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4-D flow MRI aortic 3-D hemodynamics and wall shear stress remain stable over short-term follow-up in pediatric and young adult patients with bicuspid aortic valve.

Pediatr Radiol. 2018 Sep 10;:

Authors: Rose MJ, Rigsby CK, Berhane H, Bollache E, Jarvis K, Barker AJ, Schnell S, Allen BD, Robinson JD, Markl M

Abstract
BACKGROUND: Children with bicuspid aortic valve (BAV) are at risk for serious complications including aortic valve stenosis and aortic rupture. Most studies investigating biomarkers predictive of BAV complications are focused on adults.
OBJECTIVE: To investigate whether hemodynamic parameters change over time in children and young adults with BAV by comparing baseline and follow-up four-dimensional (4-D) flow MRI examinations.
MATERIALS AND METHODS: We retrospectively included 19 children and young adults with BAV who had serial 4-D flow MRI exams (mean difference in scan dates 1.8±1.0 [range, 0.6-3.4 years]). We compared aortic peak blood flow velocity, three-dimensional (3-D) wall shear stress, aortic root and ascending aortic (AAo) z-scores between baseline and follow-up exams. We generated systolic streamlines for all patients and visually compared their baseline and follow-up exams.
RESULTS: The only significant difference between baseline and follow-up exams occurred in AAo z-scores (3.12±2.62 vs. 3.59±2.76, P<0.05) indicating growth of the AAo out of proportion to somatic growth. There were no significant changes in either peak velocity or 3-D wall shear stress between baseline and follow-up exams. Ascending aortic peak velocity at baseline correlated with annual change in AAo z-score (r=0.58, P=0.009). Visual assessment revealed abnormal blood flow patterns, which were unique to each patient and remained stable between baseline and follow-up exams.
CONCLUSION: In our pediatric and young adult BAV cohort, hemodynamic markers and systolic blood flow patterns remained stable over short-term follow-up despite significant AAo growth, suggesting minimal acute disease progression. Baseline AAo peak velocity was a predictor of AAo dilation and might help in determining pediatric patients with BAV who are at risk of increased AAo growth.

PMID: 30203126 [PubMed - as supplied by publisher]

When Your 35-Year-Old Patient has a Sternotomy Scar: Anesthesia for Adult Patients with Congenital Heart Disease Presenting for Noncardiac Surgery.

Int Anesthesiol Clin. 2018;56(4):3-20

Authors: Cheema A, Ibekwe S, Nyhan D, Steppan J

PMID: 30204603 [PubMed - in process]

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Contemporary Outcomes of Combined Heart-Liver Transplant in Patients With Congenital Heart Disease.

Transplantation. 2018 02;102(2):e67-e73

Authors: Bryant R, Rizwan R, Zafar F, Shah SA, Chin C, Tweddell JS, Morales DL

Abstract
BACKGROUND: As more patients survive into adulthood with repaired congenital heart disease (CHD), transplant centers now have patients presenting with both end-stage cardiac and hepatic failure. An understanding of the contemporary outcomes with combined heart liver transplantation (CHLT) in patients with CHD is needed.
METHODS: A retrospective review of the outcomes of CHLT in CHD was conducted from October 1, 1987, to June 30, 2015, from the United Network of Organ Sharing (UNOS) database. Propensity score matched cohorts were formed for the assessment of posttransplant outcome: CHLT with CHD, CHLT without CHD, and isolated heart transplant for CHD (HT-CHD). Cohorts were matched based on age, body mass index, inotrope use, and ventilator support at the time of transplant. We assessed 30-day, 1-, 5-, and 10-year posttransplant survivals.
RESULTS: There were 61 437 heart transplants during the study period, of which 190 (0.3%) were CHLT. Among CHLT, 41(22%) patients had CHD. In 26 (63%) of these, the indication for CHLT was hepatic congestion/cirrhosis of cardiac origin. In the matched cohorts, the overall survival for CHLT with CHD at 30 days, 1, 5, and 10 years was 95%, 86%, 83%, and 83%, respectively; for CHLT without CHD, it was 100%, 92%, 92%, and 63%, respectively (vs CHLT with CHD: P = 0.49); and for HT-CHD, it was 90%, 84%, 63%, and 39% (vs CHLT with CHD: P = 0.03), respectively.
CONCLUSIONS: The posttransplant outcome of CHLT, with and without CHD, is comparable. However, there is a trend toward better survival for CHLT for CHD compared with isolated heart transplant for CHD.

