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Giovanni Di Salvo, Ziad Bulbul, Valeria Pergola, Ziad Issa, Ghassan Siblini, Nisreen Muhanna, Domenico Galzerano, Bahaa Fadel, Mansour Al Joufan, Majid Al Fayyadh, Zohair Al Halees
BACKGROUND: In patients who have undergone arterial switch operation (ASO) for d-transposition of the great arteries a gothic aortic arch (GA) morphology has been found and it has been associated with abnormal aortic bio-elastic properties. HYPOTHESIS: GA is frequent in ASO patients and may have an impact on cardiac mechanics. Our study aims were to assess 1- the occurrence of GA in a large sample of patients after ASO; 2- the association between GA and aortic bio-elastic properties; and 3- the impact of GA on left ventricular (LV) function using speckle tracking echocardiography (STE)...
March 11, 2017: International Journal of Cardiology
Maciej Słodki, Roland Axt-Fliedner, Katarzyna Zych-Krekora, Aline Wolter, Andreea Kawecki, Christian Enzensberge, Ewa Gulczyńska, Maria Respondek-Liberska
OBJECTIVE: Prenatal congenital heart disease classification system specify critical d-TGA with restriction of the foramen ovale (which requires Rashkind procedure) and planned d-TGA. However, current prenatal diagnostic criteria for post-delivery foramen ovale (FO) restriction in d-TGA are inadequate, resulting in a high false negative rate. We aim to find an echocardiographic features to predict the need for Rashkind procedure. METHODS: 98 patients from 2 European centers diagnosed prenatally with fetal d-TGA from 2006 to 2013 were analyzed and two groups were compared: 1) those in which the Rashkind procedure was performed within the first 24 hours of life; and 2) those who did not undergo a Rashkind procedure before cardiac surgery...
March 14, 2017: Ultrasound in Obstetrics & Gynecology
Frederik Helsen, Bert Vandenberk, Pieter De Meester, Alexander Van De Bruaene, Charlien Gabriels, Els Troost, Marc Gewillig, Bart Meyns, Rik Willems, Werner Budts
OBJECTIVE: To evaluate if development of fragmented QRS (fQRS) complexes, a marker of inhomogeneous ventricular activation due to myocardial fibrosis, is associated with adverse outcome in adults after Mustard/Senning repair for d-transposition of the great arteries (d-TGA). METHODS: Adults with atrial switch repair for d-TGA were selected from the database of a tertiary care hospital. Exclusion criteria were systemic right ventricular (RV) assist device or heart transplantation (HTx) before the age of 16, or fQRS already present at first visit to the Adult Congenital Heart Disease clinic...
February 9, 2017: Heart: Official Journal of the British Cardiac Society
Lucy M Safi, Ami B Bhatt
The arterial switch operation (ASO) is now the most frequently performed surgical correction in individuals with dextro-transposition of the great arteries (D-TGA). Patients who undergo this procedure as neonates have overall good clinical outcomes yet continued clinical follow-up is important to evaluate for postoperative complications. In this group, the highest mortality is in the immediate postoperative period and is generally associated with reimplantation of the coronary arteries. As these patients live into adulthood, longitudinal follow-up for other ASO complications including neo-pulmonary stenosis, right ventricular outflow tract (RVOT) obstruction, or neo-aortic root dilation and resulting aortic insufficiency should be performed...
January 2017: Current Treatment Options in Cardiovascular Medicine
Peter Klaver, Walter Knirsch, Karoline Wurmitzer, David Yoh von Allmen
This pilot study investigated neural correlates of visual working memory using functional magnetic resonance imaging (fMRI) in seven patients more than one decade after neonatal arterial switch operation for surgical correction of d-transposition of the great arteries (d-TGA, aged 10-18 years, 1 female). Compared with age and sex matched healthy controls patients showed similar visual working memory performance and a smaller increase in brain activity in the posterior parietal cortex with higher visual working memory load...
May 2016: Developmental Neuropsychology
Max E Godfrey, Kevin G Friedman, Monika Drogosz, Abraham M Rudolph, Wayne Tworetzky
OBJECTIVES: Although the postnatal physiology of D-loop transposition of the great arteries with intact ventricular septum (DTGA-IVS) is well established, little is known about fetal DTGA-IVS. In the normal fetus, the pulmonary valve (PV) is larger than the aortic valve (AoV), there is exclusive right to left flow at the foramen ovale (FO) and ductus arteriosus (DA), and the left ventricle (LV) ejects 40% of combined ventricular output (CVO) through the aorta, primarily to the brain. In DTGA-IVS, the LV ejects O2 -rich blood to the pulmonary artery, theoretically leading to pulmonary vasodilation, increased branch pulmonary arterial flow and reduced DA flow...
