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Pediatric Hypoplastic Left Heart Syndrome

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Effects of levosimendan on ventriculo-arterial coupling and cardiac efficiency in paediatric patients with single-ventricle physiology after surgical palliation: retrospective study.

Roberta Iacobelli, Zaccaria Ricci, Eleonora Marinari, Roberta Haiberger, Arianna Di Molfetta, Alessandra Toscano, Luca Di Chiara,

OBJECTIVES:The use of levosimendan for paediatric patients with low cardiac output after congenital heart surgery has been recently described. We sought to evaluate ventriculo-arterial coupling (VAC) and other ventricular energetic parameters before and after 72 h from levosimendan start in infants with single-ventricle physiology and cardiac failure after palliation with Norwood ... Read more >>

Interact Cardiovasc Thorac Surg (Interactive cardiovascular and thoracic surgery)
[2020, 30(4):623-629]

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Interstage management of pulmonary blood flow after the Norwood procedure with right ventricle-to-pulmonary artery conduit.

Takashi Yasukawa, Takaya Hoashi, Masataka Kitano, Masatoshi Shimada, Kenta Imai, Kenichi Kurosaki, Hajime Ichikawa,

OBJECTIVES:Our goal was to assess the efficacy of managing pulmonary blood flow from the Norwood procedure with a right ventricle-to-pulmonary artery (RV-PA) conduit until stage 2 palliation (S2P). METHODS:Among 48 consecutive patients undergoing the Norwood procedure between 2008 and 2018, 40 (83.3%) patients who survived to discharge were included in ... Read more >>

Eur J Cardiothorac Surg (European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery)
[2020, :]

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Health-related quality of life for children and adolescents in school age with hypoplastic left heart syndrome: a single-centre study.

Raphael D Oberhuber, Sonja Huemer, Rudolf Mair, Eva Sames-Dolzer, Michaela Kreuzer, Gerald Tulzer,

Data from neurological and radiological research show an abnormal neurological development in patients treated for hypoplastic left heart syndrome. Thus, the aim of this study was to survey the quality of life scores in comparison with healthy children and children with other heart diseases (mild, moderate, and severe heart defects, ... Read more >>

Cardiol Young (Cardiology in the young)
[2020, :1-10]

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Does ascending aorta size affect Norwood outcomes in hypoplastic left heart with aortic atresia?

Horacio G Carvajal, Matthew W Canter, Aaron M Abarbanell, Pirooz Eghtesady,

BACKGROUND:Conflicting data exists regarding the impact of ascending aorta size on outcomes following the Norwood procedure. Results from multi-institutional studies have largely relied on heterogeneous populations undergoing this surgery for different anatomic defects. Using data from the Single Ventricle Reconstruction Trial, we analyzed the impact of preoperative ascending aortic diameter ... Read more >>

Ann. Thorac. Surg. (The Annals of thoracic surgery)
[2020, :]

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Procedural, pregnancy, and short-term outcomes after fetal aortic valvuloplasty.

Neil D Patel, Stephen Nageotte, Frank F Ing, Aimee K Armstrong, Ramen Chmait, Jon A Detterich, Alberto Galindo, Helena Gardiner, Sofia Grinenco, Ulrike Herberg, Edgar Jaeggi, Shaine A Morris, Dick Oepkes, John M Simpson, Anita Moon-Grady, Jay D Pruetz,

OBJECTIVES:We aimed to evaluate the effect of technical aspects of fetal aortic valvuloplasty (FAV) on procedural risks and pregnancy outcomes. BACKGROUND:FAV is performed in cases of severe mid-gestation aortic stenosis with the goal of preventing hypoplastic left heart syndrome (HLHS). METHODS:The International Fetal Cardiac Intervention Registry was queried for fetuses ... Read more >>

Catheter Cardiovasc Interv (Catheterization and cardiovascular interventions : official journal of the Society for Cardiac Angiography & Interventions)
[2020, :]

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Choice of shunt type for the Norwood I procedure: does it make a difference?

Keti Vitanova, Stanimir Georgiev, Rüdiger Lange, Julie Cleuziou,

OBJECTIVES:This study aimed to compare pulmonary artery (PA) growth between patients who received a right ventricle-to-PA (RV-PA) shunt and those who received a modified Blalock-Taussig shunt (mBTS). METHODS:All consecutive patients with hypoplastic left heart syndrome who underwent the Norwood I procedure between 2001 and 2017 were included in the study. ... Read more >>

Interact Cardiovasc Thorac Surg (Interactive cardiovascular and thoracic surgery)
[2020, 30(4):630-635]

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Necrotizing Enterocolitis and Associated Mortality in Neonates With Congenital Heart Disease: A Multi-Institutional Study.

