Mild PAH Research Articles

Expression and Diagnostic Value of miR-3591-5p in Patients with Congenital Heart Disease-Associated Pulmonary Arterial Hypertension.

This study explored the expression and diagnostic value of differentially expressed miR-3591-5p in congenital heart disease-associated pulmonary arterial hypertension (CHD-PAH). A total of 110 CHD patients were divided into four groups based on their mean pulmonary artery pressure (PAPm). The plasma miR-3591-5p expression was determined by reverse transcription polymerase chain reaction. The correlation between the miR-3591-5p expression and various clinical indices, as well as its diagnostic value for CHD-PAH patients, were analyzed. The plasma levels of miR-3591-5p were significantly higher in the patients in the no PAH group, mild PAH group, and moderate to severe PAH group than in the control group, and they were significantly higher in the moderate to severe PAH group than in the no PAH group. Correlation analysis revealed that the miR-3591-5p expression level was significantly positively correlated with various clinical indicators, including the PAPm, pulmonary artery systolic pressure, brain natriuretic peptide, pulmonary vascular resistance, red blood cell distribution width, uric acid, Na + , systolic blood pressure, left atrial internal dimension, left ventricular end-diastolic dimension, and left ventricular end-systolic dimension. Univariate and multivariate regression analyses identified the plasma miR-3591-5p level as an independent risk factor for CHD-PAH. Receiver operating characteristic curve analysis demonstrated that the plasma miR-3591-5p level had a moderate diagnostic value for CHD-PAH, which was further improved when combined with a B-type natriuretic peptide. This study identified the expression profiles of differentially expressed plasma miRNAs in patients with CHD-PAH, focusing on the upregulation of miR-3591-5p. Bioinformatics analysis suggested that miR-3591-5p is involved in the pathogenesis of CHD-PAH and may serve as a circulating biomarker that may have diagnostic and prognostic value in CHD-PAH.

Identification of mild PAH deficiency through expanded carrier screening, undetected via newborn screening process

Identification of mild PAH deficiency through expanded carrier screening, undetected via newborn screening process

Diagnostic values of serum BNP, PTX3, and VEGF in acute pulmonary embolism complicated by pulmonary artery hypertension and their correlations with severity of pulmonary artery hypertension.

This paper aimed to unveil the diagnostic values of serum brain natriuretic peptide (BNP), pentraxin 3 (PTX3), and vascular endothelial growth factor (VEGF) in acute pulmonary embolism complicated by pulmonary artery hypertension (APE-PAH) and their correlations with severity of PAH. A total of 153 patients with APE were selected for our study and divided into the PAH and Non-PAH groups according to the measurement of pulmonary artery pressure by echocardiography. Serum BNP levels were measured by chemiluminescence immunoassay, and serum PTX3 and VEGF levels were appraised by ELISA. The predictive values of BNP, PTX3, and VEGF for APE-PAH were evaluated by applying the receiver operating characteristic (ROC) curve. Spearman test was implemented to correlate BNP, PTX3, and VEGF with the severity of PAH. Higher serum levels of BNP, PTX3, and VEGF were observed in the PAH group versus the Non-PAH group (p < .05). ROC curve analysis indicated that BNP, PTX3, and VEGF had acceptable diagnostic value for predicting APE-PAH. Higher serum levels of BNP, PTX3, and VEGF were witnessed in the moderate and severe PAH groups in contrast to the mild PAH group (p < .05), and the levels of these parameters were elevated in the severe PAH group versus the moderate PAH group (p < .05). Spearman correlation analysis signified that serum BNP (r = 0.377), PTX3 (r = 0.488), and VEGF (r = 0.575) levels were positively correlated with the severity of PAH in APE-PAH patients. Serum BNP, PTX3, and VEGF levels are significantly elevated in APE-PAH patients. Serum BNP, PTX3, and VEGF levels are of clinical value in the diagnosis of APE-PAH patients, and serum BNP, PTX3, and VEGF levels are positively correlated with the severity of PAH and can be used as predictors of the severity of PAH.

Retrospective cohort study of pulmonary arterial hypertension associated with connective tissue disease effect on patients' prognosis.

