Higher Risk Of Pulmonary Hypertension Research Articles

Association of Pulmonary Artery Pressures With Mortality in Adults With Reduced Left Ventricular Ejection Fraction

BackgroundThe independent effect of pulmonary hypertension (PHT) severity on mortality in those with reduced left ventricular ejection fraction (LVEF) is not well known. ObjectivesThe authors aimed to examine the prognostic impact of increasingly elevated pulmonary pressures in a large clinical cohort of adults with reduced LVEF. MethodsThe authors analyzed data from the National Echocardiography Database of Australia, a large clinical registry linking routine echocardiographic investigations to mortality. In 23,675 adults with a recorded tricuspid regurgitation peak velocity (TRV) and reduced LVEF (<50%), the authors evaluated the relationship between conventional thresholds of increasing risk of PHT and mortality during median follow-up of 2.9 years (Q1-Q3: 1.0-5.4 years). ResultsMean age was 70 ± 15 years, and 7,498 (31.7%) individuals were female. Overall, 8,801 (37.2%) had normal (TRV <2.5 m/s), 7,061 (29.8%) had borderline (2.5-2.8 m/s), 5,676 (24.0%) intermediate (2.9-3.4 m/s), and 2,137 (9.0%) individuals had high-risk PHT (>3.4 m/s). With increasing risk of PHT, 1- and 5-year actuarial mortality increased from 13.3% and 43.8% to 41.5% and 81.4%, respectively (P < 0.0001) from normal to severely elevated TRV. The adjusted HR of mortality increased by 1.31-fold (95% CI: 1.23-1.38), 1.82-fold (95% CI: 1.72-1.93), and 2.38-fold (95% CI: 2.21-2.56) in those with borderline, intermediate, and high risk of PHT respectively, compared with normal TRV. Further analyses suggested a distinctive threshold with a TRV reached >2.41 m/s (adjusted HR: 1.18 [95% CI: 1.04-1.33]). ConclusionsThe authors demonstrate the prevalence and negative prognostic impact of increasingly elevated TRV levels in individuals with reduced LVEF, with a threshold for mortality lying within the range of “borderline risk” PHT.

Связь системного воспаления с тяжестью обструктивного апноэ сна у мужчин пожилого возраста с хронической обструктивной болезнью легких

The combination of obstructive sleep apnea syndrome (OSA) and chronic obstructive pulmonary disease (COPD) is characterized by deep nocturnal hypoxemia and a higher risk of pulmonary hypertension. The study of the participation of blood cellular elements and indices of cellular reactivity in the inflammatory response in this comorbidity is relevant today. The purpose was to study the severity of systemic inflammation in elderly patients with chronic obstructive pulmonary disease depending on the obstructive sleep apnea syndrome severity. We examined 126 men (aged 67.3 ± 3.4 years) diagnosed with COPD 1–2 low risk of exacerbations. Depending on the presence of OSA, the patients were divided into 2 groups: group 1 — 32 people without OSA, group 2 — 94 people with OSA. Symptoms were assessed using the modified Medical Research Council (mMRC) dyspnea scale and the COPD assessment test (CAT). The severity of OSA syndrome was assessed by the frequency of respiratory events: mild – from 5 to 15 per hour; moderate — from 15 to 30 per hour; severe — more than 30 per hour. The indices were calculated: quantitative ratio of neutrophils (N) and lymphocytes (L) — NLR = N/L; ratio of platelets (P) to lymphocytes PLR = P/L, systemic inflammatory index (SII) = platelet × neutrophils / lymphocytes. It was found that as the severity of OSA increased, chronic systemic inflammation increased, which was determined by the ratio of neutrophils, lymphocytes, and platelets (NLR, PLR, SII).

