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Respiration All my notes > Pulmonary Hypertension > Flashcards

Pulmonary Hypertension Flashcards

1
Q

Question 1:
What is pulmonary hypertension, and what is the defining value for mean pulmonary artery pressure (mPAP) in this condition?

A

Answer:

Pulmonary hypertension is high blood pressure within the pulmonary circulation.

The defining value for mean pulmonary artery pressure (mPAP) in pulmonary hypertension is ≥ 25 mmHg.

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2
Q

Question 2:
Differentiate between pulmonary hypertension and systemic hypertension.

A

Answer:

  • Pulmonary hypertension is high blood pressure within the pulmonary circulation, while
  • systemic hypertension is high blood pressure within the systemic circulation.
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3
Q

Question 3:
What is Type I Pulmonary Artery Hypertension, and what are its key characteristics?

A

Answer:

  • Type I Pulmonary Artery Hypertension is characterized by super vasoconstriction in the pulmonary vessels, leading to very high pulmonary vascular resistance.
  • This results in an increase in mean pulmonary artery pressure (mPAP ≥ 25 mmHg) and puts strain on the right heart, leading to right ventricular hypertrophy and eventually right heart failure.
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4
Q

Question 4:
What are the factors responsible for intense vasoconstriction in Type I Pulmonary Artery Hypertension?

A

Answer:

  • intense vasoconstriction is caused by endothelin-1 (ET-1), a potent vasoconstrictor produced by endothelial cells.
  • ET-1 causes smooth muscle contraction, leading to decreased vessel diameter, increased vasoconstriction, and increased peripheral vascular resistance.
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5
Q

Question 5:
What are the factors responsible for decreased vasodilation in Type I Pulmonary Artery Hypertension?

A

Answer:
* result of reduced production of nitric oxide (NO) and prostacyclin (PGI2).

  • Both NO and PGI2 act on smooth muscle and cause relaxation, increasing vessel diameter and decreasing peripheral vascular resistance.
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6
Q

Question 6:
What are the etiological factors for Type I Pulmonary Artery Hypertension?

A

Answer:

(i) Idiopathic (most common, diagnosed by exclusion)
(ii) Hereditary - caused by the BMRR2 gene, leading to increased ET-1 production and decreased NO and PGI2 production
(iii) Connective tissue diseases, such as systemic lupus erythematosus (SLE) and scleroderma

(iv) HIV (mechanism not well-known)

(v) Porto-pulmonary hypertension, often seen in patients with cirrhosis, where liver failure leads to vasoactive molecules not being cleared, causing vasoconstriction and decreased vasodilation.

(vi) Left-to-right shunts, especially observed in infants with large ventricular septal defects (VSD), atrial septal defects (ASD), and patent foramen ovale.

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7
Q

Question 1:
What are the two primary pathophysiological changes in pulmonary hypertension?

A

Answer:
increased vasoconstriction processes and decreased vasodilation processes.

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8
Q

Question 2:
What is the consequence of increased peripheral vascular resistance in pulmonary hypertension?

A

Answer:
Increased peripheral vascular resistance in pulmonary hypertension leads to an increase in pulmonary artery pressure.

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9
Q

Question 3:
How does pulmonary hypertension affect the right heart?

A

Answer:
Pulmonary hypertension puts strain on the right heart, leading to right ventricular hypertrophy and eventually right heart failure.

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10
Q

Question 4:
List the different causes of pulmonary hypertension.

A

Answer:

(i) Idiopathic (most common)
(ii) Hereditary
(iii) Connective tissue diseases
(iv) HIV
(v) Porto-pulmonary hypertension
(vi) Left-to-right shunting

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11
Q

Question 5:
What is Type II Pulmonary Artery Hypertension, and what are its common causes?

A

Answer:

  • Type II Pulmonary Artery Hypertension is caused by left heart disease, including conditions such as left heart failure and valvular heart disease.
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12
Q

Question 6:
What is the pathophysiological mechanism behind Type II Pulmonary Artery Hypertension?

