Peak Tricuspid Regurgitant Jet Velocity Research Articles

Pulmonary hypertension screening in children with sickle cell disease.

Screening for pulmonary hypertension (PHT) is recommended in children with sickle cell disease (SCD). However, best approaches are poorly described. We examined the utility of PHT symptoms, echocardiogram (ECHO), N-terminal-pro hormone brain natriuretic peptide (NT-proBNP), and BNP to screen for PHT in the SCD pediatric population. Children (8-18years old) with SCD-HbSS and HbSthal° were prospectively included and underwent PHT screening. The screening consisted of a comprehensive PHT symptoms evaluation, ECHO measurement, and NT-proBNP and BNP levels. A total of 73 patients were included (mean age 12±5.7years; >80% on hydroxyurea), of which 37% had a symptom consistent with PHT, including exertional dyspnea (26.5%), fatigue (17.6%), palpitation (14.7%), and chest pain (10.3%). ECHO was obtained in 53 (72.6%) patients, with only ECHO of 48 patients included in the final analysis. Elevated ECHO peak tricuspid regurgitant jet velocity (TRV) >2.5m/s or indirect findings to suggest PHT were seen in only two of 48 (4.2%). No significant differences were seen between those with and without PHT symptoms when compared for NT-proBNP, BNP, hemoglobin, pulmonary function testing, fractional exhaled nitric oxide, asthma, oxygen saturation, and sleep apnea. PHT symptoms are not consistent with ECHO, NT-proBNP nor BNP findings in children with SCD. PHT prevalence based on TRV was low in children on hydroxyurea, therefore screening may not be warranted for this group.

Association of increased arterial stiffness with diastolic dysfunction in ischemic stroke patients: the Norwegian Stroke in the Young Study.

Young and middle-aged ischemic stroke survivors have a high prevalence of hypertension, increased arterial stiffness and abnormal left ventricular (LV) geometry, which all are associated with the presence of LV diastolic dysfunction. However, the prevalence and covariates of diastolic dysfunction in these patients have not been reported. To explore diastolic dysfunction in ischemic stroke patients aged 15-60 years included in the Norwegian Stroke in the Young Study. Data from 260 patients with acute ischemic stroke was analyzed. Diastolic dysfunction was assessed by combining transmitral peak early flow (E), early diastolic mitral annular velocity (e'), E/e' ratio, left atrial volume index and peak tricuspid regurgitant jet velocity, following current European guidelines. Carotid-femoral pulse wave velocity at least 10 m/s by aplanation tonometry was defined as increased arterial stiffness. Prevalent diastolic dysfunction was found in 20% of patients (13% with diastolic dysfunction grade 1 and 7% with diastolic dysfunction grades 2-3). Patients with diastolic dysfunction were older and more likely to have hypertension, overweight, increased arterial stiffness, higher LV mass and less percentage nightly reduction in mean blood pressure (BP) (all P < 0.001). In a multivariable logistic regression analysis, diastolic dysfunction was associated with increased arterial stiffness [odds ratio 2.86 (95% confidence interval 1.05-7.79), P < 0.05] independent of age more than 45 years, overweight, hypertension, night-time BP reduction and LV mass. Among young and middle-aged ischemic stroke survivors, diastolic dysfunction was found in 20%. The presence of diastolic dysfunction was associated with increased arterial stiffness independent of higher age, overweight, hypertension, night-time BP reduction and LV mass.

89: Fetal endoscopic tracheal occlusion (FETO) reduces pulmonary hypertension in severe congenital diaphragmatic hernia (CDH)