The objectives of this study are to clarify clinical characteristics and recognize prognostic factors of CTD-PAH patients. A retrospective cohort study of consecutive patients with documented CTD-PAH diagnosis from Jan 2014 to Dec 2019 was conducted, the ones who have other comorbid conditions that cause PH were excluded. Survival functions were plotted using the Kaplan-Meier method. Univariable and multivariable Cox regression analysis was applied to determine the survival-related factors. In 144 patients with CTD-PAH analyzed, the median sPAP value was 52.5 (44.0, 71.0) mmHg, the overall targeted drug usage rate was 55.6%, and only 27.5% patients were given combination. Twenty-four non-PAH-CTD patients with sPAP value were included as the control group. Compared with non-PAH-CTD groups, CTD-PAH patients had worse cardiac function, higher NT-pro BNP and γ-globulin level, and lower PaCO2 level. Compared with the mild PAH group, the moderate-severe PAH group had worse cardiac function; increased Hb, HCT, and NP-pro BNP level; and decreased PaO2. Kaplan-Meier analysis showed significant difference for survival among non-PAH-CTD, mild CTD-PAH, and moderate-severe CTD-PAH groups. The univariate analyses showed that Hb, pH, and Ln (NT-pro BNP) were identified as factors significantly associated with survival, and Hb and pH showed significant association with risk of death in the multivariate model. Kaplan-Meier analysis also showed that Hb > 109.0 g/L and pH > 7.457 affected CTD-PAH patients' survival significantly. PAH is not rare in CTDs patients; PAH affects CTD patients' prognosis significantly. Higher Hb and pH were associated with an increased risk of death. Key Points • Pulmonary arterial hypertension affects connective tissue disease patients' prognosis significantly. • The significantly factors associated with survival is hemoglobin, pH, and Ln (NT-pro BNP).

Multisystem Inflammatory Syndrome in Children [MIS-C] - A Case Report

After the appearance of COVID 19 infection, surging number of nations reported children with inflammatory syndrome, presented with clinical features that overlaps with Kawasaki disease, Toxic shock syndrome, Macrophage Activation Syndrome. Intravenous immunoglobulin with or without steroid or aspirin is the preferred management for MIS-C. Here we report a case of 6-y-old girl who was managed by intravenous steroids alone. On examination, the child had bilateral Conjunctival congestion with persisting high grade fever. Initial investigations exhibited elevated inflammatory parameters such as ESR and CRP. Echocardiography displayed grade 2 Mitral regurgitation and grade 2-3 tricuspid regurgitation with mild PAH. Cardiac isoenzymes CK-MB was also elevated, suggesting myocardial cell wall injury. She was treated with antibiotics, steroids and antipyretic. RT PCR for SARS-CoV-2 was negative but antibody test was positive. Her clinical condition improved and became afebrile from day 5, inflammatory parameters were decreased and was discharged after 11 days of hospital stay.

Evaluation of pulmonary arterial pressure in patients with connective tissue disease-associated pulmonary arterial hypertension by myocardial perfusion imaging.

BackgroundPulmonary arterial hypertension (PAH) is a complex and severe complication of connective tissue disease (CTD). We aimed to evaluate the application value of myocardial perfusion imaging (MPI) in evaluating CTD‐associated PAH (CTD‐PAH).MethodsWe retrospectively included 88 patients who were diagnosed with CTD between January 2018 and December 2020 at our hospital. Fifty‐eight patients had PAH and were included into the CTD‐PAH group. Thirty patients without PAH were included in the control group. All patients received routine physical examination, biochemical tests and cardiac function evaluation, right heart catheterization (RHC), and 99mTc‐MIBI MPI. PAH patients were divided into the mild, moderate, and severe PAH group according to their mean pulmonary artery pressures by RHC. Pearson correlation analysis was used to calculate the correlation between the right ventricle target/background (T/B) and right ventricle stroke volume (RV‐SV), total pulmonary resistance (TPR), pulmonary vascular resistance (PVR), mean pulmonary arterial pressure (mPAP), 6‐minute walk distance (6‐MWD), and N‐terminal B‐type natriuretic peptide (NT‐proBNP). The ROC curves of T/B and pulmonary artery pressure classification were plotted and the sensitivity and specificity of T/B in diagnosing PAH of different severities were analyzed.ResultsThe analysis of correlation revealed that T/B correlated negatively with 6‐MWD and positively with NT‐proBNP and exhibited good positive correlation with mPAP, TPR, and PVR by RHC and negative correlation with RV‐SV. T/B was of the most diagnostic value for severe PAH, and its correlation with severe PAH was stronger than that with mild PAH and moderate PAH.ConclusionsTarget/background is a noninvasive method that can simultaneously evaluate pulmonary arterial pressure and myocardial perfusion of CTD‐CHD patients and is particularly of relatively high value for severe PAH patients.