Pulmonary vascular dysfunction in systemic sclerosis

PurposeThis study aimed to determine if any cases of pulmonary arterial hypertension (PAH) were missed using routine screening of systemic sclerosis (SSc). We assessed the potential benefits of adding the DETECT algorithm and invasive hemodynamic exercise testing into the local screening process in a Danish single-center cohort without known PAH. MethodsSeventy-three SSc patients (aged 60 ± 10 years; 56 (77%) females) underwent echocardiography. Among them, 24/73 had an elevated STEP 2 score of whom 3 had a high risk of pulmonary hypertension on echocardiographic assessment. Four patients declined right heart catheterization. Twenty patients (aged 62 ± 10 years, 13 (65%) females) underwent invasive haemodynamic assessment at rest. Nineteen patients participated in invasive haemodynamic exercise testing. ResultsAt rest, 3/20 patients met the 2022 ESC guidelines for PAH (mean pulmonary arterial pressure (mPAP) > 20 mmHg, pulmonary arterial wedge pressure (PAWP) ≤ 15 mmHg, and pulmonary vascular resistance (PVR) > 2 Wood units). According to the 2015 ESC guidelines, only 1/19 patients had PAH. During exercise, 18/19 experienced a mPAP of > 30 mmHg, with the majority (13/19) showing an abnormal pulmonary pressure-flow relationship > 3 mmHg/L/min. Interestingly, seven out of nine patients with no pulmonary hypertension (PH) at rest had exercise-induced pulmonary hypertension. One-quarter of the patients had pulmonary hypertension associated with left ventricular dysfunction (Group 2 PH, WHO classification). ConclusionIn a Danish cohort of 73 SSc patients already undergoing bi-annual PAH screening, applying the DETECT algorithm identified three patients meeting the 2022 ESC/ERS guideline criteria for PAH at rest, one of whom demonstrated increased PAWP with exercise. However, many patients exhibited an abnormal pulmonary vascular response to exercise and/or signs of Group 2 PH.

Symptom-based portopulmonary hypertension screening questionnaire in Japanese patients with chronic liver disease.

As the exact prevalence of portopulmonary hypertension (PoPH) and the etiology of chronic liver disease (CLD) remain unknown, the present study aimed to clarify these points in Japanese patients with CLD using symptom-based questionnaire screening. Patients with CLD were asked to complete an eight-item written questionnaire on pulmonary hypertension (PH) symptoms. If at least one item response was "yes," the patient was offered ultrasonic echocardiography (UCG). Patients identified as having an intermediate or high risk of PH by UCG were referred to a cardiologist for further evaluation, whereby a definitive diagnosis of PoPH was made using right heart catheterization (RHC) findings. A total of 1111 patients with CLD completed the survey. Of the 566 symptomatic patients with at least one question answered as "yes," approximately half agreed to undergo UCG (n = 267). Compared with asymptomatic patients, symptomatic patients were significantly older, predominantly female, and more frequently exhibited cirrhosis. Based on UCG findings, 228, 12, and 8 patients had a low, intermediate, or high risk for PH, respectively. Intermediate-/high-risk patients showed significantly more advanced disease progression status than low-risk patients. The frequencies of answer to the eight questions were comparable. Ultimately, three patients were diagnosed as having PoPH (1.1% of UCG cases), one with underlying hepatitis C virus (HCV) infection and two with primary biliary cholangitis (PBC). This symptom-based PoPH screening study clarified the prevalence of PoPH in CLD patients according to a PH symptom questionnaire, UCG, and RHC. Patients with HCV and PBC may have a higher risk of PoPH.

Sedation Prior to Intubation at Birth in Infants with Congenital Diaphragmatic Hernia: An International Survey on Current Practices

Introduction: Infants with congenital diaphragmatic hernia (CDH) are commonly intubated immediately after birth. Consensus on whether to provide sedation prior to intubation in the delivery room is lacking, although avoidance of stress is especially important in this population with high risk of pulmonary hypertension. We aimed at obtaining an overview of local pharmacological interventions and at providing guidance on delivery room management. Methods: An electronic survey was sent to international clinicians in referral centres for prenatal and postnatally diagnosed infants with CDH. This survey addressed demographic information, use of sedation and/or muscle relaxant prior to intubation, and use of pain scales in the delivery room. Results: We received 93 relevant responses from 59 centres. Most centres were from Europe (n = 33, 56%), followed by North America (n = 16, 27%), Asia (n = 6, 10%), Australia (n = 2, 3%), and South America (n = 2, 3%). A total of 19% (11/59) of the centres routinely provided sedation prior to intubation in the delivery room, with midazolam and fentanyl being most often used. Methods of administration varied for all medications provided. Only 5 of 11 centres using sedation reported an adequate sedative effect prior to intubation. Muscle relaxants prior to intubation were used in 12% (7/59) of the centres, although not always in combination with sedation. Conclusion: This international survey shows a substantial variation in sedation practices in the delivery room and scarce use of both sedative agents and muscle relaxants prior to intubation of CDH infants. We provide guidance on developing protocols for pre-intubation medication in this population.

