Pulmonary Hypertension

and Various Treatment

Options
Presented by: Cory
Johanboeke
 Overview
Pulmonary hypertension is
characterized by increased pulmonary
arterial pressure and secondary right
ventricular failure.
PH is defined as a mean pulmonary
artery pressure greater 25mmHg at
rest or 30mmHg with exercise.
Classified into 5 groups according to
the mechanistic basis of the disease.
 Classification
Group I Pulmonary arterial hypertension
(PAH), this group includes those with sporadic
idiopathic pulmonary arterial hypertension,
PAH due to collagen vascular disease (RA,
SLE, scleroderma), congenital heart defects
causing systemic to pulmonary shunt (ASD,
VSD), portal hypertension.
Pulmonary vascular resistance (PVR)
>120dynes/sec/cm, Pulmonary capillary
wedge pressure (PCWP)<15
 Classification
Group II pulmonary venous
hypertension, which is pulmonary
hypertension due to left atrial,
ventricular, or valvular heart disease.
Group III - pulmonary hypertension is due
to disorders of the respiratory system or
hypoxemia (COPD, sleep apnea).
Group IV pulmonary hypertension is due
to chronic thrombotic or embolic disease
of the pulmonary vasculature
 Classification
Group V pulmonary hypertension
due to inflammation, mechanical
obstruction, or extrinsic compression
of the pulmonary vasculature
( sarcoidosis, and fibrosing
mediastinitis)
 Pathophysiology
Increased blood flow in
the pulmonary
circulation
Hypoxic
vasoconstriction
Vascular fibrosis
Inflammatory mediators
causing vasoconstriction and
activating platelets
Smooth muscle cell
propagation
Increased vasculature
pressure
Right heart hypertrophy and
failure
 Diagnostic test
Chest X-ray- enlargement of the central
pulmonary arteries, enlargement of the
right ventricle
EKG- signs of right atrial and ventricular
hypertrophy, right axis deviation
Echocardiography- used to estimate
pulmonary artery systolic pressure, and
right ventricle size and thickness, check for
shunts, valve function, and pericardial
effusions.
 Diagnostic test
Pulmonary function test- to determine lung
disease as a cause.
Overnight oximetry- check for obstructive
sleep apnea
V/Q scan- check for thromboembolic disease.
Six minute walk to evaluate which NYHA
functional class the patient is in.
Right heart catheterization to confirm
diagnosis
 Treatment options
primary therapy
Divided into two groups primary
therapy and advanced therapy.
Primary therapy is directed at
resolving the underlying cause.
Fix the underlying heart defect for
those in group 2
Diagnosis and treat the underlying
lung condition for those in group 3
 Treatment options
primary therapy
Anticoagulation with warfarin for those in
class 4 (consider using with all classes) INR
goal of 2
Reversal of or treatment of underlying cause
in group 5
Use diuretics for peripheral edema and
hepatic congestion.
Oxygen 1-4 liters to maintain Sats. of 90% or
higher.
Exercise to improve functional capacity
 Treatment options
advanced therapy
Indicated for all patients in New York Heart
Association (NYHA) functional class levels
3 or 4 (usually those with IPAH).
CCBs, Nifedipine and diltiazem, titrated up
to reduce patient to functional class 1 or 2
Prostonoid drugs, induce smooth muscle
relaxation and inhibit smooth muscle cell
and platelet aggregation. IV Epoprostenol,
SQ Treprostinil, inhaled Iloprost
 Advanced therapy
Bosentan- Non-selective endothelin
receptor blocker (endothelin potent
vasoconstrictor)
Sixtaxentan, ambrisentan- selective
endothelin type A antagonist ( wont
block the vasodilator effects
mediated by type b receptors).
These drugs still under review by FDA
 Advanced treatment
Sildenafil- phosphodiesterase type 5
inhibitor, prolongs the vasodilator effects of
Nitric oxide.
Milirone - phosphodiesterase type 3
inhibitor (vasodilator effect on both large
and small pulmonary arteries)
Nicorandil- potassium channel opener with
nitrate properties. (hyperpolarizes the
smooth muscle wall and calcium
antagonist).
 Advanced therapy
Fasudil- approved to treat vasospasm
after arachnoid hemorrhage in Japan,
blocks Rho-kinase which is a
mediator of smooth muscle
contraction.
 Surgical treatment options
Atrial septostomy- creates a right to left
shunt this increase systemic blood flow
and reduces pulmonary congestion.
Cadaveric lung transplantation
Living donor lobar lung transplantation-
harvesting lower lobes from healthy
donors and implanting them in the patient.