A

Answer:

  • In Type II Pulmonary Artery Hypertension, left heart disease causes an increase in pulmonary venous backflow from the left heart into the pulmonary vein.
  • This leads to congestion in the pulmonary venous circulation, increasing pulmonary artery pressure and straining the right heart, ultimately resulting in right ventricular hypertrophy and right heart failure.
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13
Q

Question 7:
Which lung diseases are associated with Type III Pulmonary Artery Hypertension?

A

Answer:
Type III Pulmonary Artery Hypertension is associated with underlying lung diseases, including:
(i) COPD (Chronic Obstructive Pulmonary Disease)
(ii) Interstitial lung disease
(iii) Obstructive sleep apnea
(iv) Obesity hypoventilation syndrome

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14
Q

Question 8:
Explain the pathophysiological process of Type III Pulmonary Artery Hypertension in patients with lung diseases.

A

Answer:

  • In Type III Pulmonary Artery Hypertension, inadequate ventilation in multiple lung parenchymal areas leads to hypoxic vasoconstriction.
  • This causes pulmonary vessels to constrict, resulting in increased pulmonary vascular resistance and pulmonary artery pressure.
  • The right heart is strained, leading to right ventricular hypertrophy and eventual right heart failure.
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15
Q

Question 1:
What are the key characteristics of Type IV Pulmonary Artery Hypertension, and what is its primary cause?

A

Answer:

  • Type IV Pulmonary Artery Hypertension is characterized by an increase in peripheral vascular resistance due to chronic pulmonary emboli (distal blood clots).
  • Unlike Type III, which is due to hypoxia, Type IV’s increased vascular resistance is caused by clots obstructing blood flow.
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16
Q

Question 2:
Explain the difference between vasoconstriction in Type I and Type IV Pulmonary Artery Hypertension.

A

Answer:
In Type I Pulmonary Artery Hypertension, vasoconstriction is due to increased levels of endothelin-1, while in Type IV, vasoconstriction occurs due to clots obstructing blood flow.

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17
Q

Question 3:
Which lung diseases are associated with Type V Pulmonary Artery Hypertension?

A

Answer:
Type V Pulmonary Artery Hypertension is associated with lung diseases such as sarcoidosis, where granulomas compress the pulmonary vessels, and mediastinal tumors (e.g., tracheal, bronchial tumor, lymphoma) that compress the pulmonary artery.

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18
Q

Question 4:
How does Type V Pulmonary Artery Hypertension affect the right heart?

A

Answer:
Type V Pulmonary Artery Hypertension increases pulmonary artery pressure, putting strain on the right heart, leading to right ventricular hypertrophy, and eventually, right heart failure.

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19
Q

Question 5:
Summarize the primary causes and key characteristics of each type of Pulmonary Artery Hypertension.

A

Answer:

Type I: Idiopathic, increased vasoconstrictors (endothelin-1), decreased vasodilators (NO and PGI2).
Type II: Left heart disease, resulting in pulmonary venous backflow congestion.
Type III: Lung diseases causing hypoxic vasoconstriction.
Type IV: Chronic pulmonary emboli leading to increased peripheral vascular resistance due to clots.
Type V: Sarcoidosis or mediastinal tumors compressing the pulmonary artery.

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20
Q

Question 1:
What are the classic clinical features of right heart failure in patients with pulmonary hypertension?

A

Answer:

  • Jugular venous distention due to blood backing up into the superior vena cava and jugular vein.
  • Ascites and hepatomegaly due to blood backing up into the liver through the inferior vena cava.
  • Pedal edema caused by blood accumulating in the interstitial spaces of the lower extremities.
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21
Q

Question 2:
How do auscultation findings differ between patients with systemic hypertension and pulmonary hypertension?

A

Answer:

  • In patients with systemic hypertension, severe hypertension causes the aortic valve to snap hard, leading to a loud second heart sound (S2) with a particularly pronounced A2 component.
  • In contrast, in patients with pulmonary hypertension, the high pulmonary pressure causes the pulmonary valve to close quickly during diastole, resulting in a loud S2 with a particularly pronounced P2 component.
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22
Q

Question 3:
What is the primary cause of exertional dyspnea in patients with pulmonary hypertension?

