Mild Pulmonary Arterial Hypertension Research Articles

Effects of maternal pulmonary arterial hypertension on fetal hemodynamics and maternal-fetal outcome in late pregnancy.

The aim of this study was to investigate the effects of maternal pulmonary arterial hypertension (PAH) on fetal hemodynamics in the third trimester and to identify hemodynamic indicators associated with adverse maternal and fetal outcomes. We recruited 48 pregnant women with PAH in the third trimester and 32 women with normal pregnancies as controls matched for age and gestational week. Fetal growth and hemodynamic parameters were assessed by two-dimensional and color Doppler. All cases were followed up until delivery and maternal and fetal outcomes were collected. High throughput sequencing method was used to determine differential miRNA patterns in plasma exposed to pulmonary arterial hypertension (PAH) in pregnant women. We then performed the validated of key differentially expressed miRNAs by real-time PCR. Compared with the normal and mild PAH groups, resistance index (RI), pulsatility index (PI) of the fetal umbilical artery (UA) and quantitative ductus venosus (QDV) blood flow were increased in subjects with moderate to severe PAH, while PI and the ratio of peak systolic velocity (PSV) to end-diastolic velocity (EDV) (S/D) of the middle cerebral artery (MCA) were decreased. Compared with the normal group, subjects in the mild and moderate PAH groups had lower neonatal weight, shorter neonatal height, and higher preterm birth rates. In addition, miRNA sequencing data showed that PAH affected the levels of 23 miRNAs in plasma. At the same time, we showed that PAH significantly decreased the level of miR-1255a and increased the level of miR-548ar-3p by real-time PCR. In the group of pregnant women with moderate to severe pulmonary hypertension, there was a higher proportion of preterm births and low birth weight babies. Hemodynamic changes in the fetal UA, MCA, and ductus venosus (DV) during late pregnancy were associated with adverse fetal outcomes. At the same time, miRNA sequencing results showed that miR-1255a and miR-548ar-3p may play an important role in the development of PAH.

Prevalence of pulmonary arterial hypertension in post-tuberculosis lung fibrosis patients: A cross-sectional observational study

Introduction: Post-tuberculosis (TB) lung fibrosis is an infrequently reported cause of Pulmonary arterial hypertension (PAH). Pulmonary TB still being an important global health problem, especially in developing countries, PAH developing after pulmonary TB deserves attention. This study was undertaken to know the prevalence of PAH among patients with post-TB lung fibrosis. Methods: A cross-sectional observational study was carried out in two tertiary care hospitals in Kolkata over a period of 1 year. Sixty adult post-TB lung fibrosis patients without any cardiovascular or other pulmonary comorbidities were included in this study. Results: Out of the 60 patients with varying degrees of lung fibrosis confirmed radiologically, eight patients were found to have PAH (13.33% of the study population). Among the eight cases, six were males and two were females. Five of the males were smokers with a mean of 17.6 pack years of smoking. Out of the eight diagnosed PAH cases, three were mild PAH, four were moderate, and one case had severe PAH (grading based on 2D echocardiography findings). Pulmonary function test (PFT) revealed obstructive pattern in three cases, restrictive pattern in three cases, and mixed in two cases. On PFT, smokers with PAH exhibited obstructive pattern and mixed pattern, whereas the nonsmoker PAH cases exhibited restrictive pattern. Conclusion: All post-TB lung fibrosis cases must be followed up at regular intervals and screened to rule out PAH. Early diagnosis and prompt treatment of the cases will help to reduce morbidity and mortality of the disease which otherwise has a poor prognosis.

Severe hemolytic exacerbations of Chinese PNH patients infected SARS-CoV-2 Omicron.