PMID: 29077655 [PubMed - indexed for MEDLINE]

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Parental Uncertainty About Transferring Their Adolescent with Congenital Heart Disease to Adult Care.

J Adv Nurs. 2018 Sep 12;:

Authors: Burström Å, Acuna Mora M, Öjmyr-Joelsson M, Sparud-Lundin C, Rydberg A, Hanseus K, Frenckner B, Nisell M, Moons P, Bratt EL

Abstract
AIMS: To study parent's levels of uncertainty related to the transfer from pediatric to adult care in adolescents with Congenital Heart Disease (CHD) and to identify potentially correlating factors.
BACKGROUND: Parents acknowledge that during transition they struggle with finding ways of feeling secure in handing over the responsibility and letting go of control. Well-prepared and informed parents who feel secure are most likely better skilled to support their adolescent and to hand over the responsibility.
DESIGN: A cross-sectional study.
METHODS: Overall, 351 parents were included (35% response rate). Parental uncertainty was assessed using a Linear Analogue Scale (0-100). Data was collected between January - August 2016. Potential correlates were assessed using the readiness for transition questionnaire and sociodemographic data.
RESULTS: The mean parental uncertainty score was 42.5. Twenty-four percent of the parents had a very low level of uncertainty (score 0-10) and 7% had a very high level (score 91-100). Overall, 26% of the mothers and 36% of the fathers indicated that they had not started thinking of the transfer yet. The level of uncertainty was negatively associated with the level of perceived overall readiness. Adolescents' age, sex, CHD complexity and parental age were not related to uncertainty.
CONCLUSION: A wide range in the levels of uncertainty was found. Parents who were less involved in the care, or perceived their adolescent as readier for the transition, felt less uncertain. Still, thirty percent of the parents had not started to think about the transfer to adult care. This article is protected by copyright. All rights reserved.

PMID: 30209810 [PubMed - as supplied by publisher]

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Differences in Obesity Among Black and White Children, Adolescents, and Adults With Congenital Heart Disease.

J Cardiovasc Nurs. 2018 Sep 11;:

Authors: Jackson JL, Harrison T, Keim SA

Abstract
BACKGROUND: Congenital heart disease (CHD) survivors have an elevated risk for obesity-related comorbidities, but little is known about racial differences in obesity rates for this population.
OBJECTIVE: The authors aimed to compare rates of obesity in CHD survivors to national estimates using National Health and Nutrition Examination Assessment Survey (NHANES) and to characterize racial disparities in obesity among CHD survivors across age ranges.
METHODS: Retrospective chart review included 4496 CHD survivors (4050 white and 446 black) with a range of lesion severities from a pediatric and an adult medical center.
RESULTS: White children with CHD had a higher prevalence of obesity compared with NHANES estimates. In contrast, white young adults with CHD had a lower prevalence of obesity compared with NHANES. Blacks with CHD had a 58% increased risk of obesity in young adulthood and a 33% increased risk in late adulthood compared with whites with CHD.
CONCLUSIONS: Obesity interventions are needed among CHD survivors across the lifespan, particularly among adult non-Hispanic blacks.

PMID: 30211814 [PubMed - as supplied by publisher]

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Blood troponin levels in acute cardiac events depends on space weather activity components (a correlative study).