A

Answer:

  • Exertional dyspnea in patients with pulmonary hypertension is primarily due to the high pulmonary artery pressure,
  • which leads to reduced blood flow through the pulmonary arterial circulation and decreased delivery of oxygen to the left heart,
  • resulting in less left ventricular cardiac output and decreased oxygen delivery to the tissues.
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23
Q

Question 4:
Why may patients with pulmonary hypertension experience exertional syncope?

A

Answer:

  • Patients with pulmonary hypertension may experience exertional syncope due to inadequate oxygen delivery to the brain tissue during exertion, resulting from the high pulmonary artery pressure and reduced cardiac output.
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24
Q

Question 5:
What is the most common exertional symptom in patients with pulmonary hypertension?

A

Answer:
Exertional dyspnea often leading to misdiagnosis as chronic heart failure or coronary artery disease.

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25
Q

Question 1:
What are the typical symptoms that should raise suspicion for pulmonary artery hypertension?

A

Answer:
* exertional dyspnea, exertional syncope, and exertional angina.

  • Additionally, evidence of right heart failure due to high pulmonary pressure, such as right ventricular hypertrophy, should be considered.
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26
Q

Question 2:
What are the key classification criteria used to differentiate types of pulmonary artery hypertension?

A

Answer:

Type I: Increased vasoconstrictors and decreased vasodilators.
Type II: Left heart disease.
Type III: Lung disease.
Type IV: Chronic pulmonary emboli.
Type V: Compression due to mediastinal tumor or sarcoidosis.

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27
Q

Question 3:
What is the primary purpose of performing an echocardiogram in the diagnostic approach to pulmonary artery hypertension?

A

Answer:
* to rule out Type II Pulmonary Artery Hypertension, which is associated with left heart disease.

* The echocardiogram can identify wall motion abnormalities, reduced ejection fraction, and valvular heart disease, and it helps to assess the right heart's structure and function.
28
Q

Question 4:
What are the expected echocardiogram findings in patients with high pulmonary artery pressure?

A

Answer:
* In patients with high pulmonary artery pressure, an echocardiogram is expected to show right ventricular hypertrophy initially.

  • Over time, evidence of right heart failure may become apparent, such as right ventricular dilation and right atrial enlargement.
29
Q

Question 5:
Why might patients with pulmonary artery hypertension have exertional dyspnea?

A

Answer:
due to high pulmonary artery pressure, leading to reduced blood flow through the pulmonary arterial circulation, decreased oxygen delivery to the left heart, and subsequently, less left ventricular cardiac output and oxygen delivery to the tissues.

30
Q

Question 1:
What are the significant findings on the echocardiogram that suggest high right-sided pressures in pulmonary artery hypertension?

A

Answer:

  • right ventricular dilation, a little bit of hypertrophy, and significant right atrial enlargement.
  • The appearance of a plethoric inferior vena cava is also supportive of high right-sided pressure.
31
Q

Question 2:
Why is a V/Q (Ventilation/Perfusion) scan preferred over a CTPA (Computed Tomography Pulmonary Angiography) in ruling out Type IV pulmonary artery hypertension due to chronic pulmonary emboli?

A

Answer:

V/Q scans have been shown to be more effective in detecting chronic distal pulmonary emboli.

32
Q

Question 3:
How does a high-resolution CT scan aid in the diagnostic approach to pulmonary artery hypertension?

A

Answer:

  • A high-resolution CT scan is helpful in ruling out Type III pulmonary artery hypertension and Type V pulmonary artery hypertension caused by mediastinal tumors or sarcoidosis.
  • It provides a detailed image of the lung parenchyma and mediastinum, allowing for the identification of pruning of the pulmonary arteries and dilation of the pulmonary arteries due to high pulmonary artery pressures.
33
Q

Question 4:
What are the common features that may be observed in a chest X-ray regarding pulmonary artery hypertension?

