Vasodilator Therapy Research Articles

Outcomes of adults with congenital heart disease and pulmonary hypertension in the advanced pulmonary vasodilator era. A regional multicenter registry (RACCA)

Abstract The objective of this study is to evaluate mortality, causes and risk factors of death in adults with congenital heart disease (ACHD) and PHT on Pulmonary vasodilator therapy (PVT). Methods 170 cases with PHT were identified in RACCA (Left heart disease were excluded (n=20)). We examined mortality, causes of death and complications during a mean follow-up (FU) of 7.9+4.6 years. For each patient demographic data, shunt location, clinical PHT group and functional class (FC) were collected. In an unselected sample of 103 patients, O2Sat, NT-proBNP, right ventricle function (RV) and 6-minute-test (6min) were retrospectively reviewed at 2 data collection time points: evaluation at baseline and the most recent data preceding death or at last clinical visit. Survival was assessed with Kaplan-Meier curves and differences between groups using the log-rank test. To look for predictors of death, Cox regression analysis was performed. Results Patients with PHT were predominantly women (61%), aged 45 years (IQR 33–58.5). The distribution of PHT group by underlying defects were 58 Eisenmenger (ES) (Complex 50%) and 45 non-ES (pretricuspid 46.7% (p=0,0002). PHT-pretricuspid defects occurred more in women with unrepaired simple defects and at older ages. In the sample, 85% were commenced on therapy with PVT (5% on initial dual therapy). The treated patients presented worse functional situation, more desaturation of O2 and worse TAPSE. In the group of treatment, 53% required treatment escalation during the FU. Initially, 82% were in sinus rhythm, in FC I-II 53% and in FC III-IV 39%. 10% had moderate-severe RV dysfunction. At the last visit, 38% were on monotherapy, 40% on dual therapy and 22% on triple therapy. FC improved from III-IV to I-II in 35% and 79% remained in FC-I-II without clinical deterioration. The Δ6min was +53±72 mts in those patients alive at the end of FU, and −126±26 mts in exitus cases (p=0,003), but O2 saturation did not change significantly. Over the FU, 29 patients died. The main cause of death was heart failure, followed by sudden death. The event-free survival was 84% at 5 years. Survival was significantly worst for patients presenting with arrhythmias and more advanced FC at initiation of PVT and for those who developed RV dysfunction over FU. At multivariate analysis, syndromic forms, pretricuspid shunt, non-ES physiology, PVT and monotherapy at baseline, FC III-IV and desaturation were independent predictors for death. Conclusions Although a positive clinical response was observed, mortality of PHT in CHD under advanced PVT remains high at mid-term FU. Heart failure is the leading cause of death. In terms of mortality, non ES patients and pretricuspid shunts were less responsive to treatment than patients with ES. Our results may suggest that patients with more advanced disease at the initiation of therapies do not respond properly and support the need for early treatment and initial dual therapy. Funding Acknowledgement Type of funding source: None

Case of Hypogonadotropic Hypogonadism (HH), Obesity, Diabetes Type 2, Hyperlipidemia Associated with BBS Genes

While the surgical stages of single ventricle (SV) palliation serve to separate pulmonary venous and systemic venous return, and to volume-unload the SV, staged palliation also results in transition from parallel to series circulation, increasing total vascular resistance. How this transition affects pressure loading of the SV is as yet unreported.We performed a retrospective chart review of Stage I, II, and III cardiac catheterization (CC) and echocardiographic data from 2001-2017 in all SV pts, with focus on systemic, pulmonary, and total vascular resistance (SVR, PVR, TVR respectively). Longitudinal analyses were performed with log-transformed variables. Effects of SVR-lowering medications were analyzed using Wilcoxon rank-sum testing.There were 372 total patients who underwent CC at a Stage I (median age of 4.4 months, n=310), Stage II (median age 2.7 years, n = 244), and Stage III (median age 7.3 years, n = 113). Total volume loading decreases with progression to Stage III (P< 0.001). While PVR gradually increases from Stage II to Stage III, and SVR increases from Stage I to Stage III, TVR dramatically increases with progress towards series circulation. TVR was not affected by use of systemic vasodilator therapy. TVR, PVR, SVR, and CI did not correlate with indices of SV function at Stage III.TVR steadily increases with an increasing contribution from SVR over progressive stages. TVR was not affected by systemic vasodilator agents. TVR did not correlate with echo-based indices of SV function. Further studies are needed to see if modulating TVR can improve exercise tolerance and outcomes.

