R. Allen Ligon^1,*, Larry A. Latson¹, Mark M. Ruzmetov², Kak-Chen Chan¹, Todd Roth¹, Immanuel I. Turner², Frank G. Scholl², Steve Bibevski²

Congenital Heart Disease, Vol.16, No.3, pp. 285-297, 2021, DOI:10.32604/CHD.2021.014373

Abstract Background: Pulmonary valve replacement (PVR) can be accomplished via surgical, transcatheter, or hybrid approaches. There are inherent advantages to transcatheter PVR and hybrid PVR without cardiopulmonary bypass. We review the methods and results of a standardized institutional approach to PVR. Methods: Retrospective review of all PVR cases between February 2017 and February 2020. Hybrid PVR entailed off-pump RVOT plication with percutaneous transcatheter PVR. Results: Primary transcatheter PVR was attempted in 37, hybrid PVR was performed in 11, and on-pump surgical PVR was performed in 9. Median age at PVR was 27 years (6–65). Primary transcatheter PVR was successful in 35/37… More >

Se Yong Jung^#, Doyoung Jung^#, Ah Young Kim, Jae Hee Seol, Jung Min Park, Jo Won Jung, Jae Young Choi^*

Congenital Heart Disease, Vol.16, No.3, pp. 233-244, 2021, DOI:10.32604/CHD.2021.014272

Abstract Purpose: This study examined the efficacy and safety of selexipag in treating pulmonary arterial hypertension (PAH) associated with congenital heart disease (CHD). Materials and Methods: We conducted a retrospective study of patients with CHD-associated PAH, treated with selexipag since December 2017. Thirteen adult patients (mean age, 45.4 years; women, 77%) were treated with selexipag as add-on therapy. Baseline characteristics, World Health Organization functional class, 6-minute walking distance (6MWD) test results, N-terminal pro-B-type natriuretic peptide levels, echocardiographic data, and incidence of side effects were assessed. Results: The majority of patients (12/13, 92.3%) experienced more than one treatment-associated complication; one patient dropped… More >

Ruikun Zou¹, Yifei Wang^1,*, Chengcheng Pang², Yunxia Sun¹, Chen Chen¹, Jian Zhuang³

Congenital Heart Disease, Vol.16, No.2, pp. 159-170, 2021, DOI:10.32604/CHD.2021.015050

Abstract Aim: To determine the profiles of clinical features including four-limb blood pressure (BP), saturations of peripheral oxygen (SpO₂), and echocardiographic features in infants with coarctation of aorta (CoA) to facilitate congenital heart diseases screening. Methods: The charts of infants with CoA were retrospectively reviewed. All in-hospital infants suspected of congenital heart diseases by clinical teams were prospectively measured of four limbs BPs and SpO₂ in a regional cardiac transferring center during 2013 and 2019. Echocardiography as a gold standard test was followed within 2 days after suspicion. All infants were divided into non-significant CoA group or significant CoA group based… More >

Reghan Conrey^1,*, Sebastian Tume², Carlos Bonilla-Ramirez³, Seema Lalani⁴, Dean McKenzie^3,#, Marc Anders^2,#

Congenital Heart Disease, Vol.16, No.2, pp. 171-181, 2021, DOI:10.32604/CHD.2021.014409

Abstract Background: Patients with genetic syndromes who undergo surgery to correct congenital heart defects can be at risk for increased morbidity or mortality. Surgical outcomes and postoperative courses following congenital heart surgery in patients with Kabuki-Syndrome (KS) have not been well studied. Objectives: The purpose of this study was to describe the postoperative courses and associated outcomes in the largest set of KS patients undergoing congenital heart surgery to date. Methods: Patients with a confirmed molecular diagnosis of KS and a diagnosis of a CHD admitted to Texas Children’s Hospital between January 1, 2000 and January 1, 2020 were included (n… More >

Christopher M. Day^1,*, Neda Mulla², Timothy Martens³, Brent M. Gordon²

Congenital Heart Disease, Vol.16, No.2, pp. 183-187, 2021, DOI:10.32604/CHD.2021.014337

Abstract Marfan syndrome patients have connective tissue abnormalities that predispose them to intracardiac defects and postoperative complications. We present a case of late onset ASD device failure secondary to device movement within the atrial septum in a girl with Marfan syndrome. This case study suggests that further studies are necessary to determine the optimal device and approach for ASD repair in this patient cohort. More >

Félix Collard¹, Dimitrios Buklas², Pascale Maragnes¹, Fabien Labombarda^1,*

Congenital Heart Disease, Vol.16, No.2, pp. 141-146, 2021, DOI:10.32604/CHD.2021.013180

