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1

Rychik, Jack y Gil Wernovsky, eds. Hypoplastic Left Heart Syndrome. Boston, MA: Springer US, 2003. http://dx.doi.org/10.1007/978-1-4615-0253-1.

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2

1959-, Rychik Jack y Wernovsky Gil 1956-, eds. Hypoplastic left heart syndrome. Boston: Kluwer Academic Pub., 2003.

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3

Qiao, Bin, Zhong Min Liu, Yu Guo Weng y Ajit P. Yoganathan, eds. Surgical Atlas of Functional Single Ventricle and Hypoplastic Left Heart Syndrome. Singapore: Springer Singapore, 2018. http://dx.doi.org/10.1007/978-981-10-8435-5.

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4

Wernovsky, Gil y Jack Rychik. Hypoplastic Left Heart Syndrome. Springer London, Limited, 2012.

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5

Navaratnam, M. y C. Ramamoorthy. Hypoplastic Left Heart Syndrome. Editado por Kirk Lalwani, Ira Todd Cohen, Ellen Y. Choi y Vidya T. Raman. Oxford University Press, 2018. http://dx.doi.org/10.1093/med/9780190685157.003.0009.

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Approximately 960 babies are born per year in the United States with hypoplastic left heart syndrome. Over the last 20 years, advances in surgical techniques, perioperative care, cardiopulmonary bypass, and intensive care unit management have converted this previously fatal condition to one with a neonatal survival rate of 90% to 92% for standard risk patients. Understanding the factors affecting the balance of pulmonary blood flow and systemic blood flow and ensuring adequate cardiac output and end-organ perfusion is critical to successful outcomes. Extracorporeal membrane oxygenation remains an important support modality following stage I palliation. This chapter discusses this syndrome and describes treatment options.
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6

Hypoplastic Left Heart Syndrome. London: Springer-Verlag, 2005. http://dx.doi.org/10.1007/b138429.

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7

Wernovsky, Gil y Jack Rychik. Hypoplastic Left Heart Syndrome. Springer London, Limited, 2012.

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8

Anderson, Robert H., Marco Pozzi y Suzie Hutchinson. Hypoplastic Left Heart Syndrome. Springer London, Limited, 2005.

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9

Hennein, Hani A. y Edward L. Bove. Hypoplastic Left Heart Syndrome. Wiley & Sons, Incorporated, John, 2008.

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10

(Editor), Hani A. Hennein y Edward L. Bove (Editor), eds. Hypoplastic Left Heart Syndrome. Blackwell Publishing Limited, 2002.

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11

Anderson, Robert H., Marco Pozzi y Suzie Hutchinson. Hypoplastic Left Heart Syndrome. Springer, 2004.

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12

(Editor), Jack Rychik y Gil Wernovsky (Editor), eds. Hypoplastic Left Heart Syndrome (Developments in Cardiovascular Medicine). Springer, 2002.

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13

(Illustrator), Frank Jaworski, ed. Hypoplastic Left Heart Syndrome: A Handbook for Parents. Baby Hearts Press, 1997.

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14

Jaworski, Anna Marie. Hypoplastic Left Heart Syndrome: A Handbook for Parents. Baby Hearts Press, 1996.

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15

Yoganathan, Ajit P., Bin Qiao, Zhong Min Liu y Yu Guo Weng. Surgical Atlas of Functional Single Ventricle and Hypoplastic Left Heart Syndrome. Springer, 2019.

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16

Yoganathan, Ajit P., Bin Qiao, Zhong Min Liu y Yu Guo Weng. Surgical Atlas of Functional Single Ventricle and Hypoplastic Left Heart Syndrome. Springer, 2018.

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17

Archer, Nick y Nicky Manning. Left-sided abnormalities. Oxford University Press, 2018. http://dx.doi.org/10.1093/med/9780198766520.003.0010.