Single-cell RNA sequencing reveals thatBMPR2mutation regulates right ventricular functionvia IDgenes

BackgroundMutations in bone morphogenetic protein type II receptor (BMPR2) have been found in patients with congenital heart disease-associated pulmonary arterial hypertension (CHD-PAH). Our study aimed to clarify whether deficient BMPR2 signalling acts through downstream effectors, inhibitors of DNA-binding proteins (IDs) during heart development to contribute to the progress of PAH in CHD patients.MethodsTo confirm that IDs are downstream effectors of BMPR2 signalling in cardiac mesoderm progenitors (CMPs) and contribute to PAH, we generated cardiomyocyte-specific Id 1/3 knockout mice (Ids cDKO), and 12 out of 25 developed mild PAH with altered haemodynamic indices and pulmonary vascular remodelling. Moreover, we generated ID1 and ID3 double-knockout (IDs KO) human embryonic stem cells that recapitulated the BMPR2 signalling deficiency of CHD-PAH induced pluripotent stem cells (iPSCs).ResultsCardiomyocytes differentiated from iPSCs derived from CHD-PAH patients with BMP receptor mutations exhibited dysfunctional cardiac differentiation and reduced calcium (Ca2+) transients, as evidenced by confocal microscopy experiments. Smad1/5 phosphorylation and ID1 and ID3 expression were reduced in CHD-PAH iPSCs and in Bmpr2+/– rat right ventricles. Moreover, ultrasound revealed that 33% of Ids cDKO mice had detectable defects in their ventricular septum and pulmonary regurgitation. Cardiomyocytes isolated from mouse right ventricles also showed reduced Ca2+ transients and shortened sarcomeres. Single-cell RNA sequencing analysis revealed impaired differentiation of CMPs and downregulated USP9X expression in IDs KO cells compared with wild-type cells.ConclusionWe found that BMPR2 signals through IDs and USP9X to regulate cardiac differentiation, and the loss of ID1 and ID3 expression contributes to cardiomyocyte dysfunction in CHD-PAH patients with BMPR2 mutations.

BMPR2 promoter methylation and its expression in valvular heart disease complicated with pulmonary artery hypertension.

Valvular heart disease (VHD) is a common heart disease that affects blood flow. It usually requires heart surgery. Valvular heart disease complicated with pulmonary artery hypertension (VHD-PAH) may be lethal due to heart failure that results from increased heart burden. It is important for these patients to seek early treatment in order to minimize the heart damage. However, there is no reliable diagnosis method in VHD. In this study, we found DNA methylation was increased at the promoter of BMPR2 gene in the VHD patients compared with the healthy controls. This finding was confirmed by an independent cohort study of VHD patients and healthy controls. In addition, BMPR2 mRNA levels were reduced in the plasma of the VHD patients. There is strong correlation between BMPR2 promoter DNA methylation and the severity of VHD. Indeed, we found that both BMPR2 promoter DNA methylation and BMPR2 mRNA levels in the plasma are good biomarkers of VHD by themselves, with the respective AUC value of 0.879 and 0.725, respectively. When they were used in combination, the diagnostic value was even better, with the AUC value of 0.93. Consistent with the results in the VHD patients, we observed decreased BMPR2 and increased fibrosis in the lung of a PAH model mouse. BMPR2 was also decreased in the hearts of the PAH mice, whereas BMP4 was increased. Furthermore, BMPR2 was reduced in the heart valve tissue samples of human VHD patients after valve replacement with moderate/severe PAH compared with those with mild PAH. There was also increased apoptosis in the hearts of the PAH mice. BMPR2 promoter DNA methylation and its expression appear to be good biomarkers for VHD. Our results also suggest that DNA methylation may cause PAH through deregulation of BMP signaling and increased apoptosis.