Predictors of Cardiovascular Risk in Subjects with Type-2 Diabetes Mellitus: Evidence from a Cross-sectional Study

While the surgical stages of single ventricle (SV) palliation serve to separate pulmonary venous and systemic venous return, and to volume-unload the SV, staged palliation also results in transition from parallel to series circulation, increasing total vascular resistance. How this transition affects pressure loading of the SV is as yet unreported.We performed a retrospective chart review of Stage I, II, and III cardiac catheterization (CC) and echocardiographic data from 2001-2017 in all SV pts, with focus on systemic, pulmonary, and total vascular resistance (SVR, PVR, TVR respectively). Longitudinal analyses were performed with log-transformed variables. Effects of SVR-lowering medications were analyzed using Wilcoxon rank-sum testing.There were 372 total patients who underwent CC at a Stage I (median age of 4.4 months, n=310), Stage II (median age 2.7 years, n = 244), and Stage III (median age 7.3 years, n = 113). Total volume loading decreases with progression to Stage III (P< 0.001). While PVR gradually increases from Stage II to Stage III, and SVR increases from Stage I to Stage III, TVR dramatically increases with progress towards series circulation. TVR was not affected by use of systemic vasodilator therapy. TVR, PVR, SVR, and CI did not correlate with indices of SV function at Stage III.TVR steadily increases with an increasing contribution from SVR over progressive stages. TVR was not affected by systemic vasodilator agents. TVR did not correlate with echo-based indices of SV function. Further studies are needed to see if modulating TVR can improve exercise tolerance and outcomes.

Visceral Adiposity Index And Cardiometabolic Risk

While the surgical stages of single ventricle (SV) palliation serve to separate pulmonary venous and systemic venous return, and to volume-unload the SV, staged palliation also results in transition from parallel to series circulation, increasing total vascular resistance. How this transition affects pressure loading of the SV is as yet unreported.We performed a retrospective chart review of Stage I, II, and III cardiac catheterization (CC) and echocardiographic data from 2001-2017 in all SV pts, with focus on systemic, pulmonary, and total vascular resistance (SVR, PVR, TVR respectively). Longitudinal analyses were performed with log-transformed variables. Effects of SVR-lowering medications were analyzed using Wilcoxon rank-sum testing.There were 372 total patients who underwent CC at a Stage I (median age of 4.4 months, n=310), Stage II (median age 2.7 years, n = 244), and Stage III (median age 7.3 years, n = 113). Total volume loading decreases with progression to Stage III (P< 0.001). While PVR gradually increases from Stage II to Stage III, and SVR increases from Stage I to Stage III, TVR dramatically increases with progress towards series circulation. TVR was not affected by use of systemic vasodilator therapy. TVR, PVR, SVR, and CI did not correlate with indices of SV function at Stage III.TVR steadily increases with an increasing contribution from SVR over progressive stages. TVR was not affected by systemic vasodilator agents. TVR did not correlate with echo-based indices of SV function. Further studies are needed to see if modulating TVR can improve exercise tolerance and outcomes.

Circulating Expression of the Myocardial Infarction (MI)-related Long Noncoding RNAs (lncRNAs) in Type 2 Diabetes (T2D)

While the surgical stages of single ventricle (SV) palliation serve to separate pulmonary venous and systemic venous return, and to volume-unload the SV, staged palliation also results in transition from parallel to series circulation, increasing total vascular resistance. How this transition affects pressure loading of the SV is as yet unreported.We performed a retrospective chart review of Stage I, II, and III cardiac catheterization (CC) and echocardiographic data from 2001-2017 in all SV pts, with focus on systemic, pulmonary, and total vascular resistance (SVR, PVR, TVR respectively). Longitudinal analyses were performed with log-transformed variables. Effects of SVR-lowering medications were analyzed using Wilcoxon rank-sum testing.There were 372 total patients who underwent CC at a Stage I (median age of 4.4 months, n=310), Stage II (median age 2.7 years, n = 244), and Stage III (median age 7.3 years, n = 113). Total volume loading decreases with progression to Stage III (P< 0.001). While PVR gradually increases from Stage II to Stage III, and SVR increases from Stage I to Stage III, TVR dramatically increases with progress towards series circulation. TVR was not affected by use of systemic vasodilator therapy. TVR, PVR, SVR, and CI did not correlate with indices of SV function at Stage III.TVR steadily increases with an increasing contribution from SVR over progressive stages. TVR was not affected by systemic vasodilator agents. TVR did not correlate with echo-based indices of SV function. Further studies are needed to see if modulating TVR can improve exercise tolerance and outcomes.

Thiamine-Responsive Acute Pulmonary Hypertension of Early Infancy (TRAPHEI)-A Case Series and Clinical Review.

Persistent pulmonary hypertension of the newborn (PPHN) is a syndrome of high pulmonary vascular resistance (PVR) commonly seen all over the world in the immediate newborn period. Several case reports from India have recently described severe pulmonary hypertension among infants in the postneonatal period. These cases typically present with respiratory distress in 1–6-month-old infants, breastfed by mothers on a polished rice-based diet. Predisposing factors include respiratory tract infection such as acute laryngotracheobronchitis with change in voice, leading to pulmonary hypertension, right atrial and ventricular dilation, pulmonary edema and hepatomegaly. Mortality is high without specific therapy. Respiratory support, pulmonary vasodilator therapy, inotropes, diuretics and thiamine infusion have improved the outcome of these infants. This review outlines four typical patients with thiamine-responsive acute pulmonary hypertension of early infancy (TRAPHEI) due to thiamine deficiency and discusses pathophysiology, clinical features, diagnostic criteria and therapeutic options.

