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https://www.readbyqxmd.com/read/28925321/an-unusual-combination-of-truncus-arteriosus-interrupted-aortic-arch-and-hypoplastic-left-ventricle
#1
Supreet P Marathe, Sanjeev H Naganur, Sabarinath Menon, Yishay Orr, Stephen G Cooper, David S Winlaw
Truncus arteriosus (common arterial trunk) is known to be associated with interrupted aortic arch in 10% to 15% of cases. However, the association of either of these lesions with a hypoplastic left ventricle is rare. The combination of all three of these lesions along with an intact interventricular septum is virtually unknown with only two cases reported in the literature. We report such a case with its anatomical and surgical management aspects.
January 1, 2017: World Journal for Pediatric & Congenital Heart Surgery
https://www.readbyqxmd.com/read/28924546/sirenomelia-associated-with-hypoplastic-left-heart-in-a-newborn
#2
H Turgut, R Ozdemir, I K Gokce, C Karakurt, A Karadag
Sirenomelia, also known as "mermaid malformation/syndrome," is a rare, serious congenital anomaly characterized by variable degrees of fusion of the lower limbs and associated with severe malformations of vertebral, genitourinary, cardiovascular system and single umbilical artery. The first pregnancy of a 25-year-old woman resulted in one twin born by Cesarian section at 32 weeks' gestation, who was referred to our hospital with cyanosis, a congenital anomaly and respiratory distress. On physical examination, there was no urogenital region and anal fissure and gender was indeterminate...
June 30, 2017: Balkan Journal of Medical Genetics: BJMG
https://www.readbyqxmd.com/read/28924274/quantitative-volumetric-analysis-of-a-retinoic-acid-induced-hypoplastic-model-of-chick-thymus-using-image-j
#3
Ayesha Haque, Muhammad Yunus Khan
OBJECTIVE: To assess the total volume change in a retinoic acid-induced, hypoplastic model of a chick thymus using Image-J. METHODS: This experimental study was carried out at the anatomy department of College of Physicians and Surgeons, Islamabad, Pakistan, from February 2009 to February 2010, and comprised fertilised chicken eggs. The eggs were divided into experimental group A and control group C. Group A was injected with 0.3µg of retinoic acid via yolk sac to induce a defective model of a thymus with hypoplasia...
September 2017: JPMA. the Journal of the Pakistan Medical Association
https://www.readbyqxmd.com/read/28922269/extubation-failure-is-associated-with-increased-mortality-following-first-stage-single-ventricle-reconstruction-operation
#4
Thomas Scodellaro, Jane M McKenzie, Yves d'Udekem, Warwick Butt, Siva P Namachivayam
OBJECTIVES: To identify the prevalence, causes, risk factors, and outcomes associated with extubation failure following first stage single ventricle reconstruction surgery. DESIGN: Retrospective cohort analysis of neonates who underwent a first stage single ventricle reconstruction operation. Extubation failure was defined as endotracheal reintubation within 48 hours of first extubation attempt. SETTING: The Royal Children's Hospital, Melbourne...
September 15, 2017: Pediatric Critical Care Medicine
https://www.readbyqxmd.com/read/28921168/chromosomal-abnormalities-affect-the-surgical-outcome-in-infants-with-hypoplastic-left-heart-syndrome-a-large-cohort-analysis
#5
Dala Zakaria, Xinyu Tang, Rupal Bhakta, Nahed O ElHassan, Parthak Prodhan
Patients with hypoplastic left heart syndrome (HLHS) can have associated genetic abnormalities. This study evaluated the incidence of genetic abnormalities among infants with HLHS and the short-term outcomes of this population during the first hospitalization. This is a retrospective analysis of the multi-center Pediatric Heath Information System database of infants with HLHS who underwent Stage I Norwood, Hybrid, or heart transplant during their first hospitalization from 2004 through 2013. We compared clinical data between infants with and without genetic abnormality, among the three most common chromosomal abnormalities, and between survivors and non-survivors...
September 18, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28918207/the-association-between-cardiac-physiology-acquired-brain-injury-and-postnatal-brain-growth-in-critical-congenital-heart-disease
#6
Shabnam Peyvandi, Hosung Kim, Joanne Lau, A James Barkovich, Andrew Campbell, Steven Miller, Duan Xu, Patrick McQuillen
OBJECTIVE: To assess the trajectory of perioperative brain growth in relationship to cardiac diagnosis and acquired brain injuries. METHODS: This was a cohort study of term neonates with hypoplastic left heart syndrome (HLHS) and transposition of the great arteries (TGA). Subjects underwent magnetic resonance imaging of the brain pre- and postoperatively to determine the severity of brain injury and total and regional brain volumes by the use of automated morphometry...
