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R S Monteiro, D P Dob, M R Cauldwell, M A Gatzoulis
Women with a single ventricle circulation palliated with the Fontan operation require specialist multidisciplinary management. We report 14 such cases with successful pregnancies and detail the pathophysiology encountered. A combined obstetric and cardiac service between Chelsea and Westminster Hospital and Royal Brompton Hospital provides care for women with heart disease, and maintains a prospective database of referred women. We searched this database for women with a known Fontan circulation and reviewed the case notes and electronic patient records between January 1994 and December 2015...
September 7, 2016: International Journal of Obstetric Anesthesia
Carla Frescura, Gaetano Thiene
Transposition of the great arteries (TGA) is a cardiac condition in which the arterial trunks arise from the inappropriate ventricle: the aorta from the right ventricle and the pulmonary trunk from the left ventricle [discordant ventriculo-arterial (VA) connection]. In complete TGA, the discordant VA connection is associated with situs solitus or inversus and concordant atrioventricular (AV) connection. The hemodynamic consequence of these combined connections is that systemic and pulmonary circulations function in "parallel" rather than in "series"...
2016: Frontiers in Pediatrics
Christophe P Teuwen, Tim I M Korevaar, Rosa L Coolen, Twan van der Wel, Charlotte A Houck, Reinder Evertz, Ameeta Yaksh, Jolien W Roos-Hesselink, Ad J J C Bogers, Natasja M S de Groot
AIMS: Atrial fibrillation (AF) is increasingly observed in patients with congenital heart defects (CHDs) who survive nowadays into adulthood. Yet, predictors of AF are scarce in this high-risk population. This study therefore examined the predictive ability of atrial extrasystole (AES) for development of AF in CHD patients. METHODS AND RESULTS: Adult CHD patients who had a 24 h Holter registration were followed to determine who developed AF. A total of 573 patients (49% male, mean age 35 ± 12 years) were included; they had a simple/complete repaired CHD (n = 279), complex repaired CHD (n = 251), or univentricular heart (UVH, n = 43)...
October 4, 2016: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
Eva Hlavackova, Martin Liska, Hana Jicinska, Jiri Navratil, Jiri Litzman
The Fontan operation or total cavopulmonal connection (TCPC) is a palliative surgical correction of rare and complex inborn cardiac malformations that are characterized by univentricular circulation. Protein-losing enteropathy (PLE) develops in 4-13% of patients after the Fontan procedure. Fontan-related PLE leads to secondary combined immunodeficiency marked by hypogammaglobulinemia and predominantly CD4+ lymphocytopenia. Here, we present 3 case reports of patients with secondary immunodeficiency after the Fontan operation...
September 30, 2016: International Archives of Allergy and Immunology
Sara E Peterson, Amy K Bennett, Barbara Sargent
No abstract text is available yet for this article.
2016: Pediatric Physical Therapy
Heidi Mäenpää, Arja Häkkinen, Anne Sarajuuri
PURPOSE: To compare changes in motor development from 1 to 5 years of age among 18 children with hypoplastic left heart syndrome and 12 with univentricular heart to 42 children without heart defect. METHODS: Motor development was assessed with the Alberta Infant Motor Scale and Movement Assessment Battery for Children (Movement ABC). RESULTS: Children with hypoplastic left heart syndrome or univentricular heart had significantly lower scores on the Alberta Infant Motor Scale test at the age of 1 and on the Movement ABC test at the age of 5 years compared with controls...
2016: Pediatric Physical Therapy
Anil K Singhi, Sivakumar Kothandum
Failing Fontan physiology in univentricular hearts manifest with protein-losing enteropathy, plastic bronchitis, low cardiac output, and recurrent effusions. Transcatheter creation of fenestration in a failing Fontan may be useful in alleviating the symptoms by improving the cardiac output. It is traditionally achieved by puncturing through the conduit from femoral or jugular venous access. In the absence of good venous path, transhepatic access provides a direct route for needle puncture of the conduit. If marked intimal ingrowth into the conduit results in increasing rigidity and makes the conduit nonyielding, alternative approaches may be needed...
