Changes in Right Ventricular Function Measured by Cardiac Magnetic Resonance Imaging in Patients Receiving Pulmonary Arterial Hypertension–Targeted Therapy

Peacock, Andrew J.; Crawley, Stephen F.; McLure, L. E. R.; Blyth, Kevin G.; Vizza, Carmine Dario; Poscia, Roberto; Francone, Marco; Iacucci, Ilaria; Olschewski, Horst; Kovàcs, Gabór; Noordegraaf, Anton Vonk; Marcus, J. Tim; Veerdonk, Mariëlle C. van de; Oosterveer, Frank

doi:10.1161/circimaging.113.000629

Cited by 136 publications

(114 citation statements)

References 43 publications

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“…47 Cardiac MRI-derived parameters, such as right ventricular and left ventricular volumes and ratios, mass, and ejection fraction correlate with traditional measures of functional status, including 6MWD, 46,48,49 hemodynamics, 50 and survival. [50][51][52][53][54] In addition, viewing MRI parameters in the context of other standard risk parameters may allow us a better spatial assessment of risk of the continuum of each individual factor ( Figure 6).…”

Section: Imagingmentioning

confidence: 99%

Risk Assessment in Pulmonary Arterial Hypertension Patients: The Long and Short of it

Benza

Lohmueller

Kraisangka

et al. 2018

Advances in Pulmonary Hypertension

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Pulmonary arterial hypertension (PAH) is a chronic and rapidly progressive disease that is characterized by extensive narrowing of the pulmonary vasculature, leading to increases in pulmonary vascular resistance, subsequent right ventricular dysfunction, and eventual death. There are currently multiple approved drugs—developed as single or combination therapies in the last few years—that have improved outcome and functionality in PAH. However, despite improvement in short-term survival with these new effective therapies, PAH remains an incurable disease with a median survival of 7 years (Figure 1).1 This chronic disease state may be characterized by morbid events such as hospitalizations that herald rapid disease progression and account for a significant disease burden (Figure 2).23 Physician ability to predict PAH disease progression is critical for determining optimal care of patients. Accurate risk assessment allows clinicians to determine the patient's prognosis, identify treatment goals, and monitor disease progression and the patient's response to treatment. Risk assessment for PAH patients should include a range of clinical, hemodynamic, and exercise parameters, performed in a serial fashion over the treatment course. Patient risk stratification can also help physicians better allocate treatment resources in settings where they are scarce. If widely adopted, risk prediction can enhance the consistency of treatment approaches across PAH practitioners and improve the timeliness of referral for lung transplantation. Hence, along with advancing PAH treatment options, comprehensive risk prediction is essential to make individualized treatment decisions in the current treatment era. Several tools are currently available for assessing risk in PAH (Figure 3). These include the 2015 European Society of Cardiology/European Respiratory Society pulmonary hypertension guidelines' risk variables,4 the French registry equation,5 the National Institutes of Health risk equation,6 or a risk score such as the one derived from the Registry to Evaluate Early And Long-term PAH Disease Management.1 These registries and evaluations of clinical trial sets have provided important insights into the importance of both modifiable (eg, 6-minute walk distance, functional class, brain natriuretic peptide, and nonmodifiable (eg, age, gender, PAH etiology) risk factors that predict survival. The following review explores commonly cited risk factors, both modifiable and nonmodifiable, and their implications for patient outcomes.

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Section: Imagingmentioning

confidence: 99%

Risk Assessment in Pulmonary Arterial Hypertension Patients: The Long and Short of it

Benza

Lohmueller

Kraisangka

et al. 2018

Advances in Pulmonary Hypertension

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“…74 The prognostic value of changes in RV mass in PAH is small and seems limited to patients with scleroderma PAH. 69,75,76 The limited clinical value of the assessment of RV mass compared with RV volumes is perhaps explained by the fact that RV hypertrophy both reflects the severity of pulmonary vascular remodeling (negative impact) and is part of the normal adaptive process to an increased load (positive response). 77 For example, despite a similar elevation in PAP, patients with Eisenmenger syndrome have a greater amount of RV hypertrophy compared with patients with idiopathic PAH, which is associated with better RV function and survival.…”

