Symptomatic Pulmonary Hypertension Research Articles

Under pressure: symptomatic pulmonary hypertension is a predictor of poor outcome following hip fracture.

Pulmonary hypertension (PHTN) is associated with increased morbidity and mortality in noncardiac surgery and elective surgery. This population of patients has a low physiological reserve and is prone to cardiac arrest as a result. This study aims to identify the impact that PHTN has on outcomes among geriatric hip fracture patients. A 3:1 propensity-score-matched retrospective case (PHTN)-control (no PHTN [N]) study of hip fracture patients from 2014 to 2022 was performed. Patients were matched utilizing propensity score matching of a validated geriatric trauma risk assessment tool (STTGMA). All patients were reviewed for hospital quality measures and outcomes. Comparative univariable and multivariable analyses were conducted between the two matched cohorts. A sub-analysis compared patients across PHTN severity levels (mild, moderate, severe) based on pulmonary artery systolic pressures (PASP) as measured by transthoracic echocardiogram. PHTN patients (n = 67) experienced a higher rate of inpatient, 30-day, and 1-year mortality, major complications, and 90-day readmissions as compared to the N cohort (n = 201). PHTN patients with a PASP > 60 experienced a significantly higher rate of major complications, need for ICU, longer admission length, and worse 1-year functional outcomes. Pulmonary hypertension was found to be independently associated with a 3.5 × higher rate of 30-day mortality (p = 0.016), 2.7 × higher rate of 1-year mortality (p = 0.008), 2.5 × higher rate of a major inpatient complication (p = 0.028), and 1.2 × higher rate of 90-day readmission (p = 0.044). Patients who had a prior diagnosis of pulmonary hypertension before sustaining their hip fracture experienced significantly worse inpatient and post-discharge outcomes. Those with a PASP > 60mmHg had worse outcomes within the PHTN cohort. Providers must recognize these at-risk patients at the time of arrival to adjust care planning accordingly. III.

Effect of hydrogen inhalation on cardiovascular and interstitial components of pulmonary hypertension in rats

Hydrogen is known to have selective antioxidant properties. It binds highly reactive hydroxyl radicals. The pathogenesis of the monocrotaline animal model of pulmonary hypertension is associated with oxidative stress and leads to all the symptoms of pulmonary hypertension (PH) and interstitial lung disease (ILD) associated with hypertension.The aim of this work was to study the effect of 4% hydrogen inhalations on the symptoms of PH and ILD in rats.Methods. To model monocrotaline-induced pulmonary hypertension (MCT-PH), two groups of animals received a single subcutaneous injection of monocrotaline (MCT) on day 1. The control group was injected subcutaneously with MCT solvent only. The animals receiving MCT were further divided into 2 subgroups. Subgroup 1 rats breathed room air and subgroup 2 rats breathed a mixture of room air and 4% hydrogen. The regular inhalations continued until day 21. On day 21, hemodynamic parameters were measured under urethane anesthesia, the heart and its components and the lungs were weighed, and lung tissue was preserved for morphological study.Results. The inhalation had no effect on the main cardiovascular symptoms of PH, but a positive effect on the state of the connective tissue of the lungs affected by PH was shown. The mast cell response was reduced both quantitatively and functionally. There was a decrease in tryptase expression by mast cells, with predominance of the forms without signs of degranulation. TGF-β secretion was also significantly reduced and visualized by immunopositive cells in alveolar cellular structures and vessel walls.Conclusion. Inhalation of 4% hydrogen reduces inflammation and fibrosis of lung tissue during the development of MCT-PH.

Transient abnormal myelopoiesis requiring advanced neonatal intensive care treatment.