A

Answer:

  • A chest X-ray may show features similar to a CT scan, but it might not be as detailed.
  • It can help identify cardiomegaly, enlargement of the pulmonary arteries, and signs of right heart strain.
34
Q

Question 5:
Which diagnostic tests are helpful in ruling out specific types of pulmonary artery hypertension?

A

Answer:

  • Echocardiogram: Rules out Type II pulmonary artery hypertension and indicates right ventricular dilation and right atrial enlargement.
  • V/Q Scan: Rules out Type IV pulmonary artery hypertension due to chronic pulmonary emboli.
  • High-Resolution CT Scan: Helps rule out Type III and Type V pulmonary artery hypertension, as it provides detailed imaging of lung parenchyma and mediastinum.
35
Q

Question 1:
What findings can be seen on chest X-rays or high-resolution CT scans in patients with pulmonary artery hypertension?

A

Answer:
pruning of the pulmonary arteries (dilation) due to extremely high pulmonary artery pressure.

36
Q

Question 2:
Why is a high-resolution CT scan preferred over a chest X-ray in the diagnostic approach to pulmonary artery hypertension?

A

Answer:
because it provides more detailed imaging and allows for the evaluation of mediastinal tumors, sarcoidosis, COPD (obstructive lung disease), and interstitial lung disease (restrictive lung disease).

37
Q

Question 3:
What is the role of a polysomnogram (sleep study) in the diagnostic approach to pulmonary artery hypertension?

A

Answer:
A polysomnogram helps rule out Type III pulmonary artery hypertension caused by obstructive sleep apnea (OSA) or possibly associated obesity hypoventilation.

38
Q

Question 4:
How can pulmonary function tests (PFTs) help differentiate between obstructive lung disease, restrictive lung disease, and pulmonary hypertension?

A

Answer:

Obstructive lung disease: FEV1/FVC ratio < 70%, high total lung capacity (TLC), and residual volume (RV), and low DLCO (diffusing capacity of the lung for carbon monoxide).

Restrictive lung disease: FEV1/FVC ratio ≥ 70%, very small TLC, RV, and functional vital capacity (FVC), and low DLCO.

  • Pulmonary hypertension: Normal FEV1/FVC ratio, normal TLC and RV, and decreased DLCO.
39
Q

Question 5:
What does a decreased DLCO on PFTs suggest in patients with pulmonary hypertension?

A

Answer:

  • A decreased DLCO (diffusing capacity of the lung for carbon monoxide) on pulmonary function tests (PFTs) suggests pulmonary hypertension.
  • In the context of normal FEV1/FVC ratio, TLC, and RV, a decreased DLCO points toward the presence of pulmonary hypertension rather than obstructive or restrictive lung diseases.
40
Q

Question 1:
What are some findings on an EKG that may suggest right heart strain in patients with pulmonary artery hypertension?

A

Answer:
right ventricular enlargement, right ventricular hypertrophy, and right bundle branch block.

41
Q

Question 2:
Which diagnostic tests help rule out specific causes of pulmonary artery hypertension?

A

Answer:

  • Liver function test (LFT): Rules out port-pulmonary hypertension due to cirrhosis.
  • HIV test: Helps rule out HIV-related pulmonary artery hypertension.
  • ANA (Antinuclear Antibody) test: Provides information about systemic lupus erythematosus.
  • Scleroderma antibodies: Assists in diagnosing scleroderma.
42
Q

Question 3:
What is the role of right heart catheterization in the diagnostic approach to pulmonary artery hypertension?

A

Answer:

  • Right heart catheterization is performed to confirm the diagnosis of pulmonary artery hypertension after ruling out other types (Type II-V) and other potential causes.
  • It involves measuring the pulmonary artery pressure and pulmonary capillary wedge pressure to calculate the transpulmonary pressure gradient.
43
Q

Question 4:
What does a high transpulmonary pressure gradient suggest in the context of pulmonary artery hypertension?