Safety and efficacy of an integrated endovascular treatment strategy for early hepatic artery occlusion after liver transplantation

BackgroundHepatic artery occlusion (HAO) after liver transplantation (LT) is typically comprised of hepatic artery thrombosis (HAT) and stenosis (HAS), both of which are severe complications that coexist and interdependent. This study aimed to evaluate an integrated endovascular treatment (EVT) strategy for the resolution of early HAO and identify the risk factors associated with early HAO as well as the procedural challenge encountered in the treatment strategy. MethodsConsecutive orthotopic LT recipients (n = 366) who underwent transplantation between June 2017 and December 2018 were retrospectively investigated. EVT was performed using an integrated strategy that involved thrombolytic therapy, shunt artery embolization plus vasodilator therapy, percutaneous transluminal angioplasty, and/or stent placement. Simple EVT was defined as the clinical resolution of HAO by one round of EVT with thrombolytic therapy and/or shunt artery embolization plus vasodilator therapy. Otherwise, it was defined as complex EVT. ResultsTwenty-six patients (median age 52 years) underwent EVT for early HAO that occurred within 30 days post-LT. The median interval from LT to EVT was 7 (6–16) days. Revascularization time (OR = 1.027; 95% CI: 1.005–1.050; P = 0.018) and the need for conduit (OR = 3.558; 95% CI: 1.241–10.203, P = 0.018) were independent predictors for early HAO. HAT was diagnosed in eight patients, and four out of those presented with concomitant HAS. We achieved 100% technical success and recanalization by performing simple EVT in 19 patients (3 HAT+/HAS- and 16 HAT-/HAS+) and by performing complex EVT in seven patients (1 HAT+/HAS-, 4 HAT+/HAS+, and 2 HAT-/HAS+), without major complications. The primary assisted patency rates at 1, 6, and 12 months were all 100%. The cumulative overall survival rates at 1, 6, and 12 months were 88.5%, 88.5%, and 80.8%, respectively. Autologous transfusion < 600 mL (94.74% vs. 42.86%, P = 0.010) and interrupted suture for hepatic artery anastomosis (78.95% vs. 14.29%, P = 0.005) were more prevalent in simple EVT. ConclusionsThe integrated EVT strategy was a feasible approach providing effective resolution with excellent safety for early HAO after LT. Appropriate autologous transfusion and interrupted suture technique helped simplify EVT.

Clinical characteristics and therapeutic outcomes of pulmonary arterial hypertension secondary to congenital portosystemic shunts.

The aim of this retrospective study was to investigate the clinical characteristics and therapeutic outcomes of pulmonary arterial hypertension (PAH) secondary to congenital portosystemic shunts (CPSS). Thirty-three pediatric patients diagnosed in our institution with CPSS between 2012 and 2019 were enrolled in this study. The patients were divided into PAH and non-PAH groups. The PAH group included 15 patients who presented with unexplained PAH when CPSS was diagnosed. Two patients with microangiopathic hemolytic anemia died of right heart failure shortly after diagnosis. One patient received a liver transplant at the age of 4.3 years and showed a mild decrease in pulmonary artery pressure (PAP) 4 years after the operation. Seven patients underwent one-stage shunt closure at a median age of 2.8 years (1.4-13 years). Follow-up examinations, from 1.6 to 4.1 years after intervention, showed marked reduction of PAP in one patient and stabilization of PAH in six others. However, in one patient who underwent two-stage shunt closure, a marked increase in PAP was noted after partial ligation of the shunt. The remaining four patients received only pulmonary vasodilator therapy, and one of them died of right heart failure 12 years after the PAH diagnosis. The non-PAH group included 18 patients without evidence of PAH upon CPSS diagnosis. Shunt closure was carried out in eight of these patients, but one patient subsequently developed PAH after the resolution of hepatopulmonary syndrome.Conclusion: CPSS may be a more likely cause of unexplained PAH in pediatric patients than previously thought. Shunt closure or liver transplantation may prevent the progression of PAH, or even improve it for the majority of CPSS patients.

EFFICACY OF NITROGLYCERINE INFUSION VERSUS SUBLINGUAL NIFEDIPINE IN SEVERE PRE-ECLAMPSIA

Background: Pre-eclampsia is a pregnancy-specific syndrome of reduced organ perfusion secondary to vasospasm and endothelial activation. The incidence of preeclampsia is estimated to range between 2.5 and 5%. An accurate estimate, as well as its global burden, is difficult to obtain, due to lack of data from several countries and standardization of diagnostic criteria. Objective: To compare between the uses of continuous i.v. administration of nitroglycerine versus sublingual nifedipine in the management of women with severe pre-eclampsia who were being managed with controlled plasma volume expansion and MgSO4 loading and maintenance doses. Patients and Methods: Patients were taken from Sayed Galal Hospital. This study was carried out on 100 women with severe pre-eclampsia who were being managed with controlled plasma volume expansion and MgSO4 loading and maintenance doses. Those women were randomly assigned into two equal groups: Nitroglycerine or nifedipine. Both groups were compared as regard maternal adverse effects of vasodilator therapy, mean arterial pressure changes within one hour after vasodilator therapy, post therapy changes in fetal cardiotocography (CTG) and for feto-maternal complications including gestational age of termination of pregnancy, time of maternal intensive care unit (ICU) stay and the need of neonatal ICU. Results: Statistical analysis of the magnitude and time course of maternal hypotensive responses after initiation of the vasodilator therapy showed that, in nitroglycerine group, a statistically significant decrease in both systolic blood pressure (SBP) and diastolic blood pressure (DBP) started just 5 min. after the initiation of nitroglycerine infusion, while in nifedipine group, the significant decrease in SBP and DBP started 15 min. after oral nifedipine. The SBP and DBP measurements in nitroglycerine group were significantly lower than those in nifedipine group. The feto-maternal safety margin observed was similar in the two study groups. Conclusion: IV infusion of nitroglycerine was effective, safe and alternative therapy for severe pre-eclampsia.