November 22, 2016: Ultrasound in Obstetrics & Gynecology
Paul Khairy
PURPOSE OF REVIEW: The literature on sudden death in transposition of the great arteries (D-TGA) with atrial switch surgery is reviewed and a pathophysiological mechanism is proposed. RECENT FINDINGS: Over 80% of sudden deaths in patients with D-TGA and Mustard or Senning baffles occur during exercise. Factors most consistently associated with ventricular arrhythmias and sudden death include heart failure parameters and atrial arrhythmias. Atrial arrhythmias have been observed to trigger malignant ventricular arrhythmias...
January 2017: Current Opinion in Cardiology
L Talemal, M T Donofrio
D-transposition of the great arteries (D-TGA) is the most commonly diagnosed cyanotic congenital heart disease presenting in the neonatal period. The survival after an arterial switch operation, with freedom from adverse cardiovascular events, has been reported to be as high as 93% at 25 years. However, despite excellent surgical outcomes, there continues to be significant preoperative morbidity and potential mortality due to compromise in the delivery room from foramen ovale closure requiring urgent balloon atrial septostomy for stabilization in the first minutes of life...
September 16, 2016: Journal of Neonatal-perinatal Medicine
Konrad Paczkowski, Ireneusz Haponiuk, Maciej Chojnicki, Radosław Jaworski
We present a case of a 2.5-year-old-girl with complex congenital heart disease: tricuspid atresia (TA), bulboventricular septal defect (VSD), hypoplastic right ventricle, d-transposition of the great arteries (d-TGA) with aortic outflow from redundant RV. Due to II/III degree atrioventricular block induced after diagnostic cardiac catheterization, an epicardial pacemaker was implanted during the Glenn procedure. Because of severe left ventricle outflow tract obstruction, she was finally referred for extracardiac TCPC (extracardiac Fontan type) with recruitment of PV and Damus-Kaye-Stansel anastomosis...
August 23, 2016: Heart Surgery Forum
Vincent J Schmithorst, Ashok Panigrahy, J William Gaynor, Christopher G Watson, Vince Lee, David C Bellinger, Michael J Rivkin, Jane W Newburger
OBJECTIVE: Little is currently known about the impact of congenital heart disease (CHD) on the organization of large-scale brain networks in relation to neurobehavioral outcome. We investigated whether CHD might impact ADHD symptoms via changes in brain structural network topology in a cohort of adolescents with d-transposition of the great arteries (d-TGA) repaired with the arterial switch operation in early infancy and referent subjects. We also explored whether these effects might be modified by apolipoprotein E (APOE) genotype, as the APOE ε2 allele has been associated with worse neurodevelopmental outcomes after repair of d-TGA in infancy...
August 2016: Brain and Behavior
David Backhoff, Gunter Kerst, Andrea Peters, Monika Lüdemann, Christian Frische, Michaela Horndasch, Gabriele Hessling, Thomas Paul, Ulrich Krause
BACKGROUND: Sudden cardiac death (SCD) is the most important cause of late mortality after atrial baffle procedure for d-transposition of the great arteries (d-TGA). Experience with internal cardioverter defibrillator (ICD) therapy in this population is limited. We conducted a multicenter cohort study to determine the current state of ICD therapy in individuals after atrial baffle procedure. METHODS: Demographic and clinical data as well as data on device implantation, programming, ICD discharges, and complications after atrial baffle procedure for d-TGA from four German centers were analyzed retrospectively...
October 2016: Pacing and Clinical Electrophysiology: PACE
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)...
November 2016: Pediatric Research
Sachin Talwar, Manikala Vinod Kumar, Amolkumar Bhoje, Shiv Kumar Choudhary, Shyam Sunder Kothari, Rajnish Juneja, Anita Saxena, Balram Airan
OBJECTIVES: In developing countries, where patients present late, the atrial switch operation is still a preferred palliation for d-transposition of great arteries (d-TGA). In this report, we present our experience in patients with d-TGA who were 5 years of age or older. METHODS: Twenty-seven patients underwent an atrial switch procedure between January 2004 and December 2014. The standard technique consisted of a combination of the Senning and Mustard's repair with Schumacker's in situ modification for construction of the pulmonary venous baffle...
November 2016: Interactive Cardiovascular and Thoracic Surgery
Yuji Nakajima
D-transposition of the great arteries (TGA) is one of the most common conotruncal heart defects at birth and is characterized by a discordant ventriculoarterial connection with a concordant atrioventricular connection. The morphological etiology of TGA is an inverted or arrested rotation of the heart outflow tract (OFT, conotruncus), by which the aorta is transposed in the right ventral direction to the pulmonary trunk. The rotational defect of the OFT is thought to be attributed to hypoplasia of the subpulmonic conus, which originates from the left anterior heart field (AHF) residing in the mesodermal core of the first and second pharyngeal arches...