Joseph A Spinner, Shaine A Morris, Deipanjan Nandi, Andrew T Costarino, Bradley S Marino, Joseph W Rossano, Pirouz Shamszad,

OBJECTIVE:There are scarce data about the prevalence and mortality of necrotizing enterocolitis in neonates with congenital heart disease. The purpose of this study is to provide a multi-institutional description and comparison of the overall prevalence and mortality of necrotizing enterocolitis in neonates with congenital heart disease. DESIGN:Retrospective multi-institutional study. SETTING:The ... Read more >>

Pediatr Crit Care Med (Pediatric critical care medicine : a journal of the Society of Critical Care Medicine and the World Federation of Pediatric Intensive and Critical Care Societies)
[2020, 21(3):228-234]

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Provider perspective on the role of palliative care in hypoplastic left heart syndrome.

Sarah Lowenstein, Robert Macauley, Kathleen Perko, Christina Ronai,

OBJECTIVE:Hypoplastic left heart syndrome is a single ventricle defect. While staged surgical palliative treatments have revolutionised care, patients with hypoplastic left heart syndrome continue to have significant morbidity and mortality. In 2017, the National Pediatric Cardiology Quality Improvement Collaborative recommended all single ventricle patients to receive a prenatal palliative care ... Read more >>

Cardiol Young (Cardiology in the young)
[2020, 30(3):377-382]

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Arch intervention following stage 1 palliation in hypoplastic left heart syndrome is associated with slower feed advancement: a report from the National Pediatric Quality Cardiology Improvement Collaborative.

Alicia H Chaves, Carissa M Baker-Smith, Geoffrey L Rosenthal,

INTRODUCTION:Infants undergoing stage 1 palliation for hypoplastic left heart syndrome may have post-operative feeding difficulties. Although the cause of feeding difficulties in these patients is multi-factorial, residual arch obstruction may affect gut perfusion, contributing to feeding intolerance. We hypothesised that undergoing arch reintervention following stage 1 palliation would be associated ... Read more >>

Cardiol Young (Cardiology in the young)
[2020, 30(3):396-401]

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Clinical Outcomes and Risk Factors for In-Hospital Mortality in Neonates with Hypoplastic Left Heart Syndrome.

Mohammed Hamzah, Hasan F Othman, Esraa Elsamny, Hemant Agarwal, Hany Aly,

The objective of this study was to identify patient and hospitalization characteristics associated with in-hospital mortality in infants with hypoplastic left heart syndrome (HLHS). We conducted a retrospective analysis of a large administrative database, the National Inpatient Sample dataset of the Healthcare Cost and Utilization Project for the years 2002-2016. Neonates ... Read more >>

Pediatr Cardiol (Pediatric cardiology)
[2020, :]

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Geometry of the pulmonary arteries before the Fontan operation: can we influence it during the Norwood procedure?

Katarzyna Januszewska, Pawel Nawrocki, Anja Lehner, Julia Stegger, Felix Kleinerueschkamp, Edward Malec,

OBJECTIVES:The right ventricle-to-pulmonary artery (RV-PA) shunt provides stable haemodynamics after the Norwood procedure but can influence development of the central pulmonary arteries (PAs). The goal of this study was to analyse the geometry of the central PAs in children with hypoplastic left heart syndrome before the Fontan operation with respect ... Read more >>

Eur J Cardiothorac Surg (European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery)
[2020, :]

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Tricuspid valve repair in children with hypoplastic left heart syndrome: impact of timing and mechanism on outcome.

Masamichi Ono, Benedikt Mayr, Melchior Burri, Nicole Piber, Christoph Röhlig, Martina Strbad, Julie Cleuziou, Alfred Hager, Jürgen Hörer, Rüdiger Lange,

OBJECTIVES:Our aim was to evaluate the results of tricuspid valve repair (TVr) in patients with hypoplastic left heart syndrome during staged reconstruction, focussing on the timing of the repair and the mechanisms of tricuspid regurgitation (TR). METHODS:Records of 44 children with hypoplastic left heart syndrome who underwent a total of ... Read more >>

Eur J Cardiothorac Surg (European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery)
[2020, :]

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Sinus Superflex DS© early collapse in systemic arterial duct.

