New Technologies in Pulmonary Arterial Hypertension

Summary

Pulmonary arterial hypertension (PAH) is a rare cardiopulmonary disease that is associated with remodeling within the small pulmonary arteries, increased pulmonary vascular resistance, and right ventricular failure. Identifying PAH is a challenge for clinicians, in part due to its nonspecific symptoms, such as dyspnea, chest pain, heart failure, and palpitations. As a result, the accurate diagnosis of PAH is often delayed up to 2 years after symptom onset. This article describes the role of new imaging technology in improving the diagnosis and assessment of patients with PAH.

  • Hypertensive Disease Clinical Trials

Pulmonary arterial hypertension (PAH) is a rare cardiopulmonary disease that is associated with remodeling within the small pulmonary arteries, increased pulmonary vascular resistance, and right ventricular failure. Identifying PAH is a challenge for clinicians, in part due to its nonspecific symptoms, such as dyspnea, chest pain, heart failure, and palpitations. As a result, the accurate diagnosis of PAH is often delayed up to 2 years after symptom onset. Pierre Gacon, MD, Centre Hospitalier Universitaire, Dijon, France, described the role of new imaging technology in improving the diagnosis and assessment of patients with PAH.

Among Caribbean patients with PAH, approximately 40% has idiopathic disease. Additional etiologies include connective tissue disease (15%), HIV infection (10%), and hemoglobinopathy (5%). Current guidelines from the American College of Chest Physicians recommend Doppler echocardiography as a noninvasive screening test for patients who are suspected of having PAH. Echocardiographic abnormalities, such as pericardial effusion, right atrial enlargement, septal displacement, and left ventricular eccentricity index, predict adverse outcomes in patients with PAH and identify patients who may be candidates for more intensive medical therapy or earlier transplantation.

Additional tools are also emerging as valuable options for patient assessment. For instance, echocardiographic measurement of the tricuspid annular plane systolic excursion (TAPSE) adds valuable prognostic information about RV systolic function in patients with PAH. The measurement of TAPSE is easy to obtain in patients with PAH, irrespective of heart rate and rhythm, and significantly improves the prognostic value of other echocardiographic measures.

New technologies for measuring key prognostic variables, such as regional contractility, left ventricular eccentricity index, and myocardial performance index, can also improve risk assessment in patients with PAH. Compared with conventional Doppler echocardiography, new options, such as Doppler tissue imaging, 2-dimensional strain echocardiography, and speckle tracking echocardiography, provide more accurate prognostic information for determining PAH severity.

Improving outcomes for patients with PAH will require collaboration among primary care physicians, cardiologists, and other members of the health care team. By incorporating new technologies, physicians can improve the diagnosis, risk assessment, and management of patients with PAH.

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