An increasing number of children with congenital heart disease survive to adulthood, but many adults require surgical intervention and can present complex management challenges in the perioperative period. This review will address common considerations that surgeons, anesthesiologists, and intensivists are likely to face in caring for this growing population.
MoonsPBovijnLBudtsWBelmansAGewilligM. Temporal trends in survival to adulthood among patients born with congenital heart disease from 1970 to 1992 in Belgium. Circulation. 2010;122:2264-2272.
2.
MarelliAJMackieASIonescu-IttuRRahmeEPiloteL. Congenital heart disease in the general population: changing prevalence and age distribution. Circulation. 2007;115:163-172.
3.
MarelliAJIonescu-IttuRMackieASGuoLDendukuriNKaouacheM. Lifetime prevalence of congenital heart disease in the general population from 2000 to 2010. Circulation. 2014;130:749-756.
4.
AfilaloJTherrienJPiloteLIonescu-IttuRMartucciGMarelliAJ. Geriatric congenital heart disease: burden of disease and predictors of mortality. J Am Coll Cardiol. 2011;58:1509-1515.
5.
WarnesCA. The adult with congenital heart disease: born to be bad?J Am Coll Cardiol. 2005;46:1-8.
6.
KaramlouTDiggsBSPersonTUngerleiderRMWelkeKF. National practice patterns for management of adult congenital heart disease: operation by pediatric heart surgeons decreases in-hospital death. Circulation. 2008;118:2345-2352.
7.
KogonBEPlattnerCLeongT. Adult congenital heart surgery: adult or pediatric facility? Adult or pediatric surgeon?Ann Thorac Surg. 2009;87:833-840.
8.
WarnesCAWilliamsRGBashoreTM. ACC/AHA 2008 guidelines for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines. J Am Coll Cardiol. 2008;52:e143-e263.
9.
MaxwellBGWilliamsGDRamamoorthyC. Knowledge and attitudes of anesthesia providers about noncardiac surgery in adults with congenital heart disease. Congenit Heart Dis. 2013;9:45-53.
10.
DinardoJAAndropoulosDBBaumVC. Special article: a proposal for training in pediatric cardiac anesthesia. Anesth Analg. 2010;110:1121-1125.
11.
MascioCEPasqualiSKJacobsJPJacobsMLAustinEH. Outcomes in adult congenital heart surgery: analysis of the Society of Thoracic Surgeons Database. J Thorac Cardiovasc Surg. 2011;142:1090-1097.
12.
MaxwellBGPosnerKLWongJK. Factors contributing to adverse perioperative events in adults with congenital heart disease: a structured analysis of cases from the Closed Claims Project [published online May28, 2014]. Congenit Heart Dis. doi:10.1111/chd.12188.
13.
BouchardyJTherrienJPiloteL. Atrial arrhythmias in adults with congenital heart disease. Circulation. 2009;120:1679-1686.
14.
KoyakZAchterberghRCAde GrootJR. Postoperative arrhythmias in adults with congenital heart disease: incidence and risk factors. Int J Cardiol. 2013;169:139-144.
15.
FabioGPietroB. Perioperative haemodynamic management: is echocardiography the right tool?Curr Opin Crit Care. 2014;20:431-437.
16.
KuttySAtteberyJEYeagerEM. Transthoracic echocardiography in pediatric intensive care: impact on medical and surgical management. Pediatr Crit Care Med. 2014;15:329-335.
17.
MaskatiaSAFeinsteinJANewmanBHanleyFLRothSJ. Pulmonary reperfusion injury after the unifocalization procedure for tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collateral arteries. J Thorac Cardiovasc Surg. 2012;144:184-189.
18.
AsijaRRothSJHanleyFL. Reperfusion pulmonary edema in children with tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collateral arteries undergoing unifocalization procedures: a pilot study examining potential pathophysiologic mechanisms and clinical significance. J Thorac Cardiovasc Surg. 2014;148:1560-1565.
19.
YacoubySMeadorMMossadE. Lung reperfusion injury in patients after balloon angioplasty for pulmonary artery stenosis. J Cardiothorac Vasc Anesth. 2014;28:502-505.
20.
LeeKCChoYLLeeSY. Reperfusion pulmonary edema after pulmonary endarterectomy. Acta Anaesthesiol Sin. 2001;39:97-101.
21.
DobsonRDantonMNicolaWHamishW. The natural and unnatural history of the systemic right ventricle in adult survivors. J Thorac Cardiovasc Surg. 2013;145:1493-1501.
22.
FontanFBaudetE. Surgical repair of tricuspid atresia. Thorax. 1971;26:240-248.
23.
SteinDGLaksHDrinkwaterDC. Results of total cavopulmonary connection in the treatment of patients with a functional single ventricle. J Thorac Cardiovasc Surg. 1991;102:280-286.
24.
MarcellettiCFHanleyFLMavroudisC. Revision of previous Fontan connections to total extracardiac cavopulmonary anastomosis: a multicenter experience. J Thorac Cardiovasc Surg. 2000;119:340-346.
25.
SheikhAMTangATMRomanK. The failing Fontan circulation: successful conversion of atriopulmonary connections. J Thorac Cardiovasc Surg. 2004;128:60-66.
26.
GamillschegAZobelGUrlesbergerB. Inhaled nitric oxide in patients with critical pulmonary perfusion after Fontan-type procedures and bidirectional Glenn anastomosis. J Thorac Cardiovasc Surg. 1997;113:435-442.
BalintOHSammanAHabererK. Outcomes in patients with pulmonary hypertension undergoing percutaneous atrial septal defect closure. Heart. 2008;94:1189-1193.
29.