September 2016: Annals of Pediatric Cardiology
Kevin J Morine, Vikram Paruchuri, Xiaoying Qiao, Mark Aronovitz, Gordon S Huggins, David DeNofrio, Michael S Kiernan, Richard H Karas, Navin K Kapur
INTRODUCTION: Transient receptor potential (TRP) channels are broadly expressed cation channels that mediate diverse physiological stimuli and include canonical (TRPC), melastatin (TRPM), and vanilloid (TRPV) subtypes. Recent studies have implicated a role for TRPC6 channels as an important component of signaling via the cytokine, transforming growth factor beta 1 (TGFβ1) in right (RV) or left ventricular (LV) failure. Endoglin (Eng) is a transmembrane glycoprotein that promotes TRPC6 expression and TGFβ1 activity...
August 21, 2016: Cardiovascular Pathology: the Official Journal of the Society for Cardiovascular Pathology
Miguel Urencio, Ali Dodge-Khatami, Chris E Greenleaf, Giorgio Aru, Jorge D Salazar
For repair of interrupted aortic arch, unfavorable anatomy challenges a tension-free anastomosis. We describe a useful alternative surgical technique used in five neonates/infants, involving splitting the ascending aorta from the sinotubular junction to the arch origin, leftward and posterior "sliding" of the flap with anastomosis to the distal arch creating a native tissue bridge, and reconstruction with a patch. With wide interruption gaps between proximal and distal aortic portions, the ascending aortic slide is a safe and reproducible technique, providing a tension-free native tissue bridge with potential for growth, and a scaffold for patch augmentation in biventricular hearts, or for Norwood stage I in univentricular palliation...
September 2016: World Journal for Pediatric & Congenital Heart Surgery
Sayaka Mii, Kazushi Yasuda, Hiroomi Murayama
A 2-year-old girl with a functionally univentricular heart associated with a pulmonary sequestration underwent right lower lobectomy after which increased lung volume with low mean pulmonary artery pressure and pulmonary vascular resistance was documented. A cardiac catheterisation performed after a subsequent total cavopulmonary connection demonstrated favourable Fontan haemodynamics. Lobectomy may have induced compensatory lung growth, contributing to the maintenance of haemodynamics favourable for the long-term success of the Fontan procedure...
August 30, 2016: Cardiology in the Young
Olivier Villemain, Damien Bonnet, Lucile Houyel, Mathieu Vergnat, Magalie Ladouceur, Virginie Lambert, Zakaria Jalal, Pascal Vouhé, Emre Belli
The management of double-outlet right ventricle associated with anatomically noncommitted ventricular septal defect constitutes a surgical challenge. The limits for, and the specific outcomes after anatomical vs univentricular repair still remain to be established. Between 1993 and 2011, 36 consecutive patients presenting with double-outlet right ventricle or noncommitted ventricular septal defect (21 inlet, 10 muscular, and 5 central perimembranous) and 2 adequately sized ventricles underwent surgical repair at 2 centers...
2016: Seminars in Thoracic and Cardiovascular Surgery
Lianne M Geerdink, Gideon J du Marchie Sarvaas, Irene M Kuipers, Willem A Helbing, Tammo Delhaas, Henriette Ter Heide, Lieke Rozendaal, Chris L de Korte, Sandeep K Singh, Tjark Ebels, Mark G Hazekamp, Felix Haas, Ad J J C Bogers, Livia Kapusta
OBJECTIVE: Surgical outcomes of pediatric patients with Ebstein's anomaly are often described as part of all-age-inclusive series. Our objective is to focus on patients treated surgically in childhood (0-18 y). We study the intended treatment (biventricular or 1.5 ventricle repair or univentricular palliation), freedom from unplanned reoperation and survival of this specific age group, in a nationwide study. DESIGN: Records of all Ebstein's anomaly patients born between 1980 and 2013 were reviewed...
August 25, 2016: Congenital Heart Disease
Daniel J Penny, Rajesh Krishnamurthy
OBJECTIVES: The objectives of this review are to discuss the pathophysiology of the circulation with a functionally univentricular heart, with a focus on the unique physiologic characteristics, which provide the underpinnings for the management of these complex patients. DATA SOURCE: MEDLINE and PubMed. CONCLUSIONS: The circulation of the patient with a functionally univentricular heart displays unique physiologic characteristics, which are quite different from those of the normal biventricular circulation...
