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Chapter 49 - Long QT Syndrome
- from Section 7 - Miscellaneous Lesions and Syndromes
- Edited by Laura K. Berenstain, James P. Spaeth
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- Book:
- Congenital Cardiac Anesthesia
- Published online:
- 09 September 2021
- Print publication:
- 16 September 2021, pp 374-385
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Summary
Congenital long QT syndrome is a group of genetically transmitted disorders characterized by abnormal cardiac repolarization resulting in QT interval prolongation that predisposes patients to the acute onset of ventricular arrhythmias, most notably torsades de pointes, which may cause syncope or sudden cardiac death. Long QT syndrome is usually transmitted in an autosomal dominant pattern. Diagnosis remains challenging, as roughly 40% of patients with genotype-positive LQTS do not demonstrate QT prolongation on resting ECG. Clinical manifestations are heterogenous and include presyncope, syncope, aborted cardiac arrest, cardiac arrest, and sudden cardiac death but many patients are completely asymptomatic. Many of the medications administered during an anesthetic affect the QT interval. Additionally, patients with long QT syndrome may require the placement of pacemakers, implantable cardioverter-defibrillators and/or cardiovascular implantable electronic devices. This chapter discusses the perioperative management of patients with long QT syndrome and appropriate management of implanted devices during the perioperative period.
Risk factors for mortality in paediatric cardiac ICU patients managed with extracorporeal membrane oxygenation
- Barbara-Jo Achuff, Matthew D. Elias, Richard F. Ittenbach, Chitra Ravishankar, Susan C. Nicolson, Thomas L. Spray, Stephanie Fuller, J. William Gaynor, Matthew J. O’Connor
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- Journal:
- Cardiology in the Young / Volume 29 / Issue 1 / January 2019
- Published online by Cambridge University Press:
- 31 October 2018, pp. 40-47
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Background
Veno-arterial extracorporeal membrane oxygenation is frequently used in patients with cardiac disease. We evaluated short-term outcomes and identified factors associated with hospital mortality in cardiac patients supported with veno-arterial extracorporeal membrane oxygenation.
MethodsA retrospective review of patients supported with veno-arterial extracorporeal membrane oxygenation at a university-affiliated children’s hospital was performed.
ResultsA total of 253 patients with cardiac disease managed with extracorporeal membrane oxygenation were identified; survival to discharge was 48%, which significantly improved from 39% in an earlier era (1995–2001) (p=0.01). Patients were categorised into surgical versus non-surgical groups on the basis of whether they had undergone cardiac surgery before or not, respectively. The most common indication for extracorporeal membrane oxygenation was extracorporeal cardiopulmonary resuscitation: 96 (51%) in the surgical group and 45 (68%) in the non-surgical group. In a multiple covariate analysis, single-ventricle physiology (p=0.01), duration of extracorporeal membrane oxygenation (p<0.01), and length of hospital stay (p=0.03) were associated with hospital mortality. Weekend or night shift cannulation was associated with mortality in non-surgical patients (p=0.05).
ConclusionWe report improvement in survival compared with an earlier era in cardiac patients supported with extracorporeal membrane oxygenation. Single-ventricle physiology continues to negatively impact survival, along with evidence of organ dysfunction during extracorporeal membrane oxygenation, duration of extracorporeal membrane oxygenation, and length of stay.
Early postoperative remodelling following repair of tetralogy of Fallot utilising unsedated cardiac magnetic resonance: a pilot study
- Michael P. DiLorenzo, Elizabeth Goldmuntz, Susan C. Nicolson, Mark A. Fogel, Laura Mercer-Rosa
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- Journal:
- Cardiology in the Young / Volume 28 / Issue 5 / May 2018
- Published online by Cambridge University Press:
- 15 February 2018, pp. 697-701
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Introduction
The right ventricular adaptations early after surgery in infants with tetralogy of Fallot are important to understand the changes that occur later on in life; this physiology has not been fully delineated. We sought to assess early postoperative right ventricular remodelling in patients with tetralogy of Fallot by cardiac MRI.
Materials and methodSubjects with tetralogy of Fallot under 1 year of age were recruited following complete surgical repair for tetralogy of Fallot. Protocol-based cardiac MRI to assess anatomy, function, and flows was performed before hospital discharge using the feed and sleep technique, an unsedated imaging technique.
ResultsMRI was completed in 16 subjects at a median age of 77 days (interquartile range 114). There was normal ventricular ejection fraction and indexed right ventricular end-diastolic volume (48±13 cc/m2), but elevated right ventricular mass (z score 6.2±2.4). Subjects requiring a transannular patch or right ventricle to pulmonary artery conduit had moderate pulmonary insufficiency (regurgitant fraction 27±16%).
