scholarly journals Clinical study of patients with primary pulmonary hypertension (PPH)

2018 ◽  
Vol 60 (2) ◽  
pp. 80-84
Author(s):  
Alaa A. Abbood AL-Kinani
Keyword(s):  
Pulmonary Hypertension ◽  
Ventricular Hypertrophy ◽  
Primary Pulmonary Hypertension ◽  
Term Therapy ◽  
Heart Catheterization ◽  
Mean Pulmonary Arterial Pressure ◽  
Pulmonary Arterial ◽  
The Mean ◽  
Long Term Therapy

Background: Pulmonary hypertension (PH) is a hemodynamic and pathophysiological conditiondefined as an increase in mean pulmonary arterial pressure (PAP) ≥25 mmHg at rest as assessed byright heart catheterization (RHC). Although there is some underestimation and overestimation of PAPbetween transthoracic Doppler echo (DE) and RHC, Doppler echo remains an indispensable screeningtool for the assessment of PH.Objective: clinical evaluation of patients with primary pulmonary hypertension (PPH) and assessvasoreactivity testing to identify patients who may benefit from long term therapy with calcium channelblockers (CCBs).Patients and methods: This prospective study was performed in the cardiac catheterization division inAl-Zahraa teaching hospital in Al-Kut. We studied the prevalence of certain variables among forty twopatients with PPH from "March 2014 to Nov 2016" including the clinical triggers, electrocardiographic(ECG) changes, Echocardiographic variables , RHC and vasoreactivity test with intravenous adenosineto identify acute positive responders and long term responders to CCB.Results: A total of forty two patients, female to male ratio were 2.8:1 with a mean age of 38±10(years).Dyspnea is a common clinical trigger (85%). Abnormal ECG was found in (90.5%) of patients, themajority had right ventricular hypertrophy (RVH) (76.2%). Echocardiographically all patients hadRVH. There was some differences in mean PAP (36±4.9mmhg) derived by DE from that obtained byRHC (47±4.78mmhg). RHC reveal that 6 patients (15.78%) were acute positive responders tointravenous adenosine and about 4 patients (66%) were long term responders to CCB during 3monthsfollow up echocardiography.Conclusions: There is some discrepancy in the mean PAP between Doppler echo and RHC within ±10mm Hg for pulmonary artery pressure estimates. 15.7% of patients at RHC were acute positiveresponder to intravenous adenosine and half of them were long term responder to CCB.

scholarly journals Clinical study of patients with primary pulmonary hypertension (PPH)

2018 ◽  
Vol 60 (2) ◽  
pp. 80-84
Author(s):  
Alaa A. Abbood AL-Kinani
Keyword(s):  
Pulmonary Hypertension ◽  
Ventricular Hypertrophy ◽  
Primary Pulmonary Hypertension ◽  
Term Therapy ◽  
Heart Catheterization ◽  
Mean Pulmonary Arterial Pressure ◽  
Pulmonary Arterial ◽  
The Mean ◽  
Long Term Therapy

Background: Pulmonary hypertension (PH) is a hemodynamic and pathophysiological conditiondefined as an increase in mean pulmonary arterial pressure (PAP) ≥25 mmHg at rest as assessed byright heart catheterization (RHC). Although there is some underestimation and overestimation of PAPbetween transthoracic Doppler echo (DE) and RHC, Doppler echo remains an indispensable screeningtool for the assessment of PH.Objective: clinical evaluation of patients with primary pulmonary hypertension (PPH) and assessvasoreactivity testing to identify patients who may benefit from long term therapy with calcium channelblockers (CCBs).Patients and methods: This prospective study was performed in the cardiac catheterization division inAl-Zahraa teaching hospital in Al-Kut. We studied the prevalence of certain variables among forty twopatients with PPH from "March 2014 to Nov 2016" including the clinical triggers, electrocardiographic(ECG) changes, Echocardiographic variables , RHC and vasoreactivity test with intravenous adenosineto identify acute positive responders and long term responders to CCB.Results: A total of forty two patients, female to male ratio were 2.8:1 with a mean age of 38±10(years).Dyspnea is a common clinical trigger (85%). Abnormal ECG was found in (90.5%) of patients, themajority had right ventricular hypertrophy (RVH) (76.2%). Echocardiographically all patients hadRVH. There was some differences in mean PAP (36±4.9mmhg) derived by DE from that obtained byRHC (47±4.78mmhg). RHC reveal that 6 patients (15.78%) were acute positive responders tointravenous adenosine and about 4 patients (66%) were long term responders to CCB during 3monthsfollow up echocardiography.Conclusions: There is some discrepancy in the mean PAP between Doppler echo and RHC within ±10mm Hg for pulmonary artery pressure estimates. 15.7% of patients at RHC were acute positiveresponder to intravenous adenosine and half of them were long term responder to CCB.

