Medical Treatment of Pulmonary Hypertension in 2004

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US Cardiology 2004;2004:1(1):1-7

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Pulmonary hypertension (PH) is a hemodynamic condition defined by pulmonary arterial systolic pressure (PASP) above an upper limit of 35mmHg, or by pulmonary arterial mean pressure (PAMP) exceeding 25mmHg at rest.1,2 Pulmonary arterial hypertension (PAH) is present when pre-capillary pulmonary vascular pressure is elevated in the absence of pulmonary capillary wedge pressure >15mmHg, such as that associated with left ventricular dysfunction or valve disease, or obstruction of the pulmonary veins. PH can be suspected on the basis of symptomatic presentation (though may be present in the absence of symptoms), physical examination and transthoracic Doppler-echocardiogram. Chest X-ray and electrocardiogram, while important for assessment of co-existing conditions, may be supportive of a diagnosis of PH but is not definitive in determining the presence of PH.3 Echocardiography, by assessment of the tricuspid and pulmonary regurgitant velocities and application of the modified Bernoulli equation, estimates PASP and pulmonary artery diastolic pressure. Although the estimated value correlates well with invasively measured hemodynamics,4 individual variation and inaccuracy requires that right heart catheterization be performed for definitive evaluation of pulmonary hemodynamics, including response to vasoactive agents.

Pulmonary hypertensive disorders have been classified according to the World Health Organization (WHO) definition of 1998 and modified by an international symposium in 2003 (see Table 1).5 The schema of classification is based on a combination of pathological features, clinical constellation, hemodynamics, and management strategies. Idiopathic pulmonary arterial hypertension (IPAH; also called primary pulmonary hypertension (PPH)) is a rare illness affecting all ages, but predominantly young to middle-aged women. A genetic type of IPAH has been identified, associated with mutations of the gene coding for the bone morphogenic protein receptor.6,7 The low incidence of both sporadic and familial IPAH (approximately one to five per million in the US) and subtle signs at early stages makes it difficult to recognize. Improving physician awareness and widespread availability of non-invasive screening by echocardiography, however, are shortening the delay to the time of diagnosis. When other types of PAH are considered, the prevalence increases substantially. Moreover, the diagnosis of PH based on echocardiography includes patients who may have left-sided cardiac abnormalities, hypoxemic or parenchymal lung disease, or chronic pulmonary thromboembolic disease. Since the management of PAH (idiopathic or in association with connective tissue disease, HIV infection, prior appetite suppressant use or other toxic exposures, EisenmengerÔÇÖs syndrome, or portal hypertension) differs from PH due to chronic thromboembolic disease, lung disease, and left heart etiologies, accurate diagnostic distinction is essential.A general process for evaluating PH is illustrated in Figure 1.

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