DiscussionEarly right ventricular remodelling after surgical repair for tetralogy of Fallot is characterised by significant pulmonary regurgitation, right ventricular hypertrophy, and lack of dilation. Performing cardiac MRI using the feed and sleep technique is feasible in infants younger than 5 months. These results might open new avenues to study longitudinal right ventricular changes in tetralogy of Fallot and to further explore the utility of unsedated MRI in patients with other types of CHDs.
A vascular endothelial growth factor A genetic variant is associated with improved ventricular function and transplant-free survival after surgery for non-syndromic CHD
- Constantine D. Mavroudis, Daniel Seung Kim, Nancy Burnham, Alexandra H. Morss, Jerry H. Kim, Amber A. Burt, David R. Crosslin, Donna M. McDonald-McGinn, Elaine H. Zackai, Meryl S. Cohen, Susan C. Nicolson, Thomas L. Spray, Ian B. Stanaway, Deborah A. Nickerson, Mark W. Russell, Hakon Hakonarson, Gail P. Jarvik, J. William Gaynor
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- Journal:
- Cardiology in the Young / Volume 28 / Issue 1 / January 2018
- Published online by Cambridge University Press:
- 20 September 2017, pp. 39-45
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Background
We have previously shown that the minor alleles of vascular endothelial growth factor A (VEGFA) single-nucleotide polymorphism rs833069 and superoxide dismutase 2 (SOD2) single-nucleotide polymorphism rs2758331 are both associated with improved transplant-free survival after surgery for CHD in infants, but the underlying mechanisms are unknown. We hypothesised that one or both of these minor alleles are associated with better systemic ventricular function, resulting in improved survival.
MethodsThis study is a follow-up analysis of 422 non-syndromic CHD patients who underwent neonatal cardiac surgery with cardiopulmonary bypass. Echocardiographic reports were reviewed. Systemic ventricular function was subjectively categorised as normal, or as mildly, moderately, or severely depressed. The change in function was calculated as the change from the preoperative study to the last available study. Stepwise linear regression, adjusting for covariates, was performed for the outcome of change in ventricular function. Model comparison was performed using Akaike’s information criterion. Only variables that improved the model prediction of change in systemic ventricular function were retained in the final model.
ResultsGenetic and echocardiographic data were available for 335/422 subjects (79%). Of them, 33 (9.9%) developed worse systemic ventricular function during a mean follow-up period of 13.5 years. After covariate adjustment, the presence of the VEGFA minor allele was associated with preserved ventricular function (p=0.011).
ConclusionsThese data support the hypothesis that the mechanism by which the VEGFA single-nucleotide polymorphism rs833069 minor allele improves survival may be the preservation of ventricular function. Further studies are needed to validate this genotype–phenotype association and to determine whether this mechanism is related to increased vascular endothelial growth factor production.
Improved early results with cavopulmonary connections
- Antonio R. Mott, Thomas L. Spray, J. William Gaynor, Rodolfo I. Godinez, Susan C. Nicolson, James M. Steven, William M. DeCampli, Gregory J. Schears, Gil Wernovsky
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- Journal:
- Cardiology in the Young / Volume 11 / Issue 1 / January 2001
- Published online by Cambridge University Press:
- 01 July 2011, pp. 3-11
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Background: We describe the recent results in a large cohort of patients with functionally single ventricle who underwent various modifications of cavopulmonary connections. Methods: Using the database at our institution, we identified all children who underwent cavopulmonary connection operations between June 1995 and June 1997. Demographic data, surgical history, and perioperative course were reviewed. Results: We performed 130 consecutive operations in 113 patients. The procedures included superior cavopulmonary connections in the form of the HemiFontan procedure in 45 instances, and bidirectional Glenn procedures in 11, and bilateral superior cavopulmonary connections in 7. The median age of these patients was 7.0 months. We completed Fontan operations using a fenestrated lateral tunnel on 47 occasions, and using an extracardiac conduit 9 times, 5 of which were fenestrated. A lateral tunnel without fenestration was constructed inone patient. The median age for these procedures was 19.5 months. In the remaining 10 instances, we revised Fontan procedures at a median age of 8 years. Diagnoses included hypoplastic left heart syndrome in 43 patients, double outlet right ventricle in 22, heterotaxy in 13, tricuspid atresia in 13, and a miscellaneous group accounting for the other 22. One death (0.7%) occurred within 30 days of surgery. Clinical seizures occurred in 7 children (5.3%), 6 had no residual neurologic deficits. Atrial pacing was needed in 14 children (10.7%) because of transient junctional rhythm, and 2 received treatment for supraventricular tachycardia. Pleural effusions were diagnosed radiographically after 31 of 130 (24%) procedures. Diuretic therapy resolved the effusion in 21 of these, with only 6 children requiring thoracostomy catheter drainage, and 4 undergoing thoracentesis alone. The median length of stay on the intensive care unit was 2 days, with a range from 1 to 30 days, and median stay in hospital was 6 days, with a range from 3 to 58 days. Conclusion: Mortality and perioperative morbidity after cavopulmonary connections have decreased dramatically in the current era. The long-term results of staged reconstruction for functionally single ventricle, nonetheless, await ongoing study.