Growing evidence indicates fetal endoscopic tracheal occlusion (FETO) improves perinatal survival in fetuses with severe congenital diaphragmatic hernia (CDH).This study evaluates the impact of FETO on the resolution of pulmonary hypertension (PH) in fetuses with isolated severe left or right-sided CDH. All fetuses evaluated for CDH between January 2004 and July 2017 at a single institution were included. The resolution of PH by 1 year of life was compared in a cohort of infants with severe CDH who underwent FETO to a non-FETO cohort. The non-FETO cohort included CDH neonates stratified by prenatal MRI severity indices, [observed to expected total lung volume (O/E TLV) and % liver herniation] into mild, moderate, and severe groups. Postnatal echocardiograms were used to determine presence of PH, defined as elevation of pulmonary arterial pressure > 2/3 systemic systolic pressure based on Doppler parameters of the ductus arteriosus, interventicular septal position, and/or peak tricuspid regurgitant jet velocity. Patients were included if they underwent at least one prenatal MRI, had serial echocardiograms (the first within 48 hours of life) and had no other major structural or chromosomal anomalies. ANOVA and Dunnett’s post hoc tests were used for comparisons. A total of 254 CDH cases were evaluated; 57% (n=145) had no major structural or chromosomal anomalies and 106 met inclusion criteria. The FETO cohort consisted of 18 severe CHD cases and the non-FETO cohort consisted of 35% mild (n=31), 36% moderate (n=32), 29% severe (n=25) CHD cases. The proportion of left-or right-sided CDH cases was not different among groups. All infants with severe CDH, with and without FETO, had evidence of neonatal PH (see Table). By 1 year of life, a higher proportion of infants with severe CDH who underwent FETO had resolution of PH compared to infants with severe CDH in the non-FETO cohort (66.7% vs. 28%; p: 0.021). Of note, this proportion was similar to that of the moderate cases in the non-FETO cohort (see Table). FETO was also associated with a reduced need for ECMO in severe CHD cases regardless of the site of the CDH lesion (p: 0.009). In severe CDH infants, FETO is associated with resolution of PH by 1 year of life akin to moderate CDH cases. FETO may induce changes in fetal lung architecture facilitating resolution of PH in severe CDH.View Large Image Figure ViewerDownload Hi-res image Download (PPT)

Elevated Tricuspid Regurgitant Jet Velocity In Lebanese Patients With Sickle Cell Disease Is Associated With Severe Disease and Is Clustered In Families

Introduction Pulmonary hypertension (PHT) is a known complication of sickle cell disease (SCD), and it is indirectly assessed by measurement of the tricuspid regurgitant jet velocity (TRV). Despite the fact that PHT was found to be associated with early mortality in adults SCD patients, the association between elevated TRV and SCD severity and mortality is still controversial particularly in children and adolescents. The objective of this study was to investigate the relationship between elevated TRV and parameters of disease severity in children, adolescents and young adults with SCD. Methods The study is a retrospective review of medical records of patients with SCD followed at the comprehensive sickle cell clinic at the American University of Beirut Medical Center between April 2002 and January 2012. These include 115 patients with homozygous hemoglobin S disease, 8 with sickle β0-thalassemia, and 24 with sickle β+-thalassemia who have had at least one echocardiogram done as part of their routine medical care. All echocardiograms were done at steady state, at least four weeks after the last vasoocclusive crisis (VOC) or acute chest syndrome (ACS). Elevated TRV was defined as peak TRV of 2.5 meters per second or higher. Disease severity was assessed by comparing hemolysis biomarkers, rate of transfusions, VOCs and ACS and other SCD complications. In patients with sequential echocardiograms, changes in hydroxyurea (HU) doses were examined against TRV variation. Results 147 patients were studied over a mean number of 3.77 ± 1.96 years. The mean age was 15.07 ± 8.62 years, ranging from 18 to 43.4 years, with 66% being below 18 years of age. Elevated TRV was found in 57 patients (38.8%). T-tests and chi-square tests did not show any statistically significant differences between the patients with normal and elevated TRV with respect to age, gender, SCD subtype, time of follow-up, presence of stroke, AVN, ulcers and splenectomy. In addition, hemoglobin (mean=9.43±1.46 in normal TRV group versus 9.20±1.19 in high TRV, p=0.309) mean corpuscular volume (MCV, mean=93.04±16.96 vs. 96.56±15.03, p=0.203), reticulocyte count (mean= 9.51 ± 4.81 vs 9.97±5.55, p=0.596), bilirubin (indirect bilirubin mean=1.40±1.28 vs. 1.44±1.04, p=0.883) and lactate dehydrogenase (LDH, mean=423.20±192.00 vs. 451.40±399.40, p=0.398) did not show any significant differences between the two groups. Among patients with elevated TRV, 71.9% were on HU at a mean dose of 16.27 mg/kg/day compared to 70% of patients with low TRV who were on a mean HU dose of 15.13 mg/kg/day. HU was started in both groups for indications including pain crises, ACS and low hemoglobin but not TRV alone. Initially markers of hemolysis including MCV, reticulocyte count, bilirubin and LDH were significantly higher and hemoglobin lower in patients with elevated TRV, but these differences between the two groups were no longer evident after the dose of HU was optimized. In the 99 patients with 2 or more echocardiograms, no trend in TRV variation was observed after HU dose adjustment and sequential TRV readings. The mean transfusion rate per patient did not differ between the two groups. However, RBC alloimmunization was found in 6.2% of patients with normal TRV compared to 15% of patients with elevated TRV. Results of the Poisson models demonstrated that the VOC rate was 1.4 times higher among subjects with TRV ≥ 2.5 compared to subjects with TRV &lt; 2.5 (rate ratio = 1.4; 95% CI = 1.04 to 2.02; p=0.029). Additionally, ACS rate was almost 3 times higher among subjects with high TRV (rate ratio = 2.8; 95% CI = 1.3 to 6.2; p=0.01). No deaths were reported in either study groups. Fisher's exact test and the generalized linear mixed model (GLMM) both indicated the presence of familial clustering of elevated TRV (Fisher's p-value: 0.009; GLMM p-value: 0.026). Conclusion The overall prevalence of elevated TRV in our study population was 38.8%. Despite improvement in hemolyis after introducing and optimizing hydroxyurea doses, patients with elevated TRV still had more severe disease as defined by higher rates of VOC, ACS and alloimmunization. Familial clustering of elevated TRV was observed in our highly consanguineous population. These findings suggest that elevated TRV is a marker that defines patients with more severe SCD even among children and young adults. Disclosures: No relevant conflicts of interest to declare.