August 24, 2017: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/28905979/-cyanosis-in-a-calf-with-a-double-outlet-right-ventricle
#7
Nicola Wiedemann, Nicolai Hildebrandt, Manfred Henrich, Estelle Henrich, Matthias Schneider
A 4-day-old female Holstein Friesian calf was presented for evaluation of cyanosis and dyspnea. On auscultation, severe bronchovesicular sounds and a systolic heart murmur of grade IV/VI above the tricuspid valve were found. On echocardiography, a marked dextroposition of the aorta (> 50% originating from the right ventricle), leading to both great arteries arising from the right ventricle - a so-called double-outlet right ventricle - was detected. Two ventricular septal defects were present, one in the perimembranous, subpulmonary region, the other non-committed in the muscular region...
September 13, 2017: Tierärztliche Praxis. Ausgabe G, Grosstiere/Nutztiere
https://www.readbyqxmd.com/read/28904928/prenatal-sonographic-diagnosis-of-hypoplastic-left-heart-syndrome
#8
Raghu Teja Sadineni, B Santh Kumar, N B Chander, Durga Mahita Boppana
Hypoplastic left heart syndrome (HLHS) represents a variety of cardiac malformations that may result from errors in the early stages of cardiac development. HLHS includes a wide spectrum of cardiac malformations including hypoplasia of the left ventricle, ascending aorta, hypoplasia, or atresia of the aortic and mitral valves. Over the recent years, the improved resolution of advanced equipment with awareness and increased performance of second-trimester ultrasound examinations for the assessment of fetal anomalies have helped in understanding the spectrum and have expanded our knowledge of HLHS...
July 2017: International Journal of Applied and Basic Medical Research
https://www.readbyqxmd.com/read/28901225/promoting-pulmonary-arterial-growth-via-right-ventricle-to-pulmonary-artery-connection-in-children-with-pulmonary-atresia-ventricular-septal-defect-and-hypoplastic-pulmonary-arteries
#9
Edon J Rabinowitz, Shilpi Epstein, Nina Kohn, David B Meyer
BACKGROUND: Complete repair of pulmonary atresia (PA) ventricular septal defect (VSD) with hypoplastic or absent native pulmonary arteries, often with major aortopulmonary collateral arteries (MAPCAs), involves construction of an adequate sized pulmonary arterial tree. We report our results with a previously described staged strategy using initial right ventricle (RV)-to-reconstructed pulmonary arterial tree (RV-PA) connection to promote pulmonary arterial growth and facilitate later ventricular septation...
September 2017: World Journal for Pediatric & Congenital Heart Surgery
https://www.readbyqxmd.com/read/28893257/preoperative-single-ventricle-function-determines-early-outcome-after-second-stage-palliation-of-single-ventricle-heart
#10
Jacek Pająk, Michał Buczyński, Piotr Stanek, Grzegorz Zalewski, Marek Wites, Lesław Szydłowski, Bogusław Mazurek, Lidia Tomkiewicz-Pająk
BACKGROUND: Second-stage palliation with hemi-Fontan or bidirectional Glenn procedures has improved the outcomes of patients treated for single-ventricle heart disease. The aim of this study was to retrospectively analyze risk factors for death after second-stage palliation of single-ventricle heart and to compare therapeutic results achieved with the hemi-Fontan and bidirectional Glenn procedures. MATERIAL AND METHODS: We analyzed 60 patients who had undergone second-stage palliation for single-ventricle heart...
