Operative repair of an atrioventricular septal defect is not without risk. The purpose of this review is to highlight various key topics in the perioperative treatment of patients with atrioventricular septal defects, including challenges related to Down syndrome, postoperative arrhythmias, pulmonary hypertension, hypothyroidism, postoperative residual and recurrent lesions, including systemic atrioventricular valvar regurgitation and left ventricular outflow tract obstruction, sedation and analgesia, and vascular access.
JacobsJPBurkeRPQuintessenzaJAMavroudisC. Congenital Heart Surgery Nomenclature and Database Project: atrioventricular canal defect. Ann Thorac Surg. 2000;69(4 suppl):S36–S43.
2.
AndersonRHWesselsAVettukattilJJ. The morphology and morphogenesis of atrioventricular septal defect with common atrioventricular junction. World Journal for Pediatric and Congenital Heart Surgery. 2010,1(1).
3.
JacobsJPJacobsMLMavroudisC. Atrioventricular septal defects: lessons learned about practice patterns and outcomes from the congenital heart surgery database of the Society of Thoracic Surgeons. World Journal for Pediatric and Congenital Heart Surgery. 2010,1(1).
4.
MorrisCDMagilkeDRellerM. Down’s syndrome affects results of surgical correction of complete atrioventricular canal. Pediatr Cardiol. 1992;13:80–84.
5.
VisJCDuffelsMGWinterMM. Down syndrome: a cardiovascular perspective. J Intellect Disabil Res. 2009;53:419–425.
6.
JonesKL. Smith’s Recognizable Patterns of Human Malformation 2005. P: Elsevier Saunders; 2005.
7.
LoukanovTSebeningCSpringerWUlmerHHaglS. Simultaneous management of congenital tracheal stenosis and cardiac anomalies in infants. J Thorac Cardiovasc Surg. 2005; 130:1537–1541.
8.
American Academy of Pediatrics Committee on Genetics. Health supervision for children with Down syndrome. Pediatrics. 2001;107:442–449.
9.
SuzukiTBoveELDevaneyEJ. Results of definitive repair of complete atrioventricular septal defect in neonates and infants. Ann Thorac Surg. 2008;86:596–602.
10.
LindbergLOlssonAKJogiPJonmarkerC. How common is severe pulmonary hypertension after pediatric cardiac surgery?J Thorac Cardiovasc Surg. 2002;123:1155–1163.
11.
DelaneyJWMoltedoJMDziuraJDKopfGSSnyderCS. Early postoperative arrhythmias after pediatric cardiac surgery. J Thorac Cardiovasc Surg. 2006;131:1296–1301.
12.
Dodge-KhatamiAMillerOIAndersonRH. Surgical substrates of postoperative junctional ectopic tachycardia in congenital heart defects. J Thorac Cardiovasc Surg. 2002;123:624–630.
13.
HoffmanTMWernovskyGWieandTS. The incidence of arrhythmias in a pediatric cardiac intensive care unit. Pediatr Cardiol. 2002;23:598–604.
14.
HaasNACamphausenCK. Impact of early and standardized treatment with amiodarone on therapeutic success and outcome in pediatric patients with postoperative tachyarrhythmia. J Thorac Cardiovasc Surg. 2008;136:1215–1222.
WeindlingS. Duration of complete atrioventricular block after congenital heart disease surgery. Am J Cardiol. 1998;82:525–527.
17.
BandoKTurrentineMWSharpTG. Pulmonary hypertension after operations for congenital heart disease: analysis of risk factors and management. J Thorac Cardiovasc Surg. 2002;123:1155–1163.
18.
LakeCLBookerPD.
Pediatric Cardiac Anesthesia.New York, NY: Lippincott Williams & Wilkins; 2004.
CannonBCFeltesTFFraleyJK. Nitric oxide in the evaluation of congenital heart disease with pulmonary hypertension: factors related to nitric oxide response. Pediatr Cardiol. 2005; 26:565–569.
21.
JournoisDBaufretonCMauriatP. Effects of inhales nitric oxide administration on early postoperative mortality in patients operated for correction of atrioventricular canal defects. Chest. 2005;128:3537–3544.
22.
MillerOITangSFKeechA. Inhaled nitric oxide and prevention of pulmonary hypertension after congenital heart surgery: a randomised double-blind study. Lancet. 2000;356:1464–1469.
23.
NamachivayamPTheilenUButtWWCooperSMPennyDJShekerdemianLS. Sildenafil prevents rebound pulmonary hypertension after withdrawal of nitric oxide in children. Am J Respir Crit Care Med. 2006;174:1042–1047.
24.
RubelloDPozzanGBCasaraD. Natural course of subclinical hypothyroidism in Down’s syndrome: prospective study results and therapeutic considerations. J Endocrinol Invest. 1995;17:35–40.
