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Yosuke Fukushima, Kenji Baba, Maiko Kondo, Yoshihiko Kurita, Takahiro Eitoku, Shigemitsu Yusuke, Kenta Hirai, Hirokazu Tsukahara, Tatsuo Iwasaki, Shingo Kasahara, Kotani Yasuhiro, Shinichi Otsuki
BACKGROUND: Rashkind balloon atrial septostomy (BAS) can be challenging in infants with hypoplastic left heart syndrome (HLHS) and small atrial septal defects (ASD). METHODS: We retrospectively reviewed all infants with HLHS who underwent surgery and BAS between January 2006 and December 2015. The infants were divided into three groups: no BAS, catheter BAS, and open BAS. Infants who underwent catheter BAS were divided into two groups based on atrial septal anatomy: standard and complex...
October 19, 2018: Pediatrics International: Official Journal of the Japan Pediatric Society
Brian H Morray, Erin L Albers, Thomas K Jones, Mariska S Kemna, Lester C Permut, Yuk M Law
BACKGROUND: The hybrid stage 1 palliation for hypoplastic left heart syndrome (HLHS) was first described in 1993 as a bridge to heart transplant for HLHS. There are limited data on this strategy as primary heart transplantation for HLHS has become less common. METHODS: This is an observational, single-center study comparing pre- and post-transplant outcomes of patients listed for transplant following hybrid palliation with those following surgical stage 1 palliation...
October 19, 2018: Pediatric Transplantation
James M Meza, Martijn Slieker, Eugene H Blackstone, Luc Mertens, William M DeCampli, James K Kirklin, Mohsen Karimi, Pirooz Eghtesady, Kamal Pourmoghadam, Richard W Kim, Phillip T Burch, Marshall L Jacobs, Tara Karamlou, Brian W McCrindle
BACKGROUND: Qualitative features of aortic and mitral valvar pathology have traditionally been used to classify congenital cardiac anomalies for which the left heart structures are unable to sustain adequate systemic cardiac output. We aimed to determine if novel groups of patients with greater clinical relevance could be defined within this population of patients with critical left heart obstruction (CLHO) using a data-driven approach based on both qualitative and quantitative echocardiographic measures...
October 2018: Computer Methods and Programs in Biomedicine
Ming Wei, Yuxiang Ma, Long Yin
INTRODUCTION: The endovascular treatment of intracranial atherosclerotic stenosis has become a common intervention. However, few reports are available regarding stenting performed for hypoplastic vertebral artery (HVA) with multiple atherosclerotic lesions. Combining multiple neuroendovascular devices provides more possibilities for technical success. We describe a stenting technique using a distal intracranial catheter (Navien) for providing an approach to an HVA with multiple existing atherosclerotic lesions...
October 2018: Medicine (Baltimore)
Hidetoshi Matsukawa, Hiroyasu Kamiyama, Kosumo Noda, Nakao Ota, Osamu Takahashi, Takaharu Shonai, Sadahisa Tokuda, Rokuya Tanikawa
The pathogenesis of basilar apex aneurysm (BAA) are still poorly understood. Embryologically, basilar apex anatomical disposition is formed by the fusion of both caudal internal carotid divisions on the midline. To compare basilar apex morphology by embryological classification among patients with BAAs, anterior circulation aneurysms (ACAs), and controls. Prospectively collected data of 47 consecutive patients with unruptured BAAs (42 females and five males), age- and gender-matched 47 patients with unruptured ACAs, and 47 controls without any aneurysms were analyzed...
October 13, 2018: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
Néstor F Sandoval, Catalina Vargas Acevedo, Juan Bernardo Umaña, Ivonne Pineda, Albert Guerrero, Carlos Obando, Juan P Umaña, Jaime Camacho
BACKGROUND: The approach to coarctation of the aorta with hypoplastic aortic arch is controversial. We evaluated the outcomes in patients with coarctation of the aorta with or without hypoplastic aortic arch operated through a posterior left lateral thoracotomy. METHODS: A retrospective cohort of patients with aortic coarctation, who underwent repair between January 2009 and October 2017, was analyzed. Preoperative, postoperative, and echocardiographic characteristics were reviewed...
