Evaluation Of Pulmonary Artery Pressure Research Articles

Pulmonary artery acceleration time in young children is determined by heart rate and transpulmonary gradient but not by pulmonary blood flow: A simultaneous echocardiography—cardiac catheterization study

Pulmonary artery acceleration time (PAAT) is considered useful for the non-invasive evaluation of pulmonary artery pressure (PAP) and pulmonary vascular resistance (PVR). PAAT is dependent on PAP, PVR, pulmonary artery compliance, stroke volume, and heart rate. Its relative dependency on these determinants may differ between young and older children, raising uncertainty regarding its utility in young children. We aim to identify the primary determinants of the PAAT in children less than 36 months undergoing cardiac catheterization and its utility for the diagnosis of elevated PVR. We prospectively studied 42 children undergoing cardiac catheterization and simultaneous echocardiography. We determined the correlations of PAAT to the above-mentioned determinants and evaluated receiver operator characteristic (ROC) curves for diagnosis of PVR indexed to body surface area (PVRi) ≥3 Wu*m2 . Median age was 11.5 (IQR 5.2, 21.2) months. Moderate correlations were found between PAAT and mean PAP (R = -.66, p < .001), PVRi (R = -.54, p = .004), pulmonary artery compliance (R = .65, p < .001), transpulmonary gradient (R = -.67, p < .001), stroke volume (R = .61, p = .002), and heart rate (R = -.63, p < .001). In multivariate regression modeling, only transpulmonary gradient and heart rate were independent determinants of PAAT. PAAT ≤77 msec had acceptable utility for diagnosing PVRi ≥ 3 Wu*m2 (AUC .8 [.64, .95], n = 36), low sensitivity (59%), and excellent specificity (94%). Transpulmonary gradient and heart rate, but not pulmonary blood flow, are important determinants of PAAT in children <36 months undergoing cardiac catheterization. PAAT has low sensitivity for diagnosing elevated PVRi, therefore, should not be solely relied upon in screening for elevated PVRi in young children.

Evaluation of pulmonary arterial pressure in patients with connective tissue disease-associated pulmonary arterial hypertension by myocardial perfusion imaging.

BackgroundPulmonary arterial hypertension (PAH) is a complex and severe complication of connective tissue disease (CTD). We aimed to evaluate the application value of myocardial perfusion imaging (MPI) in evaluating CTD‐associated PAH (CTD‐PAH).MethodsWe retrospectively included 88 patients who were diagnosed with CTD between January 2018 and December 2020 at our hospital. Fifty‐eight patients had PAH and were included into the CTD‐PAH group. Thirty patients without PAH were included in the control group. All patients received routine physical examination, biochemical tests and cardiac function evaluation, right heart catheterization (RHC), and 99mTc‐MIBI MPI. PAH patients were divided into the mild, moderate, and severe PAH group according to their mean pulmonary artery pressures by RHC. Pearson correlation analysis was used to calculate the correlation between the right ventricle target/background (T/B) and right ventricle stroke volume (RV‐SV), total pulmonary resistance (TPR), pulmonary vascular resistance (PVR), mean pulmonary arterial pressure (mPAP), 6‐minute walk distance (6‐MWD), and N‐terminal B‐type natriuretic peptide (NT‐proBNP). The ROC curves of T/B and pulmonary artery pressure classification were plotted and the sensitivity and specificity of T/B in diagnosing PAH of different severities were analyzed.ResultsThe analysis of correlation revealed that T/B correlated negatively with 6‐MWD and positively with NT‐proBNP and exhibited good positive correlation with mPAP, TPR, and PVR by RHC and negative correlation with RV‐SV. T/B was of the most diagnostic value for severe PAH, and its correlation with severe PAH was stronger than that with mild PAH and moderate PAH.ConclusionsTarget/background is a noninvasive method that can simultaneously evaluate pulmonary arterial pressure and myocardial perfusion of CTD‐CHD patients and is particularly of relatively high value for severe PAH patients.