EP04 Is this scleroderma lung or COVID-19 lung?

Case report - IntroductionThis is a case of Pakistani female with limited systemic sclerosis and associated mild interstitial lung disease. The lung disease was complicated by SARS-COV-2 related pneumonitis in April 2020 and that led to treatment challenges.She was previously seen in multiple private hospitals and labelled as Rheumatoid arthritis. She was being treated with long term steroids and Methotrexate. After her initial presentation to our Rheumatology services, her diagnosis was correctly revised to Systemic Sclerosis with phenotype of CREST. Her treatment was adjusted to Vasodilators and Mycophenolate due to skin and Lung involvement. Case report - Case descriptionThis is a case of 40-year-old Pakistani female who had been having multiple joint pains since 2010. She also experienced severe Raynaud’s.She presented to our Rheumatology clinic in December 2018. Her symptoms included recurrent digital ulcers, tight and tough skin at fingers and Raynaud’s worse during winter months. Her examination confirmed peripheral cyanosis with multiple digital ulcers with superimposed infection, marked sclerodactyly and calcinosis. She was started on Vasodilator therapy including calcium channel blocker and PDE5 inhibitor due to severity of ulceration. Infection was managed with prolonged course of antimicrobial therapy. Her immunology showed positive anti nRNP/Sm. Anti-centromere and anti Scl 70 were negative. Her condition fit description of CREST (Calcinosis, RP, Oesophageal dysmotility, telangiectasia). Her management included weaning off Methotrexate and reduction in the dose of corticosteroids. In February 2019, Respiratory work up showed normal Chest radiograph, High resolution CT chest showing no significant abnormality and FEV1 82%, FVC 86%, and DLCO 77%. Her PASP was 25mmHg. Overall, her condition remained stable over the course of next year. Her medication included Cellcept, low dose prednisolone, hydroxychloroquine, and Sildenafil. More importantly, Digital ulcers have been well controlled with combined vasodilator therapy. In April 2020, she developed SARS-CoV-2 with mild respiratory symptoms and was admitted to a different hospital. Fortunately, she responded well to ward based supportive and symptomatic treatment with no need for respiratory support. Subsequently, she has seen a different respiratory physician and had repeat imaging of chest which has led to dilemma whether the ground glass opacities in both lungs is due to scleroderma lung or COVID-19 related lung disease. She was given high dose prednisolone by the respiratory physician which has been reduced in rheumatology clinic. The new findings on chest imaging are sequelae of SARS-COV-2.Case report - DiscussionThis case highlights few important points as below: Systemic sclerosis diagnosis was not made for many years even though she has had severe digital ulcers for a long time. She was being managed as Rheumatoid arthritis. Systemic sclerosis remains a difficult disease to diagnose and is still under recognised. SARS-COV-2 related illness has not affected this patient adversely despite the fact of being on long term maintenance prednisolone of 7.5mg daily dose and Cellcept 2gm. Her cellcept was temporarily stopped during acute illness. We know that viral pneumonitis can present with typical ground glass opacities in bilateral areas of lungs and differential diagnosis does include connective tissue related lung disease but this lady had no significant respiratory involvement prior to COVID-19 illness and follow up scan will help to decide if this is disease progression or related to viral cause.Case report - Key learning pointsThere are multiple learning points in this case: Continuity of care under same primary team can avoid confusion related to diagnosis and diagnosis related complications. This lady had none, or mild subclinical lung involvement related to systemic sclerosis prior to contracting COVID-19 illness. Her CT chest findings after the episode of SARS-COV-2 were attributed to systemic sclerosis as she was seen by different respiratory team. This continuity is not always possible, but MDT collaboration needs to be improved across hospitals and across various departments. Systemic sclerosis remains an under diagnosed and under recognized complex rheumatic disorder and more primary care physicians need to be educated so they can appropriately refer these cases to Rheumatology services.Multi-disciplinary collaboration between Rheumatology, Respiratory and other specialties is the key point to manage these complex cases. This case also highlights an interesting observation that presence of significant immune disorder and immunosuppressant medication does not always equate to worse outcome if patient contracts SARS-COV-2. Supportive care, appropriate observation, and temporary suspension of DMARD in such cases can avoid any further complications.