August 2016: Pediatric Critical Care Medicine
Nathalie H P Claessens, Pim Moeskops, Andreas Buchmann, Beatrice Latal, Walter Knirsch, Ianina Scheer, Ivana Išgum, Linda S de Vries, Manon J N L Benders, Michael von Rhein
BACKGROUND: This study aimed to assess cortical gray matter growth and maturation in neonates with congenital heart disease (CHD). METHODS: Thirty-one (near) term neonates with severe CHD (8 univentricular heart malformation (UVH), 21 d-transposition of great arteries (d-TGA) and 2 aortic coarctation) underwent cerebral MRI before (postnatal-day 7) and after (postnatal-day 24) surgery. Eighteen controls with similar gestational age had one MRI (postnatal-day 23)...
August 24, 2016: Pediatric Research
Kerry L Gaskin, David J Barron, Amanda Daniels
UNLABELLED: Aim The aim of this study was to explore parental preparedness for discharge and their experiences of going home with their infant after the first-stage surgery for a functionally univentricular heart. BACKGROUND: Technological advances worldwide have improved outcomes for infants with a functionally univentricular heart over the last 3 decades; however, concern remains regarding mortality in the period between the first and second stages of surgery...
October 2016: Cardiology in the Young
Hoi Lam She, Arno A W Roest, Emmeline E Calkoen, Pieter J van den Boogaard, Rob J van der Geest, Mark G Hazekamp, Albert de Roos, Jos J M Westenberg
PURPOSE: To evaluate the inflow pattern and flow quantification in patients with functional univentricular heart after Fontan's operation using 4D flow magnetic resonance imaging (MRI) with streamline visualization when compared with the conventional 2D flow approach. METHOD: Seven patients with functional univentricular heart after Fontan's operation and twenty-three healthy controls underwent 4D flow MRI. In two orthogonal two-chamber planes, streamline visualization was applied, and inflow angles with peak inflow velocity (PIV) were measured...
July 18, 2016: Congenital Heart Disease
Ujjwal Kumar Chowdhury, Poonam Malhotra Kapoor, Keerthi Rao, Parag Gharde, Mukesh Kumawat, Priya Jagia
We report a rare complication of massive aneurysm of the proximal ligated end of the main pulmonary artery which occurred in the setting of a patient with a functionally univentricular heart and increased pulmonary blood flow undergoing superior cavopulmonary connection. Awareness of this possibility may guide others to electively transect the pulmonary artery in such a clinical setting.
July 2016: Annals of Cardiac Anaesthesia
Rohit S Loomba, Peter C Kouretas, Robert H Anderson
Isomerism, also known as heterotaxy, is a unique clinical entity in which there are mirror imaged findings in the thoracic. In the abdomen, the arrangement of organs can be random. The anatomic findings also have functional consequences. Isomerism has been known to increase morbidity and mortality in those with functionally univentricular hearts. The aim of this study was to determine the impact of isomerism on the Glenn hospitalization. Data from the 1997 to 2012 Kids' Inpatient Database were utilized for this cross-sectional study...
July 8, 2016: Pediatric Cardiology
David S Cooper, Stuart L Goldstein, David M Kwiatkowski
No abstract text is available yet for this article.
July 2016: Pediatric Critical Care Medicine
Jinghao Zheng, Botao Gao, Zhongqun Zhu, Guocheng Shi, Zhiwei Xu, Jinfen Liu, Xiaomin He
OBJECTIVES: The optimal surgical strategies for pulmonary atresia with intact ventricular septum (PAIVS) are still not well established. This study reviewed our 15-year experience in the management of PAIVS. METHODS: Between July 1999 and June 2014, 170 patients were treated for PAIVS in our heart centre. Based on the morphology of the right ventricle (RV), age and surgical approaches, the patients were divided into two groups: the one-stage surgery group (n = 33) and the staged surgery group (n = 137), in which patients received definitive repair, including biventricular repair, 1...
July 1, 2016: European Journal of Cardio-thoracic Surgery
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