Evaluation of the Benefit of Extended Catheter-Directed Thrombolysis with Serial Angiography for Acute Pulmonary Embolism

PurposeTo evaluate whether extended catheter-directed thrombolysis (CDT) with repeat visits to the angiography suite provide added benefit in treatment of acute pulmonary embolism (PE). Materials and MethodsThis was a retrospective review of CDT procedures performed for acute PE in 156 patients (age 56.1 y ± 15.3, 46.2% women) between 2009 and 2019. All patients underwent at least 1 follow-up visit to the angiography suite for evaluation of pulmonary artery pressure (PAP) and thrombus burden (Miller score) before termination (111/156, 71.2%) or continuation of CDT (45/156, 28.8%). ResultsPatients who had CDT extended beyond the first follow-up visit required a higher total dose of tissue plasminogen activator (40.7 mg ± 14.3 vs 22.6 mg ± 9.9, P < .001) to achieve a similar final Miller score (6.4 ± 3.8 vs 7.6 ± 3.9, P = .1) and a similar reduction in systolic PAP (−14.4 mm Hg ± 10.2 vs −12.6 mm Hg ± 11.9, P = .6). The initial Miller scores were similar in both groups (19.7 ± 5.8 vs 19 ± 4, P = .4) but were significantly higher during the first follow-up visit (after 18 hours ± 5.5 vs 20 hours ± 4.8, P = .06) in patients requiring multiple follow-up visits (12.2 ± 5 vs 7.6 ± 3.9, P < .001). Multiple regression analyses identified heart rate > 100 beats/min and systolic PAP > 55 mm Hg as associated with the need for extended CDT. Extended CDT did not result in a higher hemorrhagic complication rate (1/45 vs 6/111, P = .7). ConclusionsPatients presenting with higher heart rates and systolic PAP may benefit from extended CDT to achieve similar reductions in PAP and thrombus burden, without clear added risk of hemorrhage.

Performance of echocardiography for detection of portopulmonary hypertension among liver transplant candidates: Meta-analysis.

Evaluation of pulmonary arterial pressure is crucial among cirrhotic patients, considering that moderate portopulmonary hypertension (POPH) is a contraindication for liver transplantation. Although right heart catheterization (RHC) is the most accurate method to diagnose POPH, it is invasive. The aim of the study is to evaluate the performance of echocardiography in detecting POPH in liver transplant candidates. A Literature search was performed, and pooled sensitivity, specificity, positive likelihood ratio (LR), negative LR, and area under the summary receiver operating curve (AUC) were calculated. Subgroup analyses were performed based on different cutoff values for echocardiography and diagnostic criteria of RHC. Sensitivity, specificity, positive LR, negative LR, and AUC of echocardiography for detection of POPH were 0.86 (0.74, 0.94), 0.87 (0.84, 0.90), 7.17 (3.59, 14.31), 0.22 (0.13, 0.38), and 0.807 while they were 0.82 (0.74, 0.89), 0.81 (0.78, 0.84), 117.75 (16.03, 865.08), 0.28 (0.16, 0.50), and 0.876for detection of moderate POPH, respectively. Performance of echocardiography was not significantly different in the subgroup analyses of stringency of POPH criteria and pulmonary arterial systolic pressure (ePASP) cutoffs. Our meta-analysis supports utilization of echocardiography for screening of POPH. However, RHC remains essential in highly suspicious cases. Echocardiographic data other than ePASP should be evaluated in future studies.

Neonatal functional echocardiography.

The role of echocardiography has dramatically changed over the past decade and use of functional echocardiography has become increasingly popular among neonatologists and pediatric intensivists in making clinical decisions in sick infants and children. The purpose of this review is to outline the current capabilities and limitations of functional echocardiography, best practices for its clinical application, and evidence for its utility. Functional echocardiography can provide direct assessment of hemodynamics at bedside and can be used as a modern hemodynamic monitoring tool in the neonatal intensive care unit. It is now being regarded as a useful extension to the clinical examination and other monitoring tools in the critically ill infant. The anatomic, physiological, and hemodynamic information functional echocardiography provides can be used in targeting specific interventions and evaluating response to treatment. This review article is focused on an overview of the most common applications of functional neonatal echocardiography also called targeted neonatal echocardiography or neonatologist performed echocardiography. It will review assessment of hemodynamics and cardiac function in neonatal hypotension and shock, suspected patent ductus arteriosus, suspected persistent pulmonary hypertension of the newborn with evaluation of pulmonary artery pressure and assessment of pericardial effusion. It will also emphasize the specific requirements for the practice and training of functional neonatal echocardiography.

