High prevalence of occult left heart disease in scleroderma-pulmonary hypertension

Fox, Benjamin D.; Shimony, Avi; Langleben, David; Hirsch, Andrew; Rudski, Lawrence; Schlesinger, Robert; Eisenberg, Mark J.; Joyal, Dominique; Hudson, Marie; Boutet, Kim; Serban, Alexandrina; Masetto, Ariel; Baron, Murray

doi:10.1183/09031936.00091212

Cited by 132 publications

(107 citation statements)

References 25 publications

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“…20 Fluid challenge during RHC to elicit latent pulmonary venous hypertension (PVH), discussed in a recent summary statement on group 2 PH, 21 is routinely performed in a minority of centers. However, there is increasing evidence that this simple test may be useful in identifying latent PVH, 22,23 with over 20% of patients exhibiting an increase in PWP >15 mmHg after rapid infusion of 500 mL normal saline. 22,24 Given that exercise testing and fluid challenge are not commonly performed at most PH centers, additional study of these maneuvers in patients with PAH and group 2 PH to define sensitivity and specificity is needed before firm consensus recommendations and widespread adoption of this practice should occur.…”

Section: Discussionmentioning

confidence: 99%

Variability in Hemodynamic Evaluation of Pulmonary Hypertension at Large Referral Centers

et al. 2014

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Despite consensus guidelines for right heart catheterization (RHC) in the diagnosis of pulmonary arterial hypertension (PAH), considerable differences exist in the performance of RHC, interpretation of hemodynamic data, and frequency of RHC performance in patients with established disease. These differences may lead to variability in diagnosis or treatment of PAH. We sought to gather information on the standard practice of RHC for the diagnosis and management of PAH from experienced pulmonary vascular disease specialists. We developed a semiquantitative online survey of diagnosis and treatment patterns of pulmonary hypertension and distributed it to physicians at pulmonary hypertension centers in the United States. Thirty of 50 physicians completed the survey: 20 pulmonologists and 10 cardiologists, all of whom reported treating >100 patients with PAH in the past year. All respondents perform RHC in ≥90% of patients with suspected PAH. All physicians determine the pulmonary wedge pressure at end expiration; however, only half of respondents personally review tracings. Physicians differed in frequency of vasodilator testing (8 of 24 performed testing in >90% of patients with PAH), fluid challenge and exercise (19 of 30 performed testing in <25% of patients with PAH for both). Most physicians (70%) report repeating RHC between 6 months and 1 year after PAH treatment initiation. Variability exists in the interpretation of hemodynamic tracings and performance of vasodilator, fluid, and exercise challenges in the management of PAH by experienced physicians in the United States. Additional consensus guidelines delineating appropriate adjunctive testing to standardize the diagnosis of PAH are needed.

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Section: Discussionmentioning

confidence: 99%

Variability in Hemodynamic Evaluation of Pulmonary Hypertension at Large Referral Centers

et al. 2014

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“…Based on PAWP, 29 out of 53 had PAH and 24 had post-capillary PH. However, after fluid challenge, 11 PAH patients were reclassified as displaying occult post-capillary PH [34] although there was no follow-up data precluding any firm conclusion. Such an occult post-capillary component may explain, at least in part, the worst outcomes of SSc-associated PAH in randomised clinical trials.…”

Section: Cardiac Involvementmentioning

confidence: 99%

“…Myocardial fibrosis can lead to left-ventricular dysfunction and could either explain the development of pure post-capillary PH or contribute to a post-capillary component in SSc patients with pulmonary arteriopathy. In a recent study by FOX et al [34], the authors assessed the prevalence of occult left-heart disease in SSc-PH. In a population of 107 patients with SSc, 53 had PH confirmed by right-heart catheterisation.…”