860 TRICUSPID REGURGITATION RELATED TO CARDIAC IMPLANTABLE DEVICES: A NEW ENTITY?

Abstract Background. The incidence of cardiac implantable electronic device (CIED) implantation is currently growing. Patients who develop a moderate to severe CIED-induced tricuspid regurgitation (TR) are at higher risk of pulmonary hypertension, right heart failure (HR) and have higher mortality. Transthoracic echocardiography (TTE) is the most common method for TR grading, while transesophageal echocardiography (TOE) with three-dimensional (3D) imaging is nowadays gaining importance to reconstruct valve morphology and to determine the TR pathophysiology. Case Summary. We present the case of an 83-year-old man with multiple comorbidities, affected by non-ischemic dilated cardiomyopathy, for which a biventricular defibrillator (ICD-CRT) was implanted in 2007 in primary prevention. Over the years, the patient has been a CRT-responder, with partial recovery of the ejection fraction (40% in 2022 versus 25%). In 2018 he had an episode of acute pulmonary oedema and ventricular fibrillation with ICD intervention; during that hospitalization, the right ventricular catheter (EC) was replaced due to dysfunction. In 2022, the patient presented to our hospital for anasarca due to right HF. TTE and TOE showed new onset of right heart dilation and dysfunction with severe TR, which was predominantly caused from lead impingement of the septal tricuspid valve (TV) leaflet and later from secondary dilation of the TV annulus. The patient responded well to medical therapy and was discharged. Discussion. CIED-related TR is getting more relevant since its growing prevalence and its prognostic impact, even if little is known about why some patients develop moderate-to-severe CIED-related TR. Clinicians should be aware of this entity and follow the patients with TR over the time with periodic echocardiograms. TOE and 3D imaging should always be performed to understand the mechanism of the TR and to select the optimal treatment. Currently, no guidelines nor randomized data are available in order to guide treatment in these patients. Figure 1:4-chamber views with and without color Doppler showing the ventricular EC passing the TV through with the presence of peri-catheter TR.Figure 2:trans-gastric TOE xPlane views showing how the ventricular EC determines a restrictive movement of the septal TV leaflet, causing the severe TR to form.

Systemic Sclerosis-Specific Antibodies: Novel and Classical Biomarkers

Disease-specific autoantibodies are considered the most important biomarkers for systemic sclerosis (SSc), due to their ability to stratify patients with different severity and prognosis. Anti-nuclear antibodies (ANA), occurring in subjects with isolated Raynuad’s phenomenon, are considered the strongest independent predictors of definite SSc and digital microvascular damage, as observed by nailfold videocapillaroscopy. ANA are present in more than 90% of SSc, but ANA negativity does not exclude SSc diagnosis: a little rate of SSc ANA negative exists and shows a distinct subtype of disease, with less vasculopathy, but more frequent lower gastrointestinal involvement and severe disease course. Anti-centromere, anti-Th/To, and anti-Topoisomerase I antibodies could be considered as classical biomarkers, covering about 60% of SSc and defining patients with well-described cardio-pulmonary complications. In particular, anti-Topoisomerase I represent a risk factor for development of diffuse cutaneous involvement and digital ulcers in the first 3 years of disease, as well as severe interstitial lung disease (ILD). Anti-RNA polymerase III is a biomarker with new clinical implications: very rapid skin thickness progression, gastric antral vascular ectasia, the occurrence of synchronous cancers, and possible association with silicone breast implants rupture. Moreover, novel SSc specific autoantibodies have been globally described in about 10% of “seronegative” SSc patients: anti-elF2B, anti-RuvBL1/2 complex, anti-U11/U12 RNP, and anti-BICD2 depict specific SSc subtypes with severe organ complications. Many autoantibodies could be considered markers of overlap syndromes, including SSc. Anti-Ku are found in 2–7% of SSc, strictly defining the PM/SSc overlap. They are associated with synovitis, joint contractures, myositis, and negatively associated with vascular manifestation of disease. Anti-U3RNP are associated with a well-defined clinical phenotype: Afro-Caribbean male patients, younger at diagnosis, and higher risk of pulmonary hypertension and gastrointestinal involvement. Anti-PM/Scl define SSc patients with high frequency of ILD, calcinosis, dermatomyositis skin changes, and severe myositis. The accurate detection of autoantibodies SSc specific and associated with overlap syndromes is crucial for patients’ stratification. ANA should be correctly identified using indirect immunofluorescent assay and a standardized way of patterns’ interpretation. The gold-standard technique for autoantibodies’ identification in SSc is still considered immunoprecipitation, for its high sensitivity and specificity, but other assays have been widely used in routine practice. The identification of SSc autoantibodies with high diagnostic specificity and high predictive value is mandatory for early diagnosis, a specific follow-up and the possible definition of the best therapy for every SSc subsets. In addition, the validation of novel autoantibodies is mandatory in wider cohorts in order to restrict the gap of so-called seronegative SSc patients.

