> ankle edema 9)<\/strong> tachypnea 10)<\/strong> nonproductive cough 11)<\/strong> chest pain<\/p>\n

Characteristic Test Findings<\/strong><\/p>\n

Radiology<\/em> – 1)<\/strong> scattered infiltrates consistent with hemorrhage and hemosiderosis on chest radiograph 2)<\/strong> enlarged central pulmonary arteries EKG<\/em> – 3)<\/strong> right axis deviation with right ventricular hypertrophy Echocardiography<\/em> – 4)<\/strong> right ventricular enlargement with decreased left ventricular size Laboratory – hypoxemia<\/p>\n

Histology\/Gross Pathology<\/strong><\/p>\n

1)<\/strong> early changes are reversible if underlying conditions are corrected (e.g., with heart surgery) 2)<\/strong> grade 1 – smooth muscle hypertrophy of arteries 3)<\/strong> grade 2 – intimal proliferation exists 4)<\/strong> grade 3 – intimal fibrosis has begun 5)<\/strong> grade 4 – dilation and thinning of pulmonary arteries with formation of nodular plexiform lesions 6)<\/strong> grade 5 – pulmonary artery rupture with hemorrhage into lung parenchyma 7)<\/strong> grade 6 – fibrinoid necrosis of arteries\/arterioles<\/p>\n

Associated Conditions<\/strong><\/p>\n

1)<\/strong> right-sided heart failure 2)<\/strong> generalized venous congestion 3)<\/strong> diet drugs fenfluramine and phentermine<\/p>\n

Biochemistry<\/strong><\/p>\n

1)<\/strong> recurrent pulmonary embolism causes pulmonary hypertension through mechanical blockage of the arteries with resultant vasoconstriction and cytokine release 2)<\/strong> at birth the vasculature of the lungs thin and dilate as the lung moves to a high-volume\/low pressure system from a low-volume\/high pressure system in the fetus that shunts blood through the ductus arteriosus<\/p>\n

Inheritance\/Epidemiology<\/strong><\/p>\n

1)<\/strong> idiopathic pulmonary hypertension affects mostly women in the 20s and 30s 2)<\/strong> a few cases are familial with involved defect likely a trinucleotide repeat expansion on 2q31-32<\/p>\n

Treatment<\/strong><\/p>\n

1)<\/strong> previously idiopathic from was relentlessly progressive and required heart-lung transplantation 2)<\/strong> new treatment regimens can at least slow the progression and includes – IV adenosine, IV prostacyclin, calcium channel blockers (nifedipine,diltiazem), anticoagulation, digoxin, diuretics, avoidance of strenuous activity<\/p>\n

Tips for USMLE<\/strong><\/p>\n

1)<\/strong> isolated right-sided failure can occur in absence of left-sided failure 2)<\/strong> in pulmonary hypertension the right ventricular fraction does not increase with exercise as it would in a normal patient<\/p>\n","protected":false},"excerpt":{"rendered":"

Pathophysiology 1) increased arterial pressure in lungs 2) primary or idiopathic form occurs in absence of known precipitating causes 3) secondary form caused by – mitral stenosis, pulmonary venous occlusive disease, extreme obesity (Pickwickian syndrome), chronic hypoxemia, recurrent pulmonary embolism, kyphosis, infiltrative lung disease, chronic bronchitis, long periods at high altitude<\/p>\n","protected":false},"author":1,"featured_media":0,"comment_status":"open","ping_status":"open","sticky":false,"template":"","format":"standard","meta":{"_exactmetrics_skip_tracking":false,"_exactmetrics_sitenote_active":false,"_exactmetrics_sitenote_note":"","_exactmetrics_sitenote_category":0,"_genesis_hide_title":false,"_genesis_hide_breadcrumbs":false,"_genesis_hide_singular_image":false,"_genesis_hide_footer_widgets":false,"_genesis_custom_body_class":"","_genesis_custom_post_class":"","_genesis_layout":"","footnotes":""},"categories":[5076],"tags":[5337,5341,4268,5339,5338,3028,5340],"yoast_head":"\nPulmonary Hypertension - 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