Pulmonary Vascular Disease

Question 1

Pulmonary hypertension

vs

Pulmonary arterial hypertension

Question 2

Classification of pulmonary hypertension

Answer 2

• pre-capillary - pulmonary ARTERIAL hypertension
• primary respiratory
• post-capillary - most often - left sided heart dz

Question 3

WHO Classification of

Pulmonary Hypertension

Answer 3

I

• idiopathic/familial
• collagen vascular
• portal hypertension
• HIV
• toxins
• myloproliferative d/o
• uncorrected congenital heart disease

II pulmonary venous congestiosn - left sided heart

III hypoxic induced PH - ILD, COPD, etc

IV chronic thromboembolic disease

V misc - dz affecting vasculature directly - schistosomiasis, pulmonary capillary hemangiomatosis, sarcoidosis

Question 4

Definition of Pulmonary arterial HTN

Answer 4

Mean PAP > 25 mmHg

Mean PAP > 30 mmHg with exercise

Question 5

Pulmonary arterial hypertension

vs pulmonary hypertension

Answer 5

pulmonary ARTERIAL hypertension

• pre-capillary

Question 6

HIV is associated PAH

Answer 6

HIV is associated PAH

Question 7

CXR of pulmonary arterial hypertension

Answer 7

• enlargement of central pulmonary arteries
• but with peripheral pruning
• right interlobar artery > 17mm
• calcificatiion of the pulmonary artery
• right ventricular enlargement
• Parenchymal abnormalities
  
  • septal lines
  • small effusions
  • occasional a/s opacities

Question 8

Other collateral findings in patients with PH

Answer 8

• calcification
• pulmonary artery aneurysm
• chronic emboli
• pericardial effusion
• bronchial artery collaterals

Question 9

HRCT findings of PAH

Answer 9

• mosaic attenuation (oligemic areas with vessel pruning)
• parenchymal disease
• peripheral pruning of pulmonary arteries

Question 10

“Primary” pulmonary hypertension

Answer 10

• scleroderma
• SLE
• medications - fenfluramine
• chronic liver disease and portal HTN
• HIV

Question 11

Eisenmenger’s Syndrome

Answer 11

• dilated central pulmonary arteries, may even have aneurysmal dilatation of pulmonary artery
• calcification
• secondary thromboses
• cardiac abnormalities

Question 12

Most common cause of pulmonary arterial hypertension worldwide?

Answer 12

Schistosomiasis

Question 13

Pulmonary hypertension due to disorders

directly affecting the pulmonary vasculature

Answer 13

• inflammatory
• schistosomiasis
• sarcoidosis
• other (talc-graunulomatous vasculitis)
• pulmonary capillary hemangiomatosis

Question 14

CT appearance of talcosis?

Answer 14

almost like miliary TB

random distribution of small nodules

Question 15

Pulmonary capillary hemangiomatosis

Answer 15

• rare disease
• proliferation of capillaries
• invade walls of pulmonary arteries and veins
• CT
  
  • small nodules - ill-defined
  • mosaic attenuation