Background: Pulmonary arteriovenous malformations (PAVMs) are direct connections between the pulmonary artery and the pulmonary vein, mostly associated with hereditary hemorrhagic telangiectasia (HHT). PAVMs can lead to severe neurologic complications such as stroke and brain abscess. The risk of complications decreases after embolization. Therefore, screening for PAVMs using transthoracic contrast echocardiography (TTCE) is recommended, including a rescreening interval of 5 years.
Research Question: Is extension of the interval for rescreening patients without a pulmonary right-to-left shunt (RLS) of up to 10 years appropriate?
Study Design And Methods: Adult patients with HHT with 5- or 10-year follow-up TTCE, or both, were included. Patients who underwent PAVM embolization in the past or at baseline were excluded. The RLS grades and presence of a treatable PAVM were compared with baseline.
Results: In total, 387 patients (median age, 45 years [interquartile range, 33-54 years]; 56% women) involving 5- and 10-year follow-up data in 363 and 166 patients, respectively, were included. None of the patients (n = 148) without a pulmonary RLS at baseline demonstrated a treatable PAVM after 5 and 10 years. Of the patients with a pulmonary RLS at baseline, 20 patients (9%) and three patients (3%) demonstrated a treatable PAVM at the 5- and 10-year follow-up, respectively. In most patients, the RLS grade remained stable over time.
Interpretation: On the basis of the results of this retrospective study, we believe that the rescreening interval for patients with HHT without a pulmonary RLS at initial screening may be extended to 10 years. Those with a pulmonary RLS should be rescreened every 5 years because treatable PAVMs can evolve.
Download full-text PDF |
Source |
|---|---|
| http://dx.doi.org/10.1016/j.chest.2022.11.005 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Design, synthesis, biological evaluation and multi spectroscopic studies of novel 2-styrylquinoline-carboxamide derivatives as potential DNA intercalating anticancer agents.
Bioorg Chem
December 2024
Biotechnology Research Center, Pharmaceutical Technology Institute, Mashhad University of Medical Sciences, Mashhad, Iran; Department of Medicinal Chemistry, School of Pharmacy, Mashhad University of Medical Sciences, Mashhad, Iran. Electronic address:
In this study, novel 2-styrylquinoline derivatives possessing a planar aromatic system and a flexible side chain with an amino substituent were designed and synthesized as DNA-intercalating antitumor agents. The cytotoxic activity of the synthesized compounds was evaluated against four cancer cell lines including MCF-7 (breast cancer cells), A549 (lung epithelial cancer cells), HCT116 (colon cancer cells) and normal cell line L929 (mouse fibroblast cell line). The results displayed that the anti-cancer activity of the target quinolines is sensitive to the lipophilic nature of the C-6 and C-7 quinoline substituents.
View Article and Find Full Text PDFCorrelation of right ventricular outflow tract residual disease on post-op transesophageal echocardiography and discharge transthoracic echocardiography after tetralogy of fallot repair.
Int J Cardiovasc Imaging
December 2024
Divisions of Cardiology, Children's National Hospital, The George Washington University School of Medicine and Health Science, Washington, DC, USA.
Post-operative transesophageal echocardiography (TEE) is standard of care to evaluate for residual disease and quality of surgical repair. The residual lesion score (RLS) as defined by the pediatric heart network explored the impact of residual lesions on outcomes using discharge transthoracic echocardiogram (TTE). In tetralogy of fallot with pulmonary stenosis (TOF/PS), the residual right ventricular outflow tract (RVOT) gradient is one marker of quality of repair.
View Article and Find Full Text PDFLong-term follow-up of hereditary hemorrhagic telangiectasia patients without significant pulmonary right-to-left shunt at screening.
Eur J Intern Med
December 2024
HHT Unit. Hospital Universitari Bellvitge, Barcelona, Spain; Internal Medicine Department. Hospital Universitari Bellvitge, Barcelona, Spain; Bellvitge Biomedical Research Institute (IDIBELL), Barcelona, Spain; Clinical Sciences Department. Faculty of Medicine and Health Sciences. Universitat de Barcelona, Barcelona, Spain; Center for Biomedical Research in Obesity and Nutrition Physiopathology Network (CIBEROBN). Carlos III Health Institute, Madrid, Spain. Electronic address:
Objectives: To describe the incidence of pulmonary arteriovenous malformations (PAVM)-related complications, right-to-left shunt (RLS) progression at transthoracic contrast echocardiography (TTCE) and development of treatable PAVM during long-term follow-up in hereditary hemorrhagic telangiectasia (HHT) patients with RLS grades 0-1 at screening TTCE.
Methods: Observational prospective study including adult HHT patients with grades 0-1 RLS at screening TTCE. Those requiring previous embolization of PAVM were excluded.
Sickle Trait and Alpha Thalassemia Increase NOS-Dependent Vasodilation of Human Arteries Through Disruption of Endothelial Hemoglobin-eNOS Interactions.
Circulation
January 2025
Physiology Unit, Laboratory of Malaria and Vector Research (S.D.B., A.P.R., X.Z., M.A.H., L.A.R., R.L.S., M.J., J.N.d.R., A.J.M., J.M.J., R.O.E., N.T., K.L., H.C.A.), National Institute of Allergy and Infectious Diseases, Rockville, MD.
Background: Severe malaria is associated with impaired nitric oxide (NO) synthase (NOS)-dependent vasodilation, and reversal of this deficit improves survival in murine models. Malaria might have selected for genetic polymorphisms that increase endothelial NO signaling and now contribute to heterogeneity in vascular function among humans. One protein potentially selected for is alpha globin, which, in mouse models, interacts with endothelial NOS (eNOS) to negatively regulate NO signaling.
View Article and Find Full Text PDFSleep-related movement disorders in children: recent updates.
World J Pediatr
November 2024
Sleep Center, Division of Pulmonary and Sleep Medicine, Cincinnati Children's Hospital Medical Center, 3333 Burnet Ave, MLC 7041, Cincinnati, OH, USA.
Article Synopsis
- Sleep-related movement disorders (SRMDs) like restless legs syndrome (RLS) and periodic limb movement disorder (PLMD) are common in children but often go unrecognized; a new disorder called restless sleep disorder (RSD) has also been identified.
- The causes of these disorders include genetic factors, problems with dopamine function, and iron deficiency, with RLS and RSD possibly sharing similar mechanisms.
- Effective treatments are available, including iron therapy and specific medications, but further research is necessary to better understand RSD and improve care for affected children.
Want AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!