J Basic Clin Physiol Pharmacol. 2018 Jun 27;29(3):257-263

Authors: Stoupel E, Radishauskas R, Bernotiene G, Tamoshiunas A, Virvichiute D

Abstract
BACKGROUND: Many biological processes are influenced by space weather activity components such as solar activity (SA), geomagnetic activity (GMA) and cosmic ray activity (CRA). Examples are total mortality, acute myocardial infarction (AMI), stroke (cerebrovascular accident), sudden cardiac death, some congenital maladies (congenital heart disease and Down syndrome), many events in neonatology, ophtalmology, blood pressure regulation, blood coagulation, inflammation, etc. The aim of this study was to check if the level of blood troponins (Tns) - markers of myocardial damage and recognized components of modern description of AMI - is connected with the mentioned space weather parameters.
METHODS: Patients admitted to a 3000-bed tertiary university hospital in Kaunas, Lithuania, with suspected AMI were the object of the study. Data for the time between 2008 and 2013 - 72 consecutive months - were studied. Of the patients, 1896 (1398 male, 498 female) had elevated troponin I (Tn I) or troponin T (Tn T, sensitive Tn) levels. Normal values were 0.00-0.03 ng/mL for Tn I and 0.00-14.00 ng/mL for Tn T. Monthly means and standard deviation of Tn I and Tn T were compared with monthly markers of SA, GMA and CRA. Pearson correlation coefficients and their probabilities were established (in addition to the consecutive graphs of both comparing physical and biological data). The cosmophysical data came from space service institutions in the United States, Russia and Finland.
RESULTS: AMI was diagnosed in 1188 patients (62.66%), and intermediate coronary syndrome in 698 patients (36.81%). There were significant links of the Tn blood levels with four SA indices and CRA (neutron activity in imp/min); there was no significant correlation with GMA indices Ap and Cp (p=0.27 and p=0.235). Tn T levels significantly correlated with the GMA indices and not with the SA and CRA levels (Ap: r=0.77, p=0.0021; Cp: r=0.729, p=0.0047).
CONCLUSIONS: First, the monthly level of blood Tn I in ACS is significantly correlated with the indices of SA (inverse) and with CRA (neutron); second, no significant correlation with the GMA indices was found; and third, the Tn T levels showed significant links with the GMA indices and none with SA and CRA (neutron).

PMID: 29397386 [PubMed - indexed for MEDLINE]

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Implementing and Assessing a Standardized Protocol for Weaning Children Successfully From Extracorporeal Life Support.

Artif Organs. 2018 Apr;42(4):394-400

Authors: Kubicki R, Höhn R, Grohmann J, Fleck T, Reineker K, Kroll J, Siepe M, Benk C, Klemm R, Humburger F, Stiller B

Abstract
Extracorporeal life support (ECLS) weaning is a complex interdisciplinary process with no clear guidelines. To assess ventricular and pulmonary function as well as hemodynamics including end-organ recovery during ECLS weaning, we developed a standardized weaning protocol. We reviewed our experience 2 years later to assess its feasibility and efficacy. In 2015 we established an inter-professional, standardized, stepwise protocol for weaning from ECLS. If the patient did not require further surgery, weaning was conducted bedside in the intensive care unit (ICU). Most of the weaning procedures are guided via echocardiography. Data acquisition began at baseline level, followed by four-step course (each step lasting 10 min), entailing flow-reduction and ending 30 min after decannulation. Moreover, data from the preprotocol era are presented. Between May 2015 and 2017, 26 consecutive patients (18 male), median age 177 days (2 days-20 years) required ECLS with median support of 4 (2-11) days. Excluding eight not weanable patients, 21 standardized weaning procedures were protocolled in the remaining 18 children. Our generally successful protocol-guided weaning rate (with at least 24-h survival) was 89%, with a discharge home rate of 58%. Practical application of the novel standard protocol seems to facilitate ECLS weaning and to improve its success rate. The protocol can be administered as part of standard bedside ICU assessment.

PMID: 29423912 [PubMed - indexed for MEDLINE]

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Efficiency of the home cardiac rehabilitation program for adults with complex congenital heart disease.

Congenit Heart Dis. 2018 Sep 14;:

Authors: Bhasipol A, Sanjaroensuttikul N, Pornsuriyasak P, Yamwong S, Tangcharoen T

