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Four-dimensional Virtual Catheter: Noninvasive Assessment of Intra-aortic Hemodynamics in Bicuspid Aortic Valve Disease.

Radiology. 2019 12;293(3):541-550

Authors: Elbaz MSM, Scott MB, Barker AJ, McCarthy P, Malaisrie C, Collins JD, Bonow RO, Carr J, Markl M

Background Four-dimensional (4D) flow MRI enables the evaluation of blood flow alterations in patients with congenital bicuspid aortic valve (BAV). However, current analysis methods are cumbersome and lack the use of the volumetric data from 4D MRI. Purpose To investigate the feasibility and reproducibility of a technique that uses a catheter-like mathematical model (virtual catheter) to assess volumetric intra-aortic hemodynamics from 4D flow MRI in patients with BAV. Materials and Methods In this retrospective study, data were collected from adult patients with BAV and healthy participants who underwent aortic 4D flow MRI from November 2011 through August 2014. Reproducibility was tested in healthy study participants who underwent test-retest examinations within 2 weeks. Patients were grouped on the basis of the severity of aortic valve regurgitation (AVR) and aortic valve stenosis (AVS). A 4D virtual catheter mathematical model for probing intra-aortic hemodynamic flow was constructed as a tube with an automatically derived radius along the entire thoracic aorta centerline. Volumetric intra-aortic hemodynamics were computed from 4D flow MRI only within the virtual catheter, and the following volume-normalized systolic peaks were derived: kinetic energy (KE), viscous energy loss rate (VELR), and vorticity. Hemodynamic data were presented as medians with interquartile ranges and compared by using Mann-Whitney U test and Kruskal-Wallis test. Results The study included 91 participants (57 patients [mean age, 46 years ± 12], 18 women; 34 healthy participants [mean age: 44 years ± 14], 12 women; 15 healthy participants underwent test-retest examinations). Patients showed higher VELR values compared with healthy participants (median, 31 W/m3 [interquartile range, 21-72] vs 23 W/m3 [interquartile range, 17-30], respectively; P < .001) and vorticity (69 sec-1 [interquartile range, 59-87] vs 60 sec-1 [interquartile range, 50-67], respectively; P < .001). Four-dimensional virtual catheter showed differences among different AVS and AVR grades with the highest VELR (120 W/m3; interquartile range, 99-166; P < .001) and vorticity (108 sec-1; interquartile range, 84-151; P < .001) found in severe AVS. High test-retest reproducibility was found for all virtual catheter-derived metrics (intraclass correlation, 0.80 ± 0.07; coefficient of variation, 9% ± 3). Conclusion The proposed four-dimensional (4D) virtual catheter technique enabled reproducible automated evaluation of volumetric intra-aortic hemodynamics alterations from 4D flow MRI in patients with bicuspid aortic valve. © RSNA, 2019 Online supplemental material is available for this article. See also the editorial by Mitsouras and Hope in this issue.

PMID: 31592729 [PubMed - indexed for MEDLINE]

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Frequency and Outcomes of Elevated Perioperative Lactate Levels in Adult Congenital Heart Disease Patients Undergoing Cardiac Surgery.

J Cardiothorac Vasc Anesth. 2020 Feb 01;:

Authors: Kim J, Wu A, Grogan T, Wingert T, Scovotti J, Kratzert W, Neelankavil JP

OBJECTIVES: To assess whether lactate levels are associated with clinical outcomes in adult congenital heart disease patients who undergo cardiac surgery.
DESIGN: Retrospective study.
SETTING: Single quaternary academic referral center.
PARTICIPANTS: Adult congenital heart disease patients (≥18 y old) with congenital heart disease undergoing cardiac surgery with cardiopulmonary bypass.
INTERVENTIONS: Participants were classified into 3 groups according to their peak arterial lactate level within the first 48 hours of surgery.
MEASUREMENTS AND MAIN RESULTS: In-hospital 30-day mortality, hospital and intensive care unit length of stay, duration of mechanical ventilation after surgery, acute kidney injury defined by Acute Kidney Injury Network criteria, and intensive care unit and hospital readmission within 30 days of surgery were examined. There was no significant difference among different lactate level groups in acute kidney injury, hospital length of stay, intensive care unit length of stay, hours of mechanical ventilation, need for redo surgery, or rates of hospital or intensive care unit readmission. In multivariable analysis, which included cardiopulmonary bypass time, redo surgery, nonelective case, and the adult congenital heart disease complexity score, lactate levels were not a significant predictor of either acute kidney injury or hospital length of stay.
CONCLUSIONS: The appeal of using lactate levels to risk stratify-patients or to develop a model to predict mortality and morbidity has potential merit, but currently there is insufficient evidence to use lactate levels as a predictor of outcomes in adult patients with congenital heart disease undergoing cardiac surgery.