PULMONARY HYPERTENSION IN PATIENTS WITH CANCER

SESSION TITLE: Pulmonary Vascular Disease Posters SESSION TYPE: Original Investigation Posters PRESENTED ON: October 18-21, 2020 PURPOSE: Pulmonary hypertension (PH) may be an underrecognized issue in cancer patients. Use of tyrosine kinase inhibitors is now a recognized etiology for World Health Organization (WHO) Group I Pulmonary Arterial Hypertension (PAH). Rare case reports/series describe tumor microemboli, pulmonary veno-occlusive disease, and myeloproliferative disease resulting in PH. Our objective was to evaluate the prevalence of PH in cancer patients based on right heart catheterization (RHC), and to describe demographic, cancer, and clinical characteristics. METHODS: A retrospective review of all cancer patients undergoing RHC from 1/2011 to 2/2020 was performed. Demographic data, cancer history, clinical characteristics, and diagnostic studies were analyzed. WHO guidelines from 2019 were used to classify patients into groups. RESULTS: Out of 180 patients that underwent RHC, 133 had PH, with 60.2% women, and a median age of 67 years (23 to 91). New York Heart Association functional class was I in 6.8%, II in 63.2%, III in 29.3%, and IV in 0.8%. Cancer diagnoses included solid organ tumors in 48.1%, hematologic in 42.0%, concurrent cancers in 2.3%, and 7.6% had no active cancer. Most (62.4%) had undergone active cancer treatment in the last 3 months. PH was classified as follows: 22.9% Group I PAH; 50.4% Group 2 PH (secondary to left-sided heart disease); 15.3% Group 3 PH (secondary to hypoxia); 6.9% Group 4 PH (secondary to thromboembolic disease); and 4.6% Group 5 PH (due to miscellaneous causes). In the 29 cases of PAH, 20 were related to therapy, 4 had autoimmune disease, and 5 were either idiopathic or related to myeloproliferative disorder. One patient had autopsy-proven tumor emboli classified as Group 5, while another had pulmonary veno-occlusive disease documented on lung tissue without evidence of PH on RHC. Treatment with pulmonary vasodilator therapy was initiated in 35.6% and included an endothelin receptor antagonist in 13, a phosphodiesterase-5 inhibitor in 41, and combination therapy in 9. Favorable response to therapy was noted in 21.3% of patients based on functional status, and in 38.9% based on echocardiographic findings. CONCLUSIONS: In our cohort, left-sided heart disease was the leading etiology of PH, followed by PAH mostly from tyrosine kinase inhibitors, although autoimmune disease and idiopathic PAH (some potentially related to myeloproliferative disease) were also present. Pulmonary vasodilator therapy was initiated in those meeting criteria. CLINICAL IMPLICATIONS: PH in cancer patients may be related to the underlying malignancy or its therapies, although autoimmune, underlying lung, or left-sided heart disease may coexist. A high clinical suspicion for PH with subsequent diagnosis and classification can help facilitate treatment and possibly improve functional status. DISCLOSURES: No relevant relationships by Lara Bashoura, source=Web Response No relevant relationships by Saadia Faiz, source=Web Response No relevant relationships by Cezar Iliescu, source=Web Response No relevant relationships by Maryam Kaous, source=Web Response No relevant relationships by Juan Lopez-Mattei, source=Web Response No relevant relationships by Nicolas Palaskas, source=Web Response Research relationship with United Therapeutics; PhaseBio Please note: $5001 - $20000 Added 06/01/2020 by Bela Patel, source=Web Response, value=Grant/Research No relevant relationships by Oriana Salamo, source=Web Response No relevant relationships by Lilit Sargsyan, source=Web Response

Non-arteritic anterior ischaemic optic neuropathy: treatment and risk factors.

To ascertain whether various therapeutic procedures in non-arteritic anterior ischaemic optic neuropathy (NAION) have an impact on the resulting visual acuity of the affected eye. To assess the prevalence of risk factors that accompany this disease according to the literature. The retrospective study enrolled 55eyes of 53 patients (41 men, 12 women) with an age range of 46 to 85 years (mean 64.9; median 64.0) who were hospitalized at the Department of Ophthalmology of the Faculty of Medicine and Dentistry and the University Hospital in Olomouc with the diagnosis of NAION between 2005 and 2016, and who received systemic treatment with intravenous vasodilators, either alone or in combination with intravenous corticosteroids. Central visual acuity (CVA) prior to treatment and immediately after its termination was evaluated. CVA was measured using the Snellen chart and is presented in decimal values. Using medical history data and medical records, the presence of systemic disease, namely hypertension, type2 diabetes mellitus, and hypercholesterolaemia, was studied in these patients and evaluated for apossible association withNAION. In the group of patients who were treated with intravenous vasodilators, the resulting CVA improved by 0.083 on average. In the group of patients who, in addition to vasodilator therapy, also received treatment with corticosteroids, the resulting CVA improved by only 0.03 on average. Although there was amore prominent improvement in CVA in the group treated with intravenous vasodilators alone, this difference was not statistically significant. At least one risk factor was found in the vast majority of the patients (96%). Eighty percent of the patients had hypertension, 43.6% of them were treated for diabetes mellitus, and 72.7% of the patients took drugs for hypercholesterolaemia. Acombination of all these conditions was found in 36.4% of the patients. The proportion of smokers and past smokers did not exceed that of non-smokers. The mean improvement in the resulting CVA in patients after systemic therapy with vasodilators alone was greater than in those treated with acombination of vasodilators and corticosteroids; however, this difference was not statistically significant. In most patients in the group, at least one systemic risk factor was noted, most frequently hypertension. The prevalence rate of systemic risk factors was comparable to that reported in theliterature.

