Pulmonary arterial hypertension

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Hipertension Arterial Pulmonar incluye definicion, fisiopatologia, diagnostico y tratamiento

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Pulmonary arterial hypertension

  1. 1. PULMONARYARTERIALHYPERTENSIONAllan Murillo – Médico Interno - UNICAH
  2. 2. Definition  PAH is defined as a sustained elevation of pulmonary arterial pressure to more than 25 mm Hg at rest or to more than 30 mm Hg with exercise, with a mean pulmonary-capillary wedge pressure and left ventricular end-diastolic pressure of less than 15 mm Hg  Puede ser consecuencia de insuficiencia en la mitad izquierda del corazón, enfermedad del parénquima o de vasos pulmonares, tromboembolia o una combinación de talesNEJM 351;16 www.nejm.org october 14, 2004 - Review Article - Mechanisms ofdiseasefactores Arterial Hypertension Harrison W. Farber, M.D., and Joseph in Pulmonary
  3. 3. Pulmonary arterial hypertension comprises: Pulmonary Arterial Hypertension in the Idiopathic Pulmonary setting of Collagen Portal Hypertension Arterial Hypertension Vascular Disease (e.g. CREST syndrome) Persistent pulmonary Congenital left-to-right Infection with HIV hypertension of the intracardiac shunts newbornNEJM 351;16 www.nejm.org october 14, 2004 - Review Article - Mechanisms ofdisease in Pulmonary Arterial Hypertension Harrison W. Farber, M.D., and Joseph
  4. 4. FisiopatologíaCambios HistológicosMediadores de la respuesta a la PAH
  5. 5. Histological Findings Intimal Fibrosis Increased Medial Thickness Pulmonary Arteriolar Occlusion Plexiform LesionsNEJM 351;16 www.nejm.org october 14, 2004 - Review Article - Mechanisms ofdisease in Pulmonary Arterial Hypertension Harrison W. Farber, M.D., and Joseph
  6. 6. Vascular changes Smooth-muscle cell and Vasoconstriction Thrombosis endothelial-cell proliferationNEJM 351;16 www.nejm.org october 14, 2004 - Review Article - Mechanisms ofdisease in Pulmonary Arterial Hypertension Harrison W. Farber, M.D., and Joseph
  7. 7. Mediators of Pulmonary Vascular Responses in Pulmonary Arterial Hypertension  Thromboxane A2  Prostaglandin I  Nitric oxide  Endothelin-1  Serotonin  Vascular Endothelial Growth Factor  Vasoactive Intestinal PeptideNEJM 351;16 www.nejm.org october 14, 2004 - Review Article - Mechanisms ofdisease in Pulmonary Arterial Hypertension Harrison W. Farber, M.D., and Joseph
  8. 8. Prostacyclin/Thromboxane A2  Prostacyclin: potent vasodilator, inhibits platelet activation and has antiproliferative properties  Thromboxane A2 is a potent vasoconstrictor and platelet agonist.  In PAH, the imbalance between these two molecules is shifted toward thromboxane A2.  The production of prostacyclin synthase is decreased in the small and medium-sized pulmonary arteries of patients with pulmonary hypertension, particularly those with idiopathic PAHNEJM 351;16 www.nejm.org october 14, 2004 - Review Article - Mechanisms ofdisease in Pulmonary Arterial Hypertension Harrison W. Farber, M.D., and Joseph
  9. 9. Endothelin-1  Potent vasoconstrictor, stimulates proliferation of pulmonary-artery smooth-muscle cells  The plasma levels of endothelin-1 are increased in PAH and the level of endothelin-1 is inversely proportional to the magnitude of the pulmonary blood flow and cardiac outputNEJM 351;16 www.nejm.org october 14, 2004 - Review Article - Mechanisms ofdisease in Pulmonary Arterial Hypertension Harrison W. Farber, M.D., and Joseph
  10. 10. Nitric Oxide  Potent vasodilator and inhibitor of platelet activation and vascular smooth-muscle cell proliferation  Decreased levels of nitric oxide synthase have been observed in the pulmonary vascular tissue of patients with idiopathic PAH  Endothelial nitric oxide synthase is, howerver, increased in the plexiform lesions of idiopathic PAH, where it probably promotes pulmonary endothelial-cell proliferationNEJM 351;16 www.nejm.org october 14, 2004 - Review Article - Mechanisms ofdisease in Pulmonary Arterial Hypertension Harrison W. Farber, M.D., and Joseph
  11. 11. Serotonin  Vasoconstrictor that promotes smooth-muscle cell hypertrophy and hyperplasia.  ↑ levels of plasma serotonin and ↓ content of serotonin in platelets have been found in idiopathic PAH and persist even after the normalization of pulmonary-artery pressures following lung transplantation.  The level of serotonin itself is probably not a determinant of pulmonary hypertension, because selective serotonin- reuptake inhibitors (SSRIs), which increase serotonin levels but inhibit serotonin transport, are not associated with an increased incidence of pulmonary hypertension and may, in fact, be protective in the setting of hypoxiaNEJM 351;16 www.nejm.org october 14, 2004 - Review Article - Mechanisms ofdisease in Pulmonary Arterial Hypertension Harrison W. Farber, M.D., and Joseph
  12. 12. Adrenomedullin  Dilates pulmonary vessels, increases the pulmonary blood flow, and is synthesized by several cell populations in the normal lung  The plasma levels of adrenomedullin are elevated in both PAH and PH associated with hypoxemia, and the elevation correlates with increases in the mean right atrial pressure, pulmonary vascular resistance, and the mean pulmonary arterial pressure.  Current data suggest that increased adrenomedullin is a marker of PH, rather than aNEJM 351;16 www.nejm.org october 14, 2004 - Review Article - Mechanisms of cause.disease in Pulmonary Arterial Hypertension Harrison W. Farber, M.D., and Joseph
