RESUMO
Aortic wall injuries may occur after interventional treatment of aortic coarctation (CoA), especially after balloon angioplasty. We reported on a patient who presented with an intra-stent aneurysm formation after direct stenting of a native near atretic aortic CoA by using a BeGraft Aortic stent. This evidence supports the need to maintain a strict follow-up protocol. A computed tomography scan is mandatory, after covered stent implantation as well, especially in high-risk cases and even in the absence of any immediate apparent complication.
Assuntos
Aneurisma Aórtico , Coartação Aórtica , Humanos , Resultado do Tratamento , Aorta/lesões , Aneurisma Aórtico/diagnóstico por imagem , Aneurisma Aórtico/etiologia , Aneurisma Aórtico/cirurgia , Stents/efeitos adversos , Coartação Aórtica/diagnóstico por imagem , Coartação Aórtica/cirurgiaRESUMO
AIM: To describe our initial experience with the indications and results of the 5/7 Occlutech® duct Occluder (ODO, Occlutech International AB, Helsingborg, Sweden). A small incremental increase in occluder sizes is of utmost importance for successful outcomes, especially in smaller patients in whom protrusion of the distal disk towards the aorta should be minimised. METHODS: Retrospective study of all patients undergoing PDA closure with the 5/7 ODO in three institutions since 2018. RESULTS: The 5/7 ODO was used in 18 patients with median age and weight at the time of the procedure of 17.5 months (interquartile range 25th to 75th percentile 8 months- 4.4 years) and 13.6 kg (interquartile range 25th to 75th percentile 6.4-22.5 kg) respectively. All cases were successful. There were no cases of device embolisation, haemolysis, or flow disturbance of the LPA or the aorta. CONCLUSIONS: This small retrospective study demonstrated an excellent outcome of transcatheter PDA closure with the 5/7 ODO. The device is a beneficial complement to the existing sizes of PDA devices, filling the gap between the 4/6 and 6/8 ODO and avoiding protrusion of a larger disk in the aortic isthmus.
Assuntos
Permeabilidade do Canal Arterial , Dispositivo para Oclusão Septal , Humanos , Pré-Escolar , Estudos Retrospectivos , Resultado do Tratamento , Desenho de Prótese , Cateterismo Cardíaco/efeitos adversos , Cateterismo Cardíaco/métodosRESUMO
Postmortem toxicology is an ever-changing landscape presenting challenges for toxicologists and medical examiners. Trends can vary for certain benzodiazepines. While diazepam use tends to remain constant, alprazolam and clonazepam ebb and flow depending on prescription trends and street popularity. Novel benzodiazepines like etizolam vary in casework depending on legal restrictions and black-market availability. In May 2022, the first case of the designer benzodiazepine, bromazolam, was detected in Jefferson County Coroner/Medical Examiner's Office (JCCMEO) casework. Between then and December of 2023, an additional nine cases have been observed. Bromazolam, is the brominated analog of the low dose benzodiazepine alprazolam and is likely similar in potency. As bromazolam is a potent benzodiazepine, low concentrations of this novel drug are likely to contribute to CNS depression in opioid overdose cases. We present ten cases in which bromazolam was detected in postmortem samples at the University of Alabama at Birmingham (UAB) toxicology laboratory. The decedents ranged in age from 20 to 41 years of age. Most of the decedents were White (n = 8; 80%) and male (n = 7; 70%). Bromazolam concentrations ranged from 21 to 3,220 ng/mL (mean 401 ng/mL). All but one case were polydrug and all deaths were related to drug toxicity. Fentanyl was detected in 8 of the 10 decedents, with concentrations ranging from <2.5 to 97 ng/mL (mean 30 ng/mL). Additional drugs detected were methamphetamine, ethanol, oxycodone, methadone, cocaine, amphetamine, morphine, and diphenhydramine. While all manners of death were ruled as accidental, bromazolam was included in the cause of death statements in nine of the ten cases. Capturing important emerging drug trends on the death certificate is critical to help inform public health and medical colleagues for preventive measures and treatment in the continued drug epidemic.
