Effects of a short Cardiovascular Rehabilitation program in Hypertensive subjects: A Pilot Study
Published on: 2nd May, 2017
OCLC Number/Unique Identifier: 7286350945
Systemic arterial hypertension (SAH) is considered an important risk factor for the development of cardiovascular diseases. The aim of the present study was to verify the effects of a short cardiovascular rehabilitation program (CR) in hypertensive subjects. The clinical pilot study involved a sample composed of 11 hypertensive subjects. It was evaluated the weight, height, body mass index (BMI), waist and hip circumference, waist hip ratio, systolic blood pressure (SBP), diastolic blood pressure (DBP) and six-minute walk test (6-MWT) before and after CR. CR was performed twice a week for 60 minutes. The results shown that after CR occurred a reduction of waist circumference (99.86±8.7 to 95.2±8.6 cm, p=0.0002) and hip circumference (110.18±14.75 to 105.00±12.7 cm p=0.01) values. About the mean distance walked in the 6-MWT there was an increase after the CR program (335.9±123.5 m to 554.56±87.9 m, p=0.000). In conclusion, the results suggest that a short CR is an effective for the treatment of hypertensive subjects. After 16 CR sessions, functional and musculoskeletal capacity was improved, evaluated by 6-MWT. Furthermore, the short CR program decreased waist and hip circumferences, being an important option for these subjects. Although. There were no changes in baseline blood pressure levels.
Non-hemodynamic factors associated to the risk of developing hypertensive cardiopathy
Published on: 20th September, 2017
OCLC Number/Unique Identifier: 7286431107
Introduction: Hypertensive cardiopathy is the target organ lesion caused by arterial hypertension (HTN) that exhibits the highest morbidity and mortality rates. Although the importance of hemodynamic overload exerted by HTN on the onset of cardiopathy is well established, several non-hemodynamic factors may contribute significantly to its development.
Objective: To evaluate the influence of different non-hemodynamic risk factors in the development of hypertensive cardiopathy.
Methods: A prospective cohort study was carried out in hypertensive patients assisted at the specialized arterial hypertension physicians’ office of the “Carlos Manuel de Céspedes” Specialty Policlinic attached to the General University Hospital, Bayamo Municipality, Granma Province, Cuba from January 5, 2006 to December 31, 2015. The study included 18-to-55-year-old hypertensive patients with a stage 1 arterial hypertension diagnosis for less than a year1.
Results: The multivariate analysis showed a significant and independent relation among the majority of the factors studied and the risk of developing cardiopathy. The major factor was C-reactive protein (HR: 5.020; IC 95%: 3.383-7,448; p<0.005) followed by microalbuminuria (HR: 2.649; IC 95%: 1.932-3.631; p<0.005). The area under the model ROC curve was 0.887 (p<0,005).
Conclusions: The results showed that it is possible to estimate the risk of developing hypertensive cardiopathy with the application of the regression model to major risk factors.
Design and validation of an Index to predict the development of Hypertensive Cardiopathy
Published on: 16th February, 2018
OCLC Number/Unique Identifier: 7347023220
Introduction: The high morbidity and mortality by hypertensive cardiopathy demand the construction and validation of tools to stratify the risk of developing this condition.
Objective: To design and validate an index, based on risk factors, that permits to predict the development of hypertensive cardiopathy in patients with a diagnosis of essential arterial hypertension.
Methods: A prospective cohort study was done in hypertensive patients assisted at the specialized arterial hypertension physicians’ office of the “Carlos Manuel de Céspedes” Specialty Policlinic attached to the General University Hospital, Bayamo Municipality, Granma Province, Cuba from January 1st, 2010 to December 31, 2016. Internal and external validity and the internal consistency of the index were determined.
Results: The index sensitivity was of 97, 20 (IC: 93, 93-94.09) and specificity of 65, 38 (IC: 76, 25-76, 20). Both the index discriminative capacity (area under the ROC curve= 0,944; interval of confidence: 0.932-0.956; p<0.0005) and calibration (p=0.751) were adequate.
Conclusions: The present study proposes an index to predict the risk of developing hypertensive cardiopathy, with adequate discriminative capacity and calibration (external validity). The index can be used as a tool of clinical and epidemiological surveillance since it permits to identify subjects with greater probability of developing the condition and to stratify the risk.
A rare case of abdominal pain and hematuria from retroaortic left renal vein
Published on: 7th December, 2019
OCLC Number/Unique Identifier: 9272398140
62-year-old female with a history of arterial hypertension, attended the emergency department due to pain in the left flank. On physical examination no showed signs of peritoneal irritation. Urinalysis was indicated, that reported microscopic hematuria and negative abdominal ultrasound.