CLINICAL CHARACTERISTICS, TREATMENT RESPONSE, AND SURVIVAL OF PATIENTS WITH IDIOPATHIC INFLAMMATORY MYOSITIS AND PULMONARY ARTERIAL HYPERTENSION

CLINICAL CHARACTERISTICS, TREATMENT RESPONSE, AND SURVIVAL OF PATIENTS WITH IDIOPATHIC INFLAMMATORY MYOSITIS AND PULMONARY ARTERIAL HYPERTENSION

S1677 Topical Cannabidiol (CBD) for the Treatment of Anal Fissures: A Case Series

INTRODUCTION: An anal fissure is defined as a longitudinal tear in the anal canal distal to the dentate line. It is a common benign condition that may be primary, resulting from local trauma, or secondary, stemming from an underlying disorder such as a malignancy, sexually transmitted infection, or inflammatory bowel disease. The primary symptom is anal pain particularly during defecation but may last for several hours. While the pathophysiology remains unclear, a mechanism of internal anal sphincter spasm, reduced perfusion and local ischemia has been proposed. Current first-line therapy involves a combination of a topical vasodilator therapy (nitroglycerin or calcium channel blockers) along with supportive measures such as a high fiber diet, sitz baths, local anesthesia, and stool softeners. Topical vasodilators are effective in 50% of cases but are often limited by side effects. The vasoactive properties of Cannabidiol (CBD) has been described in both human and animal models. CBD induced vasodilation has been shown in renal, mesenteric, opthalmic and cerebral arteries. The vasorelaxant properties of CBD have yet to be exploited in the management of anal fissures. We report five patients with anal fissures that were successfully treated with topical CBD. CASE DESCRIPTION/METHODS: Five consecutive patients who presented or were referred to a single Gastroenterology practice for an anal fissure were prescribed topical cannabidiol (1.4–2 mg/g) twice daily and supplemental fiber. One patient was female. Mean age was 49 (Range 31–63). Duration of symptoms was greater than 6 weeks in all patients. Each patient was asked to complete a modified HEMO-FISS QoL questionnaire to measure symptom improvement after two weeks of therapy. All patients reported a significant improvement in symptoms at follow-up. No side effects were reported. One patient later had a recurrence of symptoms that required treatment with Botulinum toxin injection. DISCUSSION: Cannabidiol has been shown to induce endothelium dependent vasorelaxation in human mesenteric arteries through activation of CB1 receptors. It is likely that the vasoactive properties of CBD reduce local ischemia and promote healing of chronic anal fissures. As far as we are aware, this is the first report showing a potential role for CBD in the management of acute and chronic anal fissures. Given the limited options for topical therapy and significant side effects, CBD provides an attractive alternative to conventional first line treatment. Further studies are warranted.

Heart rate monitoring improves clinical assessment during 6-min walk.

Right ventricular (RV) function is a predictor of outcomes in pulmonary arterial hypertension (PAH). The 6-min walk test (6MWT) is likely an indirect measure of RV function during exercise, but changes in absolute walk distance can also be influenced by factors like effort and musculoskeletal disease. Paired 6MWT with continuous electrocardiogram monitoring was performed in stable PAH patients, patients adding PAH therapies, and healthy controls. Heart rate expenditure (HRE) was calculated (integrating pulse during 6MWT) and then divided by walk distance (HRE/d). We also evaluated changes in peak heart rate, time above age-adjusted maximum predicted heart rate, and heart rate at 6 min. HRE/d was compared to invasive hemodynamic measures in patients who had right heart catheterization performed within seven days, WHO functional class assessment, and Emphasis 10 questionnaire. We measured two 6MWT in 15 stable PAH patients, 13 treatment intensification patients, and 8 healthy controls. HRE/d was reproducible in the stable PAH group (median difference, −0.79%), while it decreased (median difference, 23%, p = 0.0001) after adding vasodilator therapy. In 11 patients with right heart catheterization, HRE/d correlated strongly with stroke volume, r = −0.72, p = 0.01. Peak heart rate decreased after adding vasodilator therapy. HRE/d also correlated with WHO functional class and Emphasis 10 score. Continuous heart rate monitoring during 6MWT provides valuable physiologic data accounting for effort. HRE/d appears to enhance test reproducibility in stable patients while detecting change after adding therapy as compared to walk distance alone.

