Pathology of Pulmonary Hypertension & Embolism Flashcards
What gene mutation is associated with primary pulmonary hypertension?
Bone morphogenic protein receptor 2 (BMPR2)
A 30yo female presents to the ER with shortness of breath, JVD, and heptaosplenomegaly. The patient has no prior medical history. What is the likely diagnosis?
Idiopathic pulmonary hypertension - most common in women 20-40yo
A woman experiences a complicated labor and delivers a stillborn fetus. During labor the woman experiences respiratory distress and expires. What is the likely COD?
Amniotic fluid embolism
What is Homans sign?
Indicative of a DVT - pain in the calf with dorsiflexion of the foot with the knee extended
A 25yo man with 7-year pack history presents to the ER with hemoptysis and hematuria. Kidney biopsy reveals linear deposits of IgG and crescent-shaped cell proliferation. What is the diagnosis?
Goodpasture Syndrome
True/False. Atherosclerotic lesions in pulmonary arteries are always pathologic.
True - atheromatous deposits in the pulmonary artery are a cause of secondary pulmonary hypertension
What is the function of BMPR2?
This gene inhibits proliferation and favors apoptosis. When the gene is mutated in primary pulmonary hypertension, vascular proliferation and concentric laminar intimal fibrosis occurs
This type of thromboembolism lodges at the bifurcation of the R/L bronchi.
Saddle embolus
This vascular disorder is characterized by autoantibodies against collagen IV that destroy the basement membrane.
Goodpasture Syndrome
How can Wegener’s granulomatosis be differentiated from other vasculitis based on clinical presentation?
URI symptoms (sinusitis, otitis media, nasal ulceration)
How can idiopathic pulmonary hemosiderosis be differentiated from Goodpasture syndrome?
Absence of vasculitis, renal damage, and anti-basement membrane autoantibodies, presentation in young children
A patient presents to the ED with respiratory distress and petechial hemorrhages. PMH is significant for a R femur fracture 3 days ago. What is the diagnosis?
Fat embolism - arises 1-3 days after trauma
What histologic findings would present on examination of a pulmonary vessel in a patient with pulmonary hypertension.
Medial hypertrophy and intimal thickening
How do symptoms of arterial and venous thromboemboli differ?
Arterial - pain, pulselessness, parathesia, pallor, paralysis
Venous - pain, edema, erythema, warmth
What antibodies are present with Wegener’s granulomatosis?
c-ANCA (PR3-ANCA)
What are the four possible outcomes of a thrombus?
Propagation, embolization, dissolution, or recanalization
