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.;… 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… 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… 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]),… More >

Varun Aggarwal¹, Christopher J. Petit², Andrew C. Glatz^3，4, Bryan H. Goldstein⁵, Athar M. Qureshi¹

Congenital Heart Disease, Vol.14, No.1, pp. 110-115, 2019, DOI:10.1111/chd.12709

Abstract The use of prostaglandin‐E1 immediately after birth and subsequent surgical crea‐ tion of the modified Blalock‐Taussig shunt (BTS) shunt have remarkably improved the prognosis and survival of children with congenital heart disease and ductal‐depend‐ ent pulmonary blood flow (PBF). Despite the advancement in surgical techniques, bypass strategies, and postoperative management, significant morbidity and mortal‐ ity after BTS still remain. Patent ductus arteriosus stenting has been shown to be as an acceptable alternative to BTS placement in select infants with ductal‐dependent PBF. Newer procedural techniques and equipment, along with operator experience have all contributed to procedural More >

Dana M. Boucek¹, Athar M. Qureshi², Bryan H. Goldstein ³, Christopher J. Petit⁴, Andrew C. Glatz^1,5

Congenital Heart Disease, Vol.14, No.1, pp. 105-109, 2019, DOI:10.1111/chd.12707

Abstract Background: Infants with ductal-dependent pulmonary blood flow (PBF) often undergo a palliative procedure to provide a stable source of PBF prior to definitive palliation or repair. In the current era, a surgical shunt or ductal stent is used to provide PBF. We aimed to review the current literature comparing ductal stents to surgical shunts.
Methods and Results: Four small, single-center studies and two larger multicenter studies were identified comparing ductal stent to surgical shunt. Combined, these studies showed ductal stent resulted in similar or improved pulmonary artery growth, fewer complications, shorter length of stay, less diuretic use, More >

Sara Salehyar^{1, †}, Nickolas Forsch^1,†,*, Kathleen Gilbert^2,3, Alistair A. Young^3,4, James C. Perry⁵, Sanjeet Hegde⁵, Jeffrey H. Omens^1,6, Andrew D. McCulloch^1,6

Molecular & Cellular Biomechanics, Vol.16, No.3, pp. 179-183, 2019, DOI:10.32604/mcb.2019.07384

Abstract Tetralogy of Fallot (TOF) is the most common form of cyanotic congenital heart disease. Infants diagnosed with TOF require surgical interventions to survive into adulthood. However, as a result of postoperative structural malformations and long-term ventricular remodeling, further interventions are often required later in life. To help identify those at risk of disease progression, serial cardiac magnetic resonance (CMR) imaging is used to monitor these patients. However, most of the detailed information on cardiac shape and biomechanics contained in these large four-dimensional (4D) data sets goes unused in clinical practice for lack of efficient and… More >

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 >

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 over time.
… More >

Shiraz A. Maskatia¹, Christopher J. Petit^2,3, Curtis D. Travers², David J. Goldberg⁴, Lindsay S. Rogers⁴, Andrew C. Glatz⁴, Athar M. Qureshi⁶, Bryan H. Goldstein⁵, Jingning Ao^2,3, Ritu Sachdeva^2,3

Congenital Heart Disease, Vol.13, No.6, pp. 892-902, 2018, DOI:10.1111/chd.12671

Abstract Background: In patients with pulmonary atresia, intact ventricular septum (PA/IVS) following right ventricular (RV) decompression, RV size and morphology drive clinical outcome. Our objectives were to (1) identify baseline and postdecompression echo‐ cardiographic parameters associated with 2V circulation, (2) identify echocardio‐ graphic parameters associated with RV growth and (3) describe changes in measures of RV size and changes in RV loading conditions.
Methods: We performed a retrospective analysis of patients who underwent RV de‐ compression for PA/IVS at four centers. We analyzed echocardiograms at baseline, postdecompression, and at follow up (closest to 1‐year or prior to Glenn… More >