Regurgitant Jet Research Articles

Unrecognized pulmonary arterial hypertension in hospitalized patients.

Pulmonary hypertension (PH) is an underdiagnosed and potentially fatal condition. The utility of screening for PH in hospitalized patients undergoing echocardiography is unknown. The goal of this study was to determine the prevalence of undiagnosed pulmonary hypertension (PH) and probable pulmonary arterial hypertension (PAH) in hospitalized patients undergoing echocardiography for any indication. All hospitalized patients undergoing echocardiography were identified and echocardiographs reviewed prospectively for the presence of a tricuspid regurgitant (TR) jet. Electronic medical records (EMR) of patients with a TR jet ≥ 3m/s were reviewed for identifiable causes of pulmonary hypertension. Patients with no identifiable cause were classified as presumptive World Health Organization (WHO) Group 1 PH (also known as PAH). These PAH patients were compared to other PH patients for baseline demographic characteristics and comorbidities as well as 30-day readmission and mortality. The admitting physicians of patients classified as PH were advised to consider further evaluation including right heart catheterization. We reviewed 4417 consecutive echocardiograms and identified 448 with a TR jet ≥ 3m/s. Of these 448 patients with PH, 47 were identified as "presumptive PAH" and the other 401 as having PH belonging to WHO Groups 2-5. Presumptive PAH represented 1% of screened echocardiograms and 10.5% of those identified to have an elevated TR jet. Of the patients identified as presumptive PAH, 8 underwent further evaluation including a right heart catheterization, where 5 were confirmed to have PAH. Kaplan-Meier analysis revealed 30-day readmission was higher among those classified as PAH. Our data shows that pulmonary hypertension, as defined by TR jet ≥ 3m/s, is frequently encountered in hospitalized patients undergoing echocardiography for any reason. A careful review of echocardiogram findings and clinical history suggested 10.5% of those with PH (and 1% of all screened patients) may meet the criteria for PAH. Considering PH is a fatal condition which is frequently missed, a hospital screening program seems feasible.

Abstract 14185: Targeting Lower Oxygen Saturation Parameters in Premature Infants Does Not Increase Incidence of Pulmonary Hypertension in the Neonatal Intensive Care Unit

Introduction: Lower oxygen saturation targets in preterm infants have been associated with decreased incidence of bronchopulmonary dysplasia (BPD) but increased risk of pulmonary hypertension (PH). Studies have shown that targets of <90% are associated with higher incidence of PH, however data on the optimal saturation target >90% are lacking. In this study, we compared the rate of BPD and PH in two cohorts with saturation targets of 94-98% and 92-97%. We hypothesized that BPD rate would be lower and PH rate would be unchanged at the lower saturation target. Methods: We performed a retrospective cohort study comparing PH and BPD rates among two cohorts of infants born at ≤32 weeks gestation at Brigham and Women’s Hospital: cohort 1 with saturation target of 94-98% (n = 129, July 2017-July 2018), cohort 2 with saturation target of 92-97% (n = 124, July 2018-July 2019). PH was defined by echocardiographic evidence of systolic septal flattening or right ventricular pressure ≥35 mmHg (estimated by tricuspid regurgitant jet velocity or shunt velocity) at gestational age (GA) ≥36 weeks. Comparisons between groups were carried out by Chi-square test, t-test, and multivariable logistic regression. Results: Subjects had a GA of 23-32 weeks; 46% were female. Groups did not differ with respect to GA, sex, or birth weight. There was no difference in rate of PH (2.4% vs. 4.2%, p = 0.12) or BPD (25% vs. 20%, p = 0.31) between cohort 1 and cohort 2. Other clinical parameters were not different between groups, including presence of patent ductus arteriosus, presence of atrial septal defect, use of diuretics, or use of steroids. After controlling for GA, birth weight, sex, and diagnosis of BPD using logistic regression, there was no difference in rate of PH between groups (p = 0.47), but there was a positive association of BPD with PH (OR 3.45; 95% CI, 1.18-10.09; p = 0.02). Conclusions: A lower oxygen saturation target was not associated with a higher rate of PH or lower rate of BPD in preterm infants. The overall rate of PH was much lower than rates previously reported at saturation targets <90%. Given our low incidence of PH, and the lack of a significant difference in rate of PH between groups, a saturation target of 92-97% may be safe while also minimizing need for supplemental oxygen in this population.

The Grndad Registry: Contemporary Natural History Data and an Analysis of Real-World Patterns of Use and Limitations of Disease Modifying Therapy in Adults with SCD

The Grndad Registry: Contemporary Natural History Data and an Analysis of Real-World Patterns of Use and Limitations of Disease Modifying Therapy in Adults with SCD