PMID: 32139342 [PubMed - as supplied by publisher]

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Structural brain abnormalities in adults with congenital heart disease: Prevalence and association with estimated intelligence quotient.

Int J Cardiol. 2020 Feb 24;:

Authors: Kessler N, Feldmann M, Schlosser L, Rometsch S, Brugger P, Kottke R, Knirsch W, Oxenius A, Greutmann M, Latal B

BACKGROUND: Little is known about the prevalence of structural brain abnormalities and cognitive functioning in the growing population of patients with adult congenital heart disease (ACHD). Thus, our aim was to assess structural abnormalities on brain magnetic resonance imaging (MRI) and their association with intelligence quotient (IQ) in ACHD patients.
METHODS: Cross-sectional study in ACHD patients and healthy controls as comparison group. Brain MRI was performed on a 3 T MR scanner, and inspection of structural abnormalities was performed blinded to ACHD or control status. IQ was estimated using the vocabulary and matrix reasoning subtests from the Wechsler Adult Intelligence Scale, Fourth Edition.
RESULTS: A total number of 67 (55% males) ACHD patients and 55 (51% males) controls were included (mean age 26.9 and 26.0 years respectively). Abnormalities on brain MRI were detected in 29 of 46 (63%) ACHD patients and in none of the controls. Abnormalities consisted of focal infarction or atrophy, white matter lesions, microhemorrhages, and global atrophy. Mean estimated IQ was significantly lower in ACHD patients than in controls (98.51 versus 104.38; 95% CI: -10.09 to -1.66; P value = 0.007). Comparison between patients with and without cerebral abnormalities revealed no significant difference in estimated IQ.
CONCLUSION: Our findings indicate a high prevalence and wide spectrum of structural brain abnormalities in ACHD patients. Furthermore, this population is at a higher risk of impaired intellectual functioning than healthy controls. However, the present study could not establish a statistically significant association between MRI findings and estimated IQ.

PMID: 32143921 [PubMed - as supplied by publisher]

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Canadian Cardiovascular Society/Canadian Cardiac Transplant Network Position Statement on Heart Transplantation: Patient Eligibility, Selection, and Post-Transplantation Care.

Can J Cardiol. 2020 Mar;36(3):335-356

Authors: Chih S, McDonald M, Dipchand A, Kim D, Ducharme A, Kaan A, Abbey S, Toma M, Anderson K, Davey R, Mielniczuk L, Campbell P, Zieroth S, Bourgault C, Badiwala M, Clarke B, Belanger E, Carrier M, Conway J, Doucette K, Giannetti N, Isaac D, MacArthur R, Senechal M

Significant practice-changing developments have occurred in the care of heart transplantation candidates and recipients over the past decade. This Canadian Cardiovascular Society/Canadian Cardiac Transplant Network Position Statement provides evidence-based, expert panel recommendations with values and preferences, and practical tips on: (1) patient selection criteria; (2) selected patient populations; and (3) post transplantation surveillance. The recommendations were developed through systematic review of the literature and using the Grading of Recommendations Assessment, Development, and Evaluation (GRADE) system. The evolving areas of importance addressed include transplant recipient age, frailty assessment, pulmonary hypertension evaluation, cannabis use, combined heart and other solid organ transplantation, adult congenital heart disease, cardiac amyloidosis, high sensitization, and post-transplantation management of antibodies to human leukocyte antigen, rejection, cardiac allograft vasculopathy, and long-term noncardiac care. Attention is also given to Canadian-specific management strategies including the prioritization of highly sensitized transplant candidates (status 4S) and heart organ allocation algorithms. The focus topics in this position statement highlight the increased complexity of patients who undergo evaluation for heart transplantation as well as improved patient selection, and advances in post-transplantation management and surveillance that have led to better long-term outcomes for heart transplant recipients.

PMID: 32145863 [PubMed - as supplied by publisher]

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22q11.2 deletion syndrome is associated with increased mortality in adults with tetralogy of Fallot and pulmonary atresia with ventricular septal defect.

