Concomitant pulmonary vein isolation and percutaneous closure of atrial septal defects: A pilot project

Background: Patients with an atrial septal defect (ASD) are at increased risk of developing atrial fibrillation (AF). Currently percutaneous ASD closure is the preferred therapeutic strategy and although pulmonary vein isolation (PVI) for AF is feasible after ASD closure, the transseptal puncture can be technically challenging and probably increases the perioperative risk. A staged approach, with PVI several months before ASD closure, has been recommended for patients already scheduled for closure, but no data are available on combined procedures. Purpose: This pilot study evaluates the feasibility of a combined procedure of PVI and ASD closure in patients with a hemodynamic important ASD and documented AF. Methods: In one procedure, PVI was performed prior to placement of the ASD closure device. Transseptal access for PVI was obtained via wire passage through the ASD in all patients. Patients were followed with 5-day-holter monitoring at 3, 6, and 12 months. Recurrence of AF was defined as a documented, symptomatic episode of AF. Results: The study population consisted of five patients (four females, mean age: 58 (±3) years). Acute PVI was achieved in all patients. Only one patient had a small residual ASD after closure. Besides a small groin hematoma in two patients, no complications occurred. After 12-month follow-up, three patients were free of AF recurrence (60%). Conclusion: This study shows that a combined PVI with ASD closure is feasible with an acceptable success rate of AF free survival. These preliminary results in a small patient group warrants a larger trial

Transthoracic 3D echocardiographic left heart chamber quantification in patients with bicuspid aortic valve disease

Integration of volumetric heart chamber quantification by 3D echocardiography into clinical practice has been hampered by several factors which a new fully automated algorithm (Left Heart Model, (LHM)) may help overcome. This study therefore aims to evaluate the feasibility and accuracy of the LHM software in quantifying left atrial and left ventricular volumes and left ventricular ejection fraction in a cohort of patients with a bicuspid aortic valve. Patients with a bicuspid aortic valve were prospectively included. All patients underwent 2D and 3D transthoracic echocardiography and computed tomography. Left atrial and ventricular volumes were obtained using t

Left ventricular global longitudinal strain in bicupsid aortic valve patients: head-to-head comparison between computed tomography, 4D flow cardiovascular magnetic resonance and speckle-tracking echocardiography

Left ventricular global longitudinal strain (LVGLS) analysis is a sensitive measurement of myocardial deformation most often done using speckle-tracking transthoracic echocardiography (TTE). We propose a novel approach to measure LVGLS using feature-tracking software on the magnitude dataset of 4D flow cardiovascular magnetic resonance (CMR) and compare it to dynamic computed tomography (CT) and speckle tracking TTE derived measurements. In this prospective cohort study 59 consecutive adult patients with a bicuspid aortic valve (BAV) were included. The study protocol consisted of TTE, CT, and CMR on the same day. Image analysis was done using dedicated feature-tracking (4D flow CMR and CT) and speckle-tracking (TTE) software, on apical 2-, 3-, and 4-chamber long-axis multiplanar reconstructions (4D flow CMR and CT) or standard apical 2-, 3-, and 4-chamber acquisitions (TTE). CMR and CT GLS analysis was feasible in all patients. Good correlations were observed for GLS measured by CMR (− 21 ± 3%) and CT (− 20 ± 3%) versus TTE (− 20 ± 3%, Pearson’s r: 0.67 and 0.65, p 0.61, p < 0.001). Feature-tracking GLS analysis is feasible using the magnitude images acquired with 4D flow CMR. GLS measurement by CMR correlates well with CT and speckle-tracking 2D TTE. GLS analysis on 4D flow CMR allows for an integrative approach, integrating flow and functional data in a single sequence. Not applicable, observational study

Expert consensus recommendations on the cardiogenetic care for patients with thoracic aortic disease and their first-degree