Early Outcome of Mitral Valve Replacement in Patient Having Mitral Stenosis with Moderate Pulmonary Hypertension

Background: Mitral valve replacement (MVR) plays a central role in the management of patients with mitral stenosis with moderate to severe pulmonary hypertension. Pulmonary hypertension has an impact on short term outcome of MVR. It can influence left ventricular function (low output syndrome), incidence of arrhythmia, ARDS leading to respiratory failure and right ventricular failure which may be irreversible.&#x0D; Methods: The immediate postoperative hemodynamics in 40 patients with moderate to severe pulmonary arterial hypertension who underwent mitral valve replacement (BLMV) between July 2010 and June 2012 were studied prospectively. Patients were divided into two groups: Group A (n=20): Patient having MS with moderate pulmonary hypertension (PASP: 40-59 mm-Hg) and Group B (n=20): Patient having MS with severe pulmonary hypertension (PASP e” 60mm-Hg). Total two follow up were done- 1st follow up after 10 days and 2nd follow up after 1 month of MVR. Each patient was assessed by medical history, clinical examination &amp; color doppler echocardiogram.&#x0D; Results: It was shown that surgery can be beneficial for the patients if MVR is done in the state of mild PAH irrespective of age but beyond this level of PAH, the patients may still remain with mild pulmonary hypertension which may trigger the cascade of pulmonary vascular Disease may be the cause of unsatisfactory outcome. So, early surgical outcome of mitral stenosis with moderate pulmonary hypertension is better than mitral stenosis with severe pulmonary hypertension.&#x0D; Conclusion: We conclude that MVR in patients having MS with moderate PAH is a safe and effective measures for preventing pulmonary hypertension related complications.&#x0D; Cardiovasc. j. 2021; 13(2): 177-182

Abstract 15259: Breast Cancer and Heritable Pah Due to Bmpr2 Mutation: An Unexpected Discovery

Introduction: BMPR2 mutation is reported in 80% of familial PAH and 20% of idiopathic cases. Recently, a rat model of hereditary Pulmonary Arterial Hypertension with a BMPR2 loss-of-function mutation has been generated. Similarly to human, only around 20% of the rats spontaneously developed mild PAH. Unexpectedly, we observed that 20% (95%CI:12-28) of the 82 Bmpr2+/Δ71 rats (93% of females) versus 4% (95%CI:0-9)of the 84 age-matched wild type littermates spontaneously developed mammary masses requiring euthanasia. Interestingly, rats with tumor had elevated PA and clearly exhibit PAH signs. Hypothesis: We hypothesized that mammary tumor exacerbates the penetrance of PAH in the BMPR2 mutated female rats. Methods/Results: BMPR2 mutated and wild-type female young rats were treated with the breast cancer inducer 7, 12- diméthylbenz(a)anthracene (DMBA) or Vehicle. DMBA causes on average more tumors development in BMPR2 mutated rats versus WT rats (6,2 vs. 4) P=0,0275. Compare to both vehicle-treated and DMBA rats (without tumor) BMPR2 mutated rats with DMBA-induced tumors had a significant increase in mean PA pressure (p&lt;0,0001); total pulmonary resistance (p&lt;0,0001) and a significant decrease in cardiac output (P=0,0293). Histologically, these changes were associated with an increase of pulmonary artery remodelling assessed by immunofluorescence. Mechanistically, development of PAH in the BMPR2 mutated rats with tumor was associated with a significant upregulation of BRD4 (western blot) (p=0,0149); IL-1β (P=0,0052), BRD3 (P=0,0791) and BIRC5 (P=0,0166) mRNA expression levels. Co-culture experiments with breast cancer cells and PASMC from both WT and BMPR2 mutated rats are ongoing to identify whether cancer cells are realising factors responsible for BRD4 activation and thus PASMC proliferation and resistance to apoptosis. Conclusions: We demonstrated for the first time that breast cancer exacerbates PAH development in BMPR2 mutated rats. Increased BRDs driven pro-inflammatory signalling is associated with PAH development in BMPR2 mutated rats. We are currently exploring whether BMPR2 mutated patients are more prone to breast cancer development or not and whether PAH development occurs more frequently in breast cancer patients.

