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.

scholarly journals The Effects of Dexmedetomidine Prescription in Paediatric Patients With Pulmonary Hypertension Under Congenital Heart Surgery

2020 ◽  
Author(s):  
Bahram Ghasemzadeh ◽  
Bahador Azizi ◽  
Simin Azemati ◽  
Mostafa Bagherinasab
Keyword(s):  
Blood Pressure ◽  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Pulmonary Artery ◽  
Arterial Hypertension ◽  
Systolic Blood Pressure ◽  
Systolic Pressure ◽  
Pulmonary Artery Systolic Pressure ◽  
Anesthetic Care ◽  
Pulmonary Arterial

Anesthetized patient management for pediatric patients with pulmonary arterial hypertension (PAH) is a major challenge. The aim of this study was to evaluate the ability of dexmedetomidine to reduce pulmonary arterial hypertension in patients with pulmonary arterial hypertension undergoing cardiac surgery. Sixty-six patients with pulmonary arterial hypertension underwent the study. Patients were randomly divided into two groups: group D received a dexmedetomidine injection in a dose of 1 μg/kg in the first hour and then decreased to 0.5 μg/kg/hr, injection continued after surgery until extubation in the post-anesthetic care unit (PACU). Group C received normal saline 0.9% in a similar volume. Pulmonary artery systolic pressure (PASP) and systemic systolic blood pressure (SSBP) were recorded during and after the surgery in the postanesthetic care unit. Needing vasodilators, sedatives, extubation time, and the length of ICU stay were recorded for all patients. Patients in the dexmedetomidine group showed a significant reduction in Pulmonary artery systolic pressure and Pulmonary artery systolic pressure/systemic systolic blood pressure rates during surgery and during the first 24 hours in the post-anesthetic care unit (P<0.001). The dexmedetomidine group, in comparison with the control group, needed a significantly lower dose of a vasodilator (P<0.001) and a lower dose of sedation (P<0.001). It is concluded that the use of dexmedetomidine during the surgery in children with pulmonary hypertension reduces pulmonary artery systolic pressure during and after the surgery.

scholarly journals The effects of ductal size on the severity of pulmonary hypertension in children with patent ductus arteriosus (PDA): a multi-center study

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Josephat M. Chinawa ◽  
Bartholomew F. Chukwu ◽  
Awoere T. Chinawa ◽  
Chika O. Duru
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Artery ◽  
Patent Ductus Arteriosus ◽  
Ductus Arteriosus ◽  
Systolic Pressure ◽  
Pulmonary Artery Systolic Pressure ◽  
Severe Pulmonary Hypertension ◽  
The Mean ◽  
Hypertension In Children ◽  
Patent Ductus

Abstract Introduction Patent ductus arteriosus (PDA) is a common acyanotic heart disease that presents with variable symptoms. Objectives This study is therefore aimed at determining the relationship between gender, age, and size of PDA and pulmonary hypertension. This study also seeks to determine the prevalence of elevated pulmonary artery systolic pressure in children with PDA. Patients and methods A descriptive study of children with patent ductus arteriosus was carried out from 2016 to 2020 in three institutions. The data were analysed with the IBM SPSS statistics for windows, version 20 (IBM Corp, Chicago) Result The mean ductal size was 3.78 (2.39) mm, with a minimum of 1.0 mm and a maximum size of 10.0 mm. The mean ductal size for males, 4.02 (2.53) mm was comparable with that of the females, 3.61 (2.28) mm (Student T-test = 0.8, 0.4). The mean pulmonary artery systolic pressure (PASP) of the patients was 43.36 (24.46) mmHg. Also the mean PASP was comparable among the males and the females, 48.37 (26.69) mmHg versus 39.63 (22.16) mmHg (Student T-test = 1.81, p = 0.07). There was no correlation between age and PASP (correlation coefficient = 0.009, p = 0.92). Sixty point two percent (60.2%) (62/103) of children with PDA had pulmonary hypertension. The proportion of males with pulmonary hypertension, 48.39% (30/62) was comparable with that of the females, 51.61% (32/62) (Chi2 = 2.05, p = 0.15) and females are 1.8 times more likely to have pulmonary hypertension as males (odds ratio 1.81, 95% CI 0.8–4.1). There was a positive correlation between ductal size and PASP (Pearson correlation coefficient = 0.26, p value = 0.007). Those with moderate and large sized duct tend to have moderate and severe pulmonary hypertension respectively and this is statistically significant. Chi2 = 17.85, p = 0.007 Conclusion The prevalence of pulmonary hypertension in children with PDA is 60.2%. Moderate and large size duct presents with moderate and severe pulmonary hypertension respectively. Females are 1.8 times more likely to have pulmonary hypertension than the males.

