Tetralogy of Fallot

Author(s):  
Renee Nierman Kreeger ◽  
James P Spaeth

Patients with congenital heart disease are frequently encountered by the pediatric anesthesiologist for non-cardiac surgery. Fortunately, the majority of these patients have already undergone definitive repair of their cardiac lesions and can often be managed using traditional anesthetic methods. However, given the known association of congenital heart disease with other congenital malformations and syndromes, there is a relatively high likelihood that a pediatric anesthesiologist will encounter a situation involving a child with an unrepaired lesion. With the reported increased mortality rate for patients with complex cardiac lesions undergoing non-cardiac procedures (see Chapter 30), an understanding of the pathophysiology and anatomy, as well as the potential effects of the anesthetic medications and techniques chosen, is paramount to their successful care.

Author(s):  
Michael Clifford

It is estimated that up to 1 million children in the United States have congenital heart disease (CHD). These children range from those who are essentially normal functionally with anatomically repaired hearts, and hence minimal impact for anesthesia, to those that have had complex and numerous surgical procedures with significant residual abnormalities in circulation and cardiac function, and a range of comorbidities. These latter children have many issues that will affect anesthesia for non-cardiac surgery. When presented with a child with CHD for non-cardiac surgery, the general pediatric anesthesiologist should be able to perform a tailored cardiac preoperative evaluation and plan an appropriate anesthetic with suitable anesthetic techniques, agents, and monitoring. Not every child with CHD has a single ventricle with all its complexity (see Chapter 31), but every child with CHD will offer challenges for the pediatric anesthesiologist.


2021 ◽  
Vol 11 (6) ◽  
pp. 562
Author(s):  
Olga María Diz ◽  
Rocio Toro ◽  
Sergi Cesar ◽  
Olga Gomez ◽  
Georgia Sarquella-Brugada ◽  
...  

Congenital heart disease is a group of pathologies characterized by structural malformations of the heart or great vessels. These alterations occur during the embryonic period and are the most frequently observed severe congenital malformations, the main cause of neonatal mortality due to malformation, and the second most frequent congenital malformations overall after malformations of the central nervous system. The severity of different types of congenital heart disease varies depending on the combination of associated anatomical defects. The causes of these malformations are usually considered multifactorial, but genetic variants play a key role. Currently, use of high-throughput genetic technologies allows identification of pathogenic aneuploidies, deletions/duplications of large segments, as well as rare single nucleotide variants. The high incidence of congenital heart disease as well as the associated complications makes it necessary to establish a diagnosis as early as possible to adopt the most appropriate measures in a personalized approach. In this review, we provide an exhaustive update of the genetic bases of the most frequent congenital heart diseases as well as other syndromes associated with congenital heart defects, and how genetic data can be translated to clinical practice in a personalized approach.


BMC Surgery ◽  
2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Xiaoqiang Yin ◽  
Mei Xin ◽  
Sheng Ding ◽  
Feng Gao ◽  
Fan Wu ◽  
...  

Abstract Background We aimed to explore the relationship between the neutrophil to lymphocyte ratio (NLR) and the early clinical outcomes in children with congenital heart disease (CHD) associated with pulmonary arterial hypertension (PAH) after cardiac surgery. Methods A retrospective observational study involving 190 children from January 2013 to August 2019 was conducted. Perioperative clinical and biochemical data were collected. Results We found that pre-operative NLR was significantly correlated with AST, STB, CR and UA (P < 0.05), while post-operative NLR was significantly correlated with ALT, AST, BUN (P < 0.05). Increased post-operative neutrophil count and NLR as well as decreased lymphocyte count could be observed after cardiac surgery (P < 0.05). Level of pre-operative NLR was significantly correlated with mechanical ventilation time, ICU stay time and total length of stay (P < 0.05), while level of post-operative NLR was only significantly correlated to the first two (P < 0.05). By using ROC curve analysis, relevant areas under the curve for predicting prolonged mechanical ventilation time beyond 24 h, 48 h and 72 h by NLR were statistically significant (P < 0.05). Conclusion For patients with CHD-PAH, NLR was closely related to early post-operative complications and clinical outcomes, and could act as a novel marker to predict the occurrence of prolonged mechanical ventilation.