Risk factors for the flare of systemic lupus erythematosus and its influence on prognosis: a single-center retrospective analysis

ObjectivesTo explore the risk factors for systemic lupus erythematosus (SLE) flare and their impact on prognosis.MethodsThe clinical characteristics, laboratory results, and treatment plans of 121 patients with SLE flare were retrospectively analyzed. Ninety-eight SLE outpatients with sustained remission during the same period were selected as controls. Logistic multivariate regression analysis was employed to screen for risk factors for SLE flare.ResultsInfection, thrombocytopenia, arthritis, anti-nucleosome antibodies positive, anti-β2-glycoprotein I (IgG) antibodies positive, and patient’s self-discontinuation of medicine maintenance therapy might be risk factors for SLE flare. Patients who discontinued medicine maintenance therapy by themselves had a significantly higher rate of severe SLE flare than patients with regular medicine maintenance therapy (P = 0.033). The incidence of anemia associated with SLE (P = 0.001), serositis (P = 0.005), and pulmonary hypertension (P = 0.003) in patients who discontinued medicine maintenance therapy were significantly higher than patients with regular medicine maintenance therapy. SLE patients with regular medicine maintenance therapy for less than 3 years had a higher risk of pulmonary hypertension than those with regular medicine maintenance therapy longer than 3 years (P = 0.034).ConclusionsThe accompanying thrombocytopenia, arthritis, anti-nucleosome antibodies positive and anti-β2-glycoprotein I (IgG) antibodies positive at the onset of SLE may affect the prognosis of SLE. Patient’s self-discontinuation of medicine maintenance therapy is the main cause of SLE flare, which may induce severe flare in SLE patients and lead to a significantly higher incidence of pulmonary hypertension.

Flow field around bubbles on formation of air embolism in small vessels

An air embolism is induced by intravascular bubbles that block the blood flow in vessels, which causes a high risk of pulmonary hypertension and myocardial and cerebral infarction. However, it is still unclear how a moving bubble is stopped in the blood flow to form an air embolism in small vessels. In this work, microfluidic experiments, in vivo and in vitro, are performed in small vessels, where bubbles are seen to deform and stop gradually in the flow. A clot is always found to originate at the tail of a moving bubble, which is attributed to the special flow field around the bubble. As the clot grows, it breaks the lubrication film between the bubble and the channel wall; thus, the friction force is increased to stop the bubble. This study illustrates the stopping process of elongated bubbles in small vessels and brings insight into the formation of air embolism.

Association between HIV and incident pulmonary hypertension in US Veterans: a retrospective cohort study

SummaryBackgroundPulmonary hypertension incidence based on echocardiographic estimates of pulmonary artery systolic pressure in people living with HIV remains unstudied. We aimed to determine whether people living with HIV have higher incidence and risk of pulmonary hypertension than uninfected individuals.MethodsIn this retrospective cohort study, we evaluated data from participants in the Veterans Aging Cohort Study (VACS) referred for echocardiography with baseline pulmonary artery systolic pressure measures of 35 mm Hg or less. Incident pulmonary hypertension was defined as pulmonary artery systolic pressure higher than 35 mm Hg on subsequent echocardiogram. We used Poisson regression to estimate incidence rates (IRs) of pulmonary hypertension by HIV status. We then estimated hazard ratios (HRs) by HIV status using Cox proportional hazards regression. We further categorised veterans with HIV by CD4 count or HIV viral load to assess the association between pulmonary hypertension risk and HIV severity. Models included age, sex, race or ethnicity, prevalent heart failure, chronic obstructive pulmonary disease, hypertension, smoking status, diabetes, body-mass index, estimated glomerular filtration rate, hepatitis C virus infection, liver cirrhosis, and drug use as covariates.FindingsOf 21 314 VACS participants with at least one measured PASP on or after April 1, 2003, 13 028 VACS participants were included in the analytic sample (4174 [32%] with HIV and 8854 [68%] without HIV). Median age was 58 years and 12 657 (97%) were male. Median follow-up time was 3·1 years (IQR 0·9–6·8) spanning from April 1, 2003, to Sept 30, 2017. Unadjusted IRs per 1000 person-years were higher in veterans with HIV (IR 28·6 [95% CI 26·1–31·3]) than in veterans without HIV (IR 23·4 [21·9–24·9]; p=0·0004). The risk of incident pulmonary hypertension was higher among veterans with HIV than among veterans without HIV (unadjusted HR 1·25 [95% CI 1·12–1·40], p<0·0001). After multivariable adjustment, this association was slightly attenuated but remained significant (HR 1·18 [1·05–1·34], p=0·0062). Veterans with HIV who had a CD4 count lower than 200 cells per μL or of 200–499 cells per μL had a higher risk of pulmonary hypertension than did veterans without HIV (HR 1·94 [1·49–2·54], p<0·0001, for those with <200 cell μL and HR 1·29 [1·08–1·53], p=0·0048, for those with 200–499 cells per μL). Similarly, veterans with HIV who had HIV viral loads of 500 copies per mL or more had a higher risk of pulmonary hypertension than did veterans without HIV (HR 1·88 [1·46–2·42], p<0·0001).InterpretationHIV is associated with pulmonary hypertension incidence, adjusting for risk factors. Low CD4 cell count and high HIV viral load contribute to increased pulmonary hypertension risk among veterans with HIV. Thus, as with other cardiopulmonary diseases, suppression of HIV should be prioritised to lessen the burden of pulmonary hypertension in people living with HIV.FundingNational Heart, Lung, and Blood Institute (National Institutes of Health, USA); National Institute on Alcohol Abuse and Alcoholism (National Institutes of Health, USA).