Five to thirty percent of neonates with trisomy 21 develop transient abnormal myelopoiesis (TAM) with a high mortality rate. The aim of the study was to identify contributing factors that determine mortality and need for chemotherapy in this patient group. Six-year, single-centre, retrospective study of neonatal TAM cases requiring admission to intensive care. Data were collected from electronic patient records, laboratory and genetic results. The odds ratio was calculated to assess the likelihood of neonates with certain clinical characteristics having short-term mortality and needing chemotherapy. Twenty-one neonates were studied with a mortality rate of 28%. Neonates requiring inotropic support (OR 19, 95% CI: 0.9-399, p = 0.05) and inhaled nitric oxide (iNO) (OR 13, 95% CI: 1.4-124.3, p = 0.03) were less likely to survive to discharge. Neonates needing mechanical ventilation (OR 14, 95% CI: 1.1-185.5, p = 0.04), or a white cell count >50 × 109/L (OR 27, 95% CI: 1.2-605.7, p = 0.04) were more likely to receive chemotherapy. A high mortality rate was identified in TAM neonates with symptomatic pulmonary hypertension (PH) needing active treatment strategies, such as inotropes and iNO. The presence of PH should be considered in the clinical management, prognosis and parental counselling.

Recurrent pulmonary hypertension after balloon pulmonary angioplasty for inoperable chronic thromboembolic pulmonary hypertension

Balloon pulmonary angioplasty improves the hemodynamics of patients with inoperable chronic thromboembolic pulmonary hypertension; however, the clinical impact of recurrent pulmonary hypertension after balloon pulmonary angioplasty remains unclear. We retrospectively reviewed 262 consecutive patients with chronic thromboembolic pulmonary hypertension who underwent balloon pulmonary angioplasty between July 2009 and December 2020; 158 (65±12years; males, 20%; median follow-up period, 45 [26, 66]months) with follow-up right heart catheterization and no residual pulmonary hypertension were included. Recurrent pulmonary hypertension was defined as mean pulmonary arterial pressure <25mm Hg at the first evaluation after balloon pulmonary angioplasty and ≥25mm Hg at follow-up evaluation requiring additional treatment with balloon pulmonary angioplasty or pulmonary vasodilators. Recurrent pulmonary hypertension was observed in 11 patients; the state occupation probability of recurrence at 5years was 9.0% (95% confidence interval: 5.0%-18.9%). Only 1 case (0.6%) of recurrent pulmonary hypertension showed vascular restenosis and reocclusion of previously treated lesions, with more significant hemodynamic and exercise capacity deterioration than the other cases. Additional treatments for recurrent pulmonary hypertension (balloon pulmonary angioplasty in 9 patients, pulmonary vasodilators in 4 patients) improved the mean pulmonary arterial pressure from 27 [26, 29] to 22 [19, 23] mm Hg (p<0.01). Recurrence had a low probability of transitioning to death in an illness-death model. No specific risk factors for recurrent pulmonary hypertension were identified. Symptomatic recurrent pulmonary hypertension due to vascular restenosis or reocclusion after balloon pulmonary angioplasty was extremely rare. Most cases of recurrent pulmonary hypertension were mild, did not worsen clinically, and had favorable prognoses.

Surgical and Device Interventions in the Treatment of Chronic Thromboembolic Disease.

Chronic thromboembolic pulmonary disease (CTEPD) is characterized by unresolved clot burden in large pulmonary arteries, obstructive disease in smaller arteries, and increased downstream clot burden. This occurs in the setting of abnormal fibrinolysis or hematological disorders. Up to 50% of patients in some studies are unaware of a self-history of a deep venous thrombosis or pulmonary embolism. Ultimately, they present with symptoms of pulmonary hypertension (PH), which can result in right heart failure (RHF). Pulmonary endarterectomy (PEA) is curative, though many patients have prohibitive surgical risk or surgically inaccessible disease, warranting other interventions such as balloon pulmonary angioplasty (BPA) and medical therapy. Rarely, other treatment options may be implemented. We focus this review on PEA and BPA, with an overview of the history of CTEPD and the evolution of these procedures. We will briefly discuss other treatment modalities.

