Screening For Pulmonary Hypertension Research Articles

The Application of Point of Care Ultrasound to Screen for Pulmonary Hypertension: A Narrative Review.

Pulmonary Hypertension (PH) is a condition with several cardiopulmonary etiologies that has the potential of progressing to right heart failure without proper intervention. After a history, physical exam, and investigations, cases of suspected PH typically undergo imaging via a transthoracic echocardiogram (TTE). This is a resource-intensive procedure that is less accessible in remote communities. However, point of care ultrasound (POCUS), a portable ultrasound administered at the bedside, has potential to aid in the diagnostic process of PH. The MEDLINE, Embase, and CENTRAL databases were searched to screen the intersection of POCUS and PH. Studies involved adult patients, and only English articles were accepted. Reviews, case reports, unfinished research, and conference abstracts were excluded. Our aim was to identify primary studies that correlated POCUS scan results and additional clinical findings related to PH. Nine studies were included after our search. In these studies, POCUS was effective in identifying dilatation of inferior vena cava (IVC); internal jugular vein (IJV); and hepatic, portal, and intrarenal veins in patients with PH. The presence of pericardial effusion, pleural effusion, or b-lines on POCUS are also associated with PH. This review suggests important potential for the use of POCUS in the initial screening of PH. IVC and basic cardiopulmonary POCUS exams are key for PH screening in patients with dyspnea. Right-heart dilatation can be visualized, and peripheral veins may be scanned based on clinical suspicion. POCUS offers screening as an extension of a physical exam, with direct visualization of cardiac morphology. However, more studies are required to develop a statistically validated POCUS exam for PH diagnosis. More studies should also be conducted at the primary-care level to evaluate the value of screening using POCUS for PH in less-differentiated patients.

Diagnosis of pulmonary hypertension associated with congenital heart disease based on statistical features of the second heart sound

Aiming at the problems of obscure clinical auscultation features of pulmonary hypertension associated with congenital heart disease and the complexity of existing machine-aided diagnostic algorithms, an algorithm based on the statistical characteristics of the high-frequency components of the second heart sound signal is proposed. Firstly, an endpoint detection adaptive segmentation method is employed to extract the second heart sounds. Subsequently, the high-frequency component of the heart sound is decomposed using the discrete wavelet transform. Statistical features including the Hurst exponent, Lempel-Ziv information and sample entropy are extracted from this component. Finally, the extracted features are utilized to train an extreme gradient boosting algorithm (XGBoost) classifier, which achieves an accuracy of 80.45% in triple classification. Notably, this method eliminates the need for a noise reduction algorithm, allows for swift feature extraction, and achieves effective multi-classification using only three features. It is promising for early screening of pulmonary hypertension associated with congenital heart disease.

ECG patterns to predict pulmonary arterial hypertension in patients with severe tricuspid regurgitation

IntroductionEchocardiographic evaluation of tricuspid regurgitation (TR) velocity is a key measure in screening for pulmonary hypertension. Based on its value and additional features of right ventricle overload patients are classified into low, intermediate or high probability of pulmonary hypertension which transfers into decisions of further invasive evaluation. However, in the presence of severe TR echocardiography underestimates pulmonary artery pressure and therefore pulmonary hypertension may be overlooked in some patients. Accordingly, in the present study we aimed to assess the role of electrocardiography in predicting the presence of pulmonary arterial hypertension (PAH) in patients with severe TR. ResultsWe analysed 83 consecutive patients with severe TR who were diagnosed in our centre between February 2008 and 2021 and who underwent right heart catheterization. Of them 58 had PAH while 25 had isolated TR (iTR).We found that the following ECG criteria supported the diagnosis of PAH as opposed to the diagnosis of iTR: R:SV1 > 1.0, max RV1 or 2 + max S I or aVL –SV1 > 6 mm, SI/RI > 1 in I. For these parameters using ROC analysis we found that the optimal thresholds suggesting the presence of pulmonary hypertension were: R:SV1 > 1.5 (AUC = 0.74, p = 0.0004, sensitivity 57.1%,specificity of 85%), max RV1 or 2 + max S I or aVL – SV1 > 3 mm (AUC = 0.76, p < 0.0001, sensitivity 91.4%, specificity of 60%) and for SI:RI > 0.71 (AUC = 0.79, p < 0.0001, sensitivity 82.5%,specificity of 70.8%). Presence of atrial fibrillation predicted iTR with 76% sensitivity and 81% specificity. ConclusionsECG analysis can improve the diagnostic process for patients with severe TR. The presence of atrial fibrillation facilitates the diagnosis of isolated tricuspid regurgitation (iTR), while increased values of R:SV1, R:SI, and increased max RV1 or 2 + max SI or aVL – SV1 favor the diagnosis of TR secondary to PAH.

