scholarly journals Right ventricle performances with echocardiography and 99mTc myocardial perfusion imaging in pulmonary arterial hypertension patients

2018 ◽  
Vol 243 (9) ◽  
pp. 754-761
Author(s):  
Jie Liu ◽  
Lei Fei ◽  
Guang-Qing Huang ◽  
Xiao-Ke Shang ◽  
Mei Liu ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Myocardial Perfusion ◽  
Arterial Pressure ◽  
Right Ventricle ◽  
Arterial Hypertension ◽  
Pulmonary Arterial Pressure ◽  
Right Heart Catheterization ◽  
Heart Catheterization ◽  
Mean Pulmonary Arterial Pressure ◽  
Pulmonary Arterial

Right heart catheterization is commonly used to measure right ventricle hemodynamic parameters and is the gold standard for pulmonary arterial hypertension diagnosis; however, it is not suitable for patients’ long-term follow-up. Non-invasive echocardiography and nuclear medicine have been applied to measure right ventricle anatomy and function, but the guidelines for the usefulness of clinical parameters remain to be established. The goal of this study is to identify reliable clinical parameters of right ventricle function in pulmonary arterial hypertension patients and analyze the relationship of these clinical parameters with the disease severity of pulmonary arterial hypertension. In this study, 23 normal subjects and 23 pulmonary arterial hypertension patients were recruited from January 2015 to March 2016. Pulmonary arterial hypertension patients were classified into moderate and severe pulmonary arterial hypertension groups according to their mean pulmonary arterial pressure levels. All the subjects were subjected to physical examination, chest X-ray, 12-lead electrocardiogram, right heart catheterization, two-dimensional echocardiography, and technetium 99m (99mTc) myocardial perfusion imaging. Compared to normal subjects, the right heart catheterization indexes including right ventricle systolic pressure, right ventricle end diastolic pressure, pulmonary artery systolic pressure, pulmonary artery diastolic pressure, pulmonary vascular resistance, and right ventricle end systolic pressure increased in pulmonary arterial hypertension patients and were correlated with mean pulmonary arterial pressure levels. Echocardiography parameters, including tricuspid regurgitation peak velocity, tricuspid regurgitation pressure gradient, tricuspid annular plane systolic excursion and fractional area, right ventricle-myocardial performance index, were significantly associated with the mean pulmonary arterial pressure levels in pulmonary arterial hypertension patients. Furthermore, myocardial perfusion imaging was not observed in the normal subjects but in pulmonary arterial hypertension patients, especially severe pulmonary arterial hypertension subgroup, and showed potential diagnostic properties for pulmonary arterial hypertension. In conclusion, mean pulmonary arterial pressure levels are correlated with several right heart catheterization and echocardiography markers in pulmonary arterial hypertension patients; echocardiography and 99mTc myocardial perfusion can be used to evaluate right ventricle performance in pulmonary arterial hypertension patients. Impact statement In this study, we analyzed the clinical parameters for evaluating RV function, including right ventricle catheterization (RHC), echocardiography, and technetium 99m (99mTc) myocardial perfusion imaging (MPI) in normal Asian subjects and PAH patients ( n = 23 for each group). Our results demonstrated that six RHC indexes, four echocardiography indexes and MPI index were significantly altered in PAH patients and correlated with the levels of mean pulmonary arterial pressure. Importantly, we evaluated the diagnostic performance of MPI and found that MPI has a strong diagnostic accuracy in PAH patients. The findings from this study will be of interest to clinical investigators who make diagnosis and therapeutic strategies for PAH patients.

scholarly journals Acute Deterioration of Pulmonary Arterial Hypertension (PAH) in a Patient with Neurofibromatosis Type 1 (NF1)

2019 ◽  
Vol 2019 ◽  
pp. 1-4
Author(s):  
Seiya Tanaka ◽  
Fuko Kawahara ◽  
Taro Miyamoto ◽  
Satoshi Tsurusaki ◽  
Yoshihito Sanuki ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Pressure ◽  
Arterial Hypertension ◽  
Neurofibromatosis Type 1 ◽  
Pulmonary Arterial Pressure ◽  
Neurofibromatosis Type ◽  
Mean Pulmonary Arterial Pressure ◽  
Pulmonary Arterial