ETA Receptor Blockade and Vascular Function in Patients with Sickle Cell Disease

Endothelin-1 (ET1) is a potent vasoconstrictor, mitogen, proinflammatory mediator, and a mediator of nociception, whose synthesis is increased by hypoxia, ischemia, shear stress, oxidative stress and reduced nitric oxide (NO) bioavailability, all of which are well documented mechanisms in the pathophysiology of sickle cell disease (SCD). We earlier reported on our studies on the role of ET-1 in SCD, and in transgenic mouse models showed that ETA receptor blockade with ambrisentan provides renal protection by preventing the development of glomerular hyperfiltration and proteinuria (Kasztan et al, 2017, Taylor et al, 2019, Kasztan and Pollock, 2019). ETA receptor blockade was also shown to decrease pulmonary inflammation in response to hypoxia/reoxygenation and LPS (Meiler et al.). Additionally, Lutz et al (2018) showed that pharmacologic inhibition or neuron specific knockdown of ETA receptor in primary sensory neurons of dorsal root ganglia in Berk mice alleviated basal and post-hypoxia evoked pain sensitivity. More recently (Kutlar et al, Blood 2019, 617,130036) we reported the preliminary results of a placebo controlled, double-blind phase I trial of ambrisentan in 26 subjects with SCD (SS and S-ß0 thalassemia) and microalbuminuria, and showed that the drug was well tolerated without any dose limiting toxicities and patients on ambrisentan had a reduction in microalbuminuria, especially in the subgroup who had been on concomitant ACEi/ARB therapy at a stable dose for at least six months (n=6, 286.1 mg/g Cr at baseline to 197.7 mg/g on day 85, p=0.06). FMD measurements showed increased arterial diameter, and improved microvascular function. We analyzed data collected on secondary end points during the phase I study, including Tricuspid regurgitant jet velocity (TRV), Transcranial Doppler (TCD) velocities in distal internal carotid (dICA) and middle cerebral (MCA) arteries, and eGFR (calculated with CKD-Epi formula). Fig. 1a shows eGFRs in ambrisentan and placebo groups; Fig. 1b and Fig. 1c show a breakdown of ambrisentan and placebo groups based on concomitant ACEi/ARB usage. There is a reduction in glomerular hyperfiltration in the ambrisentan group compared to placebo, more pronounced in the subgroup who have not been on ACEi/ARBs. Fig. 1d depicts the change in flow velocity in MCA (TAMMV, Time Averaged Mean Maximal Velocity, cm/sec) between the ambrisentan and placebo groups; similarly, Fig. 1e and 1f show the breakdown of baseline and Day 85 TAMMVs in MCA according to ACEi/ARB usage. A similar trend is also observed in dICA flow (fig. 1g-1i), and suggests a synergistic effect of ETA receptor blockade with ACEi/ARBs in preventing an increase in blood flow velocities. TRV was available on 7 subjects, 6 of which were in the ambrisentan group. Fig. 1j shows the change in TRV in the ambrisentan group, and again is suggestive of a synergistic effect of ambrisentan and ACEi/ARBs in decreasing TRV. These data are clearly very preliminary, and are obtained on a small number of subjects, and as such, do not warrant any conclusions and or speculations. Nevertheless, an interesting observation is the apparent interaction of ETA receptor blockade and ACEi/ARBs in altering vascular flow/function in SCD patients. Decrease in microalbuminuria has been reported with ACEi and ARBs in SCD (Yee et al, 2018), without any effect on GFR. A reduction in hyperfiltration would likely have a significant renoprotective effect, at an earlier stage in the development of sickle nephropathy. ETA receptor antagonists are approved for the treatment of pulmonary arterial hypertension; thus, a decrease in TRV would have a beneficial effect. Increase in blood flow velocity in major intracranial vessels is a well established risk factor for ischemic stroke in children with SCD; however, much less is known in adults. In summary, the effect of ETA receptor blockade with or without ACEi/ARB, may have a significant effect on vascular function/blood flow in different organ systems, and should be explored in a large, multi-center phase II trial, with and without concomitant and or serial ACEi/ARBs, for a longer period of time, with a dose escalation, to further clarify the pleiotropic effects on multiple aspects of SCD pathology. Figure 1 Disclosures Kutlar: NIH/NHLBI (SCDIC): Research Funding; Global Blood Therapeutics: Research Funding, Speakers Bureau; Micelle Biopharma: Consultancy; Novartis Pharmaceuticals: Consultancy, Research Funding; Novo Nordisk: Research Funding; Forma Therapeutics: Research Funding; REACH: Other: DSMB Member; NOHARM: Other: DSMB Member; Bluebird Bio: Other: DSMB Member.

Allogeneic Hematopoietic Stem Cell Transplant Versus Gene Therapy in Sickle Cell Disease: Updated Results from a Systematic Review

