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https://www.readbyqxmd.com/read/29035962/effectiveness-of-interstage-home-monitoring-on-healthcare-outcomes-in-infants-with-hypoplastic-left-heart-syndrome-a-systematic-review-protocol
#1
Chelsey Andries, Michelle Palokas
What is the effectiveness of home monitoring on interstage survival rates, weight gain, hospital admissions and cost savings in infants with hypoplastic left heart syndrome?
October 2017: JBI Database of Systematic Reviews and Implementation Reports
https://www.readbyqxmd.com/read/29016838/the-hand1-frameshift-a126fs-mutation-does-not-cause-hypoplastic-left-heart-syndrome-in-mice
#2
Beth A Firulli, Kevin P Toolan, Jade Harkin, Hannah Millar, Santiago Pineda, Anthony B Firulli
Aims: To test if a human Hand1 frame shift mutation identified in human samples is causative of hypoplastic left heart syndrome (HLHS). Methods and results: HLHS is a poorly understood single ventricle congenital heart defect that affects two to three infants in every 10 000 live births. The aetiologies of HLHS are largely unknown. The basic helix-loop-helix transcription factor HAND1 is required for normal heart development. Interrogation of HAND1 sequence from fixed HLHS tissues identified a somatic frame-shift mutation at Alanine 126 (NP_004812...
August 31, 2017: Cardiovascular Research
https://www.readbyqxmd.com/read/28993061/risk-comparison-for-prenatal-use-of-analgesics-and-selected-birth-defects-national-birth-defects-prevention-study-1997-2011
#3
Julia D Interrante, Elizabeth C Ailes, Jennifer N Lind, Marlene Anderka, Marcia L Feldkamp, Martha M Werler, Lockwood G Taylor, James Trinidad, Suzanne M Gilboa, Cheryl S Broussard
PURPOSE: To compare the use of nonsteroidal anti-inflammatory drugs (NSAIDs) and/or opioids to the use of acetaminophen without NSAIDs or opioids with respect to associations with birth defects. METHODS: We used data from the National Birth Defects Prevention Study (1997-2011). Exposure was self-reported maternal analgesic use from the month before through the third month of pregnancy (periconceptional). Adjusted odds ratios (aORs) were calculated to examine associations with 16 birth defects...
September 20, 2017: Annals of Epidemiology
https://www.readbyqxmd.com/read/28976608/cerebrovascular-response-to-maternal-hyperoxygenation-mh-in-fetuses-with-hypoplastic-left-heart-syndrome-hlhs-depends-upon-gestational-age-ga-and-baseline-cerebrovascular-resistance
#4
Anita Szwast, Mary Putt, J William Gaynor, Daniel Licht, Jack Rychik
OBJECTIVES: Compared to normal fetuses, HLHS fetuses have smaller brain volumes and more brain injury, possibly due to diminished cerebral blood flow and oxygen content. By increasing cerebral oxygen delivery, maternal hyperoxygenation (MH) might improve brain development and reduce brain injury in these fetuses. This study investigates whether gestational age (GA) and baseline cerebrovascular resistance impact response to MH in HLHS fetuses. METHODS: The middle cerebral artery (MCA) pulsatility index (PI), a surrogate measure of cerebrovascular resistance, was assessed in 43 HLHS fetuses...
October 4, 2017: Ultrasound in Obstetrics & Gynecology
https://www.readbyqxmd.com/read/28971557/prenatal-diagnosis-of-hypoplastic-left-heart-and-coarctation-of-the-aorta-with-color-doppler-fine
#5
L Yeo, R Romero
No abstract text is available yet for this article.
October 2017: Ultrasound in Obstetrics & Gynecology
https://www.readbyqxmd.com/read/28964428/surgical-interventions-in-infants-born-preterm-with-congenital-heart-defects-an-analysis-of-the-kids-inpatient-database
#6
Jagdish Desai, Sanjeev Aggarwal, Steven Lipshultz, Prashant Agarwal, Paulos Yigazu, Riddhiben Patel, Samantha Seals, Girija Natarajan
OBJECTIVE: To evaluate short-term outcomes in infants born preterm with congenital heart defects (CHDs) and the factors associated with surgery, survival, and length of hospitalization in this population. STUDY DESIGN: We analyzed data from infants born preterm (gestational age <37 weeks) enrolled in the multicenter Kids' Inpatient Database of the Healthcare Cost and Utilization Project who were admitted to the hospital within 30 days after birth. Infants with atrial septal defects were excluded...
