scholarly journals Effect of bosentan in pulmonary hypertension development in systemic sclerosis patients with digital ulcers

PLoS ONE ◽  
2020 ◽  
Vol 15 (12) ◽  
pp. e0243651
Author(s):  
Ivan Castellví ◽  
Carmen Pilar Simeón ◽  
Monica Sarmiento ◽  
Jordi Casademont ◽  
Hèctor Corominas ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Systemic Sclerosis ◽  
Statistical Significance ◽  
Vascular Complications ◽  
Digital Ulcers ◽  
Entire Cohort ◽  
Increased Risk ◽  
Pulmonary Arterial ◽  
Hypertension Development

Systemic sclerosis is a disease where microcirculation damage is critical in their beginning and vascular complications have similar pathogenic findings. Digital ulcers are a frequent complication in systemic sclerosis patients and pulmonary hypertension is one of the leading causes of death. The use of bosentan has been shown to be useful for the treatment of pulmonary arterial hypertension and to prevent new digital ulcers. However, is unknown if bosentan can prevent pulmonary hypertension. Our objective was to determine if bosentan is useful to prevent pulmonary hypertension in SSc patients. A retrospective study in 237 systemic sclerosis patients with digital ulcers history treated or not with bosentan to prevent it was made. We analyzed the occurrence of pulmonary hypertension defined by an echocardiogram pulmonary arterial pressure > 40 mmHg in the entire cohort. Demographic, clinical, and treatment variables were recorded for all patients. Statistical significance was denoted by p values < 0.05. Fifty-nine patients were treated with bosentan a median of 34 months. 13.8% of treated patients had pulmonary hypertension vs 23.7% of untreated patients (p 0.13) during the follow up. In multivariate analysis patients not treated with bosentan had 3.9fold-increased risk of pulmonary hypertension compared with patients under bosentan treatment (p < 0.02). Moreover the percentage carbon monoxide diffusing capacity (DLCO) in bosentan treated patients did not decrease from baseline to the end of follow-up (61.8±14% vs 57±20.1%, p = 0.89). We concluded that Systemic sclerosis patients with digital ulcers treated with bosentan seems to have less risk to develop pulmonary hypertension and to stabilize DLCO

P6478Resting and exercise pulmonary artery systolic pressure to rule out later development of pulmonary arterial hypertension in systemic sclerosis

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
L Gargani ◽  
V Codullo ◽  
P Argiento ◽  
A Moreo ◽  
F Pieri ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Systemic Sclerosis ◽  
Pulmonary Arterial Hypertension ◽  
Pulmonary Artery ◽  
Peak Exercise ◽  
Exercise Doppler Echocardiography ◽  
Pulmonary Arterial ◽  
Further Development ◽  
Rule Out

Abstract Background Patients with systemic sclerosis (SSc) are at risk of developing pulmonary arterial hypertension (PAH), which is often diagnosed late when the benefits of vasoactive therapies are limited. The concept of exercise pulmonary hypertension as a possible transitional phase anticipating resting PAH has been assessed in several pathologies, but has not been endorsed by the latest European Guidelines, because not supported by sufficient data. Purpose To evaluate whether PASP values at rest and at peak exercise, estimated at echocardiography, could be predictors of further development of PAH. Methods Four hundred and twenty-nine SSc patients without a previous diagnosis of PAH, enrolled at 4 referral Centres, underwent standard exercise Doppler echocardiography with PASP estimation at rest and at peak stress. Patients were then followed-up to assess the development of PAH, as diagnosed by a complete diagnostic work-up including right heart catheterization. PAH was defined by pre-capillary pulmonary hypertension (mean pulmonary artery pressure ≥25 mmHg with pulmonary arterial wedge pressure ≤15 mmHg), without significant interstitial lung disease and/or left heart disease. Results During the median follow-up of 75 months (IQR 29–114), 16 patients developed PAH. A combined cut-off of ≥24 mmHg as resting PASP and ≥40 as peak PASP was identified as the best predictor of further development of PAH (see Figure). Both resting PASP and peak PASP were predictors of PAH at univariate analysis (resting PASP OR 1.13, 95% C.I. 1.07–1.19, p<0.0001; peak PASP OR 1.13, 95% C.I. 1.07–1.18, p<0.0001). At multivariate analysis, only peak PASP was independently associated to PAH development (OR 1.13, 95% C.I. 1.04–1.18, p<0.001). Only one patient among those with resting PASP <24 mmHg and peak PASP <40 mmHg (34.7% of the total population) developed PAH during the follow-up (after 10 years from normal exercise Doppler echocardiography). Kaplan-Meier curves Conclusions Exercise increase in PASP is an independent predictor of later development of PAH in SSc. An increase in exercise PASP is frequent and is not necessarily associated with a later development of PAH, whereas the very high negative predictive value of a normal PASP both at rest and at peak exercise can be used in the clinical practice to confidently rule out about one third of patients. Acknowledgement/Funding Italian Ministry of Health (Ricerca Finalizzata 2011-2012)

