Pulmonary Arterial Hypertension Combined with a High Cardiac Output State: Three Remarkable Cases

Spruijt, Onno A.; Bogaard, Harm Jan; Vonk‐Noordegraaf, Anton

doi:10.4103/2045-8932.113185

Cited by 12 publications

(15 citation statements)

References 15 publications

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“…4,10,11 An increased prevalence of spontaneous portosystemic shunts in patients with POPH as well as the development of POPH in patients with congenital portosystemic shunts with normal liver function suggests that vasoactive factors from the splanchnic circulation, rather than the presence of liver failure per se, contribute to disease development. 12,13 These factors may represent novel therapeutic targets and may play an important role in the pathogenesis of other subtypes of PAH. The purpose of our study was to identify biomarkers of POPH disease presence, severity, and treatment response.…”

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confidence: 99%

Macrophage Migration Inhibitory Factor as a Novel Biomarker of Portopulmonary Hypertension

et al. 2016

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Portopulmonary hypertension (POPH) is a poorly understood complication of liver disease associated with significant morbidity and mortality. We sought to identify novel biomarkers of POPH disease presence and severity. We performed a prospective, multicenter, case-control study involving patients with liver disease undergoing right heart catheterization. POPH cases were defined as a mean pulmonary arterial pressure (mPAP) ≥25 mmHg and pulmonary vascular resistance (PVR) >240 dynes˙s˙cm. Plasma samples were collected from the systemic and pulmonary circulation, and antibody microarray was used to identify biomarkers. Characterization and validation of a candidate cytokine, macrophage migration inhibitory factor (MIF), was performed using enzyme-linked immunosorbent assay. Continuous variables were compared using a Mann-Whitney U test and correlated with disease severity using Spearman correlation. MIF levels were elevated in both the systemic and pulmonary circulation in patients with POPH compared with controls .53], P = 0.002). In patients with POPH, MIF levels were positively correlated with PVR (r = 0.58, P = 0.006) and inversely correlated with cardiac output (r = −0.57, P = 0.007). MIF >60 ng/mL or tricuspid regurgitation gradient >50 mmHg had a 92% sensitivity and specificity for the diagnosis of POPH, with a positive predictive value of 86% and a negative predictive value of 96%. MIF is a promising novel biomarker of POPH disease presence and severity in patients with liver disease and portal hypertension.

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confidence: 99%

Macrophage Migration Inhibitory Factor as a Novel Biomarker of Portopulmonary Hypertension

et al. 2016

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“…In reality, the heart compensates for the increased downstream resistance by remodeling, leading to increased cardiac wall thickness and hypertrophy 21,48,62 . In chronic PH a decrease in cardiac output due to these factors is expected [62][63][64] . However, it is beyond the scope of this study to attempt to model cardiac remodeling in response to PH, and as such this model provides an upper limit for the pressure response to vascular pathology within the lungs.…”

Section: Discussionmentioning

confidence: 99%

“…In addition, clinically measured pulsatility indices, which normalize by right atrial pressure, cannot be directly calculated by our model which is limited to assessing normalized pulse pressure as a proxy measure. In reality, the late-stage disease cardiac output is likely to decrease [62][63][64] In summary, our model predicts that there are potentially quantifiable differences in metrics reflecting the dynamic relationship between blood pressure and flow in the main pulmonary artery between CTEPH and PAH, but that these differences should be interpreted with care, particularly when metrics representing this dynamic response change non-linearly with disease progression (for example when fibrotic changes begin to occur in the vasculature).…”

Section: Model Limitationsmentioning

confidence: 92%

A computational model of contributors to pulmonary hypertensive disease: impacts of whole lung and focal disease distributions

et al. 2021

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Pulmonary hypertension (PH) has multiple etiologies, and so can be difficult to diagnose, prognose, and treat. Diagnosis is typically made via invasive hemodynamic measurements in the main pulmonary artery, and is based on observed elevation of mean pulmonary artery pressure. This static mean pressure enables diagnosis, but does not easily allow assessment of the severity of PH, nor the etiology of the disease, which may impact treatment. Assessment of the dynamic properties of pressure and flow data obtained from catheterization potentially allows more meaningful assessment of the strain on the right heart, and may help to distinguish between disease phenotypes. However, mechanistic understanding of how the distribution of disease in the lung leading to PH impacts the dynamics of blood flow in the main pulmonary artery and/or the pulmonary capillaries is lacking. We present a computational model of the pulmonary vasculature, parameterized to characteristic features of pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) to help understand how the two conditions differ in terms of pulmonary vascular response to disease. Our model incorporates key features known to contribute to pulmonary vascular function in health and disease, including anatomical structure and multiple contributions from gravity. The model suggests that dynamic measurements obtained from catheterization potentially distinguish between distal and proximal vasculopathy typical of PAH and CTEPH. However, the model suggests a non-linear relationship between these data and vascular structural changes typical of PAH and CTEPH which may impede analysis of these metrics to distinguish between cohorts.

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“…We report a case of POPH after TIPS in a patient with cirrhosis and portal hypertension. POPH is a recognized 6 despite the absence of portal hypertension and cirrhosis. Likewise, surgical creation of a portosystemic anastomosis is associated with PAH.…”

Section: Discussionmentioning

confidence: 99%

A Case Report of Portopulmonary Hypertension Precipitated by Transjugular Intrahepatic Portosystemic Shunt

Tatah

Weir

Prins

et al. 2021

Chest

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Pulmonary Arterial Hypertension Combined with a High Cardiac Output State: Three Remarkable Cases

Cited by 12 publications

References 15 publications

Macrophage Migration Inhibitory Factor as a Novel Biomarker of Portopulmonary Hypertension

Macrophage Migration Inhibitory Factor as a Novel Biomarker of Portopulmonary Hypertension

A computational model of contributors to pulmonary hypertensive disease: impacts of whole lung and focal disease distributions

A Case Report of Portopulmonary Hypertension Precipitated by Transjugular Intrahepatic Portosystemic Shunt

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