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This chapter explores left-sided abnormalities, discussing venoatrial abnormalities (including partial anomalous pulmonary venous drainage, total anomalous pulmonary venous drainage, and left-sided SVC), atrioventricular abnormalities (mitral atresia and mitral hypoplasia), ventriculoarterial abnormalities (including aortic stenosis, aortic atresia, and hypoplastic le. heart syndrome), and arterial abnormalities (coarctation of the aorta, interrupted aortic arch, right aortic arch, aberrant subclavian artery, double aortic arch, persistent fifth aortic arch, vascular rings, and aorto-pulmonary window).
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18

McKenzie, Ian. Single Ventricle Physiology. Oxford University Press, 2013. http://dx.doi.org/10.1093/med/9780199764495.003.0031.

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Congenital cardiac abnormalities in which there is functionally only a single ventricle are a heterogeneous group of conditions. These include patients with marked hypoplasia of one ventricle, usually with hypoplasia or atresia of the inflow of the ventricle, such as in hypoplastic left heart syndrome or conditions where surgical separation of the flow to each ventricle is not possible, such as double-inlet left ventricle. The most common pathway for palliating these conditions will be to use cavopulmonary connections to provide lung blood flow direct from systemic venous return (reliant on systemic venous pressure). The single ventricle pumps to the systemic arterial circulation. Many of these patients will be long-term survivors and present with acute surgical conditions unrelated to their cardiac condition. The safe anesthesia management of patients with single ventricle physiology and cavopulmonary connections involves assessing their cardiovascular reserve and understanding the effects of hypovolemia, anesthesia, positive-pressure ventilation, and the procedure itself on their circulation.
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19

Publications, ICON Health. Hypoplastic Left Heart Syndrome - A Medical Dictionary, Bibliography, and Annotated Research Guide to Internet References. ICON Health Publications, 2004.

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20

Mercado, Pilar, Jamey E. Eklund y Jennifer L. Anderson. Charge Syndrome. Editado por Kirk Lalwani, Ira Todd Cohen, Ellen Y. Choi y Vidya T. Raman. Oxford University Press, 2018. http://dx.doi.org/10.1093/med/9780190685157.003.0003.

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The major diagnostic features of CHARGE syndrome include coloboma of the eyes, choanal atresia or stenosis, distinctive external ears, cranial nerve abnormalities, and absent or small semicircular canals. The mnemonic refers to coloboma of the eye, heart defects, atresia of choanae, retardation of growth and development, cenitalia hypoplasia, and ear abnormalities and deafness. There is no defined etiology, though a de novo mutation on the CHD 7 gene located on Chromosome 8 is responsible for more than 50% of CHARGE cases. The incidence of CHARGE is about 1:10,000 live births with an equal distribution between males and females. The anesthetic implications of this syndrome are many and vary with the patient’s phenotype. A potential difficult airway, congenital heart defects, choanal atresia, and cranial nerve abnormalities present the most significant issues for the anesthesiologist. A multidisciplinary team must be established early to properly care for these complex patients.
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21

Firth, Helen V. y Jane A. Hurst. Pregnancy and fertility. Oxford University Press, 2017. http://dx.doi.org/10.1093/med/9780199557509.003.0006.

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This chapter describes various genetic disorders that can present in pregnancy. It discusses, among others: anterior abdominal wall defects, bowed limbs, club-foot, congenital diaphragmatic hernia, cytomegalovirus, Dandy–Walker malformation, the effects of drugs in pregnancies, fetal alcohol syndrome, fetal anticonvulsant syndrome, hyperechogenic bowel, hypoplastic left heart syndrome, low maternal serum oestriol, and the risks associated with advanced maternal and paternal age, together with many other conditions. For each of these, it gives a suggested approach to the clinical assessment, genetic advice and management, and support groups.
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22

Hraška, Viktor y Peter Murín. Surgical Management of Congenital Heart Disease II: Single Ventricle and Hypoplastic Left Heart Syndrome Aortic Arch Anomalies Septal Defects and ... of Thoracic Arteries and Veins A Video Manual. Springer, 2015.

