Pulmonary Vasculature Flashcards
Define pulmonary HTN
Elevation in pulmonary arterial pressure
WHO grouping (5)
- Pulmonary arterial HTN (PAH) secondary to various disorders
- Due to left heart dz
- Due to lung dz &/or hypoxia
- Due to chronic thromboemboli
- Miscellaneous
In pulmonary htn, what is mean pulmonary pressure at rest?
> 25
WHO - Group 1
Diseases that localize directly to the pulmonary aa –> structural changes, SM hypertrophy, & endothelial dysfxn
What are definite RFs for PAH?
- Appetite suppressants
- Rapeside oil
- Benfluorex
WHO - Group 2 is secondary to what? What is it referred to as?
L heart dz
- Referred to as pulmonary venous htn or “post-capillary” pulmonary htn
WHO - Group 2 includes what conditions?
- LV systolic & diastolic dysfxn
- Valvular heart dz
- Congenital/acquired obstruction & CMs
WHO - Group 3 is secondary to what?
Lung dz or hypoxemia
What causes lung dz & hypoxemia?
Advanced obstructive & restrictive lung disease:
- COPD
- ILD
- Pulmonary fibrosis
- Bronchiectasis
WHO - Group 4 is secondary to what?
Chronic thromboembolism
What classification is no longer included in group 4?
Pts w/ non-thrombotic occlusion (tumors, FB)
WHO - Group 5 is secondary to what?
Hematologic, systemic, metabolic, or miscellaneous causes
What are examples of hematologic disorders?
- Chronic hemolytic anemia
- Myeloproliferative disorders
- Splenectomy
What are examples of systemic disorders?
- Sarcoidosis
- Vasculitis
- Neurofibromatosis type 1
What are examples of metabolic disorders?
- Glycogen storage dz
- Gaucher disease
- Thyroid disease
What are examples of miscellaneous causes for group 5?
- Tumor embolization
- External compression of pulmonary vasculature
- End-stage renal disease on dialysis
What are signs & sx of pulmonary HTN?
- Asx for years
- # 1 = exertional dyspnea
- fatigue/weakness
- CP
- Syncope
- Nonproductive cough
- R ventricular hypertrophy & HF in advanced dz
What sx is rare but a life-threatening event for pulmonary HTN? What is it caused by?
Hemoptysis
- Caused by rupture of pulmonary artery
What is seen on PE for pulmonary HTN?
- JVD
- Paradoxical split of S2
- Accentuated pulmonary valve component of the 2nd heart sound
- R sided 3rd heart sound
- Tricuspid regurg murmur (at parasternal line)
- Strong R. ventricular impulse
- Hepatomegaly, abdominal distension, & ascites
- LE edema
What are other signs of pulmonary HTN?
Cyanosis due to shunting
How do you dx pulmonary HTN?
- EKG
- Elevated RV systolic pressure on echo
What can you use for dx of pulmonary HTN?
- Routine blood work
- Arterial blood gas
- Sleep study
- Screening for HIV & collagen vascular dz
What is the gold standard for dx of pulmonary htn? When should you perform this?
R-sided cardiac cath
- Perform prior to initiation of advance therapies
What is the transpulmonary gradient?
Drop in pressure across pulmonary circulation
What imaging is used for pulmonary HTN?
Radiographs & CTs of chest
- PFTs are useful in determining cause of htn for pts in Group 3
On PFTs, what combo suggests increased pulmonary arterial pressure?
- Decreased single-breath
- Normal FVC
- Normal TLC
- Increased wasted ventilation
What is considered the definitive dx procedure for defining the distribution & extent of disease in chronic thromboembolic pulmonary htn?
Pulmonary angiography
What test differentiates chronic thromboembolic pulmonary htn from idiopathic pulmonary arterial htn?
V/Q scan
What is 1st line therapy for group 1?
CCBs (Diltiazem, nifedipine)
*Should only be given to pts w/ + acute vasodilator response
How do you tx group 3?
Supplemental O2 for 15 hrs or more per day
How do you tx group 2 & 5?
Treat underlying condition
How do you tx group 4?
- Anticoag
- Thromboendarterectomy if no response to other therapies
What phosphodiesterase 5 inhibitors have been approved for the tx of pulmonary htn?
- Sildenafil
- Tadalafil
What is the MOA of phosphodiesterase 5 inhibitors?
cGMP accumulation –> decreased muscular tone
What is contraindicated w/ PDE-5 inhibitors?
Any drug serving as a nitric oxide donor
How do you tx the sx of pulmonary htn?
- Diuretics: Furosemide
- Warfarin (or ASA in children)
- O2
- Exercise
Pregnant women w/ PAH, should be tx w/ what?
A prostanoid
Define: Cor Pulmonale
RV alteration due to pulmonary dz &/or hypoxia that may progress to RV failure
What are the etiologies of cor pulmonale?
- COPD
- Pulmonary htn
- idiopathic pulmonary fibrosis
- thromboembolic disease
- ARDs
- Pneumoconiosis or kyphoscoliosis (less common)
Cor pulmonale has ____ pulmonary vasoconstriction & ____ pulmonary vascular bed.
- Increased
- Decreased
Define: pneumoconiosis
- Inhaled dust deposited deep in lungs
- An occupational lung dz
Pneumoconiosis includes what subcategories?
- Coal workers’ pneumoconiosis (black lung dz)
- Asbestosis
- Silicosis