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Screening performance of congenital heart defects in first trimester using simple cardiac scan, nuchal translucency, abnormal ductus venosus blood flow and tricuspid regurgitation.

Congenit Heart Dis. 2019 Nov;14(6):1094-1101

Authors: Karadzov Orlic N, Egic A, Damnjanovic-Pazin B, Lukic R, Joksic I, Mikovic Z

Abstract
OBJECTIVE: The objective of this study was to analyze if the addition of simple cardiac scan in cases with increased nuchal translucency (NT) and/or abnormal ductus venosus (DV) blood flow, and/or tricuspid regurgitation (TCR) can improve detection of congenital heart defects (CHD) in chromosomally normal fetuses without non-cardiac defects at 11-13 + 6 gestational weeks in a population of singleton pregnancies.
METHODS: During the 10 years period, all singleton pregnancies at 11-13 + 6 weeks were routinely scanned for NT, DV blood flow and TCR assessment and, if a single of these parameters was abnormal, simple cardiac scan with 2D gray scale and color and/or directional power Doppler in 4-chamber (4-CV) and 3 vessel and trachea views (3VTV) was performed.
RESULTS: The sensitivity and specificity of NT ≥ 95th + DV R/A a-wave + TCR in detecting CHD were 77% and 97%, respectively, and of simple cardiac scan, 67% and 98%, respectively. Area under the curve of receiver operating characteristic curve of NT ≥ 95th + DV R/A a-wave + TCR was 0.838, and of NT ≥ 95th + DV R/A a-wave + TCR + simple cardiac scan was 0.915.
CONCLUSIONS: In chromosomally normal fetuses without non-cardiac anomalies, addition of simple cardiac scan to the combined first trimester screening parameters improves detection of major CHD during first trimester.

PMID: 31573148 [PubMed - indexed for MEDLINE]

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Answer letter to Professor Robert Anderson by Virginija Rudiene.

Heart. 2020 02;106(3):242

Authors: Rudiene V

PMID: 31601726 [PubMed - indexed for MEDLINE]

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'Cor triatriatum', or divided left atrium.

Heart. 2020 02;106(3):242

Authors: Anderson RH

PMID: 31601727 [PubMed - indexed for MEDLINE]

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Exercise responses in children and adults with a Fontan circulation at simulated altitude.

Congenit Heart Dis. 2019 Nov;14(6):1005-1012

Authors: Takken T, Evertse A, de Waard F, Spoorenburg M, Kuijpers M, Schroer C, Hulzebos EH

Abstract
BACKGROUND: Traveling to high altitude has become more popular. High-altitude exposure causes hypobaric hypoxia. Exposure to acute high altitude, during air travel or mountain stays, seems to be safe for most patients with congenital heart disorders (CHD). Still, current guidelines for CHD patients express concerns regarding safety of altitude exposure for patients with a Fontan circulation. Therefore, investigating hemodynamic and pulmonary responses of acute high-altitude exposure (±2500 m) at rest and during maximal exercise in patients with Fontan circulation can provide clarity in this dispute and may contribute to improvement of clinical counseling.
METHODS: Twenty-one Fontan patients with 21 age-matched healthy controls, aged 8-40 years, were enrolled in an observational study. Participants performed two cardiopulmonary exercise tests on a cycle ergometer with breath-by-breath respiratory gas analyses combined with noninvasive impedance cardiac output measurements: one at sea level (±6 m) and one at simulated high altitude (±2500 m), respectively.
RESULTS: The effect of altitude exposure was different in rest for saturation (-2.3% vs -4.1%) between Fontan patients and healthy controls (P < .05). At peak exercise the effects of high altitude exposure was different on VO2 (-5.1% vs 9.6%) and AvO2 -diff (-0.3% vs -12.8%) between Fontan patients and healthy controls.
CONCLUSION: Although, acute high-altitude exposure has a detrimental effect on exercise capacity, the impact on pulmonary and hemodynamic responses of high-altitude exposure is comparable between Fontan patients and healthy controls.

PMID: 31602790 [PubMed - indexed for MEDLINE]

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Kidney injury biomarkers after cardiac angiography in children with congenital heart disease.