Successful treatment of late-onset pulmonary hypertension after atrial septal defect operation with macitentan: Our center experience
Published on: 17th August, 2018
OCLC Number/Unique Identifier: 7844628408
Background: Macitentan significantly improves pulmonary hemodynamics and survival in patients with primary pulmonary hypertension (PPH). Its beneficial effect, however, may be blunted due to the adverse impacts such as anemia and peripheral edema. Pulmonary arterial hypertension (PAH) is a significant consequence of congenital heart disease (CHD). Its presence and severity are associated with increased morbidity and mortality. We tried to evaluate that the effectiveness of the macitentan in patients with late-onset pulmonary hypertension after atrial septal defect operation in our center.
Methods: The effect of a single dose of macitentan (10 mg) on pulmonary hemodynamics, functional capacity was examined in four patients with late-onset pulmonary hypertension after atrial septal defect operation.
Results: The macitentan significantly improved mean pulmonary artery pressure (MPAP), cardiac output (CO), tricuspid annular plane systolic excursion (TAPSE), right ventricle systolic wave(RVS’), 6-minute walking test and NT-proBNP levels compared with before treatment.
Conclusions: Macitentan can be used in patients with late-onset pulmonary hypertension after shunt operation especially atrial septal defect.
Compliance of hypertensive patients with antihypertensive drug therapy at the Renaissance Hospital of N’Djamena, Chad
Published on: 23rd October, 2019
OCLC Number/Unique Identifier: 8301372454
Introduction: High blood pressure is a major cardiovascular risk factor. In hypertension, non-compliance is frequent. The objective of this work is to evaluate the therapeutic observances and to identify the predictive factors of poor compliances in Chadian hypertensive patients.
Patients and Methods: It was a prospective cross-sectional study over a six-month period from January 15 to July 15, 2019. This was performed in the outpatient Cardiology and Nephrology units at the Renaissance Hospital of N’Djamena. We included all follow-up patients who had hypertension who consulted during the study period. However, dialysis patients and children were excluded from this study. The parameters studied were demographic characteristics, economic and therapeutic data and the rate of therapeutic compliance.
Results: Eighty-seven patients were included. The average age was 50 years old. The sex ratio was 2.5. Sixty-seven percent (n = 58) of the patients were from urban areas. The predominant cardiovascular risk factors were smoking in 25% (n = 22) and diabetes in 23% (n = 20). Hypertension was uncontrolled in 76% (n = 66) patients. Adherence was poor in 66% (n = 57) of patients. The monthly cost of treatment was respectively 10,000 and 20,000 FCFA in 52% (n = 45) of cases. Combination therapy was observed in 70% of cases (n = 61) and 56% (n = 49) of patients had more than one drug intake. The adherence rate was 93% (n = 28) in the urban population (p < 0.001). All patients (n = 30) who were observing their treatment were educated (p < 0.001). The adherence rate was 20% (n = 6) in patients who had a monthly income less than 100,000 FCFA (p = 0.004). The adherence rate was 60% (n = 18) when the monthly cost was less than FCFA 10,000 (p = 0.003). The adherence rate was 77% (n = 23) in patients receiving monotherapy (p < 0.001).
Conclusion: This study showed a low level of adherence in Chadian hypertensive patients. The complexity and cost of antihypertensive therapy, poor knowledge of hypertension, and ignorance of its severity have been the main factors of poor compliance.
Impact of mandibular advancement device in quantitative electroencephalogram and sleep quality in mild to severe obstructive sleep apnea
Published on: 30th December, 2020
OCLC Number/Unique Identifier: 8899350400
Sleep related breathing disorders (SRBD) are among seven well-established major categories of sleep disorders defined in the third edition of The International Classification of Sleep Disorders (ICSD-3), and Obstructive Sleep Apnea (OSA) is the most common SRBD [1,2]. Several studies have demonstrated that obstructive sleep apnea treatment increases the quality of life in OSA patients [3-8]. Indeed, excessive daytime sleepiness (EDS), cognitive impairment (e.g., deficits in attention-concentration, memory, dexterity, and creativity), traffic accidents, and deterioration of social activities are frequently reported in untreated patients [9-11]. Furthermore, an increase in cardiovascular morbidities and mortality (systemic hypertension, stroke, cardiac arrhythmias, pulmonary arterial hypertension, heart failure) [12], metabolic dysfunction, cerebrovascular ischemic events and chemical/structural central nervous system cellular injuries (gray/white matter) has been reported in OSA patients [13-17].