Thoracic Visceral Adipose Tissue Area and Pulmonary Hypertension in Lung Transplant Candidates. The Lung Transplant Body Composition Study.

Rationale: Obesity is associated with an increased risk of pulmonary hypertension (PH); however, regional adipose tissue deposition is heterogeneous with distinct cardiovascular phenotypes.Objectives: To determine the association of body mass index (BMI) and thoracic visceral and subcutaneous adipose tissue areas (VAT and SAT, respectively) with PH in patients with advanced lung disease referred for lung transplantation.Methods: We studied patients undergoing evaluation for lung transplantation at three centers from the Lung Transplant Body Composition Study. PH was defined as mean pulmonary artery pressure >20 mm Hg and pulmonary vascular resistance ≥3 Wood units. VAT and SAT were measured on chest computed tomography and normalized to height squared.Results: One hundred thirty-seven (34%) of 399 patients included in our study had PH. Doubling of thoracic VAT was associated with significantly lower pulmonary vascular resistance (β, -0.24; 95% confidence interval [95% CI], -0.46 to -0.02; P = 0.04), higher pulmonary arterial wedge pressure (β, 0.79; 95% CI, 0.32 to 1.26; P = 0.001), and decreased risk of PH (relative risk, 0.86; 95% CI, 0.74 to 0.99; P = 0.04) after multivariate adjustment. Vaspin levels were higher in patients without PH (median, 101.8 vs. 92.0 pg/ml; P < 0.001) but did not mediate the association between VAT and the risk of PH. SAT and BMI were not independently associated with risk of PH.Conclusions: Lower thoracic VAT was associated with a higher risk of PH in patients with advanced lung disease undergoing evaluation for lung transplantation. The role of adipokines in the pulmonary vascular disease remains to be evaluated.

0718 Hypoxemia and Pulmonary Hypertension in Patients with Concomitant Restrictive Ventilatory Defect and Sleep Apnea: The Overlap Syndrome

Abstract Introduction Patients with severe restrictive ventilatory defect (RVD) have hypoxemia and a high risk of pulmonary hypertension (PHTN). Sleep apnea (SA) aggravates the severity of nocturnal desaturation significantly. The aim of this study was to investigate the severity of hypoxemia and prevalence of PHTN in patient with the overlap syndrome (OS) of RVD and SA. Methods Patients referred for both sleep test and spirometry for suspected SA and RVD or obstructive ventilatory defect (OVD) were recruited prospectively from January-December, 2018. SA was determined by an apnea-hypopnea index ≥5/h; mean nocturnal oxygen saturation (meanSaO2), minimum oxygen saturation (minSaO2), saturation lower than 90% (T90) were calculated automatically. RVD was diagnosed in the presence of forced expiratory volume in the first second/forced vital capacity (FVC) &amp;gt;0.7 and FVC&amp;lt;80% predicted value. PHTN was defined by systolic pulmonary arterial pressure (SPAP) ≥ 50mmHg, documented by noninvasive transthoracic echocardiography. Patients with PHTN secondary to extrapulmonary factors were excluded. Results Of 65 patients who completed the investigation, 16 (24.6%) subjects were diagnosed with isolated SA (without RVD or OVD), and 28 (43.1%) subjects were verified to have RVD, in which 22 (78.6%) were diagnosed with OS and 6 (21.4%) presented as isolated RVD. Patients with OS vs. those with isolated RVD had lower minSaO2 (78.3% vs. 88.7%, p=0.003) and meanSaO2 (91.5% vs. 95.8%, p=0.007) but higher T90 (37.2% vs. 0.3%, p=0.009). Patients with OS vs. those with isolated RVD or with isolated SA had higher SPAP (62.6 mmHg vs. 45.3 mmHg or 35.9 mmHg, p=0.334 or p=0.016 respectively). Higher proportion of patients with OS were diagnosed with PHTN than those with isolated RVD or isolated SA (8 [36.4%] vs. 1 [16%] or 1 [6.25%], p=0.360 or p=0.031, respectively). T90 was the only polysomnographic data associated with the prevalence of PHTN after adjusting for age and sex (OR 4.90, 95% CI 1.23-25.56, p=0.023). Conclusion Patients with the OS of RVD and SA had high odds of PHTN, which is probably associated with severe hypoxemia. Further investigation is needed to discern whether therapeutic strategies toward OS might eliminate PHTN in this cohort. Support