Int J Cardiol. 2020 Feb 27;:

Authors: Kauw D, Woudstra OI, van Engelen K, Meijboom FJ, Mulder BJM, Schuuring MJ, Bouma BJ

BACKGROUND: 22q11.2 Deletion syndrome (22q11.2DS) is common in patients with tetralogy of Fallot (TOF) or pulmonary atresia with ventricular septal defect (PA/VSD) and is associated with worse outcomes in children. Whether this impaired prognosis is also translated into adulthood is unknown, as data in adult patients are limited. We aimed to compare long-term outcomes in adults with TOF or PA/VSD both with and without 22q11.2DS.
METHODS: This study prospectively followed a nationwide multicenter cohort of TOF or PA/VSD patients with genetically confirmed presence or absence of 22q11.2DS, from inclusion in the Dutch national CONCOR registry for adults with congenital heart disease (CHD) onward. Outcome measures included all-cause mortality, cardiac mortality, need for pulmonary valve replacement (PVR), ventricular arrhythmias (VA), pacemaker implantation, and ICD implantation.
RESULTS: In total, 479 patients were included (277 (58%) male, median age 28 [IQR; 21-37] years, 62 (13%) with PA/VSD, 34 (7%) with 22q11.2DS). During a median follow-up of 11 [IQR; 6-13] years, 52 (11%) patients died (8 with 22q11.2DS and 44 without 22q11.2DS). Patients with 22q11.2DS had significant decreased survival after 12 years (76% [95% CI; 62-93]) compared to patients without 22q11.2DS (89% [95% CI; 86-92], p = 0.008). 22q11.2DS was associated with increased risk of all-cause mortality and cardiac-mortality, independent of age, sex, and PA/VSD. No association was found between 22q11.2DS and late complications i.e. PVR, VA, pacemaker, or ICD implantation.
CONCLUSIONS: Adults with TOF or PA/VSD with 22q11.2DS have a significantly worse survival than adults without this deletion. In patients with TOF or PA/VSD, genetic analysis for the presence of 22q11.2DS is important for risk stratification and genetic counseling.

PMID: 32145937 [PubMed - as supplied by publisher]

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The role of illness uncertainty in the relationship between disease knowledge and patient-reported outcomes among adolescents and adults with congenital heart disease.

Heart Lung. 2019 Jul - Aug;48(4):325-330

Authors: Schiele SE, Emery CF, Jackson JL

BACKGROUND: Greater general disease knowledge predicts better patient-reported outcomes (PROs) among congenital heart disease (CHD) survivors, but higher illness uncertainty is associated with elevated emotional distress and poorer well-being among patients with chronic disease.
OBJECTIVES: This study explored the relationship of illness uncertainty and disease knowledge with emotional distress and health-related quality of life (HRQoL) among patients with CHD.
METHODS: Individuals with CHD (N = 169, ages 15-39) completed self-report measures of disease knowledge (general and risk-related), illness uncertainty, depressive and anxiety symptoms, and HRQoL. Pearson correlations and regressions analyses were utilized.
RESULTS: Greater risk-related knowledge was associated with greater anxiety (b = .41, p = .03, 95% CI = [.04, .77]) and poorer emotional HRQoL (b = -.53, p = .03, 95% CI = [-1.02, -.05]) when illness uncertainty was higher.
CONCLUSION: When individuals with CHD feel uncertain about their disease course and outcomes, knowledge about future cardiovascular risks may result in higher levels of distress.

PMID: 30471879 [PubMed - indexed for MEDLINE]

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Trends and Outcomes of Infective Endocarditis in Adults With Tetralogy of Fallot: A Review of the National Inpatient Sample Database.

Can J Cardiol. 2019 06;35(6):721-726

Authors: Egbe AC, Vallabhajosyula S, Akintoye E, Connolly HM

BACKGROUND: Lesion complexity and prosthetic valves are known risk factors for infective endocarditis in patients with congenital heart disease. Tetralogy of Fallot (TOF) is the most common complex/cyanotic congenital heart disease and often requires prosthetic valve implantation. Population-based risk of endocarditis in TOF patients is unknown.
METHODS: We reviewed the National Inpatient Sample (NIS) and identified admissions in TOF patients (>18 years), 2000 to 2014. The primary outcome was to describe incidence of endocarditis-related admissions. To assess trends, we divided the study period into tertiles: early (2000 to 2004), mid (2005 to 2009) and late (2010 to 2014) eras. The secondary outcome was to compare in-hospital mortality, complications, and health care resource utilization between admissions with and without endocarditis.
RESULTS: There were 393 (2.1%) endocarditis-related admissions among 18,353 admissions, and the incidence of endocarditis-related admissions increased over time: 1.9% (early era) vs 2.2% (mid-era) vs 2.4% (late era), P < 0.001. Overall in-hospital mortality was 6%. In addition to previously described risk factors for endocarditis, such as previous pacemaker/defibrillator or prosthetic valve implantation, we observed an association between endocarditis-related admissions and male gender, black race, and lower socioeconomic class. In comparison with admissions without endocarditis, the endocarditis-related admissions had higher in-hospital mortality, complications, and health care resource utilization measured by length of stay, inflation-adjusted hospitalization cost, and type of hospital discharge.
CONCLUSIONS: Incidence of endocarditis-related admissions increased over time and was associated with higher mortality, complications, and health care resource utilization. Further studies are required to investigate the observed temporal increase in incidence of endocarditis and explore new strategies to improve outcomes.