Continuous positive airway pressure (CPAP) therapy is considered the gold standard for treatment of moderate-severe OSA, nevertheless there is an increasing body of evidence supporting the usefulness of mandibular advancement devices (MADs) for improving quality of life and respiratory parameters even among patients with a high severity of OSA burden [5,10,18,19]. According to the standard of care of the American Academy of Sleep Medicine (AASM), MADs are indicated for mild to moderate OSA particularly in the context of CPAP intolerance or refusal, surgical contraindication, or the need for a short-term substitute therapy [9,15,20-22]. In Cuba, CPAP machines are not readily available; they are expensive and the majority of OSA patients cannot obtain this mode of therapy. Taking into account this problem, our hypothesis was based in the scientific evidences of MAD effectiveness, considering that low cost MADs could offer a reasonable alternative treatment for patients with OSA where CPAP technology are not handy. In this way our purpose was to assess the efficacy of one of the most simple, low cost, manufactured monoblock MAD models (SAS de Zúrich) in terms of improvements in cerebral function, sleep quality and drowsiness reports in a group of Cuban OSA patients with mild to severe disease. Outcome measures included changes in the brain electrical activity, sleep quality, and respiratory parameters, measured by EEG recording with qEEG analysis and polysomnographic studies correspondingly, which were recorded before and during treatment with an MAD, as well as subjective/objective improvements in daytime alertness.
Frequency of renal tubular acidosis in children with down syndrome
Published on: 2nd October, 2020
OCLC Number/Unique Identifier: 8691889815
Background: Down syndrome (DS) is associated with various congenital diseases and malformations, including those of the kidneys and urinary tract. It has been thought that renal tubular acidosis (RTA) is more frequent in this population. The objective of this study was to assess the frequency of RTA and, secondarily, of other renal and urological disorders in persons with DS.
Method: An observational, ambispective, descriptive and cross-sectional study of patients diagnosed with RTA, or suspected kidney or urological disorders, was carried out from July 2016 to September 2017 at the Down syndrome clinic of the Mexican National Institute of Paediatrics. Urinalysis was performed, along with analyses of venous blood gas, sodium, potassium, chlorine, calcium, phosphorus, albumin and creatinine. Those with any abnormal values were referred to nephrology for diagnostic evaluation.
Results: Of a total of 700 patients seen at the clinic, 47 met the selection criteria. Of these, 32 had no RTA or other renal or urological alterations. The remaining 15 continued to the second phase of the study, where 6 were diagnosed with nephropathy or uropathy (RTA, systemic arterial hypertension, monosymptomatic familial haematuria, mild renal failure secondary to reflux nephropathy, urinary tract infection or right ureteropelvic stenosis). Four had mild metabolic acidosis without meeting the criteria for diagnosis of RTA.
Conclusion: RTA is not more common in children with Down syndrome. Nephropathies and uropathies should be investigated in the evaluation of DS patients.
Control of arterial hypertension and risk of new-onset of atrial fibrillation in patients with metabolic syndrome
Published on: 10th March, 2022
Background: An association between Atrial Fibrillation (AF) and Metabolic Syndrome (MS) a constellation of abnormalities (high blood pressure, hyperglycemia, dyslipidemia, and abdominal obesity), has been demonstrated. There have been many studies that have shown that elevated blood pressure (BP), was significantly associated with an increased risk of AF. It is uncertain whether maintaining the optimal BP levels can prevent AF in the patients with MS categorized as ‘high-risk’ patients.Objective: The aim of this study was to evaluate the influence of control of BP on the occurrence of new-onset atrial fibrillation in patients with Metabolic Syndrome.Methods: Into this observational study, was enrolled 435 consecutive patients (210 males and 225 females) aged 45-79 years who fulfilled criteria for MS. Participants were selected among primary and secondary care patients, who were receiving ongoing care for arterial hypertension in the period from November 2018 till November 2021. The study was conducted at outpatients in 5 Health Care Clinics (3 Secondary Health Care Clinics and 2 Primary Health Clinics). Patient were categorized according to their BP levels as Group 1-patients with controlled BP, {(patients aged < 65 years Systolic Blood Pressure (SBP) of 120 - 130 mmHg, patients aged ≥ 65 years SBP of 130 - 139 mmHg)} and Diastolic Blood Pressure (DBP), {(patients aged < 65 years of < 80 mmHg. but