Prognostic Value of the Echocardiographic Probability of Pulmonary Hypertension in Patients with Acute Decompensated Heart Failure.

The prognostic value of pulmonary hypertension (PH) estimated by echocardiography in unselected patients with acute decompensated heart failure (ADHF) is poorly studied. Between November 2014 and September 2018, 657 patients were recruited in a prospective registry of ADHF (ClinicalTrials.gov NCT02444416). The probability of pulmonary hypertension was based on European Society of Cardiology (ESC) guidelines for echocardiographic evaluation. The median survival without all-cause mortality or readmission was 7 months. During the median follow-up period of 15 months, there were 450 events including 185 deaths. In multivariate analysis, the hazard ratio (HR) of all-cause mortality or readmission for patients with a high probability of PH was 1.67 (95% CI 1.29–2.17, p < 0.001) as compared to patients with a low or intermediate probability. The left ventricular ejection fraction (LVEF) and right ventricular function (RVF) were not associated with the primary outcome—HR 1.02 (95% CI 0.81–1.29; p = 0.84) and 0.96 (95% CI 0.76–1.23; p = 0.77) respectively. In patients admitted for ADHF, a high probability of PH as evaluated by echocardiography provided the highest independent prognostic value for mortality and readmission, whereas LVEF and RVF were not associated with prognosis. The identification of patients at high risk of PH by non-invasive measurement conveys important prognostic information and may guide management.

Pulmonary Artery Involvement in Patients with Takayasu Arteritis.

This study was performed to explore the clinical manifestations and longterm prognosis in patients with Takayasu arteritis (TA) with pulmonary artery involvement (PAI). The medical records of 194 patients with TA who underwent traditional catheter angiography or computed tomography of pulmonary artery from 2009 to 2016 were retrospectively reviewed. The clinical manifestations, angiographic features, and mortality of 128 patients with TA with PAI were further analyzed. Patients with TA with PAI had a higher risk of pulmonary hypertension (PH) than patients with TA alone (61.7% vs 7.6%, p < 0.001). Patients with PAI and PH more frequently developed dyspnea, hemoptysis, and lower limbs edema (all p < 0.05) than those without PH. Patients with PH also had a higher incidence of bilateral PAI (84.8% vs 34.7%, p < 0.001) and a higher pulmonary artery obstruction index [23 (interquartile range 20-27) vs 10 (6-15), p < 0.001]. Left heart disease was presented in 39 (30.5%) patients with TA with PAI. During the median followup of 38 (21-58) months, 19 and 2 deaths occurred among patients with and without PH, respectively. Among patients with PAI, the mortality rate was 7 times higher in patients with than without PH (p = 0.009). Independent predictors of mortality were the disease duration (p = 0.047), New York Heart Association class III/IV (p = 0.019), right ventricular systolic dysfunction (p = 0.019), and respiratory failure (p = 0.007). Patients with TA with PAI have a higher risk of developing PH than patients with TA alone. The presence of PH in patients with PAI increases the risk of early mortality.

