Right atrial size and tricuspid regurgitation severity predict mortality or transplantation in primary pulmonary hypertension

Bustamante-Labarta, Miguel H.; Perrone, Sergio V.; Fuente, Ricardo León de la; Stutzbach, Pablo; Hoz, Ricardo Pérez de la; Torino, Augusto; Favaloro, Roberto

doi:10.1067/mje.2002.123962

Cited by 169 publications

(137 citation statements)

References 12 publications

(16 reference statements)

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“…An increase in PAPs does not necessarily reflect disease progression and a decrease in PAPs does not necessarily signal improvement. A comprehensive echocardiographic assessment includes a description of chamber sizes, particularly of the RA and RV area, the magnitude of tricuspid regurgitation, the LV eccentricity index and RV contractility, which can be determined by several variables, including RV longitudinal systolic strain/strain rate and RV fractional area change, Tei index and tricuspid annular plane systolic excursion (TAPSE) [101][102][103][104][105][106][107][108].…”

Section: The World Health Organization Functional Class (Who-fc) (Webmentioning

confidence: 99%

2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension

et al. 2015

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Guidelines summarize and evaluate all available evidence on a particular issue at the time of the writing process, with the aim of assisting health professionals in selecting the best management strategies for an individual patient with a given condition, taking into account the impact on outcome, as well as the risk–benefit ratio of particular diagnostic or therapeutic means. Guidelines and recommendations should help health professionals to make decisions in their daily practice. However, the final decisions concerning an individual patient must be made by the responsible health professional(s) in consultation with the patient and caregiver as appropriate.

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Section: The World Health Organization Functional Class (Who-fc) (Webmentioning

confidence: 99%

2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension

et al. 2015

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“…Studies have shown that a variety of atrial, ventricular, and Doppler parameters have been associated with an increased risk of death or transplantation in patients with PAH [37,38,44,63,[74][75][76]. While pericardial effusion presence and size are also associated with death or transplantation, this parameter is unlikely to be sufficiently discriminating for research purposes [32,38,44,45,47,77].…”

Section: Echocardiographymentioning

confidence: 99%

Surrogate End Points in Pulmonary Arterial Hypertension: Assessing the Response to Therapy

Snow¹,

Kawut²

2007

Clinics in Chest Medicine

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Pulmonary arterial hypertension (PAH) is a rare disease which is characterized by increased pulmonary vascular resistance and right heart failure. Recent discoveries in disease pathophysiology have been translated into effective therapies tested in clinical trials. The studies which have led to the regulatory board approval of therapies for PAH have focused on surrogate and intermediate end points, thought to reflect quantity and quality of life, respectively. However, validation of many of these surrogates is incomplete. It is also unknown which indicators of function or long-term survival should be used to formulate decisions regarding addition, discontinuation, or combination of therapies. Identification of suitable end points would therefore not only help investigators design appropriate clinical trials, but also assist clinicians in caring for patients with PAH. Hemodynamic, cardiac imaging, plasma biomarkers, and exercise testing hold some promise as potential surrogate end points for PAH. Functional status and quality of life assessments may also have important roles. Future studies should validate the most promising surrogate markers, so that patients, clinicians, subjects, and investigators may benefit from the advantages they confer on clinical care and on clinical trials.The past several years have yielded an explosion of randomized clinical trials (RCTs) of several new medical therapies for pulmonary arterial hypertension (PAH). The hopes for these and all treatments for potentially fatal diseases are 1) to improve how a patient feels or functions and/ or 2) to prolong survival. While simply stated, the assessment of progress towards these goals may be quite complex [1][2][3][4][5][6].New PAH therapies have gained regulatory board approval based on studies with intermediate and surrogate end points as primary outcomes, believed to reflect the impact of these drugs on quality of life (QOL) and survival, respectively. While hemodynamic, plasma, cardiac imaging, exercise, functional, and QOL parameters are promising candidates, the reliability and validity of these measures are not clearly established. In addition, there are no RCTs addressing the use of such end points in guiding management decisions in PAH, such as changing, intensifying, escalating, or combining therapies. Despite this lack of evidence, clinicians who

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“…An RA area > 20 cm 2 has been shown to be abnormal, and an RA area > 27 cm 2 showed a higher rate of death in adults with IPAH. 110,111 In adult patients with chronic PAH, the RA size significantly increases and parallels signs of activation of the Frank-Starling mechanism in both right chambers. The RA size has been shown to be positively related to larger RV dimensions.…”

Section: Rv and Ra Enlargementmentioning

confidence: 99%

“…10 in a 16-year-old adolescent with PH), indicates poor RV compliance, increased mean RAP, or both in adults. 110 The measurement of RA dimensions, including RA diameter and area, in an apical 4-chamber view is a simple variable. An RA area > 20 cm 2 has been shown to be abnormal, and an RA area > 27 cm 2 showed a higher rate of death in adults with IPAH.…”

Section: Rv and Ra Enlargementmentioning

confidence: 99%

Transthoracic Echocardiography in the Evaluation of Pediatric Pulmonary Hypertension and Ventricular Dysfunction

et al. 2016

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Transthoracic echocardiography (TTE) is the most accessible noninvasive diagnostic procedure for the initial assessment of pediatric pulmonary hypertension (PH). This review focuses on principles and use of TTE to determine morphologic and functional parameters that are also useful for follow-up investigations in pediatric PH patients. A basic echocardiographic study of a patient with PH commonly includes the hemodynamic calculation of the systolic pulmonary artery pressure (PAP), the mean and diastolic PAP, the pulmonary artery acceleration time, and the presence of a pericardial effusion. A more detailed TTE investigation of the right ventricle (RV) includes assessment of its size and function. RV function can be evaluated by RV longitudinal systolic performance (e.g., tricuspid annular plane systolic excursion), the tricuspid regurgitation velocity/right ventricular outflow tract velocity time integral ratio, the fractional area change, tissue Doppler imaging-derived parameters, strain measurements, the systolic-to-diastolic duration ratio, the myocardial performance (Tei) index, the RV/left ventricle (LV) diameter ratio, the LV eccentricity index, determination of an enlarged right atrium and RV size, and RV volume determination by 3-dimensional echocardiography. Here, we discuss the potential use and limitations of TTE techniques in children with PH and/or ventricular dysfunction. We suggest a protocol for TTE assessment of PH and myocardial function that helps to identify PH patients and their response to pharmacotherapy. The outlined protocol focuses on the detailed assessment of the hypertensive RV; RV-LV crosstalk must be analyzed separately in the evaluation of different pathologies that account for pediatric PH.

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Right atrial size and tricuspid regurgitation severity predict mortality or transplantation in primary pulmonary hypertension

Cited by 169 publications

References 12 publications

2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension

2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension

Surrogate End Points in Pulmonary Arterial Hypertension: Assessing the Response to Therapy

Transthoracic Echocardiography in the Evaluation of Pediatric Pulmonary Hypertension and Ventricular Dysfunction

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