Yield of genetic evaluation in non-syndromic pediatric moyamoya patients.

Few guidelines exist for genetic testing of patients with moyamoya arteriopathy. This study aims to characterize the yield of genetic testing of non-syndromic moyamoya patients given the current pre-test probability. All pediatric moyamoya patients who received revascularization surgery at one institution between 2018 and 2022 were retrospectively reviewed. Patients with previously diagnosed moyamoya syndromes or therapeutic cranial radiation were excluded. Of 117 patients with moyamoya, 74 non-syndromic patients (44 females, 59%) were eligible. The median age at surgery was 8.1years. Neurosurgeons referred 18 (24%) patients for neurogenetic evaluation. Eleven (61%) patients subsequently underwent genetic testing. Eight (73%) patients had available testing results. Five (62.5%) of these patients had developmental delay compared to 16 (22%) of the entire cohort. Six (75%) patients who underwent genetic testing were found to have at least one genetic variant. These results led to diagnosis of a new genetic disorder for 1 (12.5%) patient and screening recommendations for 2 (25%) patients. An RNF213 variant in one patient led to recommendations for family member screening and pulmonary hypertension screening. Another patient was diagnosed with CBL disorder and referred for cancer screening. The median age at surgery in patients with clinically actionable findings was 4.6years compared to 9.2years in those who were referred for genetic testing. All 3 patients who had an actionable finding had developmental delay. It may be beneficial to refer moyamoya patients under 5 for genetic screening given the high likelihood of discovering actionable mutations.

Echocardiographic analysis of pulmonary arterial systolic pressure in pulmonary hypertension.

The reliability of pulmonary arterial systolic pressure by transthoracic echocardiography is limited by its variability to define pulmonary hypertension. To know the variability of pulmonary arterial systolic pressure estimated by echocardiography in pulmonary hypertension. Their demographic variables were obtained. From 2016-2020 subjects with pulmonary hypertension were recruited, with pulmonary artery systolic pressure estimated by transthoracic echocardiography and by right heart catheterization. Data were analyzed using the Bland-Altman descriptive statistic and the intraclass correlation coefficient (95% confidence interval). 152 subjects, age 60 ± 12 years, were studied. Body mass index 27.64 ± 4.69 kg/m2. The pulmonary artery systolic pressure estimated by transthoracic echocardiography 58.99 ± 18.62 vs. cardiac catheterization 55.43 ± 16.79 mmHg. Mean difference (bias) -3.6 (29.1, -36.2) and intraclass correlation coefficient 0.717 (0.610, 0.794). Variability is wide, and agreement is substantial for pulmonary artery systolic pressure. It is recommended to estimate only as screening for pulmonary hypertension.

Is relevant to add stress echocardiography with semi-supine bicycle at the screening of pulmonary hypertension in systemic sclerosis patients?

Abstract Funding Acknowledgements Type of funding sources: None. Introduction Pulmonary hypertension (PH) is the leading cause of death in systemic sclerosis, with a 50% mortality rate within three years of PH diagnosis. The 3-year survival of patients with SSc and PH is estimated to be 56% compared with 94% for those without PH.(1,2). Patients with SSc are at risk of developing group 1 to 3 PH. Detection of PH with echocardiography at rest remains the essential clinical tool; several studies suggested that in patients with SSc, an even earlier change would be the abnormal increase in pulmonary pressures with exercise. (3–6) Purpose Exercise-induced pulmonary hypertension may represent an early but clinically relevant phase in the spectrum of pulmonary vascular disease. On the other hand, increases in mean pulmonary artery pressure on exercise echocardiography can be followed over time, indicating the progression of the pulmonary vascular disease before PH. Methods Observational, prospective, cross-sectional, and analytical study. A basal transthoracic echocardiogram (TTE) and physical stress echocardiogram test were performed, and a TTE follow-up at four years. Results 40 patients. A follow-up was 48 months ED 6 months. Women (93%). Mean age of 49.8 ED 11.4 years. 9.8% of the patients had systemic arterial hypertension; no patient had diabetes mellitus or other chronic-degenerative diseases. 28 (68.3%) patients had the limited cutaneous variety, and 13 (31.7%) patients had the diffuse type. In the basal TTE at the first visit, all had a low probability of presenting pulmonary hypertension; in the follow-up echocardiogram, 11 (26.8%) patients showed a high likelihood of pulmonary hypertension. TTE with a cycle ergometer at first visit: 19 (46.3%) patients presented PSAP &amp;gt;50 mmHg, 16 (39%) patients gave DeltaPSAP &amp;gt;24 mmHg, 19 (46.3%) patients showed a relationship mPAP &amp;gt;30 mmHg + PVR &amp;gt; 3 mmHg/L/min The correlation between stress test variables and the high probability of pulmonary hypertension at follow-up was PSAP&amp;gt;50 mmHg exercise (r 0.58, p&amp;lt;0.001). Delta PSAP &amp;gt;24 mmHg exercise(r 0.40, p 0.011). mPAP&amp;gt;30 mmHg + PVR&amp;gt;3 mmHg/L/min exercises (r 0.45, p 0.003). PVR and &amp;gt;3 mmHg/L/min (r 0.15, p 0.310). Conclusion(s) Exercise-induced pulmonary hypertension is a promising tool in screening patients with systemic sclerosis for the detection of pulmonary hypertension at earlier preclinical stages. Identifying patients with closer follow-up for TTE at rest every 3 to 6 months and not every year as suggested in this disease.

