scholarly journals Pulmonary Arterial Hypertension Specific Therapy in Patients with Combined Post- and Precapillary Pulmonary Hypertension

2018 ◽  
Vol 2018 ◽  
pp. 1-7 ◽  
Author(s):  
Hassan Alfraidi ◽  
Sultan Qanash ◽  
Zoheir Bshouty
Keyword(s):  
Functional Class ◽  
Initial Assessment ◽  
Specific Therapy ◽  
Potential Efficacy ◽  
Short And Long Term ◽  
Pulmonary Arterial ◽  
The Mean ◽  
Minute Walk Distance

Background. Specific therapy for patients with PAH is associated with good outcomes. Little is known about the effect of this treatment in patients with Cpc-PH (PAPm ≥ 25 mmHg, PAWP > 15 mmHg, DPG ≥ 7 mmHg, and/or PVR > 3 WU). This study evaluates the outcome of treating patients with Cpc-PH using PAH specific therapy. Methods. The primary outcome was survival. Secondary outcomes were WHO functional class and 6-minute walk distance (6-MWD). Results. Twenty-six patients with Cpc-PH (half with VHD and half with HF) received PAHST. Six patients did not tolerate treatment due to pulmonary edema. No predictors for treatment intolerance were identified. In twenty patients who tolerated the treatment, the mean WHO functional class improved from 2.70±0.21 at initial assessment to 2.22±0.21 (p<0.04) and 2.06±0.21 (p<0.03) at 6 and 9 months, respectively. Mean 6-MWD improved from 276.0±38.50 meters at initial assessment to 343.9±22.99 meters (p<0.04) and 364.6±34.85 meters (p=0.07) at 6 and 9 months, respectively. Twelve patients died during the follow-up period. Mean survival for all patients was 1279.7±193.60 days. Conclusion. PAHST may be beneficial in the treatment of Cpc-PH (both short and long term). Prospective randomized controlled trials of PAHST in this population are needed to assess its potential efficacy.

scholarly journals Effect of Bosentan on Exercise Capacity and Clinical Worsening in Patients with Dual down and Eisenmenger Syndrome

2013 ◽  
Vol 7 ◽  
pp. CMC.S10237 ◽  
Author(s):  
Giorgio Serino ◽  
Marco Guazzi ◽  
Angelo Micheletti ◽  
Carlo Lombardi ◽  
Rossella Danesi ◽  
...  
Keyword(s):  
Doppler Echocardiography ◽  
Laboratory Tests ◽  
Functional Class ◽  
Walk Distance ◽  
Pulmonary Arterial ◽  
Minute Walk Distance ◽  
Minute Walk ◽  
Clinical Worsening

This single-center, retrospective analysis evaluated long-term bosentan treatment in adult patients (n = 7) with both Down and Eisenmenger syndromes (DS-ES). Laboratory tests, 6-minute walk distance (6MWD), functional class, and Doppler echocardiography were assessed at baseline and during 2 years' follow-up. Improvements or maintenance of 6MWD were observed (68 m improvement from baseline at month 12) after bosentan initiation. 6MWD was maintained up to year 2. Overall, 6 patients experienced a significant improvement in functional class during 2 years' therapy ( P = 0.01). There were no significant changes in parameters measured by Doppler echocardiography. None of the patients required either hospitalization or additional pulmonary arterial hypertension (PAH) therapy because of PAH progression. Bosentan treatment was generally well tolerated; no liver function abnormalities or serious adverse drug reactions were noted. In this DS-ES cohort, bosentan seemed to be well tolerated and clinically effective.

