Pulm V - p.HTTN, Cor Pulmonale, + Chronic PE

Question 1

Pulmonary HTTN - (pHTN or PH)

Answer 1

• abnormal elevation in mean pulmonary artery pressure (mPAP)
• Normal mPAP = 8-20 mmHg, measured via RT heart cath
• > 20 mmHg @ rest = high

Question 2

Etiology pHTTN

Answer 2

• MC of pHTN in adults is Lung Dz
• generally a feat of advanced dz
• important to determine underlying etiology

Question 3

Patho pHTTN

Answer 3

(3) ways pulmonary circulation can be affected:
1. Cx of INC Pulmonary Vascular Resistance (PVR);
- Occlusive vasculopathy, DEC SA of pulm vas bed (PE, inter lung dz), induction of hypoxic vasoconstriction (hypov syndrome or paryn lung dz)
2. Cx of INC flow;
- congenital heart defects w/ L-to-R shunts (ASD, VSD, PDA), liver cirrhosis, + anemia (inc in fluid production, to inc BP)
3. Cx of INC Pulmonary Venous Pressure;
- mitral v dz, LV systolic or dias dys, constrict pericarditis, restrictive cardiom, pulmonary venous obstruction

**Combo of chronic increase in flow and/or pulmonary venous pressure > inc PVR
>
pHTTN > decreased compliance of pulm vascul >
- progress inc in RV afterload -> RV hypertrophy
- RV dilation > DEC contract > DEC CO

Question 4

Group 1 - Pulmonary Artery HTTN (PAH)

Answer 4

[Etio]
1. Schistosomiasis*; 
parasitic flatworm, #1 cx WW 
(w/o worm) 
2. Idiopathic - 50%
3. Hereditary - 10%
[Epi]
*Rare, W>M, younger adults, W>AA>H>A
- SEVERE elevation in PAP 
[Patho]
*Increased PVR is the primary cx 
- abormalities in pulmonary vascular endothelial + smooth muscle cells causes: vasoconstrict, vascular prolif, thrombosis, + inflam
- May have some coagulation stuff

Question 5

Group 2 - due to LT heart dz

Answer 5

[Etio]
- HF p EF, HF r EF
- Valvular dz (**mitral>aortic)
- Congen/acquired cardio conditions > post-capillary pHTN
[Epi]
*LT sided HF MC cause of pHTN; 70% of G2 causes
[Patho]
*ELE LT heart filling pressures > increases in post-cap pressure > dec pulmonary arterial compliance - stiff

Question 6

Group 3 - chronic lung dz

Answer 6

[Etio]
- Obstructive; COPD, sleep apnea
- Restrictive 
- Other with mixed ^
- Hypoxia w/o lung dz (elev altitudes) 
- Dvp lung disorders 
[Epi] 
*typ have mild-mod (20-35 mPAP) elevation in PAP 
*MC cx
1. COPD 
2. Interstitial Lung DZ 
[Patho]
*ST hypoxia > immediate precapillary arteriole vasoconstrict (can be reserved with O2)
*CHRONIC > pulmonary vasoconstrict + vascular remodeling

Question 7

Group 4 - pulmonary artery obstruction

Answer 7

[Etio]

• Chronic thromboembolic pHTTN due > thromboembolic occlusion of the proximal or distal pulmonary A’s
• thromboe causes decease of SA of the pulm vas bed > inc PVR

Question 8

Group 5 - unclear multifactorial mech, unrelated to 1-4

Answer 8

[Etio]
Hematological - myeloproliferative dis
Systemic - sarcoidosis 
Metabolic disorders - glycogen storage dz 
Miscellaneous - Sickle Cell dz

Question 9

NYHA classification system of pHTTN

Answer 9

Class I: pHTTN w/o limitations of physical activity
Class II: resulting in slight limitations, no rest sx
Class III: resulting in marked limitations of PA, no rest syx
Class IV: inability to perform activites; SX @ rest, symptoms of RT HF

Question 10

Sx of pHTTN

Answer 10

*Non-specific
- DOE
- Angina pain
- Nonprod cough
- Malaise
- Fatigue
- Syncope > if related to insufficient CO
`Saddle PE, LV failure, longstanding pHTTN causing RV failure

Question 11

Signs of pHTTN

Answer 11

• **Hemoptysis: rare, but life-threat PA rupture (100% fatal)
• Cyanosis: R-t-L shunt in late dz
• Cardiac: angina pain, fixed split S2, RT s S3 (RT HF), TR murmur, MS murmur, MR murmur
• Elevated JVD: inc RT atria pressures
• Hepatomegaly: from portal vein congestion
• LL: edema, from venous congestion
• Lungs: unremarkable, may be wheezes and rales