OP0227 Features and longitudinal analysis of systemic sclerosis patients with inappropriate exercise-induced increase in pulmonary artery pressure estimated by echocardiography

BackgroundPatients with Systemic Sclerosis (SSc) are at risk of developing pulmonary hypertension (PH). The role of exercise testing in predicting this severe complication is still debated. In SSc, a subset...

Pressure reflection in the pulmonary circulation in patients with severe mitral regurgitation indicates adverse postoperative outcome

Severe pulmonary hypertension (PH) is a known risk factor in valvular surgery. In the present study, we hypothesized that the assessment of pressure reflection (PR) in the pulmonary circulation, indicating increased pulmonary vascular resistance, might improve the identification of patients with increased morbidity and mortality following surgery for severe mitral regurgitation. A total of 103 patients without atrial fibrillation were divided into three groups: Group 1 (n = 48), patients without PR; Group 2 (n = 36), patients with PR and pulmonary artery systolic pressure (PASP) ≤ 60 mmHg and Group 3 (n = 19), patients with PR and PASP >60 mmHg. Three variables related to PR were selected: the acceleration time in the right ventricular outflow tract (RVOT), the interval between peak velocity in the RVOT and peak tricuspid regurgitant jet velocity and the right ventricular pressure increase after peak RVOT velocity. There were no differences between groups in age, ejection fraction, need for coronary bypass grafting or creatinine. Patients with PR (Groups 2 and 3) had more use of vasoactive drugs (overall P < 0.0001, Group 1 vs Group 2 P = 0.018). The proportion of patients with >24 h in the intensive care unit was 27% in Group 1, 54% in Group 2 and 84% in Group 3 (overall P < 0.0001, Group 1 vs Group 2 P = 0.006). The in-hospital mortality in patients without PR (n = 49) was 0% compared with 10.9% in patients with PR (P = 0.029). Echocardiography assessment of PR in the pulmonary circulation and severe PH may identify patients with adverse outcome following mitral surgery.

Pulmonary hypertension diagnosed by right heart catheterisation in sickle cell disease

Recent studies have recognised the importance of pulmonary hypertension (PH) in sickle cell disease (SCD). The aim of this study was to determine the prevalence and prognostic impact of PH and its features in patients with SCD. 80 patients with SCD underwent baseline clinical evaluation, laboratory testing, 6-min walk tests (6MWTs) and echocardiography. Patients with a peak tricuspid regurgitant jet velocity (TRV) of ≥ 2.5 m·s(-1) were further evaluated through right heart catheterisation (RHC) to assure the diagnosis of PH. Our study evidenced a 40% prevalence of patients with elevated TRV at echocardiography. RHC (performed in 25 out of 32 patients) confirmed PH in 10% (95% CI 3.4-16.5%) of all patients, with a prevalence of post-capillary PH of 6.25% (95% CI 0.95-11.55%) and pre-capillary PH of 3.75% (95% CI -0.4-7.9%). Patients with PH were older, had worse performance in 6MWTs, and more pronounced anaemia, haemolysis and renal dysfunction. Survival was shorter in patients with PH. Our study reinforced the use of echocardiography as a screening tool for PH in SCD and the mandatory role of RHC for proper diagnosis. Our findings confirmed the prognostic significance of PH in SCD as its association to pronounced haemolytic profile.