Pedro Betrián-Blasco, Gerard Marti-Aguasca, Gemma Giralt-Garcia,

We implanted two sinus Superflex DS© stents in a systemic non-stenotic arterial duct of a newborn with hypoplastic left heart syndrome. Forty-eight hours later an important collapse of the stent was detected and treated with a balloon expandable stent. Sinus Superflex DS© has been specifically designed for systemic ducts. This ... Read more >>

Cardiol Young (Cardiology in the young)
[2020, 30(3):436-437]

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Beating heart thoracic aortic surgery under selective myocardial perfusion for patients with congenital aortic anomalies.

Tai Fuchigami, Masahiko Nishioka, Yutaka Tamashiro, Nobuhiro Nagata,

OBJECTIVE:We evaluated beating heart thoracic aortic surgery (BHTAS) using selective myocardial perfusion (SMP) in patients with aortic anomalies with complex surgical needs. METHODS:Between 2012 and 2018, 27 infants with aortic anomalies underwent BHTAS using SMP. RESULTS:Median body weight was 3.5 kg (range 2.6-5.2). In total, 15 cases of aortic coarctation, 7 ... Read more >>

Gen Thorac Cardiovasc Surg (General thoracic and cardiovascular surgery)
[2020, :]

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Survival, Neurocognitive, and Functional Outcomes After Completion of Staged Surgical Palliation in a Cohort of Patients With Hypoplastic Left Heart Syndrome.

Joseph Atallah, Gonzalo Garcia Guerra, Ari R Joffe, Gwen Y Bond, Sunjidatul Islam, M Florencia Ricci, Mohammed AlAklabi, Ivan M Rebeyka, Charlene M T Robertson, ,

Background Management of patients with hypoplastic left heart syndrome has benefited from advancements in medical and surgical care. Outcomes have improved, although survival and long-term functional and cognitive deficits remain a concern. Methods and Results This is a cohort study of all consecutive patients with hypoplastic left heart syndrome undergoing ... Read more >>

J Am Heart Assoc (Journal of the American Heart Association)
[2020, 9(4):e013632]

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Heart Failure in Single Right Ventricle Congenital Heart Disease: Physiologic and Molecular Considerations.

Anastacia M Garcia, Jonathan-Thomas Beatty, Stephanie Jialing Nakano,

Due to remarkable surgical and medical advances over the past several decades, there are growing numbers of infants and children living with single ventricle congenital heart disease (SV), where there is only one functional cardiac pumping chamber. Nevertheless, cardiac dysfunction and ultimately heart failure is a common complication in the ... Read more >>

Am. J. Physiol. Heart Circ. Physiol. (American journal of physiology. Heart and circulatory physiology)
[2020, :]

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Atrial septum stenting in a foetus with hypoplastic left heart syndrome and restrictive foramen ovale: an alternative to emergency atrioseptectomy in the newborn-a case report.

Andreas Tulzer, Wolfgang Arzt, Christoph Prandstetter, Gerald Tulzer,

Background:In patients with hypoplastic left heart syndrome (HLHS) premature closure or restriction of the interatrial communication causes severe cyanosis directly after birth with rapid deterioration in clinical state. An ex-utero intrapartum treatment (EXIT) procedure, extracorporal membrane oxygenation (ECMO), and emergency interventional cardiac catheterization or cardiac surgery has to be anticipated ... Read more >>

Eur Heart J Case Rep (European heart journal. Case reports)
[2020, 4(1):1-4]

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Surgical Repair of Peripheral Pulmonary Artery Stenosis in Patients Without Williams or Alagille Syndromes.

Elisabeth Martin, Richard D Mainwaring, R Thomas Collins, Kirstie L MacMillen, Frank L Hanley,

Peripheral pulmonary artery stenosis is a relatively rare form of congenital heart disease typically associated with genetic syndromes, such as Williams or Alagille syndromes. However, some patients present with severe stenosis without associated syndromes. The purpose of the study was to review our surgical experience in such patients. This was ... Read more >>

Semin. Thorac. Cardiovasc. Surg. (Seminars in thoracic and cardiovascular surgery)
[2020, :]

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Counseling for Prenatal Congenital Heart Disease-Recommendations Based on Empirical Assessment of Counseling Success.