Use of red cell distribution width in a population at high risk for pulmonary hypertension

Pulmonary hypertension (PH) often presents with non-specific symptoms making early diagnosis difficult. Red cell distribution width (RDW) is a parameter routinely reported on an automated complete blood cell count that has been associated with numerous disease states. The purpose of this study was to further evaluate RDW as a biomarker for PH in at-risk populations. In a retrospective, cross-sectional analysis of patients seen at a PH center over 1 year, we examined both patients with PH and patients at risk for but without PH (e.g. systemic sclerosis, [SSc]). We also studied a group of age-and sex-matched, non-diseased controls. Relevant characteristics were compared among the 3 groups using one-way ANOVA. Similar comparisons were made across World Health Organization (WHO) PH groups 1-4. RDW was highest in the PH patients (n = 181), intermediate in the at-risk for PH patients (n = 52), and lowest in matched controls (n = 100) (15.9 ± 2.8 vs 14.8 ± 2.8 vs 14.2 ± 1.1%, respectively; p < 0.0001). There were no significant differences in RDW across WHO PH groups (p = 0.50). SSc patients with PH had significantly higher RDW values compared to SSc patients without PH (16.0 ± 2.2 vs 14.4 ± 1.9%, respectively; p = 0.03). RDW is significantly higher in PH patients, without regard to disease etiology, when compared to age- and sex-matched non-diseased controls. Importantly, RDW is also higher in PH patients compared to at-risk patients, particularly in the SSc cohort. The ease of obtaining RDW as a biomarker may help detect incident PH at earlier stages among patients who are at high risk for development of PH.

Echocardiographic evaluation of prevalence of pulmonary hypertension in β-thalassemia major: A cross sectional study

Introduction: Pulmonary hypertension is a common complication associated with thalassemia syndromes and it may play an important role in the pathogenesis of right ventricle failure. The true prevalence of pulmonary hypertension in patients with thalassemia major remains unclear and has been reported to be between 2 and 79%.Materials and methods: In total, 70 patients with thalassemia major were initially examined. Patients with valvular left heart disease, congenital heart diseases such as atrial septal defect (ASD) and ventricular septal defect (VSD), left heart failure, and chronic embolism were excluded. All patients with thalassemia major underwent echocardiography. Based on tricuspid regurgitation velocity (TRV), the patients were divided into the following three groups: low, medium, and high risk of pulmonary hypertension.Results: The mean age of the subjects was 24 y; 60.6% of the subjects were males and 39.4% of the subjects were females. Overall, three (4.5%) subjects were considered at a high risk of pulmonary hypertension. The mean hemoglobin level in the patients with a high probability of pulmonary hypertension was 8.2 g/dL and that in the patients with a low or medium probability of pulmonary hypertension was 9.1 g/dL. No significant difference was observed between the groups (p = .059).Conclusion: This study showed that, based on new echocardiography criteria, the prevalence of pulmonary hypertension secondary to β-thalassemia was 4.5% and there was no correlation between TRV and the number of received blood units or disease duration.

Predictive value of pulmonary function testing in the evaluation of pulmonary hypertension in sarcoidosis.

Background: In sarcoidosis patients, pulmonary hypertension (PH) is associated with significant morbidity and mortality. Early identification of sarcoidosis-associated pulmonary hypertension (SAPH) has substantial clinical implications. While a number of pulmonary function testing (PFT) variables have been associated with SAPH, the optimal use of PFT’s in screening for SAPH is unknown. Objectives: To examine the predictive value of PFT’s for echocardiographic PH in a cohort of sarcoidosis patients. Methods: We conducted a retrospective cohort study of patients with sarcoidosis from a single center over a period of five years. All consecutive adult patients with a diagnosis of biopsy-proven sarcoidosis (determined by review of the medical chart) who underwent PFT and echocardiographic testing were included. Echocardiographic risk of PH (either intermediate or high) was determined by the presence of echocardiographic PH signs and tricuspid regurgitant jet velocity. Data analysis was performed using multivariate logistic regression analysis with least absolute shrinkage and selection operator. Results: Of the 156 patients included in the study, 42 (27%) met the criteria for echocardiographic PH. Roughly equal proportions met the criteria for intermediate risk (45%) as did for high risk of PH (55%). The percent predicted of diffusion capacity for carbon monoxide (%DLCO) and forced vital capacity (%FVC) were predictive of echocardiographic PH. No other PFT variables outperformed these two markers, and the incorporation of additional PFT variables failed to significantly enhance the model. Conclusions: The %FVC and %DLCO emerged as being predictive of echocardiographic PH in this cohort of biopsy-proven sarcoidosis patients. Potentially reflecting the multifactorial pathogenesis of PH in sarcoidosis, incorporation of other PFT variables failed to enhance screening for PH in this population. (Sarcoidosis Vasc Diffuse Lung Dis 2018; 35: 308-316)

Abstract 19137: Association of Pulmonary Hypertension and Left Ventricular Dysfunction: Impact of Demographics