Diagnosis and clinical investigation of patients presenting with pulmonary hypertension

ESC CardioMed ◽  
2018 ◽  
pp. 2507-2511 ◽  
Author(s):  
Daniela Calderaro ◽  
Luis Felipe Prada ◽  
Rogério Souza
Keyword(s):  
Pulmonary Hypertension ◽  
Clinical Investigation ◽  
Right Heart Catheterization ◽  
Heart Catheterization ◽  
Right Heart ◽  
Reference Centre ◽  
Mean Pulmonary Arterial Pressure ◽  
Invasive Test ◽  
Pulmonary Arterial

The diagnosis of pulmonary hypertension (PH) relies on the haemodynamic criterion of mean pulmonary arterial pressure greater than or equal to 25 mmHg, assessed by right heart catheterization. The scope of this chapter is to discuss the key elements of clinical assessment of PH patients and the decision process to indicate right heart catheterization. Investigation must get through all the possible causes of PH according to their probability and frequency in the population. Echocardiography is the most important non-invasive test as an indicator for further diagnostic evaluation. Patients who are eligible for right heart catheterization should always be referred to PH centres, where technical skills and standardized procedures will enable maximal reliability of haemodynamic measurement. In the reference centre, a multidisciplinary team will discuss clinical and haemodynamic data, to propose the best therapeutic and follow-up schedule.

scholarly journals Impact of the updated hemodynamic definitions on diagnosis rates of pulmonary hypertension

2020 ◽  
Vol 10 (3) ◽  
pp. 204589402093129
Author(s):  
Seda Tanyeri ◽  
Ozgur Y. Akbal ◽  
Berhan Keskin ◽  
Aykun Hakgor ◽  
Ali Karagoz ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Arterial Pressure ◽  
European Society ◽  
Pulmonary Arterial Pressure ◽  
Heart Catheterization ◽  
Mean Pulmonary Arterial Pressure ◽  
Pressure Threshold ◽  
Normal Mean ◽  
Pulmonary Arterial ◽  
European Society Of Cardiology

We evaluated whether updated pulmonary hypertension definitive criteria proposed in sixth World Symposium on Pulmonary Hypertension had an impact on diagnosis of overall pulmonary hypertension and pre-capillary and combined pre- and post-capillary phenotypes as compared to those in European Society of Cardiology/European Respiratory Society 2015 pulmonary hypertension Guidelines. Study group comprised the retrospectively evaluated 1300 patients (age 53.1 ± 18.8 years, female 807, 62.1%) who underwent right heart catheterization with different indications between 2006 and 2018. Mean pulmonary arterial pressure ≥25 mmHg (European Society of Cardiology) and PAMP (mean pulmonary arterial pressure) >20 mmHg (World Symposium on Pulmonary Hypertension) right heart catheterization definitions criteria were used, respectively. For pre-capillary pulmonary hypertension, pulmonary artery wedge pressure ≤15 mmHg and pulmonary vascular resistance ≥3 Wood units criteria were included in the both definitions. Normal mean pulmonary arterial pressure (<21 mmHg), borderline mean pulmonary arterial pressure elevation (21–24 mmHg), and overt pulmonary hypertension (≥25 mmHg) were documented in 21.1, 9.8, and 69.1% of the patients, respectively. The pre-capillary and combined pre- and post-capillary pulmonary hypertension were noted in 2.9 and 1.1%, 8.7 and 2.5%, and 34.6 and 36.6% of the patients with normal mean pulmonary arterial pressure, borderline, and overt pulmonary hypertension subgroups, respectively. The World Symposium on Pulmonary Hypertension versus European Society of Cardiology/European Respiratory Society definitions resulted in a net 9.8% increase in the diagnosis of overall pulmonary hypertension whereas increases in the pre-capillary pulmonary hypertension and combined pre- and post-capillary pulmonary hypertension diagnosis were only 0.8 and 0.3%, respectively. The re-definition of mean pulmonary arterial pressure threshold seems to increase the frequency of the overall pulmonary hypertension diagnosis. However, this increase was mainly originated from those in post-capillary pulmonary hypertension subgroup whereas its impact on pre-capillary and combined pre- and post-capillary pulmonary hypertension was negligible. Moreover, criteria of pre-capillary pulmonary vascular disease and combined pre- and post-capillary phenotypes were still detectable even in the presence of normal mean pulmonary arterial pressure. The obligatory criteria of pulmonary vascular resistance ≥3 Wood units seems to keep specificity for discrimination between pre-capillary versus post-C pulmonary hypertension after lowering the definitive mean pulmonary arterial pressure threshold to 20 mmHg.