25.
KarlssonBGustafssonJHedovGIvarssonS-AAnnerenG. Thyroid dysfunction in Down’s syndrome: relation to age and thyroid autoimmunity. Arch Dis Child. 1998;79:242–245.
26.
BettendorfMSchmidtKGTiefenbacherU. Transient secondary hypothyroidism in children after cardiac surgery. Pediatr Res. 1997;41:375–379.
27.
DimmickSJBadawiNRandellT. Thyroid hormone supplementation for the prevention of morbidity in infants undergoing cardiac surgery. Cochrane Database Syst Rev. 2004;3:CD004220.
28.
ChowdhuryDOjamaaKParnellVA. A prospective randomized clinical study of thyroid hormone treatment after operations for complex congenital heart disease. J Thorac Cardiovasc Surg. 2001;122:1023–1025.
29.
BettendorfMSchmidtKGGrulich-HennJUlnerHEHeinrichUE. Tri-iodothyronine treatment in children after cardiac surgery: a double blind, randomized, placebo-controlled study. Lancet. 2000;356:529–534.
30.
BiondiBFazioSPalmieriEACarellaC. Left ventricular diastolic dysfunction in patients with subclinical hypothyroidism. J Clin Endocrin Metab. 1999;84:2064–2067.
31.
DiBelloVMonazniFGiorgiD. Ultrasonic myocardial textural analysis in subclinical hypothyroidism. J Am Soc Echocardiogr. 2000;13:832–840.
32.
KahalyGJ. Cardiovascular and atherogenic aspects of subclinical hypothyroidism. Thyroid. 2000;10:665–679.
33.
ToscanoEPacileoGLimongelliG. Subclinical hypothyroidism and Down’s syndrome: studies on myocardial structure and function. Arch Dis Child. 2003;88:1005–1008.
34.
RosenfeldHMGentlesTLWernovskyG. Utility of intraoperative transesophageal echocardiography in the assessment of residual cardiac defects. Pediatr Cardiol. 1998; 19:346–351.
35.
HonioOKotaniYOsakiS. Discrepancy between intraoperative transesophageal echocardiography and postoperative transthoracic echocardiography in assessing congenital valve surgery. Ann Thorac Surg. 2006;82:2240–2246.
36.
MalhotraSPLacour-GayetFMitchellMB. Reoperation for left atrioventricular valve regurgitation after atrioventricular septal defect repair. Ann Thorac Surg. 2008;86:147–152.
37.
ChowdhuryUKAiranBMalhotraA. Specific issues after surgical repair of partial atrioventricular septal defect: actuarial survival, freedom from reoperation, fate of the left atrioventricular valve, prevalence of left ventricular outflow tract obstruction, and other events. J Thorac Cardiovasc Surg. 2009;137:548–555.
38.
MommaK. ACE inhibitors in pediatric patients with heart failure. Pediatr Drugs. 2006;8:5–69.
39.
PisacaneCPacileoGSantoroG. New insights in the pathophysiology of mitral and aortic regurgitation in pediatric age: role of angiotensive-converting enzyme inhibitor therapy. Ital Heart J. 2001;2:100–106.
40.
GillespieMJMarinoBSCohenMS. Risk factors for adverse outcomes after surgery on the systemic atrioventricular valve in 109 children. Cardiol Young. 2006;16:35–42.
41.
MafricaFFodaleV. Opioids and Down’s syndrome. J Opioid Manag. 2006;2:93–98.
42.
BejianSValasekCNigroJJClevelandDCWillisBC. Prolonged use of dexmedetomidine in the paediatric cardiothoracic intensive care unit. Cardiol Young. 2009;19:98–104.
43.
ChrysostomouCBeermanLShiderlyDBerryDMorellVMunozR. Dexmedetomidine: a novel drug for the treatment of atrial and junctional tachyarrhythmias during the perioperative period for congenital cardiac surgery: a preliminary study. Pediatr Anesth. 2008;107:1514–1522.
44.
SendasguptaCMakhijaNKiranUChoudharySKLakshmyRDasSN. Caudal epidural sufentanil and bupivacaine decreases stress response in paediatric cardiac surgery. Ann Cardiol Anaesth. 2009;12:27–33.
45.
LeyviGTaylorDGReithEStockACrookeGWasnickJD. Caudal anesthesia in pediatric cardiac surgery: does it affect outcome?J Cardiothorac Vasc Anesth. 2005; 19:734–738.
46.
SulemanjiDSDonmezAAkpekEAAlicY. Vascular catheterization is difficult in infants with Down syndrome. Acta Anaesthesiol Scand. 2009;53:98–100.