Bosentan for the treatment of pulmonary arterial hypertension associated with congenital cardiac disease

2006 ◽  
Vol 16 (3) ◽  
pp. 268-274 ◽  
Author(s):  
Eugene Kotlyar ◽  
Raymond Sy ◽  
Anne M. Keogh ◽  
Fiona Kermeen ◽  
Peter S. Macdonald ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Functional Class ◽  
Walk Distance ◽  
Specific Therapy ◽  
Eisenmenger’S Syndrome ◽  
Pulmonary Arterial ◽  
Minute Walk Distance ◽  
Minute Walk

Aims:Bosentan is efficacious in idiopathic pulmonary arterial hypertension, and the variants associated with connective tissue disease, but not currently approved for treatment of pulmonary arterial hypertension due to Eisenmenger's syndrome. We sought to evaluate its effect in adults with Eisenmenger's syndrome.Methods:We administered bosentan on the basis of compassionate use in 23 patients with Eisenmenger's syndrome, aged 37 plus or minus 14 years. Of the patients, 17 had never received specific treatment for pulmonary arterial hypertension, five were transitioned from treprostinil, and one from beraprost to bosentan. We measured functional class, saturation of oxygen, haemoglobin levels and six-minute walk distance at baseline, one, six months and at most recent follow-up.Results:Baseline functional class was IV in three, III in fifteen, and II in five patients. At follow-up, with a mean of 15 plus or minus 10 months, 13 of the 23 patients (57%) had improved by at least one functional class, from a median baseline of III to II (p equal to 0.016), mean saturation of oxygen at rest had increased from 81% to 84% (p equal to 0.001), and levels of haemoglobin had decreased from 178 plus or minus 26 grams per litre to 167 plus or minus 19 grams per litre (p equal to 0.001). Overall, the six-minute walk distance did not change from baseline of 335 metres. The distance walked by those not previously receiving specific therapy, however, improved from 318 plus or minus 129 to 345 plus or minus 123 metres (p equal to 0.03).Conclusion:Treatment of adults with Eisenmenger's syndrome using bosentan significantly improved functional class, saturation of oxygen at rest, and decreased levels of haemoglobin. Treatment with bosentan was associated with improvement in six-minute walk distance in those not previously receiving specific therapy. In patients already in receipt of specific therapy, transition to bosentan resulted in no clinical deterioration.

scholarly journals Bariatric surgery in patients with type 1 diabetes: special considerations are warranted

2019 ◽  
Vol 10 ◽  
pp. 204201881882220 ◽  
Author(s):  
Zohar Landau ◽  
Galit Kowen-Sandbank ◽  
Daniela Jakubowicz ◽  
Asnat Raziel ◽  
Nasser Sakran ◽  
...  
Keyword(s):  
Bariatric Surgery ◽  
Type 1 Diabetes ◽  
Diabetic Ketoacidosis ◽  
Glucose Control ◽  
Healthcare Services ◽  
Short And Long Term ◽  
The Mean

Objective: We examined short and long-term outcomes of bariatric surgery in patients with obesity and type 1 diabetes mellitus (T1DM). Methods: We reviewed the records of all adults insured by Maccabi Healthcare Services during 2010 -2015, with body mass index (BMI) ⩾30 kg/m2 and T1DM; and compared weight reduction and glucose control according to the performance of bariatric surgery. BMI and glycated hemoglobin (HbA1c) levels were extracted for baseline and every 6 months, for a mean 3.5 years. Results: Of 52 patients, 26(50%) underwent bariatric surgery. Those who underwent surgery were more often female and with a longer duration of diabetes. Immediately postoperative, 4(15%) developed diabetic ketoacidosis, while 6(23%) experienced severe hypoglycemic episodes. The mean BMI decreased among surgery patients: from 39.5±4.4 to 30.1±5.0 kg/m2 ( p < 0.0001); and increased among those who did not undergo surgery: from 33.6±3.9 to 35.1±4.4 kg/m2 ( p = 0.49). The mean HbA1c level decreased during the first 6 months postoperative: from 8.5±0.9% to 7.9±0.9%; however, at the end of follow-up, was similar to baseline, 8.6±2.0% (p = 0.87). For patients who did not undergo surgery, the mean HbA1c increased from 7.9±1.9% to 8.6±1.5% ( p = 0.09). Conclusions: Among individuals with obesity and T1DM, weight loss was successful after bariatric surgery, but glucose control did not improve. The postoperative risks of diabetic ketoacidosis and severe hypoglycemic episodes should be considering when performing bariatric surgery in this population.

