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Double Outlet Right Ventricle With Normally Related Great Arteries

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Rare Association of Absent Pulmonary Valve Syndrome with Double Outlet Right Ventricle and Hypoplastic Left Heart Complex.

Erik L Frandsen, Arpan R Doshi, Sujatha Buddhe, Bhawna Arya, Sathish M Chikkabyrappa,

Kans J Med (Kansas journal of medicine)
[2020, 13:21-22]

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The science and art of aortic and/or pulmonary root translocation.

Supreet P Marathe, Sachin Talwar,

This review aims to present and compare different surgical techniques of root translocation of the great arteries except the Ross procedure. The historical aspects, technical considerations, and results are briefly elucidated. ... Read more >>

Ann Pediatr Cardiol (Annals of pediatric cardiology)
[2020, 13(1):56-66]

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Loss of Asb2 Impairs Cardiomyocyte Differentiation and Leads to Congenital Double Outlet Right Ventricle.

Abir Yamak, Dongjian Hu, Nikhil Mittal, Jan W Buikema, Sheraz Ditta, Pierre G Lutz, Christel Moog-Lutz, Patrick T Ellinor, Ibrahim J Domian,

Defining the pathways that control cardiac development facilitates understanding the pathogenesis of congenital heart disease. Herein, we identify enrichment of a Cullin5 Ub ligase key subunit, Asb2, in myocardial progenitors and differentiated cardiomyocytes. Using two conditional murine knockouts, Nkx+/Cre.Asb2fl/fl and AHF-Cre.Asb2fl/fl, and tissue clarifying technique, we reveal Asb2 requirement for embryonic ... Read more >>

iScience (iScience)
[2020, 23(3):100959]

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EDUCATIONAL SERIES IN CONGENITAL HEART DISEASE: Echocardiographic assessment of transposition of the great arteries and congenitally corrected transposition of the great arteries

Meryl S Cohen, Luc L Mertens,

Echocardiographic assessment of patients with transposition of the great arteries and congenitally corrected transposition requires awareness of the morphology and commonly associated lesions. The pre-operative echocardiography should include a full segmental and sequential analysis. Post-operative assessment is not possible without awareness of the type of surgical procedure performed and consists ... Read more >>

Echo Res Pract (Echo research and practice)
[2019, 6(4):R107-R119]

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Imaging of the aortic root on high-pitch non-gated and ECG-gated CT: awareness is the key!

Prashant Nagpal, Mukta D Agrawal, Sachin S Saboo, Sandeep Hedgire, Sarv Priya, Michael L Steigner,

The aortic pathologies are well recognized on imaging. However, conventionally cardiac and proximal aortic abnormalities were only seen on dedicated cardiac or aortic studies due to need for ECG gating. Advances in CT technology have allowed motionless imaging of the chest and abdomen, leading to an increased visualization of cardiac ... Read more >>

Insights Imaging (Insights into imaging)
[2020, 11(1):51]

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Virtual three-dimensional model for preoperative planning in a complex case of a double outlet right ventricle.

Swati Garekar, Alpa Bharati, Firoza Kothari, Sachin Patil, Shyam Dhake, Shivaji Mali, Amit Mhatre, Dilip Bind, Ashwini Joshi, Bharat Soni, Dhananjay Malankar,

A 3-year-old child with a complex variant of double outlet right ventricle achieved a successful biventricular repair after detailed delineation of the intracardiac anatomy on multimodality imaging. A virtual three-dimensional (3D) model based on computed tomography was used successfully in the absence of an actual 3D-printed model. This case report ... Read more >>

Ann Pediatr Cardiol (Annals of pediatric cardiology)
[2019, 12(3):295-297]

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Essential role of the Crk family-dosage in DiGeorge-like anomaly and metabolic homeostasis.

Akira Imamoto, Sewon Ki, Leiming Li, Kazunari Iwamoto, Venkat Maruthamuthu, John Devany, Ocean Lu, Tomomi Kanazawa, Suxiang Zhang, Takuji Yamada, Akiyoshi Hirayama, Shinji Fukuda, Yutaka Suzuki, Mariko Okada,

CRK and CRKL (CRK-like) encode adapter proteins with similar biochemical properties. Here, we show that a 50% reduction of the family-combined dosage generates developmental defects, including aspects of DiGeorge/del22q11 syndrome in mice. Like the mouse homologs of two 22q11.21 genes CRKL and TBX1, Crk and Tbx1 also genetically interact, thus ... Read more >>

Life Sci Alliance (Life science alliance)
[2020, 3(2):]

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Tetralogy of Fallot with isolated levocardia in a young female.