Subclinical progression of systemic sclerosis-related cardiomyopathy

2020 ◽  
Vol 27 (17) ◽  
pp. 1876-1886
Author(s):  
Giulia Stronati ◽  
Lucia Manfredi ◽  
Alessia Ferrarini ◽  
Lucia Zuliani ◽  
Marco Fogante ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Systemic Sclerosis ◽  
Speckle Tracking ◽  
Cardiac Involvement ◽  
Global Longitudinal Strain ◽  
Prospective Longitudinal Study ◽  
Heart Involvement ◽  
Increased Risk ◽  
Prospective Longitudinal

Aims Cardiac involvement in patients with systemic sclerosis (SSc) is frequent and represents a negative prognostic factor. Recent studies have described subclinical heart involvement of both the right ventricle (RV) and left ventricle (LV) via speckle-tracking-derived global longitudinal strain (GLS). It is currently unknown if SSc-related cardiomyopathy progresses through time. Our aim was to assess the progression of subclinical cardiac involvement in patients with SSc via speckle-tracking-derived GLS. Methods This was a prospective longitudinal study enrolling 72 consecutive patients with a diagnosis of SSc and no structural heart disease nor pulmonary hypertension. A standard echocardiographic exam and GLS calculations were performed at baseline and at follow-up. Results Traditional echocardiographic parameters did not differ from baseline to 20-month follow-up. LV GLS, despite being already impaired at baseline, worsened significantly during follow-up (from –19.8 ± 3.5% to –18.7 ± 3.5%, p = .034). RV GLS impairment progressed through the follow-up period (from –20.9 ± 6.1% to –18.7 ± 5.4%, p = .013). The impairment was more pronounced for the endocardial layers of both LV (from –22.5 ± 3.9% to –21.4 ± 3.9%, p = .041) and RV (–24.2 ± 6.2% to –20.6 ± 5.9%, p = .001). A 1% worsening in RV GLS was associated with an 18% increased risk of all-cause death or major cardiovascular event ( p = .03) and with a 55% increased risk of pulmonary hypertension ( p = .043). Conclusion SSC-related cardiomyopathy progresses over time and can be detected by speckle-tracking GLS. The highest progression towards reduced deformation was registered for the endocardial layers, which supports the hypothesis that microvascular dysfunction is the main determinant of heart involvement in SSc patients and starts well before overt pulmonary hypertension.

scholarly journals Incidence of pulmonary hypertension and determining factors in patients with systemic sclerosis

2018 ◽  
Vol 51 (4) ◽  
pp. 1701197 ◽  
Author(s):  
J. Gerry Coghlan ◽  
Matthias Wolf ◽  
Oliver Distler ◽  
Christopher P. Denton ◽  
Martin Doelberg ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Systemic Sclerosis ◽  
Vena Cava ◽  
Diffusion Capacity ◽  
Mean Pulmonary Arterial Pressure ◽  
Right Heart Catheterisation ◽  
Determining Factors ◽  
Pulmonary Arterial ◽  
Velocity Diffusion

