Contemporary Trends in the Diagnosis and Management of PAH: An Initiative To Close the Care Gap

McLaughlin, Vallerie V.; Langer, Anatoly; Dragomir, Alina; Casanova, Amparo; Tan, Mary; Clements, Philip J.; Oudiz, Ronald J.; Tapson, Victor F.; Channick, Richard N.; Rubin, Lewis J.

doi:10.1378/chest.9901

Cited by 43 publications

(64 citation statements)

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“…These results therefore suggest that, on the basis of the guideline recommendations, a high proportion of patients in this study did not have a confirmed diagnosis of CTEPH, highlighting the difficulties in diagnosing CTEPH, even in specialist centers, and a potential need for increased education and/or resources. The low V/Q scanning rates found in this study may also indirectly support previous findings from PH registries indicating that knowledge gaps exist in correctly diagnosing PH, 8 as it can be speculated that incorrect decisions made during PH diagnosis may subsequently affect the correct diagnostic workup for CTEPH, particularly the use of V/Q scanning.…”

Section: Discussionsupporting

confidence: 82%

An International Physician Survey of Chronic Thromboembolic Pulmonary Hypertension Management

et al. 2016

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Abstract:We conducted an international study to evaluate practices in the diagnosis and management of patients with chronic thromboembolic pulmonary hypertension (CTEPH) globally across different regions. Between August and October 2012, CTEPH-treating physicians completed a 15-minute online questionnaire and provided patient record data for their 2-5 most recent patients with CTEPH. Overall, 496 physicians (Europe: 260; United States: 152; Argentina: 52; Japan: 32) completed the questionnaire and provided patient record data for 1,748 patients. The proportion of physicians who described themselves as working in or affiliated with a specialized pulmonary hypertension (PH) center ranged from 38% in France and Italy to 83% in the United States. A large proportion of patients did not undergo ventilation/perfusion scanning (46%-67%) or right heart catheterization (24%-57%) for the diagnosis of CTEPH. Referral rates for pulmonary endarterectomy evaluation ranged from 25% in Japan to 44% in Europe, with higher referral rates in PH centers; the main reasons for lack of referral were that surgery was not considered unless medical treatment was failing and patient refusal. Other variations in management included greater use of phosphodiesterase 5 inhibitors in the United States than in Europe and Japan and greater use of combination treatment in the United States than in Europe. Physicians' perceptions of their treatment strategy were generally consistent with patient record data. Results from this study, which includes a global aspect of CTEPH care, demonstrate not only regional differences in CTEPH management but, more importantly, considerable nonadherence to the diagnosis and treatment guidelines for CTEPH, even in PH centers.Keywords: chronic thromboembolic pulmonary hypertension, pulmonary arterial hypertension-specific therapies, diagnosis, clinical practice, guidelines. Chronic thromboembolic pulmonary hypertension (CTEPH) is a disease of obstructive pulmonary artery remodeling as a consequence of major vessel thromboembolism. 1 A ventilation/perfusion (V/Q) scan is recommended in the workup of all patients with pulmonary hypertension (PH) to screen for CTEPH. 1 Diagnosis of CTEPH is challenging for several reasons, including nonspecific presenting symptoms, which may occur late in the progression of the disease, and a lack of prior pulmonary embolism in a high percentage of patients. 2 Once CTEPH is diagnosed, pulmonary endarterectomy (PEA) is the gold-standard treatment for these patients and is potentially curative. 1,3 However, between 20% and 40% of these patients are considered to have inoperable CTEPH, 4 and approximately 30% may have residual PH after PEA (defined as mean pulmonary artery pressure of ≥30 mmHg at 3-month follow-up after surgery). 5 Balloon pulmonary angioplasty (BPA) is an emerging treatment option for patients with inoperable or persistent CTEPH. 3 However, the role of BPA in CTEPH has not been investigated in randomized trials, and further studies are warranted; current guidelines state ...