RESUMO
BACKGROUND: Stent implantation has become the preferred method of treatment for treating vessel stenosis in congenital heart diseases. The availability of covered stents may decrease complications and have an important role in the management of patients with complex anatomy. AIM: This study aims to evaluate the feasibility and safety of the pre-mounted cobalt-chromium stent-graft-covered ePTFE Aortic BeGraft in a broad spectrum of vascular lesions. METHODS: This is a multicenter retrospective results analysis of 107 implanted BeGraft stents between 2016 and 2022 in six different European centers. RESULTS: One hundred and four patients with a mean age of thirteen years (range 1-70 years) and with the body weight of 56.5 kg (range 11-115 kg) underwent the BeGraft stent implantation. Stents were implanted in the following conditions: aortic coarctation (74 patients), RVOT dysfunction (12 patients), Fontan circulation (7 patients), and miscellaneous (11 subjects with complex CHD). All the stents were implanted successfully. The median stent diameter was 16 mm (range 7-24 mm), and the median length was 39 mm (range 19-49 mm). Major complications occurred in five subjects (4.7%). During a median follow-up of fourteen (1-70) months, stents' re-dilatation was performed in five patients. CONCLUSIONS: The BeGraft stent can be used safely and effectively in a wide spectrum of congenital heart diseases. Whether these good results will be stable in the longer term still needs to be investigated in a follow-up given its recent introduction into clinical practice, in particular regarding stent fracture or neointimal proliferation.
RESUMO
Bicuspid aortic valve (BAV) is the most common congenital heart malformation in adults but can also cause childhood-onset complications. In multicenter study, we found that adults who experience significant complications of BAV disease before age 30 are distinguished from the majority of BAV cases that manifest after age 50 by a relatively severe clinical course, with higher rates of surgical interventions, more frequent second interventions, and a greater burden of congenital heart malformations. These observations highlight the need for prompt recognition, regular lifelong surveillance, and targeted interventions to address the significant health burdens of patients with early onset BAV complications.
RESUMO
Background: The Occlutech Atrial Flow Regulator (AFR) is a self-expandable double-disc nitinol device with a central fenestration. Its use has been approved in the adult population with heart failure and described for pulmonary hypertension (PH). Only case reports and small series have been published about its use in the paediatric population and for congenital heart disease (CHD). Objectives: The authors sought to investigate the feasibility, safety, and short-term follow-up of AFR implantation in patients with CHD or children with PH or cardiomyopathy. Methods: This is a multicenter retrospective study involving 10 centers worldwide. Patients of any age with CHD or patients aged < 18 years with PH or cardiomyopathy needing AFR implantation were included. Results: A total of 40 patients underwent AFR implantation. The median age of the population at the time of the procedure was 58.5 months (IQR: 31.5-142.5) and the median weight was 17â kg (IQR: 10-46). A total of 26 (65.0%) patients had CHD, nine (22.5%) children, a cardiomyopathy, and five (12.5%), a structurally normal heart. The implantation success rate was 100%. There were two early and one late device thrombosis. Two patients (5.0%) with dilated cardiomyopathy on extracorporeal membrane oxygenator (ECMO) died during the hospital stay. At a median follow-up of 330 days (IQR: 125-593), 37 (92.5%) patients were alive. At follow-up, 20 patients improved their New York Heart Association (NYHA) class, 12 patients did not change their NYHA class, and one patient with idiopathic PH worsened. Conclusions: AFR implantation in patients with CHD and children with severe PH or cardiomyopathy is promising and seems to have beneficial effects at short-term follow-up.