2012 ◽  
Vol 43 (2) ◽  
pp. 344-351 ◽  
Author(s):  
Andrew Robert Harper ◽  
David Steven Crossland ◽  
Gianluigi Perri ◽  
John Jude O'Sullivan ◽  
Milind Pralhad Chaudhari ◽  
...  

2021 ◽  
Vol 5 (1) ◽  
Author(s):  
Dennis R. Delany ◽  
Stephanie S. Gaydos ◽  
Deborah A. Romeo ◽  
Heather T. Henderson ◽  
Kristi L. Fogg ◽  
...  

AbstractApproximately 50% of newborns with Down syndrome have congenital heart disease. Non-cardiac comorbidities may also be present. Many of the principles and strategies of perioperative evaluation and management for patients with congenital heart disease apply to those with Down syndrome. Nevertheless, careful planning for cardiac surgery is required, evaluating for both cardiac and noncardiac disease, with careful consideration of the risk for pulmonary hypertension. In this manuscript, for children with Down syndrome and hemodynamically significant congenital heart disease, we will summarize the epidemiology of heart defects that warrant intervention. We will review perioperative planning for this unique population, including anesthetic considerations, common postoperative issues, nutritional strategies, and discharge planning. Special considerations for single ventricle palliation and heart transplantation evaluation will also be discussed. Overall, the risk of mortality with cardiac surgery in pediatric patients with Down syndrome is no more than the general population, except for those with functional single ventricle heart defects. Underlying comorbidities may contribute to postoperative complications and increased length of stay. A strong understanding of cardiac and non-cardiac considerations in children with Down syndrome will help clinicians optimize perioperative care and long-term outcomes.


2017 ◽  
Vol 167 (11-12) ◽  
pp. 251-255
Author(s):  
Sascha Meyer ◽  
Martin Poryo ◽  
Mohammed Shatat ◽  
Ludwig Gortner ◽  
Hashim Abdul-Khaliq

2013 ◽  
Vol 61 (10) ◽  
pp. E446
Author(s):  
Mark J. Schuuring ◽  
David Koolbergen ◽  
Mark Hazekamp ◽  
Petr Symersky ◽  
Wim Lagrand ◽  
...  

2017 ◽  
Vol 8 (2) ◽  
pp. 182-188
Author(s):  
Madurra Perinpanayagam ◽  
Signe H. Larsen ◽  
Kristian Emmertsen ◽  
Marianne B. Møller ◽  
Vibeke E. Hjortdal

Background: Adults with congenital heart disease are a growing population. We describe surgical interventions, short- and long-term mortality and morbidity, and risk factors for adverse events in a population-based cohort. Methods: Patients over or equal to 18 years with congenital heart disease who underwent cardiac surgery at Aarhus University Hospital, Denmark, from 1994 to 2012 were included in the study. Diagnoses, surgical procedures, postoperative complications, and survival were identified in hospital databases, medical records, and the Danish Civil Registration System. Results: Four hundred seventy-four surgeries were performed in 445 adults (50% men). The median age was 39 years (range 18-83). Thirty-nine percent had previous surgical or catheter-based interventions. Thirty-day and in-hospital mortality were 1.1%. Postoperative complications occurred in 50% of cases, most were minor such as temporary arrhythmias and pneumonia. Major complications included postoperative bleeding necessitating intervention (6%), stroke (2%), and acute temporary renal failure (1%). Multivariate analysis identified RACHS-1 categories over or equal to 3 compared to category 1 (odds ratio (OR) = 2.3; 95% confidence interval (CI): 1.5-3.7), New York Heart Association functional class III and IV compared to class I (OR = 2.2; 95% CI: 1.3-3.7) and age at surgery (OR = 1.03, 95% CI: 1.01-1.04), as risk factors for adverse events. Survival during a median follow-up of 7.8 years (range 0 days-21.4 years) was 85% (95% CI: 80%-89%). Conclusion: Adults with congenital heart disease constitute a growing population with the need for cardiac surgery. Postoperative complications are frequent but early and late mortality are low.


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