ECHOCARDIOGRAPHIC EVALUATION OF THE RIGHT HEART IN THE PULMONARY HYPERTENSION

Introduction: A comprehensive assessment of right ventricular size and function, as well as evaluation of pulmonary artery pressures is an integral part of every echocardiographic examination. It is important to know the relevant guidelines but also the pitfalls of echocardiography. The aim: To determine the significance of echocardiography in the diagnostic process, prognosis and evaluation of treatment effectiveness in pulmonary hypertension. Review and Discussion: The gold standard for evaluation of size, ejection fraction, and stroke volume of the right ventricle is cardiac magnetic resonance. Whereas, the gold standard for the assessments of pulmonary artery pressures is right heart catheterization. However, echocardiography is the first diagnostic modality in the assessment of size and function of the right heart. Conclusions: Echocardiographic evaluation of the right heart plays a fundamental role in the diagnostic process of pulmonary hypertension. Echocardiography is essential to predict the course of the disease and assess the treatment efficiency.

Noninvasive evaluation of pulmonary artery pressure during exercise: the importance of right atrial hypertension.

Identification of elevated pulmonary artery pressures during exercise has important diagnostic, prognostic and therapeutic implications. Stress echocardiography is frequently used to estimate pulmonary artery pressures during exercise testing, but data supporting this practice are limited. This study examined the accuracy of Doppler echocardiography for the estimation of pulmonary artery pressures at rest and during exercise. Simultaneous cardiac catheterisation-echocardiographic studies were performed at rest and during exercise in 97 subjects with dyspnoea. Echocardiography-estimated pulmonary artery systolic pressure (ePASP) was calculated from the right ventricular (RV) to right atrial (RA) pressure gradient and estimated RA pressure (eRAP), and then compared with directly measured PASP and RAP. Estimated PASP was obtainable in 57% of subjects at rest, but feasibility decreased to 15-16% during exercise, due mainly to an inability to obtain eRAP during stress. Estimated PASP correlated well with direct PASP at rest (r=0.76, p<0.0001; bias -1 mmHg) and during exercise (r=0.76, p=0.001; bias +3 mmHg). When assuming eRAP of 10 mmHg, ePASP correlated with direct PASP (r=0.70, p<0.0001), but substantially underestimated true values (bias +9 mmHg), with the greatest underestimation among patients with severe exercise-induced pulmonary hypertension (EIPH). Estimation of eRAP during exercise from resting eRAP improved discrimination of patients with or without EIPH (area under the curve 0.81), with minimal bias (5 mmHg), but wide limits of agreement (-14-25 mmHg). The RV-RA pressure gradient can be estimated with reasonable accuracy during exercise when measurable. However, RA hypertension frequently develops in patients with EIPH, and the inability to noninvasively account for this leads to substantial underestimation of exercise pulmonary artery pressures.

Reassessment of the Accuracy of Cardiac Doppler Pulmonary Artery Pressure Measurements in Ventilated ICU Patients: A Simultaneous Doppler-Catheterization Study*