scholarly journals Serum-Free Thyroxine Levels Were Associated with Pulmonary Hypertension and Pulmonary Artery Systolic Pressure in Euthyroid Patients with Coronary Artery Disease

2017 ◽  
Vol 2017 ◽  
pp. 1-8
Author(s):  
Bingjie Wu ◽  
Jingjing Jiang ◽  
Minghui Gui ◽  
Lin Liu ◽  
Qiqige Aleteng ◽  
...  
Keyword(s):  
Coronary Artery Disease ◽  
Pulmonary Hypertension ◽  
Regression Analysis ◽  
Coronary Artery ◽  
Pulmonary Artery ◽  
Systolic Pressure ◽  
Free Thyroxine ◽  
Pulmonary Artery Systolic Pressure ◽  
Serum Free ◽  
Artery Disease

The aim of this study was to evaluate the association between thyroid hormone levels, pulmonary hypertension (PH), and pulmonary artery systolic pressure (PASP) in euthyroid patients with coronary artery disease (CAD). A cross-sectional study was conducted in individuals who underwent coronary angiography and were diagnosed as CAD from March 2013 to November 2013. 811 subjects (185 women and 626 men) were included in this study. PASP was measured by transthoracic Doppler echocardiography. 86 patients were diagnosed as PH and had significantly higher free thyroxine (FT4) levels than those without PH. Multiple logistic regression analysis demonstrated an independent association of FT4 levels with PH after adjustment of gender, age, body mass index, systolic blood pressure, left ventricular ejection fraction, hypertension, and medication use of calcium channel blockers, ACE inhibitors, angiotensin II receptor antagonists, and nitrates. Serum-free triiodothyronine (FT3) and thyroid-stimulating hormone (TSH) were not associated with PH. Furthermore, multivariate linear regression analysis showed that FT4 levels emerged as an independent predictor for PASP, while FT3 and TSH levels were not associated with PASP. Our study demonstrated that, in euthyroid patients with CAD, FT4 was an independent risk factor for PH, and FT4 levels were independently associated with PASP.

Abstract 15388: Persistent Pulmonary Hypertension After Transcatheter Aortic Valve Implantation: Impact on Mortality

Circulation ◽  
2015 ◽  
Vol 132 (suppl_3) ◽  
Author(s):  
Maria Drakopoulou ◽  
Konstantinos Stathogiannis ◽  
Konstantinos Toutouzas ◽  
George Latsios ◽  
Andreas Synetos ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Aortic Stenosis ◽  
Pulmonary Artery ◽  
Severe Aortic Stenosis ◽  
Systolic Pressure ◽  
Right Atrial ◽  
Pulmonary Artery Systolic Pressure ◽  
End Point ◽  
The Right ◽  
The Impact

Objective: Severe aortic stenosis leads to increased pulmonary arterial systolic pressure. A controversy still remains regarding the impact of persistent pulmonary hypertension (PHT) on prognosis of patients undergoing transcatheter aortic valve implantation (TAVI). We sought to investigate the impact of persistent PHT on 2-year all-cause mortality of patients with severe aortic stenosis following TAVI. Methods: Patients with severe and symptomatic aortic stenosis (effective orifice area [EOA]≤1 cm 2 ) who were scheduled for TAVI with a self-expanding valve at our institution were prospectively enrolled. Prospectively collected echocardiographic data before and after TAVI were retrospectively analyzed in all patients. Pulmonary artery systolic pressure was estimated as the sum of the right ventricular to the right atrial gradient during systole and the right atrial pressure. PHT following TAVI was classified as absent if <35 mmHg and persistent if ≥35 mmHg. Primary clinical end-point was 2-year all-cause mortality defined according to the criteria proposed by the Valve Academic Research Consortium-2. Results: Hundred and forty patients (mean age: 82±9 years) were included in the study. The primary clinical end point occurred in 17 patients (12%) during a median follow-up period of 2 years. Mean pulmonary artery systolic pressure was reduced in all patients following TAVI (45±9 versus 41±6 mmHg, p<0.01). Mortality rate was higher in patients with persistent PHT compared to patients with normal pulmonary artery systolic pressure following TAVI (26% versus 14 %, p<0.01). Patients that reached the primary clinical end point had a higher post procedural mean systolic pulmonary pressure (43±9 versus 39±6 mmHg, p=0.02). In multivariate regression analysis, persistence of PHT (OR: 2.51, 95% CI: 1.109-7.224, p=0.01) was an independent predictor of long-term mortality. Conclusions: The persistence of pulmonary hypertension after TAVI is associated with long term mortality. Identifying the population that will clearly benefit from TAVI is still need to be validated by larger trials.