Sindrom kongenitalne centralne hipoventilacije - heterogenost kliničke prezentacije, modaliteti ventilatorne potpore i ishodi

Introduction/aim: Central congenital hypoventilation syndrome (CCHS) is a rare genetic disorder characterized by autonomic dysregulation and alveolar hypoventilation with ventilatory support being the cornerstone of long-term survival. The aim was to present different ventilatory strategies in CCHS. Material and methods: The study included retrospectively analyzed medical records of five patients diagnosed with CCHS in a national pediatric center. Alveolar hypoventilation was evidenced by noninvasive continuous transcutaneous capnometry and central sleep-disordered breathing documented by polygraphy. Clinical evaluation included cardiac evaluation, rectal biopsies, and urinalysis of catecholamine levels. Life-threatening cardiac arrhythmias were indications for pacemaker implantation. Genetic analyses of alanine residues in paired-like homeobox 2B gene (PHOX2B) confirmed the diagnosis. Results: A range of pathogenic changes in the PHOX2B gene resulted in varying clinical outcomes. 3/4 (75%) of patients with an early onset were ventilated continuously through a tracheostomy tube, while one patient was successfully treated with noninvasive ventilation (NIV) as the preferred option. Additionally, NIV was applied in one child with early-onset disease after decannulation. Finally, NIV was also feasible in a case with late-onset disease presented by the time of four years with symptoms of pulmonary hypertension. There were no serious side effects of ventilation, and one patient died due to cardiac arrhythmias. Conclusion: Invasive mechanical ventilation remains the treatment of choice in most children with early-onset disease. However, the indications for NIV have been widened from overnight ventilation in the late-onset course to selected cases with early-onset disease. The timely switch from IMV to NIV has been popularized in recent years worldwide.

Effect of acetazolamide on pulmonary vascular haemodynamics in patients with COPD going to altitude: a randomised, placebo-controlled, double-blind trial.

COPD may predispose to symptomatic pulmonary hypertension at high altitude. We investigated haemodynamic changes in lowlanders with COPD ascending to 3100 m and evaluated whether preventive acetazolamide treatment would attenuate the altitude-induced increase in pulmonary artery pressure (PAP). In this randomised, placebo-controlled, double-blind, parallel-group trial, patients with COPD Global Initiative for Chronic Obstructive Lung Disease grades 2-3 who were living <800 m and had peripheral oxygen saturation (S pO2 ) >92% and arterial carbon dioxide tension <6 kPa were randomised to receive either acetazolamide (125-250 mg·day-1) or placebo capsules, starting 24 h before ascent from 760 m and during a 2-day stay at 3100 m. Echocardiography, pulse oximetry and clinical assessments were performed at 760 m and after the first night at 3100 m. Primary outcome was PAP assessed by tricuspid regurgitation pressure gradient (TRPG). 112 patients (68% men, mean±sd age 59±8 years, forced expiratory volume in 1 s (FEV1) 61±12% pred, S pO2 95±2%) were included. Mean±sd TRPG increased from 22±7 to 30±10 mmHg in 54 patients allocated to placebo and from 20±5 to 24±7 mmHg in 58 patients allocated to acetazolamide (both p<0.05) resulting in a mean (95% CI) treatment effect of -5 (-9 to -1) mmHg (p=0.015). In patients assigned to placebo at 760/3100 m, mean±sd S pO2 was 95±2%/88±3%; in the acetazolamide group, the respective values were 94±2%/90±3% (both p<0.05), resulting in a treatment effect of +2 (1 to 3)% (p=0.001). In lowlanders with COPD travelling to 3100 m, preventive acetazolamide treatment attenuated the altitude-induced rise in PAP and improved oxygenation.

Inter-Specialty Controversies on the Treatment of Cardiovascular Diseases during Pregnancy: A Questionnaire Study.

Background and Objectives: Management of cardiovascular disease (CVD) during pregnancy is challenging and usually requires eminence-based decisions due to limited strong-evidence data in this field. The purpose of our study was to compare the attitudes of anaesthesiologists, cardiologists, and gynaecologists towards the diagnosis and treatment of potentially life-threatening CVDs during pregnancy. Materials and Methods: A cross-sectional, questionnaire-based study was performed among 111 doctors (55 anaesthesiologists, 36 cardiologists, 20 gynaecologists). Personal opinions on the recommendations (n = 19) regarding rare, potentially life-threatening CVDs during pregnancy were recorded using a five-item Likert scale. Results: Opinions regarding eight statements (42%) varied substantially between specialties (p < 0.05). The most distinctive differences between physicians concerned the following recommendations: “thrombolysis should only be used in pulmonary embolism with cardiogenic shock” (agree: 52.7% of anaesthesiologists, 80.4% of cardiologists, 25.0% of gynaecologists; p < 0.001); “women with the antiphospholipid syndrome should restart treatment with vitamin K antagonists from the second trimester of pregnancy” (agree: 12.7% of anaesthesiologists, 69.4% of cardiologists, 20.0% of gynaecologists; p < 0.001); “women with symptomatic pulmonary hypertension should have a Swan–Ganz catheter inserted for labour” (agree: 20.0% of anaesthesiologists, 11.1% of cardiologists, 55.0% of gynaecologists; p = 0.001). Conclusions: Physicians’ opinions regarding diagnostics and treatment of CVDs in pregnancy remain controversial. A multidisciplinary approach is recommended to ensure the safety and effectiveness of management in these unique medical conditions.