Paroxysmal nocturnal hemoglobinuria (PNH) is characterized by hemolytic anemia, bone marrow failure, thrombophilia. COVID-19, caused by a novel severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) with many variants including Omicron. This study collected demographic and clinical data of 20 PNH patients with SARS-CoV-2 Omicron infection. They all were with high disease activity, and LDH level exceeded any documented since the diagnosis of PNH, and those reported in the literature for previously stable treatment with complement inhibitors. D-dimer level elevated in 10 patients. 2 patients developed mild pulmonary artery hypertension. Glomerular filtration rate declined in 5 patients. 1 patient developed acute renal failure and underwent hemodialysis. Anemia and hemolysis were improved in 5 patients treated with eculizumab. Hemolytic exacerbation of PNH with COVID-19 is severe and eculizumab may be an effective treatment.

Neonatal outcomes in pregnant women with pulmonary arterial hypertension associated with heart disease.

As an increasing number of women with pulmonary arterial hypertension (PAH) choose to become pregnant, outcomes in newborns have become a priority. The main purpose of this study was to compare the neonatal outcomes in pregnant women with PAH associated with heart disease. A single-center retrospective study was performed. Pregnancy with heart disease is divided into three groups: no PAH, mild PAH and severe PAH. Neonatal outcomes of pregnant women were compared among groups. Meanwhile, multivariable analyses were used to investigate the association between maternal PAH and adverse neonatal events. A total of 127 pregnant women with heart disease were enrolled. Of these, 82 (64.6%) had no PAH, 19 (15%) had mild PAH and 26 (20.4%) had severe PAH. The offspring of women with severe PAH had a higher risk of preterm delivery, low birth weight, neonatal respiratory distress syndrome (NRDS), neonatal intensive care unit (NICU) admission and recurrence of congenital heart disease (CHD). Compared to the women without PAH, only the risk of preterm delivery (32-36 weeks) and NICU admission were slightly higher in mothers with mild PAH; other neonatal events were similar. Multivariate regression analyses showed that the risk of preterm delivery (<37 weeks) increased with the increasing severity of maternal PAH, with an OR of 3.1 (95% CI, 1.1-8.8) for mild and 21.9 (95%CI, 4.8-99.4) for severe PAH. The same pattern was observed for NICU admission. Mothers with severe PAH were independently associated with low birth weight (OR 13, 95%CI 4.3-39, P<0.001), NRDS (OR 17.9, 95%CI 5.5-58.9, P<0.001) and recurrence of CHD (OR 4.47, 95%CI 1.7-11.6, P=0.002). Pregnancy in women with severe PAH can significantly increase the risks of neonatal events. While neonatal outcomes in pregnant women with mild PAH were considered optimistic in the present study, a multidisciplinary management of PAH in pregnancy would be necessary to have consistently good outcomes.

Pregnancy feasibility in women with mild pulmonary arterial hypertension: a systematic review and meta-analysis

BackgroundThe aim of this study was to evaluate the pregnancy feasibility of women with mild pulmonary hypertension according to pregnancy outcomes.MethodsThis systematic review and meta-analysis compared the differences in maternal and fetal outcomes between mild and moderate-to-severe pulmonary hypertension. Relevant English and Chinese literature were searched in the PubMed, Embase, Cochrane Central Register of Controlled Trials (COCHRANE), CNKI, WanFang Data, and VIP databases between January 1st, 1990 and April 18th, 2023, and the references of the included articles and relevant systematic reviews were reviewed to determine whether studies were missed. The inclusion criteria were randomized controlled and observational studies (including case-control studies and cohort studies) examining maternal and fetal pregnancy outcomes with pulmonary hypertension. Conference abstracts, case reports, case series reports, non-comparative studies, and review articles were excluded.ResultsThis meta-analysis included 32 studies. In this study, maternal and fetal outcomes were better in the mild pulmonary hypertension group than in the moderate-to-severe group. Regarding maternal mortality, the mild group was much lower than the moderate to severe group. We found a significant decrease in maternal mortality in the mild group after 2010. However, no significant difference in maternal mortality before and after 2010 was observed in the moderate to severe group. Cardiac complications, ICU admission, neonatal preterm birth, small for gestational age infants, low birth weight infants, neonatal asphyxia, and neonatal mortality were significantly lower in the mild pulmonary hypertension group than in the moderate to severe pulmonary hypertension group. The cesarean section rates of the two groups were similar. However, the vaginal delivery rate in the mild pulmonary hypertension group was significantly higher than that in the moderate to severe pulmonary hypertension group.ConclusionsThis meta-analysis confirmed that pregnancies with mild pulmonary hypertension had significantly better maternal and fetal outcomes than those with moderate to severe pulmonary hypertension. For patients with mild pulmonary hypertension and good cardiac function, continued pregnancy or even delivery should be considered under multidisciplinary monitoring. However, maternal and fetal complications with moderate to severe pulmonary hypertension significantly increase. Hence, it is essential to evaluate pregnancy risk and terminate it in time.