Surgical Pericardiectomy for Constrictive Pericarditis: A Single Tertiary Center Experience

2018 ◽  
Vol 68 (08) ◽  
pp. 730-736 ◽  
Author(s):  
Eyal Nachum ◽  
Leonid Sternik ◽  
Yigal Kassif ◽  
Ehud Raanani ◽  
Ilan Hay ◽  
...  
Keyword(s):  
Constrictive Pericarditis ◽  
Surgical Techniques ◽  
Postoperative Mortality ◽  
Functional Class ◽  
Right Atrial ◽  
Long Term Outcomes ◽  
Tertiary Center ◽  
Short And Long Term

Abstract Background Although surgery is the sole therapeutic option for patients with constrictive pericarditis (CP), reports on high postoperative mortality rates have led to hesitant surgery referral. The aim of this study was to report the short- and long-term outcomes of surgical pericardiectomy (SP) from a large tertiary center. Methods Between January 2005 and January 2017, 55 consecutive patients underwent SP after comprehensive echocardiography, computed tomography, and hemodynamic studies. Detailed clinical, imaging, surgical techniques and follow-up outcomes were recorded. Results The most common etiology was idiopathic (n = 27, 49%) and 33 patients (60%) were in functional class 3/4. Sixteen patients (29%) underwent concomitant interventions during SP, and cardiopulmonary bypass (CPB) was used in these, as well as in four additional cases. Complete resection, independent of CPB, was achieved in 96%. One patient died during the index hospitalization, and four (7%) needed re-explorations due to bleeding. While 12 patients (22%) died during a mean follow-up of 52 ± 39 months, only 1 death was due to right heart failure. Functional class significantly improved (with a p-value < 0.001), diuretics were discontinued in all, and significant reductions of right atrial pressures were recorded. None of these outcomes differed as a result of concomitant interventions at the time of SP. Conclusion Short- and long-term outcomes of SP, performed either alone or concomitantly with other procedures, indicate high safety and favorable clinical and hemodynamic efficacy for the treatment of CP.

scholarly journals Clinical and prognostic significance of the inflammatory markers GlycA and GlycB in chronic heart failure of both ischemic and non-ischemic etiologies

2021 ◽  
Vol 42 (Supplement_1) ◽  
Author(s):  
G Cediel Calderon ◽  
A Teis ◽  
P Codina ◽  
M Domingo ◽  
E Santiago-Vacas ◽  
...  
Keyword(s):  
Heart Failure ◽  
Primary Endpoint ◽  
Prognostic Significance ◽  
Functional Class ◽  
Funding Sources ◽  
The Mean ◽  
Cv Disease ◽  
Multivariable Adjustment

Abstract Background N-acetylglucosamine/galactosamine (GlycA) and sialic acid (GlycB) constitute evolving nuclear magnetic resonance (NMR) biomarkers of systemic inflammation. They have been increasingly studied and associated with cardiovascular (CV) disease and incident heart failure (HF), but little is known about its value in chronic HF population. Purpose We aimed to examine the association on long-term CV outcomes of GlycA and GlycB in chronic HF relative to aetiology. Methods We prospectively included a cohort of 429 HF patients admitted to an ambulatory HF Unit. Plasma GlycA and GlycB concentrations were determined using NMR spectroscopy. The primary endpoint was a composite of CV death and readmission due to HF. Competing risk regression models were performed with non-CV death as the competing event. Because an interaction existed between GlycA and GlycB with ischemic etiology (p&lt;0.01), we examined this further. Results The mean (SD) follow-up was 4.5±2.9 years. Median concentrations (IQR) for GlycA and GlycB were 5.4 (4.9–6.2) mmol/L and 1.9 (1.7–2.2) mmol/L, respectively. A total of 92 (41.1%) and 123 (60.0%) patients from non-ischemic and ischemic etiology, respectively, the clinical endpoint. In ischaemic HF patients (47.8%; n=205) both markers were not associated with the primary endpoint. Conversely, in non-ischemic HF patients (52.2%; n=224), GlycA and GlycB exhibited association with the primary endpoint in univariable and after multivariable adjustment (HR 1.14; 95% confidence interval [CI]: 1.02–1.28, p=0.018 and HR 1.91; 95% CI: 1.27–2.88, p=0.002; respectively. Figure 1 and 2). In this subgroup, a correlation analysis with well-known biomarkers (NT-proBNP, hs-TnT and ST2) only evidenced a positively and significantly correlation of GlycB with ST2 (r=0.26, p&lt;0.001). No association was found with NYHA functional class. Conclusions GlycA and GlycB represent an evolving approach of inflammation status with prognostic value of long-term CV related events in non-ischemic HF patients. FUNDunding Acknowledgement Type of funding sources: None. Figure 1. Probability of MACE by GlycA tertiles Figure 2. Probability of MACE by GlycB tertiles