The association between left ventricular diastolic dysfunction (LVDD) and pulmonary hypertension (PH) is shown in the literature, studies on the linear association between LVDD and PH severity and effect of demographics on this association are lacking. We aimed to investigate the correlation of LVDD with presence and/or severity of PH and to examine whether patient’s demographics modifies this association. Methods: Study population included 2702 randomly selected patients with ejection fraction (EF) ≥ 50%, who presented to the echocardiographic lab at a tertiary care academic center between 2008 and 2011. Diastolic function was assessed based on echocardiographic characteristics. PH was diagnosed if right ventricle systolic pressure (RSVP) was ≥ 40 mmHg. Using logistic regression analysis, the association between diastolic function and presence and severity of PH was examined after adjusting for potential demographic and clinical confounders. Then, we examined the interaction term of demographics (age, sex and combination of age and sex) in regards to the association between LVDD and PH. Results: Among 2702 patients, 1301 (48.4%) were female, 842 (31.2%) had normal LVDD and 694 (25.7%) had PH. Patients with LVDD had higher frequency of PH compared to those with normal diastolic function (28.1% vs. 18.6%, OR: 1.7; 95%CI: 1.4, 2.1; p&lt; 0.0001). RVSP increases at higher stages of LVDD (p&lt;0.0001). Among patients with LVDD, PH was found in 24.7% patients with impaired relaxation, 44.5% of pseudonormal, and 52.5% of those with restrictive. Subgroup analysis showed that in patients 60 (OR: 1.9 vs. 0.9, p&lt;0.0001 and 0.8820, respectively). Besides, the association of LVDD and PH was higher among female patients &lt; 60, compared to males &lt;60 (OR: 2.5 vs 1.5, p&lt;0.0001 and 0.0800, respectively). Sex did not modify the association between LVDD and PH. Conclusion: Patients with LVDD have higher risk of PH, compared to those patients with normal diastolic function. The severity of PH also increases with higher stages of the LVDD. In addition, these findings were more pronounced in middle age females.

Surgical Repair for D-Transposition of the Great Arteries Associated With an Aortopulmonary Window Using the Fenestrated Ventricular Septal Defect Patch as a Safety Adjunct

Transposition of the great arteries with a ventricular septal defect and an associated aortopulmonary window is a rare anatomic combination having a high risk for pulmonary hypertension. Arterial switch with closure of the ventricular septal defect and repair of the aortopulmonary window is the procedure of choice, but a postoperative pulmonary hypertensive crisis is a common occurrence associated with significant morbidity and mortality. This report describes one case of such an anatomic lesion, which was repaired successfully with a fenestrated ventricular septal defect patch as an adjunct to decrease the risk of a postoperative pulmonary hypertensive crisis.

Preliminary Experience in the Use of Preoperative Echo-guided Left Stellate Ganglion Block in Patients Undergoing Cardiac Surgery

Pulmonary arterial (PA) vasoconstriction in cardiac surgery can originate from the action of combined humoral, endothelial, and sympathetic tone changes. The consequence of PA vasoconstriction is pulmonary hypertension (PHT) and, when present after cardiopulmonary bypass (CPB), can predispose to right ventricular dysfunction. Right ventricular dysfunction after CPB is a serious complication with high mortality rates. The extent to which sympathetic blockade could reduce PA vasoconstriction and reduce PHT is unknown. Pharmacologic stellate ganglion block (SGB) has been associated with a reduction in PHT, but its role and mechanism in cardiac surgery have not been described. Thus, the goal of the study was to test the hypothesis that echo-guided left SGB, performed before the induction of general anesthesia, could prevent PA pressure increases during CPB weaning. A prospective cohort study in cardiac surgical patients. A tertiary care university hospital. Forty cardiac surgical patients. A left SGB was performed immediately before the induction of general anesthesia under ultrasound guidance and was compared with matched control patients. Standard hemodynamic and electrocardiographic monitoring was performed, and blood gas samples were drawn at specific predetermined time points for analysis. Rhythm disorders, echocardiographic parameters that included wall motion abnormalities, and biochemical parameters of myocardial ischemia were measured by an observer blinded to the allocated group. Marked improvement in the PaO(2)/F(I)O(2) ratio in the SGB group was observed (mean difference = 77 mmHg, p = 0.0001). There were no differences between the groups in PA pressure over time during the procedure; central venous pressure was higher in the SGB group (p =0.0184). Reductions of right ventricular fractional area change (p = 0.0331) and tricuspid annulus displacement (p = 0.0048) were observed in the SGB group. The CK-MB was 1.5 times higher in the SGB group (p = 0.0211), but no patients developed myocardial infarction. Left SBG was associated with improved oxygenation that could partially explain its mechanism in acute PHT. Further studies are necessary to evaluate the usefulness of this technique in patients with a high risk of PHT during separation from CPB.