September 2016: Congenital Anomalies
Emile C A Nyns, Andreea Dragulescu, Shi-Joon Yoo, Lars Grosse-Wortmann
Right ventricular (RV) volume and function evaluation is essential in the follow-up of patients after arterial switch operation (ASO) for dextro-transposition of the great arteries (d-TGA). Cardiac magnetic resonance (CMR) imaging using the Simpson's method is the gold-standard for measuring these parameters. However, this method can be challenging and time-consuming, especially in congenital heart disease. Knowledge-based reconstruction (KBR) is an alternative method to derive volumes from CMR datasets. It is based on the identification of a finite number of anatomical RV landmarks in various planes, followed by computer-based reconstruction of the endocardial contours by matching these landmarks with a reference library of representative RV shapes...
September 2016: International Journal of Cardiovascular Imaging
Kai Ma, Shoujun Li, Shengshou Hu, Zhongdong Hua, Keming Yang, Jun Yan, Hao Zhang, Qiuming Chen, Sen Zhang, Lei Qi
BACKGROUND: Report results of neoaortic regurgitation (NAR) after arterial switch for patients with d-transposition of the great arteries (d-TGA) and corrected transposition of the great arteries. METHODS: From 2003 to 2013, 583 patients who underwent arterial switch operation for d-TGA and 31 patients who underwent double switch (DS) for congenitally corrected transposition of the great arteries (cc-TGA) were included in this retrospective study. Since 2011, concomitant neoaortic sinotubular junction reconstruction was performed if aorta and pulmonary artery discrepancy was present in patients with d-TGA...
August 2016: Annals of Thoracic Surgery
Magalie Ladouceur, Alban Redheuil, Gilles Soulat, Christophe Delclaux, Michel Azizi, Mehul Patel, Gilles Chatellier, Antoine Legendre, Laurence Iserin, Younes Boudjemline, Damien Bonnet, Elie Mousseaux
BACKGROUND: Systemic right ventricle (sRV) dysfunction in d-transposition of the great arteries following atrial switch (d-TGA) is associated with increased mortality. We aimed to characterize maladaptive sRV mechanisms in d-TGA patients, analyzing relation of echocardiographic parameters of sRV systolic function to objective measurements of exercise capacity. METHODS: Forty-seven adult patients with d-TGA and atrial switch (mean age 31.6±4.2years) underwent conventional echocardiography, bidimensional strain (2D-strain), cardiac magnetic resonance (CMR) imaging and cardiopulmonary exercise evaluation on the same day...
August 15, 2016: International Journal of Cardiology
Dai Kimura, Mario Briceno-Medina, T K Susheel Kumar, Christopher J Knott-Craig
Neonatal arterial switch operation for simple dextro-transposition of the great arteries (d-TGA) has almost eliminated the occurrence of pulmonary vascular obstructive disease compared to patients who underwent Mustard or Senning procedure at an older age. We report a case of a neonate with d-TGA and intact ventricular septum who underwent arterial switch operation and yet developed severe pulmonary vascular obstructive disease within two months.
January 2017: World Journal for Pediatric & Congenital Heart Surgery
Adam R Cassidy, Matthew T White, David R DeMaso, Jane W Newburger, David C Bellinger
OBJECTIVE: To establish executive function (EF) structure/organization and test a longitudinal developmental cascade model linking processing speed (PS) and EF skills at 8-years of age to academic achievement outcomes, both at 8- and 16-years, in a large sample of children/adolescents with surgically repaired dextro-transposition of the great arteries (d-TGA). METHOD: Data for this study come from the 8- (n = 155) and 16-year (n = 139) time points of the Boston Circulatory Arrest Study and included WISC-III, Trail Making Test, Test of Variables of Attention, and WIAT/WIAT-II tasks...
October 2016: Neuropsychology
Magdalena Lipczyńska, Piotr Szymański, Olga Trojnarska, Lidia Tomkiewicz-Pająk, Bronisława Pietrzak, Anna Klisiewicz, Magdalena Kumor, Piotr Podolec, Piotr Hoffman
OBJECTIVE: We sought to identify maternal/neonatal and cardiovascular complications in pregnant women with complete transposition of great arteries (D-TGA) following atrial switch. METHODS: Clinical records of all women with D-TGA after the Mustard/Senning (M/S) operation who were followed at the three largest Adult Congenital Heart Disease (ACHD) centers in Poland were reviewed. RESULTS: Fifteen of the fifty-nine women followed had a total of 24 pregnancies, including two spontaneous miscarriages...
March 2017: Journal of Maternal-fetal & Neonatal Medicine
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