September 27, 2017: Journal of Pediatrics
https://www.readbyqxmd.com/read/28960425/two-cases-of-prenatally-diagnosed-membranous-and-muscular-ventricular-septal-aneurysms
#7
Olga Ivanitskaya, Elena Andreeva, Natalia Odegova
A ventricular septal aneurysm is a rare heart defect located in the muscular or membranous part of the septum. Muscular ventricular septal aneurysms are usually isolated, with a favorable prognosis. Membranous ventricular septal aneurysms are often associated with other heart anomalies, could result in serious complications, and may require surgical treatment. We describe 2 cases of prenatally diagnosed ventricular septal aneurysms: an isolated membranous ventricular septal aneurysm with a good outcome, which was initially misdiagnosed as an atrioventricular septal defect; and a muscular ventricular septal aneurysm associated with a hypoplastic aortic arch and severe hydrocephaly, which resulted in termination of the pregnancy...
September 29, 2017: Journal of Ultrasound in Medicine: Official Journal of the American Institute of Ultrasound in Medicine
https://www.readbyqxmd.com/read/28957534/combining-computer-modelling-and-cardiac-imaging-to-understand-right-ventricular-pump-function
#8
John Walmsley, Wouter van Everdingen, Maarten J Cramer, Frits W Prinzen, Tammo Delhaas, Joost Lumens
Right ventricular (RV) dysfunction is a strong predictor of outcome in heart failure and is a key determinant of exercise capacity. Despite these crucial findings, the RV remains understudied in the clinical, experimental, and computer modelling literature. This review outlines how recent advances in using computer modelling and cardiac imaging synergistically help to understand RV function in health and disease. We begin by highlighting the complexity of interactions that make modelling the RV both challenging and necessary, and then summarize the multiscale modelling approaches used to date to simulate RV pump function in the context of these interactions...
October 1, 2017: Cardiovascular Research
https://www.readbyqxmd.com/read/28938963/study-design-and-rationale-for-elpis-a-phase-i-iib-randomized-pilot-study-of-allogeneic-human-mesenchymal-stem-cell-injection-in-patients-with-hypoplastic-left-heart-syndrome
#9
RANDOMIZED CONTROLLED TRIAL
Sunjay Kaushal, Brody Wehman, Nicholas Pietris, Casey Naughton, Soren M Bentzen, Grace Bigham, Rachana Mishra, Sudhish Sharma, Luca Vricella, Allen D Everett, Kristopher B Deatrick, Sihong Huang, Helina Mehta, William A Ravekes, Naru Hibino, Darcy L Difede, Aisha Khan, Joshua M Hare
Despite advances in surgical technique and postoperative care, long-term survival of children born with hypoplastic left heart syndrome (HLHS) remains limited, with cardiac transplantation as the only alternative for patients with failing single ventricle circulations. Maintenance of systemic right ventricular function is crucial for long-term survival, and interventions that improve ventricular function and avoid or defer transplantation in patients with HLHS are urgently needed. We hypothesize that the young myocardium of the HLHS patient is responsive to the biological cues delivered by bone marrow-derived mesenchymal stem cells (MSCs) to improve and preserve right ventricle function...
October 2017: American Heart Journal
https://www.readbyqxmd.com/read/28936753/long-term-results-of-bilateral-pulmonary-artery-banding-versus-primary-norwood-procedure
#10
Yasutaka Hirata, Hiroaki Miyata, Norimichi Hirahara, Arata Murakami, Hideaki Kado, Kisaburo Sakamoto, Shunji Sano, Shinichi Takamoto
Bilateral pulmonary artery banding (PAB) has emerged to be an attractive option as an initial procedure for the treatment of hypoplastic left heart syndrome (HLHS), and some centers report excellent survival. However, its usage is variable among institutions and its true efficacy is unknown due to reporting biases. We aimed to describe the results of bilateral PAB use, preoperative risk factors, and long-term outcomes compared with primary Norwood procedure, using a national database. Infants who underwent bilateral PAB or Norwood procedure as an initial palliation for HLHS between January 2008 and December 2012 listed in the Japan Congenital Cardiovascular Surgery Database (JCCVSD) were included...
September 21, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28928623/hypoplastic-left-heart-syndrome-with-coronary-cameral-fistulas-echocardiographic-demonstration-of-coronary-artery-steal-and-successful-interventional-treatment
#11
Arshid Mir, Mary Niu, Merick Yamada, Ed Overholt
We report a case of a patient with hypoplastic left heart syndrome with significant coronary-cameral fistulas and exertional symptoms from coronary steal. Symptoms resolved following successful coil occlusion of his left ventricle.
September 2017: Annals of Pediatric Cardiology
https://www.readbyqxmd.com/read/28924546/sirenomelia-associated-with-hypoplastic-left-heart-in-a-newborn
#12
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/28922269/extubation-failure-is-associated-with-increased-mortality-following-first-stage-single-ventricle-reconstruction-operation
#13
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
#14
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
#15
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
#16
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
#17
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/28893257/preoperative-single-ventricle-function-determines-early-outcome-after-second-stage-palliation-of-single-ventricle-heart
#18
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/28889833/acute-kidney-injury-following-first-stage-palliation-in-hypoplastic-left-heart-syndrome-hybrid-versus-norwood-palliation
#19
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
#20
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
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