A 56-year-old woman was diagnosed as having chronic obstructive pulmonary disease with heavy smoking. Mild pulmonary hypertension (mean pulmonary arterial pressure: 31 mmHg) was detected at the first visit. She was diagnosed with pulmonary hypertension due to pulmonary disease and medicated only with bronchodilators. Simultaneous, multiple freckling in the trunk of her body and café au lait macules in her back with some cutaneous neurofibromas were also detected. A plastic surgeon removed one of the neurofibromas and pathologically diagnosed it as neurofibromatosis type 1 (NF1). We finally rediagnosed her with pulmonary hypertension with unclear and/or multifactorial factors when she deteriorated 1 year after being treated only with bronchodilators. We then administrated upfront combination therapy with macitentan and tadalafil. Mean pulmonary arterial pressure rapidly improved. Learning Objective. Pulmonary arterial hypertension (PAH) in neurofibromatosis type 1 (NF1) can occur due to lung disease or due to certain involvement of pulmonary arteries, or a combination of both. Increased awareness of PAH in NF1 is very important for patients survival. The current therapeutic strategy is almost identical to that of idiopathic PAH; however, there is no clinical evidence. Insights gained from clinical experiences should help identify promising novel therapeutic approaches in NF1-PAH.

scholarly journals New potential diagnostic biomarkers for pulmonary hypertension

2015 ◽  
Vol 46 (5) ◽  
pp. 1390-1396 ◽  
Author(s):  
Svenja L. Tiede ◽  
Henning Gall ◽  
Oliver Dörr ◽  
Marina dos Santos Guilherme ◽  
Christian Troidl ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Growth Factor ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Pressure ◽  
Arterial Hypertension ◽  
Lung Disease ◽  
Pulmonary Arterial Pressure ◽  
Diagnostic Biomarkers ◽  
Mean Pulmonary Arterial Pressure ◽  
Pulmonary Arterial

This study aimed to determine whether the vascular endothelial growth factor (VEGF) family members soluble VEGF receptor 1 (also called soluble fms-like tyrosine kinase 1 (sFlt-1)) and placental growth factor (PlGF) could be used as biomarkers for pulmonary hypertension (PH).Consecutive patients undergoing right heart catheterisation were enrolled (those with mean pulmonary arterial pressure ≥25 mmHg were classed as having PH; those with mean pulmonary arterial pressure <25 mmHg acted as non-PH controls). Plasma from the time of PH diagnosis was analysed for PlGF and sFlt-1 using enzyme immunoassays.In total, 247 patients with PH were enrolled: 62 with idiopathic pulmonary arterial hypertension (IPAH), 14 with associated pulmonary arterial hypertension (APAH), 21 with collagen vascular disease (CVD), 26 with pulmonary venous hypertension, 67 with lung disease-associated PH and 57 with chronic thromboembolic PH. The non-PH control group consisted of 40 patients. sFlt-1 plasma levels were significantly higher in patients with IPAH, APAH, CVD and lung disease-associated PH versus controls; PlGF levels were significantly higher in all PH groups versus controls. The combination of sFlt-1 and PlGF resulted in a sensitivity of 83.7% with specificity of 100% for pulmonary arterial hypertension. There was no association between sFlt-1 or PlGF and haemodynamic parameters, 6-min walking distance or survival.In summary, PlGF and sFlt-1 are promising diagnostic biomarkers for PH.

scholarly journals Effects of oxygen therapy in a pediatric normoxemic patient with pulmonary arterial hypertension and congenital heart disease

2019 ◽  
Vol 9 (2) ◽  
pp. 1177_2045894019 ◽  
Author(s):  
Simon Green ◽  
Deidre Stuart
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Oxygen Therapy ◽  
Septal Defect ◽  
Right Heart Catheterization ◽  
Heart Catheterization ◽  
Mean Pulmonary Arterial Pressure ◽  
Rapid Fall ◽  
Pulmonary Arterial ◽  
Observation Period

