Emmanuel Akintoye¹, Gruschen R. Veldtman², William R. Miranda³, Heidi M. Connolly³, Alexander C. Egbe³

Congenital Heart Disease, Vol.14, No.2, pp. 138-139, 2019, DOI:10.1111/chd.12690

Abstract The purpose of this study was to determine the optimal age for performing Fontan operation using data from the National Inpatient Sample. Our results showed that although the Fontan operation was most commonly performed at age 2 in the United States, age 3 is the optimum age for this procedure as evident by lower rate of in‐hospital mortality, procedure‐related complications, and rate of nonroutine home discharge when procedure is performed at age 3 years. More >

Mariana M. Clavé¹, Nair Y. Maeda², Ana M. Thomaz¹, Sergio P. Bydlowski³, Antonio A. Lopes¹

Congenital Heart Disease, Vol.14, No.2, pp. 246-255, 2019, DOI:10.1111/chd.12688

Abstract Background: Ideally, vasodilator therapies for pulmonary arterial hypertension (PAH) should have a favorable impact on markers of vascular dysfunction, in addition to their known effects on hemodynamics, cardiac function, and patient’s physical capacity.
Methods: We analyzed circulating (plasma) markers of endothelial and platelet activation/dys‐ function (enzyme‐linked immunoassays) in the specific setting of advanced PAH associated with congenital heart disease, during the course of sildenafil and tadalafil therapies. Thirty‐one patients were enrolled (age 10‐54 years), most of them with chronic hypoxemia and elevated hematocrit. Drugs were administered orally for 6 months (sildenafil [n = 16], 20 mg t.i.d.; tada‐ lafil [n… More >

Soham Dasgupta¹, Shae Anderson¹, Michael Kelleman², Ritu Sachdeva¹

Congenital Heart Disease, Vol.14, No.2, pp. 230-235, 2019, DOI:10.1111/chd.12687

Abstract Introduction: In the pediatric Appropriate Use Criteria (AUC), abnormal electrocardiogram (ECG) in an asymptomatic patient has been rated as an “Appropriate” indication for transthoracic echocardiogram (TTE). We hypothesized that the yield of abnormal findings on TTE for this indication will be low.
Methods: All asymptomatic patients (≤ 18 years) from January 1, 2015 to December 31, 2017 who underwent initial outpatient evaluation at our center and had a TTE ordered for an abnormal ECG, were included. Clinic records were reviewed to obtain ECG and TTE findings.
Results: Of the 199 study patients, 13 (6.5%) had abnormal findings. Incomplete right bundle… More >

Samantha C. Butler^1,4, Anjali Sadhwani^1,4, Christian Stopp², Jayne Singer^1,3,4, David Wypij^2,5,6, Carolyn Dunbar‐Masterson², Janice Ware^1,3,4, Jane W. Newburger^2,5

Congenital Heart Disease, Vol.14, No.2, pp. 236-245, 2019, DOI:10.1111/chd.12686

Abstract Objective: Mortality rates for children with congenital heart disease (CHD) have significantly declined, resulting in a growing population with associated neurodevelopmental disabilities. American Heart Association guidelines recommend systematic developmental screening for children with CHD. The present study describes results of inpatient newborn neurodevelopmental assessment of infants after open heart surgery.
Outcome measures: We evaluated the neurodevelopment of a convenience sample of high‐risk infants following cardiac surgery but before hospital discharge using an adaptation of the Newborn Behavioral Observation. Factor analysis examined relationships among assessment items and consolidated them into domains of development.
Results: We assessed 237 infants at a median… More >

Georges Ephrem¹, Camden Hebson², Anitha John³, Estella Moore⁴, Maan Jokhadar⁴, Ryan Ford⁵, Gruschen Veldtman⁶, Yoav Dori⁷, Michelle Gurvitz^8,9, Brian Kogon¹⁰, Adrienne Kovacs¹¹, Meghan Roswick, Michael McConnell¹², Wendy M. Book⁴, Fred Rodriguez III^4,12

Congenital Heart Disease, Vol.14, No.2, pp. 128-137, 2019, DOI:10.1111/chd.12685

Abstract The initial “Frontiers in Fontan Failure” conference in 2015 in Atlanta, Georgia, provided an opportunity for experts in the field of pediatric cardiology and adult congenital heart disease to focus on the etiology, physiology, and potential interventions for patients with “Failing Fontan” physiology. Four types of “Fontan Failure” were described and then published by Dr Book et al. The acknowledgment that even Dr Fontan himself realized that the Fontan proce‐ dure “imposed a gradually declining functional capacity and premature late death after an initial period of often excellent palliation.” The purpose of the second “Frontiers in Fontan Failure” was to… More >