  13. 13. Vasoactive Intestinal Peptide  Potent systemic vasodilator, decreases pulmonary-artery pressure and pulmonary vascular resistance; it also inhibits platelet activation and vascular smooth-muscle cell proliferation.  A recent study reported decreased levels of vasoactive intestinal peptide in the serum and the lungs in patients with PAH; treatment with inhaled vasoactive intestinal peptide improved the clinical course and the hemodynamics in these patients.NEJM 351;16 www.nejm.org october 14, 2004 - Review Article - Mechanisms ofdisease in Pulmonary Arterial Hypertension Harrison W. Farber, M.D., and Joseph
  14. 14. Vascular Endothelial Growth Factor  In acute and chronic hypoxia, the production of vascular endothelial growth factor (VEGF) is increased and that of its receptors, VEGF receptor-1 and VEGF receptor-2 in the lung  In PAH, disordered angiogenic responses appear to underlie the formation of plexiform lesions and the clonal expansion of endothelial cells within the lesions  There is an imbalance of the vascular effectors in PAH that favors vasoconstriction, vascular-cell proliferation, and thrombosisNEJM 351;16 www.nejm.org october 14, 2004 - Review Article - Mechanisms ofdisease in Pulmonary Arterial Hypertension Harrison W. Farber, M.D., and Joseph
  15. 15. ESC Guidelines - Guidelines for the diagnosis and treatment of pulmonaryhypertension - European Heart Journal (2009) 30, 2493–2537
  16. 16. DiagnósticoDatos ClínicosEstudios de Laboratorio e Imagen
  17. 17. Clinical presentationThe symptoms of PAH are non- The physical signs of PAHspecific and include include Breathlessness  Accentuated pulmonary component of 2º hrt sound Fatigue  Pansystolic murmur of tricuspid regurgitation Weakness  Diastolic murmur of pulmonary insufficiency Angina  RV third sound  Jugular vein distension Syncope  Hepatomegaly Abdominal distension  Peripheral edema  Ascites Cool extremities Symptoms at rest are  Lung sounds are usually normal reported only in very  Telangiectasia advanced cases   Digital ulceration  Sclerodactyly are seen in scleroderma  The stigmata of liver disease such as spider naevi, testicular atrophy, and palmar erythemaESC Guidelines - Guidelines for the diagnosis should be considered and treatment of pulmonaryhypertension - European Heart Journal (2009) 30, 2493–2537
  18. 18. Electrocardiogram  The ECG may provide suggestive or supportive evidence of PH:  RV hypertrophy on ECG is present in 87% and right axis deviation in 79% of patients with IPAH  Supraventricular arrhythmias may be present in advanced stages, in particular atrial flutter, but also atrial fibrillation, which almost invariably leads to further clinical deterioration.  BRDHH de grado avanzado  The absence of these findings does not exclude the presence of PH nor does it exclude severe haemodynamic abnormalities.ESC Guidelines - Guidelines for the diagnosis and treatment of pulmonaryhypertension - European Heart Journal (2009) 30, 2493–2537
  19. 19. Chest radiograph  In 90% of patients with IPAH the chest radiograph is abnormal at the time of diagnosis.  Findings include:  Central pulmonary arterial dilatation  Right atrium and RV enlargement in advanced cases.ESC Guidelines - Guidelines for the diagnosis and treatment of pulmonaryhypertension - European Heart Journal (2009) 30, 2493–2537
  20. 20. Severe pulmonary hypertensionProminent main pulmonary artery segment (MPA), which appears to beaneurysmally dilated. Right pulmonary artery (RPA) is also enlarged.
  21. 21. En la PAH no se puedeobservar la presenciade vasos sanguineosen la periferia pulmonar
  22. 22. Pulmonary function tests and Arterial Blood Gases  Patients with PAH usually have decreased lung diffusion capacity for carbon monoxide and mild to moderate reduction of lung volumes  PAO2 is normal or only slightly lower than normal at rest and PACO2 is decreased because of alveolar hyperventilationESC Guidelines - Guidelines for the diagnosis and treatment of pulmonaryhypertension - European Heart Journal (2009) 30, 2493–2537
  23. 23. Echocardiography  Transthoracic echocardiography provides several variables which correlate with right heart haemodynamics including PAP, and should always be performed in the case of suspected PHESC Guidelines - Guidelines for the diagnosis and treatment of pulmonaryhypertension - European Heart Journal (2009) 30, 2493–2537
  24. 24. High-resolution CT  Provides detailed views of the lung parenchyma and facilitates the diagnosis of interstitial lung disease and emphysema.  Characteristic changes of interstitial oedema with diffuse central ground-glass opacification and thickening of interlobular septa suggest PVOD; additional findings may include lymphadenopathy and pleural effusion.  Pulmonary capillary haemangiomatosis is suggested by diffuse bilateral thickening of the interlobular septa and the presence of small, centrilobular, poorly circumscribed nodular opacities.ESC Guidelines - Guidelines for the diagnosis and treatment of pulmonaryhypertension - European Heart Journal (2009) 30, 2493–2537
  25. 25. ESC Guidelines - Guidelines for the diagnosis and treatment of pulmonaryhypertension - European Heart Journal (2009) 30, 2493–2537
  26. 26. FIN … WUJU!!!

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