To shed light on the efficacy of oxygen therapy in pulmonary arterial hypertension (PAH) when hypoxemia is not present, we report seven years of observational data for a female patient recorded between February 2012 and February 2019 when she was aged 7.8–14.8 years. The patient was born with an atrial septal defect (closed spontaneously at 2.3 years) and ventricular septal defect (surgically repaired at 8.3 years) and then diagnosed with PAH at 8.9 years . The patient was prescribed bosentan soon after diagnosis and for the next 4.8 years, during which a first phase of oxygen therapy (nocturnal) was trialed for 2.8 years. Mean pulmonary arterial pressure (mPAP) and systolic PAP (sPAP) remained stable and at mild levels when oxygen was administered, but then increased progressively to severe levels over two years without oxygen. This coincided with worsening right ventricular pathology during the later part of this period without oxygen. Re-initiation of more intensive oxygen therapy while the patient was still on bosentan and before pharmocotherapy was changed coincided with a large and rapid fall in sPAP, confirmed by right heart catheterization measurements of mPAP. During this entire observation period, the patient remained normoxemic. These observations challenge the notion that oxygen therapy should be restricted to patients with hypoxemia and strengthen calls for further study of oxygen therapy in PAH.

Continuous real-time monitoring of pulmonary arterial pressure using a tip deflecting microcatheter in an infant with pulmonary arterial hypertension

2020 ◽  
Vol 30 (7) ◽  
pp. 1032-1034
Author(s):  
Atsushi Tanaka ◽  
Yuichiro Sugitani ◽  
Jun Muneuchi
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Pressure ◽  
Arterial Hypertension ◽  
Real Time ◽  
Pulmonary Arterial Pressure ◽  
Real Time Monitoring ◽  
Mean Pulmonary Arterial Pressure ◽  
Pulmonary Arterial ◽  
Anomalous Pulmonary Venous Connection ◽  
Severe Pulmonary Arterial Hypertension

AbstractWe present the case of a 3-month-old boy with pulmonary arterial hypertension after corrective repair of total anomalous pulmonary venous connection. The patient developed severe pulmonary arterial hypertension with a high mean pulmonary arterial pressure of 45 mmHg. We performed continuous monitoring of pulmonary arterial pressure using a tip deflecting microcatheter in the intensive care unit. We successfully managed this patient based on real-time pulmonary arterial pressure measurements. Continuous real-time monitoring of pulmonary arterial pressure using this microcatheter enables individualized targeted therapy for infants with pulmonary arterial hypertension.

scholarly journals Hemodynamic trajectories and outcomes in patients with pulmonary arterial hypertension

2020 ◽  
Vol 10 (4) ◽  
pp. 204589402094134
Author(s):  
Myriam Amsallem ◽  
Shadi P. Bagherzadeh ◽  
David Boulate ◽  
Andrew J. Sweatt ◽  
Kristina T. Kudelko ◽  
...  
Keyword(s):  
New York ◽  
Mean Arterial Pressure ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Pressure ◽  
Arterial Hypertension ◽  
Pulmonary Arterial Pressure ◽  
Heart Association ◽  
Mean Pulmonary Arterial Pressure ◽  
Pulmonary Arterial

The relative pulmonary to systemic pressure ratio (mean pulmonary arterial pressure/mean arterial pressure) has been proven to be valuable in cardiac surgery. Little is known on the prognostic value of baseline and trajectory of mean pulmonary arterial pressure/mean arterial pressure in pulmonary arterial hypertension. Patients with confirmed idiopathic, familial, drug and toxins, or connective tissue disease-related pulmonary arterial hypertension and at least one complete right heart catheterization were included and prospectively followed-up for 5.9 ± 4.03 years. Correlates of the primary end point (i.e. death or lung transplant need) during follow-up were determined using Cox regression modeling. Results showed that among the 308 patients included, 187 had at least one follow-up catheterization (median time between catheterizations: 2.16 (1.16–3.19) years). In the total cohort (mean age 47.3 ± 14.9 years, 82.8% of female and 58.1% in New York Heart Association class 3 or 4), mean pulmonary arterial pressure/mean arterial pressure (1.38 (1.07–1.77)) was associated with outcome ( p = 0.01). Mean pulmonary arterial pressure/mean arterial pressure was incremental to a basic model (including right atrial pressure, systolic blood pressure, New York Heart Association class 3 or 4, and connective tissue disease) for outcome prediction, while mean pulmonary arterial pressure was not. In the 187 patients with a follow-up catheterization, both delta mean pulmonary arterial pressure and delta mean pulmonary arterial pressure/mean arterial pressure were associated with outcome (1.32 (1.11–1.58) and 1.31 (1.1–1.57) respectively, p < 0.01). Mean pulmonary arterial pressure and mean pulmonary arterial pressure/mean arterial pressure were both incremental to the basic model, while worsening in mean pulmonary arterial pressure or mean pulmonary arterial pressure/mean arterial pressure did not reach significance. In conclusion, mean pulmonary arterial pressure/mean arterial pressure at baseline prognosticates long-term outcome with a significant, albeit modest, incremental value to basic variables.