Muscle strength is reduced in children with pulmonary arterial hypertension.

Muscle strength is decreased in adults with pulmonary arterial hypertension (PAH). We aim to investigate muscle strength in children with PAH in relation to a cohort of healthy children, and investigate correlations with disease severity markers. This prospective study included children with PAH aged 4-18 years, who visited the Dutch National Referral Center for Pulmonary Hypertension in Childhood between October 2015 and March 2016. Muscle strength was assessed using handgrip strength and maximum voluntary isometric contractility (MVIC) of four peripheral muscles. Dynamic muscle function was evaluated with the Bruininks-Oseretsky test of motor proficiency (BOT-2). These measurements were compared with those in two cohorts of healthy children and correlated with 6-minute walk distance (6MWD), World Health Organization functional class (WHO-FC), N-terminal pro-brain natriuretic peptide (NT-proBNP), and time since diagnosis. Eighteen children with PAH aged 14.0 [interquartile range: 9.9-16.0] years showed reduced muscle strength. Handgrip strength z-score -2.4 ± 1.2, p < 0.001, total MVIC z-score -2.9 ± 1.2, p < 0.001, and BOT-2 z-score -1.0 ± 0.9, p < 0.001. 6MWD (67 ± 11% predicted) correlated with most muscle measurements (r = 0.49-0.71, p = 0.001). Dynamic muscle function (BOT-2) differed between WHO-FC, whereas handgrip strength and MVIC did not. NT-proBNP and time since diagnosis did not show significant correlations with muscle strength measurements. Muscle strength was significantly reduced in children with PAH and correlated with 6MWD, but not with disease severity markers WHO-FC and NT-pro-BNP. The nature of this reduced muscle strength is yet unclear, but its occurrence in children with seemingly mild or well-controlled PAH supports the concept of PAH being a systemic syndrome involving peripheral skeletal muscles.

Isolated Insular Stroke: An Interesting Report of Two Cases.

Isolated Insular Stroke: An Interesting Report of Two Cases.

Clinical outcomes of pulmonary hypertension in pregnancy among women with congenital heart disease in China

Absrtact Objective Pulmonary arterial hypertension (PAH) increases the risk for perinatal women and newborns, especially in women with congenital heart disease (CHD). We explored the maternal, perinatal, and postneonatal outcomes of PAH in pregnant women with CHD in China. Methods A total of 95 pregnant women with CHD-PAH in Beijing Anzhen Hospital from 2009 to 2013 were included retrospectively. We described their characteristics and examined the associations between the grade of PAH and maternal, perinatal, and postneonatal outcomes. Results The New York Heart Association (NYHA) classification grade, delivery mode, and infant outcomes in CHD-PAH patients were analyzed. Overall 95 patients with CHD-PAH, there were 17 patients in mild group(17.7%), 27 patients in moderate group (28.1%), and 51 patients in severe group (53.1%)。The CHD patients with mild PAH, were mostly NYHA class I–II and CHD patients with severe PAH were NYHA class II–IVs. Cesarean section (67.7%) was the most common method of delivery. The rate of therapeutic abortion in the severe group (76.9%) was obviously higher than that in other groups (11.5% in mild group and moderate group respectively), whereas there was no term delivery in severe group, with 2 cases in mild group and moderated group respectively . The rates of heart failure and therapeutic abortion in pregnant women were positively associated with the severity of PAH. The rate of term delivery was higher in patients whose CHD had been corrected by cardiac surgery (83.3%) before pregnancy. Conclusion Patients with severe PAH have poor cardiac adaptability, poor maternal and fetal outcomes, and are contraindicated during pregnancy. Patients with mild PAH are not absolutely contraindicated during pregnancy, but their heart burden increases significantly in the third trimester. Patients in mid-gestation should preferentially be delivered by cesarean section. When pregnant patients prefer to continue pregnancy, their close monitoring is essential. We provide useful data for guiding management of pregnancy and delivery in patients with CHD in China.

