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.
Jeany P Villamizar, Laiqua Khalid, Emilia N Faraj, Thomas J Harrington and David J De La Zerda*
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.
Your journal has accomplished its intended mission of providing very effective and efficient goals in dealing with submissions, conducting the reviewing process and in publishing accepted manuscripts in a timely manner. Keep up the great work and services that you provide.
University of Jacqmar, Inc., USA
John St. Cyr
''Co-operation of Archives of Surgery and Clinical Research journal is appreciable. I'm impressed at the promptness of the publishing staff and the professionalism displayed. Thank you very much for your support, help and encouragement.''
Anıl Gokce
You are such a nice person. Your journal co-operation is very appreciable and motivational.
Department of Biotechnology, Uttaranchal college of Applied and Life Sciences, Uttaranchal University, Dehradun, Uttarakhand, India
Archna Dhasmana
The Clinical Journal of Obstetrics and Gynecology is an open access journal focused on scientific knowledge publication with emphasis laid on the fields of Gynecology and Obstetrics. Their services toward us have been encouraging through their kindness and respect. Great consideration has been given to us as young budding researchers and we are very grateful for this.
Carole Assontsa
"It was a pleasure to work with the editorial team of the journal on the submission of the manuscript. The team was professional, fast, and to the point".
NC A&T State University, USA
Moran Sciamama-Saghiv
Many thanks for publishing my article in your great journal and the friendly and hassle-free publication process, the constructive peer-review, the regular feedback system, and the Quick response to any queries.
Azab Elsayed Azab
We really appreciate your efforts towards our article, the professional way you handle our request for exemption from charges.
It was a great honor for us to publish in your magazine.
Achraf elbakkaly
"This is my first time publishing with the journal/publisher. I am impressed at the promptness of the publishing staff and the professionalism displayed. Thank you for encouraging young researchers like me!"
Ajite Kayode
Your journal co-operation is very appreciable and motivational. I am really thankful to your journal and team members for the motivation and collaboration to publish my work.
Assistant Professor, UCLAS Uttaranchal University, Dehradun, India
Archna Dhasmana
My candid opinion is that the service you render is second to none. My favourite part is the prompt response to issue, really i value that.
If you are already a member of our network and need to keep track of any developments regarding a question you have already submitted, click "take me to my Query."