Elevated Estimated Pulmonary Arterial Pressures in Patients with Chuvash Polycythemia.

Blood ◽  
2004 ◽  
Vol 104 (11) ◽  
pp. 1634-1634
Author(s):  
Victor R. Gordeuk ◽  
Adelina I. Sergueeva ◽  
Galina Y. Miasnikova ◽  
Lydia A. Polyakova ◽  
Daniel J. Okhotin ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Artery ◽  
Tricuspid Regurgitation ◽  
Chronic Hypoxia ◽  
Primary Pulmonary Hypertension ◽  
Systolic Pressure ◽  
Pulmonary Artery Systolic Pressure ◽  
Normal Range ◽  
Pulmonary Arterial ◽  
The Mean

Abstract Chuvash polycythemia is characterized by a homozygous 598C>T mutation in VHL and up regulation of HIF-1α during normoxia. Disorders of chronic hypoxia may be complicated by the development of pulmonary hypertension. Because of the up regulation of the hypoxic response in Chuvash polycythemia, we postulated that there may be a tendency to increased pulmonary artery pressures in this condition as well. To test this hypothesis, we analyzed results for Doppler echocardiography in 15 patients with Chuvash polycythemia and 15 Chuvash individuals without polycythemia. The tricuspid regurgitation velocity (TRV) allows estimation of pulmonary artery systolic pressure. A TRV of 2.5 m/sec or higher corresponds to a pulmonary artery systolic pressure of at least 35 mm Hg (normal up to 32 mm Hg), while a TRV of 3.0 m/sec or higher to a pressure of at least 46 mm Hg. The results are summarized in the Table. Pulmonary artery pressures as estimated by tricuspid regurgitation velocity (TRV) in Chuvash subjects with and without polycythemia Chuvash polycythemia (n = 15) Controls (n = 15) P Age in years; mean (SD) 35 (17) 35 (17) 1.0 Female sex in no. (%) 8 (53%) 8 (53%) 1.0 Hemoglobin in g/dL; mean (SD) 16.7 (2.3) 13.3 (1.2) <0.001 TRV in m/sec; mean (SD) 2.2 (0.6) 1.4 (0.6) 0.001 TRV > 2.4 m/sec in no. (%) 4 (27%) 0 (0%) 0.1 Most of the patients with Chuvash polycythemia were receiving phlebotomy therapy and therefore many had hemoglobin concentrations in the upper normal range. Four of the patients with Chuvash polycythemia and none of the others had TRV ≥ 2.5 m/sec (range of 2.5 to 3.0), and mean TRVs were significantly higher in the patients with Chuvash polycythemia. Interestingly, the mean ± SD TRV in these 15 patients with Chuvash polycythemia was identical to the mean ± SD TRV that was recently reported in 195 American patients with sickle cell disease (Gladwin et al, NEJM2004;350:886), another hematological condition with a tendency to pulmonary hypertension. While the pulmonary arterial pressures detected so far in Chuvash polycythemia patients are lower than those in patients with primary pulmonary hypertension, our results suggest that pulmonary hypertension may be an unrecognized complication of Chuvash polycythemia.