Echocardiographically-derived septal positional angle (EDSPA) as a measure of elevated right ventricular systolic pressure

BackgroundPulmonary hypertension is a significant yet rare disease that can have many long-term consequences, including death. Cardiac catheterization is the gold standard for measuring pulmonary artery mean pressures (PAMP), but is invasive and risks potentially serious complications. This study hypothesizes that a semi-quantitative, non-invasive echocardiographic measure of septal positioning can be used to assess PAMP. ObjectiveTo assess an alternative echocardiographic measure of right ventricular systolic pressure. MethodsThis study was a retrospective study of patients with and without pulmonary hypertension who had a transthoracic echocardiogram and cardiac catheterization. Patients undergoing atrial septal defect closure represented controls. Two blinded readers calculated the “Echocardiographically-Derived Septal Positional Angle (EDSPA),” which was compared to corresponding catheterization data, including mean pulmonary artery pressures. ResultsA total of 159 children were included, of which 150 had interpretable echocardiographic data based on image quality or axis of imaging plane. 39 children were identified as having pulmonary hypertension while 111 children had an atrial septal defect. Patient age ranged from a minimum of 54 days and maximum of 19 years [mean 7.1 years (SD = 5.30)]. Inter-observer variability between two readers [Pearson correlation coefficient of 0.939 (p < .001)] and intra-observer variability were low [intraclass correlation coefficient (ICC) of 0.95 and 0.96 for each observer, respectively]. An EDSPA of ≤39° predicted a PAMP>20 mm Hg (as measured by cardiac catheterization) with a 76 % sensitivity and 76 % specificity (AUC 0.846). ConclusionsEDSPA is a useful, non-invasive, and reproducible echocardiographic measure of PAMP that is easy to perform. With a sensitivity and specificity near 80 %, it has significant utility in screening for pulmonary hypertension and determining which patients should undergo further invasive diagnostic testing.

IMPLICATION OF PULMONARY ARTERY SYSTOLIC PRESSURE FOR PROLONGED CARDIOPULMONARY BYPASS DURATION IN PATIENTS UNDERGOING RE-REPAIR FOR DEGENERATIVE MITRAL REGURGITATION

Objective: Emerging evidence revealed that the longer duration of cardiopulmonary bypass (CPB) during cardiac surgery renders patients at higher risk of adverse outcomes. However, little is known regarding the risk factor of prolonged CPB in patients undergoing re-repair of degenerative mitral regurgitation (DMR). We aimed to explore the prognostic significance of pulmonary artery systolic pressure (PASP) on CPB duration in patients undergoing re-repair of DMR. Design and method: Patients with DMR undergoing re-repair in our center were consecutively enrolled from Jan 2009 to Dec 2021. Patients were stratified into the prolonged CPB group (CPB time &lt; 132 min, n = 61) and the shorter CPB (CPB time &lt; 132, n = 59). Preoperative Univariable and multivariable logistic regression analyses were used to evaluate the impact of PASP on the risk of prolonged CPB duration. Results: Of the 120 patients enrolled, 61 had prolonged CPB time (50.8%). The mean preoperative PASP was higher in the prolonged CPB group compared with the shorter CPB group (45.2±21.9 vs. 37.7±15.1, P &lt; 0.05). CPB time increased with higher preoperative PASP (P &lt; 0.05). For per 10 units of pre-PASP value increase in patients undergoing re-repair for DMR, there were 1.25 times more likely to be associated with a prolonged CPB time (adjusted odds ratio [OR] 1.25, 95% confidence interval [CI] 1.01-1.56, P &lt; 0.05). Patients with preoperative PASP &gt; 50mmHg had an adjusted OR of 3.66 (95% CI 1.36-9.84, P &lt; 0.05) for a prolonged CPB period compared with those with preoperative PASP &lt; 50mmHg. Conclusions: Preoperative PASP in patients requiring re-repair for DMR was associated with a higher likelihood of an extended time of CPB. Regular screening of pulmonary hypertension may aid in perioperative risk-stratification in patients prior to re-repair for DMR.