Doppler echocardiography is a well-recognized technique for the noninvasive evaluation of pulmonary artery pressure; however, little information is available concerning patients receiving mechanical ventilation. Furthermore, recent studies have debatable results regarding the relevance of this technique to assess pulmonary artery pressure. The aim of our study was to reassess the accuracy of Doppler echocardiography to evaluate pulmonary artery pressure and to predict pulmonary hypertension. Prospective observational study. Amiens ICU, France. ICU patients receiving mechanical ventilation. In 40 patients, we simultaneously recorded Doppler echocardiography variables (including tricuspid regurgitation and pulmonary regurgitation) and invasive central venous pressure, systolic pulmonary artery pressure, diastolic pulmonary artery pressure, and mean pulmonary artery pressure. Systolic pulmonary artery pressure assessed from the tricuspid regurgitation derived maximal pressure gradient added to the central venous pressure demonstrated the best correlation with the invasive systolic pulmonary artery pressure (r = 0.87) with a small bias (-3 mm Hg) and a precision of 9 mm Hg. A Doppler echocardiography systolic pulmonary artery pressure greater than 39 mm Hg predicted pulmonary hypertension (mean pulmonary artery pressure ≥ 25 mm Hg) with 100% sensitivity and specificity. Tricuspid regurgitation maximal velocity greater than 2.82 m/s as well as tricuspid regurgitation pressure gradient greater than 32 mm Hg predicted the presence of pulmonary hypertension. Pulmonary regurgitation was recorded in 10 patients (25%). No correlation was found between pulmonary regurgitation velocities and either mean pulmonary artery pressure or diastolic pulmonary artery pressure. Pulmonary acceleration time less than 57 ms and isovolumic relaxation time less than 40 ms respectively predicted pulmonary hypertension 100% of the time and had a 100% negative predictive value. Tricuspid regurgitation maximal velocity pressure gradient added to invasive central venous pressure accurately estimates systolic pulmonary artery pressure and mean pulmonary artery pressure in ICU patients receiving mechanical ventilation and may predict pulmonary hypertension.

Evaluation of pulmonary artery pressure in the aged population

Evaluation of pulmonary artery pressure in the aged population

Evaluation of pulmonary artery pressure in patients with juvenile systemic lupus erythematosus (jSLE).

Juvenile systemic lupus erythematosus (jSLE) is a chronic multisystemic autoimmune disease. Previous studies among adults have shown impaired right ventricular (RV) function in patients with SLE. Also, these patients may develop pulmonary artery hypertension (PAH), which is one of the most threatening complications of SLE. Nevertheless, studies on PAH among jSLE patients are still rare. The aim of this study was to assess the RV function in jSLE patients by Doppler echocardiography (Echo Doppler). We also estimated pulmonary artery systolic pressure (PASP) and mean pulmonary artery pressure (mPAP) in these patients. A total of 38 jSLE patients and 40 sex- and age-matched controls were retrospectively analyzed. All patients underwent combined M-mode, cross-sectional echo, and Doppler Echo examination. The RV function was significantly impaired in jSLE patients compared to controls. PASP and mPAP were normal in 37 out of 38 patients (97.37%), however, the mean values of PASP and mPAP were significantly higher in jSLE patients compared to controls (26.90 mmHg versus 21.71 mmHg and 12.63 mmHg versus 9.89 mmHg, respectively) [p < 0.05]. Only one patient (2.6 %) had elevated mPAP (60 mmHg). The right cardiac catheterization confirmed PAH in this patient. Although PAH was detected only in one patient, there was a marked increase of PAP in our jSLE patients. Overall, PASP and mPAP were significantly higher in jSLE patients compared to healthy controls. Prospective studies with ethnically diverse cohorts could give more insight on the relevance of PAP and PHT in patients with jSLE.

A modified noninvasive screening protocol for pulmonary hypertension in children with sickle cell disease—Who should be sent for invasive evaluation?

Invasive studies have shown that prevalence and severity of pulmonary hypertension (PH) in patients with sickle cell disease (SCD) tend to be overestimated if based exclusively on Doppler-derived tricuspid regurgitant velocity (TRV) as surrogate noninvasive marker with a cutoff ≥2.5 m/s. We aimed to better define a subgroup of pediatric SCD patients who should be sent for invasive evaluation of pulmonary artery pressure (PAP) based on a modified echocardiographic PH screening protocol that implements evidence from Doppler-catheter comparative studies. Charts of 121 pediatric patients with stable SCD were reviewed regarding echocardiographically assessed risk for elevated PAP/PH and associated clinical characteristics. TRV cutoff was refined at ≥2.9 m/s to avoid overestimating the risk for PH. TRV was combined with additional echocardiographic parameters to avoid underestimating the PH risk. Ninety-one patients qualified for analysis. Based on our modified echocardiographic protocol, 5.5% of patients qualified for at least moderate risk for elevated PAP (compatible with PH) as opposed to 20.9% if based exclusively on TRV ≥2.5 m/s. These patients were older, homozygous for hemoglobin S (HbSS), and more anemic. No subject had an echocardiographic risk constellation suggesting more than mild PH. Our modified noninvasive screening protocol-if confirmed by invasive studies-may help to better identify a subgroup of pediatric SCD patients in whom evaluation by catheterization appears justified. Unlike estimates based on the conventional protocol, the size of the targeted subgroup compares favorably with catheterization-confirmed PH prevalence rates. Characteristics associated with an increased PH risk were also identified.