Range in Pulmonary Artery Systolic Pressure Among Highly Trained Athletes

Although the physiologic range of pulmonary artery systolic pressure (PASP) has been reported, data on how it is manifested in athletes are limited. The aim of the present study was to explore the full spectrum of PASP and the long-term training impact in a large population of highly trained athletes. Six hundred fifteen consecutive athletes (370 endurance-trained athletes [ATEs] vs 245 strength-trained athletes [ATSs]; 28.4 ± 10.1 years old) and 230 healthy control subjects (27.5 ± 11.3 years old) underwent transthoracic echocardiography. PASP was estimated by measuring maximal tricuspid regurgitant jet velocity (TRV) with the modified Bernoulli equation. The ratio of TRV to right ventricular outflow tract time-velocity integral (TRV/RVOTTVI) was obtained as a correlate of pulmonary vascular resistance (PVR). Left ventricular (LV) mass index and ejection fraction did not differ significantly between the two groups of athletes. Conversely, ATSs showed an increased sum of wall thickness and relative wall thickness, whereas LV end-diastolic diameter, LV stroke volume, peak TRV, and PASP were significantly higher in ATEs. The ratio between transmitral E wave and tissue Doppler e' wave was not different among the three groups. The ratio TRV/RVOTTVI was ≤ 0.2 (ie, normal PVR) in all subjects. A TRV value > 2.5 m/s was observed in 76 athletes (12.3%). By multivariable analysis, age (P < .01), type and duration of training (P < .01), and LV stroke volume (P < .001) were the only independent predictors of PASP in athletes. This study delineates the full range of resting TRV and derived PASP in highly trained athletes. The upper physiologic limit of PASP in endurance athletes may reach 40 mm Hg, in line with the greater increase in stroke volume. This should be considered a "physiologic phenomenon" when evaluating athletes for sports eligibility.

Inappropriate exercise-induced increase in pulmonary artery pressure in patients with systemic sclerosis

BackgroundRecent data show that there is an unexpectedly high prevalence of ‘inappropriate’ pulmonary responses to exercise among patients with systemic sclerosis (SS). However, no consensus exists as to which threshold...

Increased Pulmonary Pressures and Myocardial Wall Stress in Children with Severe Malaria

Chronic intravascular hemolysis leads to nitric oxide (NO) depletion and pulmonary hypertension in sickle cell disease. To test whether this pathophysiology occurs in malaria, we examined in Mali 53 children who were admitted to the hospital with severe malaria (excluding cerebral malaria) and 31 age-matched controls. Severity of hemolysis was assessed from plasma levels of free hemoglobin and arginase-1. NO metabolism was assessed by whole-blood nitrite levels and plasma NO consumption. Effects on the cardiovascular system and endothelial function were assessed by using echocardiography to measure peak tricuspid regurgitant jet velocity and by evaluating plasma levels of N-terminal prohormone brain natriuretic peptide (NT-proBNP) and soluble vascular cell adhesion molecule-1. Children with severe malaria had higher plasma levels of hemoglobin and arginase-1, reduced whole-blood levels of nitrite, and increased NO consumption relative to controls. They also had increased pulmonary arterial pressures (P< .05) with elevated levels of NT-proBNP and soluble vascular cell adhesion molecule-1 (P< .001). Children with severe malaria have increased pulmonary pressures and myocardial wall stress. These complications are consistent with NO depletion from intravascular hemolysis, and they indicate that the pathophysiologic cascade from intravascular hemolysis to NO depletion and its cardiopulmonary effects is activated in children with severe malaria.