RESUMO
Bicuspid aortic valve (BAV), the most common congenital heart defect, is a major cause of aortic valve disease requiring valve interventions and thoracic aortic aneurysms predisposing to acute aortic dissections. The spectrum of BAV ranges from early onset valve and aortic complications (EBAV) to sporadic late onset disease. Rare genomic copy number variants (CNVs) have previously been implicated in the development of BAV and thoracic aortic aneurysms. We determined the frequency and gene content of rare CNVs in EBAV probands (n = 272) using genome-wide SNP microarray analysis and three complementary CNV detection algorithms (cnvPartition, PennCNV, and QuantiSNP). Unselected control genotypes from the Database of Genotypes and Phenotypes were analyzed using identical methods. We filtered the data to select large genic CNVs that were detected by multiple algorithms. Findings were replicated in cohorts with late onset sporadic disease (n = 5040). We identified 34 large and rare (< 1:1000 in controls) CNVs in EBAV probands. The burden of CNVs intersecting with genes known to cause BAV when mutated was increased in case-control analysis. CNVs intersecting with GATA4 and DSCAM were enriched in cases, recurrent in other datasets, and segregated with disease in families. In total, we identified potentially pathogenic CNVs in 8% of EBAV cases, implicating alterations of candidate genes at these loci in the pathogenesis of BAV.
RESUMO
BACKGROUND: Heritable thoracic aortic diseases (HTAD), typically entailing aortic complications, can be caused by pathogenic variants or likely pathogenic variants (PV/LPVs) in several genes, including fibrillin1 (FBN1), Actin Alpha2 (ACTA2) and genes encoding components of the transforming growth factor (TGF)-ß signaling pathway. In addition to aortic complications, non-aortic cardiac disease such as impaired myocardial function and/or arrhythmia have been increasingly reported, mainly in Marfan syndrome with underlying FBN1 PV/LPVs and are acknowledged as additional causes of morbidity and mortality. The prevalence of these manifestations in the various HTAD entities is largely unknown. METHODS: This international multicentre retrospective study collected data on patients with HTAD presenting non-aortic cardiac disease. A total of 9 centers from 7 different countries participated. Patients 12 years or older carrying a PV/LPV in one of the following genes: FBN1, TGFBR1, TGFBR2, TGFB2, TGFB3, SMAD3 and ACTA2 were screened. Non-aortic cardiac disease included impaired myocardial function and/or arrhythmia. Impaired myocardial function was defined as (a)symptomatic reduced ejection fraction (EF<50%). Arrhythmias included atrial fibrillation (AF), atrial flutter (AFL), ventricular tachycardia (VT), ventricular fibrillation (VF) and (aborted) sudden cardiac death (presumed arrhythmogenic) (SCD). RESULTS: Medical records of 3219 patients with HTAD were screened (2761, 385 and 73 carrying a PV/LPV in FBN1, in a TGF-ß signaling gene and in ACTA2 respectively). Non-aortic cardiac disease was reported 142 times in 101 patients (3.1%) (age 37 [range 12-77] years, 39% female): 88 patients carrying an FBN1 PV/LPV and 13 carrying a PV/LPV in one of the TGF-ß signaling genes. Neither impaired myocardial function nor arrhythmia was reported in screened patients carrying a PV/LPV in ACTA2. Among the 142 reported non-aortic cardiac diseases, 68 (48%) were impaired myocardial function, 47 (33%) were AF/AFL and 27 (19%) were VT/VF/SCD. Among the patients with non-aortic cardiac disease, prior cardiac surgery was noted in 80% and severe valvular disease (valvular surgery or severe valvular regurgitation) in 58%, while 18% of the patients developed non-aortic cardiac disease in the absence of any of the latter. CONCLUSIONS: In patients with HTAD, arrhythmia and impaired myocardial function was reported in patients with PV/LPVs in FBN1 and in the TGF-ß signaling genes and not in patients harboring PV/LPVs in ACTA2. Though infrequent, non-aortic cardiac disease should be acknowledged as potentially severe, also occurring in young patients with no underlying significant valvular or aortic disease.