Background: Patients with sickle cell disease (SCD) have reduced life expectancy and high morbidity, despite treatments such as hydroxyurea, transfusion, vaccination, and antibiotic prophylaxis. Both allogeneic hematopoietic stem cell transplant (HSCT) and more recently, gene therapy (GT) via ex vivo manipulation of autologous hematopoietic stem cells (HSC), have emerged as curative strategies for SCD. However, both therapies carry significant risks. HSCT and GT have both demonstrated efficacy against SCD in clinical trials, but have not been compared. Here, we present the updated results of a systematic review comparing HSCT and GT in SCD. Methods: We searched the MEDLINE and EMBASE databases for studies reporting HSCT and GT outcomes in SCD. Phase I-III trials, retrospective reviews, and case reports with >1 SCD patient undergoing HSCT or GT were included. All HSC donor types and conditioning regimens were included. References of included studies were screened for additional relevant studies. All titles/abstracts were screened for full text retrieval. Non-English and preclinical studies were excluded. Primary outcomes were overall survival (OS), event-free survival (EFS), graft failure, transplant-related mortality (TRM), graft-versus-host disease (GVHD), and secondary malignancies. Secondary outcomes were end-organ function, acute chest syndrome/vaso-occlusive episodes (ACS/VOE), and health-related quality of life (HRQOL). We used descriptive statistics to analyze data on study characteristics, quality, and effects. Risk of bias was assessed using the Newcastle-Ottawa Scale. Outcome heterogeneity was assessed using sensitivity and subgroup analyses. Publication bias was assessed with funnel plots, where applicable. Results: We identified 949 titles through database searching and 12 titles from references of included studies (Figure). After removal of duplicate citations (n=147), exclusion of ineligible records (n=716), and merging 42 studies with their primary article, 56 studies were included for data extraction. Of these 56 studies, 53 reported outcomes post-HSCT (n=1149) and 3 reported outcomes post-GT (n=28). Length of follow-up was 3718.6 patient-years and 43.4 patient-years in the HSCT and GT groups, respectively. Median age was 11.5 across HSCT studies; 26/28 GT patients were >18 years. HSCT donor source was matched related donor (n=812), matched unrelated donor (n=73), haploidentical (n=127), or mismatched unrelated donor (n=11) among 1023 HSCT patients. Two-year OS and EFS for HSCT was 91% and 87%, respectively. OS and EFS were not reported for GT studies. TRM was 74/1042 (7.1%) over a median of 3 years of observation for HSCT, and 0/28 (0%) over a median of 1.6 years of observation for GT. Secondary malignancies were reported in 4 SCD patients from the HSCT group and 1 SCD patient from the GT group. While the vast majority of HSCT studies reported the absence of ACS/VOE in engrafted patients, the lack of published data on pre- and post-transplant ACS/VOE frequency precluded further analysis. Median annual ACS/VOE episodes decreased from 5.3 to 0 post-transplant in 1 GT study, while a second GT study reported 2 episodes of ACS and 1 VOE post-GT. Fourteen studies (n=375), all from the HSCT group, reported 6 strokes/TIAs out of 2030.5 patient-years of post-transplant follow-up, with 1/6 strokes occurring in engrafted patients. None of the GT studies explicitly commented on stroke as an outcome. Only 1 study quantified changes in tricuspid regurgitant jet velocity post-transplant, and 1 study reported on renal function pre- and post-transplant, both from the HSCT group. There was no change in pulmonary function testing values post-transplant, as reported by 2 HSCT studies. Post-transplant HRQOL was reported for 8 HSCT studies and 0 GT studies, but due to heterogeneity of reporting, meta-analysis could not be performed. Conclusions: While long-term HSCT and early GT data demonstrate regimen efficacy, reporting of other important post-transplant outcomes such as end-organ function, persistence of SCD-related complications, and patient-important outcomes is lacking for both strategies. Small sample sizes and differential reporting of outcomes preclude comparison between subgroups of HSC sources and conditioning regimens, a limitation of our study. We advocate for standardized reporting to better compare outcomes within and between treatment groups. Figure 1 Disclosures Kuo: Celgene: Consultancy; Pfizer: Consultancy, Research Funding; Alexion: Consultancy, Honoraria; Novartis: Consultancy, Honoraria; Bioverativ: Membership on an entity's Board of Directors or advisory committees; Agios: Consultancy, Membership on an entity's Board of Directors or advisory committees; Bluebird Bio: Consultancy; Apellis: Consultancy.

Cardiac evaluation in patients with scleroderma_results from screening of pulmonary hypertension in systemic lupus erythermatosus (SOPHIE) study

Abstract Background Pulmonary hypertension (PH) and cardiac involvement were considered as the important prognostic factors in patients with scleroderma. The purpose of this study is to investigate the PH prevalence and related cardiovascular events in patients with scleroderma who were enrolled for screening of PH in systemic lupus erythermatosus (SOPHIE) study. Methods One hundred and ninety-four patients with scleroderma were enrolled in this study. Clinical characteristics were collected, all of patients were received echocardiography for screening of the probability of PH using tricuspid regurgitation jet velocity and other echocardiographic “PH” signs. Additionally, Blood test, B type natriuretic peptide (BNP), serum autoantibodies, and 6 minutes walk test, as well as electrocardiograph, were performed in those patients. Results Forty (20.6%) patients were divided into intermediate/ high probability PH group (IHPH), 154 Patients were divided into none/ low probability PH group (NLPH). Patients in IHPH group were older compared to NLPH group and has a higher proportion of males (20% VS 7.8%.NLPH), dermatomyositis (10% VS 1.9%.NLPH), and stage 3 chronic kidney disease (7.5% VS 1.3%.NLPH), with a shorter 6MWT distance (390.2±108.3 VS 444.9±88.5.NLPH). B-type natriuretic peptide (BNP) level (181.4±323.8 VS 51.1±61.8. NLPH) and the proportion of BNP level larger than 100 pg/ml (40% VS 10.3%.NLPH) were increased in IHPH group. No significant difference was observed in serum autoantibodies including anti-Scl-70, anti-centromere, anti-RNP, and antiphospholipid antibody between two groups. Thirty-six (18.6%) patients occurred arrythmias, 3 patients (1.6%) with atrial fibrillation and all belong to the IHPH group, patients in IHPH group presented a higher proportion of premature atrial complex (7.5% VS 1.0%.NLPH) and sinus tachycardia (5% VS 0.6%.NLPH), no significant difference was observed in arrythmias in terms of atrial-ventricular blockage or ventricular conduction blockage. Forty patients (20.6%) occurred abnormal electrical activities, the main difference between two groups were lie in T wave abnormality (20% vs 3.9%. NLPH) and P wave high voltage (7.5% VS 0%.NLPH). Receiver operating characteristic (ROC) curve revealed that BNP larger than 100pg/ml to predict PH with sensitivity (52.9%) and specificity (88.2%). Multiple logistic regression analysis indicates age (OR, 1.1; 95% CI, 1.0, 1.10), BNP (OR, 1.0; 95% CI, 1.0, 1.02), diastolic blood pressure (OR, 1.0; 95% CI, 0.91, 0.98) significantly affected right ventricular systolic pressure. Conclusions Patients with scleroderma belonged to IHPH group are relatively older than that of NLPH group and manifested with a higher proportion of males, dermatomyositis, more severely chronic kidney disease, arrhythmias, and abnormal electrical activities. Increased age, BNP levels, and diastolic blood pressure can serve as the predicting factors for pulmonary hypertension in patients with scleroderma. Figure 1 Funding Acknowledgement Type of funding source: None