Reproducibility of tricuspid regurgitant jet velocity measurements in children and young adults with sickle cell disease undergoing screening for pulmonary hypertension

The reproducibility of tricuspid regurgitant jet velocity (TRJV) measurements by Doppler echocardiography has not been subjected to systematic evaluation among individuals with sickle cell disease (SCD) undergoing screening for pulmonary hypertension. We examined sources of disagreement associated with peak TRJV in children and young adults with SCD. Peak TRJV was independently measured and interpreted a week apart by separate sonographers and readers, respectively, in 30 subjects (mean age, 15.8 ± 3.3 years) who provided 120 observations. We assessed intra-/inter-reader, intra-/inter-sonographer, sonographer-reader, and within subject agreement using Intraclass Correlation Coefficient (ICC) and Cohen's kappa (κ). Agreement was examined graphically using Bland-Altman plots. Although sonographers could estimate and measure peak TRJV in all subjects, readers designated tricuspid regurgitation nonquantifiable in 10-17% of their final interpretations. Intra-reader agreement was highest (ICC = 0.93 [95% CI 0.86, 0.97], P = 0.0001) and within subject agreement lowest (ICC = 0.36 [95% CI 0.02, 0.64], P = 0.021) for single TRJV measurements. Similarly, intra-reader agreement was highest (κ = 0.74 [95% CI 0.53, 0.95], P = 0.0001) and within subject lowest (κ = 0.14 [95% CI -0.17, 0.46], P = 0.38) when sonographers and readers categorized TRJV measurements. On Bland-Altman plots, absolute differences in observations increased with higher mean TRJV readings for intra-/inter-reader agreement. Peak TRJV measurements in individual children and young adults with SCD are affected by several sources of disagreement, underscoring the need for methodological improvements that ensure reproducibility of this screening modality for making clinical decisions in this population.

Factors influencing the echocardiographic estimate of right ventricular systolic pressure in normal patients and clinically relevant ranges according to age

Previous studies have shown that in the absence of underlying cardiac pathology, the echocardiographic estimate of right ventricular systolic pressure (RVSP) increases progressively and normally with age. There are limited data in patients older than 60 years of age. To define the ranges of RVSP according to age and to include more elderly patients than have previously been reported. All patients undergoing echocardiography since May 26, 1999, at the Kingston Heart Clinic (Kingston, Ontario) have had their data entered into a locally designed cardiology database (CARDIOfile; Registered trademark, Kingston Heart Clinic). RVSP was calculated from the peak tricuspid regurgitant jet velocity (V) using the modified Bernoulli equation (RVSP = 4V2 + RAP), with the mean right atrial pressure (RAP) estimated to be 10 mmHg. Of the 22,628 patients who had undergone echocardiography, 10,905 had RVSP measured. All abnormal echocardiograms were excluded, leaving 1559 echocardiograms for analysis. Patient age ranged from 15 to 93 years. The mean age was 49 years. RVSP increased significantly only after the age of 50 years. The mean (+/- SD) RVSP for those younger than 50 years, 50 to 75 years, and older than 75 years of age was 27.3+/-5.7 mmHg, 30.2+/-7.6 mmHg and 34.8+/-8.7 mmHg, respectively (P<0.0001 among all age groups). The normal range (95% CI) of RVSP in those younger than 50 years, 50 to 75 years, and older than 75 years of age was 16 mmHg to 39 mmHg, 15 mmHg to 45 mmHg, and 17 mmHg to 52 mmHg, respectively. Multivariate analysis indicated that age, mitral diastolic early-to-late filling velocity ratio, ejection fraction, aortic size and early mitral filling velocity/ early diastolic mitral annular velocity were the only significant independent variables. There were significant changes in diastolic function with increasing age, which may have been responsible for the changes in RVSP. RVSP remains stable in both men and women until the age of 50 years. Thereafter, RVSP increases progressively in a linear manner with age and is significantly higher in patients older than 75 years of age. The changes may relate to changes in diastolic function. These ranges should be taken into account when using echocardiogram-derived RVSP for the diagnosis of pulmonary hypertension in the absence of cardiovascular disease.

Tricuspid regurgitant jet velocity elevation and its relationship to lung function in pediatric sickle cell disease

