Pulmonary Vascular Disease Flashcards
Acute PE and DVT general characteristics:
- Common
- Often Fatal
- Idiopathic
- Recognized Risk Factors: Thrombophilias
- Many Preventable !– DVT prophylaxis
Where do DVT usually originate?
60-90% of PE originate in proximal (above popiteal) deep veins of the legs
Calf vein thrombus usually small and deteriorates but can still be a problem if it propagtates
What is virchow’s triad?
Stasis, Injury, Hypercoagulability
What are risk factors for DVT?
Virchow’s Triad, thrombophilias and medical risks
What are some thrombophilias?
Prot C, S, ATIII, Factor V Leiden, Prothrombin gene mutation, MTHFR; Factor VIII
What are some medical risks?
– Hip, Knee Surgery – Immobility – CHF, Obesity – Malignancy ( may be cause of “Idiopathic VTE”) – Acquired Hypercoagulability
What can be the results of a PE?
- 30% Mortality if untreated
- 2.5% In-Hospital Mortality, due to: Recurrent, Acute PE, Massive Obstruction of Vessels, RV Failure, Infarct
- Vast Majority will resolve with treatment
- 1-3% with Chronic PE
What are the symptoms of PE?
Usually Nonspecific
- Dyspnea; Acute or Subacute
- Dizziness, Syncope: with Large PE, Massive, “Saddle”
- Chest Pain – Pleuritic, (Infarct, Bloody Effusion)
- Palpitations, Tachycardia
- Hemoptysis (infarct)
How do you diagnose a PE & DVT?
• DVT: Doppler Ultrasound (US)
• PE: Ventilation
perfusion scans &CT pulmonary angiogram
What is the Pulmonary Hypertension: Chronic Disorders Classification scale?
I. Pulmonary Arterial Hypertension (PAH)
II. Pulmonary Venous Hypertension, Left Heart
(Most Common)
III. Pulm HTN: Respiratory Disorders (FVC < 70%)
IV. Pulm HTN: Chronic Thromboembolic Disorders
V. Pulm HTN: Miscellaneous
What is the definition of type I. Pulmonary Arterial Hypertension (PAH)?
Mean pulmonary arterial pressure (mPAP) >25 mm Hg at rest, with a normal wedge pressure (PCWP), “pre-capillary pattern.
How do we diagnose PAH?
Echocardiogram is the best non-invasive test.
Able to see Right atrial and Right Ventricular Changes, Right Ventricular Overload, Estimate PA Pressure, Congenital Ht Dis: PFO or shunt (bubble study) and Pericardial Effusion
What hemodynamics are present in PAH?
Decreased RV function: right atrial pressure > 10 mmHg and Cardiac Index < 2.2 L/min m2
poor prognosis if RAP is high and CI (CO) is low
What can cause type 1 PAH?
Idiopathic = IPAH
Heritable
Associated with = APAH things like CVD (Scleroderma, SLE, RA), Congenital Heart Disease, Portal Hypertension, HIV, Drugs, Other Disorders
What are the heritable causes of PAH?
Bone Morphogenic Protein Receptor-2 (BMPR2)
~55% familial PHT have mutations in this gene with Autosomal dominant and low penetrance
What is Bone Morphogenic Protein Receptor-2 (BMPR2)?
TGF-beta superfamily of receptors
Symptoms do not limit physical activity. Ordinary physical activity does not cause undue discomfort
What functional class of PAH?
Class 1
Inability to carry out any physical activity. The patient may experience symptoms even at rest. Discomfort is increased by any physical activity. These patients manifest signs of right heart failure.
What functional class of PAH?
Class 4
Marked limitation of physical activity. The patient is comfortable at rest, yet experiences symptoms with minimal physical activity.
What functional class of PAH?
Class 3
Slight limitation of physical activity. The patient is comfortable at rest, yet experiences symptoms with ordinary physical activity
What functional class of PAH?
Class 2
What are some symptoms of PAH?
- Breathlessness
- Fatigue
- Near Syncope, Syncope (suggestive if particularly with exertion in young adult)
- Chest Pain: Angina
- Palpitations
What pathological changes occur with PAH?
- Large Pulm. Artery Thickening
- Medial Wall Smooth Muscle Hypertrophy
- ** “Plexiform” Lesion in small vessel ***
What endothelial cell defects occur in PAH?
– Decreased Prostacyclin
– Increased Endothelins
– Decreased Nitric Oxide Synthase
How can we change the Prostacyclin (vasodilates and antiproliferation) pathway to treat PAH?
add back prostacylin derivaties
How can we change the Nitric Oxide (vasodilates and antiproliferation) pathway to treat PAH?
add exogenous nitric oxide or inhibit phosphodiesterase5 which usually decreased cGMP
How can we change the Endothelin (vasoconstricts and proliferation) pathway to treat PAH?
endothelin receptor antagonists
Why is Prostacyclin (Epoprostenol , Flolan) tough to administer?
it’s IV because prostacyclin is highly unstable so pt need to wear pumps
What is Bosentan?
Endothelin Receptor Antagonist
What is Sildenafil?
NO pathway, Increase cGMP
What is Riociguat?
NO pathway, Soluble guanylate cyclase stimulator
How can pulmonary vasculitis manifest?
- Pulmonary and Alveolar Hemorrhage (ex.: Goodpasture’s Syndrome)
- Lung Infitrates (nodular, cavitary lesions, associated ILD, Acute, bilateral, diffuse)
- Pulmonary Hypertension
What are the classifications of aveolar (capillary) hemorrhage syndromes?
- Antibasement Membrane Antibody Disease (Goodpasture’s Syndrome)
- Anti Neutrophil Cytoplasmic Antibody Associated Vasculitis
- Idiopathic Pulmonary Hemosiderosis
- Collagen Vascular Diseases
What is the classic triad of alveolar hemorrhage syndromes?
- hemoptysis
- pulmonary infiltrates
- anemia
How can you diagnose alveolar hemorrhage syndromes?
- sputum, tracheal aspirate
- Urine; other organ problems
- Serial Hgb: - 2 gm in 24 hr
- Serial Chest x-rays;
- Serologies: ABMAb, ANCA, ANA, other
- Bronchoalveolar lavage, Open Lung Biopsy
- OTHER ORGANS: Skin Biopsy or Renal Biopsy
What is Wegener’s Granulomatosis?
Systemic vasculitis - granulomatous inflammation
Upper (73%) before Lower Respiratory (45%) Tract; and Kidney (18%)
SIGNS/SYMPTOMS: Nasal, sinus, otitis, Cough, hemoptysis, Constitutional (joint, fever)
When does Wegener’s Vasculitis occur?
5th decade; male > female
How do you diagnose Wegener’s?
– tissue biopsy
– Serologies (c-ANCA)
What happens in the lungs in Wegener’s?
- Capillaritis, Alveolar Hemorrhage
- Nodules, Cavitary Lesions
- Large Airways
- Interstitial lung disease