Section: Cardiac Involvementmentioning

confidence: 99%

Pulmonary hypertension in systemic sclerosis: different phenotypes

et al. 2017

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“…However, pulmonary hyperten sion in systemic sclerosis is complicated by the fact that elevation of pulmonary pressure can also be caused -or at least be aggravated -by parenchymal lung disease and left heart failure making it very diffi cult to identify the causative factor that results in ele vated pulmonary pressure [21]. Additional vasodilata tion of the pulmonary circulation may aggravate left heart failure and explain why patients with pulmonary hypertension associated with systemic sclerosis have a worse outcome when treated with PHtarget therapies compared to those with pulmonary arterial hyperten sion due to other aetiologies [22]. Patients with connec tive tissue disease should be screened with annual echocardiography or when developing symptoms.…”

Section: Connective Tissue Diseasementioning

confidence: 99%

Pulmonary hypertension: Classification and pathobiology

2014

Cardiovasc Med

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SummaryPulmonary hypertension is defined as an increase of the mean pulmonary arterial pressure above 25 mm Hg and, as such, the diagnosis requires invasive haemo dynamic measurement by right heart catheterisation. More than just a single disease, pulmonary hyperten sion is an umbrella term that includes many different disorders and pathophysiological entities. However, most forms of pulmonary hypertension share a final common pathway, in particular the trias of vasocon striction, microthrombosis and vascular remodelling. The classification of pulmonary hypertension has been subjected to many changes within the last decade. At the same time, major achievements in our understand ing of the complex pathobiology have been made. Here, these developments are discussed in the light of the re cently published report from the fifth World Sympo sium on pulmonary hypertension. Definition and haemodynamicsPulmonary arterial pressure (PAP) is a function of car diac output, left atrial pressure and pulmonary vascu lar resistance. The pulmonary circulation is a lowpres sure system and the mean pulmonary arterial pressure (mPAP) does not exceed 20 mm Hg in healthy individ uals [1]. Pulmonary hypertension is defined as an in crease of the mPAP above 25 mm Hg. Pressure values ranging from 21 to 24 mm Hg have been associated with a high risk to develop overt pulmonary hyperten sion in a subgroup of patients, for example those with connective tissue disease [2], but otherwise these pa tients are not well defined and not much data exist on the prognostic and therapeutic consequences. Use of the term "borderline pulmonary hypertension" is thus not recom mended [3]. Increasing age [4] and body weight [5] have been associ ated with a rise of systolic pulmo nary arterial pressure (sPAP) and might explain the gap between nor mal mPAP and pulmonary hyper tension.The presence of pulmonary hypertension might be determined by echocardiography, which, depending on the underlying cause of elevated pulmonary pressure, provides an excellent screening tool. However, sensitiv ity and specificity of this method is limited [6]. Diagno sis is thus confirmed by invasive haemodynamic as sessment with right heart catheterisation at rest. An inadequate elevation of pulmonary pressure upon ex ercise has been part of former definitions of pulmonary hypertension but, due to lack of standardisation, is no longer a criterion.The differentiation of pulmonary hypertension due to left heart disease from the other forms is crucial. As suggested by guidelines, the pulmonary arterial wedge pressure (PAWP, also known as "wedge pressure") is used to estimate left atrial pressure in clinical practice. In healthy subjects, PAWP reflects left atrial pressure, which is extensively determined by left ventricular enddiastolic pressure (LVEDP). Several conditions in terfere with this assumption and may lead to misclas sification of pulmonary hypertension. Since pulmonary vessels are not stiff pipes but rather compressible tubes, an increase of the alveolar pressure (e.g., ...

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High prevalence of occult left heart disease in scleroderma-pulmonary hypertension

Cited by 132 publications

References 25 publications

Variability in Hemodynamic Evaluation of Pulmonary Hypertension at Large Referral Centers

Variability in Hemodynamic Evaluation of Pulmonary Hypertension at Large Referral Centers

Pulmonary hypertension in systemic sclerosis: different phenotypes

Pulmonary hypertension: Classification and pathobiology

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