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Pulmonary Vasculature Disorders

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Pulmonary Hypertension (PH) due to Left Heart Disease

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Clinical features: Dyspnea, fatigue, evidence of left heart disease. Diagnostic approach: Echocardiography, cardiac MRI. Management: Treatment of underlying left heart disease, diuretics.

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Pulmonary Arteriovenous Malformation (PAVM)

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Clinical features: Cyanosis, clubbing, dyspnea. Diagnostic approach: Contrast echocardiography, CT angiography. Management: Embolization or surgical resection.

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Pulmonary Infarction

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Clinical features: Pleuritic chest pain, hemoptysis, fever. Diagnostic approach: CT pulmonary angiography, ECG, chest X-ray. Management: Anticoagulation, pain management.

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Sarcoidosis

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Clinical features: Dry cough, dyspnea, chest pain, extrapulmonary symptoms like skin lesions and eye problems. Diagnostic approach: Chest X-ray, biopsy of affected tissue. Management: Corticosteroids, immunosuppressive agents.

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Pulmonary Venous Thromboembolism (PVTE)

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Clinical features: Similar to PE with dyspnea, chest pain. Diagnostic approach: Imaging with CT or MRI, sometimes invasive venography. Management: Anticoagulation, managing underlying causes.

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Pulmonary Capillary Hemangiomatosis (PCH)

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Clinical features: Dyspnea, fatigue, signs of right heart failure. Diagnostic approach: Lung biopsy, imaging studies. Management: Similar to PAH, lung transplantation.

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Eisenmenger Syndrome

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Clinical features: Cyanosis, polycythemia, clubbing. Diagnostic approach: Echocardiography, cardiac catheterization. Management: Avoidance of dehydration, phlebotomy for symptomatic polycythemia, transplantation in select cases.

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Pulmonary Arterial Hypertension (PAH)

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Clinical features: Dyspnea, chest pain, syncope. Diagnostic approach: Echocardiography, right heart catheterization. Management: Vasodilators, anticoagulation, lung transplantation in severe cases.

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Pulmonary Embolism (PE)

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Clinical features: Sudden onset dyspnea, chest pain, hemoptysis. Diagnostic approach: D-dimer test, CT pulmonary angiography. Management: Anticoagulation, thrombolytic therapy, Inferior Vena Cava (IVC) filter if needed.

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Pulmonary Edema

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Clinical features: Acute shortness of breath, orthopnea, pink frothy sputum. Diagnostic approach: Chest X-ray, brain natriuretic peptide (BNP) levels. Management: Oxygen, diuretics, treat underlying cause (often heart failure).

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Pulmonary Lymphangioleiomyomatosis (LAM)

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Clinical features: Dyspnea, recurrent pneumothoraces, chylous pleural effusions. Diagnostic approach: High-resolution CT scan, lung biopsy. Management: Sirolimus, supportive care, lung transplantation if severe.

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Chronic Thromboembolic Pulmonary Hypertension (CTEPH)

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Clinical features: Progressive dyspnea on exertion, fatigue, right heart failure signs. Diagnostic approach: V/Q scan, pulmonary angiography. Management: Anticoagulation, pulmonary thromboendarterectomy.

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