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Section: Discussionsupporting

confidence: 82%

An International Physician Survey of Chronic Thromboembolic Pulmonary Hypertension Management

et al. 2016

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“…and s.c. prostanoids in patients with severe disease. Despite the known benefits of parenteral prostanoids, in clinical practice they have been underused [35,36]. A recent single-centre study indicated that this may not necessarily be the case in expert PAH centres [37], highlighting the importance of patient referral to, and co-management with, expert centres.…”

Section: Combination Therapy For Optimal Pah Managementmentioning

confidence: 99%

“…or s.c. prostanoids due to their routes of administration, and this may contribute to their underuse [25,35,36]. To address this, it is imperative to educate the patient on the benefits of parenteral prostanoids, and to support them throughout the process [25,39].…”

Section: Combination Therapy For Optimal Pah Managementmentioning

confidence: 99%

Pulmonary arterial hypertension: tailoring treatment to risk in the current era

Gaine

McLaughlin

2017

Eur Respir Rev

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Recent advances in the treatment of pulmonary arterial hypertension (PAH) have led to improved patient outcomes. Multiple PAH therapies are now available and optimising the use of these drugs in clinical practice is vital. In this review, we discuss the management of PAH patients in the context of current treatment guidelines and supporting clinical evidence. In clinical practice, considerable emphasis is placed on the importance of making treatment decisions guided by each patient's risk status, which should be assessed using multiple prognostic parameters. As PAH is a progressive disease, regular assessments are essential to ensure that any change in risk is detected in a timely manner and treatment is adjusted accordingly. With the availability of therapies that target three different pathogenic pathways, combination therapy is now the standard of care. For most patients, this involves dual combination therapy with agents targeting the endothelin and nitric oxide pathways. Therapies targeting the prostacyclin pathway should be added for patients receiving dual combination therapy who do not achieve a low-risk status. There is also a need for a holistic approach to treatment beyond pharmacological therapies. Implementation of all these approaches will ensure that PAH patients receive maximal benefit from currently available therapies.

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“…The 2013 Pulmonary Arterial Hypertension Quality Enhancement Research Initiative reported high rates of RHC utilisation across academic (96.3%) and community (84.4%) centres [44]. However, there was some disparity in RHC use between the two types of centres (i.e.…”

Section: Evolving Utilisation and Application Of Rhc In Clinical Pracmentioning

confidence: 99%

Right heart catheterisation: best practice and pitfalls in pulmonary hypertension

Rosenkranz¹,

2015

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Right heart catheterisation (RHC) plays a central role in identifying pulmonary hypertension (PH) disorders, and is required to definitively diagnose pulmonary arterial hypertension (PAH). Despite widespread acceptance, there is a lack of guidance regarding the best practice for performing RHC in clinical practice. In order to ensure the correct evaluation of haemodynamic parameters directly measured or calculated from RHC, attention should be drawn to standardising procedures such as the position of the pressure transducer and catheter balloon inflation volume. Measurement of pulmonary arterial wedge pressure, in particular, is vulnerable to over-or under-wedging, which can give rise to false readings. In turn, errors in RHC measurement and data interpretation can complicate the differentiation of PAH from other PH disorders and lead to misdiagnosis. In addition to diagnosis, the role of RHC in conjunction with noninvasive tests is widening rapidly to encompass monitoring of treatment response and establishing prognosis of patients diagnosed with PAH. However, further standardisation of RHC is warranted to ensure optimal use in routine clinical practice. @ERSpublications Right heart catheterisation is required to diagnose pulmonary arterial hypertension, but standardisation is needed http://ow.ly/TI6Gr

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Contemporary Trends in the Diagnosis and Management of PAH: An Initiative To Close the Care Gap

Cited by 43 publications

References 0 publications

An International Physician Survey of Chronic Thromboembolic Pulmonary Hypertension Management

An International Physician Survey of Chronic Thromboembolic Pulmonary Hypertension Management

Pulmonary arterial hypertension: tailoring treatment to risk in the current era

Right heart catheterisation: best practice and pitfalls in pulmonary hypertension

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