Safety and effectiveness of long-term therapy with the oral iron chelator deferiprone

Blood ◽  
2003 ◽  
Vol 102 (5) ◽  
pp. 1583-1587 ◽  
Author(s):  
Alan R. Cohen ◽  
Renzo Galanello ◽  
Antonio Piga ◽  
Vincenzo De Sanctis ◽  
Fernando Tricta
Keyword(s):  
Early Death ◽  
Thalassemia Major ◽  
Iron Chelator ◽  
Term Therapy ◽  
First Year ◽  
Intention To Treat ◽  
The Mean ◽  
Multicenter Prospective Study ◽  
Long Term Therapy

AbstractThe identification of a safe, orally active iron chelator is critically important for the prevention of morbidity and early death in patients receiving regular red cell transfusions. Based on our findings in a 1-year multicenter, prospective study of the safety and efficacy of deferiprone in patients with thalassemia major, we have extended the treatment period to 4 years. The mean dose of the chelator was 73 mg/kg per day during 531 patient-years. The rates of agranulocytosis (absolute neutrophil count [ANC] &lt; 500 × 109/L) and milder forms of neutropenia (ANC, 500-1500 × 109/L) were 0.2 and 2.8 per 100 patient-years, respectively. Neutropenia occurred significantly more commonly in patients with intact spleens. Gastrointestinal and joint symptoms decreased significantly after the first year of therapy, and led to discontinuation of deferiprone in only one patient in years 2 to 4. The mean alanine aminotransferase (ALT) value of 71 U/L after 4 years of therapy was significantly higher than the baseline value of 61 U/L. Trend analysis showed no increase in the ALT levels or the percentage of patients with ALT levels greater than twice the upper limit of the reference range. Ferritin levels did not change significantly from the values at the time of change from deferoxamine to deferiprone in either the intention-to-treat analysis or in the 84 patients who completed 4 years of therapy. Because of concerns regarding the effectiveness of the studied dose of deferiprone, 47 patients discontinued therapy, whereas 15 patients interrupted therapy because of concerns regarding low iron levels. The results of this study help to define the safety and effectiveness of long-term therapy with deferiprone.

scholarly journals High Tissue Factor Microparticle Level in Major Thalassemic Patients with Pulmonary Hypertension

2019 ◽  
Vol 7 (2) ◽  
pp. 88-95
Author(s):  
Dimmy Prasetya ◽  
Pandji Irani Fianza ◽  
Erwan Martanto ◽  
Teddy Arnold Sihite
Keyword(s):  
Pulmonary Hypertension ◽  
Tissue Factor ◽  
Pulmonary Arterial Pressure ◽  
Secondary Data ◽  
Primary Data ◽  
Mean Pulmonary Arterial Pressure ◽  
Significant Difference ◽  
Pulmonary Arterial ◽  
The Mean ◽  
Clinical Characteristic

Objective: To analyze the correlation between tissue factor microparticles (TF-MP) levels and pulmonary hypertension (PH) in adult thalassemic patients. Methods: This study was conducted from September to October 2018, using secondary and primary data. The secondary data consisted of the PH parameter, which was retrieved from a 2017 previous study entitled ‘Clinical Characteristic and Complication due to Iron Overload in Thalassaemic Patients‘in 2017 while the primary data were the TF-MP, which were obtained from the analysis of frozen serum of the same population using ELISA method. The mean pulmonary arterial pressure (mPAP) values were obtained from echocardiography results and PH was defined as mPAP >25 mmHg. Results: Seven (16.7%) major thalassemic patients experienced PH. The median values of TF-MP levels were higher among major thalassemic patients with PH when compared to the non-PH patients (1569 vs 11.5 pg/dL; p=0.023). No significant difference was observed in the median TF-MP levels between subjects with splenectomy and subjects without splenectomy (11.6 vs 12.3 pg/dL; p=0.44). There was also no difference in mPAP values between subjects with splenectomy and subjects without splenectomy (18.0 vs 17.0 mmHg; p=0.663). When the median TF-MP levels among major thalassemic patients were analyzed in terms of correlation with transfusion level, no statistically significant difference was seen between subjects who received sufficient transfusions (≥180 mL/kgbb/year) and those who received insufficient transfusions (<180 mL/kgbb/year) (r= 0.138; p=0.390). Conclusions: There is a positive correlation between the TF-MP levels and PH in adult major thalassemic subjects.

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