Constanze Pfitzer^1,2,3, Paul C. Helm⁴, Hannah Ferentzi^1,5, Lisa-Maria Rosenthal¹, Ulrike M. M. Bauer^4,6, Felix Berger^1,3,7, Katharina R. L. Schmitt^1,3

Congenital Heart Disease, Vol.12, No.6, pp. 787-793, 2017, DOI:10.1111/chd.12515

Abstract Objective: To assess the prevalence of congenital heart disease (CHD) in Germany in relation to phenotypes, severity and gender.
Design: Cross-sectional registry study.
Setting: We analyzed data from patients with CHD born between 1996 and 2015.
Patients: A total of 26 630 patients, registered with the NRCHD, were born between 1996 and 2015. 10 927 patients were excluded from the current analysis due to prior registration with the NRCHD under the German PAN Prevalence Study, which showed a potential bias in the inclusion of this patient population (proportion of mild cardiac lesions was comparatively high due to improved diagnostic capability… More >

Athanasios Koutsoukis¹, Xavier Halna du Fretay², Patrick Dupouy³, Phalla Ou⁴, Jean-Pierre Laissy⁴, Jean-Michel Juliard⁵, Fabien Hyafil⁶, Pierre Aubry⁵

Congenital Heart Disease, Vol.12, No.6, pp. 726-732, 2017, DOI:10.1111/chd.12504

Abstract Objective: The diagnosis of anomalous connections of the coronary arteries (ANOCOR) requires an appropriate identification for the management of the patients involved. We studied the observer variability in the description and classification of ANOCOR between a nonexpert group of physicians and a group of expert physicians, using the ANOCOR cohort.
Patients and design: Consecutive patients identified by 71 referring cardiologists were included in the ANOCOR cohort. Anomalous connection was diagnosed by invasive and/or computed tomography coronary angiography. Angiographic images were reviewed by an angiographic committee with experience in this field. Both investigators and angiographic committee filled out a questionnaire to… More >

Andrew N. Redington

Congenital Heart Disease, Vol.12, No.5, pp. 559-560, 2017, DOI:10.1111/chd.12527

Abstract This article has no abstract. More >

Ritu Sachdeva¹, Pamela S. Douglas², Michael S. Kelleman¹, Courtney E. McCracken¹, Leo Lopez³, Kenan W.D. Stern⁴, Benjamin W. Eidem⁵, Oscar J. Benavidez⁶, Rory B. Weiner⁶, Elizabeth Welch³, Robert M. Campbell¹, Wyman W. Lai⁷

Congenital Heart Disease, Vol.12, No.3, pp. 373-381, 2017, DOI:10.1111/chd.12455

Abstract Objective: The objective of this study was to evaluate effectiveness of educational intervention (EI) in the Pediatric Appropriate Use of Echocardiography (PAUSE) study to improve appropriateness of transthoracic echocardiograms (TTEs) ordered in pediatric cardiology clinics.
Design: Data were prospectively collected after the publication of the Appropriate Use Criteria (AUC) document during 2 phases: the pre-EI phase (1/1/15 to 4/30/15) and the post-EI phase (7/ 1/15 to 10/30/15). Pre-EI, site-investigators (SI) determined AUC indications, by reviewing the clinic records. Post-EI, providers assigned indications prior to obtaining TTE.
Setting: Pediatric cardiology clinics at six centers.
Patients: Those ≤18 years old, receiving initial… More >

Rohit S. Loomba¹, Gabrielle Geddes¹, Amanda J. Shillingford², David A. Hehir²

Congenital Heart Disease, Vol.12, No.3, pp. 332-339, 2017, DOI:10.1111/chd.12448

Abstract Background: Splenic dysfunction is common in heterotaxy syndrome, and increases the risk of bacteremia and bacteremia related mortality. Despite the risks associated with bacteremia in this setting, best practice guidelines for management of infectious concerns are lacking. We conducted a survey of pediatric cardiologists to characterize practice regarding the diagnosis of splenic dysfunction, approach to antibiotic prophylaxis, and management of possible bacterial infection.
Methods: A 22-item web-based survey was distributed via email to pediatric cardiologists in North America.
Results: We received 230 responses from 63 centers, for a response rate of 22%. The majority (83%) always obtain abdominal ultrasound to… More >