Pulmonary Hypertension as a Risk Factor for Death in Patients with Sickle Cell Anemia

Blood ◽  
2008 ◽  
Vol 112 (11) ◽  
pp. 4819-4819
Author(s):  
Rodolfo D Cancado ◽  
Maria Cristina A Olivato ◽  
Newton Nunes Lima Filho ◽  
Orlando Campos ◽  
Carlos Chiattone
Keyword(s):  
Pulmonary Hypertension ◽  
Sickle Cell Disease ◽  
Pulmonary Artery ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Systolic Pressure ◽  
Pulmonary Artery Systolic Pressure ◽  
Cell Disease ◽  
Risk Of Death ◽  
Hydroxyurea Therapy

Abstract Pulmonary hypertension develops in most forms of hereditary and chronic hemolytic anemia, including sickle cell disease, thalassemia, hereditary spherocytosis, and paroxysmal nocturnal hemoglobinuria, suggesting that there is a clinical syndrome of hemolysis-associated pulmonary hypertension. Retrospective studies from tertiary care referral centers suggest a prevalence of pulmonary hypertension in adults with sickle cell disease ranging from 20 to 40%. Despite the fact the elevations in pulmonary artery pressures are slight, morbidity and mortality are high. In adult sickle cell anemia patients, pulmonary hypertension is emerging as a major risk factor for death. We performed Doppler echocardiographic assessments of pulmonary-artery systolic pressure in 80 consecutive patients (20 men and 60 women; mean [±SD] age, 30 ± 10.8 years) between 1/20/2006 and 1/20/2008. The genotype on the basis of hematologic and hemoglobin characteristics was hemoglobin SS in all patients. Pulmonary hypertension was prospectively defined as a tricuspid regurgitant Jet velocity (TFJV) of at least 2.5 m per second. Patients were followed for a mean of 18 months (6–24 months), and data were censored at the time of death or loss to follow-up. Doppler-defined pulmonary hypertension occurred in 37.5 percent of patients (30/80). Multiple logistic-regression analysis, with the use of the dichotomous variable of a tricuspid regurgitant jet velocity of less than 2.5 m per second or 2.5 m per second or more, identified age, female sex, deferasirox therapy, left ventricular mass index, pulmonary artery systolic pressure, reticulocytes, white-cell count, platelet count, lactate dehydrogenase (a marker of hemolysis), blood urea nitrogen, creatinine, uric acid and self-reported history of cardiovascular complication, billiary stones, retinopathy and acute chest syndrome, as significant independent correlates of pulmonary hypertension. The hemoglobin level, fetal hemoglobin level, hydroxyurea therapy and serum ferritin level were unrelated to pulmonary hypertension. Hazard rate for death according to the TFJV of at least 2.5 m per second, as compared with a velocity of less than 2.5 m per second, was associated with an increased risk of death (0.00 versus 2.54; P=0.998). Mortality rate in 24 months was 6.7% (2/30) for patients with TRJ velocity ≥ 2.5 m/sec versus 0.0% (0/50) for patients without pulmonary hypertension. Pulmonary hypertension, diagnosed by Doppler echocardiography, is common in adults with sickle cell disease. It appears to be a complication of chronic hemolysis, is resistant to hydroxyurea therapy, and confers a high risk of death. Large trials evaluating the effects of treatment for pulmonary hypertension in the sickle cell anemia population are indicated.

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.

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