Open Access

REVIEW

Elio Caruso^*, Silvia Farruggio, Salvatore Agati, Corrado Di Mambro
Congenital Heart Disease, Vol.16, No.4, pp. 309-331, 2021, DOI:10.32604/CHD.2021.015470
Abstract Fetal arrhythmias reach up around 10% of the total third-level perinatal cardiology references. Sustained bradycardia is defined as a baseline fetal heart rate (FHR) of less than 110 bpm sustained for at least 10 min. The overall incidence of malignant fetal bradyarrhythmias, such as complete atrioventricular block (AVB) and channellopathies, is relatively rare, 1:5000 pregnancies, but represents a serious emergency for the gynecologist, neonatologists, and pediatric cardiologists. Fetal complete AVB is strongly associated with maternal connective tissue disease, but it can be also associated with congenital heart disease and usually with a poorer prognosis with high risk of fetal hydrops… More >

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ARTICLE

Cheryl Raskind-Hood^1,*, Kashaine A. Gray^2,3, Jayne Morgan³, Wendy M. Book^4,*
Congenital Heart Disease, Vol.16, No.4, pp. 333-355, 2021, DOI:10.32604/CHD.2021.014384
Abstract Early intervention to prevent premature mortality is vital for adults with congenital heart defects (CHD). Anatomic complexity and comorbid conditions are thought to contribute to CHD mortality. Since hypertension (HTN) and heart failure (HF) are the comorbid conditions among the most prevalent causes of death in the United States, and commonly accompany CHD, it is crucial to evaluate whether they are reliable predictors of mortality for adults with CHD (ACHD) independent of anatomic CHD complexity. A retrospective cross-sectional analysis of ACHD, aged 18–64, with concomitant HTN and/or HF and at least one health care encounter during 2008–2010 were assessed. Of… More >

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ARTICLE

Allard T. van den Hoven¹, Sultan Yilmazer¹, Lidia R. Bons¹, Roderick W. J. van Grootel¹, L. W. Geenen¹, An M. van Berendoncks¹, Anthonie L. Duijnhouwer², Hans-Marc J. Siebelink³, Jolien W. Roos-Hesselink¹, Annemien E. van den Bosch^1,*
Congenital Heart Disease, Vol.16, No.4, pp. 357-368, 2021, DOI:10.32604/CHD.2021.014462
Abstract Aim: This study aimed to describe the systolic left ventricular global longitudinal strain (LvGLS) and left ventricular peak early diastolic strain rate (Sre) in adult women with Turner syndrome (TS) and to determine its relationship with exercise capacity and clinical parameters. Methods: In this cross-sectional cohort study, consecutively included adult TS women underwent an electrocardiogram, transthoracic echocardiogram (TTE) and cardiopulmonary exercise test (CPET) on the same day. LvGLS and Sre were measured using 2D speckle tracking analysis (STE) and compared with age-matched healthy female controls. Results: Ninety-four adult women (age 36 ± 13 years) with TS and 32 healthy age-matched… More >

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876

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CASE REPORT

Kotaro Hine^*, Kohei Ogata, Keiko Saitou, Norio Mizukaki, Hiroko Arai, Hitoshi Yoda
Congenital Heart Disease, Vol.16, No.4, pp. 369-371, 2021, DOI:10.32604/CHD.2021.014516
Abstract Abnormal branching of the aorta associated with the right aortic arch (RAA) has been reported as isolation of left subclavian artery (ILSA), isolation of left common carotid artery, isolation of brachiocephalic artery. ILSA is a rare aortic branch anomaly that originates in the left subclavian artery from the pulmonary artery via ductus arteriosus. Several reports have described ILSA associated with 22q11.2 deletion syndrome and tetralogy of Fallot. Here, we present a very unusual case of RAA with ILSA associated with D-transposition of the great arteries and inferior vena cava interrupted with azygos continuation. More >

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ARTICLE

Dongdong Zheng^1,#, Chi Shen^1,2,#, Wenshi Liu¹, Wenjing Lv¹, Xiaofei Li^1,*
Congenital Heart Disease, Vol.16, No.4, pp. 373-381, 2021, DOI:10.32604/CHD.2021.015260
Abstract Objectives: Pulmonary arterial hypertension in congenital heart disease (PAH-CHD) is the most common type of PAH and increases morbidity and mortality in patients with CHD. Right heart catheterization (RHC) is the standard method to diagnose PAH. However, RHC is an invasive and complicated method with relatively high cost. Noninvasive, feasible, and cost-efficient methods are urgently needed. The objective of this study was to evaluate three potential biomarkers of PAH-CHD: Hepatocyte growth factor (HGF), osteopontin (OPN), and suppression of tumorigenicity 2 (ST2). Methods: Plasma samples were collected from patients with CHD (n = 46) and healthy individuals (n = 22) and… More >