Concerns about the morbidity and mortality associated with tricuspid regurgitant jet velocity (TRJV) elevation, which may indicate pulmonary hypertension (PHT), in adults with sickle cell disease (SCD) have prompted growing interest in screening the pediatric sickle cell population. The goals of our study were to estimate the prevalence of TRJV elevation and determine its relationship to pulmonary function in children and young adults with SCD at baseline. Seventy-eight subjects (10-24 years old) with SCD underwent prospective screening by Doppler echocardiogram (ECHO), complete lung function evaluation, and laboratory testing as part of standard care at steady state. Tricuspid regurgitation was quantifiable in 68/78 (87%) subjects and peak TRJV was > or =2.5 m/sec in 26/78 (33.3%) evaluated. The frequency of obstruction, restriction, or abnormal gas exchange found on lung function evaluation was not significantly different in subjects with and without TRJV elevation. However, significant inverse correlations were observed between TRJV and both % predicted forced vital capacity (FVC) (r = -0.29, P = 0.022) and oxygen saturation (r = -0.26, P = 0.036). When compared to subjects without TRJV elevation, subjects with TRJV elevation had significantly lower % predicted forced expiratory volume in 1 sec (FEV(1)) (78.9 +/- 14.4 vs. 86.6 +/- 13.0%, P = 0.023), FVC (82.8 +/- 14.1 vs. 90.7 +/- 12.9%, P = 0.017), and oxygen saturation (95.8 +/- 3.2 vs. 97.5 +/- 2.4%, P = 0.016). We found that the combination of low hemoglobin and low % predicted FVC best predicted TRJV elevation (chi(2) = 17.05, P = 0.001) in our cohort, correctly identifying 70% of cases and resulting in positive and negative predictive values of 60 and 74%, respectively. We conclude that in this young population with SCD, TRJV elevation that is not significantly associated with abnormal lung function is common at baseline.

The Usefulness of Screening for Cardiopulmonary Complications in Asymptomatic Sickle Cell Patients in the United Kingdon (UK)

Background: Pulmonary hypertension (PHT) and chronic lung disease (CLD) are the chronic cardiorespiratory complications recognized in sickle cell disease (SCD). Although the incidence of these complications has been reported to be important, even in the asymptomatic patients, there is no data of the prevalence of these problems in a UK sickle population. Therefore, we established a ‘one-stop’ cardiopulmonary sickle screening clinic, which ran in conjunction with a routine review.Method: Adult patients with a diagnosis of SCD (HbSS, HbSC, HbSBthalassaemia), irrespective of symptoms were approached either during an outpatient visit, by a letter or telephone over a 4 month period. The initial screening tests consisted of an echocardiogram and pulmonary function tests (PFTs). Transthoracic Doppler echocardiography was used to measure tricuspid regurgitant jet velocity (TRV) Pulmonary hypertension was defined as a peak TRV of ≥ 2.5m/s and moderate to severe PHT was defined as a peak TRV ≥ 3m/s (Gladwin et al NEJM 2004). Spirometric tests were classified as normal, restrictive, and obstructive with a FEV1/FVC ratio of 70–80%, >80% and <70%, respectively (Eur Resp J, 1993). The patients' perspective of this approach was evaluated by a psychologist using a short semi-structured interview either in person or via telephone.Results: 62 patients agreed to take part, but only 44 (71%) attended the ‘one-stop clinic’. 29 patients had HbSS, 13 patients had HbSC, 1 patient had HbSB0 and 1 had HbSB+thalassaemia. Mean age (SD) was 36.6 years (9.5), and 20 participants were men. 30 patients had both an echocardiogram and full PFTs as planned. An additional 4 patients had an echocardiogram alone and 10 had PFTs alone. 2 patients were excluded due to an acute vasoocclusive crisis at the time of the test. Only 3/32 (9.4%) patients had abnormal echocardiograms with peak TRVs of 2.5, 2.5 and 2.6 m/s, respectively. Of the 38 patients, spirometry was normal in 6/38 (16%), restrictive in 13/38 (34%) and obstructive in 6/38 (16%). Interestingly, isolated low total lung capacity (TLC) was found in 13/38 (34%). Of the patients who a TRV≥ 2.5m/s, 2/3 had obstructive spirometry and 1/3 had an isolated low TLC. There was no association with type of SCD, use of hydroxyurea or previous chest crises. Finally, 18/44 (41%) of patients completed the interview with the majority reporting that the increased time spent in clinic was not obviously beneficial.Conclusion: The uptake of the ‘one-stop’ clinic for asymptomatic patients with SCD was reasonable. Although we found 32/38 (84%) patients had an abnormality of their PFTs, the echocardiographic data do not concur with the published data with respect to the prevalence of PHT in the asymptomatic SCD population. This needs further assessment on a large scale. Finally, the ‘one stop’ cardiopulmonary screening clinic does not appear a clinically effective use of resources in the UK with little perceived benefit by the patients.