not < 70 mmHg; patients aged ≥ 65 years of 85 - 89 mmHg)}, or Group 2-patients with uncontrolled BP(> 130/80 mmHg),and in patients aged ≥ 65 years BP (≥ 140/90 mmHg ). Results: New-onset of AF, was more frequent in participants with uncontrolled BP, respectively (34.7% vs. 19.5%, p = 0.009).Patients with uncontrolled BP have more frequent persistent AF (15.2% vs. 0.04%) and permanent AF (0.08% vs. 0.02%), whereas there was not significant changes between groups in relation to frequency of paroxysmal AF, respectively (12.8% vs. 10.9%, p = 0.29). There was observed significant association of uncontrolled BP with: increased frequency of AF (OR = 2.193; 95% CI 1.390 - 3.439), persistent AF (OR = 3.931; 95% CI 1.771 - 8.084), permanent AF (OR = 4.138; 95% CI 1.383-12.381), LA. Dimension ≥ 2.2 cm/m2 (OR = 2.089, 95% CI 1.330 - 3.252), BMI (OR = 5.226, 95% CI 3.155 - 8.659) and 5-risk factors for MS, respectively (OR = 2.998, 95% CI 1.833 - 4.901).Conclusion: Optimal BP levels, can reduce the frequency of new-onset AF in patients with MS categorized as ‘high-risk’ patients. Uncontrolled BP was associated with an increased risk of both subtypes of AF (persistent and permanent) in the patients with MS categorized as ‘high-risk’ patients.
Does global longitudinal strain improve stratification risk in heart failure with preserved ejection fraction?
Published on: 8th September, 2022
Background: Heart Failure with Preserved Ejection Fraction (HFPEF) accounts for more than half of the cases of heart failure.Long regarded as an abnormality of left ventricular diastolic function, recent studies using longitudinal strain (two-dimensional speckle tracking mode) have suggested that left ventricular longitudinal systolic function is altered in HFPEF.Despite these interesting pathophysiological perspectives, the data in the literature on the prognostic value of the alteration of longitudinal strain are controversial. Given these conflicting results, it is difficult to confirm the magnitude and prevalence of impaired LV longitudinal systolic function in patients with HFPEF and its prognostic relevance. Purposes: This work aims to study the prognostic value of Global the left ventricle’s Global Longitudinal Strain (GLS) Algerian cohort of patients with HFPEF. Patients and methods: We conducted a monocentric prospective observational study from April 2018 to April 2020, with a minimum follow-up of 1 year for each recruited patient. We included patients over the age of 18 referred to the echocardiography laboratory for chronic or acute HFpEF, defined according to the criteria of ESC 2016. 153 consecutive patients underwent clinical examination, biological tests, and echocardiography with measurement of GLS at rest, in addition to routine management by the attending physicians.Results: 153 patients were collected. The average age of our patients is 73 +/- 11 years ranging from 42 to 91 years old. The female population is predominant with a rate of 67%. Comorbidities are predominant mainly by arterial hypertension (86%) and diabetes (64%), with a history of atrial fibrillation (46%).63% of patients have impaired GLS (< 16%). Contrary to our hypothesis, GLS was not shown to be a powerful predictor of cardiovascular events in HFPEF patients either in dichotomous analysis (OR = 0.79; p = 0.64) or in continuous analysis (OR = 0.97; p = 0.69).We were able to identify that congestive venous signs, anemia, and pulmonary hypertension, are the main independent prognostic factors in our Algerian population study. Conclusion: We were unable to demonstrate the prognostic role of mpaired GLS in our population of patients with HFPEF.
Centralized aerobic-anaerobic energy balance compensation theory perspective in biomedicine
Published on: 13th September, 2022
OCLC Number/Unique Identifier: 9617835348
Recently announced centralized aerobic-anaerobic energy balance compensation (CAAEBC) theory has already demonstrated achievements in the treatment of arterial hypertension (AHT), diabetes myelitis (DM) and osteochondrosis. Such demonstration lifts the necessity to check the applicability of this theory to other non-communicable diseases (NCDs) and develop the proper way to model the main idea of CAAEBC.
Left ventricular hypertrophy linked with arterial hypertension through centralized aerobic-anaerobic energy balance compensation theory
Published on: 26th October, 2022
We demonstrated intrinsic connections between left ventricular hypertrophy (LVH) and arterial hypertension (AHT) through the recently announced centralized aerobic-anaerobic energy balance compensation (CAAEBC) theory. CAAEBC has already demonstrated achievements in the treatment of AHT, diabetes myelitis (DM), and osteochondrosis. Such demonstration lifts the necessity to check the applicability of this theory to other non-communicable diseases (NCDs) and develop the proper way to model the main idea of CAAEBC.
