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Cor Pulmonale

PULMONARY HYPERTENSION AND COR PULMONALE פרופ' נוויל ברקמן מכון הריאה ביה"ח האוניברסיטאי הדסה עין-כרם. Cor Pulmonale. Hypertrophy and/ or dilatation of the right ventricle secondary to respiratory disease. Invariably associated with pulmonary hypertension.

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Cor Pulmonale

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  1. PULMONARY HYPERTENSION AND COR PULMONALEפרופ' נוויל ברקמןמכון הריאהביה"ח האוניברסיטאי הדסה עין-כרם

  2. Cor Pulmonale • Hypertrophy and/ or dilatation of the right ventricle secondary to respiratory disease. • Invariably associated with pulmonary hypertension. • Right ventricular failure is a late consequence of chronic pulmonary hypertension.

  3. NORMAL Large cross-sectional area low resistance Low pressure PULMONARY HYPERTENSION Reduced cross-sectional area high resistance high pressure Pulmonary Vascular Bed

  4. Pulmonary Hypertension • Normal pulmonary pressures: 15-30/4-12; mean 9-18. • Pulmonary hypertension: mean pressure >25mm Hg at rest (no values on exercise).

  5. Pulmonary Hypertension Pressure = Cardiac OutputResistance

  6. Updated clinical classification Dana Point 2009 1. PULMONARY ARTERIAL HYPERTENSION Idiopathic (IPAH) Familial (FPAH) Associated with (APAH): connective tissue disorders congenital systemic-pulmonary shunts Portal hypertension HIV Drugs and toxins Other (thyroid disorders, Gaucher’s, glycogen storage dis, HHT, hemoglobinopathies, myeloproliferative disorders, splenectomy) PAH with significant venous and/or capillary involvement pulmonary veno-occlusive disease (PVOD) pulmonary capillary hemangiomatosis (PCH) Persistent PHN

  7. Updated clinical classification - cont 2. PULMONARY HYPERTENSION WITH LEFT HEART DISEASE Atrial or ventricular heart disease Valvular heart disease 3. PULMONARY HYPERTENSION WITH LUNG DISEASE AND/OR HYPOXEMIA Chronic obstructive pulmonary disease Interstitial lung disease Sleep disorders; Alveolar hypoventilation ; Chronic exposure to high altitude Developmental Abnormalities

  8. Updated clinical classification –cont. 4. PH DUE TO CHRONIC THROMBOTIC AND/OR EMBOLIC DISEASE Thromboembolic obstruction of proximal pulmonary arteries Thromboembolic obstruction of distal pulmonary arteries Pulmonary embolism (tumor, parasites, foreign material) 5. MISCELLANEOUS Sarcoidosis, histiocytosis X, lymphangiomatosis, compression of pulmonary vessels (adenopathies and tumors, fibrosing mediastinitis)

  9. Pulmonary Arterial Hypertension- Pathology • Vasocostriction • In-situ thrombosis. • Intimal and medial thickening with obliteration. • Plexiform lesions

  10. Pulmonary Hypertension

  11. Pathophysiology • Increased vasoconstriction • Thromboxane A2 • Fibrinopeptide • endothelin-1 • Serotonin • Reduced vasodilatation • Decreased prostacyclin • Decreased Nitric Oxide • Impaired K+- ion channel • Enhanced proliferation • Endothelium • smooth muscle

  12. The PAH gene! • 6% of IPAH are familial • Linkage analysis localised the PAH gene to chromosome 2q33. • Autosomal dominant; low penetrance.

  13. The PAH gene! • Gene identified as the “Bone Morphogenetic Protein Receptor II gene (BMPRII)” Deng,Am J Hum Genet, 2000

  14. BMPR-II • 72% of PAH families have an identified BMPR2 mutation or show linkage to this locus. • Mutations of the BMPR2 gene are present in at least 26% of patient with sporadic PAH.

  15. Mechanism of Idiopathic PAH • Member of the TGF- receptor family. • Important in the regulation of cell growth, differentiation and apoptosis. • Other factors; genetic or enviromental are required for the disease to develop.

  16. PULMONARY HYPERTENSION Increased pulmonary Vascular resistance Enhanced proliferation of smooth muscle cells

  17. Idiopathic PAH- Clinical • Young females • 1-2/ million/ yr. • Familial in 6-10% (chromosome 2q 31-32) • Immune features (ANF+ in 20-30%) • DELAYED DIAGNOSIS

  18. Pulmonary Arterial Hypertension- symptoms • Dyspnea on exertion • Chest pain • Syncope • Right heart failure • hemoptysis, palpitations.

  19. Pulmonary Arterial Hypertension - signs • Loud pulmonary second heart sound (P2), often with fixed splitting. • Right ventricular heave • Tricuspid insufficiency • Third or fourth heart sound. • Right sided cardiac failure: elevated JVP, hepatomegally, peripheral edema. • Clear lungs • Seldom clubbing

  20. Pulmonary Arterial Hypertension- diagnosis • ECG:- right ventricular hypertrophy, strain, RBBB, right axis deviation. • X-ray:- enlarged pulmonary arteries. • Low diffusion capacity, normal lung volumes • Echocardiography. • Right heart catheterisation. • Exclude other causes: vasculitis, pulmonary emboli, cardiac shunt, pulmonary parenchymal disease.

  21. Pulmonary Arterial Hypertension- Treatment • Oxygen • Anticoagulants • Diuretics • Ca-channel blockers (nifedipine, diltiazem) • Prostacyclins (epoprostenol, treprostinil, iloprost) • Endothelin receptor antagonists (bosentan, ambrisentan) • Phosphodiesterase -5 inhibitors (sildenafil) • Transplantation, atrial septostomy

  22. Humbert et al; N Engl J Med 2004 Treatment

  23. PPH- Prognosis • Poor : 50% 3 year survival. • Poor predictors of survival: high mean PAP, high RAP, low CO, low diffusion capacity. • Responders to vasodilators do well! • Continuous intravenous epoprostanol prolongs survival.

  24. Vasoreactivity PAH, Class III/IV Conventional therapy (C) (oral anticoagulants ± diuretics ± O2) Yes No Oral CCBs (C) Class III Class IV Sustained Response Endothelin R Antagonists Bosentan (A) or Prostanoid Analogues Iloprost inh (B), Treprostinil (B), Beraprost (B), or Epoprostenol (A) Epoprostenol (A) Bosentan (A) Treprostinil (B) Iloprost iv (C) Yes No No improvement or deterioration (Combination Therapy?) ? Continue CCB(< 10%) Atrioseptostomy (C) and/or Lung Transplant (C) PDE5 inhibitors Sildenafil (C)

  25. BMPR-II signaling PROLIFERATION PROLIFERATION Humbert et al. Eur Respir J 2002;20:741-9

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