Crescentic Proximal Metatarsal Osteotomy for Moderate to Severe Hallux Valgus: A Mean 12.2 Year Follow-Up Study

2001 ◽  
Vol 22 (10) ◽  
pp. 817-822 ◽  
Author(s):  
J.P. Veri ◽  
S.P. Pirani ◽  
R. Claridge
Keyword(s):  
Hallux Valgus ◽  
Long Term Results ◽  
Soft Tissue Reconstruction ◽  
Short Term ◽  
Long Term Follow Up ◽  
Short And Long Term ◽  
The Mean ◽  
Severe Hallux Valgus

The senior author's (R.C.) first 25 patients (37 feet) treated with a combination proximal crescentic osteotomy and distal soft-tissue reconstruction made up the patient cohort. All 25 patients were reviewed at a minimum of one year post-op (short-term follow-up) and 20/25 (31/37 feet) were reviewed again at a mean 12.2 years (range 11.4 to 13.0 yrs) post-op (long-term follow-up). This allowed for a comparison of short- and long-term results and led to a long-term follow-up rate of 84% (31/37 feet, mean 12.2 yrs). Clinical, radiographic and patient outcome measures were obtained and compared pre-op and at short- and long-term follow-up. The mean preoperative hallux valgus (HVA) and intermetatarsal angles (IMA) were 37° and 16° respectively. The mean HVA correction was 24° and IMA correction 10° at long-term follow-up with no tendency toward recurrence. Sesamoid position and first MTP subluxation was markedly improved postoperatively and the correction was maintained at long-term follow-up. Patients were asked about their satisfaction in terms of pain, appearance and motion. At long-term follow-up, more than 90% of patients were completely satisfied with pain and motion and greater than 80% with their appearance. Ninety-four percent of patients said they would have the operation again. The AOFAS clinical rating scale for the hallux was calculated retrospectively for pre-op and short-term follow-up and prospectively for long-term follow-up. The mean pre-op score was 37/100 (16 to 60) which significantly improved to 92/100 (67 to 100) at both follow-up periods, suggesting no evidence of decrease in outcome over time. Complications included two patients (5%) that were over-corrected into varus (one symptomatic, one asymptomatic), and four patients (11%) that were undercorrected, developing asymptomatic recurrences (>10° increase HVA) at long-term follow-up. In addition, two patients (5%) developed new transfer lesions postoperatively, likely related to technical error (one varus overcorrection, one dorsiflexion malunion). In conclusion, the long-term results, with a mean follow-up of 12.2 years, of the resection realignment procedure for moderate to severe hallux valgus are generally excellent and the complication rate is low and acceptable. Attention to detail, avoiding both undercorrection, which can lead to recurrence, and overcorrection, which can cause symptomatic varus, is essential.

Smoking Cessation and Weight Gain in Smokers Participating in a Behavioral Treatment at 3-Year Follow-up

1998 ◽  
Vol 82 (3) ◽  
pp. 999-1005 ◽  
Author(s):  
Elisardo Becoña ◽  
Fernando L. Vazquez
Keyword(s):  
Smoking Cessation ◽  
Weight Gain ◽  
Behavioral Treatment ◽  
Smoking Cessation Program ◽  
Short And Long Term ◽  
The Mean ◽  
Mean Differences

