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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.

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Presentation Transcript
overview
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
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
classification1
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
classification2
Classification
  • Group V – pulmonary hypertension due to inflammation, mechanical obstruction, or extrinsic compression of the pulmonary vasculature ( sarcoidosis, and fibrosing mediastinitis)
pathophysiology
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
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 test1
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
Treatment optionsprimary 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 therapy1
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
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
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
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 therapy1
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
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.