Efficacy and safety of inhaled nitrite in addition to sildenafil in thalassemia patients with pulmonary hypertension: A 12-week randomized, double-blind placebo-controlled clinical trial

Pulmonary hypertension is a significant complication in thalassemia patients. Recent studies showed that inhaled nebulized nitrite could rapidly decrease pulmonary artery pressure. We conducted a multicenter, randomized, double-blind, placebo-controlled trial in thalassemia patients with symptomatic pulmonary hypertension diagnosed by right heart catheterization. Eleven patients were recruited; five were assigned to the nitrite group and six to the placebo group. Patients were treated with the optimal doses of sildenafil for pulmonary hypertension and randomly assigned into the placebo or nitrite groups. Patients in the nitrite group were given inhaled nebulized 30 mg sodium nitrite twice a day for 12 weeks. The clinical outcomes measured at week 12 were the changes in 6-min walk distance (6MWD), mean pulmonary artery pressure (MPAP), and N-terminal pro B-type natriuretic peptide. The MPAP estimated by echocardiography was significantly reduced from 33.6 ± 7.5 mmHg to 25.8 ± 6.0 mmHg (mean difference = 7.76 ± 3.69 mmHg, p = 0.009 by paired t-test). Furthermore, 6MWD was slightly increased from 382.0 ± 54.0 m to 432 ± 53.9 m (mean difference = 50.0 ± 42.8 m, p = 0.059 by paired t-test) in the nitrite group. At week 12, the nitrite group had lower MPAP than the placebo group (25.8 ± 6.0 vs. 45.7 ± 18.5 mmHg, p = 0.048 by unpaired t-test). No significant difference in 6MWD and N-terminal pro B-type natriuretic peptide between the two groups was observed at week 12. There was no hypotension or other significant adverse effects in the nitrite group.

Methylene Blue to Neonatal Septic Shock treatment in neonate pigs,

ObjectiveThe authors design an animal model of neonatal sepsis to analyze the treatment of neonatal septic shock with Methylene Blue (MB) in a swine model. MethodsThe study design included twenty male newborn pigs divided into four groups: 1) The control group; 2) The sepsis group (induced with lipopolysaccharide); 3) The MB group, and 4) The MB-treated sepsis group. Septic shock was defined as Blood Pressure (BP) dropping 20% below the baseline value. Continuous Blood Pressure (BP), Nitric Oxide (NO) levels, cyclic Guanosine Monophosphate (cGMP), malondialdehyde acid, base excess, lactate, arterial blood gases, hematocrit, and echocardiography were analyzed. ResultsThe BP of the sepsis group treated with MB showed a slight improvement in the first hour after treatment; however, a significant difference was not observed compared to the untreated sepsis group. Besides hemodynamic stability, the current study did not show symptomatic pulmonary hypertension, suggesting that MB was safe in neonates and children. An improvement in Base Excel (BE) levels after MB administration in septic animals may indicate a possible improvement in microcirculation. ConclusionThe MB improved biomarkers related to septic shock prognosis, although an improvement in the blood levels could not be detected. MB might be a beneficial drug for hemodynamic instability in infants.

Echocardiography in Pulmonary Arterial Hypertension: Comprehensive Evaluation and Technical Considerations.