Prevalence and Risk Factors of Elevated Pulmonary Artery Pressures in Children With Sickle Cell Disease

The objectives of this study were (1) to determine the prevalence and risk factors of elevated pulmonary artery pressures in children with homozygous SS or Sbeta(0) thalassemia using Doppler echocardiography and (2) to determine a correlation between abnormal transcranial Doppler examinations and elevated pulmonary artery pressures. Screening echocardiograms were prospectively performed during an annual comprehensive clinic visit on children who were older than 6 years and had homozygous SS or Sbeta(0) thalassemia. Detailed history, examination, and laboratory tests were done, and transcranial Doppler examinations were obtained in children 2 to 14 years of age. Pulmonary hypertension was defined as pulmonary artery systolic pressure of at least 30 mmHg corresponding to a peak tricuspid regurgitant jet velocity of > or = 2.5 m/second. Mild pulmonary hypertension was defined as tricuspid regurgitant jet velocity > or = 2.5 to 2.9 m/second. Moderate pulmonary hypertension was defined as tricuspid regurgitant jet velocity > or = 3 m/second. Patients with pulmonary stenosis or right outflow obstruction were excluded. Characteristics were compared between patients with mild, moderate, and no pulmonary hypertension using 1-way analysis of variance for continuous variable and Fisher's exact test for categorical variables. Of the 75 patients who had homozygous SS/Sbeta(0) thalassemia and were older than 6 years, echocardiograms were obtained for 62 (82.6%). Thirty percent (19 of 62) of patients had elevated tricuspid regurgitant jet velocity > or = 2.5 m/second. One third of these patients had tricuspid regurgitant jet velocity > or = 3 m/second. All patients with elevated tricuspid regurgitant jet velocity had SS disease. A high reticulocyte count, low oxygen saturation, and a high platelet count were significantly associated with elevated pulmonary artery pressures. There was no difference in age, gender, history of acute chest syndrome, hydroxyurea therapy, chronic blood transfusion, stroke, hemoglobin, and bilirubin between patients with and without elevated pulmonary artery pressures. A total of 47% patients with elevated tricuspid regurgitant jet velocity and 57% without elevated tricuspid regurgitant jet velocity had screening transcranial Doppler examinations. Transcranial Doppler examinations were normal for all patients. High pulmonary artery pressures do occur in children with sickle cell disease. Screening by echocardiography can lead to early detection and intervention that may potentially reverse this disease process. There was no correlation between elevated pulmonary artery pressures and abnormal transcranial Doppler examination in our study.

Correlation between Right Ventricular Indices and Clinical Improvement in Epoprostenol Treated Pulmonary Hypertension Patients

The aim of this study was to evaluate which parameter of right ventricular (RV) echocardiographic best mirrors the clinical status of patients with pulmonary arterial hypertension. Patients with pulmonary arterial hypertension on epoprostenol therapy were identified via hospital registry. Twenty patients, (16 females, 4 males) were included in the study, 9 with primary pulmonary hypertension and 11 with other diseases. Echocardiograms before therapy and at 22.7 (+/-9.3) months into therapy were compared. The right ventricular myocardial performance index (RVMPI) was measured as the sum of the isometric contraction time and the isometric relaxation time divided by right ventricular ejection time. Other measures included peak tricuspid regurgitation jet velocity (TRV), pulmonary artery systolic pressure (PASP), pulmonary valve velocity time integral (PVVTI), PASP/PVVTI (as an index of total pulmonary resistance) and symptoms by New York Heart Association (NYHA) functional class. Echo parameters of right ventricular function were analyzed in patients, before and during therapy. There was significant improvement of NYHA class in patients following epoprostenol therapy (P < 0.0001). Peak tricuspid regurgitant jet velocity (pre 4.2 +/- 0.6 m/sec, post 3.8 +/- 0.7 m/sec, P = 0.02) and PASP/PVVTI (pre 6.7 +/- 3.3 mmHg/m per second, post 4.8 +/- 2.2 mmHg/m per second, P < 0.0001) were significantly improved during treatment. RVMPI did not improve (pre 0.6 +/- 0.3, post 0.6 +/- 0.3, P = 0.54). Changes in NYHA class did not correlate with changes in RVMPI (P = 0.33) or changes in PASP/PVVTI (P = 0.58). Despite significant improvements in TRV, PASP/PVVTI, and NYHA class, there was no significant change in RVMPI on epoprostenol therapy. Changes in right ventricular indices were not correlated with changes in NYHA class.