Risk Factors for Mortality and Circulatory Outcome Among Neonates Prenatally Diagnosed With Ebstein Anomaly or Tricuspid Valve Dysplasia: A Multicenter Study.

BackgroundIn a recent multicenter study of perinatal outcome in fetuses with Ebstein anomaly or tricuspid valve dysplasia, we found that one third of live‐born patients died before hospital discharge. We sought to further describe postnatal management strategies and to define risk factors for neonatal mortality and circulatory outcome at discharge.Methods and ResultsThis 23‐center, retrospective study from 2005 to 2011 included 243 fetuses with Ebstein anomaly or tricuspid valve dysplasia. Among live‐born patients, clinical and echocardiographic factors were evaluated for association with neonatal mortality and palliated versus biventricular circulation at discharge. Of 176 live‐born patients, 7 received comfort care, 11 died <24 hours after birth, and 4 had insufficient data. Among 154 remaining patients, 38 (25%) did not survive to discharge. Nearly half (46%) underwent intervention. Mortality differed by procedure; no deaths occurred in patients who underwent right ventricular exclusion. At discharge, 56% of the cohort had a biventricular circulation (13% following intervention) and 19% were palliated. Lower tricuspid regurgitation jet velocity (odds ratio [OR], 2.3 [1.1–5.0], 95% CI, per m/s; P=0.025) and lack of antegrade flow across the pulmonary valve (OR, 4.5 [1.3–14.2]; P=0.015) were associated with neonatal mortality by multivariable logistic regression. These variables, along with smaller pulmonary valve dimension, were also associated with a palliated outcome.ConclusionsAmong neonates with Ebstein anomaly or tricuspid valve dysplasia diagnosed in utero, a variety of management strategies were used across centers, with poor outcomes overall. High‐risk patients with low tricuspid regurgitation jet velocity and no antegrade pulmonary blood flow should be considered for right ventricular exclusion to optimize their chance of survival.

Associated factors of early-onset pulmonary hypertension and clinical difference between early- and late-onset pulmonary hypertension in Thai systemic sclerosis

Objectives Pulmonary hypertension (PH) is a major cause of death in systemic sclerosis (SSc). Detection of early-onset PH and its associated factors would be helpful for improving patient care. Our aims were to determine the factors associated with early-onset PH and to define the differences between early- and late-onset PH among SSc patients. Methods A cohort study was conducted of 409 adult SSc patients who had followed-up between January 2014 and December 2016. Early-PH is defined when the onset of PH is diagnosed within 5 years of the disease. Logistic regression analysis was applied to determine the factors associated with early-PH. Results In 3409 person-years, we diagnosed 50 cases with PH confirmation by right heart catheterization, of whom 26 were early-PH (incidence 0.7 per 100 person-years; 95%CI:0.5–1.1). Among SSc with early-PH, 69.2% had the diffuse cutaneous SSc subset and the most common PH classification was PH due to interstitial lung disease (18 cases;69.2%). According to a logistic regression analysis, early-PH was associated with a WHO functional class (WHO-FC) II and higher, cardiomegaly according to chest radiography, and tricuspid regurgitation jet maximum velocity (TRVmax)>2.8 m/s with the respective OR of 20.12 (95%CI:1.59–255.35), 7.42 (95%CI:1.35–40.88), and 8.20 (95%CI:1.17–57.64). To contrast, early-PH had a negative association with gastrointestinal involvement (OR 0.08; 95%CI:0.01–0.56). Conclusions Early-PH is prevalent among SSc patients and the most common cause is interstitial lung disease. A poor WHO-FC, cardiomegaly, and a high TRVmax are associated with early-PH. Gastrointestinal involvement is a protective factor for early-PH in SSc.