Intravenous leiomyomatosis of the uterus: still discovered on anatomopathological examination
Published on: 2nd September, 2022
Background: Leiomyomas beyond the uterus are defined by benign smooth muscle cell tumors outside of the uterus. Intravenous leiomyomatosis is a rare type of uterine leiomyoma and is characterized by the formation and growth of benign leiomyoma tissue within the vascular wall. Herein, we present a case of Intravenous leiomyomatosis successfully treated by surgical removal and a review of actual medical recommendations.Case presentation: A 49 - year-old woman, maghrébin, G3 P2, no family history of uterine myomas mentioned, having systemic arterial hypertension, presented to our department with hypogastric pain and abnormal uterine bleeding in the prior five months resulting in anemia which required iron supplementation. On physical examination the vital signs were normal. A palpable mass in the hypogastrium was noted. The rest of the exam was unremarkable. Pelvic ultrasound showed a huge uterus with multiple heterogeneous leiomyomas, including at least one intracavity. Computed tomography scans and magnetic resonance imaging were not done initially due to the unaffordability of the patient. The initial diagnosis was leiomyoma. The decision to perform a total abdominal hysterectomy and bilateral salpingo-oophorectomy was taken. The abdomen was opened by a midline vertical incision. During surgery, multiple subserosal, intramural and submucosal fibroids ranging from 2 cm × 3 cm to 10 cm × 10 cm were seen. On pathological examination, the uterus measured 19 cm in the largest diameter and weighed 1.3 kg. The cut section showed white nodular myometrial masses. Microscopically, intravascular growth of benign smooth muscle cells is found within venous channels lined by endothelium. The diagnosis of Intravenous leiomyomatosis of the uterus without malignant transformation was retained. The patient was monitored for 14 months and subsequent computed tomography did not reveal any evidence of tumor recurrence. The follow-up will be performed annually till the age of menopause.Conclusion: Intravenous leiomyomatosis is a benign, rare and potentially lethal pathology. It especially affects premenopausal women with a history of uterine myoma, whether operated on or not. They require close and prolonged follow-up because of the high risk of recurrence.
Clinical characteristics of patients with respiratory disease and probable COVID-19 at the General Hospital Zacatecas Mexico
Published on: 4th May, 2023
Introduction: The spread of SARS-CoV-2 cases grew exponentially. In Mexico, it focused mainly on containing the disease and adopting activities and actions to mitigate it. Hospital reconversion was a fundamental strategy in the management of care for patients with COVID-19.Objective: To know the clinical characteristics of patients admitted with respiratory disease and probable COVID-19 in the Zacatecas General Hospital “Luz González Cosío” México.Material and methods: Descriptive, cross-sectional, and analytical study, at the General Hospital, from March 2019 to September 2021; using data from the Respiratory Disease Surveillance System. Data from patients admitted with a diagnosis of some pathology of respiratory disease and probable COVID-19 were analyzed.Results: We included 2,678 diagnosed with respiratory disease and a mean age; of 47.6 ± 21.6 gender distribution was almost equal; women 1,344 (51.0%). positive result to COVID-19 by PCR; 1,654 negatives; 900 and 124 without result. 193 (7.0%) required mechanical ventilation. The presence of comorbidities was evaluated; type 2 diabetes mellitus, systemic arterial hypertension, obesity, alone and together. Also the association of the age factor, as well as the lethality index; was 531 (19.6%).Discussion: What has been published in other studies about comorbidities and their influence on the severity of COVID-19 is confirmed, disagreeing on the case fatality rate; 20.7% against what was reported; 17.6% in other countries for COVID-19 hospitalized. An age variable was used as a risk factor with a cut-off point > 45 years; (FRE), obtaining; RR 3.42 (95% CI 2.79 to 4.19) and an odds ratio of 4.015 in binary logistic regression analysis. Reported male mortality (OR = 1.45; 95% CI: 1.41–1.51) according to our OR results; 1,45.Conclusion: The present study shows how certain chronic diseases influenced respiratory disease to present a serious state, regardless of the positive or negative result of COVID-19.
