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Hypoplastic left heart

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https://www.readbyqxmd.com/read/28410632/evolution-of-left-ventricular-size-in-late-survivors-of-surgery-for-hypoplastic-left-heart-syndrome
#1
Markus Bjurbom, Ajay J Iyengar, Florian Moenkemeyer, Igor E Konstantinov, Christian P Brizard, Yves d'Udekem
BACKGROUND: Aortic atresia and mitral atresia are predictors of adverse events in early life in patients with hypoplastic left heart syndrome, but it is unclear whether late outcomes are also affected, and whether this impact is related to a small hypertrophied left ventricle (LV) that impairs right ventricle (RV) function. METHODS: Thirty patients with hypoplastic left heart syndrome surviving with a Fontan procedure were identified. Follow-up echocardiograms were available at birth, before the Fontan procedure, and several years after...
April 12, 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28408455/cell-therapy-trials-in-congenital-heart-disease
#2
REVIEW
Hidemasa Oh
Dramatic evolution in medical and catheter interventions and complex surgeries to treat children with congenital heart disease (CHD) has led to a growing number of patients with a multitude of long-term complications associated with morbidity and mortality. Heart failure in patients with hypoplastic left heart syndrome predicated by functional single ventricle lesions is associated with an increase in CHD prevalence and remains a significant challenge. Pathophysiological mechanisms contributing to the progression of CHD, including single ventricle lesions and dilated cardiomyopathy, and adult heart disease may inevitably differ...
April 14, 2017: Circulation Research
https://www.readbyqxmd.com/read/28402400/unplanned-cardiac-reoperations-and-interventions-during-long-term-follow-up-after-the-norwood-procedure%C3%A2
#3
Eva Sames-Dolzer, Gregor Gierlinger, Michaela Kreuzer, Julia Schrempf, Roland Gitter, Christoph Prandstetter, Gerald Tulzer, Rudolf Mair
OBJECTIVES: Patients with hypoplastic left heart syndrome or related malformations are predominantly treated with a 3-stage palliation. Anatomic or physiologic problems can lead to unplanned additional surgical or catheter interventions during single ventricle palliation. Changes in operative technique may have an impact on the reoperation rate. METHODS: Between 1997 and 2014, 317 Norwood procedures were performed at our centre. A retrospective single centre investigation was carried out concerning incidence, timing, indication and type of unplanned interstage cardiac reoperations and catheter interventions during follow-up of Norwood patients...
April 11, 2017: European Journal of Cardio-thoracic Surgery
https://www.readbyqxmd.com/read/28393581/the-effect-of-transport-on-the-physiologic-stability-of-neonates-withductal-dependent-single-ventricle-lesions
#4
Silvestre R Duran, Sanjeev Aggarwal, Girija Natarajan
OBJECTIVE: To compare the status of infants with hypoplastic left heart syndrome (HLHS) or pulmonary atresia-hypoplastic right heart (PA-HRH) before and following transport using the validated Transport Risk Index of Physiologic Stability (TRIPS) score. METHODS: In this retrospective review of infants with HLHS or PA-HRH transported to a Children's Hospital by a pediatric transport team, an increase in TRIPS score (temperature, blood pressure, respiratory status, and response to stimuli) following transport was defined as deterioration...
April 10, 2017: Journal of Maternal-fetal & Neonatal Medicine
https://www.readbyqxmd.com/read/28371265/oral-facial-digital-syndrome-type-1-in-males-congenital-heart-defects-are-included-in-its-phenotypic-spectrum
#5
Arjan Bouman, Mariëlle Alders, Roelof Jan Oostra, Elisabeth van Leeuwen, Nikki Thuijs, Anne-Marie van der Kevie-Kersemaekers, Merel van Maarle
Oral-facial-digital syndrome type 1 (OFD1; OMIM# 311200) is an X-linked dominant ciliopathy caused by mutations in the OFD1 gene. This condition is characterized by facial anomalies and abnormalities of oral tissues, digits, brain, and kidneys. Almost all affected patients are female, as OFD1 is presumed to be lethal in males, mostly in the first or second trimester of pregnancy. Live born males with OFD1 are a rare occurrence, with only five reported patients to date. In four patients the presence of a congenital heart defect (CHD) was observed...