A CASE OF ACUTE SILICOSIS WITH SEVERE PULMONARY HYPERTENSION

SESSION TITLE: Medical Student/Resident Occupational and Environmental Lung Disease SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: October 18-21, 2020 INTRODUCTION: Silicosis occurs from inhalation of silica dust particles. Acute and accelerated silicosis share nodular formations as seen in chronic silicosis but have granular lipo-proteinaceous collections in the alveolar spaces [1]. These collections impair gas exchange resulting in cyanosis, and ultimately respiratory failure usually exacerbated by respiratory infections. We report a case of a young patient developing acute silicosis from working in a gold mine. CASE PRESENTATION: A 30-year-old African man presented with a 2-week history of worsening shortness of breath associated with productive cough and pleuritic chest pain. The patient was previously diagnosed with progressive massive fibrosis (PMF) and silicosis via lung biopsy at an outside hospital, attributed to a 3-year long silica exposure from gold mining in West Africa. Our patient was initiated on intravenous glucocorticoids, broad spectrum antibiotics, and noninvasive positive pressure ventilation for acute hypoxic and hypercarbic respiratory failure. Chest radiograph revealed extensive bilateral airspace opacities (figure 1). CT-chest showed extensive bilateral perihilar parenchymal opacities with traction bronchiectasis, consistent with PMF (figure 2)(figure 3). Transthoracic echocardiogram showed septal wall abnormalities and a right ventricular systolic pressure of 173 mmHg measured via tricuspid regurgitant jet. The patient’s condition stabilized during hospitalization and the patient was discharged home on prednisone and oxygen supplementation via nasal cannula. Three weeks after discharge, the patient presented again with recurrent acute respiratory failure with a positive viral PCR for RSV requiring intubation and mechanical ventilation. Shortly after intubation, the patient went into cardiac arrest and expired. DISCUSSION: Generally, silicosis presents as a chronic condition but rarely, acute silicosis, most often related to massive exposure, can present within a few years . Lung transplantation is the only definitive treatment of advanced silicosis with studies showing successful lung transplantation with the 6-month, 1-year and 3-year survival rates of 86, 86, and 76 percent respectively [2]. One case report examined initiation of intravenous glucocorticoid therapy followed by oral prednisone which showed transient improvement in chest radiography and pulmonary function tests [3]. Unfortunately, treatment with glucocorticoid therapy was unable to alter the outcome of our patient. Lung transplantation was in the process for the patient but was unable to be obtained. CONCLUSIONS: This case presents a rare case of acute silicosis/PMF with severe pulmonary hypertension, who developed respiratory failure after RSV infection leading to death. Reference #1: Ozkan M, Ayan A, Arik D, Balkan A, Ongürü O, Gümüş S. FDG PET findings in a case with acute pulmonary silicosis. Ann Nucl Med 2009;23:883–886. Reference #2: Singer, J. P., et al. “Survival Following Lung Transplantation for Silicosis and Other Occupational Lung Diseases.” Occup Med (Lond) 62.2 (2012): 134-7. Print. Reference #3: Goodman, George B., et al. “Acute Silicosis Responding to Corticosteroid Therapy.” Chest 101.2 (1992): 366-70. Print. DISCLOSURES: No relevant relationships by Khaled Abu-Ihweij, source=Web Response No relevant relationships by Sung Choi, source=Web Response no disclosure on file for Keith Guevarra; No relevant relationships by John Wisener, source=Web Response

Improved Delineation of Cardiac Pathology Using a Novel Three-Dimensional Echocardiographic Tissue Transparency Tool

Accurate visualization of cardiac valves and lesions by three-dimensional (3D) echocardiography is integral for optimal guidance of structural procedures and appropriate selection of closure devices. A new 3D rendering tool known as transillumination (TI), which integrates a virtual light source into the data set, was recently reported to effectively enhance depth perception and orifice definition. We hypothesized that adding the ability to adjust transparency to this tool would result in improved visualization and delineation of anatomy and pathology and improved localization of regurgitant jets compared with TI without transparency and standard 3D rendering. We prospectively studied 30 patients with a spectrum of structural heart disease who underwent 3D transesophageal imaging (EPIQ system, Philips) with standard acquisition and TI with and without the transparency feature. Six experienced cardiologists and sonographers were shown randomized images of all three display types in a blinded fashion. Each image was scored independently by all experts using a Likert scale from 1 to 5, while assessing each of the following aspects: (1) ability to recognize anatomy, (2) ability to identify pathology, including regurgitant jet origin, (3) depth perception, and (4) quality of border delineation. TI images with transparency were successfully obtained in all cases. All experts perceived an incremental value of the transparency mode, compared with TI without transparency and standard 3D rendering, in terms of ability to recognize anatomy (respective scores: 4.5±1.1 vs 4.1±1.1 vs 3.6±1.1, P<.05), ability to identify pathology (4.1±1.1 vs 3.9±1.2 vs 3.3±1, P<.05), depth perception (4.6±0.7 vs 4.1±0.8 vs 3.2±1.0, P<.05), and border delineation (4.6±0.8 vs 4.1±1.0 vs 3.1±1.1, P<.05). The addition of the transparency mode to TI rendering significantly improves the diagnostic and clinical utility of 3D echocardiography and has the potential to markedly enhance echocardiographic guidance of cardiac structural interventions.