A

Answer:

  • A high transpulmonary pressure gradient, indicated by a high pulmonary artery pressure (mPAP ≥ 25mmHg) and low pulmonary capillary wedge pressure (< 15mmHg), suggests pulmonary artery hypertension.
  • This finding supports the diagnosis of Type I pulmonary artery hypertension, which is idiopathic or heritable in nature.
44
Q

Question 5:
What diagnostic information does a high pulmonary capillary wedge pressure provide?

A

Answer:

indicative of left atrial pressure and suggests Type II pulmonary hypertension, which is associated with left heart disease.

45
Q

Question 1:
What is the primary treatment approach for Type I pulmonary artery hypertension?

A

Answer:
focused on managing the underlying causes and associated conditions, such as HIV, porto-pulmonary hypertension, left-to-right shunt, scleroderma, and systemic lupus erythematosus.

46
Q

Question 2:
How are other types of pulmonary artery hypertension (Type II-V) treated?

A

Answer:

Type II: Treating the underlying left heart disease.
Type III: Treating the underlying lung disease.
Type IV: Treating the underlying chronic pulmonary emboli.
Type V: Treating the underlying sarcoidosis or mediastinal tumor.

47
Q

Question 3:
What is the rationale behind focusing on Type I pulmonary artery hypertension treatment?

A

Answer:

  • Type I pulmonary artery hypertension is idiopathic or heritable, and there may not be any specific underlying condition to target.
  • Thus, the treatment focuses on managing associated conditions that can contribute to pulmonary artery hypertension, such as HIV, porto-pulmonary hypertension, left-to-right shunt, scleroderma, and systemic lupus erythematosus.
48
Q

Question 1:
What is the primary treatment approach for idiopathic Type I pulmonary artery hypertension?

A

Answer:
reducing intense vasoconstriction and increasing vasodilation to decrease pulmonary vascular resistance, lower pulmonary artery pressure, and reduce the risk of right heart failure.

49
Q

Question 2:
How is vasoreactivity tested in patients with idiopathic Type I pulmonary artery hypertension?

A

Answer:

  • Vasoreactivity is tested by giving patients vasodilators and assessing the response.
  • The goal is to determine if the vasodilator effectively dilates the pulmonary vessels, reducing mean pulmonary artery pressure.
50
Q

Question 3:
What are the criteria for a positive vasoreactive test result in idiopathic Type I pulmonary artery hypertension?

A

Answer:

  • A positive vasoreactive test result includes meeting all three criteria:
  • Mean pulmonary artery pressure (mPAP) (absolute) < 40mmHg.
  • mPAP decrease > 10mmHg from the initial value after vasodilator administration.
  • Right ventricular cardiac output increases or stays the same.
51
Q

Question 4:
What are the treatment options for patients with idiopathic Type I pulmonary artery hypertension who have a negative vasoreactive test?

A

Answer:

  • For patients with a negative vasoreactive test, treatment aims to increase vasodilation and decrease vasoconstriction.
  • This can be achieved through the use of medications that inhibit endothelin-1 receptors (endothelin-1 inhibitors -sentan), increase prostacyclin (prostacyclin analogs -prost-), or mimic nitric oxide action (PDE inhibitors -afil).
52
Q

Question 5:
What precautions should be taken when using PDE inhibitors (phosphodiesterase inhibitors) to treat pulmonary artery hypertension?

A

Answer:

When using PDE inhibitors like Sildenafil and Tadalafil to treat pulmonary artery hypertension, avoid giving nitrates or α-blockers simultaneously, as this can lead to a significant drop in blood pressure due to excessive vasodilation.

53
Q

Question:
What is the last resort treatment option for refractory pulmonary artery hypertension?

A

Answer:
a bilateral lung and heart transplant.

This procedure involves removing the affected pulmonary vasculature from the lungs and replacing them with healthy ones, along with a fresh right heart to alleviate the severe impact of the disease.

54
Q

Question 1:
What are the two main consequences of pulmonary hypertension?