September 11, 2017: Cardiovascular Ultrasound
https://www.readbyqxmd.com/read/28891083/a-nationwide-survey-of-hypoplastic-myelodysplastic-syndrome-a-multicenter-retrospective-study
#11
Takashi Kobayashi, Yasuhito Nannya, Motoshi Ichikawa, Kenji Oritani, Yuzuru Kanakura, Akihiro Tomita, Hitoshi Kiyoi, Masayoshi Kobune, Junji Kato, Hiroshi Kawabata, Motohiro Shindo, Yoshihiro Torimoto, Yuji Yonemura, Nobuyoshi Hanaoka, Hideki Nakakuma, Daisuke Hasegawa, Atsushi Manabe, Naohito Fujishima, Nobuharu Fujii, Mitsune Tanimoto, Yasuyoshi Morita, Akira Matsuda, Atsushi Fujieda, Naoyuki Katayama, Haruhiko Ohashi, Hirokazu Nagai, Yoshiki Terada, Masayuki Hino, Ken Sato, Naoshi Obara, Shigeru Chiba, Kensuke Usuki, Masatsugu Ohta, Osamu Imataki, Makiko Uemura, Tomoiku Takaku, Norio Komatsu, Akira Kitanaka, Kazuya Shimoda, Kenichiro Watanabe, Kaoru Tohyama, Akifumi Takaori-Kondo, Hideo Harigae, Shunya Arai, Yasushi Miyazaki, Keiya Ozawa, Mineo Kurokawa
Hypoplastic myelodysplastic syndrome (hMDS) is a distinct entity with bone marrow (BM) hypocellularity and the risk of death from BM failure (BMF). To elucidate the characteristics of hMDS, the data of 129 patients diagnosed between April 2003 and March 2012 were collected from 20 institutions and the central review team of the National Research Group on Idiopathic Bone Marrow Failure Syndromes, and compared with 115 non-hMDS patients. More RA and fewer CMMoL and RAEB-t in French-American-British (FAB) and more RCUD and MDS-U and fewer RCMD in World Health Organization (WHO) classifications were found in hMDS than non-hMDS with significant differences...
September 11, 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/28889833/acute-kidney-injury-following-first-stage-palliation-in-hypoplastic-left-heart-syndrome-hybrid-versus-norwood-palliation
#12
Richard U Garcia, Girija Natarajan, Henry L Walters, Ralph E Delius, Sanjeev Aggarwal
OBJECTIVE: The aim of this study was to evaluate the prevalence of acute kidney injury after first-stage surgical palliation in patients with a single ventricle and to explore associated risk factors and outcomes. Design and patients This single-centre retrospective study included neonates who underwent either Norwood or Hybrid procedure from 2008 to 2015 for a single ventricle. Postoperative acute kidney injury was defined using the paediatric risk, injury, failure, loss, end-stage renal disease (pRIFLE), criteria within 72 hours of the procedure...
September 11, 2017: Cardiology in the Young
https://www.readbyqxmd.com/read/28889828/atrial-septal-defect-morphology-and-stenting-in-hypoplastic-left-heart-syndrome-after-hybrid-palliation
#13
Lilia Oreto, Giuseppe Mandraffino, Lucia Manuri, Michele B Saitta, Salvatore Agati, Concetta Zito, Fiore S Iorio, Scipione Carerj, Paolo Guccione
Aims The aim of this study was to describe atrial septal defect morphology in hypoplastic left heart syndrome, to report the incidence of restrictiveness and its relationship with defect morphology, to correlate restriction with midterm outcome, and to describe our interventional approach to restrictive defect. Methods and results From 2011 to 2015, 31 neonates with hypoplastic left heart syndrome underwent hybrid procedure with pulmonary artery banding and ductal stenting at our Institution. Restrictive physiology of the atrial septal defect was based on Doppler gradient >6 mmHg through the defect and on clinical signs of pulmonary hypertension...
September 11, 2017: Cardiology in the Young
https://www.readbyqxmd.com/read/28889813/unique-levoatriocardinal-veins-in-neonates-with-hypoplastic-left-heart-syndrome-and-intact-atrial-septum
#14
Chiaki Iida, Jun Muneuchi, Mamie Watanabe
We had two cases of neonates with hypoplastic left heart syndrome and intact atrial septum who had unique levoatriocardinal veins. Contrast-enhanced CT and angiography revealed that previously unknown communicating vessels ran from the top of the left atrium and drained into the right atrium. We emphasise that transcatheter atrial septostomy should be performed not through these communicating vessels but using the trans-septal approach in neonates with hypoplastic left heart syndrome and an intact atrial septum...
September 11, 2017: Cardiology in the Young
https://www.readbyqxmd.com/read/28885315/tuberous-breast-past-present-and-future-personal-classification-treatment-and-surgical-outcomes
#15
Alessandro Innocenti, Marco Innocenti, Francesco Mori, Dario Melita, Francesco Ciancio, Adriana Cordova
BACKGROUND: Tuberous breast (TB) is a rare congenital deformity, which may appear in different clinical forms representing various degrees of a single pathological entity. The worst cases are characterized by severe hypoplasia. Following a detailed analysis of the available relevant literature and a significant number of treated cases, in this article, the authors propose a new classification, with the aim of summarizing and simplifying a more intuitive categorization of the malformation, considering all the clinical aspects and including all types of TBs, even the minor ones, thus allowing a more immediate diagnosis and surgical planning...