36. Scoliosis surgery normalizes cardiac function in AIS patients

BACKGROUND CONTEXT Spinal deformity in adolescent idiopathic scoliosis (AIS) can increase right atrial and ventricular pressures secondary to restrictive lung disease. Pulmonary hypertension leading to cor pulmonale is the most feared outcome; however, mild pulmonary hypertension in AIS patients has been reported in the past. No study has examined these changes including the improvement of right heart function following scoliosis surgery. PURPOSE To determine if AIS patients, compared to healthy patients, will have a higher incidence of pulmonary hypertension that worsens with increasing curve angle and improves following scoliosis surgery. STUDY DESIGN/SETTING Retrospective review. PATIENT SAMPLE A total of 202 healthy AIS patients undergoing posterior spinal fusion from 2009-2013 at a single institution (Group 1), 179 healthy age-matched control patients, who did not undergo spinal deformity correction, at the same institution (Group 2) and 47 AIS patients from with elevated preoperative tricuspid regurgitant jet velocity (TRV) undergoing posterior spinal fusion (PSF). OUTCOME MEASURES Right ventricular systolic pressure (RVSP) value, TRV value, aortic root dimensions, PFT. METHODS Cobb angle, 2D-echo signs of structural heart disease, aortic root dimensions, tricuspid regurgitant jet velocity (TRV), PFTs, ABG, and patient demographics were reviewed. Right ventricular systolic pressure (RVSP) was estimated using Bernoulli's equation [4(TRV)2] and right atrial pressure. RVSP ≥ 36mmHg is a surrogate marker for pulmonary hypertension. Logistic regression was used to assess for differences in TRV between groups. RESULTS Mean preoperative RVSP was significantly elevated in AIS patients (26.9±0.49; p CONCLUSIONS This study found that 21% of patients with AIS had elevated TRV while age-matched controls had no TRV abnormalities. Additionally, RVSP measurements demonstrated mild pulmonary hypertension in AIS patients. These abnormal values normalized postoperatively, indicating the benefits of scoliosis surgery on cardiac function in AIS. FDA DEVICE/DRUG STATUS This abstract does not discuss or include any applicable devices or drugs.

Echocardiography Screening for Latent Rheumatic Heart Disease: What Can We Do in Indonesia?

Rheumatic heart disease (RHD), a sequela of acute rheumatic fever (ARF), is a preventable disease but remains a significant health problem, especially in developing countries. It causes disability, poor quality of life, early mortality, and national economic burden. The World Heart Federation (WHF) aimed to achieve a 25% reduction in premature deaths from ARF and RHD among individuals aged <25 years by 2025. Primordial and primary prophylaxis of RHD is aimed to prevent the occurrence of ARF, while the goal of secondary and tertiary prophylaxis is to limit the progression and reduce the consequences of RHD. Early recognition of RHD is important for early prophylaxis strategies to inhibit any progression to advanced stages. In 2012, WHF introduced the latest echocardiographic criteria to recognize the early stage of RHD. This includes the evaluation of pathological regurgitation jet and morphological features of RHD based on 2D, color, and spectral Doppler criteria. In remote areas, portable echocardiography is preferable for RHD screening. Previous portable devices were only capable of producing 2D and color images. Hence, a simplified echocardiographic criterion without spectral Doppler evaluation is needed in selected areas. Indonesia is a developing country, an archipelago with a population of over 250 million. Currently, there are no data on ARF incidence and RHD prevalence nationwide. The only data available are the number of patients in advanced stages who came to referral centers for further management. The screening program has to be introduced in Indonesia as part of national RHD prophylaxis.

Color Doppler Splay: A Clue to the Presence of Significant Mitral Regurgitation

The authors describe a previously unreported Doppler signal associated with mitral regurgitation (MR) as imaged using transthoracic echocardiography. Horizontal "splay" of the color Doppler signal along the atrial surface of the valve may indicate significant regurgitation when the MR jet otherwise appears benign. Splay was defined as a nonphysiologic arc of color centered at the point at which the MR jet emerges into the left atrium. The authors present a series of 10 cases of clinically significant MR (moderately severe or severe as defined by transesophageal echocardiography) that were misclassified on transthoracic echocardiography as less than moderate. The splay signal was present on at least one standard transthoracic view in each case. To better characterize the splay signal, two groups were created from existing clinically driven transthoracic echocardiograms: 100 consecutive patients with severe MR and 100 with mild MR. Splay was present in the majority of severe MR cases (81%) regardless of vendor machine, ejection fraction, or MR etiology. Splay was particularly prevalent among patients with wall-hugging jets (28 of 30 [93%]). In patients with mild MR, splay was present less often (16%), on fewer frames per clip, and had smaller dimensions compared with severe MR. Color scale did not differ between subjects with and those without splay, but color gain was higher when splay was present (P=.04). Machine settings were further explored in a single subject with prominent splay: increasing transducer frequency reduced splay, while increasing color gain increased it. The authors describe a new transthoracic echocardiographic sign of MR. Horizontal splay may be a clue to the presence of severe MR when the main body of the jet is out of the imaging plane. Splay is likely generated as a side-lobe artifact due to a high-flux regurgitant jet.