Congenit Heart Dis. 2019 Nov;14(6):1087-1093

Authors: Nishida M, Kubo S, Morishita Y, Nishikawa K, Ikeda K, Itoi T, Hosoi H

Abstract
OBJECTIVE: This study aims to investigate the changes in renal function and levels of urinary biomarkers before and after cardiac angiography in children with congenital heart disease (CHD).
SETTING: Children with CHD are at a risk for kidney injury during contrast exposure in cardiac angiography.
OUTCOME MEASURES: We measured urinary protein, albumin, N-acetyl-β-D-glucosaminidase (NAG), β2-microglobulin (BMG), and liver-type fatty acid-binding protein (L-FABP) levels, as well as serum creatinine and cystatin C levels, before and after cardiac angiography in 33 children with CHD.
RESULTS: No significant decrease was noted in either the creatinine-based or cystatin C-based estimated glomerular filtration rate at 24 hours after angiography compared with that before angiography. Urinary protein, NAG, BMG, and L-FABP levels were significantly increased at 24 hours after angiography, all of which returned to baseline levels at more than 7 days after angiography. An increase in urinary level of protein, albumin, NAG, or BMG was mostly associated with increased urinary L-FABP level. An increase in both urinary BMG and L-FABP, but not that in urinary L-FABP alone, was associated with increased levels of urinary protein and NAG, as well as the greater dose of contrast media.
CONCLUSIONS: Transient increases of kidney injury biomarkers following cardiac angiography are not necessarily associated with the impairment of renal function in a short time period; however, the increase in urinary protein, albumin, NAG, or BMG level may indicate greater stresses to the kidneys than the increase in urinary L-FABP alone in children with CHD.

PMID: 31605509 [PubMed - indexed for MEDLINE]

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Left cardiac sympathetic denervation in the management of long QT syndrome and catecholaminergic polymorphic ventricular tachycardia: A meta-regression.

Congenit Heart Dis. 2019 Nov;14(6):1102-1112

Authors: Sgrò A, Drake TM, Lopez-Ayala P, Phan K

Abstract
BACKGROUND: Left cardiac sympathetic denervation (LCSD) has been proposed as useful therapy for long QT syndrome (LQTS) and catecholaminergic polymorphic ventricular tachycardia (CPVT), in addition to anti-arrhythmic agents and implantable cardioverter defibrillators. This study aimed to assess the current evidence for LCSD and compare the open vs the video-assisted thoracoscopic surgery (VATS) approaches.
METHODS: MEDLINE, Embase and Cochrane library databases were searched up to December 2018 for studies reporting the long-term outcomes of LCSD in LQTS, CPVT patients. The incidence of cardiac events (CEs) before and after surgery, the change in QTc interval, and surgical complications were pooled to estimate the efficacy of LCSD. Meta-regression was used to estimate the effects of surgical approach (open vs VATS) on outcomes following LCSD.
RESULTS: Twenty-seven papers met our inclusion criteria (647 patients). VATS was used in 408 patients (63.1%), open surgery in 239 (36.9%). Mean follow-up was 32.3 ± 32.5 months. Postsurgery, 398/585 patients (68.0%) were free of CEs and QTc decreased from 522 ± 61.6 ms to 494 ± 52.3 ms. Meta-regression showed no differences between the two approaches in the incidence of CEs and surgical complications. VATS was associated with a smaller reduction in QTc (β-coefficient -20.04, 95% CI -36.82 to -3.27, P = .019).
CONCLUSIONS: LCSD was associated with a reduction in the incidence of CEs in LQTS, CPVT patients and in the duration of QTc. Open surgery was associated with a greater reduction in QTc. Due to the limitations that hindered our study, a randomized trial is warranted to fully establish LCSD safety and efficacy.

PMID: 31621201 [PubMed - indexed for MEDLINE]

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Surveillance and screening practices of New England congenital cardiologists for patients after the Fontan operation.

Congenit Heart Dis. 2019 Nov;14(6):1013-1023

Authors: Davey BT, Toro-Salazar OH, Gauthier N, Valente AM, Elder RW, Wu FM, Berman N, Pollack P, Lee JH, Rathod RH

Abstract
INTRODUCTION: Surveillance and management guidelines for Fontan patients are lacking due to the paucity of evidence in the literature of screening efficacy on outcome measures.
METHODS: The Fontan Working Group within the New England Congenital Cardiology Association designed an electronic survey to assess surveillance practices for patients with Fontan procedures among New England congenital cardiologists and to explore variability in screening low-risk vs high-risk Fontan patients across regional programs.
RESULTS: Fifty-six cardiologists representing 12 regional programs responded to the survey, comprising ~40% of the total New England congenital cardiac physicians. The majority of desired testing and consultation was available within 50 miles of the patient's home institution with some limitations of cardiac catheterization and cardiac magnetic resonance imaging availability. Surveillance and screening were less frequent in low-risk Fontan patients compared to high-risk Fontan patients. Counseling practices were similar for both low-risk and high-risk Fontan patients. Aspirin monotherapy was recommended by 82% of providers for low-risk Fontan patients, while anticoagulation regimens were more varied for the high-risk population. Practitioners with ≤15 years of experience were more likely to provide quality of life testing in both low-risk and high-risk Fontan patients. There were no other major differences in testing frequencies by years of practice, quaternary vs nonquaternary care facility, or the number of Fontan patients in a practice.
CONCLUSION: This survey provides insight into regional practices of screening and surveillance of Fontan patients. These data may be used to design future research studies and evidence-based guidelines to streamline the approach to manage these complex patients.

