Carlos E. B. Branco¹, Roney O. Sampaio¹, Flavio Tarasoutchi¹, Justin P. Zachariah²

Congenital Heart Disease, Vol.13, No.6, pp. 1069-1071, 2018, DOI:10.1111/chd.12676

Abstract In the 2017, “Cairo Accord on Rheumatic Heart Disease—From Molecules to The Global Community” experts from endemic areas enumerated an approach to reduce the population burden of rheumatic heart disease. The 10 key recommendations in‐ clude immediate logistical objectives as well as domains for further study. Echocardiographic population screening programs were relegated to research alone. Given the large body of supporting data, relegating echo screening to an investiga‐ tional modality is an opportunity lost. More >

Nikki M. Singh¹, Rohit S. Loomba², Todd M. Gudausky¹, Michael E. Mitchell³

Congenital Heart Disease, Vol.13, No.6, pp. 935-943, 2018, DOI:10.1111/chd.12670

Abstract Introduction: In patients with tetralogy of Fallot (TOF), use of transannular patch (TAP) may be required in order to relieve significant right ventricular outflow tract obstruction, subsequently resulting in pulmonary insufficiency (PI). The monocusp valve has been used to temporarily reduce insufficiency in hopes to improve short and midterm outcomes. The purpose of this study was to assess for potential benefits of the monocusp valve in this subset of patients.
Design: Between 2005 and 2016, 119 patients with TOF with pulmonary stenosis who underwent repair with TAP were analyzed, 43 (36.1%) had a monocusp valve placed. Immediate… More >

Rohit S. Loomba¹, Seth B. Gray², Saul Flores³

Congenital Heart Disease, Vol.13, No.5, pp. 646-654, 2018, DOI:10.1111/chd.12662

Abstract Introduction: Ketamine is a drug often used for procedural sedation or as adjunct agent for general sedation in children with congenital heart disease. In the clinical realm, there is often confusion regarding the effects of ketamine on hemodynamics, particularly pulmonary vascular resistance and systemic vascular resistance. We per‐ formed a meta‐analysis of studies investigating the effects of ketamine on hemodynamics.
Methods: A systematic review was conducted to identify studies characterizing the hemodynamic effects of ketamine in children with congenital heart disease. Studies were assessed for quality and those of satisfactory quality with pre‐ and postketa‐ mine hemodynamics… 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 >

Cheryl L. Brosig^1,2, Laurel Bear^1,2, Sydney Allen¹, Pippa Simpson¹, Liyun Zhang¹, Michele Frommelt^1,2, Kathleen A. Mussatto²

Congenital Heart Disease, Vol.13, No.5, pp. 700-705, 2018, DOI:10.1111/chd.12632

Abstract Background and Objectives: Children with congenital heart disease (CHD) are at risk for neurodevelopmental (ND) delays. The purpose of this study is to compare the ND testing results of children with CHD at 2 and 4 years of age and determine if rates of ND delays change over time.
Methods: Children with CHD completed the Bayley Scales of Infant Development‐III (BSID‐III) at 2 years of age, and standardized neuropsychological measures at 4 years. Scores were compared with test norms and were classified as: average (within one SD of test mean); at risk (1‐2 SDs from the test… More >

Juan Jesús Sánchez‐Barriga

Congenital Heart Disease, Vol.13, No.5, pp. 690-699, 2018, DOI:10.1111/chd.12631

Abstract Background: Congenital heart disease (CHD) represents a global health problem. In Mexico, in children <1 year of age it is the second cause of mortality. The aim was to determine mortality trends from CHD and the great vessels in children and adults nationwide, by state and socioeconomic region.
Methods: Records of mortality associated to CHD and the great vessels for 2000‐2015 were obtained from the National Institute of Statistics and Geography. This information is collected from death certificates issued nationwide. International Classification of Diseases, 10th revision, codes corresponding to the basic cause of death from CHD and… More >

Sherry L. Farr¹, Karrie F. Downing^1,2, Tiffany Riehle-Colarusso¹, Ginnie Abarbanell^1,3

Congenital Heart Disease, Vol.13, No.4, pp. 633-639, 2018, DOI:10.1111/chd.12621

Abstract Objective: To examine how cognitive and motor limitations in children with heart disease are associated with education and participation in extracurricular activities.
Design: Using 2009–2010 parent-reported data from the National Survey of Children with Special Health Care Needs (NS-CSHCN), we examined prevalence of five functional limitations (learning/ concentration, communication, self-care, gross motor skills, and fine motor skills) by diagnosed heart disease status using chi-square tests and multivariable logistic regression. Among CSHCN with heart disease, we examined the associations between severity of each functional limitation and missing ≥11 days of school in the past year, receiving special education… More >

Elizabeth C. Caris¹, Nicole Dempster², Gil Wernovsky³, Yongjie Miao², Melissa Moore-Clingenpeel², Trent Neely², Rachel Fonseca², Holly Miller-Tate², Robin Allen², Samantha Fichtner², Jamie Stewart², Clifford L. Cua²

Congenital Heart Disease, Vol.13, No.4, pp. 528-532, 2018, DOI:10.1111/chd.12619

Abstract Objectives: Siblings of children with chronic medical conditions endorse a lower quality of life compared to age-matched peers. Caregiver and sibling-self report of adjustment are often discordant. Congenital heart disease significantly affects family life. To date, there have been no studies addressing the functioning of siblings of children with hypoplastic left heart syndrome, one of the most severe forms of congenital heart disease. The goal of this study was to assess the impact of hypoplastic left heart syndrome on sibling’s quality of life as well as the caregiver’s perception of this effect.
Study Design: Cross-sectional study using… More >

Alex J. Thompson¹, Joseph A. Dearani², Jonathan N. Johnson^1,3, Hartzell V. Schaff², Eric C. Towe¹, Jared Palfreeman¹, Philip L. Wackel¹, Frank Cetta^1,3

Congenital Heart Disease, Vol.13, No.4, pp. 617-623, 2018, DOI:10.1111/chd.12618

Abstract Background: The transapical approach has been utilized in adult HCM patients with either midventricular obstruction or a small LV cavity; however, there are little data on its use in children.
Methods: We retrospectively reviewed all patients (age <21 years) with HCM who underwent a transapical myectomy from January 2002 to December 2016. Indication for surgery was midventricular obstruction in 19/23 (83%) and small LV cavity in 4 (17%). Preoperative symptoms included: dyspnea (96%), chest pain (65%), presyncope (61%), and syncope (35%). The mean age at the time of operation was 14 ± 4.0 years (range, 4–20).
Results: Overall,… More >

Timur Mese¹, Baris Guven², Murat Muhtar Yilmazer¹, Cem Karadeniz³, Rahmi Ozdemir¹, Onder Doksoz¹

Congenital Heart Disease, Vol.13, No.4, pp. 506-511, 2018, DOI:10.1111/chd.12616

Abstract Background: Mean platelet volume (MPV), platecrit, and platelet distribution width (PDW) are markers of platelet activation. Previous studies have found that platelet activation occurs in patients with pulmonary arterial hypertension. Platelet indices including MPV, PDW, and platecrit have not been studied in children with congenital heart disease associated pulmonary arterial hypertension (APAH-CHD) who survived and those who died.
Objective: The objective of this study to investigate the value of platelet indices with clinical and hemodynamic indicators predicting the disease severity and survival in children with APAH-CHD.
Methods: This was a nested case-control study. MPV, platecrit, and PDW levels… More >