Pulmonary arterial hypertension (PAH) is a rare, progressive disease in which there is a persistent, abnormal increase in pulmonary artery pressure. Symptoms of pulmonary hypertension are nonspecific and mainly associated with progressive right ventricular failure. The diagnosis of PAH is a multistep process and often requires the skillful use of several tests. The gold standard for the diagnosis of PAH is hemodynamic testing. Echocardiography currently plays an important role in the diagnostic algorithm of PAH as it is minimally invasive and readily available. Moreover, many echocardiographic parameters are closely related to pulmonary hemodynamics. It allows assessment of the right heart′s structure and function, estimation of the pressure in the right ventricle, right atrium, and pulmonary trunk, and exclusion of other causes of elevated pulmonary bed pressure. Echocardiographic techniques are constantly evolving, and recently, measurements made using new techniques, especially 3D visualization, have become increasingly important. In echocardiographic assessment, it is crucial to know current guidelines and new reports that organize the methodology and allow standardization of the examination. This review aims to discuss the different echocardiographic techniques used to evaluate patients with PAH.

Pulmonary hypertension due to interstitial lung disease or chronic obstructive pulmonary disease: a patient experience study of symptoms and their impact on quality of life.

Pulmonary hypertension resulting from chronic lung disease such as chronic obstructive pulmonary disease and interstitial lung disease is categorized by the World Health Organization as Group 3 pulmonary hypertension. To identify the symptoms and impacts of World Health Organization Group 3 pulmonary hypertension and to capture data related to the patient experience of this disease, qualitative research interviews were undertaken with 3 clinical experts and 14 individuals with pulmonary hypertension secondary to chronic obstructive pulmonary disease or interstitial lung disease. Shortness of breath, fatigue, cough, and swelling were the most frequently reported symptoms of pulmonary hypertension due to chronic obstructive pulmonary disease or interstitial lung disease, and shortness of breath was further identified as the single most bothersome symptom for most patients (71.4%). Interview participants also described experiencing a number of impacts related to pulmonary hypertension and pulmonary hypertension symptoms, including limitations in the ability to perform activities of daily living and impacts on physical functioning, family life, and social life as well as emotional impacts, which included frustration, depression, anxiety, isolation, and sadness. Results of these qualitative interviews offer an understanding of the patient experience of pulmonary hypertension due to chronic obstructive pulmonary disease or interstitial lung disease, including insight into the symptoms and impacts that are most important to patients in this population. As such, these results may help guide priorities in clinical treatment and assist researchers in their selection of patient-reported outcome measures for clinical trials in patients with pulmonary hypertension due to chronic obstructive pulmonary disease or interstitial lung disease.

Impact of lung morphology on clinical outcomes with riociguat in patients with pulmonary hypertension and idiopathic interstitial pneumonia: A post hoc subgroup analysis of the RISE-IIP study

Riociguat in Patients with Symptomatic Pulmonary Hypertension associated with Idiopathic Interstitial Pneumonias (RISE-IIP), a randomized, controlled, phase 2b trial of riociguat for pulmonary hypertension associated with idiopathic interstitial pneumonia, was terminated early due to increased mortality in riociguat-treated patients. Baseline characteristics of enrolled patients demonstrated a low diffusing capacity of the lung for carbon monoxide (DLCO) with preserved lung volumes at baseline, suggesting the presence of combined pulmonary fibrosis and emphysema (CPFE) in some patients. This post hoc analysis of RISE-IIP was undertaken to explore lung morphology, assessed by high-resolution computed tomography, and associated clinical outcomes. Available baseline/pre-baseline high-resolution computed tomography scans were reviewed centrally by 2 radiologists. The extent of emphysema and fibrosis was retrospectively scored and combined to provide the total CPFE score. Data were available for 65/147 patients (44%), including 15/27 fatal cases (56%). Of these, 41/65 patients (63%) had CPFE. Mortality was higher in patients with CPFE (12/41; 29%) than those without (3/24; 13%). Fourteen patients with CPFE had emphysema > fibrosis (4 died). No relationship was observed between CPFE score, survival status, and treatment assignment. A low DLCO, short 6-min walking distance, and high forced vital capacity:DLCO ratio at baseline also appeared to be risk factors for mortality. High parenchymal lung disease burden and the presence of more emphysema than fibrosis might have predisposed patients with pulmonary hypertension associated with idiopathic interstitial pneumonia to poor outcomes in RISE-IIP. Future studies of therapy for group 3 pulmonary hypertension should include centrally adjudicated imaging for morphologic phenotyping and disease burden evaluation during screening.

