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Fundación Colombiana de Hipertensión Pulmonar®
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Palabras claves: Causas, factores desencadenantes, factores concomitantes, tromboembolismo, embolismo, Hipertensión arterial pulmonar, Hipertensión pulmonar primaria esporádica, Hipertensión pulmonar primaria familiar, Hipertensión arterial pulmonar idiopática, Hipertensión pulmonar primaria, HPP, Hipertensión pulmonar secundaria, HTP, Hipertensión Pulmonar, HP, HAPP, HAPI, HPP, HPS. Herramientas

Symptoms Symptoms depend on the extent that the pulmonary artery is blocked and on the person's overall health. For example, people who have another disease such as chronic obstructive pulmonary disease or coronary artery disease may have more disabling symptoms. Small emboli may not cause any symptoms, but when symptoms do occur, they usually develop abruptly. Shortness of breath may be the only symptom, especially if pulmonary infarction does not develop. Often, the breathing is very rapid, and the person may feel anxious or restless and appear to have an anxiety attack. Some people have pain in the chest. In some people, the first symptoms are light-headedness, fainting, or seizures. In older people, the first symptom may be confusion or deterioration of mental function. These symptoms usually result from a sudden decrease in the heart's ability to deliver enough oxygen-rich blood to the brain and other organs. The heartbeat may become rapid, irregular, or both. With very large emboli, blood pressure may be dangerously low (shock), the skin may have a blue color (cyanosis), or the person may suddenly die. The symptoms of pulmonary infarction develop over hours. If pulmonary infarction occurs, the person experiences coughing that may produce blood-stained sputum, sharp chest pain when breathing in, and in some cases fever. Symptoms of infarction often last several days but usually become milder every day. In people who have recurring episodes of small pulmonary emboli, symptoms such as chronic shortness of breath, swelling of the ankles or legs, and weakness tend to develop progressively over weeks, months, or years. Diagnosis Doctors suspect pulmonary embolism based on the person's symptoms and risk factors, such as recent surgery, a prolonged period of bed rest, or an inherited tendency to form blood clots. A large pulmonary embolism may be relatively easy for doctors to diagnose, especially when there are obvious preconditions, such as signs of a blood clot in a leg. However, in many cases, symptoms are absent or not very characteristic, which is an important reason why pulmonary embolism is often difficult to diagnose. Indeed, pulmonary embolism is one of the most difficult serious disorders for doctors to recognize and diagnose. A chest x-ray may reveal subtle changes in the blood vessel patterns after embolism and signs of pulmonary infarction. However, the x‑ray results are often normal, and even when they are abnormal, they rarely enable doctors to establish the diagnosis with certainty. An electrocardiogram may show abnormalities, but often these abnormalities are transient and can only support the possibility of pulmonary embolism. The person's symptoms and risk factors and the results of tests help doctors estimate the likelihood of a pulmonary embolism. This estimate determines what other tests are done. Doctors try to use tests that do not involve making an incision or entering the person's body (noninvasive tests) before they use an invasive test. Noninvasive tests are usually easier to perform and carry less risk of side effects. For example, if pulmonary embolism appears unlikely, testing may be limited to a blood test that measures a substance called D‑dimer. If pulmonary embolism seems more likely or if the result of the D-dimer test is abnormal, further testing is done, which may include a CT angiogram, an ultrasound examination of the legs, or a lung perfusion scan. These are noninvasive tests. If the diagnosis is still unclear after noninvasive tests are done, an invasive test (for example, pulmonary angiography) may be done. A CT angiogram is a type of computed tomography (CT) scan. It is fast, noninvasive, and fairly accurate, particularly for large clots. In this test, contrast material is injected into a vein. The contrast material travels to the lungs, and a CT scanner generates images of blood in the arteries to determine if a pulmonary embolism is blocking blood flow. A CT angiogram is the imaging test most often used to diagnose pulmonary embolism. An ultrasound examination of the legs is noninvasive and can identify clots in the legs, which are the usual sources of pulmonary embolism. The absence of clots on this test does not rule out pulmonary embolism. However, if the ultrasound examination reveals blood clots, people are usually treated as they would be for pulmonary embolism without any further testing. A lung perfusion scan is noninvasive and fairly accurate but is not very rapid. A tiny amount of radioactive substance is injected into a vein and travels to the lungs, where it outlines the blood supply (perfusion) of the lung. Completely normal scan results indicate that the person does not have a significant blood vessel obstruction. Abnormal scan results support the possibility of pulmonary embolism but may also reflect disorders other than pulmonary embolism, such as emphysema, which can result in decreased blood flow to areas where lung tissue has been damaged. Usually, the perfusion scan is done with a lung ventilation scan. The person inhales a harmless gas containing a trace amount of radioactive material, which is distributed throughout the small air sacs of the lungs (alveoli). The areas where carbon dioxide is being released and oxygen is taken up can then be seen on a scanner. By comparing this scan to the pattern of blood supply shown on the perfusion scan, doctors can usually determine whether a person has had a pulmonary embolism. Pulmonary angiography (see Symptoms and Diagnosis of Lung Disorders: Chest Imaging) is the most accurate means of diagnosing pulmonary embolism, but it is invasive and poses some risk and is more uncomfortable than the other tests. It is usually performed only if the results of other tests are not conclusive.