The objective of this study was to evaluate the incidence of pulmonary hypertension (PH) and determining factors in patients with systemic sclerosis (SSc) and a diffusing capacity of the lung for carbon monoxide (DLCO) <60% predicted.In this bicentric, prospective cohort study, patients with SSc were clinically assessed at baseline and after 3 years, including right heart catheterisation (RHC). Analysis of determining factors for the development of PH was performed using univariate and multivariate analyses.96 patients with a mean pulmonary arterial pressure (mPAP) <25 mmHg at baseline were followed for 2.95±0.7 years (median 3 years). Of these, 71 had a second RHC; 18 of these 71 patients (25.3%) developed PH, and five (7%) developed SSc-associated pulmonary arterial hypertension. For patients with an mPAP of 21–24 mmHg at baseline, the likelihood of presenting with PH as opposed to normal pressures on follow-up was significantly higher (p=0.026). Pulmonary vascular resistance, tricuspid regurgitation velocity, diffusion capacity and the size of the inferior vena cava at baseline were independent predictors for the development of PH during follow-up.In a selected cohort of SSc patients with a DLCO <60%, pulmonary pressures appeared to rise progressively during follow-up. In this population, it was possible to identify manifest PH in almost 25% of patients using prospective RHC during follow-up. Therefore, regular clinical assessment including RHC might be useful in patients with SSc.

scholarly journals Pulmonary hypertension in systemic sclerosis: diagnosis by systematic screening and prognosis after three years follow-up

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Verônica Silva Vilela ◽  
Marcio Macri Dias ◽  
Ângelo Antunes Salgado ◽  
Bruno Rangel Antunes da Silva ◽  
Agnaldo José Lopes ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Systemic Sclerosis ◽  
Right Heart Catheterization ◽  
Diagnostic Tools ◽  
Heart Catheterization ◽  
Systematic Screening ◽  
Mean Pulmonary Arterial Pressure ◽  
Common Group ◽  
Pulmonary Arterial

Abstract Background Systemic sclerosis (SSc) is a rare disease, and the presence of pulmonary hypertension can be a determining factor in prognosis. The aim of this study was to evaluate the diagnosis, profile, and prognosis of systemic sclerosis pulmonary hypertension (SSc-PH) diagnosed by systematic screening in a Brazilian population. Methods A cohort of SSc patients underwent systematic screening for SSc-PH. Patients were referred for right heart catheterization (RHC) according to transthoracic echocardiogram or a combination of diagnostic tools. The clinical, immunological, and hemodynamic features and prognosis after 3 years were evaluated. Results Twenty patients underwent RHC. SSc pulmonary arterial hypertension (SSc-PAH) was the most common group of SSc-PH. These patients had long disease duration, high urate levels and highly elevated mean pulmonary arterial pressure (mPAP) and peripheral vascular resistance (PVR) on hemodynamics. Patients with mPAP > 20– < 25 mmHg had hemodynamic features of intermediate disease. Patients with SSc-PH associated to interstitial lung disease (SSc-ILD-PH) had signs of vasculopathy on hemodynamics. In patients with no-SSc-PH, the survival at 1, 2, and 3 years was 96%, 92% and 92%, respectively and in patients with SSc-PH it was 86.7%, 60% and 53.3%, respectively. Conclusions Patients identified with SSc-PAH and SSc-ILD-PH in our screening had severe clinical and hemodynamic features. Mortality remains high in SSc-PH but was more related to Bo-PAH and SSc-ILD-PH, while in SSc-PAH, the prognosis was better. Trial registration: Current Controlled Trials ISRCTN 72968188, July 8th, 2021. Retrospectively registered.