Reconstruction of the Left Atrioventricular Valve with Pericardial Patch Closure of the Ostium Primum Atrial Septal Defect in a Patient with Partial Atrioventricular Septal Defect under Mild Hypothermic Extracorporeal Circulation and Cardioplegic Arrest (UKC’s Modification): A Video Presentation

A 26-year-old male patient diagnosed with partial type of atrioventricular septal defect in sinus rhythm, cleft left atrioventricular valve with mild pulmonary arterial hypertension, and severe left atrioventricular valvular regurgitation successfully underwent reconstruction of the left atrioventricular valve and pericardial patch closure of the atrial septal defect using UKC’s modification. The technical details of the surgical procedure have been elaborated in detail.

Wolff-Parkinson-White syndrome in pregnancy: a case report

Wolff-Parkinson-White (WPW) syndrome is a rare congenital cardiac pre-ex citation syndrome encountered during pregnancy. However, WPW under this physiological process might be responsible for life threatening arrthymias leading to dangerous outcomes for both mother and the fetus. It is a ventricular pre-excitation syndrome of anterograde activation of the ventricle resulting from aberrant conduction pathway as well as the AV node. Very small percent of patients with WPW syndrome that is less than 1% are at risk of sudden cardiac death. Most of the antiarrthymic drugs are a threat to the fetus therefore careful choice of drug has to be made. Digoxin is the drug that is contraindicated in adults with WPW syndrome and some drugs like verapamil increase danger of ventricular fibrillation and therefore use of drugs are a topic of concern. We present a case of 25-year-old primi gravida 31 weeks 5 days woman was admitted with oligohydramnios. Patient known case of WPW syndrome not on treatment showing ECG changes of short PR interval and delta wave slurring along the QRS complex with heart rate of 98 beats/ min and echo demonstrated mild tricuspid regurgitation (TR) and mild pulmonary arterial hypertension (PAH). Patient was taken for emergency LSCS in view of Doppler changes (CPR less than 1) and delivered an alive preterm baby.

Nonischemic Super-Responders in Fusion CRT Pacing with Normal Atrioventricular Conduction.

Background: Fusion CRT pacing (FCRT) is noninferior to biventricular pacing, according to the current data. The aim of this study is to assess the response to FCRT and to identify predictors of super-responders (SRs) in a nonischemic population with normal AV conduction. Methods: LV-only CRT patients (pts) with a right atrium/left ventricle pacing system implanted in two CRT centers in Romania were included. Device interrogation, exercise tests, echocardiography, and individualized drug optimization were performed every 6 months during close follow-up. SRs pts were defined as those with left ventricular end-systolic volume (LVESV) improvement ≥30% and stable ejection fraction (LVEF) ≥45%. Results: A total of 25 out of 83 pts (31%) were SRs, with nonischemic LBBB low EF cardiomyopathy (50 male, 62 ± 9 y.o.) initially included. Mean follow-up was 5 years ± 27 months. Patients were divided in two groups: SRs and non-SRs (52 responders/6 hypo-responders). Two predictors were found in the SRs group: a higher baseline LVEF (SRs 29 ± 5% vs. non-SRs 26 ± 5%, p = 0.02) and a lower pulmonary arterial systolic pressure (SRs 38 ± 11 mm Hg vs. non-SRs 50 ± 15 mmHg, p = 0.003). Baseline severe mitral regurgitation was found in 11% of SRs vs. 64% in the non-SRs group. Conclusions: SRs in the selected NICM-FCRT group are significative high. Higher baseline LVEF and mild pulmonary arterial hypertension were independently associated with super-response.

Profiles and treatment patterns of patients with pulmonary arterial hypertension on monotherapy at experienced centres.

