Vascular (6) - Gomez Flashcards
Pulmonary Diseases Of Vascular Origin
Pulmonary Embolism, Hemorrhage and Infarction
Pulmonary Hypertension
Diffuse Pulmonary Hemorrhage Syndromes
Goodpasture Syndrome
Wegener Granulomatosis
Idiopathic Pulmonary Hemosiderosis
Other vasculitides
Pulmonary Embolism
- Significant cause of sudden death (>50K/yrUS)
10% of acute hospital deaths - Underdiagnosed
Thrombi form most often in deep leg veins in patients with underlying conditions and/or immobilized
Hypercoagulable states - Respiratory and Hemodynamic compromise
Most lower lobe
Dyspnea, fever, chest pain, hemoptysis, electromechanical dissociation with sudden death
Elevated lactate dehydrogenase (LDH) - 10% result in infarction which may also have hemoptysis
lung v/q scan
radioactive material to the lungs
then into the vasculature
area with air but no blood is blocked
Other Forms of Emboli
Fat Bone marrow Tumor Air Nitrogen Talc and metal oxides Bullets Amniotic fluid
Pulmoary hypertension
Abnormal when > 25 mm Hg
Dyspnea, fatigue, anginal chest pain &/or cyanosis when disease advanced
Eventually can lead to cor pulmonale (pulmonary hypertensive heart disease) with cardiac failure (with death in 80% within 2-5 years)
- Pulmonary arterial hypertension examples
Primary idiopathic pulmonary arterial hypertension*, autoimmune connective tissue disorders, drugs (fenfluramine/phentermine, bush tea, aminorex, toxic rapeseed oil, dexfenfluramine)
- Pulmonary hypertension secondary to left-heart failure
Cardiac malformations and acquired heart disease
- Pulmonary hypertension stemming from lung parenchymal disease or hypoxemia
Chronic obstructive or interstitial lung disease, obstructive sleep apnea
- Chronic thromboembolic pulmonary hypertension
Chronic recurrent pulmonary thromboembolism
Primary Pulmonary Hypertension
Idiopathic strikes adults (mostly females, 20-40 yr)
Rare familial form (~6%) that is autosomal dominant with only ~10-20% penetrance
Mutation in the bone morphogenic protein receptor type 2 (BMPR2)
In vascular smooth muscle cells (SMC), BMPR2 inhibits proliferation and favors apoptosis
Mutation inactivates thus favoring SMC proliferation
Seen in 50% of familial and 25% of sporadic
Secondary Pulmonary Hypertension
Endothelial dysfunction and normal reaction of pulmonary arteries to increased pressure
Increased pressure from any cause induces medial hypertrophy
If increased pressure continues then hypertrophy narrows arteries to where the narrowing further increased pressure
Eventually can lead to cor pulmonale (pulmonary hypertensive heart disease) with cardiac failure
pulmonary hypertension can lead to
atherosclerosis
A, Atheroma formation
B Medial hypertrophy.
C, Plexiform lesion (recanalization)
Some causes of Diffuse Alveolar Hemorrhage Syndrome
Goodpasture syndrome
Idiopathic pulmonary hemosiderosis
Vasculitis; e.g. Wegener granulomatosis, hypersensitivity pneumonitis, lupus erythematosus
Goodpasture Syndrome
Necrotizing hemorrhagic interstitial pneumonitis
**+ Renal involvement (focal proliferative rapidly progressive to crescentic glomerulonephritis)
Autoimmune
**Anti-basement membrane antibodies to
α3 chain of collagen IV
Serum anti-glomerular basement membrane antibodies (anti-GBM ab) available
M>F
Renal failure (uremia), most common cause of death Rx- plasmapheresis and immunosuppressants
Idiopathic Pulmonary Hemosiderosis
Rare condition usually of children
Episodes of diffuse hemorrhage
No antivascular antibodies have been found
However, immunosuppression works!
Diffuse hemosiderin deposition in macrophages and alveolar walls
Productive cough, hemoptysis, anemia, weight loss