Echocardiographic estimation of pulmonary vascular resistance in advanced lung disease.

Noninvasive assessment of pulmonary hemodynamics is often performed by echocardiographic estimation of the pulmonary artery systolic pressure (ePASP), despite limitations in the advanced lung disease population. Other noninvasive hemodynamic variables, such as echocardiographic pulmonary vascular resistance (ePVR), have not been studied in this population. We performed a retrospective analysis of 147 advanced lung disease patients who received both echocardiography and right heart catheterization for lung transplant evaluation. The ePVR was estimated by four previously described equations. Noninvasive and invasive hemodynamic parameters were compared in terms of correlation, agreement, and accuracy. The ePVR models strongly correlated with invasively determined PVR and had good accuracy with biases of <1 Wood units (WU), although with moderate precision and wide 95% limits of agreement varying from 5.9 to 7.8 Wood units. The ePVR models were accurate to within 1.9 WU in over 75% of patients. In comparison to the ePASP, ePVR models performed similarly in terms of correlation, accuracy, and precision when estimating invasive hemodynamics. In screening for pulmonary hypertension, ePVR models had equivalent testing characteristics to the ePASP. Mid-systolic notching of the right ventricular outflow tract Doppler signal identified a subgroup of 11 patients (7%) with significantly elevated PVR and mean pulmonary artery pressures without relying on the acquisition of a tricuspid regurgitation signal. Analysis of ePVR and determination of the notching pattern of the right ventricular outflow tract Doppler flow velocity envelope provide reliable insights into hemodynamics in advanced lung disease patients, although limitations in precision exist.

A Systematic Review of Echocardiographic Parameters for the Screening of Pulmonary Hypertension: What Are the Odds?

Pulmonary hypertension (PH) is an insidious disease that often presents in late stages due to nonspecific signs and symptoms. Right heart catheterization (RHC) is the gold standard diagnostic test, and echocardiogram (ECHO) is the best screening tool. However, the strength of evidence and diagnostic utility of various echocardiographic parameters to screen for is not well elucidated. This systematic review (SR) is reported in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) statement. Literature searches was performed for the period of January 1, 2016, to June 1, 2021, on seven databases. We included full-text studies with adult patients that used RHC for comparison and provided sensitivity and specificity results. Likelihood ratios (LRs) and diagnostic odds ratios (DORs) were calculated. Risk of bias was assessed using the Quality Assessment Tool for the Observational Cohort and Cross-Sectional Studies. We identified 102 studies, but only 14 satisfied our inclusion criteria. The most significant parameters identified for PH screening based on LRs are, in descending order, tricuspid regurgitation gradient peak >36mmHg, systolic pulmonary artery pressure >41mmHg, and tricuspid regurgitation velocity >2.9 m/s. There is strong correlation between LR and DOR for these parameters. This SR indicates the superiority of some ECHO parameters over others to aid in the screening and severity assessment of PH. Variables with low LR (-) ratios may help to prevent unnecessary invasive assessment for PH. Clinicians should utilize a multi-parameter approach when interpreting echocardiograms for PH assessment.

Association of Elevated Tricuspid Regurgitation Velocity with Kidney and Cerebrovascular Disease in Children with Sickle Cell Disease