Cerebral Vasculopathy Is Associated with Severe Vascular Manifestations in Systemic Sclerosis

2009 ◽  
Vol 36 (7) ◽  
pp. 1486-1494 ◽  
Author(s):  
BENJAMIN TERRIER ◽  
FRÉDÉRIQUE CHARBONNEAU ◽  
EMMANUEL TOUZÉ ◽  
ALICE BEREZNE ◽  
CHRISTIAN PAGNOUX ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Ct Scan ◽  
Vascular Complications ◽  
Scleroderma Renal Crisis ◽  
American College Of Rheumatology ◽  
Increased Risk ◽  
Brain Involvement ◽  
Pulmonary Arterial ◽  
Magnetic Resonance Imaging Mri ◽  
The Brain

Objective.To investigate brain involvement in patients with systemic sclerosis (SSc).Methods.Sixty-three patients with SSc fulfilling the American College of Rheumatology and/or Leroy and Medsger criteria were retrospectively studied, including 30 (47.6%) with limited cutaneous and 27 (42.9%) with diffuse cutaneous SSc. Forty-one patients underwent computed tomography (CT) scan and magnetic resonance imaging (MRI) of the brain, 11 patients only CT scan, and the remaining 11 patients only MRI. Cerebral vasculopathy on MRI and CT scan was defined as absent or mild (score < 1), moderate (1 ≤ score < 2), or severe (score ≥ 2) on a 4-point scale (0 to 3).Results.Cerebral vasculopathy was identified on CT scan in 22 patients (moderate in 12 and severe in 10) and on MRI in 38 patients (moderate in 28 and severe in 10). Patients with severe cerebral vasculopathy seen on MRI were more likely to have pulmonary arterial hypertension (PAH; p = 0.003) and showed a tendency to have scleroderma renal crisis (SRC; p = 0.25, test for trend p = 0.097). A similar association was found between severe cerebral vasculopathy seen on CT scan and PAH (p = 0.026) or SRC (p = 0.04). After adjusting for age and hypertension, severe cerebral vasculopathy was still associated with increased risk of severe vascular manifestations [odds ratio (OR) 32, 95% confidence interval (CI) 3.45–297, p = 0.002 for CT scan; OR 26, 95% CI 1.71–394, p = 0.019 for MRI].Conclusion.Severe cerebral vasculopathy is associated with severe vascular manifestations in SSc patients. SSc patients with severe vascular complications should undergo neuroradiological imaging assessment of brain involvement.

scholarly journals AB0401 CAN DUAL-ENERGY CT LUNG PERFUSION DETECT ABNORMALITIES AT THE LEVEL OF LUNG CIRCULATION IN SYSTEMIC SCLEROSIS (SSC)? PRELIMINARY EXPERIENCE IN 101 PATIENTS

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1229.1-1229
Author(s):  
V. Koether ◽  
A. Dupont ◽  
J. Labreuche ◽  
P. Felloni ◽  
T. Perez ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Systemic Sclerosis ◽  
Interstitial Lung Disease ◽  
Dual Energy ◽  
Lung Perfusion ◽  
Walking Distance ◽  
Dual Energy Ct ◽  
Perfusion Defects ◽  
Pulmonary Arterial