PMID: 31642600 [PubMed - indexed for MEDLINE]

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

Congenit Heart Dis. 2019 Nov;14(6):1123-1129

Authors: Evertz R, Houck CA, Ten Cate T, Duijnhouwer AL, Beukema R, Westra S, Vernooy K, de Groot NMS

Abstract
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.

PMID: 31692272 [PubMed - indexed for MEDLINE]

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Systemic Sirolimus to Prevent In-Stent Stenosis in Pediatric Pulmonary Vein Stenosis.

Pediatr Cardiol. 2020 Feb;41(2):282-289

Authors: Callahan R, Esch JJ, Wang G, Ireland CM, Gauvreau K, Jenkins KJ

Abstract
Evaluate the efficacy of systemic sirolimus (rapamycin) in preventing in-stent stenosis (ISS) in pediatric intraluminal pulmonary vein stenosis (PVS). Report the adverse events related to sirolimus therapy. There is a high incidence of ISS following stent implantation in PVS. The use of sirolimus in preventing ISS has not been reported. Retrospective review of all patients who received sirolimus (8 week course) for treatment of ISS for PVS between January 2013 and June 2018. Forty stents (37 bare metal, 3 drug-eluting) in 20 patients were treated with sirolimus; 20 at the time of implantation (primary prevention [1P]) and 20 following documented ISS requiring transcatheter reintervention (secondary prevention [2P]). Treated patients were young (median 2 y/o [0.7-5.7]) and most had PVS associated with congenital heart disease (75%, 15/20; 4/15 with TAPVC). In the 1P group, 85% (17/20) of stents were without significant (< 50%) ISS at median of 102 days (range 56-527); the growth rate of ISS in this group was 7.5 ± 7.1%/month. In the 2P group, most stents had a slower growth rate of ISS after sirolimus therapy compared to pre-treatment (median 3.7 [- 0.2 to 13.1] vs. 10.4 [1.3 to 19.5] %/month; p < 0.001). One patient developed pneumonia on drug while concurrently taking another immunosuppressive agent. No other serious adverse events were related to sirolimus therapy. Systemic sirolimus slows the growth rate of ISS following stent implantation in PVS compared to pre-treatment rates and was administered safely in a small number of pediatric patients with complex heart disease.

PMID: 31720783 [PubMed - indexed for MEDLINE]

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Baseline tubular biomarkers in young adults with congenital heart disease as compared to healthy young adults: Detecting subclinical kidney injury.

Congenit Heart Dis. 2019 Nov;14(6):963-967

Authors: Fuhrman DY, Nguyen L, Hindes M, Kellum JA

Abstract
BACKGROUND: There are significant implications for kidney disease in young adults with congenital heart disease. Prior investigations have not focused on the use of urinary tubular biomarkers for the early identification of kidney disease in this growing patient group.
OBJECTIVE: Determine if young adults with congenital heart disease have differences in the baseline concentration of urinary tubular biomarkers when compared to healthy young adults.
DESIGN/METHODS: In a pilot case control study, 30 patients from 18 to 35 years of age with congenital heart disease and a normal serum creatinine were recruited during a routine follow-up visit. In the same age group, 30 control subjects without history of heart or kidney disease were recruited. Urine samples were obtained to measure beta 2-microglobin, alpha 1-microglobin, N-acetyl-B-D-glucosaminidase, liver fatty acid binding protein, kidney injury molecule-1, insulin-like growth factor binding protein 7, and tissue inhibitor of metalloproteinases-2. Comparisons were done using Wilcoxon rank-sum or Fisher's exact test.
RESULTS: No study participants had proteinuria on urine dipstick. Median concentrations of kidney injury molecule-1 were higher (P = .01) and concentrations of insulin-like growth factor binding protein 7 (P = .001) and tissue inhibitor of metalloproteinases-2 (P = .009) were lower in the subjects with congenital heart disease when compared to the control subjects. There were no significant differences between the groups with respect to the other biomarkers.
CONCLUSION: Our data suggest that young adults with congenital heart disease may have subclinical kidney dysfunction. Lower levels of insulin-like growth factor binding protein 7 and tissue inhibitor of metalloproteinases-2 may indicate an impaired ability to respond to injury, while higher levels of kidney injury molecule-1 may reflect early tubular injury.

PMID: 31793232 [PubMed - indexed for MEDLINE]

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