Background: Thoracic aortic aneurysm (TAA) is a potentially life-threatening disorder with a strong genetic component. The number of genes implicated in TAA has increased exponentially over the last decade. Approximately 20% of patients with TAA have a positive family history. As most TAA remain asymptomatic for a long time, screening of at risk relatives is warranted to prevent complications. Existing international guidelines lack detailed instructions regarding genetic evaluation and family screening of TAA patients. We aimed to develop a consensus document to provide medical guidance for all health care professionals involved in the recognition, diagnosis and treatment of patients with thoracic aortic disease and their relatives. Methods: A multidisciplinary panel of experts including cardiologists, cardiothoracic surgeons, clinical geneticists and general practitioners, convened to review and discuss the current literature, guidelines and clinical practice on genetic testing and family screening in TAA. Results: There is a lack of high-quality evidence in the literature. This consensus statement, based on the available literature and expert opinions, summarizes our recommendations in order to standardize and optimize the cardiogenetic care for patients and families with thoracic aortic disease. In particular, we provide criteria to identify those patients most likely to have a genetic predisposition, and discuss the preferred modality and frequency of screening in their relatives. Conclusions: Age, family history, aortic size and syndromic features determine who is advised to have genetic testing as well as screening of first-degree relatives. There is a need for more prospective multicenter studies to optimize current recommendations

Abnormal Aortic Wall Properties in Women with Turner Syndrome

Background Turner syndrome (TS) is associated with aortic dilatation and dissection, but the underlying process is unclear. The aim of this study was to investigate the elastic properties and composition of the aortic wall in women with TS. Methods In this cross-sectional study, 52 women with TS aged 35 ± 13 years (50% monosomy, 12 with bicuspid aortic valve [BAV] and 4 with coarctation) were investigated using carotid-femoral pulse wave velocity (CF-PWV) by echocardiography and ascending aortic distensibility (AAD) and aortic arch pulse wave velocity (AA-PWV) by magnetic resonance imaging (MRI). As control group, 13 women with BAV without TS and 48 healthy patients were included. Results Women with TS showed a higher AA-PWV (β = 1.08, confidence interval [CI]: 0.54-1.62) after correcting for age and comorbidities compared with controls. We found no significant difference in AAD and CF-PWV. In women with TS, the presence of BAV, coarctation of the aorta, or monosomy (45, X) was not associated with aortic stiffness. In addition, aortic tissue samples were investigated with routine and immunohistochemical stains in five additional women with TS who were operated. The tissue showed more compact smooth muscle cell layers with abnormal deposition and structure of ela

The outcome of pulmonary hypertension and its association with pulmonary artery dilatation

Background: Pulmonary artery (PA) dilatation is often seen in pulmonary hypertension (PH) and is considered a long-term consequence of elevated pressure. The PA dilates over time and therefore may reflect disease severity and duration. Survival is related to the stage of the disease at the time of diagnosis and therefore PA diameter might be used to predict prognosis. This study evaluates the outcome of patients with pulmonary arterial hypertension (PAH) and chronic thrombo-embolic pulmonary hypertension (CTEPH) and investigates whether PA diameter at the time of diagnosis is associated with mortality. Methods: Patients visiting an outpatient clinic of a tertiary centre between 2004 and 2018 with a cardiac catheterisation confirmed diagnosis of PAH or CTEPH and a CT scan available for PA diameter measurement were included. PA diameter and established predictors of survival were collected (New York Heart Association (NYHA) class, N‑terminal pro-brain natriuretic peptide (NT-proBNP) level and 6‑min walking distance (6MWD)). Results: In total 217 patients were included (69% female, 71% NYHA class ≥III). During a median follow-up of 50 (22–92) months, 54% of the patients died. Overall survival was 87% at 1 year, 70% at 3 years and 58% at 5 years. The mean PA diameter was 34.2 ± 6.2 mm and was not significantly different among all the diagnosis groups. We found a weak correlation between PA diameter and mean PA pressure (r = 0.23, p < 0.001). Male sex, higher age, shorter 6MWD and higher NT-proBNP level were independently associated with mortality, but PA diameter was not. Conclusion: The prognosis of PAH and CTEPH is still poor. Known predictors of survival were confirmed, but PA diameter at diagnosis was not associated with survival in PAH or CTEPH patients