Mohd Nizam Mat Bah¹, Mohd Hanafi Sapian¹, Mohammad Tamim Jamil¹, Nisah Abdullah¹, Emieliyuza Yusnita Alias¹, Norazah Zahari²

Congenital Heart Disease, Vol.13, No.6, pp. 1012-1027, 2018, DOI:10.1111/chd.12672

Abstract Objectives: There is limited data on congenital heart disease (CHD) from the lower‐ and middle‐income country. We aim to study the epidemiology of CHD with the specific objective to estimate the birth prevalence, severity, and its trend over time.
Design: A population‐based study with data retrieved from the Pediatric Cardiology Clinical Information System, a clinical registry of acquired and congenital heart dis‐ ease for children.
Setting: State of Johor, Malaysia.
Patients: All children (0‐12 years of age) born in the state of Johor between January 2006 and December 2015.
Intervention: None.
Outcome measure: The birth prevalence, severity, and temporal trend… More >

Matthew W. Buelow^1,2, Nancy Rudd¹, Jena Tanem¹, Pippa Simpson³, Peter Bartz^1,2, Garick Hill⁴

Congenital Heart Disease, Vol.13, No.6, pp. 919-926, 2018, DOI:10.1111/chd.12655

Abstract Background: Single ventricle heart disease with aortic arch hypoplasia has high mor‐ bidity and mortality, with the greatest risk after stage 1 palliation. Residual lesions often require catheter‐based or surgical reintervention to minimize risk. We sought to describe the types, frequency, and risk factors for re‐intervention between stage 1 and stage 2 palliation, utilizing the National Pediatric Cardiology Quality Improvement Collaborative (NPC‐QIC) registry.
Methods: The NPC‐QIC registry, consisting of patients discharged after stage 1 pal‐ liation, was queried. Hybrid stage 1 palliation patients were excluded from this study. The primary risk factor was shunt type and the primary outcome was… More >

Katharina Niedermayr¹, Gerhard Pölzl², Sabine Scholl‐Bürgi¹, Christine Fauth³, Ulrich Schweigmann¹, Edda Haberlandt¹, Ursula Albrecht¹, Manuela Zlamy¹, Wolfgang Sperl⁴, Johannes A. Mayr⁴, Daniela Karall¹

Congenital Heart Disease, Vol.13, No.5, pp. 671-677, 2018, DOI:10.1111/chd.12634

Abstract Objective: In general, a mitochondrial disorder is diagnosed on the basis of symptom combinations and confirmed by genetic findings. However, patients carrying the m.3243A>G mutation in the mitochondrial tRNA leucine 1 (MT‐TL1) do not always meet all the proposed criteria for the most frequently encountered mitochondrial syndrome “MELAS,” an acronym for Mitochondrial Encephalomyopathy, Lactic Acidosis, and at least one Stroke‐like episode. We here present various phenotypic characteristics of the mitochondrial mutation m.3243A>G with particular focus on cardiac manifestations.
Methods and Results: We followed nine patients (1 month to 68 years old; median 42 years; four female and five male) from… More >

Ashley E. Neal¹, Elizabeth Lehto¹, Karen Hughes Miller², Erin Davis³, Craig Ziegler²

Congenital Heart Disease, Vol.13, No.5, pp. 788-793, 2018, DOI:10.1111/chd.12626

Abstract Objective: The 2016 American Board of Pediatrics (ABP) content outline is comprehensive, including more than 50 cardiology-specific objectives within eight content areas. This study complements the quantitative analysis of a Kentucky-wide survey of trainees, pediatricians, and pediatric cardiologists asking them to identify “most important” cardiology content by analyzing their open-ended comments about “what should be added” and “why?” within these eight categories.
Design, Methods, Outcome Measures: This cross-sectional study used an original, online survey instrument based on the 2016 ABP cardiology-specific objectives. We began an initial analysis of the qualitative data using Pandit’s version of Glaser and Strauss Grounded theory… 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 >