Role of Ultrasound-Guided Evaluation of Dyspnea in the Coronavirus Disease 2019 Pandemic

Role of Ultrasound-Guided Evaluation of Dyspnea in the Coronavirus Disease 2019 Pandemic

Novice Physician Ultrasound Evaluation of Pediatric Tricuspid Regurgitant Jet Velocity

IntroductionPulmonary hypertension, associated with high mortality in pediatric patients, is traditionally screened for by trained professionals by measuring a tricuspid regurgitant jet velocity (TRJV). Our objective was to test the feasibility of novice physician sonographers (NPS) to perform echocardiograms of adequate quality to exclude pathology (defined as TRJV > 2.5 meters per second).MethodsWe conducted a cross-sectional study of NPS to assess TRJV by echocardiogram in an urban pediatric emergency department. NPS completed an educational course consisting of a didactic curriculum and hands-on workshop. NPS enrolled a convenience sample of patients aged 7–21 years. Our primary outcome was the proportion of echocardiograms with images of adequate quality to exclude pathology. Our secondary outcome was NPS performance on four image elements. We present descriptive statistics, binomial proportions, kappa coefficients, and logistic regression analysis.ResultsEight NPS completed 80 echocardiograms. We found 82.5% (95% confidence interval [CI], 74.2–90.8) of echocardiograms had images of adequate quality to exclude pathology. Among image elements, NPS obtained a satisfactory, apical 4-chamber view in 85% (95% CI, 77.1–92.9); positioned the color box accurately 65% (95% CI, 54.5–75.5); optimized TRJV color signal 78.7% (95% CI, 69.8–87.7); and optimized continuous-wave Doppler in 55% (95% CI, 44.1–66.0) of echocardiograms.ConclusionNPS obtained images of adequate quality to exclude pathology in a majority of studies; however, optimized acquisition of specific image elements varied. This work establishes the basis for future study of NPS assessment of TRJV pathology when elevated pulmonary pressures are of clinical concern.

Outcomes of Totally Endoscopic Beating-Heart Tricuspid Repair in Redo Cardiac Surgery

The role of endoscopic surgery in treating late severe tricuspid regurgitation after cardiac surgery has not been well investigated. The aim of this study was to evaluate the outcomes of a combination of a beating-heart, minimally invasive approach and a leaflet-augmentation technique in treating tricuspid regurgitation after cardiac surgery. This was a retrospective cohort study. From January 2015 to July 2018, patients undergoing reoperative tricuspid valve repair with a totally endoscopic approach were enrolled. Procedures were performed on beating hearts with normothermic cardiopulmonary bypass (CPB). A total of 43 adults (mean age 53.4±11.4 yr; 9 men) met the inclusion criteria. The interval between prior cardiac surgery and current tricuspid repair was 17.6±6.5 years. Ten (10) patients had previous tricuspid repair and concomitant previous cardiac surgery. In the current endoscopic approach, tricuspid repair techniques included 38 leaflet augmentations, 38 annular ring placements, five artificial chordae, one cleft closure, five commissure recreations, and eight papillary muscle relaxations. Mean CPB time, median ventilation time, and median hospital stay were 128.5±54.2 minutes, 20.5 hours (range, 6-436 hrs), and 7 days (range, 4-56 d), respectively. There were only three in-hospital deaths and no follow-up mortality. The regurgitant jet area was decreased from 21.5±12.1 cm2 preoperatively to 2.4±2.2 cm2 postoperatively (p<0.001). In patients with previous tricuspid repair, although the technique of valvuloplasty seems more complex, CPB time, procedure time and hospital stay were not longer than in patients who did not have previous tricuspid repair. Beating-heart, video-assisted, minimal access tricuspid repair after previous cardiac surgery is feasible, reproducible, and associated with low mortality, even in patients who have had previous tricuspid repair.

Diagnostic value of mitral regurgitant jet volume in the assessment of mitral regurgitation severity by general imaging three-dimensional quantification

Accurate quantification of mitral regurgitation (MR) severity is critical for appropriate clinical decision making regarding surgical intervention. General imaging three-dimensional quantification (GI3DQ) method allows for direct measurement of mitral regurgitant jet volume (MRJvol) with the help of three-dimensional (3D) color flow Doppler imaging. The aim of this study was to evaluate diagnostic value of MRJvol by GI3DQ for MR grading severity, using the guideline recommended integrated approach as a reference. The study included ninety-seven patients with varying degree of MR, and all MR cases were divided into central MR group (n = 44) and eccentric MR group (n = 53). The MRJvol was measured by GI3DQ. The severity of MR was graded on the basis of recommended integrated approach as mild, moderate, or severe. As assessed by receiver operating characteristic analysis, MRJvol by GI3DQ at a cutoff value of 43.4ml yielded 76.9% of sensitivity and 86.9% of specificity to differentiate moderate from severe MR in all cases, a cutoff value of 47.5ml yielded 98.9% of sensitivity and 94.4% of specificity to differentiate moderate from severe MR in central MR, and a cutoff value of 40.7ml yielded 80.0% of sensitivity and 78.6% of specificity to differentiate moderate from severe MR in eccentric MR. MRJvol measured by GI3DQ could assess MR severity, especially in central MR group, which has higher sensitivity and specificity to differentiate moderate from severe MR.