April 3, 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/28365652/interstage-home-monitoring-after-newborn-first-stage-palliation-for-hypoplastic-left-heart-syndrome-family-education-strategies
#6
Jo Ann Nieves, Karen Uzark, Nancy A Rudd, Jennifer Strawn, Anne Schmelzer, Nancy Dobrolet
Children born with hypoplastic left heart syndrome are at high risk for serious morbidity, growth failure, and mortality during the interstage period, which is the time from discharge home after first-stage hypoplastic left heart syndrome palliation until the second-stage surgical intervention. The single-ventricle circulatory physiology is complex, fragile, and potentially unstable. Multicenter initiatives have been successfully implemented to improve outcomes and optimize growth and survival during the interstage period...
April 2017: Critical Care Nurse
https://www.readbyqxmd.com/read/28359493/twin-to-twin-heart-transplantation-a-unique-event-with-a-25-year-follow-up
#7
David Blitzer, Grace Yedlicka, Joshua Manghelli, John Dentel, Randall Caldwell, John W Brown
Solid organ transplantation in pediatric patients has been a reality since 1954, when the first kidney transplantation was successfully performed between identical twins. We report the long-term outcomes, with more than 25 years of follow-up, in a patient born with hypoplastic left heart syndrome (HLHS) who received a heart transplant from a dizygotic twin. While we would not wish for this situation to reoccur, we hope that in reporting it, we can add to the discussion surrounding pediatric heart transplantation and the management of HLHS...
April 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28345115/counseling-practices-for-fetal-hypoplastic-left-heart-syndrome
#8
Michael J Walsh, George R Verghese, M Eric Ferguson, Nora F Fino, David J Goldberg, Sonal T Owens, Nelangi Pinto, Sinai C Zyblewski, Michael D Quartermain
While counseling parents of a fetus diagnosed with hypoplastic left heart syndrome (HLHS), pediatric cardiologists play a critical role in shaping a family's expectations for the months and years to come. However, techniques for the most effective counseling practices have not been studied, and significant variation among physicians is likely present. Web-based survey of pediatric cardiologists that perform fetal echocardiography using snowball sampling. 201 physicians responded (61% male, 81% from academic centers, and 95% from the U...
March 27, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28341901/hypoplastic-left-heart-syndrome-is-not-associated-with-worse-clinical-or-neurodevelopmental-outcomes-than-other-cardiac-pathologies-after-the-norwood-sano-operation
#9
Billie-Jean Martin, I De Villiers Jonker, Ari R Joffe, Gwen Y Bond, Bryan V Acton, David B Ross, Charlene M T Robertson, Ivan M Rebeyka, Joseph Atallah
There is evidence to suggest that patients undergoing a Norwood for non-HLHS anatomy may have lower mortality than classic HLHS, but differences in neurodevelopmental outcome have not been assessed. Our objective was to compare survival and neurodevelopmental outcome during the same surgical era in a large, well-described cohort. All subjects who underwent a Norwood-Sano operation between 2005 and 2014 were included. Follow-up clinical, neurological, and developmental data were obtained from the Western Canadian Complex Pediatric Therapies Follow-up Program database...
March 24, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28329229/isolated-severe-leftward-displacement-of-the-septum-primum-anatomic-and-3d-echocardiographic-findings-and-surgical-repair
#10
Fabio Cuttone, Khaled Hadeed, François Lacour-Gayet, Hugues Lucron, Sebastien Hascoet, Philippe Acar, Bertrand Leobon, Richard Van Praagh
OBJECTIVES: Leftward displacement of the septum primum is usually described as associated with hypoplastic left heart syndrome or visceral heterotaxy. This rare malformation results in partially or totally anomalous pulmonary venous drainage with a normal connection of the pulmonary veins to the left atrium, depending on the degree of septal shift. We report the 3D echocardiographic and anatomic findings as well as the surgical repair in a series of isolated severe leftward displacement of the septum primum, responsible for totally anomalous pulmonary venous drainage...