Introduction: Adult patients with sickle cell disease (SCD) develop progressive end-organ injury leading to multi-organ disease and increased mortality. It is plausible that this multi-organ damage begins in pediatric patients. Tricuspid regurgitation velocity (TRV), measured by echocardiography is a surrogate marker for pulmonary hypertension that is a poor prognostic factor in adults with SCD. The utility of screening echocardiograms as a marker of disease severity is being studied in pediatrics. In this study, we assessed associations between elevated TRV with early development of chronic kidney disease (albuminuria), and cerebrovascular disease (CVD). Methods: We performed an IRB approved cross-sectional study to evaluate the prevalence of elevated TRV ≥ 2.5 m/s and its associations with albuminuria and CVD in patients with SCD ages 1-21 years. We included children with a diagnosis of SCD who had at least one echocardiogram performed during an outpatient clinic visit at steady-state for screening for pulmonary hypertension. We categorized patients as receiving either transfusion therapy (chronic transfusion for >12 months), SCD modifying therapy (hydroxyurea, voxelotor, crizanlizumab, or L-glutamine), or no therapy. Patients on both transfusion and a SCD modifying therapy were defined as being on transfusion therapy. We defined albuminuria as patients with two of three consecutive albumin creatinine ratios ≥30mg/g measured during standard clinic visits. Cerebrovascular disease was defined as having either 1) an overt stroke, 2) silent cerebral stroke defined by MRI, 3) vasculopathy by abnormal magnetic resonance angiography (MRA), 4) transient ischemic event defined by stroke symptoms that resolved within 24 hours, or 5) abnormal TCD (>200 cm/second). We performed bivariate analysis utilizing t-test (or Kruskal-Wallis test) and Cochran-Mantel-Haenszel test for continuous and categorical variables respectively. To determine the association between elevated TRV and albuminuria, and elevated TRV and CVD, logistic regression was fit and adjusted for variables that were significant in the bivariate analysis. Using the TRV <2.5m/sec as the reference group, adjusted odds ratios with their accompanying confidence intervals were calculated for those with albuminuria and those with CVD in separate models. All reported P values were 2-sided. Statistical significance was defined as p<0.05. All analyses were performed using Statistical Analysis Software 9.4, (SAS Institute, Cary, NC). Results: Among 166 children with echocardiography, the prevalence of an elevated TRV was 21%. On bivariate analysis, elevated TRV was significantly associated with persistent albuminuria (OR 2.85 (95% CI: 1.27-6.43) and cerebrovascular disease (OR: 2.46 (95% CI: 1.09-5.53) (Table 1). An elevated TRV was also significantly associated with increased age (15.6 vs 12.9 yrs, p=0.005), lower hemoglobin F% (2.5 vs 4.6%, p=0.01), higher absolute reticulocyte count (0.5 vs 0.4 x 106/ µL p=0.005) and chronic transfusion therapy (p=0.04). After adjusting for age, absolute reticulocyte count, HbF and treatment in a multivariable logistic regression, the associations between elevated TRV with either persistent albuminuria (OR: 1.82 (0.71 - 4.67)) and cerebrovascular disease (OR: 1.15 (0.43-3.10)) were no longer statistically significant (Table 1). Our data suggests that with an odds ratio of 2.9 and a prevalence of elevated TRV of 20%, a prospective study of 166 adolescent patients with SCD would be needed to detect the association between elevated TRV and persistent albuminuria with 80% power and 233 children would be needed to detect this difference with 90% power. Conclusion: This data provides additional evidence that around 20% of pediatric patients have an elevated TRV and those patients may also have an increased risk for developing chronic kidney disease and cerebrovascular disease. A larger, prospective cohort study should be conducted to confirm these findings and determine the role of screening echocardiography in pediatric SCD. At minimum, our data suggests that pediatric patients with known chronic kidney disease or cerebrovascular disease should be screened for cardiac complications. Furthermore, longitudinal studies are needed to evaluate the association between elevated TRV in children with early end-organ complications in adults. Figure 1View largeDownload PPTFigure 1View largeDownload PPT Close modal

IMproving PULmonary hypertension Screening by Echocardiography: IMPULSE

BackgroundThe world symposium on pulmonary hypertension (PH) has proposed that PH be defined as a mean pulmonary artery pressure (mPAP) > 20 mmHg as assessed by right heart catheterisation (RHC). Transthoracic echocardiography (TTE) is an established screening tool used for suspected PH. International guidelines recommend a multi-parameter assessment of the TTE PH probability although effectiveness has not been established using real world data.Study aimsTo determine accuracy of the European Society of Cardiology (ESC) and British Society of Echocardiography (BSE) TTE probability algorithm in detecting PH in patients attending a UK PH centre. To identify echocardiographic markers and revised algorithms to improve the detection of PH in those with low/intermediate BSE/ESC TTE PH probability.MethodsTTE followed by RHC (within 4 months after) was undertaken in patients for suspected but previously unconfirmed PH. BSE/ESC PH TTE probabilities were calculated alongside additional markers of right ventricular (RV) longitudinal and radial function, and RV diastolic function. A refined IMPULSE algorithm was devised and evaluated in patients with low and/or intermediate ESC/BSE TTE PH probability.ResultsOf 310 patients assessed, 236 (76%) had RHC-confirmed PH (average mPAP 42.8 ± 11.7). Sensitivity and specificity for detecting PH using the BSE/ESC recommendations was 89% and 68%, respectively. 36% of those with low BSE/ESC TTE probability had RHC-confirmed PH and BSE/ESC PH probability parameters did not differ amongst those with and without PH in the low probability group. Conversely, RV free wall longitudinal strain (RVFWLS) was lower in patients with vs. without PH in low BSE/ESC probability group (− 20.6 ± 4.1% vs − 23.8 ± 3.9%) (P < 0.02). Incorporating RVFWLS and TTE features of RV radial and diastolic function (RVFAC and IVRT) within the IMPULSE algorithm reduced false negatives in patients with low BSE/ESC PH probability by 29%. The IMPULSE algorithm had excellent specificity and positive predictive value in those with low (93%/80%, respectively) or intermediate (82%/86%, respectively) PH probability.ConclusionExisting TTE PH probability guidelines lack sensitivity to detect patients with milder haemodynamic forms of PH. Combining additional TTE makers assessing RV radial, longitudinal and diastolic function enhance identification of milder forms of PH, particularly in those who have a low BSE/ESC TTE PH probability.

