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Polish Archives of Internal Medicine Feb 2024
Topics: Humans; Eisenmenger Complex; Hypertension, Pulmonary
PubMed: 38133886
DOI: 10.20452/pamw.16652 -
Methodist DeBakey Cardiovascular Journal 2021Pulmonary arterial hypertension is a common complication in patients with congenital heart disease (CHD), aggravating the natural course of the underlying defect.... (Review)
Review
Pulmonary arterial hypertension is a common complication in patients with congenital heart disease (CHD), aggravating the natural course of the underlying defect. Pulmonary arterial hypertension (PAH) has a multifactorial etiology depending on the size and nature of the cardiac defect as well as environmental factors. Although progress has been made in disease-targeting therapy using pulmonary vasodilators to treat Eisenmenger syndrome, important gaps still exist in the evaluation and management of adult patients with CHD-associated PAH (PAH-CHD) who have systemic-to-pulmonary shunts. The choice of interventional, medical, or both types of therapy is an ongoing dilemma that requires further data. This review focuses on the evaluation and management of PAH-CHD in the contemporary era.
Topics: Adult; Eisenmenger Complex; Heart Defects, Congenital; Humans; Hypertension, Pulmonary; Lung; Pulmonary Arterial Hypertension
PubMed: 34326934
DOI: 10.14797/UFEJ2329 -
Journal of the Royal Society of Medicine Dec 2019
Review
Topics: Cardiac Surgical Procedures; Decision Making; Eisenmenger Complex; Humans
PubMed: 31526213
DOI: 10.1177/0141076819877551 -
Magnetic Resonance in Medicine Feb 2023To investigate the acceleration of 4D-flow MRI using a convolutional neural network (CNN) that produces three directional velocities from three flow encodings, without...
PURPOSE
To investigate the acceleration of 4D-flow MRI using a convolutional neural network (CNN) that produces three directional velocities from three flow encodings, without requiring a fourth reference scan measuring background phase.
METHODS
A fully 3D CNN using a U-net architecture was trained in a block-wise fashion to take complex images from three flow encodings and to produce three real-valued images for each velocity component. Using neurovascular 4D-flow scans (n = 144), the CNN was trained to predict velocities computed from four flow encodings by standard reconstruction including correction for residual background phase offsets. Methods to optimize loss functions were investigated, including magnitude, complex difference, and uniform velocity weightings. Subsequently, 3-point encoding was evaluated using cross validation of pixelwise correlation, flow measurements in major arteries, and in experiments with data at differing acceleration rates than the training data.
RESULTS
The CNN-produced 3-point velocities showed excellent agreements with the 4-point velocities, both qualitatively in velocity images, in flow rate measures, and quantitatively in regression analysis (slope = 0.96, R = 0.992). Optimizing the training to focus on vessel velocities rather than the global velocity error and improved the correlation of velocity within vessels themselves. The lowest error was observed when the loss function used uniform velocity weighting, in which the magnitude-weighted inverse of the velocity frequency uniformly distributed weighting across all velocity ranges. When applied to highly accelerated data, the 3-point network maintained a high correlation with ground truth data and demonstrated a denoising effect.
CONCLUSION
The 4D-flow MRI can be accelerated using machine learning requiring only three flow encodings to produce three-directional velocity maps with small errors.
Topics: Blood Flow Velocity; Reproducibility of Results; Magnetic Resonance Imaging; Machine Learning; Imaging, Three-Dimensional
PubMed: 36198027
DOI: 10.1002/mrm.29469 -
CVIR Endovascular Jun 2021To describe technical details of modifying four different Cook Zenith devices to treat complex aortic aneurysms.
PURPOSE
To describe technical details of modifying four different Cook Zenith devices to treat complex aortic aneurysms.
MATERIAL
In the first three cases, the modification process involved complete stent graft deployment on a sterile back table. Fenestrations were created using an ophthalmologic cautery and reinforced with a radiopaque snare using a double-armed 4-0 Ethibond locking suture based on measurements obtained on centerline of flow. In each instance, a nitinol wire was withdrawn and redirected through and through the fabric and used as a constraining wire. In the fourth patient, modification involved partial stent graft deployment and creation of additional two fenestrations to accommodate renal arteries. The devices are resheathed and implanted in the standard fashion.
RESULTS
Four patients underwent exclusion of their aneurysms, including thoracoabdominal aneurysms (n = 2), a contained ruptured juxtarenal aneurysm (n = 1), and a ruptured failed previous endovascular repair (n = 1). Fifteen fenestrations were successfully bridged with Atrium iCAST stent grafts. Average graft modification time, operative time, contrast volume, radiation dose, estimated blood loss, and hospital length of stay were 89 min, 155.25 min, 58.8 mL, 2451 mGy, 175 mL, and 4.3 days, respectively. One patient required a secondary intervention to treat a type Ib endoleak. During an average follow-up of 25 months, aneurysm sacs progressively shrank without additional intervention.