Pulmonary Hypertension Symptoms and Diagnostic Procedures

Pulmonary hypertension is a type of elevated blood pressure that influences the arteries in your lungs and the right side of the heart. In one form of pulmonary hypertension, called pulmonary arterial hypertension (PAH), blood vessels in the lungs are bringdown, obstructed, or destroyed. The impairment decelerates the blood flow through the body of lungs, and blood pressure in the lung arteries rises. The heart must work harder to pump the blood through the lungs and body. The extra attempt equally causes heart muscle to become sick and fail. In some people, pulmonary hypertension slowly gets very bad, and it can be life-threatening. Although there is no cure for some types of pulmonary hypertension, treatment can help bring down symptoms and improve the quality of life.

Проблемы диагностики легочной гипертензии, ассоциированной с системной склеродермией

Systemic sclerosis is the most common autoimmune disease associated with pulmonary hypertension. Pulmonary hypertension is a potential predictor of early death, and therefore, recently, interest in a comprehensive study of the diagnosis of pulmonary hypertension associated with systemic sclerosis has been increasing among researchers. Aim. Consideration of current diagnostic issues, as well as the principles of using diagnostic algorithms to verify pulmonary hypertension associated with systemic sclerosis, applied in daily clinical practice. Material and methods. In order to study the literature data, a search was carried out for information on this problem up to 10 years in depth in the Web of Science, PubMed/MEDLINE. During the search, the following terms were used individually and in combination: "systemic sclerosis", "pulmonary hypertension", "DETECT algorithm", "catheterization of the right heart chambers". The main search criteria were studies based on the study of patients with pulmonary hypertension associated with systemic sclerosis: meta-analyzes, original studies, retrospective and cohort studies. Results and discussion. The clinical symptoms of pulmonary hypertension can be nonspecific, which greatly complicates the diagnosis of the disease in the early stages. The absence of a specific clinical picture before the development of an advanced stage of pulmonary hypertension leads to late verification of the diagnosis. Key algorithms used in the daily practice of a doctor make it possible to minimize the number of undiagnosed cases of pulmonary hypertension. Conclusions. Diagnosis of PH associated with systemic sclerosis is often challenging for clinicians to practice. Early diagnosis and therefore treatment of PH are of paramount importance as they improve survival rates in patients with systemic sclerosis. Keywords: systemic sclerosis, pulmonary hypertension, screening, DETECT algorithm, right heart catheterization.

A Severe Case of Idiosyncratic Hepatotoxicity with Unfractionated Heparin.

The authors describe a case of clinically apparent idiosyncratic hepatotoxicity in association with unfractionated heparin (UFH). A 52-year-old woman with increasingly symptomatic rheumatic mitral valvular disease and severe pulmonary hypertension underwent elective minimally-invasive bioprosthetic mitral valve replacement. The patient received 42 000 units of UFH intraoperatively 10 days after receiving 3100 units during a left heart catheterization. Standard prophylactic doses of unfractionated heparin were started on POD 2 for prevention of venous thromboembolism. On the evening of postoperative day (POD) 3, the patient was lethargic, encephalopathic, and hypoglycemic with an acute liver injury and hyperlactatemia. Similar events occurred on POD 7 after clinical improvement from the initial injury and an unintentional rechallenge with UFH. Heparins are usually not suspected of idiosyncratic hepatotoxicity due to their widespread utilization and reports of milder episodes of hepatotoxicity. This case highlights the need to consider UFH in the differential of drug-induced liver injury, including severe cases.