EMERGENT THROMBECTOMY OF HIGH RISK PE

SESSION TITLE: Fellows Pulmonary Vascular Disease Posters SESSION TYPE: Fellow Case Report Posters PRESENTED ON: October 18-21, 2020 INTRODUCTION: Some physiologic consequences of high risk pulmonary emboli (PE), such as shock and refractory hypoxemia, require urgent reperfusion therapy. Delaying management in those cases may lead to catastrophic consequences including hemodynamic collapse. However, comorbid conditions often limit treatment options complicating management. Here we present two cases of acute onset severe hypoxemia attributable to PE. Neither patient was a candidate for pharmacological thrombolysis due to recent or active GI bleeding. CASE PRESENTATION: Patient A, a 52-year-old male with prior colectomy due to ulcerative colitis, was admitted with duodenal perforation and post-ERCP pancreatitis with one day of significant dyspnea and hypoxemia. He underwent testing with a computed tomography pulmonary angiogram (CTPA) which revealed pulmonary emboli. Despite intubation, FiO2 of 100%, recruitment maneuvers, and inhaled vasodilator therapy with epoprostenol, he had persistent and refractory hypoxemia (Table). After the initiation of heparin, he had recurrent gastrointestinal hemorrhage and required vasopressors. Patient B, a 71-year-old female with multiple myeloma and on chemotherapy with pancytopenia and lower GI bleed two weeks prior, presented with acute onset of dyspnea for less than one day and high oxygen requirements and labile hemodynamics with signs of hypoperfusion (Table). CTA confirmed pulmonary emboli. Point of care ultrasonography demonstrated signs of right heart strain. DISCUSSION: Both patients had bilateral pulmonary emboli and filling defects in distal left main pulmonary artery extending into left upper and lower lobar arteries and lobar and segmental arteries on the right. After rapid discussion with a multidisciplinary PE response team, percutaneous aspiration thrombectomy via the FlowTriever System was utilized in both patients. The FlowTriever System is a large bore catheter device which is FDA approved for mechanical thrombectomy in the pulmonary arteries. Both patients were successfully treated with acute clinical and technical success, with intraprocedural improvements of mean pulmonary artery pressures of > 25%. Patient A had immediate improvement of hypoxemia. Patient B had improvement in her hemodynamic stability and organ perfusion. Both patients did require packed red blood cell transfusions. This is partially attributed to both patients having active GI bleeding, but can be exacerbated aspiration of blood during thrombectomy if multiple passes are required. CONCLUSIONS: Percutaneous thrombectomy is an alternative treatment option in critically ill patients with high risk of hemodynamic collapse or refractory hypoxemia, especially in those with contraindications to thrombolysis. One limitation is the possibility of intraprocedural blood loss which should be anticipated and address with volume expansion with or without transfusion. Reference #1: Marshall PS, Mathews KS, Siegel MD. Diagnosis and management of life-threatening pulmonary embolism. J Intensive Care Med. 2011;26(5):275-294. doi:10.1177/0885066610392658 Reference #2: Wible BC, Buckley JR, Cho KH, Bunte MC, Saucier NA, Borsa JJ. Safety and Efficacy of Acute Pulmonary Embolism Treated via Large-Bore Aspiration Mechanical Thrombectomy Using the Inari FlowTriever Device. J Vasc Interv Radiol. 2019;30(9):1370-1375. doi:10.1016/j.jvir.2019.05.024 DISCLOSURES: No relevant relationships by Brian Cody Adkinson, source=Web Response Consultant relationship with Bellerophon Please note: $1001 - $5000 Added 06/01/2020 by Roger Alvarez, source=Web Response, value=Travel Advisory Committee Member relationship with United Therapeutics Please note: $1001 - $5000 Added 06/01/2020 by Roger Alvarez, source=Web Response, value=Consulting fee No relevant relationships by Adhiraj Gosine, source=Web Response No relevant relationships by Susanna Leonard, source=Web Response

Respect The Notch: Under-appreciated Pulmonary Vascular Disease and Hemodynamic Collapse Post Left Ventricular Assist Device Placement

Introduction Right ventricular (RV) failure occurs in 15-20% of patients post Left ventricular Assist Device (LVAD) implantation and has been associated with increased mortality. Herein we describe a case of severe RV failure post LVAD implantation and how proper identification of the etiology led to a positive outcome. Case description A 58-year-old male with non-ischemic cardiomyopathy is admitted with decompensated heart failure. Right heart catheterization (RHC) showed right atrial pressure (RAP) 5 mmHg, Pulmonary artery pressure (PAP): 45/25, wedge pressure (PCWP) 24 mm Hg, pulmonary vascular resistance (PVR) of 1.4 woods units and cardiac index of 1.7 on inotropes. The patient underwent HMIII LVAD implantation. Immediately postop the patient developed shock. TTE showed an akinetic and dilated RV. He was placed on VA ECMO emergently and then transitioned to a percutaneous right ventricular assist device (RVAD). The patient eventually required dialysis. Decision-making Multiple mechanisms lead to RV failure after LVAD implantation. These include increased RV preload (from increased cardiac output and venous return or blood/fluid administration perioperatively), decreased contractility (LV decompression resulting in intraventricular septal left shift and decreased septal contribution to RV contractility), RV infarct, ventricular arrhythmias, and increased RV afterload (unmasking of pulmonary hypertension or pulmonary embolism). On review of the patient's echocardiogram post RVAD placement, there was RVAD flow dependent notching of the RV outflow tract (RVOT) pulse wave doppler (Figure 1A,B). RVOT notching is a validated sign of high PVR and prompted further review of patient's pre LVAD RHCs and TTEs. Excluding the RHC and TTE immediately pre LVAD implantation, prior RHCs showed RAP:PCWP ratio of 0.7, PAP of 70s/30s and PVR as high as 5.4 with concordant RVOT notching on TTE. The narrower pulmonary pulse pressure on the RHC immediately preceding LVAD implantation suggested a failing RV and was discordant with the lower RAP:PCWP (0.25) and lower PVR. Under-occlusion of the PCWP most likely led to a falsely elevated PCWP and falsely reduced PVR. With aggressive pulmonary vasodilator therapy, hemodynamics improved, the RVAD was explanted and dialysis was successfully discontinued. Figure 1C shows improvement of RVOT notching with this treatment. Conclusion Severe RV failure post LVAD implantation has a significant impact on morbidity/mortality. The echocardiogram provides valuable pathophysiological insights and is an important asset to interpreting invasive hemodynamics, especially pertaining to RV failure.