Guideline recommendations highlight the critical role of combination therapy for the treatment of pulmonary arterial hypertension (PAH). Conversely, registry data demonstrate that a considerable number of PAH patients remain on monotherapy. The reasons for this discrepancy remain elusive. The aim of this study was to assess the patient profiles, treatment patterns, and disease characteristics of patients diagnosed with PAH who were kept on monotherapy at experienced pulmonary hypertension (PH) centres and to capture potential reasons for monotherapy. We analysed the patient profiles of 182 patients on monotherapy with PAH-targeted drugs, managed at experienced PH expert centres (Cologne, Giessen, Heidelberg, and Dresden). Patients were identified based on their latest follow-up visit and analysed retrospectively from the time of PAH diagnosis to last follow-up. Patients were dichotomized by age, and patient characteristics, treatment patterns, response to therapy, change in risk status, and drug tolerability were recorded during the course of their disease. Patients' mean age was 69.1±13.1years at the most recent follow-up (Key Time Point 1) and 64.5±14.9years at the time of diagnosis (Key Time Point 2). The mean time on monotherapy was 60.7±53.8months; 35.7/64.3% of patients were male/female. The majority (66.5%) had idiopathic PAH, followed by PAH associated with connective tissue disease (17.0%) and portopulmonary PH (8.2%). Among patients on monotherapy, there were five main clusters: (i) patients with failed escalation attempts mostly because of intolerability (26.9%); (ii) low risk on monotherapy, favourable response, and no reason for escalation (24.2%); (iii) patients with mild PAH (36.3%); (iv) elderly patients with PAH and multiple co-morbidities (38.5%); and (v) patients with associated forms of PAH where the level of evidence for combination therapies is considered low (16.5%). There were substantial differences between patients above or below the median age (68years). The most frequently used monotherapy for PAH was phosphodiesterase type 5 inhibitors (75.3%). A considerable number of PAH patients are on monotherapy at large PH expert centres, characterized by specific reasons that justify this kind of treatment. Nevertheless, as comprehensive treatment strategies have shown improved long-term outcomes even in mildly symptomatic patients, each case of monotherapy should be justified.

Transcatheter closure of ventricular septal defects: preliminary results in children weighing 10 kg or less.

Ventricular septal defect is one of the commonest heart defect in children and closure of this defect with devices has seen a rapid progression over a period of time. The availability of new and safer devices has made the transcatheter closure of ventricular septal defect a suitable option even in young children. The study was done to evaluate the feasibility and complications of device closure of ventricular septal defect in children weighing 10 kg or less with different types of devices. The present study was undertaken in a newly established dedicated Paediatric Cardiac Unit at a Tertiary Care Hospital. Relevant data were obtained retrospectively from the case files and the catheterisation records and data were analysed for first 50 patients with ventricular septal defect weighing 10 kg or less between March 2018 and March 2021. Among these 50 patients selected, device closure was successfully done in 45 (90%) cases while 5 (10%) attempts were unsuccessful for various reasons. The mean weight in this study was 7.46 ± 1.89 kg (2.3-10 kg), 21 (42%) cases were females while 29 (58%) were males; mean age was 19.4 ± 11.88 months (4-48 months). Right heart catheterisation study showed 21 (42%) patients with normal pulmonary artery pressures (no pulmonary artery hypertension). Among 29 patients with pulmonary arterial hypertension, 13 patients (22%) were having mild pulmonary arterial hypertension, 4 (8%) were with moderate pulmonary arterial hypertension, and 12 (24%) were with severe pulmonary arterial hypertension. Mean Qp/Qs was 2.73 ± 0.72 (2.5-4.5) and mean pulmonary vascular resistance was 1.5 ± 1.04 (0.6-4.6 WU). Amplatzer Duct Occluder (ADOI) was used in 15 (30%) cases, 27 (52%) cases were closed with Amplatzer Duct Occluder (ADOII), and the 3 (6%) cases closure was done with Amplatzer muscular ventricular septal defect occluder. Transcatheter closure of ventricular septal defect in children 10 kg or less is feasible and safe alternative to surgical ventricular septal defect closure. The immediate and short-term outcomes have proven this method to be safe and valid.