Factors associated with pulmonary artery hypertension among premature infants with bronchopulmonary dysplasia in Vietnam

BackgroundPulmonary artery hypertension (PAH) is a common complication among premature infants with bronchopulmonary dysplasia (BPD). Factors associated with PAH among premature Vietnamese infants with BPD are unknown. The purpose of this study was to describe the epidemiological and clinical features of premature infants with BPD who had PAH and to identify the factors associated with PAH in these patients. MethodsThis descriptive study included 150 infants with BPD admitted to VNCH from August 2017 to July 2018.Clinical data were collected for all BPD patients. PAH screening was performed by echocardiography. ResultsPAH was more common among male BPD patients (71.9%). Median gestational age was 29 ± 2.1 weeks, 96.5% of cases were <32 gestational weeks of age. Median birth weight was 1100 ± 305.1 g. The prevalence of severe, moderate, mild PAH was 52.6%; 35.1%; 12.3%, respectively. Mean pulmonary pressure was 39.9 ± 7.8 mmHg, the majority had mild PAH (66.7%).The threshold level of N-terminal-pro-brain natriuretic peptide for PAH in BPD patients was 546 ng/L. Prematurity, low birth weight, severe BPD, long-lasting oxygen-dependence were significantly related to PAH (P < 0.05).PAH-associated factors were: no prenatal corticosteroid administration (OR, 2.1;95% CI,1.1–4.1;P = 0.04), oligohydramnios (OR,4.2; 95% CI,1.4–12.8; P = 0.01), inflammation of amniotic membrane (OR, 5.7; 95% CI,2.1–15.5; P = 0.001). ConclusionsPAH was common among premature infants with BPD. Prematurity, low birth weight, severe BPD, long-lasting oxygen-dependence were related to PAH in BPD patients.

Treprostinil improves hemodynamics and symptoms in children with mild pulmonary hypertension awaiting heart transplantation.

Treprostinil, a prostacyclin analog, is a safe and effective therapy for children with PAH; however, the use of this agent in children with mild PVR elevations related to HF, including those with SV congenital heart disease awaiting HT, is understudied. We describe the hemodynamic and symptomatic changes in pediatric patients awaiting HT treated with treprostinil. Single-center retrospective review of all patients was listed for HT who received treprostinil during the listing period. Changes in hemodynamic and functional indices between the baseline catheterization (prior to drug initiation), and prior to HT, and patient outcomes were analyzed. Among 16/17 (94%) who survived to HT, 8 (50%) were female, and 10 (63%) had SV physiology. The median age at drug initiation was 9 (IQR: 1, 14) years. The median duration of therapy prior to HT was 253 (IQR: 148, 504) days. Treprostinil significantly decreased PVR (3.8 vs 3.1 WU, P=.03), while mLA or mPCW pressure did not change (11 vs 13mmHg, P=.9). HF symptoms improved in 9/15 (60%) patients without VAD support prior to drug initiation, including 4/10 (40%) who did not receive a VAD any point while awaiting HT. Treprostinil may be used safely in patients with mild PAH awaiting HT, including those with SV disease. PVR falls without substantial increases in mLA/mPCW pressure. HF symptoms improve in some patients.

Increased Pulmonary Vascular Impedance in Patients with Severe Pulmonary Arterial Hypertension