Background:Systemic sclerosis (SSc) is an autoimmune disorder that is characterized by a interplay of vascular abnormalities, immune system activation and an uncontrolled fibrotic response associated with interstitial lung disease affecting about 40% of patients. Identification of ILD relies on high-resolution CT that identify features suggestive of the histologic patterns of SSc(1). CT is used to determine pattern and extent of ILD and participates in the prediction of ILD progression(2).All group of pulmonary hypertension (PH) may occur with an overall prevalence reported in up to one fifth of patient. Whereas extensive SSc-ILD can be responsible for PH, PH can also be seen as a consequence of myocardial abnormalities or as primarily affecting small pulmonary arteries and classified as pulmonary arterial hypertension.Dual-energy CT introduction offers perspectives in the evaluation of SSc-related pulmonary manifestations. While these are not strictly perfusion images, they have been reported as adequate surrogate markers of lung perfusion (3). In the field of PH, detection of perfusion defects highly concordant with V/Q scintigraphic findings has been reported in the diagnostic approach of CTEPH but also in the differential diagnosis between PAH and peripheral forms of CTEPH (4).Objectives:To investigate lung perfusion abnormalities in patients with SSc.Methods:The study population included 101 patients who underwent dual-energy CT (DECT) angiography in the follow-up of SSc. CT examinations were obtained on a 3rd-generation dual-source CT system with reconstruction of morphologic and perfusion images. All patients underwent pulmonary function tests within two months of the follow-up CT scan. Fifteen patients had right heart catheterization-proven PH.Results:Our population included patients without SSc lung involvement (Group 1; n=37), patients with SSc-related ILD (Group 2; n=56) of variable extent (Group 2a: ≤10%: n=17; Group 2b: between 11-50%: n=31; Group 2c: >50%: n=8) and patients with PVOD/PCH (Group 3; n=8). Lung perfusion was abnormal in 8 patients in G 1 (21.6%), 14 patients in G 2 (25%) and 7 patients in G 3 (87.5%). Perfusion changes were mainly composed of bilateral perfusion defects, including patchy, PE-type perfusion defects and areas of hypoperfusion of variable size. In G 1 and G 2a (n=54): (a) patients with abnormal lung perfusion (n=14) had a significantly higher proportion of NYHA III/IV scores of dyspnea (p=0.031), a shorter mean walking distance at the 6MWT (p=0.042) and a trend towards lower mean DLCO% (p=0.055) when compared to patients with normal lung perfusion (n=40); (b) a negative albeit weak correlation was found between the iodine concentration in both lungs and the DLCO% (r=-0.27; p=0.059) whereas no correlation was found with PAPs (r=0.16; p=0.29) and walking distance during the 6MWT (r=-0.029; p=0.84).Conclusion:DECT lung perfusion provides complementary information to HRCT scans, depicting perfusion changes in SSc patients with normal or minimally infiltrated lung parenchyma.References:[1]Kim EA, Lee KS, Johkoh T, Kim TS, Suh GY, Kwon OJ, et al. Interstitial lung diseases associated with collagen vascular diseases: radiologic and histopathologic findings. Radiogr Rev Publ Radiol Soc N Am Inc. 2002 Oct;22 Spec No:S151-165.[2]Goh NSL, Desai SR, Veeraraghavan S, Hansell DM, Copley SJ, Maher TM, et al. Interstitial lung disease in systemic sclerosis: a simple staging system. Am J Respir Crit Care Med. 2008 Jun 1;177(11):1248–54.[3]Fuld MK, Halaweish AF, Haynes SE, Divekar AA, Guo J, Hoffman EA. Pulmonary perfused blood volume with dual-energy CT as surrogate for pulmonary perfusion assessed with dynamic multidetector CT. Radiology. 2013 Jun;267(3):747–56.[4]Giordano J, Khung S, Duhamel A, Hossein-Foucher C, Bellèvre D, Lamblin N, et al. Lung perfusion characteristics in pulmonary arterial hypertension and peripheral forms of chronic thromboembolic pulmonary hypertension: Dual-energy CT experience in 31 patients. Eur Radiol. 2017 Apr;27(4):1631–9.Disclosure of Interests:None declared

scholarly journals The Renal Resistive Index: A New Biomarker for the Follow-up of Vascular Modifications in Systemic Sclerosis

2020 ◽  
pp. jrheum.191101 ◽  
Author(s):  
Antonietta Gigante ◽  
Cosimo Bruni ◽  
Gemma Lepri ◽  
Giulia Tesei ◽  
Vanessa Maestripieri ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Characteristic Curve ◽  
Area Under The Curve ◽  
Resistive Index ◽  
Renal Resistive Index ◽  
Digital Ulcers ◽  
Baseline Evaluation ◽  
Pulmonary Arterial ◽  
New Onset