Morbidity and mortality after surgery for congenital cardiac disease in the infant born with low weight
- Anne M. Ades MD, Troy E. Dominguez MD, Susan C. Nicolson MD, James W. Gaynor MD, Thomas L. Spray MD, Gil Wernovsky MD, Sarah Tabbutt, MD, PhD
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- Journal:
- Cardiology in the Young / Volume 20 / Issue 1 / February 2010
- Published online by Cambridge University Press:
- 18 December 2009, pp. 8-17
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Objective
Low weight at birth is a risk factor for increased mortality in infants undergoing surgery for congenitally malformed hearts. There has been a trend towards performing surgery in patients early, and for amenable lesions, in a single stage rather than following initial palliative procedures. Our goal was to report on the current incidences of morbidities and mortality in infants born with low weight and undergoing surgery for congenital cardiac disease.
MethodsWe made a retrospective review of the data from patients meeting our criterions for entry from July, 2000, through July, 2004. The criterions for inclusion were weight at birth less than or equal to 2500 grams, and congenital cardiac malformations requiring surgery during the initial hospitalization. A criterion for exclusion was isolated persistent patency of the arterial duct. We assessed preoperative, intraoperative, and postoperative variables.
ResultsWe found a total of 105 patients meeting the criterions for inclusion. The median weight at birth was 2130 grams, and median gestational age was 36 weeks. The most common morbidity identified was infections of the blood stream. Infections, and chronic lung disease, were associated with increased length of stay. Survival overall was 76%. Patients with hypoplastic left heart syndrome, or a variant thereof, had the lowest survival, of 62%. The needs for cardiopulmonary resuscitation, or extracorporeal membrane oxygenation, post-operatively were the only factors identified as independent risk factors for mortality.
ConclusionPatients undergoing surgery during infancy for congenital cardiac disease who are born with low weight have a higher mortality and morbidity than those born with normal weight.
Inhibition and restoration of hemostasis in the young cardiac surgical patient
- David R. Jobes, Susan C. Nicolson, James M. Steven
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- Journal:
- Cardiology in the Young / Volume 3 / Issue 4 / October 1993
- Published online by Cambridge University Press:
- 19 August 2008, pp. 370-377
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For forty years, heparin has been used to temporarily inhibit normal coagulation so as to permit the use of extracorporeal circulation. It has proved effective in preventing gross thrombosis and has not been compromised by changes in adjunctive practices (such as profound hypothermia; circulatory arrest), new pharmacological therapies (such as anesthetics or antibiotics), devices (such as membrane oxygenators), or during use in patients at the extremes of age. Unfortunately, heparin has been and continues to be inefficient in preserving hemostatic function during cardiopulmonary bypass resulting in undesirable bleeding after surgery.
Postoperative course in the cardiac intensive care unit following the first stage of Norwood reconstruction
- Gil Wernovsky, Marijn Kuijpers, Maaike C. Van Rossem, Bradley S. Marino, Chitra Ravishankar, Troy Dominguez, Rodolfo I. Godinez, Kathryn M. Dodds, Richard F. Ittenbach, Susan C. Nicolson, Geoffrey L. Bird, J. William Gaynor, Thomas L. Spray, Sarah Tabbutt
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- Journal:
- Cardiology in the Young / Volume 17 / Issue 6 / December 2007
- Published online by Cambridge University Press:
- 07 November 2007, pp. 652-665
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The medical records of all patients born between 1 September, 2000, and 31 August, 2002, and undergoing the first stage of Norwood reconstruction, were retrospectively reviewed for details of the perioperative course. We found 99 consecutive patients who met the criterions for inclusion. Hospital mortality for the entire cohort was 15.2%, but was 7.3%, with 4 of 55 dying, in the setting of a “standard” risk profile, as opposed to 25.0% for those with a “high” risk profile, 11 of 44 patients dying in this group. Extracorporeal membrane oxygenation was utilized in 7 patients, with 6 deaths. Median postoperative length of stay in the hospital was 14 days, with a range from 2 to 85 days, and stay in the cardiac intensive care unit was 11 days, with a range from 2 to 85 days. Delayed sternal closure was performed in 18.2%, with a median of 1 day until closure, with a range from zero to 5 days. Excluding isolated delayed sternal closure, and cannulation and decannulation for extracorporeal support, 24 patients underwent 33 cardiothoracic reoperations, including exploration for bleeding in 12, diaphragmatic plication in 4; shunt revision in 4, and other procedures in 13. The median duration of total mechanical ventilation was 4.0 days, with a range from 0.7 to 80.5 days. Excluding those who died, the median total duration of mechanical ventilation was 3.8 days, with a range from 0.9 to 46.3 days. Reintubation for cardiorespiratory failure or upper airway obstruction was performed in 31 patients. Postoperative electroencephalographic and/or clinical seizures occurred in 13 patients, with 7 discharged on anti-convulsant medications. Postoperative renal failure, defined as a level of creatinine greater than 1.5 mg/dl, was present in 13 patients. Eleven had significant thrombocytopenia, with fewer than 20,000 platelets per μl, and injury to the vocal cords was identified in eight patients. Risk factors for longer length of stay included lower Apgar scores, preoperative intubation, early reoperations, reintubation and sepsis, but not weight at birth, genetic syndromes, the specific surgeon, or the duration of surgery.