New Model for Non-Invasive Echocardiographic Assessment of Pulmonary-Capillary Wedge Pressure in Patients With Aortic and Mitral Regurgitation

The pulmonary-capillary wedge pressure (PCWP) is a central parameter of cardiac function. In this work, we propose an equation based on a simple physical model, which should be applicable to any class of patient, provided they present some level of aortic and mitral regurgitation. We modeled the left cardio-circulatory system as three chambers placed in a series as follows: left atrium (LA), left ventricle (LV), and aorta (A). Starting from the principle of energy conservation, knowing pressure gradient between A and LV (transaortic gradient) and between LV and LA (trans mitral gradient), we can measure blood pressure in A with a non-invasive central blood pressure (BP) analysis device and then we can derive pressure in LA. With the model described, we derive the equation PCWP ≈ {[(systolic central BP + 4 maximal aortic valve outflow velocity2) − 4 maximal mitral valve regurgitation jet velocity2] + [(diastolic central BP − 4 telediastolic aortic valve regurgitation jet velocity2) + mean mitral valve inflow gradient]} / 2. Herein, we propose a new model for PCWP determination using Doppler echocardiography and a non-invasive central blood pressure (BP) analysis device. One of the main advantages of the method we proposed is that, given it is based on a simple physical model instead of multilinear regression, it should be applicable to any class of patient, provided they present some level of aortic and mitral regurgitation. Further studies are needed for its validation by parallel analysis using invasive methods.

The Assessment of Myocardial Strain by Cardiac Imaging in Healthy Infants with Acute Bronchiolitis: A Systematic Review and Meta-Analysis.

This study aims to systematically review the incidence of myocardial strain detected by echocardiography in previously healthy infants with acute bronchiolitis and its role as a predictor for adverse outcomes in this setting. Methods: Pubmed/Medline, Excerpta Medica Data Base (EMBASE), and Cochrane Library were searched in April 2020 to identify original observational prospective studies that systematically performed echocardiography for the screening of myocardial strain in healthy infants with acute bronchiolitis. Pooled estimates were generated using random-effects models. Heterogeneity within studies was assessed using Cochran’s Q and I2 statistics. Funnel plots and Egger´s regression method were constructed to evaluate publication bias. Sensitivity analyses were also conducted to evaluate potential sources of heterogeneity. Results: After a detailed screening of 305 articles, a total of 10 studies with 395 participants (mean of 40 participants per study) was included. Five of them were classified as high-quality studies. Up to 28% of cases presented adverse outcomes. The echocardiographic screening for myocardial strain was performed within the first 24 h of admission in 92% cases. Tissue Doppler imaging and Speckle-Tracking echocardiography were performed only in 20% of cases. The presence of pulmonary hypertension was evaluated with methods different from the tricuspid regurgitation jet in 64% of cases. Seven studies found some grade of myocardial strain with a pooled incidence of 21% (CI 95%, 11–31%), in the form of pulmonary hypertension (pooled incidence of 20% (CI 95%, 11–30%)), and myocardial dysfunction (pooled incidence of 5% (CI 95%, 1–9%)). The presence of these echocardiographic alterations was associated with adverse outcomes (pooled relative risk = 16; CI 95%, 8.2–31.5). After a subgroup analysis based on the echocardiographic techniques used, no significant heterogeneity across the studies was observed. There was no evidence of publication bias when assessed by Egger´s test. Cardiac biomarkers to assess myocardial strain were used in five studies. Only N-terminal-pro-brain natriuretic peptide accurately predicted the presence of myocardial strain by echocardiography. Conclusions: Myocardial strain is not infrequent in previously healthy infants with acute bronchiolitis, and it could be present at the early stages of the disease with prognostic implications. There is a need for sufficiently powered prospective studies with a similar methodology, preferably employing advanced imaging techniques, to conclusively address the usefulness of the assessment of myocardial strain in this setting.

Diastology: 2020-A practical guide.

Left ventricular (LV) diastolic function can be most conveniently assessed by echocardiography which provides reliable assessments of LV structure and function. Most patients with structural heart disease have variable degrees of myocardial dysfunction. LV structural changes as pathologic hypertrophy and systolic functional abnormalities as depressed LV long-axis systolic function are associated with diastolic dysfunction. The recognition of structural abnormalities and abnormal LV long-axis function as indices of diastolic dysfunction is an important difference between 2016 and 2009 guidelines. In addition, there are other Doppler findings indicative of diastolic dysfunction and abnormally elevated LV filling pressures. In the absence of clinical, 2D echocardiographic, and specific Doppler indices of diastolic dysfunction, mitral annulus early diastolic velocity (e'), left atrium (LA) maximum volume index, peak velocity of tricuspid regurgitation jet by continuous-wave Doppler, and ratio of mitral inflow early diastolic velocity to e' velocity can be used to draw inferences about LV diastolic function. In the presence of diastolic dysfunction, mean LA pressure and grade of diastolic dysfunction should be determined. When LA pressure at rest is normal, it is reasonable to proceed to diastolic stress testing in an attempt to identify patients with dyspnea due to heart failure. There are specific algorithms recommended in patients with atrial fibrillation, moderate or severe mitral annular calcification, and noncardiac pulmonary hypertension.