Pregnancy outcome analysis of 94 patients with pulmonary arterial hypertension during pregnancy.

The aim of the study was to analyze the pregnancy outcomes of patients with pulmonary arterial hypertension to provide a reference for clinical diagnosis and treatment. Clinical data of 94 patients with a pregnancy complicated by pulmonary hypertension were retrospectively analyzed. The means and percentages of the pregnancy outcomes were calculated, and the analysis of variance, Chi-square test, and Fisher's exact test were applied for comparison. The pregnancy outcomes were less favorable in the severe pulmonary arterial hypertension group compared to the mild and moderate groups. The more severe the pulmonary arterial hypertension, the worse the heart function. A poorer heart function was associated with a poorer prognosis across different pregnancy outcomes. A pregnancy with more severe pulmonary arterial hypertension and worse cardiac function has a poorer maternal and infant prognosis and pregnancy outcome. Cesarean section is the preferred delivery method for patients with severe pulmonary arterial hypertension, whereas vaginal delivery is preferred for patients with mild or moderate pulmonary arterial hypertension and good cardiac function.

Prevalence of pulmonary arterial hypertension in severe chronic obstructive pulmonary disease patients attending tertiary care centre Ernakulam

Background: Chronic obstructive pulmonary disease (COPD) is an irreversible bronchial inflammation of lung airways and parenchyma. Various factors play an important role in occurrence and severity of pulmonary arterial hypertension (PAH), a common in severe COPD. The objective of the study is to know the proportion of PAH in patients with severe COPD and to find the association between various factors.Methods: 180 cases of severe COPD patients admitted in Government Medical College Ernakulum from January 2019-December 2019, were enrolled into this cross-sectional descriptive study. Subject’s history, clinical examination, anthropometric measurements, vitals, Arterial Blood Gas (ABG) analysis were done.Results: Among 180 subjects, 148 (82.22%) had mild PAH, 22 (12.22%) subjects had moderate PAH and 10 (5.56%) had severe PAH. Use of accessory muscle was the most elicited sign in the study with 174 (96.67%). 170 (94.44%) had cough and 169 (93.89%) had breathlessness which were the most reported symptoms. Demographic variables and clinical features had no significant mean pulmonary artery pressure (mPAP) association. Grade 3 PAH groups were elder than others, which was statistically significant (p&lt;0.047). FEV1, Oxygen Saturation and ejection fraction were lowest in grade 3 PAH subjects. Respiratory rate, hemoglobin, PCV, tricuspid velocity, PaCO2 were highest in grade 3 PAH subjects being statistically significant (p&lt;0.0001).Conclusions: In our study, majority of severe COPD patients had mild PAH. There was an independent correlation between respiratory rate, hemoglobin, PCV, tricuspid regurgitant velocity and PaCO2 with severity of PAH.

Ventricular mass discriminates pulmonary arterial hypertension as redefined at the SixthWorld Symposium on Pulmonary Hypertension.

Cardiac magnetic resonance (CMR) measures of right ventricular (RV) mass, volumes, and function have diagnostic and prognostic value in pulmonary arterial hypertension (PAH). We hypothesized that RV mass‐based metrics would discriminate incident PAH as redefined by the lower mean pulmonary arterial pressure (mPAP) threshold of >20 mmHg at the Sixth World Symposium on Pulmonary Hypertension (6th WSPH). Eighty‐nine subjects with suspected PAH underwent CMR imaging, including 64 subjects with systemic sclerosis (SSc). CMR metrics, including RV and left ventricular (LV) mass, were measured. All subjects underwent right heart catheterization (RHC) for assessment of hemodynamics within 48 h of CMR. Using generalized linear models, associations between CMR metrics and PAH were assessed, the best subset of CMR variables for predicting PAH were identified, and relationships between mass‐based metrics, hemodynamics, and other predictive CMR metrics were examined. Fifty‐nine subjects met 6th WSPH criteria for PAH. RV mass metrics, including ventricular mass index (VMI), demonstrated the greatest magnitude difference between subjects with versus without PAH. Overall and in SSc, VMI and RV mass measured by CMR were among the most predictive variables discriminating PAH at RHC, with areas under the receiver operating characteristic curve 0.86 and 0.83. respectively. VMI increased linearly with pulmonary vascular resistance and with mPAP in PAH, including in lower ranges of mPAP associated with mild PAH. VMI ≥ 0.37 yielded a positive predictive value of 90% for discriminating PAH. RV mass metrics measured by CMR, including VMI, discriminate incident, treatment‐naïve PAH as defined by 6th WSPH criteria.