CONCLUSION
Physician-modified fenestrated/branched endografts are a safe alternative to custom made devices, especially in urgent cases and should be part of the armamentarium of any complex aortic program.
PubMed: 34061297
DOI: 10.1186/s42155-021-00233-7 -
Journal of the American Heart... Apr 2022Background The epidemiology of pulmonary hypertension (PH) in patients with adult congenital heart disease in Western countries is already known. We investigate clinical...
Background The epidemiology of pulmonary hypertension (PH) in patients with adult congenital heart disease in Western countries is already known. We investigate clinical characteristics of PH in adult congenital heart disease with emphasis on complex congenital heart disease (CHD) from an Asian cohort in Taiwan. Methods and Results All adult patients (aged >18 years) diagnosed with CHD between January 2007 and July 2018 qualified for the study. PH was determined by cardiac catheterization data or echocardiography reports. In accord with the World Symposia on Pulmonary Hypertension, CHD was further categorized as simple, severe, or complex CHD (including pulmonary atresia-ventricular septal defect and single-ventricle anomalies). There were 4301 patients (55.6% women), 15.7% with severe and 3.9% with complex CHD. The cumulative incidence of PH was 4.4% (95% CI, 3.8-5.0). Our multivariable regression model indicated 4.2-fold mortality increase (95% CI, 3.0-5.9) in the presence of PH, with age, female sex, and severe or complex CHD linked to higher incidence of PH. Only 49% of patients received PH-specific therapy. Five- and 10-year survival rates of patients with PH (n=190) were 72.3% (95% CI, 65.1%-78.4%) and 58.8% (95% CI, 50.1%-66.5%), respectively. Survival rates in those with Eisenmenger syndrome, PH after defect correction, and complex CHD were similar. Low oxygen saturation and high uric acid levels were associated with increased mortality. Conclusions In this sizable Asian adult CHD cohort, the cumulative incidence of PH was aligned with that of Western countries. Mortality proved higher in patients with PH versus without PH. Although complex CHD carried greater risk of PH compared with other adult CHD subsets, survival rate was similar.
Topics: Adolescent; Adult; Asia; Eisenmenger Complex; Female; Heart Defects, Congenital; Heart Septal Defects, Ventricular; Humans; Hypertension, Pulmonary; Male
PubMed: 35285668
DOI: 10.1161/JAHA.121.022596 -
Journal of Pregnancy 2021This study is aimed at evaluating the maternal and perinatal characteristics and pregnancy outcomes of ES. . This is a retrospective cohort study of pregnancy with...
OBJECTIVES
This study is aimed at evaluating the maternal and perinatal characteristics and pregnancy outcomes of ES. . This is a retrospective cohort study of pregnancy with Eisenmenger syndrome (ES) in Dr. Soetomo Hospital from January 2018 to December 2019. Total sampling size was obtained. We collected all baseline maternal-perinatal characteristic data, cardiac status, and pregnancy outcomes as primary outcomes. The maternal death cases were also evaluated, and we compared characteristics based on defect size (< or >3 cm).
RESULTS
During study periods, we collected 18 cases with ES from a total of 152 pregnancies with heart disease. The underlying heart disease type includes atrial septal defect (ASD), ventricle septal defect (VSD), and patent ductus arteriosus (PDA). All cases suffered pulmonary hypertension (PH), 3 cases moderate, and 15 cases as severe. 94% of cases fall into heart failure (DC FC NYHA III-IV) during treatment. The majority of cases are delivered by cesarean section (88.9%). Pregnancy complications found include preterm birth (78%), low birthweight (94%), intrauterine growth restriction (55%), oligohydramnios (16%), severe preeclampsia (33%), and placenta previa (5.5%). Large defect group has an older maternal ages (30.18 ± 4.60 vs. 24.15 ± 2.75; = 0.002), higher clinical sign (100 vs. 40%, = 0.003), and higher preterm delivery rate (100% vs. 69%, = 0.047) compared to small defect groups. The R to L or bidirectional shunt is significantly higher at the large defect group (13 vs. 5 cases, = 0.006, 95% confidence interval: -1.156 to -0.228). There were seven maternal death cases caused by shock cardiogenic.
CONCLUSIONS
Pregnancy with ES is still associated with very high maternal neonatal mortality and morbidity. The larger defect size is correlated with clinical performances and pregnancy outcomes. Effective preconception counseling is the best strategy to reduce the risk of maternal and neonatal death in ES women.