November 2018: World Journal for Pediatric & Congenital Heart Surgery
Robert H Anderson, Rodney C G Franklin, Diane E Spicer
The world of pediatric cardiac surgery and cardiac surgery as a whole lost one of the great pioneers with the passing, at the beginning of 2018, of Francis Fontan. Hence to add to the recognition of his achievements, the European Congenital Heart Surgeons Association (ECHSA) has established a lecture to be given in his memory at their annual meetings. It was a significant honor and privilege to be invited to present the initial lecture. In this report, we describe the essence of the presentation. Many patients are now palliated by construction of the Fontan circulation...
November 2018: World Journal for Pediatric & Congenital Heart Surgery
Bulent Yazici, Nilay O Yuksel, Nuri Kologlu
Diprosopus is a rare variation of conjoined twinning. In this report, ophthalmic findings in an infant with diprosopus tetraophthalmos are presented. A male infant who was born at 33 weeks of gestation to a nonconsanguineous 42-year-old mother and 47-year-old father was examined for orbital abnormalities. The infant had a large head, 2 faces, and 4 eyes, of which 2 were placed laterally and 2 were fused in the midline. In the laterally placed eyes, the pupils were unresponsive to light, and the optic discs were hypoplastic...
October 9, 2018: Ophthalmic Plastic and Reconstructive Surgery
Muhammad Saad Ahmed, Sheeza Imtiaz, Muhammad Kashif Shazlee, Muhammad Ali, Junaid Iqbal, Rahila Usman
OBJECTIVE: To assess normal venous anatomy of the cranium and its anatomical variants. METHODS: This retrospective study was conducted at Radiology Department of Dr. Ziauddin Hospital, Karachi, and comprised data of patients aged 2-75 years and having undergone magnetic resonance imaging of brain from April 2015 to April 2016. Magnetic resonance venography was reviewed to evaluate the cerebral venous system. All magnetic resonance venography examinations were performed using a contiguous two-dimensional time-of-flight venography technique, and were reviewed by two consultant radiologists...
July 2018: JPMA. the Journal of the Pakistan Medical Association
Jamie N Colombo, Michael C Spaeder, Michael R Hainstock
OBJECTIVE: We aimed to investigate the incidence and causes of readmission of infants with single-ventricle and shunt-dependent biventricular CHD following routine, outpatient cardiac catheterisation. BACKGROUND: Cardiac catheterisation is commonly performed in patients with single-ventricle and shunt-dependent biventricular CHD for haemodynamic assessment and surgical planning. Best practices for post-procedural care in this population are unknown, and substantial variation exists between centres...
October 12, 2018: Cardiology in the Young
Alejandro Javier Torres
Atrial septal defect (ASD) is one of the most common congenital cardiac anomalies. ASD can present as an isolated lesion in an otherwise normal heart or in association with other congenital heart conditions. Regardless of the type of ASD, the direction and degree of shunting across the communication is mainly determined by the difference in compliance between the right and left ventricle. Hemodynamics in children is characterized by left-to-right shunting, dilated right heart structures and normal pulmonary artery pressures (PAP)...
September 2018: Journal of Thoracic Disease
David Cleveland, C Adam Banks, Hidetaka Hara, Waldemar F Carlo, David C Mauchley, David K C Cooper
Neonatal cardiac transplantation for hypoplastic left heart syndrome (HLHS) is associated with excellent long-term survival compared to older recipients. However, heart transplantation for neonates is greatly limited by the critical shortage of donor hearts, and by the associated mortality of the long pre-transplant waiting period. This led to the development of staged surgical palliation as the first-line surgical therapy for HLHS. Recent advances in genetic engineering and xenotransplantation have provided the potential to replicate the excellent results of neonatal cardiac allotransplantation while eliminating wait-list-associated mortality through genetically modified pig-to-human neonatal cardiac xenotransplantation...
October 9, 2018: Pediatric Cardiology
Marcin Gładki, Magdalena Czerżyńska, Aleksandra Morka, Julita Sacharczuk, Janusz H Skalski
No abstract text is available yet for this article.
2018: Postępy W Kardiologii Interwencyjnej, Advances in Interventional Cardiology
A T Hadidi
PURPOSE: Perineal hypospadias correction has swung from two-stage repair in the 1960s to one-stage repair in the 1980s and back to two-stage repair in the 2000s. The author's experience with a technique in which Chordee Excision and Distal Urethroplasty (CEDU) was performed at the first operation leaving a 1-cm segment as perineal urethrotomy to be reconstructed at the second operation is presented. PATIENTS AND METHODS: Between January 2013 and December 2016, the CEDU technique was performed in 63 patients with perineal hypospadias...
