DOI: 10.1161/CIRCULATIONAHA.114.012033
Achievements in Congenital Heart Defect Surgery: A Prospective, 40 Year Study of 7038 Patients
Running title: Erikssen et al.; Postoperative mortality and reoperations Gunnar Erikssen, MD1; Knut Liestøl, PhD2; Egil Seem, MD3; Sigurd Birkeland, MD3; Kjell Johan Saatvedt, MD3; Tom Nilsen Hoel, MD3; Gaute Døhlen, MD4; Helge Skulstad, MD1; Jan Ludvig Svennevig, MD3; Erik Thaulow, MD4; Harald Lauritz Lindb Lindberg, berg, g M g, MD D3
1
Dept De pt ooff Ca Car Cardiology, rdio olo loggy, Oslo University Hospital, Rik R Rikshospitalet; ikshospitalet; 2De Dept D pt of of Informatics, University
of Oslo; Oslo; 3Dept pt ooff Th Thoracic horaacic acic S Surgery, urgeery urge ry,, Osl O Oslo slo U University niiverrsi sitty H Hospital, ospitaal, R osp Rikshospitalet; ik ksh shoosppitaale lett; t; 4Dept Dept p ooff Pe Pedi Pediatric d attric di of Ca Cardiology, ard dio iollogy logy gy,, Oslo Oslo oU University nive ni veerssit ityy Hospital, Hosp Ho spiitaal, Rikshospitalet, sp Riks Riks kshhosp hosp spit ital a et al et,, Oslo, Osllo, Norway Norw No r ay rw
Address for Correspondence: Gunnar Erikssen, MD Department of Cardiology Oslo University Hospital, Rikshospitalet Sognsvannsveien 20 0372 Oslo, Norway Tel: +47 23 07 00 00/ +47 47 68 30 00 Fax: +47 23 07 19 50 E-mail: [email protected] Journal Subject Code: Cardiovascular (CV) surgery:[41] Pediatric and congenital heart disease, including cardiovascular surgery
1 Downloaded from http://circ.ahajournals.org/ at Tulane University on January 26, 2015
DOI: 10.1161/CIRCULATIONAHA.114.012033
Abstract
Background—To update results achieved by modern surgery in congenital heart defects (CHD) over the last 40 years regarding survival and need for reoperations, especially focusing on the results from the two last decades. Methods and Results—From 1971 to 2011, all 7038 patients under age 16 years undergoing surgical treatment for CHD at Rikshospitalet (Oslo, Norway) were enrolled prospectively. CHDdiagnosis, date and type of all operations were recorded, as was all-cause mortality until December 31.2012. CHDs were classified as “simple” (3751/7038 = 53.2%), “complex” (2918/7038 marked, sequential 2918/7038 = 41.5%) or “miscellaneous” (369/7037 = 5.2%). Parallell to a mark ked, ed, se equ quen enti en tial ti al increase ncrease in operations for complex defects, median age at first operation decreased from 1.6 years year ye arss in 11971-1979 ar 9711-19979 to 0.19 years in 2000-2011. IIn 97 n total, total, 1033 died ed d prior priior tto o January 1, 2013. Cumulative 1990-2011 C um mulative survival sur urvvivall until unti till age ti age 16 years years ea s in in complex co omplexx CHD CH HD operated opperrat ateed ed in in 1971-1989 19771-1 19 -1198 9899 vs vs. 19 9900-20 -22011 was vs. 2005-11, wa as 62.4% 62.4 62 .4% % vs. v . 86.9% vs 86.99% (p< (p p< 0.0001). 0.00 0. 0 01 00 01). ). Comparing Com mpa pari ringg patients ri patie at entts operated opeerat op erat ated e inn 2000-04 ed 2 00-0 20 00-044 vs s. 20 005-1 05 11,, oone ne ne 90.7% vs. cumulative 88.8% year survivall wa wass 90 90.7 .7 7% vs s. 96 996.5% .5% .5 % (p ((p=0.003), =0.0 =0 .0 003 03)), aand nd fi five ve yyear earr cu ea umu m la lati tiive ssurvival urvi ur viva vall 88 va 88.8 .88% vs. 95.0% (p=0.0003). In simple vs. complex defects, 434 (11.6%) vs. 985 (33.8%) needed at least one reoperation before age 16 years. In complex defects, five years cumulative freedom of reoperation among patients operated in 1990-1999 vs. 2000-2011 was 66% vs. 73% (p=0.0001). Conclusions—Highly significant, sequential improvements in survival and reductions in reoperations after CHD surgery were seen. A future challenge is to find methods to reduce the need for reoperations and further reduce long term mortality.