Vitamin D Deficiency and its Correlation with the Severity of Heart Disease in Dilated Cardiomyopathy Patients
Published on: 20th June, 2023
Background: Cardiomyopathy is primarily a disorder of the cardiac muscle that causes myocardial dysfunction and is not the result of disease or dysfunction of other cardiac structures, systemic arterial hypertension and valvular stenosis or regurgitation. Aim: The present study aimed to determine the prevalence of vitamin D deficiency and its correlation with the severity of heart disease in patients with dilated cardiomyopathy (DCMP). Method: 70 ECHO-proven DCMP cases were enrolled from the medicine/ cardiology department of LHMC & associated hospitals and ABVIMS & Dr. RML Hospital, New Delhi from November 2019 to October 2021. DCMP patients with ages more than 18 years who were willing to give consent and does not meet any of the exclusion criteria were enrolled in this study. Results: Mean age of idiopathic DCMP patients was 48.3 ± 15.2. There were more males 48 (69%) than females 22 (31%). The mean ejection fraction was 26.6 ± 7.3, while the mean fractional shortening was 17.6 ± 3.1. Vitamin D deficiency was observed in 90% of patients, among which 68.5% were having moderate vitamin D deficiency and 10% were having severe vitamin D deficiency. Conclusion: In our study, vitamin D levels were inversely correlated with the severity of heart disease in DCMP patients.
Longitudinal Risk Phenotyping in Incident Systemic Sclerosis-associated Pulmonary Arterial Hypertension (SSc-PAH): An Unsupervised Cluster Analysis of the PHAROS Registry
Published on: 15th March, 2024
Background: Systemic sclerosis-associated pulmonary arterial hypertension (SSc-PAH) is characterized by pulmonary arterial remodeling resulting in right ventricular failure and death if untreated. Despite therapeutic advances, there is survival variability within the SSc-PAH population. The aim of this study was to delineate high-risk subgroups of SSc-PAH using readily available clinical parameters.Methods: We analyzed data from the PHAROS database, a prospective observational registry of incident SSc-PAH patients. Latent class modeling was performed based on trends in 6MWD over time. We compared survival between the clusters regarding baseline clinical parameters and changes in these parameters over time.Results: We identified four unique groups within 103 patients meeting our inclusion criteria, based on trajectories of 6MWD. Patients in Cluster 4 exhibited a decline in 6MWD over time and had the worst prognosis with a median survival of 3 years. Patients in Cluster 3, with the lowest baseline 6MWD, were associated with lower median survival (5 years) when compared to Clusters 1 and 2 (> 9 and 7 years, respectively), despite an improvement in 6MWD over time. There were no meaningful changes in SF-36 and WHO functional class between the clusters, but BNP trended higher over time in the higher-risk clusters. Conclusion: We identified high-risk subsets of SSc-PAH characterized by significantly worse survival. Incident SSc-PAH patients with a decline in 6MWD over time or low baseline 6MWD had worse survival when compared to SSc-PAH patients who demonstrated relatively stable or mild reduction in 6MWD over time.
Macitentan in Adults with Sickle Cell Disease and Pulmonary Hypertension: A Proof-of-Concept Study
Published on: 22nd April, 2024
Pulmonary hypertension (PH) in sickle cell disease (SCD) is associated with a mortality rate of 37%. There is an upregulation of adhesion molecules which leads to the expression of endothelin-1, a potent vasoconstrictor. A prospective, descriptive study was done to determine the safety and efficacy of macitentan in patients with SCD and PH. Continuous variables were reported as mean ± SEM or percentage where appropriate. We screened 13 patients and recruited five. All five patients were adults. Data were analyzed as appropriate by student t - test. Statistical significance was assumed at p < 0.05. Baseline pulmonary hemodynamics obtained by right heart catheterization and systemic hemodynamics were (± SEM): mean pulmonary artery pressure (MPAP) 32 ± 8 mmHg, right atrial pressure (RAP) 9 ± 4 mmHg, pulmonary vascular resistance (PVR) 257 dynes-sec/cm5 and CI 3·7 ± 0.39 l/m2. Of all parameters, only PVR and 6-min walk distance changed significantly. For the group, MPAP decreased by 15.6%, PVR by 22.5% and RAP by 25.5%. The 6-minute walk distance increased over sixteen weeks except in Patient 4 who had a 3% decrease. The mean walk distance increased in the total distance, from 464 ± 158 meters to 477 ± 190 meters (p .123). In four patients, the adverse events were mild to moderate and did not lead to study drug discontinuation. Significant improvement in pulmonary hemodynamics and exercise capacity in patients with SCD-related pulmonary arterial hypertension. We found that macitentan was safe and well tolerated.
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