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Neonatal heart surgery for HLHS

Jennifer M Lynch, Tiffany Ko, David R Busch, John J Newland, Madeline E Winters, Kobina Mensah-Brown, Timothy W Boorady, Rui Xiao, Susan C Nicolson, Lisa M Montenegro, J William Gaynor, Thomas L Spray, Arjun G Yodh, Maryam Y Naim, Daniel J Licht
BACKGROUND: Hypoxic-ischemic white matter brain injury commonly occurs in neonates with critical congenital heart disease. Recent work has shown that longer time to surgery is associated with increased risk for this injury. In this study we investigated changes in perinatal cerebral hemodynamics during the transition from fetal to neonatal circulation to ascertain mechanisms that might underlie this risk. METHODS: Neonates with either transposition of the great arteries (TGA) or hypoplastic left heart syndrome (HLHS) were recruited for preoperative noninvasive optical monitoring of cerebral oxygen saturation, cerebral oxygen extraction fraction, and cerebral blood flow using diffuse optical spectroscopy and diffuse correlation spectroscopy, 2 noninvasive optical techniques...
May 3, 2018: Journal of Thoracic and Cardiovascular Surgery
Chetan Ambastha, Gregory J Bittle, David Morales, Nathaniel Parchment, Progyaparamita Saha, Rachana Mishra, Sudhish Sharma, Alexander Vasilenko, Muthukumar Gunasekaran, Manal T Al-Suqi, Deqiang Li, Peixin Yang, Sunjay Kaushal
One of the most complex forms of congenital heart disease (CHD) involving single ventricle physiology is hypoplastic left heart syndrome (HLHS), characterized by underdevelopment of the left ventricle (LV), mitral and aortic valves, and narrowing of the ascending aorta. The underdeveloped LV is incapable of providing long-term systemic flow, and if left untreated, the condition is fatal. Current treatment for this condition consists of three consecutive staged palliative operations: the first is conducted within the first few weeks of birth, the second between 4 to 6 months, and the third and final surgery within the first 4 years...
April 2018: Translational Pediatrics
Buse Özer Bekmez, Evrim Alyamaç Dizdar, Nilüfer Okur, Mehmet Büyüktiryaki, Nurdan Uraş, Serife Suna Oğuz
BACKGROUND: Prenatal diagnosis ameliorates some preoperative and postoperative outcomes in critical congenital heart disease (CHD). Despite large variability among anatomical defect types, nearly half of them are diagnosed antenatally. We aimed to investigate the effect of the antenatal diagnosis on prereferral mortality of infants with critical CHD in a center without cardiovascular surgery clinic. METHODS: Medical records of the neonates who were diagnosed with critical CHD between the years 2010 and 2016 in Zekai Tahir Burak Women's health Education and Research Hospital were retrospectively reviewed for the study...
April 30, 2018: Journal of Maternal-fetal & Neonatal Medicine
Natasha L Lopez, Charitha Gowda, Carl H Backes, Deipanjan Nandi, Holly Miller-Tate, Samantha Fichtner, Robin Allen, Jamie Stewart, Clifford L Cua
INTRODUCTION: Neonates with hypoplastic left heart syndrome (HLHS) are at increased risk for necrotizing enterocolitis (NEC). Initial hospital outcomes are well described, but minimal midterm data exist. Goal of this study was to compare outcomes of HLHS infants with NEC (HLHS-NEC) to HLHS without NEC (HLHS-nNEC) during the interstage period. METHODS: Data were reviewed from 55 centers using the NPC-QIC database. Case-control study with one HLHS-NEC matched to HLHS-nNEC neonates in a 1:3 ratio based on institutional site, type of surgical repair, and gestational age ±1 week was performed...