YongGKhairyPDe GuiseP. Pulmonary arterial hypertension in patients with transcatheter closure of secundum atrial septal defects: a longitudinal study. Circ Cardiovasc Interv. 2009;2:455-462.
30.
HuangZFanZSunJ. The short- and medium-term results of transcatheter closure of atrial septal defect with severe pulmonary arterial hypertension. Heart Vessels. 2012;27:603-609.
31.
D’AltoMRomeoEArgientoP. Pulmonary vasoreactivity predicts long-term outcome in patients with Eisenmenger syndrome receiving bosentan therapy. Heart. 2010;96:1475-1479.
32.
PostMCJanssensSVandeWerfFBudtsW. Responsiveness to inhaled nitric oxide is a predictor for mid-term survival in adult patients with congenital heart defects and pulmonary arterial hypertension. Eur Heart J. 2004;25:1651-1656.
33.
KrasuskiRADevendraGPHartSAWangAHarrisonJKBashoreTM. Response to inhaled nitric oxide predicts survival in patients with pulmonary hypertension. J Card Fail. 2011;17:265-271.
34.
HopkinsWEOchoaLLRichardsonGWTrulockEP. Comparison of the hemodynamics and survival of adults with severe primary pulmonary hypertension or Eisenmenger syndrome. J Heart Lung Transplant. 1996;15(1 pt 1):100-105.
35.
ForbesTJKimDWDuW. Comparison of surgical, stent, and balloon angioplasty treatment of native coarctation of the aorta: an observational study by the CCISC (Congenital Cardiovascular Interventional Study Consortium). J Am Coll Cardiol. 2011;58:2664-2674.
36.
WackerAKaemmererHHollweckR. Outcome of operated and unoperated adults with congenital cardiac disease lost to follow-up for more than five years. Am J Cardiol. 2005;95:776-779.
37.
MackieASIonescu-IttuRTherrienJPiloteLAbrahamowiczMMarelliAJ. Children and adults with congenital heart disease lost to follow-up: who and when?Circulation. 2009;120:302-309.
38.
VisJCSchuuringMJvan der VeldeET. Many adults with congenital heart disease are lost to follow up. Ned Tijdschr Geneeskd. 2012;156:A3767.
39.
GindeSBartzPJHillGD. Restrictive lung disease is an independent predictor of exercise intolerance in the adult with congenital heart disease. Congenit Heart Dis. 2013;8:246-254.
40.
Alonso-GonzalezRBorgiaFDillerGP. Abnormal lung function in adults with congenital heart disease: prevalence, relation to cardiac anatomy, and association with survival. Circulation. 2013;127:882-890.
41.
DimopoulosKDillerGPKoltsidaE. Prevalence, predictors, and prognostic value of renal dysfunction in adults with congenital heart disease. Circulation. 2008;117:2320-2328.
42.
Van NieuwenhuizenRCPetersMLubbersLJTripMDTijssenJGMulderBJ. Abnormalities in liver function and coagulation profile following the Fontan procedure. Heart. 1999;82:40-46.
43.
KiesewetterCHSheronNVettukattillJJ. Hepatic changes in the failing Fontan circulation. Heart. 2007;93:579-584.
44.
RavnHBHjortdalVEStenbogEV. Increased platelet reactivity and significant changes in coagulation markers after cavopulmonary connection. Heart. 2001;85:61-65.
45.
OdegardKCMcGowanFXZurakowskiD. Procoagulant and anticoagulant factor abnormalities following the Fontan procedure: increased factor VIII may predispose to thrombosis. J Thorac Cardiovasc Surg. 2003;125:1260-1267.
46.
Tomkiewicz-PajakLHoffmanPTrojnarskaOLipczyńskaMPodolecPUndasA. Abnormalities in blood coagulation, fibrinolysis, and platelet activation in adult patients after the Fontan procedure. J Thorac Cardiovasc Surg. 2014;147:1284-1290.
47.
KompDMSparrowAW. Polycythemia in cyanotic heart disease-a study of altered coagulation. J Pediatr. 1970;76:231-236.
48.
JohnsonJACettaFGrahamRP. Identifying predictors of hepatic disease in patients after the Fontan operation: a postmortem analysis. J Thorac Cardiovasc Surg. 2013;146:140-145.
49.
FeldtRHDriscollDJOffordKP. Protein-losing enteropathy after the Fontan operation. J Thorac Cardiovasc Surg. 1996;112:672-680.
50.
CaruthersRLKempaMLooA. Demographic characteristics and estimated prevalence of Fontan-associated plastic bronchitis. Pediatr Cardiol. 2013;34:256-261.
51.
ShahNRLamWWRodriguezFH. Clinical outcomes after ventricular assist device implantation in adults with complex congenital heart disease. J Heart Lung Transplant. 2013;32:615-620.
52.
MaxwellBGWongJKSheikhAYLeePHULobatoRL. Heart transplantation with or without prior mechanical circulatory support in adults with congenital heart disease. Eur J Cardiothorac Surg. 2014;45:842-846.
53.
MoralesDLSKhanMSGottliebEAKrishnamurthyRDreyerWJAdachiI. Implantation of total artificial heart in congenital heart disease. Semin Thorac Cardiovasc Surg. 2012;24:142-143.
54.
ToblerDWilliamsWGJegatheeswaranA. Cardiac outcomes in young adult survivors of the arterial switch operation for transposition of the great arteries. J Am Coll Cardiol. 2010;56:58-64.
55.
GratzAHessJHagerA. Self-estimated physical functioning poorly predicts actual exercise capacity in adolescents and adults with congenital heart disease. Eur Heart J. 2009;30:497-504.
56.
DillerGPDimopoulosKOkonkoD. Exercise intolerance in adult congenital heart disease: comparative severity, correlates, and prognostic implication. Circulation. 2005;112:828-835.
