Pascual: Pulmonary Vascular Disease

Front 1

Pulmonary hypertension: symptoms

Back 1

Same as congestive heart failure

Exertional dyspnea
Rest dyspnea
Fatigue
Edema
Abdominal bloating and weight gain (Ascites)
Chest pain (angina)
Syncope

Front 2

Pulmonary hypertension: physical exam

Back 2

Loud pulmonic valve closure (P2), compare P2 vs. A2 and compare S1 vs S2 at the apex

Cor pulmonale
- TR murmur, PR less commonly
- Right-sided gallop (S3 or S4)
- Right ventricular (substernal) heave
- Jugular venous distention
- Peripheral edema, ascites

Front 3

Pulmonary hypertension: tests

Back 3

Contrast echocardiogram

Increased estimated systolic pulmonary artery pressure (PAP) or right ventricular systolic pressure (RVSP) or tricuspid regurgitation (TR) jet velocity
Right atrial and ventricular hypertrophy
Flattening of Intraventricular septum
Small left ventricle (LV) dimension
Normal or small left atrium (LA)
Maybe right to left or left to right shunt

Front 4

Calculating PVR (pulmonary vascular resistance)

Back 4

PVR (wood units) = mean PAP-left atrial pressure/cardiac output

PVR (dyn∙s/cm5) = PVR (wood units) X 80

Front 5

Right heart catherterization findings in pulmonary hypertension

Back 5

mPAP
->25 (normal 14)

Cardiac index
- <2.5 (normal 2.5-4)

RAP
->6 (normal 2-6)

PVR
- >3 woods (normal <2)

PCWP
- <15 (normal 8-12)

Front 6

Pulmonary hypertension: pathology

Back 6

Endothelial proliferation (Intimal) in small pulmonary arteries (branch points)
-Plexiform lesion
Intimal fibrosis
Muscular hypertrophy or hyperplasia
Reduced prostacyclin, abnormal distribution of NOS, increased endothelin
BMP-RII mutation in familial cases
Necrotizing vasculitis sometimes

Front 7

Pulmonary hypertension: classification

Back 7

1. Pulmonary Arterial Hypertension
-Primary cause
-Only group that receives pulmonary arterial hypertension drugs

2. Pulmonary Hypertension with Left Heart Disease

3. Pulmonary Hypertension associated with lung disease or hypoxemia

4. Pulmonary Hypertension due to chronic pulmonary embolism

5. Miscellaneous

Front 8

Pulmonary hypertension: treatment

Back 8

In All Patients Treat Cor Pulmonale symptoms
-Oxygen
-Diuretics
-Digoxin
-Exercise retraining (cardiac rehab) in selected cases

In PAH (WHO Group I) also can use
-Prostanoids (prostacyclin or its analogues)
-Endothelin receptor antagonists (ERA)
-Phosphodiesterase inhibitors (PDE5I)

In WHO group IV and some PAH (WHO group I): Anti-coagulation

Front 9

Pulmonary vasculitis: symptoms and signs

Back 9

Symptoms
-Inflammatory/Constitutional: fatigue, weight loss, fever
-Cough
-Hemoptysis

Signs
-Upper airway inflammation
-Crackles or wheezing
-Cardiac findings are not common

Front 10

Pulmonary vasculitis: pathogenesis

Back 10

Anti-neutrophilic cytoplasmic Ab
c-ANCA, (anti serine proteinase-3 Ab)
p-ANCA, (anti myeloperoxidase)
a-ANCA (atypical)

Front 11

Antibodies associated with lung disease

Back 11

Anti-nuclear antibody (ANA)
-Systemic Lupus Erythematosus
-Scleroderma
-Sjogren’s disease
-Rheumatoid arthritis

Anti-neutrophilic cytoplasmic antibodies (ANCA)
-Microscopic polyangitis, Churg-Strauss (pANCA)
-Wegener’s Granulomatosis (cANCA)

Anti-basement membrane antibody (anti-GBM)
-Goodpasture’s disease

Anti-receptor or mediator antibody (Anti-GM-CSF)
-Pulmonary Alveolar Proteinosis

Front 12

Granulomatosis with polyangiitis/ wegener's granulomatosis: diagnostic criteria

Back 12

Nasal or oral inflammation (upper airway)
Abnormal chest radiograph
Abnormal urinary sediment
Granulomatous inflammation
-biopsy required

Front 13

Microscopic polyangiitis

Back 13

Glomerulonephritis
Less upper respiratory involvement than WG
pANCA vs cANCA
Little or no granulomatous inflammation

Front 14

Churg-Strauss syndrome

Back 14

Asthma, eosinophilia, vasculitis
Antibodies: IgE, p-ANCA
Granulomatous vasculitis involving the heart, lungs, skin, kidneys and peripheral nerves in patients with an allergic background, usually with asthma.

Front 15

Idiopathic pauci-immune pulmonary capillaritis (IPIPC)

Back 15

Isolated to lungs, no renal involvement
Alveolar hemorrhage
Small vessel vasculitis

Front 16

Goodpasture's syndrome

Back 16

Anti-glomerular basement membrane antibodies (anti-GBM Ab) are found in the serum during active disease
Pulmonary hemorrhage + hemoptysis
diffuse alveolar infiltrates on CXR, anemia and glomerulonephritis.
M>F, most patients are young adults.
Therapy includes plasmapheresis (plasma exchange to remove the anti-GBM Ab), high dose corticosteroids and cyclophosphamide.

Front 17

Treatment of pulmonary vasculitis

Back 17

Assess severity: limited, generalized, organ dysfunction
Remission induction, Refractory, Maintenance
Corticosteroids, methotrexate, azathioprine, rituximab, cyclophosphamide
Plasmapheresis