March 9, 2018: Congenital Heart Disease
Marcin Gładki, Tomasz Składzień, Rafał Żurek, Elżbieta Broniatowska, Elżbieta Wójcik, Janusz H Skalski
Hypoplastic left heart syndrome (HLHS) is a congenital heart defect that requires 3-stage cardiac surgical treatment and multidirectional specialist care. The condition of newborns in the first postoperative days following the modified Norwood procedure is characterized by considerable hemodynamic instability that may result in a sudden cardiac arrest. It is believed that the most important cause of hemodynamic instability is the fluctuations in redistribution between pulmonary and systemic blood flow.The paper analyzes the postoperative course in 40 neonates with HLHS following the modified Norwood procedure performed under deep hypothermic cardiopulmonary bypass hospitalized in Cardiac Surgical Intensive Care Unit (CSICU) in the years 2014-2015...
August 2017: Medicine (Baltimore)
Diego A Lara, Mary K Ethen, Mark A Canfield, Wendy N Nembhard, Shaine A Morris
BACKGROUND: Hypoplastic left heart syndrome (HLHS) is strongly associated with Turner syndrome (TS); outcome data when these conditions coexist is sparse. We aimed to investigate long-term survival and causes of death in this population. METHODS: The Texas Birth Defects Registry was queried for all live born infants with HLHS during 1999-2007. We used Kaplan-Meier and Cox regression analyses to compare survival among patients with HLHS with TS (HLHS/TS+) to patients who had HLHS without genetic disorders or extracardiac birth defects (HLHS/TS-)...
January 2017: Congenital Heart Disease
Nikita M Patel, Mohamed A Mohamed, Iman K Yazdi, Ennio Tasciotti, Ravi K Birla
Current treatments in hypoplastic left heart syndrome (HLHS) include multiple surgeries to refunctionalize the right ventricle and/or transplant. The development of a tissue-engineered left ventricle (LV) would provide a therapeutic option to overcome the inefficiencies and limitations associated with current treatment options. This study provides a foundation for the development and fabrication of the bioengineered open ventricle (BEOV) model. BEOV molds were developed to emulate the human LV geometry; molds were used to produce chitosan scaffolds...
July 20, 2016: Journal of Biomedical Materials Research. Part B, Applied Biomaterials
Jae Kwang Yun, Ji Hyun Bang, Young Hwee Kim, Hyun Woo Goo, Jeong-Jun Park
Hypoplastic left heart complex (HLHC) consists of less severe underdevelopment of the left ventricle without intrinsic left valvular stenosis, i.e., a subset of hypoplastic left heart syndrome (HLHS). HLHC patients may be able to undergo biventricular repair, while HLHS requires single ventricle palliation (or transplant). However, there is no consensus regarding the likelihood of favorable outcomes in neonates with HLHC selected to undergo this surgical approach. This case report describes a neonate with HLHC, co-arctation of the aorta (CoA), and patent ductus arteriosus (PDA) who was initially palliated using bilateral pulmonary artery banding due to unstable ductus-dependent circulation...
April 2016: Korean Journal of Thoracic and Cardiovascular Surgery
Aline Wolter, Sina Nosbüsch, Andreea Kawecki, Jan Degenhardt, Christian Enzensberger, Oliver Graupner, Carina Vorisek, Hakan Akintürk, Can Yerebakan, Markus Khalil, Dietmar Schranz, Jochen Ritgen, Rüdiger Stressig, Roland Axt-Fliedner
OBJECTIVE: Functionally univentricular hearts (UVHs) represent cardiac anomalies in which either the pulmonary or systemic circulation cannot be supported independently. The purpose of our study was to determine perinatal outcomes after prenatal diagnosis of functionally UVH. METHODS: We retrospectively evaluated patients who presented between 2008 and June 2015 in our centre and in prenatal practice in Cologne. We included double inlet left ventricle (DILV), tricuspid valve atresia (TA), pulmonary valve atresia and intact ventricular septum (PA:IVS), unbalanced atrioventricular septal defect (AVSD), heterotaxy, hypoplastic left heart syndrome (HLHS) and hypoplastic left heart complex (HLHC)...