IntroductionPulmonary arterial hypertension (PAH) is characterized by increased pulmonary artery (PA) pressure and resistance due to pulmonary vascular remodeling. The functional effect of this vascular remodeling on the pulsatile pulmonary hemodynamics has not been well studied. Pulmonary vascular impedance (PVZ) represents the relationship between pulsatile pressure and flow in the pulmonary artery (PA) and provides a more comprehensive view of pulmonary arterial function. We hypothesized that decreased pulmonary vascular compliance and increased pulmonary vascular resistance (PVR) would significantly alter the pulsatile pulmonary hemodynamics in severe PAH.MethodsGroup 1 PAH patients (mean pulmonary arterial pressure (mPAP) ≥ 25 mmHg, pulmonary capillary wedge pressure ≤ 15 mmHg) and control patients (without pulmonary hypertension (PH), mPAP &lt; 25 mmHg) with both an echocardiogram including right ventricular outflow tract (RVOT) images and a right heart catheterization (RHC) within three months were identified from the University of Arizona PH registry. The PAH patients were divided into those with mild PH (PVR &lt; 7 mmHg*min/L) and severe PH (PVR ≥ 7 mmHg*min/L). RVOT Doppler flow velocity profiles were first traced using a custom MATLAB program. Flow velocity profiles were multiplied by the average PA diameter (ratio of thermodilution cardiac output and mean RVOT flow velocity) to determine PA flow profiles. PVZ was calculated in the frequency domain using RHC measured PA pressure and PA flow profiles. Characteristic Impedance (ZC) was calculated as the mean of Z2–4. Data is presented as mean ± standard error or median [interquartile range].ResultsFourteen subjects were identified (Control: n = 2, 2 female, 70 ± 3 yrs; Mild PAH: n = 6, 2 female, 63 ± 11 yrs; and Severe PAH: n = 6, 4 female, 45 ± 13 yrs, p = 0.05). The median time between the RHC and echo was 8 [1 − 15] days. Severe PAH patients have increased mPAP (60 ± 19 vs 38 ± 17 mmHg, p = 0.04) and end‐diastolic elastance (1.05 ± 0.43 vs 0.39 ± 0.3 mmHg/mL, p = 0.2), decreased stroke volume (61 ± 7 vs 91 ± 20 mL, p = 0.01) and no change in cardiac output (5.16 ± 0.81 vs 6.23 ± 1.3 L/min) compared to controls. Severe PAH showed increased 0 Hz impedance (Z0, 966 ± 138 dynes/sec/cm−5) compared to controls (288 ± 34 dynes/sec/cm−5, p = 0.02) and mild PAH (538 ± 64 dynes/sec/cm−5, p = 0.03, Figure 1). ZC in severe PAH had a non‐significant increase compared to controls (139 ± 23 vs 69 ± 19 dynes/sec/cm−5, respectively). Z1 was significantly increased in severe PAH compared to controls (52 ± 91 vs 5 ± 43 dynes/sec/cm−5, p = 0.02) but not compared to mild PAH (52 ± 91 vs 64 ± 54 dynes/sec/cm−5, p = 0.09).ConclusionPulmonary vascular impedance analysis in a small PAH cohort demonstrated that vascular resistance (Z0) and global vascular stiffness (Z1) are increased in severe PAH patients and that there is a non‐significant increase in proximal vascular stiffness (ZC).Support or Funding InformationThis work was supported in part by grants from the Arizona Biomedical Research Commission (ABRC NIA ADHS18‐198871 (RRV))This abstract is from the Experimental Biology 2019 Meeting. There is no full text article associated with this abstract published in The FASEB Journal.

PERSISTENT PULMONARY HYPERTENSION AFTER BALLOON MITRAL VALVULOPLASTY

Persistence of Pulmonary artery Hypertension (PPAH) in cases of Rheumatic Mitral stenosis following Percutaneous balloon Mitral valvuloplasty (BMV) is not uncommon. It has been found that in Patients of Mitral stenosis, with mild PAH the Pulmonary artery pressure and Pulmonary vascular resistance