Evaluation of pulmonary artery pressure and resistance by pulsed Doppler echocardiography in patients with end-stage renal disease on dialysis therapy

BackgroundPulmonary hypertension (PH) is one of the most important comorbidities in patients undergoing hemodialysis (HD). The goal of the present work is to determine the possible etiologic factors for its occurrence. MethodsThe prevalence of PH was estimated by Doppler echocardiography in a cohort of 100 patients aged 49.3±13.9years on regular HD. Mean pulmonary artery pressure was estimated from pulmonary acceleration time by Mahan’s regression equation. Pulmonary vascular resistance and pulmonary capillary wedge pressure were calculated. We focused on the effect of HD on left and right ventricle diastolic and systolic function. Right ventricle systolic function was assessed by tricuspid annular systolic excursion and pulsed Doppler myocardial performance index. Since impaired endothelial function was postulated as an underlying cause of PH, we studied the effects of HD on brachial artery endothelial function. ResultsThe current study found that pulmonary hypertension was prevalent in 70% of patients on dialysis. Left atrium diameter, left ventricle mass indexed to body surface area, and mitral E/E′ were increased in the dialysis group (4.4±0.2cm, 126.5±24.6g/m2, and 16.9±4.4, respectively, p<0.001 for all). Pulmonary artery systolic pressure was positively correlated to duration of dialysis and negatively correlated to glomerular filtration rate (p<0.001 and r=−0.991). Pulmonary vascular resistance was significantly increased in dialysis patients (1.9±0.2 Wood units vs. 1.2 Wood units in controls, p<0.001). Endothelial dysfunction, defined as brachial artery flow mediated dilatation <6%, was found in 46% of dialysis group. ConclusionIncreased pulmonary artery systolic pressure in the HD population could be attributed to left atrium dilatation and left ventricle diastolic dysfunction. Pulmonary vascular resistance was significantly increased in dialysis group. This might be explained by impaired endothelial nitric oxide synthesis that not only caused systemic vasoconstriction but also affected the pulmonary vasculature.

Stress echocardiography in paediatric cardiology.

Stress echocardiography is a well-established technique in adult cardiology and is mainly used for assessing regional myocardial function in patients with known or suspected coronary artery disease (CAD). Apart from detecting ischaemia, stress echocardiography has found its place in the assessment of the haemodynamic significance of valve disease, particularly in selected patients with aortic stenosis and mitral regurgitation. Also in the paediatric population, stress imaging is most commonly used for the detection of ischaemia in patients with CADs such as post heart transplantation, Kawasaki Disease, and abnormal origin of coronary arteries. Other paediatric indications include the haemodynamic and myocardial response in patients with different types of congenital heart disease, the early detection of myocardial dysfunction in specific populations such as patients after anthracycline exposure, and the evaluation of pulmonary artery pressures and the right ventricular functional response. Techniques have evolved over time and in different paediatric echocardiographic laboratories, exercise stress echocardiography is replacing dobutamine stress echocardiography in older children. Moreover, integrating tissue Doppler and strain technology with stress imaging allows a more quantitative analysis of regional and global systolic and diastolic function. Current clinical applications mainly include patients after transplant, suspected CAD, and hypertrophic cardiomyopathy.

Non-invasive estimation of pulmonary vascular resistance in patients of pulmonary hypertension in congenital heart disease with unobstructed pulmonary flow.