Preparado por el Comité Científico de la FCHP®


1. David B. Badesch, Steven H. Abman, Gerald Simonneau, Lewis J. Rubin and Vallerie V. McLaughlin. Medical Therapy for Pulmonary Arterial Hypertension: Updated ACCP. Evidence-Based Clinical Practice Guidelines. Chest 2007;131;1917-1928. DOI 10.1378/chest.06-2674. The online version of this article, along with updated information and services can be found online on the World Wide Web at: http://chestjournal.org/cgi/content/abstract/131/6/1917
2. (1) Medical therapy for pulmonary arterial hypertension: ACCP evidence-based clinical practice guidelines. (2) 2007 addendum.
BIBLIOGRAPHIC SOURCE(S): Badesch DB, Abman SH, Ahearn GS, Barst RJ, McCrory DC, Simonneau G, McLaughlin VV. Medical therapy for pulmonary arterial hypertension: ACCP evidence-based clinical practice guidelines. Chest 2004 Jul;126(1 Suppl):35S-62S. [191 references] PubMed
3. Guidelines on diagnosis and treatment of pulmonary arterial hypertension. The Task Force on Diagnosis and Treatment of Pulmonary Arterial Hypertension of the European Society of Cardiology. Task Force members: Nazzareno Galie (Chairperson)(Italy), Adam Torbicki (Poland), Robyn Barst (USA), Philippe Dartevelle (France), Sheila Haworth (UK), Tim Higenbottam (UK), Horst Olschewski (Germany), Andrew Peacock (UK),Giuseppe Pietra (Switzerland), Lewis J. Rubin (USA), Gerald Simonneau (Co-Chairperson) (France). ESC Committee for Practice Guidelines (CPG): Silvia G. Priori (Chairperson) (Italy), Maria Angeles Alonso Garcia (Spain), Jean-Jacques Blanc (France), Andrzej Budaj (Poland), Martin Cowie (UK), Veronica Dean (France), Jaap Deckers (The Netherlands), Enrique Fernandez Burgos (Spain), John Lekakis (Greece), Bertil Lindahl (Sweden), Gianfranco Mazzotta (Italy), Keith McGregor (France), João Morais (Portugal), Ali Oto (Turkey), Otto A. Smiseth (Norway). Document reviewers: Gianfranco Mazzotta (CPG Review Coordinator) (Italy), Joan Albert Barbera (Spain), Simon Gibbs (UK), Marius Hoeper (Germany), Marc Humbert (France), Robert Naeije (Belgium), Joanna Pepke-Zaba (UK). European Heart Journal 2004 25(24):2243-2278; doi:10.1016/j.ehj.2004.09.014
4. National Heart, Lung, and Blood Institute (NHLBI) [Instituto Nacional del Corazón, Pulmón y Sangre] - USA
5. Pulmonary Hypertension Association (PHA) [Asociación de Hipertensión Pulmonar] - USA
6. Mayo Clinic, Rochester, Minnesota [Clínica Mayo, Rochester, Minnesota] - USA
7. American Heart Association (AHA) [Asociación Americana del Corazón] - USA
8. Cleveland Clinic [Clínica Cleveland] - USA
9. Consensus statement on the management of pulmonary hypertension in clinical practice in the UK and Ireland. National Pulmonary Hypertension Centres of the UK and Ireland. Heart 2008;94;1-41 doi:10.1136/hrt.2007.132118. Downloaded from heart.bmj.com on 6 March 2008.
10. Joan A. Barberà, Pilar Escribano, Pilar Morales, Miguel Á. Gómez, Mikel Oribe,Ángel Martínez, Antonio Román, Javier Segovia, Francisco Santos y María T. Subirana. Estándares asistenciales en hipertensión pulmonar. Documento de consenso elaborado por la Sociedad Española de Neumología y Cirugía Torácica (SEPAR) y la Sociedad Española de Cardiología (SEC). Documento publicado simultáneamente en Arch Bronconeumol. 2008;44(2): 89-99. ISSN : 1579-2129. DOI artículo : 10.1157/13115748
11. Fundación Colombiana de Hipertensión Pulmonar -FCHP®- [Colombian Pulmonary Hypertension Foundation -CPHF-]

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