Zeeshan Sattar, Hafez Muhammad Abdullah, Sohaib Roomi, Waqas Ullah, Adnan Khan, Ali Ghani, Asrar Ahmad,

Tetralogy of Fallot is the most common cyanotic congenital heart disease. It consists of right ventricular outflow tract obstruction, a ventricular septal defect, abnormally located aorta and right ventricular hypertrophy. It usually occurs as an isolated anomaly with a normally placed heart and abdominal viscera. We present a case of ... Read more >>

J Community Hosp Intern Med Perspect (Journal of community hospital internal medicine perspectives)
[2019, 9(3):275-278]

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Tetralogy of Fallot: morphological variations and implications for surgical repair.

Saad M Khan, Nigel E Drury, John Stickley, David J Barron, William J Brawn, Timothy J Jones, Robert H Anderson, Adrian Crucean,

OBJECTIVES:Tetralogy of Fallot is characterized by anterocephalad deviation of the outlet septum, along with abnormal septoparietal trabeculations, which lead to subpulmonary infundibular stenosis. Archives of retained hearts are an important resource for improving our understanding of congenital heart defects and their morphological variability. This study aims to define variations in ... Read more >>

Eur J Cardiothorac Surg (European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery)
[2019, 56(1):101-109]

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Transcatheter occlusion of antegrade pulmonary blood flow in children with univentricular heart.

Gülhan Tunca Şahin, İbrahim Cansaran Tanıdır, Selman Gökalp, Alper Güzeltaş,

Background:This study aims to evaluate the results of transcatheter occlusion of antegrade pulmonary blood flow in children with univentricular heart. Methods:Medical data of a total of seven patients (4 females, 3 males; median age 11.7 years; range, 1 to 24 years) who underwent transcatheter occlusion of the antegrade pulmonary blood ... Read more >>

Turk Gogus Kalp Damar Cerrahisi Derg (Turk gogus kalp damar cerrahisi dergisi)
[2019, 27(3):274-279]

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Management of Congenital Heart Disease: State of the Art-Part II-Cyanotic Heart Defects.

P Syamasundar Rao,

In this review management of the most common cyanotic congenital heart defects (CHDs) was discussed; the management of acyanotic CHD was reviewed in Part I of this series. While the need for intervention in acyanotic CHD is by and large determined by the severity of the lesion, most cyanotic CHDs ... Read more >>

Children (Basel) (Children)
[2019, 6(4):]

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Double-outlet left ventricle: A rare case.

Ashok Sunder, Bijaya Mohanty, Manoj Kumar Sahoo,

Double-outlet left ventricle (DOLV) is a rare cardiac malformation in which both great arteries originate from the morphological left ventricle. DOLV is associated with high mortality, generally due to heart failure, myocardial infarction, or aortic thrombosis. With surgery, the 5-year survival rate is estimated at 70%-75%. Most patients will continue ... Read more >>

J Family Med Prim Care (Journal of family medicine and primary care)
[2019, 8(5):1769-1771]

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Safety and efficacy of tranexamic acid in paediatric cardiac surgery: study protocol for a double-blind randomised controlled trial.

Yu Zhang, Yuan Jia, Jia Shi, Su Yuan, Rong Wang, Zhe Zhang, Xu Wang, Jinping Liu, Jun Ran, Yuchen Zhao, Zhongdong Hua, Jun Yan, Shoujun Li, Zhe Zheng, Shengshou Hu, Yang Wang, Fuxia Yan,

INTRODUCTION:An initial retrospective study suggested that tranexamic acid (TXA) administration increased the incidence of seizures in paediatric patients undergoing cardiac surgery. However, the efficacy of TXA in paediatric cardiac surgery remains unclear owing to the small sample sizes of the studies. Therefore, this study will investigate the efficacy and safety ... Read more >>

BMJ Open (BMJ open)
[2019, 9(11):e032642]

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A new low-cost method of virtual cardiac dissection of computed tomographic datasets.

Saurabh Kumar Gupta, Diane E Spicer, Robert H Anderson,

Computed tomography has an established role in the evaluation of a variety of cardiac disorders, including congenital heart diseases. The current generation of high-speed scanners produces volumetric data at low doses of radiation. The interpretation of cardiac anatomy, however, is generally limited to multiplanar assessment of two-dimensional images. The volume ... Read more >>

Ann Pediatr Cardiol (Annals of pediatric cardiology)
[2019, 12(2):110-116]

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Postoperative brain volumes are associated with one-year neurodevelopmental outcome in children with severe congenital heart disease.

