pulmonary arterial hypertension
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PULMONARY ARTERIAL HYPERTENSION
Allan Murillo – Médico Interno - UNICAH
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 tales factores
NEJM 351;16 www.nejm.org october 14, 2004 - Review Article - Mechanisms of disease in Pulmonary Arterial Hypertension Harrison W. Farber, M.D., and Joseph Loscalzo, M.D., Ph.D.
Pulmonary arterial hypertension comprises:
Idiopathic Pulmonary
Arterial Hypertension
Pulmonary Arterial
Hypertension in the setting of
Collagen Vascular Disease (e.g.
CREST syndrome)
Portal Hypertension
Congenital left-to-right
intracardiac shunts
Infection with HIV
Persistent pulmonary
hypertension of the newborn
NEJM 351;16 www.nejm.org october 14, 2004 - Review Article - Mechanisms of disease in Pulmonary Arterial Hypertension Harrison W. Farber, M.D., and Joseph Loscalzo, M.D., Ph.D.
Cambios HistológicosMediadores de la respuesta a la PAH
Fisiopatología
Histological Findings
Intimal Fibrosis
Increased Medial ThicknessPulmonary Arteriolar Occlusion
Plexiform LesionsNEJM 351;16 www.nejm.org october 14, 2004 - Review Article - Mechanisms of disease in Pulmonary Arterial Hypertension Harrison W. Farber, M.D., and Joseph Loscalzo, M.D., Ph.D.
Vascular changes
VasoconstrictionSmooth-muscle cell and endothelial-cell
proliferationThrombosis
NEJM 351;16 www.nejm.org october 14, 2004 - Review Article - Mechanisms of disease in Pulmonary Arterial Hypertension Harrison W. Farber, M.D., and Joseph Loscalzo, M.D., Ph.D.
Mediators of Pulmonary Vascular Responsesin Pulmonary Arterial Hypertension Thromboxane A2 Prostaglandin I Nitric oxide Endothelin-1 Serotonin Vascular Endothelial Growth Factor Vasoactive Intestinal Peptide
NEJM 351;16 www.nejm.org october 14, 2004 - Review Article - Mechanisms of disease in Pulmonary Arterial Hypertension Harrison W. Farber, M.D., and Joseph Loscalzo, M.D., Ph.D.
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 PAH
NEJM 351;16 www.nejm.org october 14, 2004 - Review Article - Mechanisms of disease in Pulmonary Arterial Hypertension Harrison W. Farber, M.D., and Joseph Loscalzo, M.D., Ph.D.
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 output
NEJM 351;16 www.nejm.org october 14, 2004 - Review Article - Mechanisms of disease in Pulmonary Arterial Hypertension Harrison W. Farber, M.D., and Joseph Loscalzo, M.D., Ph.D.
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 proliferation
NEJM 351;16 www.nejm.org october 14, 2004 - Review Article - Mechanisms of disease in Pulmonary Arterial Hypertension Harrison W. Farber, M.D., and Joseph Loscalzo, M.D., Ph.D.
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 hypoxia
NEJM 351;16 www.nejm.org october 14, 2004 - Review Article - Mechanisms of disease in Pulmonary Arterial Hypertension Harrison W. Farber, M.D., and Joseph Loscalzo, M.D., Ph.D.
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 a cause.
NEJM 351;16 www.nejm.org october 14, 2004 - Review Article - Mechanisms of disease in Pulmonary Arterial Hypertension Harrison W. Farber, M.D., and Joseph Loscalzo, M.D., Ph.D.
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 of disease in Pulmonary Arterial Hypertension Harrison W. Farber, M.D., and Joseph Loscalzo, M.D., Ph.D.
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 thrombosis
NEJM 351;16 www.nejm.org october 14, 2004 - Review Article - Mechanisms of disease in Pulmonary Arterial Hypertension Harrison W. Farber, M.D., and Joseph Loscalzo, M.D., Ph.D.
ESC Guidelines - Guidelines for the diagnosis and treatment of pulmonary hypertension - European Heart Journal (2009) 30, 2493–2537
Datos ClínicosEstudios de Laboratorio e Imagen
Diagnóstico
Clinical presentation
Breathlessness Fatigue Weakness Angina Syncope Abdominal distension
Symptoms at rest are reported only in very advanced cases
Accentuated pulmonary component of 2º hrt sound
Pansystolic murmur of tricuspid regurgitation
Diastolic murmur of pulmonary insufficiency
RV third sound Jugular vein distension Hepatomegaly Peripheral edema Ascites Cool extremities Lung sounds are usually normal Telangiectasia Digital ulceration Sclerodactyly are seen in scleroderma The stigmata of liver disease such as
spider naevi, testicular atrophy, and palmar erythema should be considered
The symptoms of PAH are non-specific and include
The physical signs of PAH include
ESC Guidelines - Guidelines for the diagnosis and treatment of pulmonary hypertension - European Heart Journal (2009) 30, 2493–2537
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 pulmonary hypertension - European Heart Journal (2009) 30, 2493–2537
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 pulmonary hypertension - European Heart Journal (2009) 30, 2493–2537
Severe pulmonary hypertensionProminent main pulmonary artery segment (MPA), which appears to be aneurysmally dilated. Right pulmonary artery (RPA) is also enlarged.
En la PAH no se puede observar la presencia de vasos sanguineos en la periferia pulmonar
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 hyperventilation
ESC Guidelines - Guidelines for the diagnosis and treatment of pulmonary hypertension - European Heart Journal (2009) 30, 2493–2537
Echocardiography
Transthoracic echocardiography provides several variables which correlate with right heart haemodynamics including PAP, and should always be performed in the case of suspected PH
ESC Guidelines - Guidelines for the diagnosis and treatment of pulmonary hypertension - European Heart Journal (2009) 30, 2493–2537
High-resolution CT
ESC Guidelines - Guidelines for the diagnosis and treatment of pulmonary hypertension - European Heart Journal (2009) 30, 2493–2537
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 pulmonary hypertension - European Heart Journal (2009) 30, 2493–2537
FIN … WUJU!!!
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