Transcript Treatment options for pulmonary hypertension

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 ( won’t 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 Rhokinase 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.