Abstract Objective: Abnormal coronary artery origin (ACAO) from the opposite sinus with inter-arterial course of the ectopic proximal vessel is associated with the greatest potential for clinical manifestations, specifically sudden death. Data remain limited regarding the association between bicuspid aortic valve (BAV) and this potentially dangerous coronary variant reported in up to 0.6% in the general population. We investigated the frequency of this high-risk ACAO with inter-arterial course in our surgical series of BAV patients. Methods and Results: We conducted a retrospective study to identify BAV patients with ACAO and inter-arterial course who underwent elective aortic valve/root surgery between 2010 and… More >

Misook Seo¹, In-Kyung Song², Hye-Mee Kwon², Byungdoo Andrew Lee², Won-Jung Shin^2,*

Congenital Heart Disease, Vol.16, No.2, pp. 123-136, 2021, DOI:10.32604/CHD.2021.012626

Abstract Background: In patients with cyanotic congenital heart disease (CHD), cerebral oxygenation may be maintained by elevations in hematocrit (Hct). Hemodilution accompanying cardiopulmonary bypass (CPB), however, can disrupt cerebral oxygen balance, leading to fluctuations in cerebral oxygen saturation (ScO₂). The present study investigated the effects of Hct changes on the fluctuation of ScO₂ during CPB in cyanotic CHD using performance measurement (PM). Methods: Children with CHD (51 acyanotic and 46 cyanotic) who had undergone cardiac surgery using CPB were enrolled. Median performance error (MDPE), median absolute performance error (MDAPE), and wobble parameters of ScO₂ were calculated before (reference value), during, and… More >

Jingjing Li¹, Xiaorong Wang², Yuan Liu³, Guodong Zhao³, Ting Dai³, Hong Chen³, Haiyan Liao⁴, Haiying Qi^3,#, Jia Li^5,6,7,#,*

Congenital Heart Disease, Vol.16, No.1, pp. 45-52, 2021, DOI:10.32604/CHD.2021.013459

Abstract Background: Previous studies from high altitudes have reported significantly higher prevalence of congenital heart disease (CHD), consisting almost solely of simple CHD. Little is known about the occurrence of complex CHD. Neonates with complex CHD are likely admitted to NICU. We examined the prevalence and spectrum of complex CHD in NICU in order to depict a truer picture of CHD at high altitude. Methods: We reviewed charts of 4,214 neonates admitted to NICU in Qinghai province (average altitude 3,000 m). Echocardiography was performed in 1,943 babies when CHD was suspected based on clinical examinations. Results: CHD was diagnosed in 1,093… More >

Prisca Eser^1,*, Thomas Gruber¹, Thimo Marcin¹, Claudia Boeni^1,2, Kerstin Wustmann³, Christina DeLuigi¹, Matthias Greutmann⁴, Daniel Tobler⁵, Markus Schwerzmann³, Matthias Wilhelm¹

Congenital Heart Disease, Vol.16, No.1, pp. 73-84, 2021, DOI:10.32604/CHD.2021.013051

Abstract Background: The purpose of this study was to investigate whether patients with adult congenital heart disease (ACHD) benefit from exercise-based cardiac rehabilitation (CR) short- and long-term with regard to improvement of cardiorespiratory fitness. Methods: Cardiopulmonary exercise tests (CPET) completed by ACHD patients between January 2000 and October 2019 were analysed retrospectively. Linear mixed models were performed for peak oxygen consumption (VO₂) with patients as random effect and age, sex, disease classification, preceding surgery (≤3 months) and preceding CR (≤4 weeks for short term and >4 weeks for long term) as fixed effects. Results: 1056 CPETs of 311 ACHD patients with… More >

Nunzia Borrelli^1,2, Jolanda Sabatino^1,3, Martina Avesani^1,4, Josefa Paredes¹, Manjit Josen¹, Alain Fraisse¹, Paolo Guccione², Guido Michielon¹, Giovanni Di Salvo^1,4,*

Congenital Heart Disease, Vol.16, No.1, pp. 27-37, 2021, DOI:10.32604/CHD.2021.012526

Abstract Background: Still little is known about the impact on right ventricle function of the 2 main approaches to Norwood palliation in Hypoplastic left heart syndrome, the right ventricle-pulmonary artery shunt (RVPAS) and the modified Blalock-Taussig shunt (mBTS). Methods: The cohort included 27 patients with Hypoplastic left heart syndrome (10 in the mBTS group, 17 in the RVPAS group). Longitudinal strain, tricuspid annulus peak systolic excursion and fractional area change were evaluated before Norwood and in four different breakpoints in the steady-state after Norwood procedure (30-days, 90-days, 140-days and 200-days after Norwood). Results: No significant differences were found in all standard… More >