Section: Rv Massmentioning

confidence: 99%

Noninvasive Imaging in the Assessment of the Cardiopulmonary Vascular Unit

et al. 2015

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899N oninvasive imaging plays a key role in both the diagnosis and management of patients with pulmonary hypertension (PH). In recent years, there have been 2 major changes in perspective of imaging in PH. The first was the realization that imaging should focus on the evaluation of not only the pulmonary pressures but also the cardiopulmonary unit ( Figure 1). 1,2The second was the emergence of multimodality imaging with a complementary role for echocardiography, magnetic resonance (MRI), computed tomography, and positron emission tomography (PET).2-7 These techniques not only help in the diagnosis of PH but also help identify factors that determine risk and prognosis and gauge therapeutic effects on right ventricular (RV) function in patients with pulmonary arterial hypertension (PAH). Although echocardiography is the mainstay in the assessment of hemodynamic and ventricular function in PH, MRI has emerged as the gold standard for quantifying volumes, function, and flow in the right side of the heart. 2-7 PET is also offering novel insights into perfusion and blood flow, metabolism, neurohormonal activation, and other molecular processes in the right side of the heart but is used mainly for research at this time. Catheterization of the right side of the heart remains the gold standard for defining PH and assessing hemodynamics both at rest and with exercise. Invasive assessment of cardiac output (CO) may, however, have limited accuracy when assumed instead of measured oxygen consumption is used to derive CO (eg, using the Fick method) or when thermodilution is used in the setting of a low-CO state. [8][9][10] This review covers RV imaging studies performed in the field of PH and discusses recent advances in echocardiography and cardiac MRI and PET imaging for detailed assessment of RV function. The review starts with a discussion of important physiological considerations and unmet needs in imaging research of the right side of the heart in PH. Computed tomography angiography, which plays an important role in evaluating chronic thromboembolic PH, is not discussed extensively in this review. Physiological ConsiderationsAlthough PH is a syndrome affecting the pulmonary vasculature, survival of patients with PH is closely related to RV function. 1,[11][12][13][14] The RV initially adapts to the increased afterload by increasing its wall thickness and contractility. These mechanisms are, however, often insufficient, and RV dysfunction eventually occurs. After the recent Fifth World Symposium on Pulmonary Hypertension, a definition of failure of the right side of the heart secondary to PH was adopted: "Right heart failure in the setting of PH can be defined as a complex clinical syndrome due to a suboptimal delivery of blood or elevated systemic venous pressure at rest or exercise as a consequence of elevated RV afterload."2 The proposed definition takes into account both the systolic and diastolic characteristics of function of the right side of the heart, as well as physiological demands such as exercise.In underst...

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“…Taken together, these findings hold promise for the increased use of CMR protocols in both clinical trials and practice. 38 …”

Section: Cardiac Mrimentioning

confidence: 99%

Clinical Diagnosis of Pulmonary Hypertension

Rich

2014

Circulation

105

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1820T here has been an increased recognition of pulmonary hypertension (PH) in clinical practice over the past 30 years. It is likely that this rise in PH diagnoses is attributable to multiple factors, including increased awareness by clinicians, the routine use of diagnostic tools such as Doppler echocardiography, and the availability and marketing of the many PH-specific drugs.1 Although the increased awareness of the disease should be viewed as a favorable development, the unintended consequences include overdiagnosis, misclassification, and at times indiscriminate use of expensive, PH-specific drugs. In this review, we address the epidemiology, prognosis, and updated clinical classification of PH. We then focus on the diagnostic approach to the patient with suspected PH with an emphasis placed on highlighting key pearls for the clinician to consider. Finally, we review an evolving conceptual framework of viewing the pulmonary vasculature and the right ventricle as a single, coupled cardiopulmonary unit.

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Changes in Right Ventricular Function Measured by Cardiac Magnetic Resonance Imaging in Patients Receiving Pulmonary Arterial Hypertension–Targeted Therapy

Cited by 136 publications

References 43 publications

Risk Assessment in Pulmonary Arterial Hypertension Patients: The Long and Short of it

Risk Assessment in Pulmonary Arterial Hypertension Patients: The Long and Short of it

Noninvasive Imaging in the Assessment of the Cardiopulmonary Vascular Unit

Clinical Diagnosis of Pulmonary Hypertension

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