Objective The aim of the present retrospective observational study was to evaluate the change of Renal Resistive Index (RRI) over time (ΔRRI) and under treatment in patients with systemic sclerosis (SSc) as well as to correlate these changes with disease complications. Methods Two hundred thirty patients [29 male, median age 57 (IQR 48–67) yrs] were enrolled. At baseline and follow-up (3.43, IQR 2.81–4.45 yrs), we collected the following data: disease variables, nailfold videocapillaroscopy (NVC) pattern, forced vital capacity (FVC), diffusing lung capacity for carbon monoxide (DLCO), systolic pulmonary arterial pressure (sPAP), presence of interstitial lung disease, RRI, evaluation of glomerular filtration rate, and new onset of pulmonary arterial hypertension (PAH). Results RRI value is high in SSc patients with digital ulcers and anticentromere antibodies, active and late NVC patterns, and limited cutaneous SSc. A significant correlation was observed between ΔRRI and ΔsPAP (R = 0.17, P = 0.02), with statistically higher ΔRRI (0.08 ± 0.02 vs 0.03 ± 0.05, P = 0.04) in patients complicated by PAH onset. No other new-onset complication was associated with ΔRRI. The receiver-operating characteristic curve analysis confirmed the predictive role of ΔRRI in development of new PAH (area under the curve 0.84, 95% CI 0.75–0.93, P = 0.02). In patients with SSc never exposed to sildenafil, ΔRRI was higher (0.04 ± 0.05) compared to both patients exposed to sildenafil during the study period (0.01 ± 0.05, P = 0.03) or in those exposed at the time of baseline evaluation (0.00 ± 0.05, P = 0.01). Conclusion RRI and its variation in time are a reliable marker of SSc-related vasculopathy, both in renal and extrarenal compartments.

Study of Patients Having Splenectomy Due to Trauma for Long Term Infectious and Vascular Complications.

Blood ◽  
2007 ◽  
Vol 110 (11) ◽  
pp. 974-974
Author(s):  
Patricia L. Purcell ◽  
Shelley E. Crary ◽  
Leah Adix ◽  
Adam Alder ◽  
George R. Buchanan
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Vascular Complications ◽  
Vascular Events ◽  
Laboratory Test Results ◽  
Number Of Patients ◽  
Increased Risk ◽  
Pulmonary Arterial

Abstract Splenectomy, commonly performed for hematologic conditions, is followed by an increased risk of septicemia and arterial and deep venous thrombosis and pulmonary arterial hypertension. Most recent studies of post-splenectomy vascular complications focus primarily on patients with sickle cell disease, thalassemia, or hereditary stomatocytosis. However, the incidence and pathophysiology of complications in these patients is confounded by persistent hemolysis. Therefore, study of long term post-splenectomy infectious and vascular complications in patients without hemolysis or other serious medical conditions is indicated. Patients having splenectomy for trauma are often young and otherwise healthy so might be good candidates for investigation of post-splenectomy sequelae. To determine the feasibility of conducting a study of infectious and vascular complications following splenectomy for trauma, we retrospectively studied such patients admitted to Parkland Memorial Hospital, a 950 bed public hospital and the site of the Dallas metropolitan area’s primary trauma center. Using appropriate ICD-9 and CPT codes, three separate databases (hospital medical records, trauma registry, and professional billing compilation) were reviewed in order to identify patients ≥ age 18 yr. having total splenectomy for trauma during the past 15 years and to determine whether follow-up data were available in the records regarding subsequent vascular events or surrogate laboratory markers suggesting an increased risk of thrombosis. Between 1992 and 2006, 855 individuals had splenectomy, including 643 (75%) following trauma. The annual number of patients having splenectomy due to trauma did not decline during this period. A comprehensive chart review was performed on the 77 patients having splenectomy for trauma during 2003 and 2004. Records were unavailable in four patients, and 20 died of their injuries shortly after admission. Follow-up records of the remaining 53 patients - 26 (49%) of whom appeared to be discharged in good condition without serious sequelae - were reviewed to attempt to gather data regarding clinical features and laboratory test results. Post hospitalization follow-up occurred in only 9 (17%) of these 53 patients. Six had serious post-trauma complications (e.g., paraplegia); only three seen in follow-up more than six months following splenectomy appeared well. We conclude that complete splenectomy following trauma remains a common procedure. Retrospective review of these patients’ records does not permit evaluation of post-splenectomy complications. However, many patients were discharged shortly following splenectomy without other serious injuries. These patients could potentially be studied prospectively to assess the frequency, timing, and pathophysiology of infection, thrombosis, pulmonary arterial hypertension, and other complications of the post-splenectomy state without the confounder of hemolytic anemia.

