Cor Pulmonale & Pulmonary HTN

Question 1

Pulm circulation

general

Answer 1

Pulmonary circulation
The path of blood from the right side of the heart (right ventricle) through arteries to the lungs tobe re-saturated with oxygen to return to the heart (left atrium) through pulmonary veins

Systemic circulation
The path of oxygen-rich blood from the heart (left ventricle) to all organ systems to supply nutrients

Blood flow through systemic circulation = blood flow through pulmonary circulation

Normally, systemic blood pressure > pulmonary blood pressure
Pulmonary artery pressure 25/10 mm Hg with a mean arterial pressure of 15 mm Hg

Question 2

Cor Pulmonale

General

RIGHT SIDED HF

Answer 2

**Also known as pulmonary heart disease

Characterized by structural alteration and dysfunction of the right ventricle (RV) caused byprimary lung disease
Can be acute or chronic:
Acute cor pulmonaleis most commonly caused by massivepulmonary embolism

Chronic cor pulmonalehas a slow and progressive course resulting from underlying worsening lung disease

Etiology
Develops from increased right-sided filling pressures from long-termpulmonary hypertension(PH)associated with lung disease

structueral changes in RV that causes it not to function properly due to pulm HTN

Question 3

cor pulmonale

etiolgy

Answer 3

Develops from increased right-sided filling pressures from long-termpulmonary hypertension (PH)
A disease process normally leads to pulmonary hypertension

Question 4

Pulmonary hypertension

Answer 4

Pulmonary hypertensionis defined as:
Increased blood pressure in pulmonary circulation
Meanpulmonary arterial pressure of ≥ 25mmHg at rest or ≥ 30 during exercise (measured by right heart catheterization); normal mean pulmonary arterial pressure = 14–20mm Hg
Pulmonary hypertension is classified into one of five WHO (World Health Organization) groups

Question 5

which gourps d not lead to R HF

Answer 5

group 2 leads to L sided HF
group 1 from congential defects

Question 6

Cor Pulmonale & Pulmonary hypertension –

Pathophysiology

Answer 6

Hypoxic pulmonaryvasoconstriction
Tolimitblood flowto hypoxicalveoli, low oxygen leads to pulmonary vasoconstriction, diverting blood to the better-ventilatedalveoli

Pulmonary vascular remodeling
Vascular alterations occur: neomuscularization ofarterioles, intimal thickening, medialhypertrophy

The 2 factors above lead toincreased pulmonary vascularresistance (PVR) → pulmonary hypertension
Asresistanceincreases, as doespulmonary arterypressure and, eventually,RV afterload
RV adapts to slowly increasingpulmonary arterypressure initially by dilation → leads tohypertrophy
Consequent effect is RV dysfunction and failure

Question 7

Question 8

WHO Group 1

Answer 8

Cases of increased pulmonary arterial pressure in the absence of underlying heart or lung disease
Pulmonary arterial hypertension, which is caused when the arteries in the lungs become narrowed, thickened, or stiff
The right side of the heart must work harder to push blood through these narrowed arteries
The heart will lose its ability to pump enough blood through the lungs to meet the needs of the rest of the body

Question 9

WHO Group 1

causes

Answer 9

Causes:
Idiopathic
Inherited
Medical conditions: congenital heart disease, liver disease, HIV, connective tissue diseases (scleroderma)
Medications (past or present use): methamphetamine, cocaine

Question 10

WHO Group 2

general

Answer 10

Cases of pulmonary hypertension due to left heart disease
Problems with how the heart squeezes or relaxes, or problems with the valves on the left side of the heart

The left heart is unable to keep up with the blood returning from the lungs — causing a “backup” of blood in the pulmonary veins and capillary beds which raises pressure in the pulmonary artery

WHO Group 2 is the most common form of pulmonary hypertension

have pulm HTN but no cor pulmonale

most common group

Question 11

WHO Group 3

general

Answer 11

Cases of pulmonary hypertension due to chronic lung disease and/or hypoxia

To adapt to the lung disease, arteries in the lungs constrict so that blood can only go to areas of the lungs that are receiving the most air and oxygen (preventing a ventilation-perfusion mismatch)
Constriction leads to high blood pressure throughout the lungs

Right side of the heart must generate increased pressure to pump blood to the lungs

Question 12

WHO Group 3

causes

Answer 12

Causes:
Obstructive lung disease (COPD – most common)
Restrictive lung disease (interstitial lung disease)
Sleep-disordered breathing (Obstructive sleep apnea)
Chronic exposure to high altitude

Question 13

Question 14

WHO Group 4

general

Answer 14

Cases of pulmonary hypertension due to chronic pulmonary arterial obstruction (embolus/fat/clot)