Context:Pulmonary vascular resistance (PVR) is a critical and essential parameter during the assessment and selection of modality of treatment in patients with congenital heart disease accompanied by pulmonary arterial hypertension.Aim:The present study was planned to evaluate non-invasive echocardiographic parameters to assess pulmonary vascular resistance.Settings and Design:This prospective observational study included 44 patients admitted in the cardiology and pediatric cardiology ward of our institution for diagnostic or pre-operative catheter based evaluation of pulmonary arterial pressure and PVR.Materials and Methods:Detailed echocardiographic evaluation was carried out including tricuspid regurgitation velocity (TRV) and velocity time integral of the right-ventricular outflow tract (VTIRVOT). These parameters were correlated with catheter-based measurements of PVR.Results:The TRV/VTIRVOT ratio correlated well with PVR measured at catheterization (PVRcath) (r = 0.896, 95% confidence interval [CI] 0.816 to 0.9423, P < 0.001). Using the Bland-Altman analysis, PVR measurements derived from Doppler data showed satisfactory limits of agreement with catheterization estimated PVR. For a PVR of 6 Wood units (WU), a TRV/VTIRVOT value of 0.14 provided a sensitivity of 96.67% and a specificity of 92.86% (area under the curve 0.963, 95% confidence interval 0.858 to 0.997) and for PVR of 8 WU a TRV/VTIRVOT value of 0.17 provided a sensitivity of 79.17% and a specificity of 95% (area under the curve 0. 0.923, 95% confidence interval 0.801 to 0.982).Conclusions:Doppler-derived ratio of TRV/VTIRVOT is a simple, non-invasive index, which can be used to estimate PVR.

Accidental left circumflex artery to right lung fistula in a suspected case of pulmonary hypertension.

A 56-year-old woman was referred to the cardiology department of the Shahid Modarres hospital. The patient had a history of pulmonary thromboembolism 20 years ago which had been managed by the inferior vena cava filter and since then the patient has been on warfarin. Her chief complaint was chronic dyspnea on exertion (NYHA class II) from several years ago. Right and left heart catheterization was performed for evaluation of pulmonary artery pressure. We found rich collateral formations between LCX as well as RCA and right pulmonary artery, primarily assumed as multiple fistulas. Among patients who have chronic thromboembolic pulmonary hypertension, systemic collateral supply to the pulmonary parenchyma has been previously reported to occur from both bronchial and/or nonbronchial systemic circulations. Our patient had neither signs of heart failure nor myocardial ischemia and, thus, was a candidate for conservative management. The adenosine pulmonary reactivity test was not performed because of low pulmonary pressure which had been estimated to be high.

1701 Doppler Echocardiographic Evaluation of Pulmonary Artery Pressure in low Birth Newborn with Abdominal Compartment Syndrome after Abdominal Surgical

In this prospective study 16 newborn (ranging 1 day - 28 day) with severe compartment abdominal syndrome were evaluated by Doppler echocardiography for the presence of pulmonary hypertension (PH). The...

Not hearing is believing: novel insight into cardiopulmonary function using agitated contrast and ultrasound

the advancement of science depends heavily on the development and successful application of new techniques. Progress in ultrasound technology has allowed researchers in physiology to examine many important parameters noninvasively, including cerebral blood flow velocity, pulmonary artery systolic

Teste farmacológico pré-implante na hipertensão pulmonar elevada e fixa em candidatos a transplante heterotópico