Effects of a diuretic regimen on body weight and echocardiographic parameters of the tricuspid valve in patients scheduled for transcatheter tricuspid annuloplasty

Abstract Background Echocardiographic severity of tricuspid regurgitation is known to be highly variable and strongly related to the volume status of the individual patient. Interventional therapies for tricuspid valve repair or replacement are dependent on reliable measurements in order to select the appropriate device size. Purpose In patients scheduled for transcatheter tricuspid valve annuloplasty, we sought to evaluate the effects of a diuretic regimen started at the screening visit on body weight and tricuspid valve dimensions at the time point of transcatheter intervention. Methods After successful screening for transcatheter tricuspid annuloplasty, an individualized intensified diuretic regimen was initiated in 15 patients. At baseline and at the timepoint of the intervention, body weight and 3D echocardiographic dimensions of the tricuspid valve were documented and compared. Results The cohort comprised 6 males and 9 females (mean age 77±7 years) with severe or higher degree functional tricuspid regurgitation. In all patients not more than mild pulmonary artery hypertension was present (PaP mean 29±5 mmHg). The interval between the screening visit and the timepoint of the procedure was 64±50 days. At baseline mean body weight was 72±12 kg. In 11 patients a reduction of the body weight could be achieved, with a median weight reduction of 2.2 kg (min/max 1.2/6.4 kg). Overall, the average relative reduction of the tricuspid annulur perimeter was 13% (p&amp;lt;0,001), of the tricuspid valve area 2.7% (n.s.), the anterseptal diameter 3.3% (n.s.), while the bicommissural diameter remained almost unchanged (0.4%; n.s.). The average predicted required band length changed by only 1.5% (n.s.). The strongest correlations were found between the absolute change in body weight and the change of the tricuspid valve area (r=0.48), relative change in body weight and relative change of tricuspid valve area (r=0.52), absolute change in body weight and absolute change in predicted band length (r=0.48), and relative changes of the respective variables (r=0.52). More importantly, in 13 out of 15 patients the band size which would have been selected was identical between the screening and the annuloplasty procedure. Only in two patients a smaller device was implanted than predicted at the screening visit. Conclusion Instituting a diuretic therapy in candidates for transcatheter tricuspid annuloplasty prior to the procedure helps to keep tricuspid valve dimensions in a stable range. Changes in body weight in patients with functional tricuspid regurgitation are correlated to changes of tricuspid valve dimensions. Funding Acknowledgement Type of funding sources: None.

Erythema marginatum: an uncommon presentation of acute rheumatic fever in a Nigerian adolescent girl\u2014a case report

BackgroundErythema marginatum is an uncommon presentation in children with acute rheumatic fever and it is one of the major criteria needed to make a diagnosis. It is seen in less than 10% of cases. It is also reported to be difficult to detect in black-skinned children. This is the first and only patient to present with the above since the inception of the unit about 14 years ago and also the first to be reported in Nigeria as far as the authors are aware, after a careful literature search; hence, we report this case based on the rarity of this symptom of acute rheumatic fever.Case presentationThis is a case report of O.E, a 12-year-old Nigerian girl who presented with features of acute rheumatic fever, and these features included the rare manifestation of erythema marginatum. She presented with generalized skin eruptions on the trunk and extremities, sparing the face, migratory polyarthritis, features of congestive heart failure and high grade continuous fever. The skin lesions consisted of papules, patches, plaques and polycycles with a reticular pattern having serpiginous and raised borders. Diagnostic investigations revealed elevated erythrocyte sedimentation rate of 83mm/h, anti-streptolysin O titer of 2020IU/L and echocardiography which showed thickened mitral valves with grade II mitral regurgitation and a mild pulmonary artery hypertension. The patient was treated with anti-inflammatory and anti-failure drugs and commenced secondary prophylaxis with benzathine penicillin. Skin eruptions resolved within 3 weeks of management and are currently on follow up.ConclusionsWe present the above to increase awareness on the possibility of acute rheumatic fever presenting with erythema marginatum in our region, to encourage early diagnosis of acute rheumatic fever to reduce morbidity and mortality from its sequel, rheumatic heart disease.