PMID: 31151707 [PubMed - indexed for MEDLINE]

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Early and late presentation of coronary artery fistula: a possible natural progression?

Cardiol Young. 2019 Nov;29(11):1407-1409

Authors: Zaban NB, Elshershari H, Hoyer MH

We present two patients, one 10 years old and another 43 years old, who both had successful transcatheter closure of left main coronary artery to right atrium fistulas. The older patient had a larger fistula as well as more symptoms and a complicated post-procedure course. Closure of medium or large coronary artery fistulas should be considered at younger ages to minimise future complications.

PMID: 31502531 [PubMed - indexed for MEDLINE]

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Extremely short setting of optimal sensed atrioventricular interval in patients after Fontan procedure with implanted dual-chamber pacemaker.

Cardiol Young. 2019 Nov;29(11):1375-1379

Authors: Miyazaki A, Yoshimura SI, Matsutani H, Miyake M, Negishi J, Yamanaka K, Yamada O, Doi H, Ohuchi H

BACKGROUND: Atrioventricular interval optimisation is important in patients with dual-chamber pacing, especially with heart failure. In patients with CHD, especially in those with Fontan circulation, the systemic atrial contraction is supposed to be more important than in patients without structural heart disease.
METHODS: We retrospectively evaluated two patients after Fontan procedure with dual-chamber pacemaker with a unique setting of optimal sensed atrioventricular interval.
RESULTS: The optimal sensed atrioventricular interval determined by echocardiogram was extremely short sensed atrioventricular interval at 25 and 30 ms in both cases; however, the actual P wave and ventricular pacing interval showed 180 and 140 ms, respectively. In both cases, the atrial epicardial leads were implanted on the opposite site of the origin of their own atrial rhythm. The time differences between sensed atrioventricular interval and actual P wave and ventricular pacing interval occurred because of the site of the epicardial atrial pacing leads and the intra-atrial conduction delay.
CONCLUSION: We need to consider the origin of the atrial rhythm, the site of the epicardial atrial lead, and the atrial conduction delay by using electrocardiogram and X-ray when we set the optimal sensed atrioventricular interval in complicated CHD.

PMID: 31507257 [PubMed - indexed for MEDLINE]

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Parental reactions, distress, and sense of coherence after prenatal versus postnatal diagnosis of complex congenital heart disease.

Cardiol Young. 2019 Nov;29(11):1328-1334

Authors: Bratt EL, Järvholm S, Ekman-Joelsson BM, Johannsmeyer A, Carlsson SÅ, Mattsson LÅ, Mellander M

INTRODUCTION: A diagnosis of congenital heart disease (CHD) in offspring triggers psychological distress in parents. Results of previous studies have been inconsistent regarding the psychological impact of a prenatal versus a postnatal diagnosis. The aim of this study was to evaluate the influence of the time of diagnosis on levels of parental distress.
METHODS: Pregnant women and their partners with a fetus diagnosed with complex CHD, parents of children with postnatally diagnosed CHD, and pregnant women and their partners with uncomplicated pregnancies were invited to participate. Data were collected during pregnancy and 2-6 months after delivery using the Hospital Anxiety and Depression Scale, sense of coherence, life satisfaction, and Dyadic Adjustment Scale.
RESULTS: During pregnancy, the prenatal group scored lower sense of coherence compared to controls (p=0.044). Postnatally the prenatal group scored lower on sense of coherence compared to the postnatal group and controls (p=0.001; p=0.001). Postnatally, the prenatal and postnatal groups had higher levels of anxiety compared to controls (p=0.025; p=0.0003). Life satisfaction was lower in the prenatal group compared to that in the postnatal group and in controls (p=0.000; p=0.0004).
CONCLUSION: Parents with a prenatal diagnosis of CHD in offspring report a low sense of coherence already during pregnancy which decreased further at follow-up. The same group reported a lower satisfaction with life compared to parents of a child with postnatal diagnosis of CHD and parents of a healthy child. This motivates further efforts to improve counselling and support during pregnancy and for parents after a prenatal diagnosis.

PMID: 31522698 [PubMed - indexed for MEDLINE]