Shankar Baskar, Andrew N. Redington, Philip R. Khoury, Timothy K. Knilans, David S. Spar, Richard J. Czosek

Congenital Heart Disease, Vol.14, No.2, pp. 201-206, 2019, DOI:10.1111/chd.12684

Abstract Background: Traditional indices to evaluate biventricular (BiV) pacing are load dependent, fail to assess dynamic changes, and may not be appropriate in patients with congenital heart disease (CHD). We therefore measured the force‐frequency relationship (FFR) using tissue Doppler‐de‐ rived isovolumic acceleration (IVA) to assess the dynamic adaption of the myocardium and its vari‐ ability with different ventricular pacing strategies.
Methods: This was a prospective pilot study of pediatric and young adult CHD patients with biventricular or multisite pacing systems. Color‐coded myocardial velocities were recorded at the base of the systemic ventricular free wall. IVA was calculated at resting heart rate… More >

Masaki Sato¹, Kei Inai^1,2, Mikiko Shimizu¹, Hisashi Sugiyama¹, Toshio Nakanishi²

Congenital Heart Disease, Vol.14, No.2, pp. 167-175, 2019, DOI:10.1111/chd.12683

Abstract Objective: The recognition of fluid retention is critical in treating heart failure (HF). Bioelectrical impedance analysis (BIA) is a well-known noninvasive method; however, data on its role in managing patients with congenital heart disease (CHD) are limited. Here, we aimed to clarify the correlation between BIA and HF severity as well as the prognostic value of BIA in adult patients with CHD.
Design: This prospective single-center study included 170 patients with CHD admitted between 2013 and 2015. We evaluated BIA parameters (intra- and extracellular water, protein, and mineral levels, edema index [EI, extracellular water-to-total body water ratio]), laboratory values, and… More >

Raffaele Giordano¹, Massimiliano Cantinotti², Giuseppe Comentale¹, Luigi Di Tommaso¹, Gabriele Iannelli¹¹, Emanuele Pilato¹, Gaetano Palma¹

Congenital Heart Disease, Vol.14, No.2, pp. 162-166, 2019, DOI:10.1111/chd.12680

Abstract Background: In this study, we compared our experience about early and midterm follow‐up outcomes for right anterolateral minithoracotomy (RAMT) vs full sternot‐ omy (FS) in surgical aortic valve replacement (AVR) among adolescents with bicuspid aortic valve (BAV).
Methods: Patients were retrospectively enrolled from January 2008 to December 2017. Inclusion criteria were patients with BAV who had to undergo to AVR. They were divided in two groups: RAMT and FS. The choice of RAMT was based on indi‐ vidual surgeon’s preferences or when expressly requested by patient that was in‐ formed of nonconventional approach.
Results: We enrolled 61 patients, 23 in… More >

Shyam Sathanandam¹, Stephanie Whiting¹, Jorden Cunningham¹, David Zurakowski², Leah Apalodimas¹, B. Rush Waller¹, Ranjit Philip¹, Athar M. Qureshi³

Congenital Heart Disease, Vol.14, No.1, pp. 6-14, 2019, DOI:10.1111/chd.12729

Abstract Background: Patent ductus arteriosus (PDA) is highly prevalent in extremely low birth weight (ELBW), preterm infants. There are diverse management approaches for the PDA in ELBW infants. The objectives of this research were to identify current PDA management practices among cardiologists and neonatologists in the United States, describe any significant differences in management, and describe areas where practices align.
Methods: A survey of 10 questions based on the management of PDA in ELBW infants was conducted among 100 prominent neonatologists from 74 centers and 103 prominent cardiologists from 75 centers. Among the cardiologists, approximately 50% were interventionists who perform transcatheter… More >

Jennifer L. Shepherd, Shahab Noori

Congenital Heart Disease, Vol.14, No.1, pp. 21-26, 2019, DOI:10.1111/chd.12727

Abstract Objective: There is no consensus on the definition of a hemodynamically significant patent ductus arteriosus (hsPDA). In this review article, our objective is to discuss the main variables that one should consider when determining the hemodynamic signifi‐ cance of a PDA.
Results: We describe the various approaches that have been utilized over time to define an hsPDA and discuss the strengths and weaknesses of each echocardio‐ graphic index. Finally, we propose a comprehensive and individualized approach in determining the hemodynamic significance of the PDA.
Conclusion: There are several PDA‐related clinical, echocardiographic, and other ob‐ jective variables to take into consideration… More >