Eliane Meuwly, Maria Feldmann, Walter Knirsch, Michael von Rhein, Kelly Payette, Hitendu Dave, Ruth O' Gorman Tuura, Raimund Kottke, Cornelia Hagmann, Beatrice Latal, András Jakab, ,

Children with congenital heart disease (CHD) remain at risk for neurodevelopmental impairment despite improved perioperative care. Our prospective cohort study aimed to determine the relationship between perioperative brain volumes and neurodevelopmental outcome in neonates with severe CHD. Pre- and postoperative cerebral MRI was acquired in term born neonates with CHD ... Read more >>

Sci Rep (Scientific reports)
[2019, 9(1):10885]

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Say NO to ROS: Their Roles in Embryonic Heart Development and Pathogenesis of Congenital Heart Defects in Maternal Diabetes.

Anish Engineer, Tana Saiyin, Elizabeth R Greco, Qingping Feng,

Congenital heart defects (CHDs) are the most prevalent and serious birth defect, occurring in 1% of all live births. Pregestational maternal diabetes is a known risk factor for the development of CHDs, elevating the risk in the child by more than four-fold. As the prevalence of diabetes rapidly rises among ... Read more >>

Antioxidants (Basel) (Antioxidants (Basel, Switzerland))
[2019, 8(10):]

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Left ventricular mass estimation by real-time 3D echocardiography favourably competes with CMR in congenital left ventricular disease.

Miriam Michel, Wael Shabanah, Hermann Körperich, Andrea Kelter-Klöpping, Andreas Entenmann, Anca Racolta, Kai Thorsten Laser,

Assessment of left ventricular mass (LVM) is important in the evaluation of patients with congenital heart disease (CHD) and cardiac magnetic resonance imaging (CMR) is the gold standard. Recent software allows LVM calculation by real-time 3-dimensional echocardiography (RT3DE). We investigated the impact of different software analysis tools on LVM determination ... Read more >>

Sci Rep (Scientific reports)
[2019, 9(1):11888]

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GATA6 mutations: Characterization of two novel patients and a comprehensive overview of the GATA6 genotypic and phenotypic spectrum.

Doris Škorić-Milosavljević, Fleur V Y Tjong, Julien Barc, Ad P C M Backx, Sally-Ann B Clur, Karin van Spaendonck-Zwarts, Roelof-Jan Oostra, Najim Lahrouchi, Leander Beekman, Regina Bökenkamp, Daniela Q C M Barge-Schaapveld, Barbara J Mulder, Elisabeth M Lodder, Connie R Bezzina, Alex V Postma,

The first human mutations in GATA6 were described in a cohort of patients with persistent truncus arteriosus, and the phenotypic spectrum has expanded since then. This study underscores the broad phenotypic spectrum by presenting two patients with de novo GATA6 mutations, both exhibiting complex cardiac defects, pancreatic, and other abnormalities. ... Read more >>

Am. J. Med. Genet. A (American journal of medical genetics. Part A)
[2019, 179(9):1836-1845]

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Altered Hemorheology in Fontan Patients in Normoxia and After Acute Hypoxic Exercise.

Julian Alexander Härtel, Nicole Müller, Ulrike Herberg, Johannes Breuer, Daniel Alexander Bizjak, Wilhelm Bloch, Marijke Grau,

Background:The Fontan circulation is a unique palliation procedure for several congenital heart defects. Impaired exercise capacity has previously been demonstrated in these patients and also a higher risk for cardiopulmonary mortality. Hemorheology was shown to affect cardiopulmonary capacity and in turn to be affected by regular exercise and hypoxia but ... Read more >>

Front Physiol (Frontiers in Physiology)
[2019, 10:1443]

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EDUCATIONAL SERIES IN CONGENITAL HEART DISEASE: Three-dimensional echocardiography in congenital heart disease.

John M Simpson, Annemien van den Bosch,

Three-dimensional echocardiography is a valuable tool for the assessment of cardiac function where it permits calculation of chamber volume and function. The anatomy of valvar and septal structures can be presented in unique and intuitive ways to enhance surgical planning. Guidance of interventional procedures using the technique has now become ... Read more >>

Echo Res Pract (Echo research and practice)
[2019, 6(2):R75-R86]

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Validation of aortic valve 4D flow analysis and myocardial deformation by cardiovascular magnetic resonance in patients after the arterial switch operation.