Obstetric outcome in pregnancy complained with pulmonary hypertension

Objective: To identify whether pregnancy outcomes vary by the severity of pulmonary hypertension. Methods: A retrospective study was conducted on 78 cases of pregnancies complained with pulmonary hypertension who delivered in the First Affiliated Hospital, Sun Yat-sen University from 2006 to 2016.The selected cases were divided into three groups according to severity of pulmonary hypertension: mild pulmonary hypertension group (mild PAH group) was defined as a mean pulmonary artery pressure 30-49 mmHg, moderate pulmonary hypertension (moderate PAH group) as mean pulmonary artery pressure 50-69 mmHg and severe pulmonary hypertension (severe PAH group) as mean pulmonary artery pressure 70 mmHg or greater.The clinical features, risk pregnant complication, maternal and neonatal outcomes were described between these three groups.Analysis of variance, Chi-square test was used for statistical analysis. Results: The average age of mild, moderate and severe PAH group were (31±5) years old, (31±5) years old and (27±3) years old, respectively (P=0.050). The rate of natural fertilization (P=0.414), parity (P=0.527) and gestational age (P=0.165) were similar in these three groups. In 78 pregnancies with pulmonary hypertension, 64.9% of pregnancies in mild PAH group was NYHA Ⅰ, 50.0% of moderate PAH group was NYHA Ⅱ and 54.5% of severe PAH group was NYHA Ⅲ(P<0.001). There was no significant difference in the incidence of gestational diabetes mellitus (GDM, P=0.589), preeclampsia (P=0.942), premature rupture of membrane (PROM, P=0.276), scarred uterus (P=0.493) and postpartum hemorrhage (P=0.424). The cesarean section rate was 84.2%, 90.0% and 63.6% in three groups (P=0.208). However, neuraxial anesthesia was performed in 82.5% of cases in mild PAH and 90.0% of cases in moderate PAH, while 27.3% of cases of severe PAH underwent neuraxial anesthesia (P<0.001). The fetal outcome was similar in there groups.But the rate of admission of NICU was significantly different in three groups (P=0.011). Conclusions: Maternal and neonatal outcomes was similar in different severity of pulmonary hypertension.But the severity of pulmonary hypertension affect the type of anesthesia.Close monitoring during pregnancy and timely termination of pregnancy can improve the outcome of pregnancy.

Factors associated with disease progression in early-diagnosed pulmonary arterial hypertension associated with systemic sclerosis: longitudinal data from the DETECT cohort

ObjectivePulmonary arterial hypertension (PAH) is a severe complication of systemic sclerosis (SSc). In this longitudinal study, we aimed to identify factors associated with an unfavourable outcome in patients with SSc...

Distinctive metabolomic fingerprint in scleroderma patients with pulmonary arterial hypertension

BackgroundPulmonary arterial hypertension (PAH) in systemic sclerosis (SS) identifies a poor prognosis subset of patients. Recent studies suggested a “metabolic theory” on the development of pulmonary arterial hypertension. On this basis we performed a metabolomic study in order to evaluate whether differences in pulmonary arterial blood metabolites were identifiable in SS patients with increased pulmonary vascular resistance (PVR). MethodsWe studied 18 SS patients (age 58.7±15.6years) free of pulmonary fibrosis who underwent a right heart catheterization (RHC). A blood sample was collected during the RHC in the distal peripheral circulation of the pulmonary arteries to perform the metabolomic analysis. ResultsBased on PVR we divided the population into Group A (n=8; PVR=1.16±0.23WU) and Group B (n=10; PVR=2.67±0.67WU; p<0.001 vs Group A). No significant differences were identified in terms of anthropometric, clinical, echo and therapeutic characteristics. At RHC the 2 groups showed a difference in mean pulmonary pressure values (Group A: 20±4mmHg; Group B: 27±3.4mmHg; p=0.03), with mild PAH in Group B. We applied an OSC–PLS–DA with a clear clusterization; SSc patients with PAH showed an increase in acetate, alanine, lactate, and lipoprotein levels and a decrease in γ-aminobutyrate, arginine, betaine, choline, creatine, creatinine, glucose, glutamate, glutamine, glycine, histidine, phenylalanine, and tyrosine levels ConclusionsOur results suggest that, despite similar clinical and disease-related parameters, SSc patients who develop PAH have an unfavorable metabolic profile able to cause an impaired production of metabolites with protective effects on endothelial cells.