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23

Hraska, Viktor y Peter Murín. Surgical Management of Congenital Heart Disease II: Single Ventricle and Hypoplastic Left Heart Syndrome Aortic Arch Anomalies Septal Defects and Anomalies in Pulmonary Venous Return Anomalies of Thoracic Arteries and Veins a Video Manual. Springer London, Limited, 2015.

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24

Yarlagadda, Vamsi V. y Ravi R. Thiagarajan. Cardiac Disease in Pediatric Intensive Care. Oxford University Press, 2017. http://dx.doi.org/10.1093/med/9780199918027.003.0007.

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This chapter on cardiac disease in pediatric intensive care provides essential information on cardiovascular physiology, how to assess cardiovascular and hemodynamic status, and principles of treatment of congenital and acquired cardiac disease in children. The review of physiology includes definitions of preload, afterload, oxygen content, cardiac output, vascular resistance, blood pressure, and cardiopulmonary interactions. Formulas to calculate key parameters are provided. The authors also summarize the presentation and care of most common cyanotic and acyanotic congenital heart defects, including treatment of low cardiac output syndrome, clinical sequelae of cardiopulmonary bypass, and the key aspects of treating pre- and postoperative patients with single-ventricle lesions (e.g., hypoplastic left heart syndrome). All three stages of single-ventricle palliation are discussed, with management summaries of children undergoing the Norwood, bidirectional Glenn, and Fontan operations. Finally, the chapter includes a discussion of the clinical presentation and management of viral myocarditis and cardiomyopathy.
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25

Hernandez, Michael R. Tracheoesophageal Fistula. Editado por Kirk Lalwani, Ira Todd Cohen, Ellen Y. Choi y Vidya T. Raman. Oxford University Press, 2018. http://dx.doi.org/10.1093/med/9780190685157.003.0017.

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Tracheoesophageal fistula (TEF) in the neonate is a complex congenital disorder that may occur in isolation or as part of a larger association of findings (i.e., VACTERL association). Care of these patients must include testing to clarify the anatomic and physiologic characteristics of each finding. This is particularly important for planning of surgical and anesthetic care. Surgical options for TEF repair vary in location of incision and also whether the approach is open or minimally invasive. Patients with severe congenital heart disease, such as hypoplastic left heart syndrome, pose unique challenges to the perioperative caregiver. The anesthesia team must balance the patient’s pulmonary and systemic blood flow while still heeding the need to avoid excessive ventilation via the TEF. Regional analgesia may provide the option of early extubation after TEF repair, but risks must be weighed against the patient’s anatomic and physiologic status.
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26

Basso, Cristina, José Maria Perèz-Pomares, Gaetano Thiene y Lucile Houyel. Coronary anomalies. Editado por José Maria Pérez-Pomares, Robert G. Kelly, Maurice van den Hoff, José Luis de la Pompa, David Sedmera, Cristina Basso y Deborah Henderson. Oxford University Press, 2018. http://dx.doi.org/10.1093/med/9780198757269.003.0025.

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Coronary artery anomalies occur either in isolation or in the context of congenital heart defects (CHD). Isolated coronary artery anomalies include anomalies of connection to the pulmonary artery or to the aorta, anomalies of the intrinsic coronary arterial anatomy including anomalous orifices, and anomalies of myocardial/coronary arterial interaction including myocardial bridges and fistulae. Such defects are of major significance in clinical cardiology and cardiac surgery because of their association with myocardial ischaemia and sudden death. Coronary anomalies associated with CHD can result from three types of developmental perturbation: (1) anomalous epicardial course (in congenitally corrected transposition of the great arteries and L-looped ventricles), (2) anomalous communication with a high-pressure ventricular cavity (pulmonary atresia with intact ventricular septum and hypoplastic left heart syndrome), or (3) anomalous connection to the aorta. Outflow tract defects represents 30–40% of CHD, and their main characteristic is great artery defects influencing coronary arterial anatomy.
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