June 2016: Prenatal Diagnosis
H M Gardiner, A Kovacevic, G Tulzer, T Sarkola, U Herberg, J Dangel, A Öhman, J Bartrons, J S Carvalho, H Jicinska, V Fesslova, I Averiss, M Mellander
OBJECTIVES: Fetal aortic valvuloplasty (FV) aims to prevent fetal aortic valve stenosis progressing into hypoplastic left heart syndrome (HLHS), which results in postnatal univentricular (UV) circulation. Despite increasing numbers of FVs performed worldwide, the natural history of the disease in fetal life remains poorly defined. The primary aim of this study was to describe the natural history of fetal aortic stenosis, and a secondary aim was to test previously published criteria designed to identify cases of emerging HLHS with the potential for a biventricular (BV) outcome after FV...
September 2016: Ultrasound in Obstetrics & Gynecology
Jason M Kane, Jeff Canar, Valerie Kalinowski, Tricia J Johnson, K Sarah Hoehn
Without surgical treatment, neonatal hypoplastic left heart syndrome (HLHS) mortality in the first year of life exceeds 90 % and, in spite of improved surgical outcomes, many families still opt for non-surgical management. The purpose of this study was to investigate trends in neonatal HLHS management and to identify characteristics of patients who did not undergo surgical palliation. Neonates with HLHS were identified from a serial cross-sectional analysis using the Healthcare Cost and Utilization Project's Kids' Inpatient Database from 2000 to 2012...
February 2016: Pediatric Cardiology
Anas Taqatqa, Karim A Diab, Christopher Stuart, Louis Fogg, Michel Ilbawi, Sawsan Awad, Massimo Caputo, Zahid Amin, Ra-Id Abdulla, Damien Kenny, Ziyad M Hijazi
The hybrid approach to management of hypoplastic left heart syndrome (HLHS) was developed as an alternative to neonatal Norwood surgery, providing a less invasive initial palliation for HLHS. We describe our experience in extending the concept of the hybrid procedure to palliate neonates with anatomically compromised systemic arterial blood flow in a variety of congenital cardiac anomalies and supporting its application as first-line palliation in centers developing their HLHS programs. Retrospective review of patients undergoing therapy for HLHS at a single institution from June 2008 to December 2014 was performed...
March 2016: Pediatric Cardiology
Amira Soliman, Sanjeev Aggarwal, Girija Natarajan
OBJECTIVE: The aim of the study is to determine the number and indications of unplanned hospital readmissions and emergency room visits for children with hypoplastic left heart syndrome (HLHS) or transposition of great arteries (TGA). DESIGN: This retrospective chart review involved children with HLHS or TGA who underwent neonatal surgical repair in a single Children's Hospital. Statistical analysis (SPSS 22.0) included chi-square test, t-test, and binary logistic regression...
October 2015: American Journal of Perinatology
Sadia Malik, T Mac Bird, Robert D B Jaquiss, W Robert Morrow, James M Robbins
OBJECTIVES: The hybrid approach for the initial management of hypoplastic left heart syndrome shifts the risks of major open surgery from the vulnerable neonatal period to an older age. This study determined differences between the hybrid and the standard Norwood procedures in postoperative in-hospital mortality, renal failure, and survival to at least 2 years of age. METHODS: Data from the Pediatric Health Information System, a detailed hospital discharge database of 43 freestanding children's hospitals, were analyzed...
September 2015: Journal of Thoracic and Cardiovascular Surgery
Mohamed S Nassar, Srinivas A Narayan, Andrew Nyman, Caner Salih, Conal B Austin, David Anderson, Tarique Hussain
OBJECTIVE: Hybrid palliation for hypoplastic left heart syndrome has been developed as an alternative to neonatal Norwood surgery. At the second stage, a source of pulmonary blood flow has to be established. Options include an arterial modified Blalock-Taussig or a venous superior cavopulmonary shunt. METHODS: We retrospectively reviewed patients who received second-stage palliation after the initial hybrid. Patients were stratified according to the source of pulmonary blood supply into the arterial shunt (n = 17 patients) or venous shunt (n = 26 patients)...