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894

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ARTICLE

Yajuan Zhang, Jun Yan^*, Qiang Wang, Shoujun Li, Jing Sun, Shuo Dong, Jiachen Li
Congenital Heart Disease, Vol.16, No.4, pp. 383-392, 2021, DOI:10.32604/CHD.2021.015479
Abstract Background: The long-term outcomes of patients treated with a Kawashima procedure and keeping the antegrade pulmonary blood flow (AnPBF) in single-ventricle (SV) and interrupted inferior vena cava (IVC) heart disease is still uncertain as yet. Methods: We investigated 18 patients who underwent the Kawashima procedure with SV physiology and an interrupted IVC between January 2009 and June 2018, perioperative, operative and postoperative characteristics were recorded. Results: A total of 18 patients underwent the Kawashima procedure at a median age of 2.7 years (range 0.5–24.7 years), of which 12 (66.7%) were male and 6 (33.3%) were female. The mean saturation was… More >

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CASE REPORT

Sarah Pradhan^1,*, Eileen Broomall², Russel Hirsch¹
Congenital Heart Disease, Vol.16, No.4, pp. 393-396, 2021, DOI:10.32604/CHD.2021.015257
Abstract The differential diagnosis for irritability in children is broad. In patients with congenital heart disease, one must strongly consider cardiac etiologies such as low cardiac output or elevated central venous pressure (CVP). In patients with single-ventricle physiology, the second stage of palliation includes bidirectional Glenn, which involves anastomosis of the superior vena cava to the pulmonary artery resulting in volume offloading of the single systemic ventricle. Typically, early in the post-operative period, patients may experience a headache due to the acute increase in CVP, and symptoms improve over time. Idiopathic intracranial hypertension (IIH), also known as pseudotumor cerebri, is a… More >

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895

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ARTICLE

Junsung Park¹, Go Hun Seo², Yena Lee¹, Yunha Choi¹, Minji Kang³, Hyo-Sang Do³, Young-Hwue Kim⁴, Jeong Jin Yu⁴, Ellen Ai-Rhan Kim⁵, Euiseok Jung⁵, Byong Sop Lee⁵, Jae Suk Baek^4,#,*, Beom Hee Lee^1,6,#,*
Congenital Heart Disease, Vol.16, No.4, pp. 397-410, 2021, DOI:10.32604/CHD.2021.015167
Abstract Background and Method: The genetic cause of infantile-onset cardiomyopathy is rarely investigated. Here, we conducted whole exome sequencing (WES) and mitochondrial DNA (mtDNA) sequencing in eight patients with infantile-onset cardiomyopathy to identify genetic variations. Result: Among these patients, two (25%) had dilated cardiomyopathy (DCMP), two (25%) had left ventricular non-compaction (LVNC), and four (50%) had hypertrophic cardiomyopathy (HCMP). Except four patients identified prenatally, the remaining patients presented at a median age of 85.5 days. WES identified genetic variants in a total of seven (87.5%) patients and mtDNA sequencing in the other case. TPM1 and MYH7 variants were identified in the… More >

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CASE REPORT

Isabella Leo¹, Jolanda Sabatino^1,2,3, Sabrina La Bella¹, Antonio Strangio¹, Iolanda Aquila¹, Concetta Procopio¹, Carmen Anna Maria Spaccarotella¹, Maria Petullà⁴, Salvatore De Rosa^1,2, Ciro Indolfi^1,2,5,*
Congenital Heart Disease, Vol.16, No.4, pp. 411-416, 2021, DOI:10.32604/CHD.2021.015062
Abstract We describe a case of a rare congenital heart disorder, scimitar syndrome, diagnosed in an adult woman presenting with dyspnea on exertion, chest pain and recurrent episodes of pulmonary infections. The hallmark of the syndrome is the presence of an enlarged anomalous pulmonary vein draining into the inferior vena cava. Speckle tracking echocardiography, including the often-forgotten atrial strain evaluation, is a sensitive parameter that should be routinely used for a better clinical and prognostic evaluation of patients with congenital heart disease (CHD). More >

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CASE REPORT

Elio Caruso¹, Silvia Farruggio^1,*, Davide Calvaruso¹, Corrado Di Mambro¹, David Angel Ortiz Ruiz¹, Salvatore Agati¹, Rafie Khoargami²
Congenital Heart Disease, Vol.16, No.4, pp. 417-425, 2021, DOI:10.32604/CHD.2021.015808
Abstract We show a brief report of two common arterial trunk cases (CAT) with different arrhythmias and discuss anatomy, clinical and diagnostic management. The burden of volume and pressure overload of this cardiac malformation may predispose to different types of arrhythmia before and after surgical repair. Because of labile hemodynamic state in this group of patients, prompt diagnosis of any arrhythmia is mandatory as the devastating factor on prognosis. The first patient with a diagnosis of CAT Type II Collett and Edwards (CE) had a particular history with HIV seropositive mother assuming antiretroviral therapy during pregnancy, who presented hyperbilirubinemia and liver… More >

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987