Abstract
OBJECTIVE: We aimed to study the efficiency and safety of once-a-week outpatient rehabilitation followed by home program with tele-monitoring in patients with complex cyanotic congenital heart disease.
DESIGN: Prospective nonrandomized study.
METHOD: Patients who have been diagnosed either Eisenmenger's syndrome or inoperable complex cyanotic heart disease and able to attend 12-week cardiac rehabilitation program were included. Training with treadmill walking and bicycling under supervision at cardiac rehabilitation unit once-a-week in the first 6 weeks followed by home-based exercise program (bicycle and walking) with a target at 40%-70% of maximum heart rate (HRmax) at pretraining peak exercise for another 6 weeks was performed in the intervention group. Video and telephone calls were scheduled for evaluation of compliance and complication. Data from cardiopulmonary exercise testing (CPET) on cycle ergometry including peak oxygen consumption (peakVO2 ), oxygen pulse (O2 pulse), ventilatory equivalent for carbon dioxide (VE/CO2 at anaerobic threshold), constant work-rate endurance time (CWRET) at 75% of peak VO2 , and 6-minute walk distance (6MWD) were compared between baseline and after training by paired t test.
RESULT: Of the 400 patients in our adult congenital heart disease clinic, 60 patients met the inclusion criteria. Eleven patients who could follow program regularly were assigned home program. There was a statistically significant improvement of CWRET, O₂ pulse, and 6MWD after finishing the program (P = .003, .039, and .001, respectively). The mean difference of 6MWD change in the home-program group was significantly higher than in the control group (69.3 ± 47.9 meters vs. 4.1 ± 43.4 meters, P = .003). No serious adverse outcomes were reported during home training.
CONCLUSION: Once-a-week outpatient hospital-based exercise program followed by supervised home-based exercise program showed a significant benefit in improvement of exercise capacity in adults with complex cyanotic congenital heart disease without serious adverse outcomes.

PMID: 30216680 [PubMed - as supplied by publisher]

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Relationship of Red Cell Distribution Width to Adverse Outcomes in Adults With Congenital Heart Disease (from the Boston Adult Congenital Heart Biobank).

Am J Cardiol. 2018 Aug 03;:

Authors: Alshawabkeh L, Rajpal S, Landzberg MJ, Emani S, Ephrem G, Gray C, Singh MN, Wu F, Opotowsky AR

Abstract
Red cell distribution width (RDW), a measure of variability in red cell size, predicts adverse outcomes in acquired causes of heart failure. We examined the relation of RDW and outcomes in adults with congenital heart disease. We performed a prospective cohort study on 696 ambulatory patients ≥18 years old enrolled in the Boston Adult Congenital Heart Disease Biobank between 2012 and 2016 (mean age 38.7 ± 13.5 years; 49.9% women). The combined outcome was all-cause mortality or nonelective cardiovascular hospitalization. Most patients had moderately or severely complex congenital heart disease (42.5% and 38.5%, respectively). Mean RDW was 14.0 ± 1.3%. RDW >15% was present in 81 patients (11.6%). After median 767 days of follow-up, 115 patients sustained the primary combined outcome, including 31 who died. Higher RDW predicted both the combined outcome (hazard ratio [HR] for RDW >15% = 4.5, 95% confidence interval [CI] 3.0 to 6.6; HR per + 1SD RDW = 1.8, 95% CI 1.6 to 2.0, both p <0.0001) and death alone (HR for RDW >15% = 7.1, 95% CI 3.5 to 14.4; HR per + 1SD RDW = 1.8, 95% CI 1.6 to 2.0, both p <0.0001). RDW remained an independent predictor of the combined outcome after adjusting for age, cyanosis, congenital heart disease complexity, ventricular systolic function, New York Heart Association functional class, hemoglobin concentration, mean corpuscular volume, high-sensitivity C-reactive protein and estimated glomerular filtration rate (HR per + 1SD RDW = 1.5, 95% CI 1.2 to 1.9, p <0.0001). RDW also remained an independent predictor of mortality alone after adjustment for age plus each variable individually. In conclusion, elevated RDW is an independent predictor of all-cause mortality or nonelective cardiovascular hospitalization in adults with congenital heart disease. This simple clinical biomarker identifies increased risk for adverse events even among patients with preserved functional status.

PMID: 30217370 [PubMed - as supplied by publisher]

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Seminars in Thoracic and Cardiovascular Surgery-Current Readings: Adult Congenital Heart Surgery.

Semin Thorac Cardiovasc Surg. 2018 Sep 11;:

Authors: Fuller S

Abstract
While the incidence of congenital heart disease has remained relatively stable, survival has increased dramatically. Many of these patients require lifelong specialized cardiac care to address sequelae and residual disease. In order to best meet the increasing demands and needs of this heterogenous group of survivors, multidisciplinary specialty training, lesion specific outcomes research and both personnel and financial resources must be dedicated to their care.

PMID: 30217528 [PubMed - as supplied by publisher]

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