February 16, 2017: Interactive Cardiovascular and Thoracic Surgery
https://www.readbyqxmd.com/read/28329111/hypoplastic-left-heart-syndrome-a-novel-surgical-strategy-for-small-volume-centres
#11
Margaux Pontailler, Régis Gaudin, Marien Lenoir, Ayman Haydar, Diala Kraiche, Damien Bonnet, Pascal Vouhé, Olivier Raisky
OBJECTIVES: We describe in a prospective study, a novel surgical technique for the management of hypoplastic left heart syndrome inspired by the hybrid Norwood approach. METHODS: This new neonatal palliation comprises replacement of the patent ductus arteriosus (PDA) and aortic arch plasty with a pulmonary homograft associated with the banding of both pulmonary arteries and atrial septectomy, under cardiopulmonary bypass without aortic clamping and cardioplegia...
May 1, 2017: European Journal of Cardio-thoracic Surgery
https://www.readbyqxmd.com/read/28323386/the-association-of-maternal-lymphatic-markers-and-critical-congenital-heart-defects-in-the-fetus-a-population-based-case-control-study
#12
Martina A Steurer, Mary E Norton, Rebecca J Baer, Gary M Shaw, Sheila Keating, Anita J Moon-Grady, Christina D Chambers, Laura L Jelliffe-Pawlowski
The objective ot this study was to investigate whether lymphatic markers measured in women during the second trimester are associated with critical congenital heart defects (CCHDs) in offspring. This is a retrospective cohort study of pregnant women who participated in the California Prenatal Screening Program. CCHD data in the offspring was captured by linking birth certificate data with hospital patient discharge records. Second trimester samples were assayed for vascular endothelial growth factor (VEGF), platelet derived growth factor (PDGF) AA/BB, and PDGF AB...
March 21, 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/28314387/twin-pregnancy-complicated-by-esophageal-atresia-duodenal-atresia-gastric-perforation-and-hypoplastic-left-heart-structures-in-one-twin-a-case-report-and-review-of-the-literature
#13
Mohamad K Abou Chaar, Mariana L Meyers, Bethany D Tucker, Henry L Galan, Kenneth W Liechty, Timothy M Crombleholme, Ahmed I Marwan
BACKGROUND: The antenatal diagnosis of a combined esophageal atresia without tracheoesophageal fistula and duodenal atresia with or without gastric perforation is a rare occurrence. These diagnoses are difficult and can be suspected on ultrasound by nonspecific findings including a small stomach and polyhydramnios. Fetal magnetic resonance imaging adds significant anatomical detail and can aid in the diagnosis of these complicated cases. Upon an extensive literature review, there are no reports documenting these combined findings in a twin pregnancy...
March 18, 2017: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/28293976/evolution-of-hybrid-interventions-for-congenital-heart-disease
#14
REVIEW
Hitesh Agrawal, Wail Alkashkari, Damien Kenny
Surgical and transcatheter interventions have seen a tremendous evolution in last three decades. Hybrid technology combines the achievements of both disciplines to bring substantial hemodynamic benefit to patients with congenital heart disease (CHD) in a minimally invasive manner. This collaboration between surgeons and interventionalists will continue to grow as the technology evolves to meet the demands of CHD patients, potentially avoiding cardiopulmonary bypass and vascular access complications as well as optimizing immediate technical outcomes with exit angiography...
April 2017: Expert Review of Cardiovascular Therapy
https://www.readbyqxmd.com/read/28291606/geometry-and-growth-of-the-reconstructed-aorta-in-patients-with-hypoplastic-left-heart-syndrome-and-variants
#15
Christoph Haller, Devin Chetan, Arezou Saedi, Rachel Parker, Glen S Van Arsdell, Osami Honjo
OBJECTIVE: The interdigitating technique in aortic arch reconstruction in hypoplastic left heart syndrome and variants (HLHS) reduces the recoarctation rate. Little is known on aortic arch growth characteristics and resulting clinical impact. METHODS: A total of 139 patients with HLHS underwent staged palliation between 2007 and 2014; 73 patients underwent arch reconstruction. Dimensions of ascending aorta, transverse arch, interdigitating anastomosis, and descending aorta in pre-stage II and pre-Fontan angiograms were measured...