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.

Prognostic meaning of right ventricular function and output reserve in patients with systemic sclerosis

BackgroundThe objective of this study was to investigate the prognostic impact of right ventricular (RV) function at rest and during exercise in patients with systemic sclerosis (SSc) presenting for a screening for pulmonary hypertension (PH).MethodsIn this study, data from SSc patients who underwent routinely performed examinations for PH screening including echocardiography and right heart catheterization at rest and during exercise were analysed. Uni- and multivariable analyses were performed to identify prognostic parameters.ResultsOut of 280 SSc patients screened for PH, 225 were included in the analysis (81.3% female, mean age 58.1±13.0 years, 68% limited cutaneous SSc, WHO-FC II–III 74%, 24 manifest PH). During the observation period of 3.2±2.7 (median 2.6) years 35 patients died. Tricuspid annular plane systolic excursion (TAPSE) at rest <18 mm (p=0.001), RV output reserve as increase of cardiac index (CI) during exercise <2 l/min (p<0.0001), RV pulmonary vascular reserve (Δ mean pulmonary artery pressure/Δ cardiac output) ≥3 mmHg/l/min (p<0.0001), peak CI <5.5 l/min/m2 (p=0.001), pulmonary arterial compliance <2 ml/mmHg (p=0.002), TAPSE/systolic pulmonary arterial pressure (sPAP) ratio ≤0.6 ml/mmHg (p<0.0001) and echocardiographic qualitative RV function at rest (p<0.0001) significantly predicted worse survival. In the multivariable analysis TAPSE/sPAP ratio and diffusion capacity for carbon monoxide ≤65% were identified as independent prognostic predictors and had 75% sensitivity and 69% specificity to predict future development of pulmonary vascular disease (PVD) during follow-up.ConclusionsThis study demonstrates that assessment of RV function at rest and during exercise may provide crucial information to identify SSc patients who are at a high risk of poor outcome and for the development of PH and/or PVD.

Four-year survival rate in pulmonary sarcoidosis with extensive pulmonary hypertension screening

BackgroundSarcoidosis is a systemic disease of unknown aetiology with significant morbidity and mortality. The PULSAR study prospectively performed cardiac analysis including systematic pulmonary hypertension screening in sarcoidosis patients newly referred to a tertiary sarcoidosis center. In this manuscript we studied the four-year mortality of this population. Methods and main findingsBetween august 2015 and October 2017, 399 patients (58% male, mean age 49.4 years, 90.5% Caucasian) were included and followed for a mean period of 4.3±0.7 years. In total, 10 patients had died at the time of analysis. 1-, 2-, 3- and 4-year survival rate was 100% (n=399), 99.0% (n=399), 98.2% (n=399) and 94.6% (n=276). Most patients died of respiratory failure, other causes were heterogeneous including cardiac, neurological and non-sarcoidosis origin. A low CPI score or modified Walsh score was associated with higher mortality, similar for high PH probability on echocardiography and elevated right ventricular systolic pressure. ConclusionThis study highlights that elevated RVSP and presence of PH on echocardiography and progression of fibrotic disease with subsequent decline in pulmonary function test are important factors for mortality in sarcoidosis patients.