Soluble Receptor for Advanced Glycation End Products (sRAGE) Is a Sensitive Biomarker in Human Pulmonary Arterial Hypertension.

Pulmonary arterial hypertension (PAH) is a progressive condition with an unmet need for early diagnosis, better monitoring, and risk stratification. The receptor for advanced glycation end products (RAGE) is activated in response to hypoxia and vascular injury, and is associated with inflammation, cell proliferation and migration in PAH. For the adult cohort, we recruited 120 patients with PAH, 83 with idiopathic PAH (IPAH) and 37 with connective tissue disease-associated PAH (CTD-PAH), and 48 controls, and determined potential plasma biomarkers by enzyme-linked immunoassay. The established heart failure marker NTproBNP and IL-6 plasma levels were several-fold higher in both adult IPAH and CTD-PAH patients versus controls. Plasma soluble RAGE (sRAGE) was elevated in IPAH patients (3044 ± 215.2 pg/mL) and was even higher in CTD-PAH patients (3332 ± 321.6 pg/mL) versus controls (1766 ± 121.9 pg/mL; p < 0.01). All three markers were increased in WHO functional class II+III PAH versus controls (p < 0.001). Receiver-operating characteristic analysis revealed that sRAGE has diagnostic accuracy comparable to prognostic NTproBNP, and even outperforms NTproBNP in the distinction of PAH FC I from controls. Lung tissue RAGE expression was increased in IPAH versus controls (mRNA) and was located predominantly in the PA intima, media, and inflammatory cells in the perivascular space (immunohistochemistry). In the pediatric cohort, plasma sRAGE concentrations were higher than in adults, but were similar in PH (n = 10) and non-PH controls (n = 10). Taken together, in the largest adult sRAGE PAH study to date, we identify plasma sRAGE as a sensitive and accurate PAH biomarker with better performance than NTproBNP in the distinction of mild PAH from controls.

Systemic sclerosis-associated pulmonary arterial hypertension in children.

Systemic sclerosis (SSc) is a rare disease in childhood and is characterized by a combination of vasculopathy, inflammation, autoimmunity, and fibrogenesis with individually varying expression pattern. Pulmonary arterial hypertension (PAH) is a serious complication of SSc and affects approximately 10% of SSc patients. SSc-PAH is complex and difficult to diagnose, as symptoms are non-specific and may be complicated by other SSc-associated diseases such as interstitial lung disease or left heart disease. SSc-PAH patients can deteriorate rapidly, and disease progression can occur even in patients with mild PAH symptoms at diagnosis. Therefore, interdisciplinary care of SSc patients is essential, and treating physicians must be aware of the association between SSc and PAH. In order to detect PAH early, children with SSc should be regularly screened for PAH by pediatric cardiologists. If PAH is detected, a systematic diagnostic approach by trained PH specialists including careful phenotyping of PAH is required. Relevant interstitial lung disease and left heart disease should be ruled out in the differential diagnosis of SSc-PAH before starting any targeted therapy. Due to the progressive character of SSc-PAH known from adult studies, it appears appropriate to initiate targeted PAH-therapy in juvenile SSc-PAH early. Adapted from adult treatment algorithms, combination therapy regimens (addressing at least two pathophysiological pathways) are increasingly used for pediatric PAH patients, and there is growing evidence to support this approach also in SSc-PAH patients.