February 14, 2017: Journal of Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/28287055/congenital-pulmonary-lymphangiectasia-and-early-mortality-after-stage-1-reconstruction-procedures
#16
Jeremy L Herrmann, Mallory L Irons, Christopher E Mascio, Jack Rychik, Thomas L Spray, J William Gaynor, Jennifer E Pogoriler
OBJECTIVES: Pulmonary lymphangiectasia associated with hypoplastic left heart syndrome with an intact or restrictive atrial septum may result from increased left atrial pressure, and is associated with worse outcomes following staged reconstruction due to lung dysfunction and significant hypoxaemia. Our objective was to characterise the incidence of pulmonary lymphangiectasia in cases of early mortality following stage 1 reconstructions. METHODS: An institutional cardiac surgical database was retrospectively searched for patients who died within 30 days following a stage 1 reconstruction between 1 January, 1984 and 31 December, 2013...
March 13, 2017: Cardiology in the Young
https://www.readbyqxmd.com/read/28275592/model-of-human-fetal-growth-in-hypoplastic-left-heart-syndrome-reduced-ventricular-growth-due-to-decreased-ventricular-filling-and-altered-shape
#17
Sukriti Dewan, Adarsh Krishnamurthy, Devleena Kole, Giulia Conca, Roy Kerckhoffs, Michael D Puchalski, Jeffrey H Omens, Heather Sun, Vishal Nigam, Andrew D McCulloch
INTRODUCTION: Hypoplastic left heart syndrome (HLHS) is a congenital condition with an underdeveloped left ventricle (LV) that provides inadequate systemic blood flow postnatally. The development of HLHS is postulated to be due to altered biomechanical stimuli during gestation. Predicting LV size at birth using mid-gestation fetal echocardiography is a clinical challenge critical to prognostic counseling. HYPOTHESIS: We hypothesized that decreased ventricular filling in utero due to mitral stenosis may reduce LV growth in the fetal heart via mechanical growth signaling...
2017: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/28274521/cavopulmonary-anastomosis-during-same-hospitalization-as-stage-1-norwood-hybrid-palliative-surgery
#18
Sachin D Tadphale, Xinyu Tang, Nahed O ElHassan, Brandon Beam, Parthak Prodhan
BACKGROUND: Limited literature has examined characteristics of infants with hypoplastic left heart syndrome (HLHS) who remain hospitalized during the interstage period. We described their epidemiologic characteristics, in-hospital outcomes, and identified risk factors that predict the need for superior cavopulmonary anastomosis (SCPA) during the same hospitalization. METHODS: This retrospective multicenter database analysis included infants with HLHS who underwent stage 1 palliation from 2004 through 2013...
March 5, 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28272668/tricuspid-valve-hemangioma-associated-with-hypoplastic-left-heart-syndrome-presenting-as-sudden-infant-death-syndrome
#19
Brijnandan Gupta, Shouriyo Ghosh, Maikal Kujur, Khushbu Khetan, Tarun Kumar
Primary cardiac tumors are rare in children with a low incidence varying from 0.0017 to 0.28% in autopsy studies. Approximately 90% of the reported primary cardiac tumors in the pediatric population are benign and the most common subtype is rhabdomyomas accounting for approximately 60%, while hemangiomas are rare primary tumors with a 5% incidence. Hypoplastic left heart syndrome is abnormal development of the left-sided cardiac structures, leading to obstruction of blood flow from the left ventricle out-flow tract...
February 4, 2017: Türk Patoloji Dergisi
https://www.readbyqxmd.com/read/28262297/femoral-vein-homograft-as-right-ventricle-to-pulmonary-artery-conduit-in-stage-1-norwood-operation
#20
T K Susheel Kumar, Mario Briceno-Medina, Shyam Sathanandam, Vijaya M Joshi, Christopher J Knott-Craig
BACKGROUND: The polytetrafluoroethylene tube used as right ventricle to pulmonary artery conduit in the stage 1 Norwood operation is associated with risks of suboptimal branch pulmonary artery growth, thrombosis, free insufficiency, and long-term right ventricular dysfunction. Our experience with use of valved femoral vein homograft as right ventricle to pulmonary artery conduit is described. METHODS: Between June 2012 and December 2015, 15 neonates with hypoplastic left heart syndrome or complex single ventricle underwent stage 1 Norwood operation with valved segment of femoral vein homograft as right ventricle to pulmonary artery conduit...
March 2, 2017: Annals of Thoracic Surgery
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