Chest X-ray features facilitate screening for pulmonary hypertension caused by fibrosing mediastinitis

Misdiagnosis and underdiagnosis of pulmonary hypertension caused by fibrosing mediastinitis (PH-FM) are considerably prevalent due to unspecific symptoms and as well as the lack of awareness of this fatal disease. The aim of this study was to evaluate the diagnostic accuracy of the chest X-ray (CXR) for screening the patients with PH-FM from those with pulmonary hypertension (PH). This was a retrospective observational cohort study. The patients with suspected PH were recruited between October 2014 and October 2020. All the clinical data and CXR findings were collected. The sensitivity, specificity, and likelihood ratio of the CXR features were calculated. Logistic regression was used to identify the factors associated with the CXR characteristics and FM and to generate a prediction model. Finally, the diagnostic efficiency of the prediction model was evaluated using nomogram and internal validation. The patients with PH-FM (n = 36) and PH caused by the diseases other than FM (PH-non-FM, n = 62) were enrolled. The CXR features, including atelectasis, pleural effusion, consolidation, nodules, calcification, interlobular septal thickening, and interstitial reticulation, were more prevalent in patients with PH-FM than in those with PH-non-FM (all p < 0.05). Atelectasis had a specificity of 97%, a sensitivity of 50%, and a greater accuracy for diagnosing of PH-FM [area under the curve (AUC) = 0.720; 95% CI: 0.634-0.806] than the other factors did. The combination of tuberculosis, natural logarithmic NT-proBNP (lnBNP), atelectasis, pleural effusion, and prominent right heart border constituted a prediction model to distinguish the PH-FM from the PH-non-FM, with a sensitivity of 91.7% and a specificity of 83.9%. The model demonstrated good prediction performance by showing an AUC of 0.922 (95% CI: 0.861-0.983) in the internal validation. In this study, atelectasis was the most specific and accurate CXR characteristic for identifying PH-FM in the PH patients. The combination of atelectasis, pleural effusion, prominent right heart border, tuberculosis, and lnBNP constituted a prediction model that distinguished the PH-FM patients from the PH-non-FM ones with good performance.

Accuracy of echocardiography and chest tomography for pulmonary hypertension screening in patients awaiting lung transplantation.

ABSTRACTObjective To examine the accuracy of a pulmonary hypertension screening strategy based on a combination of echocardiographic data and tomographic measurements (pulmonary artery diameter and pulmonary artery diameter to ascending aorta diameter ratio) in patients with chronic lung disease referred for lung transplantation.Methods A retrospective observational study with patients with pulmonary emphysema or fibrosis referred for transplantation between 2012 and 2016. Pulmonary hypertension was defined as mean pulmonary artery pressure ≥25mmHg, or between 21 and 24mmHg, with pulmonary vascular resistance >3 Wood units on right heart catheterization. Tomographic measurements were made by two independent radiologists.Results This sample comprised 13 patients with emphysema and 19 patients with pulmonary fibrosis. Of these, 18 had pulmonary hypertension. The level of agreement in tomographic measurements made by radiologists was high (intraclass correlation coefficients 0.936 and 0.940, for pulmonary artery diameter and pulmonary artery diameter to ascending aorta diameter ratio, respectively). Areas under the ROC curves constructed for pulmonary artery diameter, pulmonary artery diameter to ascending aorta diameter ratio, and pulmonary artery systolic pressure as predictors of pulmonary hypertension were 0.540, 0.629 and 0.783, respectively. The sensitivity, specificity and negative predictive value of pulmonary artery systolic pressure ≥40mmHg were 67%, 79% and 65%, respectively. The combined criterion (pulmonary artery diameter to ascending aorta diameter ratio >1 and/or pulmonary artery systolic pressure ≥40mmHg) achieved sensitivity of 72%, specificity of 79%, and a negative predictive value of 69%.Conclusion Measurements of pulmonary artery and ascending aorta diameter were highly reproducible. The association of pulmonary artery and aortic diameter >1 and/or pulmonary artery systolic pressure ≥40mmHg improved the sensitivity and the negative predictive value for pulmonary hypertension screening. This strategy demands prospective validation to assess safety and cost-effectiveness.

Diagnostic accuracy, sensitivity, and specificity of CT pulmonary artery to aorta diameter ratio in screening for pulmonary hypertension in end-stage COPD patients