Topics: Cesarean Section; Eisenmenger Complex; Female; Humans; Infant Mortality; Infant, Newborn; Morbidity; Pregnancy; Pregnancy Outcome; Premature Birth; Retrospective Studies
PubMed: 34616573
DOI: 10.1155/2021/3248850 -
Journal of the American Heart... Mar 2020Background Patients with Eisenmenger syndrome are known to have a high incidence of sudden cardiac death (SCD), yet the underlying causes are not well understood. We...
Background Patients with Eisenmenger syndrome are known to have a high incidence of sudden cardiac death (SCD), yet the underlying causes are not well understood. We sought to define the predictors of SCD in this population. Methods and Results A retrospective analysis of all patients with Eisenmenger syndrome from 2 large tertiary referral centers was performed. ECGs, prolonged ambulatory recordings, echocardiograms, and clinical histories were reviewed; and the cause of death was identified. A total of 246 patients (85 [34.6%] men) with a mean age of 37.3 (±14.2) years were followed up for a median of 7 years. Over the study period, 136 patients died, with 40 experiencing SCD and 74 experiencing cardiac death (sudden and nonsudden). Age, atrial fibrillation, prolonged QRS duration, complete heart block, right atrial enlargement, right bundle branch block, increased right atrial pressure, impaired biventricular function, and the presence of a pacemaker were associated with increased risk of SCD, whereas advanced pulmonary hypertension therapies were protective. Atrial fibrillation (11.45-fold increased risk; <0.001) and QRS duration ≥120 ms (2.06-fold increased risk; =0.034) remained significant predictors of SCD in the multivariate analysis, whereas advanced pulmonary hypertension therapies were strongly protective against SCD (<0.001). Conclusions Atrial arrhythmias, impaired ventricular function, and conduction system disease were associated with increased risk of SCD in this cohort of patients with Eisenmenger syndrome, providing an opportunity for early risk stratification and potential intervention. Clinical heart failure symptoms (New York Heart Association class ≥II) were predictive of increased mortality but not of SCD, suggesting a potential arrhythmic cause behind SCD.
Topics: Adult; Arrhythmias, Cardiac; Cause of Death; Death, Sudden, Cardiac; Eisenmenger Complex; Female; Florida; Humans; Los Angeles; Male; Middle Aged; Retrospective Studies; Risk Assessment; Risk Factors; Time Factors; Young Adult
PubMed: 32174228
DOI: 10.1161/JAHA.119.014554 -
Frontiers in Cardiovascular Medicine 2022Atrial septal defect (ASD) is the most common congenital heart disease (CHD) in adults and pulmonary hypertension (PH) is an established risk factor. A decision whether...
BACKGROUND
Atrial septal defect (ASD) is the most common congenital heart disease (CHD) in adults and pulmonary hypertension (PH) is an established risk factor. A decision whether to perform ASD closure, especially in elderly patients with PH, is a complex dilemma. The aim of our study was to compare long-term survival in patients with closed and open ASD.
METHODS
A retrospective cohort study was performed on 427 patients with ASD (median age at diagnosis 38 years, IQR 18-56) out of which 186 patients (44%) manifested PH. ASD closure in patients with PH was only considered in patients without Eisenmenger syndrome with pulmonary vascular resistance < 5 WU. Median follow-up duration was 18 years (IQR 9-31 years). Kaplan-Meier and Cox proportional hazards survival analyses were performed to evaluate 12 potential predictors of survival.
RESULTS
Defect closure was associated with improved long-term survival in ASD patients both with ( < 0.001) and without PH ( = 0.01) and this association was present also in patients over 40 years. The 20-year survival since diagnosis was significantly higher in patients with PH and closed ASD compared to those with PH and open ASD (65% vs. 41%). ASD closure was a significant independent predictor of long-term survival ( = 0.003) after accounting for age at diagnosis, PH, NYHA class, Eisenmenger syndrome, and mitral regurgitation. Significant negative independent predictors of survival were older age at diagnosis ( < 0.001), Eisenmenger syndrome ( < 0.001), and PH ( = 0.03).
CONCLUSION
ASD closure appears to be associated with improved long-term survival independently of age, PH, and other clinical variables.
PubMed: 35571174
DOI: 10.3389/fcvm.2022.867012 -
Methodist DeBakey Cardiovascular Journal 2020
Topics: Adult; Cor Triatriatum; Eisenmenger Complex; Female; Heart Septal Defects, Ventricular; Humans
PubMed: 32670480
DOI: 10.14797/mdcj-16-2-168