A

Answer:

  • Strain on the right side of the heart trying to pump blood through the lungs.
  • Back pressure of blood into the systemic venous system.
55
Q

Question 2:
Why does the pulmonary system have a low incidence of atherosclerosis?

A

Answer:
due to its low pressure.

56
Q

Question 3:
What are some causes of pulmonary venous hypertension?

A

Answer:
Left ventricular systolic dysfunction
Mitral regurgitation or stenosis
Cardiomyopathy (e.g., alcohol, viral)

57
Q

Question 4:
What are the main types of pulmonary arterial hypertension?

A

Answer:
* Primary arterial hypertension (idiopathic or genetic).

  • Secondary arterial hypertension (occurs due to underlying diseases or known risk factors, such as heart disease or lung fibrosis).
58
Q

Question 1:
What are the symptoms of pulmonary hypertension?

A

Answer:
Fatigue
Dyspnoea (shortness of breath)
Chest pain
Central cyanosis if hypoxic

59
Q

Question 2:
What are the signs of pulmonary hypertension?

A

Answer:
Dependent oedema
Elevated JVP (jugular venous pressure)
Right ventricular heave at the left parasternal edge
Murmur of tricuspid regurgitation
Loud P2 (second heart sound)
Enlarged liver (pulsatile)

60
Q

Question 3:
What investigations can be done to diagnose pulmonary hypertension?

A

Answer:

  • ECHO doppler to estimate systolic pulmonary arterial pressure
    Right heart catheterization to confirm the diagnosis
  • ECG (may show right ventricular hypertrophy, right axis deviation, and right bundle branch block)
  • CXR (may show cardiomegaly, dilated pulmonary arteries, and right ventricular hypertrophy)
  • NT-proBNP blood test (indicates right ventricular failure)
  • Ascultation may reveal a split second heart sound with a loud pulmonary component.
61
Q

Question 4:
How is primary pulmonary hypertension treated?

A

Answer:
with medications such as IV prostanoids (e.g., epoprostenol),

  • endothelin receptor antagonists (e.g., macitentan and bosentan), and
  • phosphodiesterase-5 inhibitors (e.g., sildenafil).
62
Q

Question 5:
How is secondary pulmonary hypertension managed?

A

Answer:
Secondary pulmonary hypertension is managed by treating the underlying cause, such as pulmonary embolism or systemic lupus erythematosus (SLE).

63
Q

Question 1:
What is cor pulmonale?

A

Answer:

  • a condition of right heart disease that is secondary to lung disease.
  • It occurs due to chronic lung diseases such as COPD, fibrotic lung disease, or chronic ventilatory failure (e.g., obesity hypoventilation syndrome).
64
Q

Question 2:
What causes cor pulmonale?

A

Answer:

  • Cor pulmonale is caused by lung diseases that lead to pulmonary hypertension.
  • For example, in COPD, there is a mismatch between ventilation and perfusion (V/Q ratio).
  • Hypoventilated alveoli cause vasoconstriction in the adjacent pulmonary vessels, leading to increased pressure on the pulmonary artery and subsequent pulmonary hypertension.
65
Q

Question 3:
What are the pathological changes in cor pulmonale?

A

Answer:

  • right ventricular hypertrophy and dilation due to increased afterload caused by pulmonary hypertension.
  • This can eventually lead to right-sided heart failure.
66
Q

Question 4:
Which lung diseases are commonly associated with cor pulmonale?

A

Answer:

Cor pulmonale is commonly associated with chronic lung diseases such as COPD (Chronic Obstructive Pulmonary Disease), severe bronchiectasis, chronic bronchitis, and emphysema.

67
Q

Question 5:
How does pulmonary hypertension contribute to cor pulmonale?

A

Answer:

  • Pulmonary hypertension increases the afterload on the right ventricle, leading to right ventricular hypertrophy and dilation.
  • As a result, the right heart becomes less efficient in pumping blood, eventually leading to right-sided heart failure in cor pulmonale.

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