The body is not able to dissolve/resolve the obstruction in the lungs → scar tissue in the blood vessels of the lungs
Obstruction blocks normal blood flow and the scar tissue makes the right side of the heart work harder

Endothelial cells release histamine and serotonin which constricts the pulmonary arteries

Question 15

Who Group 4

causes

Answer 15

Causes:
Chronic pulmonary thromboembolism (blood clots)
Nonthrombotic pulmonary embolism
Tumorembolisms (late-stage manifestations of certain malignancies withembolizationoftumorparticles)
Foreign material (most commonly due to silicone injection)

Question 16

WHO group 5

General

Answer 16

Cases of pulmonary hypertension due to systemic disease, but the relationship is not clearly understood or thought to be multifactorial

Damage to the endothelial cells lining the pulmonary arteries

Damaged endothelial cells release endothelin-1, serotonin, and thromboxane
Constriction of the pulmonary arteries
Hypertrophy of the smooth muscle
Less production of nitric oxide and prostacyclin (pulmonary artery dilation and inhibition of smooth muscle hypertrophy)

Question 17

WHO Group 5

causes

Answer 17

Causes:
Hematological disorders: sickle cell anemia, thalassemia, splenectomy
Systemic disorders: sarcoidosis
Metabolic disorders: thyroid disorders
Other: chronic kidney disease

Question 18

Question 19

R sided failure

Clin man

Answer 19

Symptoms
Dyspnea
Underlyingpulmonary diseaseand resulting cor pulmonale
Chestpain
Increased oxygen demand and increased stress on the RV
Syncope
Inability to increasecardiac outputwith exertion or exercise

Peripheral edemaand increased abdominal girth fromright-sided heart failureand hepatic congestion
Anorexia,nausea/vomiting,right upper quadrant pain: from hepatic congestion
Additional symptoms the underlying cause

Question 20

WHO Functional class for pulmonary hypertension

Answer 20

Question 21

WHO classes 1-5

Answer 21

WHO Class I: do not suffer from the above symptoms when doing ordinary physical activity – such as climbing a flight of stairs, doing the dishes, bathing and cooking (not going for a run or going to the gym)

WHO Class II: when ‘at rest’ (sitting, laying down or standing still) there are no symptoms of breathlessness etc., however when carrying out ordinary physical activity (for example climbing a flight of stairs, shopping, or making the bed) patients suffer from any of the above symptoms

WHO Class III: when ‘at rest’ (sitting, laying down or standing still) there are no symptoms of breathlessness and so on, but there is difficulty doing general day-today activities such as household chores; patients must take breaks while carrying out daily living activities (climbing a flight of stairs, walking to the shops and so on)

WHO Class IV: suffer from symptoms even at rest (for example when sitting down) and will suffer from severe symptoms while doing any activity

Question 22

Cor pulmonale

CLin man

Answer 22

Signs
Extra heart sounds
S3 – ventricular volume overload and heart failure
S4 – ↑ resistance to ventricular filling due to ↓ ventricular compliance
Increased right-sided filling pressures:
Venous jugular distension
Right ventricular heave

Hepatic congestion from right-sided heart failure:
Hepatomegaly and/or pulsatile liver
Lower extremity edema
Ascites
Pleural effusion

Signs of hypoxemia:
Increased respiratory rate
Cyanosis
Clubbing

Question 23

cor pulmonale

Hepatic congestion from right-sided heart failure Sx

Answer 23

Hepatomegaly and/or pulsatile liver
Lower extremity edema
Ascites
Pleural effusion

Question 24

Answer 24

S3 can be normal for some, s4 is always pathologic

Question 25

cor pulmonale

X-ray, ekg, echo

Answer 25

Chest x-ray
Enlargement of the central pulmonary arteries and main branches
Cardiomegaly, pulmonary edema
Findings of the underlying lung disease
EKG
RV hypertension
Right atrial enlargement

Echocardiography
Best initial test
Ventricular and atrial size and function
Valve function
Rules out left-sidedheart failure

Question 26

Cor pulmonale

Right heart catheterization

Answer 26

Right heart catheterization
Gold standard to confirmpulmonary hypertension
Results:meanpulmonary arterial pressure(mPAP) ≥ 25 mm Hg

Question 27

cor pulmonal

Tx

Answer 27

Smoking cessation
Supplemental oxygen
Relieves pulmonaryvasoconstriction, thereby improvingcardiac outputand tissue perfusion

Treat the underlying cause!

For chronic rightheart failureor elevated RV filling pressures
Diuretics (monitor forvolume depletion)

Treatment of venousthromboembolism (WHO group 4)
Anticoagulants

Question 28