Sufficient Decrease in Pulmonary Arterial Pressure Markedly Improves Long-Term Prognosis of Patients With Pulmonary Arterial Hypertension

2012 ◽  
Vol 18 (8) ◽  
pp. S84 ◽  
Author(s):  
Masaru Hatano ◽  
Atsushi Yao ◽  
Hisataka Maki ◽  
Toshiro Inaba ◽  
Koichiro Kinugawa
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Pressure ◽  
Arterial Hypertension ◽  
Pulmonary Arterial Pressure ◽  
Pulmonary Arterial ◽  
Long Term Prognosis ◽  
Sufficient Decrease

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.

Atrial septal defect closure with an Amplatzer septal occluder fenestrated with a coronary stent in a child with pulmonary arterial hypertension

2012 ◽  
Vol 23 (5) ◽  
pp. 692-696 ◽  
Author(s):  
Gregory J. Skinner ◽  
Robert M. Tulloh ◽  
Andrew J. Tometzki ◽  
Ingram Schulze-Neick ◽  
Gareth J. Morgan
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Pressure ◽  
Arterial Hypertension ◽  
Atrial Septal Defect ◽  
Septal Defect ◽  
Pulmonary Arterial Pressure ◽  
Severe Disease ◽  
Idiopathic Pulmonary Arterial Hypertension ◽  
Stent Insertion ◽  
Pulmonary Arterial

AbstractThe presence of an atrial septal defect in pulmonary hypertension has benefits and detractions. Even in idiopathic pulmonary arterial hypertension, a significant left-to-right shunt at atrial level may increase the pulmonary arterial pressure and exacerbate the disease. However, it is well recognised that the presence of an atrial communication may be protective in subgroups with severe disease, allowing maintenance of cardiac output during times of increased pulmonary resistance. In the present paper, we present the case of a young boy with significant idiopathic pulmonary arterial hypertension and an atrial septal defect. We report our technique of septal occlusion using a device to decrease left-to-right shunting with concomitant stent insertion in that device to maintain the potential for right-to-left shunting during times of high pulmonary arterial pressure.

Pulmonary Arterial Hypertension in Systemic Lupus Erythematosus: Prevalence and Predictors

2015 ◽  
Vol 43 (2) ◽  
pp. 323-329 ◽  
Author(s):  
Gregorio Miguel Pérez-Peñate ◽  
Iñigo Rúa-Figueroa ◽  
Gabriel Juliá-Serdá ◽  
Fernándo León-Marrero ◽  
Antonio García-Quintana ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Pressure ◽  
Arterial Hypertension ◽  
Diastolic Dysfunction ◽  
Lupus Erythematosus ◽  
Pulmonary Arterial Pressure ◽  
Systemic Lupus ◽  
Pulmonary Arterial

Objective.Pulmonary arterial hypertension (PAH) prevalence has been reported to be between 0.5% and 17% in systemic lupus erythematosus (SLE). This study assessed PAH prevalence and predictors in an SLE cohort.Methods.The Borg dyspnea scale, DLCO, N-terminal pro–brain natriuretic peptide (NT-proBNP), and Doppler echocardiographic (DE) were performed. An echocardiographic Doppler exercise test was conducted in selected patients. When DE systolic pulmonary arterial pressure was ≥ 45 mmHg or increased during exercise > 20 mmHg, a right heart catheterization was performed. Hemodynamic during exercise was measured if rest mean pulmonary arterial pressure was < 25 mmHg.Results.Of the 203 patients with SLE, 152 were included. The mean age was 44.9 ± 12.3 years, and 94% were women. Three patients had known PAH. The algorithm diagnosed 1 patient with chronic thromboembolic pulmonary hypertension and 5 with exercise-induced pulmonary artery pressure increase (4 with occult left diastolic dysfunction). These patients had significantly more dyspnea, higher NT-proBNP, and lower DLCO.Conclusion.These data confirm the low prevalence of PAH in SLE. In our cohort, occult left ventricular diastolic dysfunction was a frequent diagnosis of unexplained dyspnea. Dyspnea, DLCO, and NT-proBNP could be predictors of pulmonary hypertension in patients with SLE.

scholarly journals Novel Treatment Pathways in Pulmonary Arterial Hypertension

2021 ◽  
Vol 17 (2) ◽  
pp. 106-114
Author(s):  
Kanza N Qaiser ◽  
Adriano R Tonelli
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Disease Process ◽  
Ongoing Research ◽  
Treatment Pathways ◽  
Pulmonary Vascular Remodeling ◽  
Pulmonary Arterial ◽  
Long Term Prognosis ◽  
New Treatments

Pulmonary arterial hypertension (PAH) is a severe and progressive vascular disease characterized by pulmonary vascular remodeling, proliferation, and inflammation. Despite the availability of effective treatments, PAH may culminate in right ventricular failure and death. Currently approved medications act through three well-characterized pathways: the nitric oxide, endothelin, and prostacyclin pathways. Ongoing research efforts continue to expand our understanding of the molecular pathogenesis of this complex and multifactorial disease. Based on recent discoveries in the pathobiology of PAH, several new treatments are being developed and tested with the goal of modifying the disease process and ultimately improving the long-term prognosis.

scholarly journals Role of Endothelin-1 Receptor Antagonist in Reducing Perioperative Pulmonary Arterial Hypertension after Surgical Correction of Ventricular Septal Defect

2016 ◽  
Vol 9 (1) ◽  
pp. 55-59
Author(s):  
Rampada Sarker ◽  
ASMS Islam ◽  
SC Mandal ◽  
Kazi Abul Hasan ◽  
Manoz Kumar Sarker ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Ventricular Septal Defect ◽  
Arterial Pressure ◽  
Arterial Hypertension ◽  
Septal Defect ◽  
Pulmonary Arterial Pressure ◽  
Surgical Correction ◽  
Endothelin 1 ◽  
Pulmonary Arterial

Background: This study was designed to determine the role of oral ambrisentan, an endothelin-1 receptor, in reducing perioperative pulmonary arterial hypertension during surgical correction of ventricular septal defect.Methods: This study was carried out among 54 patients of ventricular septal defect with pulmonary arterial hypertension undergoing surgical correction. The patients were divided into two groups; study group received oral ambrisentan, an endothelin-1 receptor antagonist peri-operatively, starting one week before surgery. Pulmonary arterial pressure was measured by echocardiography, cardiac catheterization and directly from pulmonary artery during surgical procedure.Results: Pulmonary arterial pressure was reduced significantly in the group in which oral amrisentan was given perioperatively. There was further reduction of pulmonary arterial pressure at discharge from hospital and at one month follow-up.Conclusion: Oral ambrisentan can reduce pulmonary arterial pressure perioperatively in patients underwent surgical correction of ventricular septal defect.Cardiovasc. j. 2016; 9(1): 55-59

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.

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