Prevalence and prognostic significance of heart failure with preserved ejection fraction in systemic sclerosis

2021 ◽  
Author(s):  
Marta Fontes Oliveira ◽  
Ana Leonor Rei ◽  
Maria Isilda Oliveira ◽  
Isabel Almeida ◽  
Mário Santos
Keyword(s):  
Heart Failure ◽  
Systemic Sclerosis ◽  
Ejection Fraction ◽  
Statistical Significance ◽  
Prognostic Significance ◽  
Left Ventricular ◽  
Preserved Ejection Fraction ◽  
Increased Risk ◽  
Atrial Enlargement

Aim: Heart failure with preserved ejection fraction (HFpEF) is a clinically relevant complication of systemic sclerosis (SSc). We aimed to examine the prevalence, correlates and prognostic significance of HFpEF in an SSc population. Materials & methods: HFpEF was defined by the presence of exertional dyspnoea, abnormal cardiac structure (left ventricular hypertrophy or left atrial enlargement) and NT-proBN (>125 pg/ml). Results: Of the 155 studied patients, 27% had HFpEF criteria. These patients were older, had more cardiovascular risk factors, and were more likely to have atrial fibrillation or interstitial lung disease. Conclusion: Over a median follow-up of 9 years, SSc patients with HFpEF had a 3.4-fold increased risk of dying (HR: 3.37, 95% CI: 1.21–9.31), although this association has lost statistical significance after adjusting for age. On the contrary, NT-proBNP was an independent predictor of a worse prognosis.

Surgical Strategy in Patients with Atrial Septal Defect and Severe Pulmonary Hypertension

2012 ◽  
Vol 15 (2) ◽  
pp. 111 ◽  
Author(s):  
Yang Hyun Cho ◽  
Tae-Gook Jun ◽  
Ji-Hyuk Yang ◽  
Pyo Won Park ◽  
June Huh ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Atrial Septal Defect ◽  
Tricuspid Regurgitation ◽  
Septal Defect ◽  
Maze Procedure ◽  
Severe Pulmonary Hypertension ◽  
Tricuspid Annuloplasty ◽  
Pulmonary Arterial ◽  
Asd Closure

The aim of the study was to review our experience with atrial septal defect (ASD) closure with a fenestrated patch in patients with severe pulmonary hypertension. Between July 2004 and February 2009, 16 patients with isolated ASD underwent closure with a fenestrated patch. All patients had a secundum type ASD and severe pulmonary hypertension. Patients ranged in age from 6 to 57 years (mean � SD, 34.9 � 13.5 years). The follow-up period was 9 to 59 months (mean, 34.5 � 13.1 months). The ranges of preoperative systolic and pulmonary arterial pressures were 63 to 119 mm Hg (mean, 83.8 � 13.9 mm Hg) and 37 to 77 mm Hg (mean, 51.1 � 10.1 mm Hg). The ranges of preoperative values for the ratio of the pulmonary flow to the systemic flow and for pulmonary arterial resistance were 1.1 to 2.7 (mean, 1.95 � 0.5) and 3.9 to 16.7 Wood units (mean, 9.8 � 2.9 Wood units), respectively. There was no early or late mortality. Tricuspid annuloplasty was performed in 14 patients (87.5%). The peak tricuspid regurgitation gradient and the ratio of the systolic pulmonary artery pressure to the systemic arterial pressure were decreased in all patients. The New York Heart Association class and the grade of tricuspid regurgitation were improved in 13 patients (81.2%) and 15 patients (93.7%), respectively. ASD closure in patients with severe pulmonary hypertension can be performed safely if we create fenestration. Tricuspid annuloplasty and a Cox maze procedure may improve the clinical result. Close observation and follow-up will be needed to validate the long-term benefits.

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