Basics

Question 1

What is a normal systolic PAP vs mean PAP

Answer 1

Mean PAP P: 10-20s
Systolic PAP: 20-30s

Question 2

Group 1-5 pulm htn

Answer 2

Group 1: Pulmonary arterial hypertension (PAH) secondary to various disorders
◦ Group 2: PH due to left heart disease
◦ Group 3: PH due to chronic lung disease & hypoxemia
◦ Group 4: PH associated with chronic thromboemboli
◦ Group 5: PH associated hematologic, systemic, metabolic and miscellaneous diseases

Question 3

Class 1-4 pulm htn

Answer 3

Class I Without limitation of physical activity. No dyspnea, fatigue chest pain or near syncope with
exertion
Class II Slight limitation of physical activity. No symptoms at rest but ordinary physical activity causes
dyspnea, fatigue, chest pain or near syncope
Class III Marked limitation of physical activity. No symptoms at rest but less than ordinary activity causes
dyspnea, fatigue, chest pain or near syncope
Class IV Inability to perform any physical activity without symptoms. Evidence of right heart failure,
dyspnea and fatigue at rest. Worsening symptoms with any activity

Question 4

What diseases an cause PAH

Answer 4

Pulmonary Hypertension
Group I
Pulmonary Arterial Hypertension (PAH)
◦ Group of diseases directly effecting pulmonary arteries
◦ Idiopathic PAH
◦ HIV
◦ Connective tissue disease

Question 5

What is a large complication of untreated PAH

Answer 5

High mortality if untreated
◦ Decompensated right heart failure

Question 6

Explain Group II PAH

Answer 6

Pulmonary Hypertension
Group II
Cardiac Etiology
◦ Left heart systolic dysfunction
◦ Aortic and mitral valve disease
◦ Heart failure with preserved ejection fraction
◦ Left heart disease
◦ Most common cause of PH in USA
Mechanism
◦ Elevated left atrial pressure
◦ Results in pulmonary venous hypertension
◦ Increases pulmonary arterial pressure

Question 7

Explain the mechanism of Group II PAH

Answer 7

Lung Function Etiology
◦ Intrinsic lung disease
◦ Chronic obstructive lung disease
◦ Interstitial lung disease
◦ Mixed obstructive/restrictive causes
◦ Sleep apnea
◦ Chronic hypoxemia
◦ Alveolar hypoventilation syndromes
◦ High-altitude exposure
Proposed Mechanism(s)
◦ Hypoxia
◦ Lung parenchyma damage

Question 8

Explain the mechanism of group IV PAH

Answer 8

Cause
◦ Chronic thromboembolism
Mechanism
◦ Occlusion of proximal & distal pulmonary arteries
◦ Body unable to dissolve clot
◦ Leads to scar tissue fibrosis in pulmonary arteries
◦ Blocks normal blood flow
◦ Subsequently leads to right side strain
◦ Pulmonary vasculature remodeling

Question 9

Explain the mechanism behind group V PAH

Answer 9

Causes
◦ Hematological
◦ Chronic hemolytic anemia
◦ Myeloproliferative disorders
◦ Splenectomy
◦ Systemic
◦ Sarcoid or vasculitis
◦ Metabolic
◦ Thyroid disease
◦ Miscellaneous causes
◦ Tumor embolization
◦ External compression of pulmonary vasculature
◦ ESRD on HD

Question 10

S/s of PAH

Answer 10

Signs & Symptoms
◦ Dyspnea & fatigue
◦ Edema, chest pain, presyncope/syncope
◦ RV failure, JVD, ascites, BLE edema
◦ Hepatomegaly
◦ Right sided S3 or S4
◦ Holosystolic tricuspid regurgitation murmur
◦ Hemoptysis
◦ Rare but life-threatening event
◦ Pulmonary artery rupture
◦ Cyanosis
◦ PFO & right to left shunt

Question 11

Lab, CT, and EKG findings indicative of PAH

Answer 11

Laboratory
◦ Abnormalities related to underlying disease
◦ Arterial blood gas
◦ Normal PaO2 at rest
◦ Hyperventilation w/ decrease PaCO2
EKG
◦ Right axis deviation & incomplete RBBB
◦ RV hypertrophy
◦ Peak P waves
◦ Right atrial enlargement
CT Imaging
◦ Enlargement right and left main pulmonary arteries
◦ Right ventricular & right atrial enlargement
◦ Advanced disease

Question 12

ECHO, RHC, and PFT results that indicate PAH

Answer 12

Echocardiogram
◦ Hypertrophied and dilated RV
◦ Elevated estimated pulmonary artery systolic pressures.
◦ Identification of specific etiologies
Right heart catheterization
◦ Gold standard for diagnosis
◦ Evaluate pulmonary pressure
Pulmonary Function Testing
◦ Identify restrictive or obstructive lung diseases as cause
6-minute walk test
◦ Degree of exertional
◦ Monitor progression & response

Question 13

RHC results that indicate PAH

Answer 13

ight heart cardiac catheterization
◦ Sustained elevation at rest PAP > 25 mmHg
◦ PVR >240 dynes/sec/cm
◦ Wedge pressure (PCWP) <15 mmHg
◦ Elevated transpulmonary gradient >12 mmHg
◦ Defining features of PAH
Right ventricular systolic pressure
◦ Mild 25-40 mmHg
◦ Moderate 41-55 mmHg
◦ Severe is the >55 mmHg
V/Q scan
◦ Differentiate chronic thromboembolism PH vs IPAH
Pulmonary angiography
◦ Defines distribution & extent chronic thromboembolic PH

Question 14

Group one PAH treatment plan

Answer 14

naging pulmonary hypertension itself
◦ Initiate management based on symptoms & functional status
◦ Calcium channel blockers
◦ Anticoagulation*
Group 1 Functional Class 2 Treatment
◦ Oral endothelin receptor antagonist (i.e. ambrisentan or macitentan)
◦ Phosphodiesterase inhibitors (i.e. sildenafil)
◦ Soluble guanylate cyclase stimulators (sGC) (i.e. riociguate)
◦ Prostacyclin analogs (epoprostenol)
Group 1 Functional Classes 3 & 4 Treatment
◦ Prostanoid agents (i.e. epoprostenol)

Question 15

Group 2 treatment plan in PAH

Answer 15

Group 2 Treatment
◦ Goal directed
◦ Decrease pulmonary venous pressure
◦ Manage heart failure & volume overload

Question 16

Group 3 treatment plan in PAH

Answer 16

Group 3 Treatment
◦ Supplemental 02
◦ 02 >15 hours per day shown to slow PH progression

Question 17

Group 4 tx plan in PAH

Answer 17

Group 4 Treatment
◦ Soluble guanylate cyclase stimulators (sGC) (i.e. riociguate)
◦ Anticoagulation
◦ Group 4 Functional Class 4
◦ Thromboendarterectomy

Question 18

Group 5 tx plan in PAH

Answer 18

Group 5 Treatment
◦ Manage underlying cause
All Groups
◦ Lung (and heart) transplant