Alexander Kovacevic, Andreas Simmelbauer, Sebastian Starystach, Michael Elsässer, Andreas Müller, Stefan Bär, Matthias Gorenflo,

Objectives: Empirical assessment of parental needs and affecting factors for counseling success after prenatal diagnosis of congenital heart disease (CHD). Methods:Counseling success after fetal diagnosis of CHD was assessed by a validated standardized questionnaire. The dependent variable "Effective Counseling" was measured in five created analytical dimensions (1. "Transfer of Medical ... Read more >>

Front Pediatr (Frontiers in Pediatrics)
[2020, 8:26]

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Variation in care for children undergoing the Fontan operation for hypoplastic left heart syndrome.

Aaron W Eckhauser, Maria I Van Rompay, Chitra Ravishankar, Jane W Newburger, S Ram Kumar, Christian Pizarro, Nancy Ghanayem, Felicia L Trachtenberg, Kristin M Burns, Garick D Hill, Andrew M Atz, Michelle S Hamstra, Mjaye Mazwi, Patsy Park, Marc E Richmond, Michael Wolf, Jeffrey D Zampi, Jeffrey P Jacobs, L LuAnn Minich, ,

BACKGROUND:The Single Ventricle Reconstruction Trial randomised neonates with hypoplastic left heart syndrome to a shunt strategy but otherwise retained standard of care. We aimed to describe centre-level practice variation at Fontan completion. METHODS:Centre-level data are reported as median or median frequency across all centres and range of medians or frequencies ... Read more >>

Cardiol Young (Cardiology in the young)
[2019, 29(12):1510-1516]

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Fontan-Associated Liver Disease: Spectrum of Disease in Children and Adolescents.

Steven L Rathgeber, Orlee R Guttman, Anna F Lee, Christine Voss, Nicole M Hemphill, Richard A Schreiber, Kevin C Harris,

Background Information is evolving on liver disease in pediatric patients with Fontan physiology. The purpose of this investigation is to evaluate the spectrum of liver disease in a pediatric population of patients with Fontan physiology and evaluate transient elastography (TE) as a noninvasive marker of liver disease. Methods and Results ... Read more >>

J Am Heart Assoc (Journal of the American Heart Association)
[2020, 9(1):e012529]

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Utility of Cervical Cannulation During Difficult Resternotomy in Congenital Heart Surgery.

Bahar Temur, Arda Davutoglu, Alper Dogruoz, Selim Aydin, Dilek Suzan, Barış Kırat, Ender Odemis, Ersin Erek,

BACKGROUND:Adhesions due to previous surgeries and some anatomical difficulties may make resternotomy dangerous in children. Femoral vessels are usually small and may not be suitable for cannulation. The aim of this report is to describe our experience with cervical cannulation during risky resternotomy in children. METHODS:Between January 2014 and January ... Read more >>

World J Pediatr Congenit Heart Surg (World journal for pediatric & congenital heart surgery)
[2020, 11(1):65-70]

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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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Recoarctation of the aorta after the Norwood procedure may be treated during the second stage of the surgical palliation.

Tomasz Mroczek, Magdalena Czerżyńska, Julita Sacharczuk, Rafał Żurek, Elżbieta Wójcik, Aleksandra Morka, Jacek Kuźma, Janusz H Skalski,

OBJECTIVES:Recoarctation of the aorta (re-CoA) after the Norwood procedure is traditionally treated during catheter-based aortoplasty (CB-A) performed as a separate procedure preceding stage II surgical palliation (S II SP). Our goal was to determine the efficacy of the protocol according to which re-CoA after the Norwood procedure in patients with ... Read more >>

Eur J Cardiothorac Surg (European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery)
[2019, 56(6):1186-1191]

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Prevalence of Sensorineural Hearing Loss in Children with Palliated or Repaired Congenital Heart Disease.

Lalitha Gopineti, Mane Paulpillai, Andrea Rosenquist, Andrew H Van Bergen,

Background Children with congenital heart disease (CHD) are at increased risk of neurodevelopmental deficits, and the presence of sensorineural hearing loss (SNHL) may further lead to poor language skills acquisition and speech delays. Prevalence of SNHL in the general pediatric population is estimated to be 0.2% at birth to 0.35% ... Read more >>

Cureus (Cureus)
[2020, 12(1):e6566]

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