September 6, 2017: Annals of Plastic Surgery
https://www.readbyqxmd.com/read/28884922/congenital-heart-defects-in-molecularly-proven-kabuki-syndrome-patients
#16
Maria Cristina Digilio, Maria Gnazzo, Francesca Lepri, Maria Lisa Dentici, Elisa Pisaneschi, Anwar Baban, Chiara Passarelli, Rossella Capolino, Adriano Angioni, Antonio Novelli, Bruno Marino, Bruno Dallapiccola
The prevalence of congenital heart defects (CHD) in Kabuki syndrome ranges from 28% to 80%. Between January 2012 and December 2015, 28 patients had a molecularly proven diagnosis of Kabuki syndrome. Pathogenic variants in KMT2D (MLL2) were detected in 27 patients, and in KDM6A gene in one. CHD was diagnosed in 19/27 (70%) patients with KMT2D (MLL2) variant, while the single patient with KDM6A change had a normal heart. The anatomic types among patients with CHD included aortic coarctation (4/19 = 21%) alone or associated with an additional CHD, bicuspid aortic valve (4/19 = 21%) alone or associated with an additional CHD, perimembranous subaortic ventricular septal defect (3/19 = 16%), atrial septal defect ostium secundum type (3/19 = 16%), conotruncal heart defects (3/19 = 16%)...
September 8, 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/28879411/serial-changes-in-anatomy-and-ventricular-function-on-dual-source-cardiac-computed-tomography-after-the-norwood-procedure-for-hypoplastic-left-heart-syndrome
#17
Hyun Woo Goo
BACKGROUND: Accurate evaluation of anatomy and ventricular function after the Norwood procedure in hypoplastic left heart syndrome is important for treatment planning and prognostication, but echocardiography and cardiac MRI have limitations. OBJECTIVE: To assess serial changes in anatomy and ventricular function on dual-source cardiac CT after the Norwood procedure for hypoplastic left heart syndrome. MATERIALS AND METHODS: In 14 consecutive patients with hypoplastic left heart syndrome, end-systolic and end-diastolic phase cardiac dual-source CT was performed before and early (average: 1 month) after the Norwood procedure, and repeated late (median: 4...
September 6, 2017: Pediatric Radiology
https://www.readbyqxmd.com/read/28870823/occipital-artery-to-middle-cerebral-artery-bypass-operative-nuances
#18
Toshikazu Kimura, Akio Morita
BACKGROUND: Superficial temporal artery-middle cerebral artery (STA-MCA) anastomosis is a common procedure for vascular neurosurgeons, and it is used in a variety of diseases. However, there are cases in which the STA is absent or is too hypoplastic to be used as a donor for revascularization. Occipital artery-MCA (OA-MCA) bypass may be a treatment option in these cases. METHODS: We encountered four cases of symptomatic cerebral ischemia in which the STA was absent or unavailable...
September 1, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/28870111/the-anatomical-variation-of-the-circulus-arteriosus-cerebri-in-a-cadaver-cohort-representing-the-population-dynamics-of-the-western-cape
#19
Karen Cilliers, Willie Vorster, Benedict John Page
The literature revealed a connection between the variations of the Circulus arteriosus cerebri (CAC) and cerebrovascular disease, ischemia, stroke, aneurysms and atherosclerosis. The diameters of the vessels forming the CAC have been classified into 22 types by previous authors. The aim of this study was to assess the variation of the CAC (diameter, length and anomalies) in a cadaver cohort representing the population of the Western Cape. Thirty-nine subjects (female n = 11, male n = 28) who had died of causes unrelated to brain trauma, were obtained from Stellenbosch University...
September 5, 2017: British Journal of Neurosurgery
https://www.readbyqxmd.com/read/28867155/the-boundaries-of-fetal-cardiac-intervention-expand-or-tighten
#20
REVIEW
Laura Gellis, Wayne Tworetzky
Fetal cardiac intervention (FCI) is a relatively new and continually evolving field, and, for select cardiac defects, offers the potential to alter the progression of the disease and improve outcomes. It is a procedure that requires a collaborative effort between maternal-fetal medicine, interventional cardiology and fetal echo/ultrasound specialists, as well as fetal and maternal anesthesiologists, nursing specialists, and social workers. This article reviews the most recently reported data and advances in FCI...
August 31, 2017: Seminars in Fetal & Neonatal Medicine
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