A RARE CASE OF PORTOPULMONARY HYPERTENSION AS A COMPLICATION OF CRYPTOGENIC CIRRHOSIS

SESSION TITLE: Medical Student/Resident Pulmonary Vascular Disease Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: October 18-21, 2020 INTRODUCTION: Portopulmonary hypertension (PoPH) is a rare and serious complication of liver cirrhosis. The prevalence of PoPH is about 0.25–4% among patients with cirrhosis. Untreated PoPH can have a poor prognosis. We report a rare case of PoPH. CASE PRESENTATION: A 35 year old female with a history of cryptogenic liver cirrhosis with portal hypertension and transjugular intrahepatic portosystemic shunt (TIPS) presented to the emergency room with complaints of dyspnea, orthopnea, and dizziness which started 2 days after her 5th caesarean section. Her exercise tolerance was reduced to one block. The patient had an elevated ProBNP of 1100. Computed tomographic pulmonary angiography was negative for pulmonary embolism. Transthoracic echocardiogram showed right ventricular systolic pressure elevated at >60mm Hg, consistent with severe pulmonary hypertension (PAH) with severely dilated right atrium. The patient was transferred to a tertiary care hospital for confirmation of the diagnosis of PoPH. The patient underwent Right Heart Catheterization which confirmed severe PAH with elevated mean pulmonary artery pressure (mPAP) of 60mmhg, normal pulmonary capillary wedge pressure of 12mmhg, elevated pulmonary vascular resistance of 17 wood units. The hepatic vein wedge pressure was 23mmHg and hepatic vein pressure was 20mmHg.The hepatic venous pressure gradient (HVPG) was 3mmhg which was normal and was consistent with the fact that the patient already underwent TIPS due to underlying portal hypertension. The patient met the diagnostic criteria for PoPH. The patient was started on treprostinil infusion for pulmonary vasodilatation. The patient improved symptomatically and was eventually discharged. In her follow up pulmonary clinic appointment her exercise tolerance improved to 10 blocks with complete resolution of orthopnea. DISCUSSION: The development of PAH in patients with cirrhosis can be explained by shunting of vasoactive mediators from splanchnic to pulmonary circulation through portosystemic shunts causing vasoconstriction of the pulmonary vasculature. The acute volume loading with mobilization of plasma volume in the immediate postpartum period may have tipped the patient into acute on chronic right ventricular failure and thereby revealed her symptomatic PAH. The prompt recognition of PoPH and initiation of appropriate medical treatment ensured the recovery of the patient. CONCLUSIONS: The diagnosis of PoPH is crucial before liver transplantation as there is a high risk of death following liver transplantation if the mPAP is more than 35 mmHg. Medical treatment is essential to lower the mPAP to less than 35mmhg before proceeding for liver transplantation. Liver transplantation can be planned after successful medical treatment of PoPH with pulmonary arterial vasomodulators like prostanoids, phosphodiesterase inhibitors, and endothelin antagonists. Reference #1: Benjaminov FS, Prentice M, Sniderman KW, et al. Portopulmonary hypertension in decompensated cirrhosis with refractory ascites. Gut 2003; 52: 1355-1362. Reference #2: Saleemi S. Portopulmonary hypertension.Ann Thorac Med. 2010 Jan-Mar; 5(1): 5–9. Reference #3: Porres-Aguilar M, Zuckerman MJ, Figueroa-Casas JB, et al.Portopulmonaryhypertension: state of the art. Ann Hepatol 2008; 7:321-330 DISCLOSURES: No relevant relationships by Antony Arumairaj, source=Web Response No relevant relationships by Diana Cativo, source=Web Response No relevant relationships by Imnett Habtes, source=Web Response

ESC guidelines 2019 on diagnostics and management of acute pulmonary embolism

This article on the new European Society of Cardiology (ESC) guidelines for diagnostics and management of acute pulmonary embolism (PE) focusses on new or changed recommendations compared to the previous version of the guidelines from 2014. The current risk-adjusted management algorithm for acute PE includes the clinical severity, aggravating comorbid conditions and right ventricular dysfunction. For low-risk patients early discharge and outpatient treatment are possible, whereas for high-risk patients reperfusion treatment and hemodynamic support have to be considered, depending on the hemodynamic situation and contraindications in the individual patient. Effective therapeutic anticoagulation for at least 3months is recommended for all patients with PE. Potential indicators for extended anticoagulation are given in the guidelines (class I or class IIa recommendations). New oral anticoagulants (NOAC) are the first choice for anticoagulation in preference to vitaminK antagonists (VKA); however, they are not recommended in patients with severe renal dysfunction, during pregnancy or lactation and in patients with antiphospholipid antibody syndrome. Furthermore, anew algorithm for the follow-up after acute PE is proposed in the guidelines. In cases of symptomatic persistent pulmonary hypertension (PH) the transfer to aspecialized center is recommended.

