scholarly journals The haemodynamic assessment of patients with pulmonary arterial hypertension

2020 ◽  
Vol 2020 (1) ◽  
Author(s):  
Stefano Ghio
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Drug Efficacy ◽  
Right Heart Catheterization ◽  
Heart Catheterization ◽  
Right Heart ◽  
Accurate Knowledge ◽  
Pulmonary Arterial ◽  
Technical Recommendations

[No abstract. Showing first paragraph of article]Right heart catheterization (RHC) has a crucial role in the management of patients with pulmonary hypertension (PH). Firstly, it is mandatory in subjects with the suspicion of PH to confirm the diagnosis. Secondly, it is strongly recommended in the follow-up of patients with confirmed pulmonary arterial hypertension (PAH) to evaluate drug efficacy and in case of clinical deterioration.In order to always perform a complete and correct RHC, clinicians carrying out the examination must be guided not only by technical recommendations, but also by the accurate knowledge of the different clinical issues which have to be addressed in these different situations.

scholarly journals Right ventricular dimension index by cardiac magnetic resonance for prognostication in connective tissue diseases and pulmonary hypertension

Rheumatology ◽  
2019 ◽  
Author(s):  
Nobuya Abe ◽  
Masaru Kato ◽  
Michihito Kono ◽  
Yuichiro Fujieda ◽  
Hiroshi Ohira ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Right Ventricular ◽  
Right Heart Catheterization ◽  
Heart Catheterization ◽  
Right Heart ◽  
Pulmonary Arterial ◽  
Dimension Index

Abstract Objectives Pulmonary hypertension (PH) in patients with CTD is a heterogeneous condition affected by left heart disease, chronic lung disease and thromboembolism as well as pulmonary vascular disease. Recent studies using cardiac magnetic resonance (CMR) have shown that right ventricular dysfunction is predictive for mortality in patients with PH, but limited to pulmonary arterial hypertension. This study aimed to analyse prognostic factors in PH-CTD. Methods This retrospective analysis comprised 84 CTD patients, including SSc, who underwent both CMR and right heart catheterization from 2008 to 2018. Demographics, laboratory findings, and haemodynamic and morphological parameters were extracted. The prognostic value of each parameter was evaluated by multivariate analysis using covariables derived from propensity score to control confounding factors. Results Of 84 patients, 65 had right heart catheterization-confirmed PH (54 pulmonary arterial hypertension, 11 non-pulmonary arterial hypertension). Nine out of these PH patients died during a median follow-up period of 25 months. In 65 patients with PH, right ventricular end-diastolic dimension index (RVEDDI) evaluated by CMR was independently associated with mortality (hazard ratio 1.24; 95% CI: 1.08–1.46; P = 0.003). In a receiver operating characteristic analysis, RVEDDI highly predicted mortality, with area under the curve of 0.87. The 0.5–2-year follow-up data revealed that RVEDDI in both survivors and non-survivors did not significantly change over the clinical course, leading to the possibility that an early determination of RVEDDI could predict the prognosis. Conclusion RVEDDI simply evaluated by CMR could serve as a significant predictor of mortality in PH-CTD. A further validation cohort study is needed to confirm its usability.

scholarly journals Seizures in Idiopathic Pulmonary Arterial Hypertension

2018 ◽  
Vol 05 (02) ◽  
pp. 107-109
Author(s):  
Jamir Pitton Rissardo ◽  
Ana Letícia Fornari Caprara
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Supplemental Oxygen ◽  
Right Heart Catheterization ◽  
Idiopathic Pulmonary Arterial Hypertension ◽  
Heart Catheterization ◽  
Right Heart ◽  
Systemic Disorder ◽  
Pulmonary Arterial

