Christopher R. Broda, Katherine B. Salciccioli, Keila N. Lopez, Peter R. Ermis, Douglas S. Moodie, Heather A. Dickerson

Congenital Heart Disease, Vol.14, No.6, pp. 885-894, 2019, DOI:10.1111/chd.12856

Abstract Background: Heterotaxy syndrome (HS) is a condition in which the thoracoabdominal organs demonstrate an abnormal lateral arrangement and is often associated with con‐ genital heart disease (CHD). Little is known about the adult HS population with CHD.
Objective: To describe the outcomes and sociodemographics of the adult CHD popu‐ lation with HS.
Methods: Records of patients 18 years of age or older with diagnoses of both CHD and HS at Texas Children's Hospital from 1964 to 2018 were reviewed.
Results: Sixty‐two patients metinclusion criteria.Median agewas 22.7 [IQR19.6‐30.0] years; 26 (42%) were female; and 13 (21%) of patients had a gap… More >

Alessandro Sgrò¹, Thomas M. Drake², Pedro Lopez‐Ayala³, Kevin Phan⁴

Congenital Heart Disease, Vol.14, No.6, pp. 1102-1112, 2019, DOI:10.1111/chd.12855

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 implant‐ able 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… More >

Brooke T. Davey¹, Olga H. Toro‐Salazar¹, Naomi Gauthier^2,3, Anne Marie Valente², Robert W. Elder⁴, Fred M. Wu², Norman Berman³, Phyllis Pollack⁵, Ji Hyun Lee¹, Rahul H. Rathod²

Congenital Heart Disease, Vol.14, No.6, pp. 1013-1023, 2019, DOI:10.1111/chd.12854

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 pa‐ tients 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… More >

Masashi Nishida, Shingo Kubo, Yuma Morishita, Kosuke Nishikawa, Kazuyuki Ikeda, Toshiyuki Itoi, Hajime Hosoi

Congenital Heart Disease, Vol.14, No.6, pp. 1087-1093, 2019, DOI:10.1111/chd.12853

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… More >

Natasa Karadzov Orlic¹, Amira Egic¹, Barbara Damnjanovic‐Pazin MD2 | Relja Lukic¹, Ivana Joksic³, Zeljko Mikovic¹

Congenital Heart Disease, Vol.14, No.6, pp. 1094-1101, 2019, DOI:10.1111/chd.12852

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 sin‐ gle of these parameters was abnormal, simple cardiac scan… More >

Ari Cedars¹, Luke Burchill², S. Lucy Roche³, Jonathan Menachem⁴, Kelly Axsom⁵, Kristen Tecson⁶

Congenital Heart Disease, Vol.14, No.6, pp. 958-962, 2019, DOI:10.1111/chd.12851

Abstract Background: There are no published data on post‐transplant outcomes in durable ven‐ tricular assist device (VAD)‐supported adult congenital heart disease (ACHD) patients.
Methods: We compared post‐transplant outcomes in VAD‐supported vs non‐VAD‐ supported ACHD patients using the Scientific Registry of Transplant Recipients.
Results: At 1 year, there was no difference in post‐transplant mortality between VAD‐supported (12 patients) and non‐VAD‐supported (671 patients) ACHD patients.
Conclusions: In appropriate ACHD patients, VAD use as a bridge to transplant is a reasonable strategy. More >

Tim Takken¹, Alyanne Evertse¹, Fleur de Waard¹, Mandy Spoorenburg¹, Martijn Kuijpers¹, Christian Schroer^2,3, Erik H. Hulzebos¹

Congenital Heart Disease, Vol.14, No.6, pp. 1005-1012, 2019, DOI:10.1111/chd.12850

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… More >

Christine M. Riley¹, Christopher W. Mastropietro², Peter Sassalos³, Jason R. Buckley⁴, John M. Costello⁴, Ilias Iliopoulos⁵, Aimee Jennings⁶, Katherine Cashen⁷, Sukumar Suguna Narasimhulu⁸, Keshava M. N. Gowda⁹, Arthur J. Smerling¹⁰, Michael Wilhelm¹¹, Aditya Badheka¹², Adnan Bakar^13,14, Elizabeth A. S. Moser¹⁵, Venu Amula¹⁶

Congenital Heart Disease, Vol.14, No.6, pp. 1078-1086, 2019, DOI:10.1111/chd.12849

Abstract Background: Elevated pulmonary vascular resistance (PVR) is common following repair of truncus arteriosus. Inhaled nitric oxide (iNO) is an effective yet costly therapy that is frequently implemented postoperatively to manage elevated PVR.
Objectives: We aimed to describe practice patterns of iNO use in a multicenter cohort of patients who underwent repair of truncus arteriosus, a lesion in which recovery is often complicated by elevated PVR. We also sought to identify patient and center factors that were more commonly associated with the use of iNO in the postoperative period.
Design: Retrospective cohort study.
Setting: 15 tertiary care pediatric referral centers.
Patients:… More >

Simone Goa Diab¹, Kristin Godang², Lil‐Sofie Ording Müller³, Runar Almaas⁴, Charlotte de Lange³, Leif Brunvand¹, Kari Margrethe Hansen¹, Anne Grethe Myhre⁵, Gaute Døhlen¹, Erik Thaulow^1,6, Jens Bollerslev^2,6, Thomas Möller¹

Congenital Heart Disease, Vol.14, No.6, pp. 996-1004, 2019, DOI:10.1111/chd.12848

Abstract Objective: We investigated bone mineral density (BMD) at different ages after the Fontan completion, and we evaluated the relationship between BMD, vitamin D levels, and pertinent patient variables.
Methods: A cross‐sectional sample of 64 patients was examined with dual‐energy X‐ray absorptiometry (DXA) scans to determine BMD. Of these patients, 24 were also examined with BoneXpert software to determine bone mass density (BMX), expressed as the bone health index (BHI). Blood samples from all patients were analyzed. Patients were divided into three different age groups; A: 4‐9 years old (n = 22), B: 10‐15 years old (n = 21), and C:… More >

Jim T. Vehmeijer¹, Zeliha Koyak¹, A. Suzanne Vink¹, Werner Budts^2,3, Louise Harris⁴, Candice K. Silversides⁴, Erwin N. Oechslin⁴, Aeilko H. Zwinderman⁵, Barbara J.M. Mulder^1,6, Joris R. de Groot¹

Congenital Heart Disease, Vol.14, No.6, pp. 952-957, 2019, DOI:10.1111/chd.12847

Abstract Objective: Adult congenital heart disease (ACHD) patients are at risk of sudden cardiac death (SCD). However, methods for risk stratification are not yet well‐ defined. The T_peak‐T_end (TpTe) interval, a measure of dispersion of ventricular repolari‐ zation, is a risk factor for SCD in non‐ACHD patients. We aim to evaluate whether TpTe can be used in risk stratification for SCD in ACHD patients.
Design: From an international multicenter cohort of 25 790 ACHD patients, we iden‐ tified all SCD cases. Cases were matched to controls by age, gender, congenital de‐ fect, and (surgical) intervention.
Outcome Measures: TpTe was measured on… More >