P3672Effects of inhaled nitrite in addition to sildenafil in thalassemia patients with pulmonary hypertension

Abstract Background Pulmonary hypertension is the major morbidity and mortality in patients with thalassemia. Recent studies have found that inhaled nebulized nitrite can significantly decrease pulmonary pressure in immediate response, but data are needed to determine its clinical benefits. Methods We conducted a multicenter, randomized, double-blind, placebo-controlled trial including thalassemia patients with symptomatic pulmonary hypertension, diagnosed by right heart catheterization and were currently treated with sildenafil. Patients continued the optimum dose of sildenafil and were randomly assigned into 2 groups; the treatment group and the control group. The treatment group was given 30 mg of inhaled nebulized nitrite twice a day for 3 months while the control group was given inhaled normal saline. The outcomes were the improvement of clinical and echocardiographic parameters at 1 and 3 months. Results A total of 12 patients were recruited, 5 were assigned to the treatment group and 7 to the control group. The median of sildenafil in both group were 60 mg/day. Treatment with the inhaled nebulized nitrite in addition to sildenafil significantly reduced the mean pulmonary artery pressure at 3 months when compared to placebo (median −7.30 mmHg in treatment group vs +2.30 mmHg in control group, P=0.016). In the treatment group, the median 6-minute walk distance was significantly increased from 380 m to 427 m. at 1 month (P=0.043) but there was no significant difference in the median change of 6-minute walk distance when compared to control group at 3 months (+48 m. in treatment group vs +17.5 m. in control group, P=0.286). No significant difference in NT-proBNP change between the two groups was detected. There were no hypotension or other major adverse effects in the treatment group. Clinical and Echoparemeters Outcome Conclusions The addition of inhaled nebulized nitrite to sildenafil can decrease mean pulmonary pressure and has a tendency to improve six minutes walk distance in thalassemia patients with pulmonary hypertension. Acknowledgement/Funding Cooley's anemia foundation, Mahidol university research funding

Exercise-Induced Changes in Pulmonary Artery Stiffness in Pulmonary Hypertension.

Background: Pulmonary hypertension causes pulmonary artery (PA) stiffening, which overloads the right ventricle (RV). Since symptoms of pulmonary hypertension (PH) are exacerbated by exercise, exercise-induced PA stiffening is relevant to cardiopulmonary status. Here, we sought to demonstrate the feasibility of using magnetic resonance imaging (MRI) for non-invasive assessment of exercise-induced changes in PA stiffness in patients with PH.Methods: MRI was performed on 7 PH patients and 8 age-matched control subjects at rest and during exercise stress. Main pulmonary artery (MPA) relative area change (RAC) and pulse wave velocity (PWV) were measured from 2D-PC images. Invasive right heart catheterization (RHC) was performed on 5 of the PH patients in conjunction with exercise stress to measure MPA pressures and stiffness index (β).Results: Heart rate and cardiac index (CI) were significantly increased with exercise in both groups. In controls, RAC decreased from 0.27 ± 0.05 at rest to 0.22 ± 0.06 with exercise (P < 0.05); a modest increase in PWV was not significant (P = 0.06). In PH patients, RAC decreased from 0.15 ± 0.02 to 0.11 ± 0.01 (P < 0.05) and PWV and β increased from 3.9 ± 0.54 m/s and 1.86 ± 0.12 at rest to 5.75 ± 0.70 m/s and 3.25 ± 0.26 with exercise (P < 0.05 for both), respectively. These results confirm increased MPA stiffness with exercise stress in both groups and the non-invasive metrics of MPA stiffness correlated well with β. Finally, as assessed by PWV but not RAC, PA stiffness of PH patients increased more than that of controls for comparable levels of moderate exercise.Conclusion: These results demonstrate the feasibility of using MRI for non-invasive assessment of exercise-induced changes in MPA stiffness in a small, heterogeneous group of PH patients in a research context. Similar measurements in a larger cohort are required to investigate differences between PWV and RAC for estimation of MPA stiffness.