August 23, 2018: Journal of Pediatric Urology
S Raghunandhan, K Madhav, A Senthilvadivu, K Natarajan, M Kameswaran
INTRODUCTION: Paediatric Auditory Brainstem Implantation (ABI) is indicated for children with congenital cochlear aplasia, absent/hypoplastic vestibulocochlear nerve, for whom cochlear implantation is not possible. Knowledge of the anatomical landmarks and variants in anatomy of the brainstem is vital for ABI surgery. METHOD: Study was done at Auditory implant centre in Madras ENT research foundation, which includes 24 children who had undergone ABI surgery and are being followed up for 1 year, post operatively...
October 4, 2018: European Annals of Otorhinolaryngology, Head and Neck Diseases
Joseph T Mechak, Erika M Edwards, Kate A Morrow, Jonathan R Swanson, Jeffrey Vergales
Prematurity increases pre- and postoperative mortality in children with congenital heart disease. There are no large, multicentered, studies that have evaluated this relation specifically in neonates with hypoplastic left heart syndrome (HLHS). We sought to determine the impact of gestational age (GA) on survival to Stage 1 palliation surgery and hospital discharge in infants with HLHS. We reviewed data from 1,913 neonates with HLHS born at or transferred to a Vermont Oxford Network expanded member hospital in the United States from 2009 to 2014...
October 1, 2018: American Journal of Cardiology
Hyun Ho Choi, Young Dae Cho, Dong Hyun Yoo, Eung Koo Yeon, Jeongjun Lee, Su Hwan Lee, Hyun-Seung Kang, Won-Sang Cho, Jeong Eun Kim, Moon Hee Han
BACKGROUND: Posterior communicating artery (PcoA) compromise may serve as adjunctive treatment in patients with hypoplastic variants of PcoA who undergo coil embolization of PcoA aneurysms. However, procedural safety and the propensity for later recanalization are still unclear. OBJECTIVE: To evaluate clinical and radiologic outcomes of coil embolization in this setting, focusing on compromise of PcoA. METHODS: As a retrospective review, we examined 250 patients harboring 291 aneurysms of hypoplastic PcoAs, all consecutively treated by coil embolization between January 2004 and June 2016...
October 5, 2018: Journal of Neurointerventional Surgery
Andreas Giannakou, Robert J Sicko, Denise M Kay, Wei Zhang, Paul A Romitti, Michele Caggana, Gary M Shaw, Laura L Jelliffe-Pawlowski, James L Mills
Hypoplastic right heart syndrome (HRHS) is a rare congenital defect characterized by underdeveloped and malformed structures of the right heart. Familial recurrence of HRHS indicates genetic factors contribute to its etiology. Our study investigates the presence of copy number variants (CNVs) in HRHS cases. We genotyped 42 HRHS cases identified from live births throughout California (2003-2010) using the Illumina HumanOmni2.5-8 array. We identified 14 candidate CNVs in 14 HRHS cases (33%) based on the genes included in the CNVs and their functions...
October 5, 2018: American Journal of Medical Genetics. Part A
Jerry I Huang, Mary Kate Thayer, Michael Paczas, Stephen H Lacey, Daniel R Cooperman
PURPOSE: The hook of the hamate is an anatomical structure that separates the ulnar border of the carpal tunnel from Guyon's canal and serves as a landmark for surgeons. The hook of the hamate is also subject to fracture from injury. We hypothesize that there are variations in the hook of the hamate in the general population. METHODS: One thousand pairs of hamates (2,000 hamates) from the Hamann-Todd Collection at the Cleveland Natural History Museum were analyzed...
October 1, 2018: Journal of Hand Surgery
Zhishuo Z Ou, Sally Kochmar, Svetlana A Yatsenko, Audrey C Woerner, Roxanne Acquaro, Damara Ortiz, Urvashi Surti, Jie Hu
We describe a 5-month-old female who presented with clinical features of 5p deletion syndrome, including high-pitched cry, microcephaly, micrognathia, bilateral preauricular tags, bifid uvula, abnormal palmar creases, bilateral hypoplastic nipples, feeding difficulties, and developmental delay. In addition, the patient also had a cardiac defect, proximal esophageal atresia, and distal tracheoesophageal fistula. aCGH of the patient revealed a 22.9-Mb deletion of chromosome 5p15.33p14.3 and an 8.28-Mb duplication of chromosome 5q12...
October 5, 2018: Cytogenetic and Genome Research
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