Evaluation of pulmonary artery pressure just before transplanting with sodium nitroprusside may allow conversion to orthotopic technique.Between 1992 and 2007, 228 transplants were performed systematically and this was used in seven patients with preoperative hemodynamic evaluation: Pre NP (mmHg) Post NP (mmHg) Systolic systemic blood pressure (PSAS) 108-78 (101.7 ± 10.9) 90-74 (79.5 ± 15.2) pulmonary arterial systolic pressure (PASP) 88-51 (69.8 ± 13.2) 70-40 (57.8 ± 9.9) Gradient transpulmonary (GTP) 16-11 (14.2 ± 1.7) 14-11 (12.4 ± 1.2) pulmonary vascular resistance (PVR/w) 7.9 to 4.8 (6.2 ± 1 0) 5.9-4.1 (5.0 ± 0.8).The intraoperative findings were: Pre NP (mmHg) e Post NP (mmHg), respectively, PSAS 91-78 (8.5 ± 5.2) and 65-59 (4.2 ± 63.8) (P = 0.017), decrease 19.9%, decrease 29.3%; PSAP 71-52 (61.8 ± 6.1) and 43-32 (37.5 ± 3.3) (P = 0.018), decrease 28%, decrease 41%. In light of these data, patients were transplanted by orthotopic technique not being observed mortality in the short and long-term evolution from 5 months to 6 years.This methodology allowed the conversion of the technique for heterotopic orthotopically, with good early and late outcomes.

Pulmonary hypertension in patients with hematological disorders following splenectomy

Prevalence of pulmonary arterial hypertension (PAH) was studied by Echocardiography and Doppler in 43 splenectomised patients with various disorders 1-20 years after splenectomy. PAH was detected only in thalassemia major, intermedia, hereditary sphereocytosis and myelofibrosis groups comprising a total of 21 patients. Six patients out of 21 was found to have PAH with mean pulmonary arterial pressure of 46.28 ± 28.17 mmHg. Twenty one controls having similar duration and type of disease also were assessed for PAH in this case control study 3/21 had PAH in this control group. The difference in number of patients showing pulmonary hypertension between case and control was not statistically significant (chi-square test p = 0.29-though the difference in pulmonary arterial pressure between case and control were significantly different (t-test p<0.0029) with control group showing a mean pulmonary arterial pressure of 25 ± 19 mmHg.Platelet count in the splenectomised group was significantly higher (p = 0.0029) than the controls. Pulmonary thromboembolism was equally high in the PAH patients with and without splenectomy. Patients undergoing splenectomy due to trauma, immune thrombocytopenia, sideroblastic anemia, extra hepatic portal hypertension, autoimmune hemolytic anemia did not show PAH after splenectomy even years after the procedure PAH following splenectomy is common after certain disorders and control patients with these diseases have tendency to develop PAH even without splenectomy. Pulmonary thromboembolism may be an important pathophysiological mechanism leading to this condition. Patients having hemolytic anemia and myelofibrosis should have regular evaluation of pulmonary arterial pressure whether he/she has been splenectomised or not. This is particularly important as availability of phosphodiesterase inhibitors like sildenafil allows one to manage these cases.

Echocardiography as an Outcome Measure in Scleroderma-related Pulmonary Arterial Hypertension: A Systematic Literature Analysis by the EPOSS Group

To assess the validation status of echocardiography with continuous Doppler (echo-Doppler) as an outcome measure in pulmonary arterial hypertension associated with systemic sclerosis (PAH-SSc). Structured literature review on full-text English articles was performed using the PubMed and Cochrane databases. Assessment of validation of echo-Doppler was based on the OMERACT filter criteria with the domains truth (face, content, construct, and criterion validity), discrimination, and feasibility. Out of 35 studies eligible for analysis, only 5 included well defined PAH-SSc subgroups (World Health Organization criteria). Echo was considered as having face validity based on expert opinion and high number of studies using echo for evaluation of patients with SSc. Echo was considered partially validated with respect to criterion validity based on significant correlations between echo measures and right-heart catheterization in patients with SSc at risk of PAH/PH. However, echo was found to lack specificity (lack of content validity), since measurements of echo pulmonary pressure may be influenced by left-heart disease and interstitial lung disease. Data from general populations of patients with scleroderma indicate that evaluation of pulmonary artery pressure by echo might not be available in all PAH-SSc patients because of technical factors. No studies enabling evaluation of the discriminant capacity over time and treatment of echo in PAH-SSc could be identified. Further studies are needed to fully validate echo-Doppler as an outcome measure in PAH-SSc. These studies would include cross-sectional analysis of baseline measures and longitudinal data of placebo and verum groups in randomized controlled trials of patients with PAH-SSc.