AimTo determine the diagnostic accuracy of pulmonary artery to aorta ratio in screening for pulmonary hypertension in advanced chronic obstructive pulmonary disease (COPD) patients.MethodsA prospective, diagnostic study was conducted in University Hospital Center Zagreb between January 2015 and March 2018. The study enrolled 100 patients who consecutively underwent chest computed tomography (CT), echocardiographic exam, and right heart catheterization. Two independent observers measured pulmonary artery and ascending aorta diameters. The correlation between the ratio and mean pulmonary artery pressure, measured invasively, was assessed. Patients with echocardiographic signs of moderate systolic or diastolic left ventricular dysfunction were excluded (n = 44).ResultsSixty-six patients (55.5% men), with a median age of 61, were identified. Median forced expiratory volume during the first second (FEV1) was 34 ± 12, FEV1/forced vital capacity <0.70. Patients with and without pulmonary hypertension had pulmonary artery diameter of 36 ± 7 mm and 27 ± 4.6 mm, respectively (P < 0.001). Median pulmonary artery/aorta (PA/A) ratios for patients with and without pulmonary hypertension were 1.05 and 0.81, respectively (P < 0.001). PA/A ratio above 0.95 was an independent predictor of pulmonary hypertension with a specificity of 100% and a sensitivity of 74.51% (area under the curve = 0.882; standard error = 0.041; P < 0.001).ConclusionPA/A ratio as measured on chest CT images can be used as a screening tool instead of echocardiography.

Noninvasive Risk Score to Screen for Pulmonary Hypertension With Elevated Pulmonary Vascular Resistance in Diseases of Chronic Volume Overload

Volume overload promotes pulmonary hypertension (PH) through pulmonary venous hypertension. However, PH with elevated pulmonary vascular resistance (hereafter PH-PVR) may develop in patients with diseases of volume overload, such as heart failure or chronic kidney disease (CKD). In such cases, volume management alone may be insufficient to slow PH progression. An accurate, noninvasive method to screen for PH-PVR in these diseases would facilitate early targeted therapy. We integrated invasive hemodynamic and echocardiography data collected from a single-center clinical cohort and identified patients with CKD or heart failure at the time of assessment. We applied penalized regression to derive a risk score of clinical parameters and echocardiography data associated with PH-PVR and categorized patients into low- (≤5 points), intermediate- (6-10 points), or high-risk (>10 points) groups. Using an internal validation strategy, we evaluated the ability of this risk score to predict PH-PVR and determined the association of this risk classification with 3-year all-cause mortality. Of 2422 patients, 42.4% had PH-PVR. In adjusted analyses, tricuspid regurgitant velocity, right ventricular function, BMI, heart rate, and hemoglobin most strongly associated with PH-PVR. The risk score significantly associated with PH-PVR (age-adjusted odds ratio 11.69 for the highest-risk group, 95% confidence interval [CI] 6.54-20.92). The high-risk group also associated with a significantly higher risk of 3-year all-cause mortality in adjusted analyses (hazard ratio 1.85, 95% CI 1.50-2.27). In conclusion, a noninvasive risk score derived from echocardiography and clinical parameters significantly associated with PH-PVR and all-cause mortality in a cohort of patients with CKD and heart failure.

Y-006. Predicting algorithm for risk of pre-eclampsia using 1st trimester circulating microRNAs

In potential liver transplant candidates, pulmonary vascular diseases, including portopulmonary hypertension (PoPH) and pulmonary venous hypertension (PVH), can be associated with high morbidity and mortality. Although there are clear guidelines regarding management and transplant listing criteria for patients with PoPH, the listing criteria for PVH are not well defined.The aim of this study was to describe and compare the perioperative and postoperative morbidity and mortality associated with PoPH and PVH in patients undergoing liver transplantation.We conducted a retrospective observational study of all patients referred for liver transplantation to our center between 2005 and 2015 who underwent a right heart catheterization (RHC) for screening for pulmonary hypertension as suggested by initial echocardiography. Based on the RHC data, the patients were grouped into no pulmonary hypertension (No PH), PoPH, and PVH categories. In patients who underwent liver transplantation, we recorded vital status intraoperatively and at 30 days and 1-year post-transplant, and we recorded the incidence of postoperative cardiopulmonary and renal complications.Of the 134 patients who underwent RHC as part of the initial transplant evaluation, 50 patients were successfully transplanted. There was 1 intraoperative death in the PoPH group. No significant difference in mortality was noted between the No PH, PoPH, and PVH groups intraoperatively and 30 days after liver transplantation. At 1 year, the survival rates were 100%, 69.2%, and 94.1% in the No PH, PoPH, and PVH groups, respectively. With respect to cardiopulmonary and renal complications, no statistically significant difference was noted among the groups, though there was a trend toward increased post-transplant reversible pulmonary complications in the PVH group.Our findings suggest that the post-transplant outcomes of patients with PoPH and PVH are similar. In light of the growing recognition of diastolic dysfunction and cirrhotic cardiomyopathy in decompensated cirrhotic patients at the time of transplant, the issue of pulmonary hypertension related to PVH will gain increasing importance as we assess these patients for transplantation. Therefore, future studies are needed to define evidence based guidelines to determine candidacy for liver transplantation in the context of PVH.