Although mortality rates after the first stage of reconstruction continue to fall, the course in the intensive care unit is remarkable for significant morbidity, especially involving the cardiac, pulmonary and central nervous systems. These patients utilize significant resources during the first hospitalization. Further studies are necessary to stratify the risks faced by patients with hypoplasia of the left heart in whom the first stage of Norwood reconstruction is planned, to determine methods to reduce perioperative morbidity, and to determine the long-term implications of short-term complications, such as diaphragmatic paresis, injury to the vocal cords, prolonged mechanical ventilation, and postoperative seizures.
Haemodynamic changes during modified ultrafiltration immediately following the first stage of the Norwood reconstruction
- J. William Gaynor, Marijn Kuypers, Maaike van Rossem, Gil Wernovsky, Bradley S. Marino, Sarah Tabbutt, Susan C. Nicolson, Thomas L. Spray
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- Journal:
- Cardiology in the Young / Volume 15 / Issue 1 / January 2005
- Published online by Cambridge University Press:
- 14 April 2005, pp. 4-7
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Background: Modified ultrafiltration has been shown to reverse haemodilution and improve ventricular function following cardiopulmonary bypass. There has been concern, however, about the safety and efficacy of modified ultrafiltration after the first stage of Norwood reconstruction for palliation of neonates with hypoplasia of the left heart and its variants. Methods: We reviewed the intraoperative course of all patients undergoing the first stage of Norwood reconstruction between September 1, 2000, and August 31, 2002. Results: The first stage of reconstruction was performed in 99 neonates, 78 with classical hypoplasia of the left heart, and 21 with variants. Mean weight at surgery was 3.1 plus or minus 0.7 kilograms. Genetic syndromes, weight less than or equal to 2.5 kilograms, and/or major additional cardiac or non-cardiac anomalies, were present in 44 patients. We deemed these patients to constitute the group at high risk. A modified Blalock–Taussig shunt was utilized in 95 patients, and a conduit from the right ventricle to the pulmonary arteries in 4. Deep hypothermic circulatory arrest was used in all patients for a mean period of 45 minutes, plus or minus 15 minutes. Total support time on cardiopulmonary bypass plus deep hypothermic circulatory arrest was 100 minutes plus or minus 26 minutes. Modified ultrafiltration was performed in all patients. The mean duration of modified ultrafiltration was 10 plus or minus 2 minutes, and the total volume of filtrate removed was 104 plus or minus 29 millilitres per kilogram. There were no complications from modified ultrafiltration, and no patient required discontinuation of modified ultrafiltration for haemodynamic instability. During modified ultrafiltration, the haematocrit increased from 31 percent plus or minus 4 to 46 percent plus or minus 6. Heart rate decreased from 170 plus or minus 17 beats per minute to 158 plus or minus 16 beats per minute. Systolic blood pressure increased from 57 plus or minus 12 to 63 plus or minus 13 millimetres of mercury, and diastolic blood pressure from 30 plus or minus 8 to 35 plus or minus 7 millimetres of mercury. All these values are significant at a p value of less than 0.0001. Hospital morality in the patients at low risk was 3 of 55 (5.5 percent), but was 12 of 44 (27.3 percent) in the patients deemed to be at high-risk. Conclusions: Modified ultrafiltration is safe procedure following the first stage of Norwood reconstruction, with improvement in all haemodynamic parameters measured. Modified ultrafiltration is an additional incremental strategy, which may contribute to the overall improvement in outcome following surgical palliation of patients with hypoplasia of the left heart or its variants.