AbstractPulmonary arterial hypertension (PAH) is a progressive pulmonary vasculopathy. A 29-year-old female patient presenting with dyspnea and syncope within 6 hours of onset was admitted to our hospital. The patient stated that she looked for a neurologist months ago because she experienced abrupt shaking limbs occurring during physical activity. She was diagnosed with focal seizure, and carbamazepine (CBZ) was started. On admission, she reported that the dyspnea had started in the last week and recurrent episodes of syncope in the last few hours. A right heart catheterization was diagnostic of PAH. She was started on spironolactone, furosemide, sildenafil, warfarin, and supplemental oxygen. On 10th admission day, the patient was seizure free and the dose of CBZ was tapered. In the follow-up, the patient remained seizure free. An investigation to search for a chronic lung disease or hypoxemia, systemic disorder, hematological disorder, and metabolic disorder was negative.

scholarly journals Schistosomiasis Pulmonary Arterial Hypertension

2020 ◽  
Vol 11 ◽  
Author(s):  
Jean Pierre Sibomana ◽  
Aloma Campeche ◽  
Roberto J. Carvalho-Filho ◽  
Ricardo Amorim Correa ◽  
Helena Duani ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Right Heart Failure ◽  
Right Heart Catheterization ◽  
Heart Catheterization ◽  
Right Heart ◽  
Systematic Screening ◽  
Pulmonary Vasodilators ◽  
Pulmonary Arterial

Pulmonary arterial hypertension (PAH) is a disease of the lung blood vessels that results in right heart failure. PAH is thought to occur in about 5% to 10% of patients with hepatosplenic schistosomiasis, particularly due to S. mansoni. The lung blood vessel injury may result from a combination of embolization of eggs through portocaval shunts into the lungs causing localized Type 2 inflammatory response and vessel remodeling, triggering of autonomous pathology that becomes independent of the antigen, and high cardiac output as seen in portopulmonary hypertension. The condition is likely underdiagnosed as there is little systematic screening, and risk factors for developing PAH are not known. Screening is done by echocardiography, and formal diagnosis requires invasive right heart catheterization. Patients with Schistosoma-associated PAH show reduced functional capacity and can be treated with pulmonary vasodilators, which improves symptoms and may improve survival. There are animal models of this disease that might help in understanding disease pathogenesis and identify novel targets to screen and treatment. Pathogenic mechanisms include Type 2 immunity and activation and signaling in the TGF-β pathway. There are still major uncertainties regarding Schistosoma-associated PAH development, course and treatment.

Pulmonary Arterial Hypertension in Systemic Sclerosis Is Associated with Profound Impairment of Microvascular Endothelium-dependent Vasodilatation

2011 ◽  
Vol 39 (1) ◽  
pp. 100-105 ◽  
Author(s):  
HERMAN M.A. HOFSTEE ◽  
ALEXANDRE E. VOSKUYL ◽  
ANTON VONK NOORDEGRAAF ◽  
YVO M. SMULDERS ◽  
PIET E. POSTMUS ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Right Heart Catheterization ◽  
Microvascular Function ◽  
Heart Catheterization ◽  
Microvascular Endothelium ◽  
Right Heart ◽  
Doppler Flowmetry ◽  
Pulmonary Arterial

Objective.Impaired microvascular function may contribute to organ complications in patients with systemic sclerosis (SSc). We investigated whether SSc patients with and without pulmonary arterial hypertension (PAH) show a graded impairment of microvascular function compared to healthy controls.Methods.Twenty-two patients with SSc and 22 controls were studied. All patients underwent right heart catheterization; 6 had no PAH (SSc-nonPAH) and 16 had PAH (SSc-PAH). Acetylcholine (ACh)-mediated endothelium-dependent vasodilatation and sodium nitroprusside (SNP)-mediated endothelium-independent vasodilatation were assessed by iontophoresis combined with laser Doppler flowmetry.Results.Compared to sex- and age-matched controls, ACh-mediated vasodilatation was reduced in SSc-PAH (340.4% vs 79.5%, respectively; p < 0.01), but not in SSc-nonPAH (340.4% vs 397.9%; p = 0.90). No significant differences were present between the groups in SNP-mediated vasodilatation.Conclusion.Systemic microvascular endothelium-dependent vasodilatation is markedly reduced in SSc complicated by PAH.

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