Novel technique of repairing right partial anomalous pulmonary venous connection with intact atrial septum using in situ interatrial septum as a flap in a 68-year-old-woman: a case report

BackgroundPartial anomalous pulmonary venous connection draining into the right atrium with an intact atrial septum is a very rare clinical entity in the adult population. Partial anomalous pulmonary venous connection must be suspected as a differential diagnosis when the cause of right heart enlargement and pulmonary artery hypertension is unknown.Case presentationThis study describes the surgical case of an isolated right partial anomalous pulmonary venous connection to the right atrium in a 68-year-old woman, who underwent tricuspid ring annuloplasty and right-sided maze procedure simultaneously. She had complaints of gradually progressing dyspnea on exertion. However, a diagnosis could not be established despite consultations at multiple hospitals for over a year. Right heart catheterization revealed severe pulmonary artery hypertension with a mean pulmonary artery pressure of 46 mmHg, step-up phenomenon of oxygen saturation at the mid-level of the right atrium with a pulmonary-to-systemic blood flow ratio of 2.4, and a pulmonary vascular resistance of 3.1 Wood Units. As medical treatment with pulmonary artery vasodilator therapy did not improve her symptoms, she underwent surgical repair. An atrial septal defect was created surgically with a curvilinear tongue-shaped cut. The right anomalous pulmonary veins were rerouted through the surgically created atrial septal defect into the left atrium with a baffle comprised of the interatrial septum flap, kept in continuity with the anterior margin and sutured while mobilizing the enlarged right atrium. The patient had an uneventful postoperative course and remains asymptomatic.ConclusionsThe described surgical technique could be considered an effective alternative for patients undergoing surgical repair for a partial anomalous pulmonary venous connection isolated to the right atrium. The indication for surgery must be judged on a case-by-case basis in these patients with prevalent systemic-to-pulmonary shunting.

Pulmonary thromboendarterectomy in Portugal: Initial experience

IntroductionSurgical treatment for chronic thromboembolic pulmonary hypertension (CTEPH) is challenging. Most Portuguese patients with CTEPH have been referred to foreign institutions for treatment, with significant social and economic costs. To meet this emerging need, the cardiothoracic surgery department of Hospital de Santa Marta, Lisbon, has developed a dedicated program for pulmonary thromboendarterectomy (PTE). We hereby present the results for the first 19 patients treated. MethodsWe conducted a retrospective analysis of all 19 patients who underwent PTE at Hospital de Santa Marta between 2008 and April 2019. ResultsSince 2008, a total of 19 patients have undergone PTE in our department. The procedure was performed with good outcomes in both survival and functional recovery. At the very beginning of the series two patients died perioperatively, before all the team underwent formal training at the Royal Papworth Hospital, UK, with no early deaths since. Postoperative complications were similar to other published series. During 11 years of follow-up, there were three late deaths, all in patients with residual pulmonary arterial hypertension. At the latest follow-up (October 2019), all surviving patients showed significant functional recovery, all in NYHA class I or II, with only one patient on vasodilator therapy with sildenafil (the first in the series, operated in 2008). ConclusionsPTE is a demanding procedure, in which outcomes are related to volume and accumulated experience, however it can be performed safely and with reproducible results by a properly prepared dedicated team with a well-controlled learning curve. More patients and multidisciplinary experience will be needed to further improve and streamline results.

Imaging digital arteries in systemic sclerosis by tomographic 3-dimensional ultrasound.

Objective methods are needed to quantify digital artery disease in systemic sclerosis (SSc) for clinical trials of vascular therapies. Our primary aim was to examine feasibility of a novel tomographic three-dimensional-(3-D) ultrasound (tUS) with high-frequency ultrasound (HFUS) or ultra-high-frequency ultrasound (UHFUS) to assess the digital arteries in patients with SSc compared to healthy controls. A secondary objective was to compare the total wall volume (TWV) as a measure of intimal/medial thickness. Eighteen patients with a confirmed diagnosis of SSc were studied by tUS HFUS (17.5MHz, n = 10) or tUS UHFUS (48 and 70MHz, n = 8) with equal numbers of healthy controls of similar age and gender. The majority of patients had limited cutaneous SSc and were representative of a spectrum of digital vasculopathy, with over half (n = 6 HFUS and n = 5 UHFUS) having previous digital ulceration. Over half were receiving oral vasodilatory therapy. TWV was measured in both digital arteries of the middle finger bilaterally. At least, two digital arteries could be identified at 17.5MHz in all patients and healthy controls. Whereas, at least two digital arteries could be identified in relatively fewer patients compared to healthy controls using 48MHz (n = 6 and 10) and especially 70MHz (n = 4 and 10) UHFUS. The median difference in TWV between patients and healthy controls was -6.49 mm3 using 17.5MHz, 1.9 mm3 at 48MHz, and -0.4 mm3 at 70MHz. tUS using UHFUS is a feasible method to measure TWV of digital arteries in SSc. Transducer frequency plays an important factor in successful digital artery measurement, with 48MHz being the optimal frequency.