This study assessed the weight gain for the short- and long-term (36 mo. follow-up) in a sample of 158 smokers (71 men and 87 women), who participated in a multicomponent behavioral smoking-cessation program. Weight was evaluated with a self-reported questionnaire, with an over-all mean pretreatment weight of 65.3 Kg ( SD = 12.6). All individuals tended to gain weight. Afterwards, the mean for quitters ( n = 42) increased by 2.0 Kg at 36-mo. follow-up and the mean for nonquitters ( n = 116) by 0.6 Kg. Among abstinent people no significant differences were found by sex at 36 mo. but significant mean differences were found in those subjects (quitters) older than 35 yr. who gained 4.1 Kg compared with 1.2 Kg in the group 35 yr. or younger.

scholarly journals Subthalamic Nucleus Stimulation in Pediatric Isolated Dystonia: A 10-Year Follow-up

2020 ◽  
Vol 47 (3) ◽  
pp. 328-335
Author(s):  
Wenying Xu ◽  
Hongxia Li ◽  
Chencheng Zhang ◽  
Bomin Sun ◽  
Yiwen Wu ◽  
...  
Keyword(s):  
Clinical Outcomes ◽  
Subthalamic Nucleus ◽  
Rating Scale ◽  
Motor Score ◽  
Age At Surgery ◽  
Short And Long Term ◽  
The Mean ◽  
Stn Dbs

ABSTRACT:Objective:To evaluate the short-term and long-term clinical effectiveness and safety of subthalamic nucleus deep brain stimulation (STN-DBS) for medically intractable pediatric isolated dystonia.Methods:Using a longitudinal retrospective design, we assessed the clinical outcomes of nine patients who underwent STN-DBS for treatment-refractory pediatric isolated dystonia one decade ago (mean age at surgery: 15.9 ± 4.5 years). The primary clinical outcome used was assessed by retrospective video analyses of patients’ dystonia symptoms using the Burke–Fahn–Marsden Dystonia Rating Scale (BFMDRS). Clinical assessments were performed at baseline, 1-year follow-up (1-yr FU), and 10-year follow-up (10-yr FU). Adverse side effects, including surgery-related, device-related, and stimulation-related effects, were also documented.Results:After STN-DBS surgery, the mean improvement in the BFMDRS motor score was 77.1 ± 26.6% at 1-yr FU and 90.4 ± 10.4% at 10-yr FU. Similarly, the mean BFMDRS disability score was improved by 69.5 ± 13.6% at 1-yr FU and by 86.5 ± 13.9% at 10-yr FU. The clinical improvements gained at 10-yr FU were significantly larger than those observed at 1-yr FU. Negative correlations were found between the duration of disease to age at surgery ratio (DD/AS) and the improvements in the BFMDRS motor score and total score at 1-yr FU and 10-yr FU.Conclusion:To our knowledge, this study provides the first clinical evidence for the short- and long-term effectiveness and safety of STN-DBS for pediatric isolated dystonia. Additionally, putative evidence is provided that earlier STN-DBS intervention in patients with refractory pediatric isolated dystonia may improve short- and long-term clinical outcomes.

Macitentan in the treatment of pulmonary arterial hypertension

2021 ◽  
Vol 17 (1) ◽  
pp. 49-58
Author(s):  
Rodrigo Zebadúa ◽  
Andrea Priscila Hernández-Pérez ◽  
Antonio García ◽  
Nayeli Zayas ◽  
Julio Sandoval ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Progressive Disease ◽  
Functional Class ◽  
Short Term ◽  
Increased Risk ◽  
Long Term Follow Up ◽  
Pulmonary Arterial

Pulmonary arterial hypertension (PAH) is an uncommon but lethal and progressive disease in which prostacyclin, nitric oxide and endothelin-1 pathways are disturbed and contribute to the pathophysiology of this disease. Endothelin receptor antagonists are a class of drugs that have been approved as PAH therapy. Macitentan is a lipophilic, tissue specific, dual receptor antagonist with a higher potency than bosentan and a reduced risk of hepatic injury. Macitentan has shown a reduction in morbidity and mortality due to PAH at long-term follow-up and improvements in hemodynamics, exercise capacity and functional class at the short term. Its main adverse events are nasopharyngitis, bronchitis and an increased risk of anemia. We review the clinical data of macitentan and its use in PAH.

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