Cardiovascular manifestations in systemic lupus erythematosus in Dakar: Descriptive study about 50 cases

Systemic lupus erythematosus is a non-specific inflammatory disorder of an organ of unknown cause and autoimmune origin. Visceral injuries, including those cardiovascular, determine the prognosis of this disease primarily affecting women. The objectives of this study were to determine the frequency and describe the cardiovascular manifestations in systemic lupus erythematosus in a lupus population of the Dakar region. This is a multicenter prospective study descriptive and analytical conducted in the region of Dakar (Senegal) from 14 February 2011 to 2 July 2012. Patients were either hospitalized or monitored as outpatients. Included were all patients with lupus and meeting at least four criteria of the American College of Rheumatology of lupus disease classification 1997. All patients underwent physical examination, an electrocardiogram and an echocardiogram looking for cardiovascular damage. The collected data were entered into the Epi Info version 3.5.1 and processed with SPSS 16.0 software. Quantitative variables are described in the median and the qualitative workforce, percentage and frequency. We have included 50 patients. The average age of the population was 36.18 years. A female predominance is noted with a sex ratio man/woman of 0.09. Cardiovascular functional symptoms were dominated by dyspnea stage II to IV NYHA (26%) and palpitations (22%). The physical signs we have found were mainly tachycardia (40%), spontaneous turgor of the jugular veins (29%), a muffling of the heart sounds (29%) and a infandibulopulmonairy shock (18%). The frequency of cardiovascular events was 46%. Electrical cardiac events were dominated by sinus tachycardia (40%) of repolarization disorders (16.3%) type of ischemia, injury, ischemia injury, necrosis and hypertrophy with 18% atrial and left ventricular hypertrophy each. Furthermore, one case of BAV first degree at 280 ms was recorded. We found 19 cases of pericarditis including 2 tamponade, 3 cases of dilated cardiomyopathy hyperkinesias with impaired ejection fraction less than 35% and 8 patients with mild PAH important. In systemic lupus erythematosus, cardiovascular events are worrying and may remain asymptomatic for awhile. Their research must be systematic in order to treat early.

A Rare Case of Spontaneous Mitral Leaflet Perforation Leading to Severe Mitral Regurgitation

AbstractSpontaneous mitral leaflet perforations are rare. We are reporting a 60 year old hypertensive female patient admitted with pulmonary edema with clinically short systolic murmur at apex without ischemic changes on ECG. There is no h/o chest pain, fever, blunt trauma of chest. TTE no RWMA, good bi- ventricular function, a posteriorly directed eccentric moderate – severe mitral regurgitation, normal LA, LV, no vegetation or prolapsed. TEE after stabilization showed whole in body of AML at its midpoint leading to severe regurgitant jet in to LA with Vena contracta of 0.8 mm. All the papillary muscles were intact and there is no prolapse of leaflets and no vegetation. Cardiac catheterization revealed increased LVEDP and PCWP with mild PAH with normal coronary arteries.

Pulmonary hypertension: prevalence and risk factors

IntroductionPulmonary arterial hypertension (PAH), defined as a systolic pulmonary artery pressure above 35mmHg, is another vascular disease entity recently described in patients receiving hemodialysis. It is a major problem due to its high prevalence and morbidity and mortality. Its pathophysiological mechanism is just known and the strategies for its supported not yet defined. AimsTo determine the prevalence of PAH in our hemodialysis patients and its risk factors. MethodologySingle center descriptive and analytical cross-sectional study, including 111 hemodialysis patients who had benefit from a trans-thoracic cardiac Doppler ultrasound during 2014. A value greater than or equal to 35mmHg is considered PAH and classified as follows: mild PAH (3550mmHg), moderate PAH (5070mmHg), and severe pulmonary hypertension (>70mmHg). Patients with a high probability of secondary PAH, especially those with the following history: chronic obstructive pulmonary disease, pulmonary embolism, were not included. ResultsThe mean age was 44.3±14.2years. Among the 111 patients, 18 had pulmonary arterial pressure above 35mmHg corresponding to 16.22% of PAH prevalence. The average pressure was 45mmHg. Of these 18 patients, 11.8% had mild PAH, 3.4% moderate PAH and 0.8% severe PAH. The average hemodialysis duration was significantly associated with PAH (p=0.003); as well as valvular calcification (p=0.000), mitral regurgitation (p=0.001) and tricuspid regurgitation (p=0.002). ConclusionPrimary pulmonary hypertension is a major problem among our hemodialysis because of its high prevalence and its risk factors.