TĂNG ÁP ĐỘNG MẠCH PHỔI, Đ H Y DƯỢC TP HCM
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PULMONARY
HYPERTENSION
Vu Minh Phuc MD.
CONTENTS
1.
2.
3.
4.
5.
6.
7.
8.
Definition
Causes
Pathogenesis
Pathophysiology
Clinical Manifestations
Natural History
Diagnosis
Management
1. DEFINITION
The
normal pulmonary arterial pressure (PAP)
of children and adults at sea level
Systolic/diastolic PAP
= 20/12 mm Hg
Mean PAP (mPAP)
= 15 mm Hg
Pulmonary
hypertension (PHTN)
mPAP 25 mm Hg at rest
mPAP 30 mm Hg during physical exercise
2. CAUSES
L-R shunt lesions (hyperkinetic PHTN)
1.
ASD, VSD, PDA, ECD
Alveolar hypoxia
2.
Pulmonary parenchymal disease
Extensive pneumonia
Primary or secondary hypoplasia of lungs
Bronchopulmonary dysplasia
Interstitial lung disease (Hamman-Rich syndrome)
Wilson-Mikity syndrome
Upper and lower airway obstruction
Inadequate ventilatory drive (central nervous system disease)
Disorders of chest wall or respiratory muscles
Kyphoscoliosis
Weakening or paralysis of skeletal muscle
High attitude (in certain hyperreactors)
2. CAUSES
Pulmonary venous hypertension
3.
Mitral stenosis, cor triatriatum, TAPVR with obstruction,
chronic left heart failure, left-sided obstructive lesions (aortic
stenosis, coarctation of the aorta)
Primary pulmonary vascular disease
4.
Persistent pulmonary hypertension of the newborn
Primary pulmonary hypertension – rare, fatal form of
pulmonary hypertension with obscure cause
Thromboembolism: ventriculoatrial shunt for
hydrocephalus, sickle cell anemia, thrombophlebitis
Collagen disease: rheumatoid arthritis, scleroderma, mixed
connective tissue disease
3- PATHOGENESIS
Endothelial dysfunction in PHTN
3- PATHOGENESIS
Imbalance of vasoactive mediators acting on the pulmonary vessels
3- PATHOGENESIS
P (pressure) = F (flow) R (resistance)
F or R or both PHTN
PHTN constriction of pulmonary arterioles PVR
RV hypertrophy
Normal RV cannot sustain sudden pressure loads over
40-50 mmHg acute right-sided heart failure if PVR
increases abruptly.
Hypertrophied RV can tolerate mild PHTN (PAPs = 50
mmHg)
Superimposed lung disease, alveolar hypoxia, acidosis
RV may fail
3- PATHOGENESIS
3.1. Hyperkinetic pulmonary hypertension
3- PATHOGENESIS
3.2. Alveolar hypoxia
3- PATHOGENESIS
3.3. Pulmonary venous hypertension
3- PATHOGENESIS
3.4.Primary pulmonary vascular disease
TREATMENT OF
PULMONARY
HYPERTENSION
Milrinone
PGE1
PGI2
Dobuta
Isoprote
PG
B2
SMOOTH CELL
ac
ATP
pde III
cAMP
inactive AMP
ETA
Vasodilation
ET-1
Vasoconstriction
ETB
GTP
NO
NOr
cGMP
gc
Ca++
A1
pde V
Nitroprusside
Nitroglycerin
B2
Sildenafil Bonsentan Calcium Tolazoline
blockers
PGI2
ETB
NO
ET-1
L-citrullin
L-arginine
NO synthetase
ENDOTHELIAL
CELL
ATP
inactive GMP
Sixtasentan
Bonsentan
Bonsentan
ABBREVIATION
A1: alpha 1 receptors
GMP: guanosine monophosphate
AMP: adenosine monophosphate
cGMP: cyclic GMP
cAMP: cyclic AMP
GTP: guanosine triphosphate
ATP: adenosine triphosphate
NOr: nitric oxide receptors
B2: beta 2 receptor
PDE III: phosphodiesterase III
ETA: ETA receptors on smooth cells
PDE V: phosphodiesterase V
ETB: ETB receptors on smooth cells
PG: prostaglandin receptors
and endothelial cells
PGE1: prostaglandin E1
ET-1: endothelial-1, an isopeptide
PGI2: prostacyclin
produced primarily in the vascular
endothelial cells
PULMONARY VASODILATORS
Oxygen, Nitric oxide
Prostacyclin
Epoprostenol, Ventavis (IV)
Iloprost (aerosol)
Anti-acidosis
Adenosine (IV)
Amrinone, Milrinone
L-arginine (IV)
Sildenafil
Dobutamin (IV)
Bonsentan
Isoproterenol (IV)
Sixtasentan
Tolazoline (IV)
Calcium blockers (nifedipine,
Nitroprusside (IV)
Nitroglycerine (IV)
diltiazem)
Salbutamol
PULMONARY VASODILATOR
TREATMENT
- mPAP > ½ mSBP and/or
- Increasing PAP causes tachycardia and falling toe temperature
- NO
- Epoprostenol/ Ventavis
YES
Acute vasodilator response Trial of calcium chanel blckers
(nifedipine or diltiazem)
NO
Incomplete response
NO
Right heart failure
Trial of:
- Iloprost
- Sildenafil
YES - Bonsentan
se
n
o
sp
e
r
- Sixtasentan
lete
p
Epoprostenol - Investigational
drugs
om
Inc