August 2015: Journal of Thoracic and Cardiovascular Surgery
Shunsuke Matsushima, Yoshihiro Oshima, Ayako Maruo, Tomomi Hasegawa, Hironori Matsuhisa, Rei Noda, Ryuma Iwaki
The presence of ventriculocoronary connections in patients with hypoplastic left heart syndrome and mitral stenosis-aortic atresia is a suggested risk factor for myocardial ischemia after surgical palliation. We describe a neonate with this anatomic variant of hypoplastic left heart syndrome who underwent a successful beating-heart Norwood operation, with continuous coronary perfusion. The ventricular condition could be visually confirmed during the procedure, and a postoperative echocardiogram showed preserved ventricular function...
November 2014: Annals of Thoracic Surgery
Brett R Anderson, Adam J Ciarleglio, Arash Salavitabar, Alejandro Torres, Emile A Bacha
OBJECTIVES: Our aim was to examine the effects of surgical timing on major morbidity, mortality, and total hospital reimbursement for late preterm and term infants with hypoplastic left heart syndrome (HLHS) undergoing stage 1 palliation within the first 2 weeks of life. METHODS: We conducted a retrospective cohort study of infants aged ≥35 weeks gestation, with HLHS, admitted to our institution at age ≤5 days, between January 1, 2003, and January 1, 2013. Children with other cardiac abnormalities or other major comorbid conditions were excluded...
January 2015: Journal of Thoracic and Cardiovascular Surgery
Lindsay M Ryerson, Andrew S Mackie, Joseph Atallah, Ari R Joffe, Ivan M Rebeyka, David B Ross, Ian Adatia
OBJECTIVE: Infants and children who undergo cardiopulmonary bypass and cardiac surgery are at risk of postoperative fluid overload. Peritoneal dialysis catheter (PDC) and peritoneal dialysis are reported to be effective means of postoperative fluid management. We sought to test the hypothesis that PDC insertion in the operating room at the time of Norwood palliation would decrease the time to achieve a negative fluid balance in a group of neonates with hypoplastic left heart syndrome...
January 2015: Journal of Thoracic and Cardiovascular Surgery
Jennifer M Lynch, Erin M Buckley, Peter J Schwab, Ann L McCarthy, Madeline E Winters, David R Busch, Rui Xiao, Donna A Goff, Susan C Nicolson, Lisa M Montenegro, Stephanie Fuller, J William Gaynor, Thomas L Spray, Arjun G Yodh, Maryam Y Naim, Daniel J Licht
OBJECTIVE: Hypoxic-ischemic white mater brain injury commonly occurs in neonates with hypoplastic left heart syndrome (HLHS). Approximately one half of HLHS survivors will exhibit neurobehavioral symptoms believed to be associated with this injury, although the exact timing of the injury is unknown. METHODS: Neonates with HLHS were recruited for pre- and postoperative monitoring of cerebral oxygen saturation, cerebral oxygen extraction fraction, and cerebral blood flow using 2 noninvasive optical-based techniques: diffuse optical spectroscopy and diffuse correlation spectroscopy...
November 2014: Journal of Thoracic and Cardiovascular Surgery
S Bergonzini, A Mendoza, M A Paz, E Garcia, J M Aguilar, F G Arlati, L Galletti, J V Comas
Hypoplastic left heart syndrome is a spectrum of structural cardiac malformations characterized by variable underdevelopment of the left heart-aorta complex. A minority of patients having a milder degree of left ventricular hypoplasia, described as hypoplastic left heart complex (HLHC), may be selected for biventricular repair. The objective of this study was to assess the outcome of the biventricular approach in HLHC. We evaluated retrospectively 30 neonates diagnosed with HLHC from the "12 de Octubre" University Hospital, following established criteria...
February 2015: Pediatric Cardiology
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