304 Pulmonary Hypertension

Question 1

Define Pulmonary Hypertension

Answer 1

It is a spectrum of diseases involving the pulmonary vasculature, and is defined as an elevation in pulmonary
arterial pressures (mean pulmonary artery pressure >22 mmHg).

Question 2

Define Pulmonary Arterial Hypertension (PAH)

Answer 2

It is a relatively rare form of PH and is characterized by symptoms of dyspnea, chest pain, and syncope.

Question 3

As the resting PVR increases, there will be a corresponding increase in mean pulmonary artery pressure (PAP) until the cardiac output (CO) is compromised and starts to fall. What happens next?

Answer 3

With a decline in CO, the PAP will fall. As CO declines as a result of increased afterload and decreased contractility, tachycardia is a compensatory response. Tachycardia decreases filling time and, thus, preload, and results in a reduced fraction of stroke volume available to distend the pulmonary vascular tree.

Question 4

Abnormalities in multiple molecular pathways and genes- what are they?

Answer 4

decreased expression of the voltage-regulated potassium channel, mutations in the bone morphogenetic protein receptor-2, increased tissue factor expression, overactivation of the serotonin transporter, hypoxia-induced activation of hypoxia-inducible factor-1α, and activation of nuclear factor of activated T cells.

Question 5

Symptoms of Pulmonary Hypertension?

Answer 5

dyspnea and/or fatigue, whereas edema, chest pain,

presyncope, and frank syncope are less common and associated with more advanced disease.

Question 6

Physical examination in Pulmonary Hypertension?

Answer 6

On examination, there may be evidence of right ventricular failure with elevated jugular venous pressure, lower extremity edema, and ascites. Additionally, the cardiovascular examination may reveal an accentuated P2 component of the second heart sound, a right-sided S3 or S4, and a holosystolic tricuspid regurgitant murmur.

Question 7

Findings in Ecocardiography in PH?

Answer 7

All forms of PH may demonstrate a hypertrophied and dilated right ventricle with elevated estimated pulmonary
artery systolic pressure.

Question 8

Findings in chest x-ray in PH?

Answer 8

A sign of PH that may be evident on chest x-ray include enlargement of the central pulmonary arteries associated with “vascular pruning,” a relative paucity of peripheral vessels. Cardiomegaly, with specific evidence of right atrial and ventricular enlargement, can often be observed. The chest x-ray may also demonstrate significant interstitial lung disease or suggest hyperinflation from obstructive lung disease, which may be the underlying cause or contributor to the development of PH.

Question 9

What other chages can you find in CT?

Answer 9

CT may also reveal signs of venous congestion including centrilobular ground-glass infiltrate and thickened septal lines.

Question 10

Lab tests in PH?

Answer 10

HIV; antinuclear antibodies, rheumatoid factor, and scl-70; liver function and hepatitis serology tests; brain natriuretic peptide (BNP) and the N-terminus of its propeptide (NT-proBNP)

Question 11

Criteria for the diagnosis of PH?

Answer 11

(1) mPAP more or equal to 25 mmHg; (2) a pulmonary capillary wedge pressure (PCWP), left atrial pressure, or left ventricular end-diastolicpressure less or equal to 15 mmHg; and (3) PVR more than 3 Wood units. Postcapillary PH is differentiated from precapillary PH by a PCWP of more or equal to 15 mmHg; this is further differentiated into passive, based on a transpulmonary gradient
less than 12 mmHg, or reactive, based on a transpulmonary gradient more than 12 mmHg and an increased PVR.

Question 12

Vasodilators in PH

Answer 12

inhaled nitric oxide, inhaled epoprostenol, or intravenous adenosine,

Question 13

% of patients that are vasoreactive during testing

Answer 13

Less than 12%

Question 14

What is a positive pulmonary vasodilator response?

Answer 14

A decrease in mPAP by more than or equal to 10 mmHg to an absolute level of less than or equal to 40 mmHg without a decrease in CO

Question 15

What is the current classification system, last revised in 2013 during the Fifth World Symposium on Pulmonary
Hypertension?

Answer 15

five categories of PH, including PAH, PH due to left heart disease, PH due to chronic lung disease, PH associated with chronic thromboemboli, and a group of miscellaneous diseases that only rarely cause PH.

Question 16

WHO Group I PH

Answer 16

group of diseases that result in pulmonary arterial precapillary remodeling marked by intimal fibrosis, increased medial thickness, pulmonary arteriolar occlusion, and classic plexiform lesions.

Question 17

average age at diagnosis of PH; % of patients with IPAH over the age of 60/70 at diagnosis

Answer 17

36 years (45 in other series);
9% (60) and 8,5% (70)

Question 18

Characterize HIV associated PH

Answer 18

Although HIV is a rare cause of PAH, this form of PAH
is indistinguishable from IPAH and is an important cause of mortality in the HIV-infected population. Importantly, there is no correlation between the stage of HIV infection and the development of PAH.

Question 19

% of patients with established portal hypertension that develop portopulmonary hypertension.

Answer 19

2–10

Question 20

WHO Group II PH

Answer 20

patients with left heart systolic failure, aortic and mitral valve disease, and heart failure with preserved ejection fraction (HFpEF)

Question 21

Diagnosis of reactive pulmonary arterial vasculopathy due to chronic pulmonary venous hypertension.

Answer 21

elevated transpulmonary gradient (more than 12 mmHg) and elevated PVR(more than 3 Wood units)

Question 22

What id the second most common cause of PH?

Answer 22

Intrinsic lung disease

Question 23

Diseases of Group III?

Answer 23

chronic obstructive lung disease and interstitial lung disease. It can also be seen in diseases with mixed obstructive/restrictive physiology: bronchiectasis, cystic fibrosis, mixed obstructive restrictive disease marked by fibrosis in the lower lung zones, and emphysema predominantly in the upper lung zones. It can also be seen in sleep-disordered breathing.