W H S van Wijk, J M P J Breur, J J M Westenberg, M M P Driessen, F J Meijboom, B Driesen, E C de Baat, P A F M Doevendans, T Leiner, H B Grotenhuis,

BACKGROUND:Aortic regurgitation (AR) and subclinical left ventricular (LV) dysfunction expressed by myocardial deformation imaging are common in patients with transposition of the great arteries after the arterial switch operation (ASO). Echocardiographic evaluation is often hampered by reduced acoustic window settings. Cardiovascular magnetic resonance (CMR) imaging provides a robust alternative as ... Read more >>

J Cardiovasc Magn Reson (Journal of cardiovascular magnetic resonance : official journal of the Society for Cardiovascular Magnetic Resonance)
[2019, 21(1):20]

Cited: 2 times

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The Role of Non-Coding RNA in Congenital Heart Diseases.

Angel Dueñas, Almudena Expósito, Amelia Aranega, Diego Franco,

Cardiovascular development is a complex developmental process starting with the formation of an early straight heart tube, followed by a rightward looping and the configuration of atrial and ventricular chambers. The subsequent step allows the separation of these cardiac chambers leading to the formation of a four-chambered organ. Impairment in ... Read more >>

J Cardiovasc Dev Dis (Journal of cardiovascular development and disease)
[2019, 6(2):]

Cited: 1 time

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2019 HRS/EHRA/APHRS/LAHRS expert consensus statement on catheter ablation of ventricular arrhythmias: Executive summary.

Edmond M Cronin, Frank M Bogun, Philippe Maury, Petr Peichl, Minglong Chen, Narayanan Namboodiri, Luis Aguinaga, Luiz Roberto Leite, Sana M Al-Khatib, Elad Anter, Antonio Berruezo, David J Callans, Mina K Chung, Phillip Cuculich, Andre d'Avila, Barbara J Deal, Paolo Della Bella, Thomas Deneke, Timm-Michael Dickfeld, Claudio Hadid, Haris M Haqqani, G Neal Kay, Rakesh Latchamsetty, Francis Marchlinski, John M Miller, Akihiko Nogami, Akash R Patel, Rajeev Kumar Pathak, Luis C Saenz Morales, Pasquale Santangeli, John L Sapp, Andrea Sarkozy, Kyoko Soejima, William G Stevenson, Usha B Tedrow, Wendy S Tzou, Niraj Varma, Katja Zeppenfeld,

Ventricular arrhythmias are an important cause of morbidity and mortality and come in a variety of forms, from single premature ventricular complexes to sustained ventricular tachycardia and fibrillation. Rapid developments have taken place over the past decade in our understanding of these arrhythmias and in our ability to diagnose and ... Read more >>

J Arrhythm (Journal of arrhythmia)
[2020, 36(1):1-58]

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Reiterative Mechanisms of Retinoic Acid Signaling during Vertebrate Heart Development.

Eliyahu Perl, Joshua S Waxman,

Tightly-regulated levels of retinoic acid (RA) are critical for promoting normal vertebrate development. The extensive history of research on RA has shown that its proper regulation is essential for cardiac progenitor specification and organogenesis. Here, we discuss the roles of RA signaling and its establishment of networks that drive both ... Read more >>

J Dev Biol (Journal of developmental biology)
[2019, 7(2):]

Cited: 1 time

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Coding of coronary arterial origin and branching in congenital heart disease: The modified Leiden Convention.

Adriana C Gittenberger-de Groot, Wilke M C Koenraadt, Margot M Bartelings, Regina Bökenkamp, Marco C DeRuiter, Mark G Hazekamp, Ad J J C Bogers, Jan M Quaegebeur, Martin J Schalij, Hubert W Vliegen, Robert E Poelmann, Monique R M Jongbloed,

OBJECTIVES:Variations in coronary anatomy are common and may relate to the position of the coronary ostium relative to the aortic sinus, the angle of coronary take-off, or the course of the coronary arterial branches. Several classification systems have been proposed. However, they all lack a simple rationale that is applicable ... Read more >>

J. Thorac. Cardiovasc. Surg. (The Journal of thoracic and cardiovascular surgery)
[2018, 156(6):2260-2269]

Cited: 1 time

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