scholarly journals SAT0240 THE PROGNOSIS OF TWO DISTINCT CLINICAL PHENOTYPES OF SLE-PAH

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1063.2-1063
Author(s):  
J. Wang ◽  
Y. Feng ◽  
Y. Lei ◽  
X. Zhang
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Lupus Erythematosus ◽  
Right Heart Catheterization ◽  
Heart Catheterization ◽  
Systemic Lupus ◽  
Clinical Phenotypes ◽  
Weighted Score ◽  
Pulmonary Arterial

Background:Based on the characteristics of systemic lupus erythematosus-associated pulmonary arterial hypertension (SLE-PAH), Sunet alhas put forward a scoring system to distinguish two clinical phenotypes as vasculitic and vasculopathic subtypes[1]. A weighted score ≥2 suggested a vasculitic subtype by combining two factors: The time interval between SLE and PAH diagnosis <2 years and ≥2 years were 1 and 0 point; SLE Disease Activity Index (SLEDAI) >9, 5-9 and <5 were 2, 1, 0 point, respectively. While the vasculitic subtype seemed to have poorer prognosis in Sun’s research, other study has shown controversial result[2].Objectives:To find out the prognosis of two distinct clinical phenotypes of SLE-PAH.Methods:Between 2008 and 2019, a SLE-PAH cohort confirmed by right heart catheterization (RHC) from Guangdong Provincial People’s Hospital was included. Other groups of pulmonary hypertension were excluded. Based on the scoring system, patients were divided into vasculitic (weighted score≥2) and vasculopathic subtypes (weighted score<2). The endpoint was PAH-related mortality. Survival status were confirmed by clinic follow-up data or phone call.Results:A total of 53 SLE-PAH patients were enrolled. The cases of vasculitic and vasculopathic subtype were 14 and 39, respectively. Ten endpoint events occurred. Eight attributed to PAH and the cause could not be traced in two which were still included in study. The pooled 1-, 3-, 5-year survival rates were 85.7%, 78.6%, 65.5% in vasculitic subtype, and 93.9%, 87.5%, 87.5% in vasculopathic subtype, respectively. Kaplan-Meier analysis showed vasculitic subtype tended to have a poorer prognosis than vasculopathic subtype (p=0.16, HR 2.4, 95%CI 0.5-13.8, figure 1).Figure 1.Survival curves for patients with systemic lupus erythematosus-pulmonary arterial hypertension (SLE-PAH) in two distinct subtypes. RHC, Right Heart Catheterization.Conclusion:The prognosis of the two phenotypes of SLE-PAH was statistically indifferent while the vasculitic subtype showed a trend of worse prognosis. Further studies are needed.References:[1]F. Sun, Y. Lei, W. Wu, L. Guo, K. Wang, Z. Chen, W. Xu, X. Wang, T. Li, X. Zhang, S. Ye, Two distinct clinical phenotypes of pulmonary arterial hypertension secondary to systemic lupus erythematosus, Ann Rheum Dis 78(1) (2019) 148-150.[2]J. Qian, M. Li, J. Zhao, Q. Wang, Z. Tian, X. Zeng, Inflammation in SLE-PAH: good news or not?, Ann Rheum Dis (2018).0:1–2. doi:10.1136/annrheumdis-2018-214605Disclosure of Interests:None declared

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