Key words: congenital heart disease, surgery, survival, reoperations
2 Downloaded from http://circ.ahajournals.org/ at Tulane University on January 26, 2015
DOI: 10.1161/CIRCULATIONAHA.114.012033
Introduction Following the surgical closures of patent ductus arteriosus (PDA) in 19381 and atrial septal defect (ASD) in 1953,2 major treatment advances have made surgical palliation or correction3 of nearly all congenital heart defects (CHDs) possible. Still, 232.000 deaths globally were attributed to CHD in 2010,4 and most victims were young. Trends in the western countries indicate, however, that this number may be substantially reduced. Nieminen et al reported major improvements in survival of children in Finland undergoing CHD surgery up to 1989.5 Later, few population based studies have specifically targeted long-term survival after CHD surgery.6,7 In Norway, CHD surgery was introduced at Oslo University Hospital, Rikshospitalet, and the he number of patients operated for CHD increased rapidly during the early 1970 1970-ies. 70-i -ies e . Up uuntil es ntil nt il 2003, about 80% of all Norwegian CHD surgery was carried out at this hospital, and after 2003 Riks Ri Rikshospitalet ksho ks hosp ho spit ital it aleet has al has been been responsible for all operations. operaatio tions. All CHD operations ope p raati tioons on in patients aged < 16 registered prospectively 1971, yyears ear ars rs were reg gis istteredd pr pros ospe os pect pe ctiivel ct ivelly fr ffrom om 19 9711, providing prrovid din ingg a basis basiss for basi for a population poopul opul ulaatio ationn based base ba sed study stud st udy dy of the Based the he changes ch han a ge gess in survival survviva vivaal after after su afte ssurgery, rger rg eryy, er y, aage ge at at operation opper e atio io on and and rreoperations. eo ope p rati rati tion o s. B on assed oon n th he 703 77038 038 38 2011, mortality Dec wee wa want patients operated opera raate tedd on from fro rom m 19 1971 7 tto o 20 2011 1, an andd mo mort rtal rt a it al ityy da data taa uuntil ntil nt il D ec 331, 1, 22012, 0 2, w 01 w nt to update these trends for different types of CHDs, especially focusing on the last two decades.
Methods Data collection The study was conducted at the Departments of Thoracic Surgery, Pediatric Cardiology, and Cardiology at Oslo University Hospital (OUS), Rikshospitalet. All operations from June 21, 1971, to December 31, 2011, were registered prospectively using a hand-written protocol from 1971 to 1989 (transferred to our electronic Datacor® database in 1990) and directly into
3 Downloaded from http://circ.ahajournals.org/ at Tulane University on January 26, 2015
DOI: 10.1161/CIRCULATIONAHA.114.012033
Datacor® thereafter. All-cause mortality until December 31, 2012, was obtained from our files and from the Norwegian population registry in Statistics Norway. The study was approved by the Data Protection Officer at OUS. Data handling was facilitated by using each patient’s unique eleven digit social security number, which includes individual birth date. CHD diagnoses were based on ICD-9 until 1998, and ICD-10 thereafter. The date of the first operation, number of operations, and type(s) of surgical correction(s) were recorded. A maximum of three diagnoses were given, and each patient was classified by the most severe diagnosis according to a diagnosis hierarchy as applied previously. 5 Data on the number of operations performed at other Norwegian hospitals lss ffrom r m 19 ro 1994 94 tto o 2003 were obtained from the Norwegian Cardiovascular Surgery Registry (operative from 1994) revealing eveeal alin ingg that in that 80.1% 80. 0 1% 0. 1% of all CHD operations in Norway Norw No way were perf performed for o meed at at Rikshospitalet during this hiss period. period. After Aft fter err 2003, 200 03, 3 all all CHD CHD H operations ope pera raatiions have haave been beenn performed perffor perf orme medd at Rikshospitalet. Rik i shos shosspi pita tale leet. From through 1998, left Fr om 11987 9887 th thr roug ughh 19 998 98, 51 ppatients a ie at ient ntss wi nt with th h hhypoplastic ypop yp opla op lassti la ticc le eft hheart e rt ssyndrome ea yndr yn d om dr o e (HLH ((HLHS) HLH LHS) S) hhad ad d their heir Fontan circulation cir ircu cula cu lati la t on ti o completed com om mpl p etted at at a surgical surg su rgic rg ical ic al centre cen entr tree in tr n tthe he U United nite ni t d St Stat States ates at es (25 (255 survivors). sur urvi v vo vi v rs).8 Thesee operations have not been included in our database. Data on mortality in all congenital heart defects in Norway from 1971 to 2011 were obtained through open access to official Norwegian mortality statistics at Statistics Norway. Rapid development occurred in the availability of diagnostic tools during the long study period. In the 1970-ies, primary pre-operative CHD diagnoses necessary for planning the operations were mainly based on clinical findings and invasive studies, but from the early 1980ies diagnostics increasingly relied on echo-Doppler techniques and computer tomography, and from the late 1980-ies also on magnetic resonance imaging. Significant refinements in all these
4 Downloaded from http://circ.ahajournals.org/ at Tulane University on January 26, 2015
DOI: 10.1161/CIRCULATIONAHA.114.012033
techniques are still taking place. However, final diagnoses (i.e. applied in this paper) were ultimately obtained during surgery. Complex defects A CHD was defined as a defect in the structure of the heart and/or the great vessels that was present at birth. If more than one CHD was present, patients were classified by the most severe diagnosis according to a diagnosis hierarchy as applied previously.5 The following diagnostic criteria were used for “complex defects” (table 1): 1.
Univentricular hearts (UVH) were characterized by the presence of one rudimentary and
one dominant ventricle (left or right), regardless of whether it had a double-inlet left or right t ventricle or a single atrioventricular inlet. 2.
Truncus arteriosus communis (TAC) was diagnosed according to Van Praagh, 9 including
TA AC associated asso as sooci ciat ated at d with wit i h an interrupted aortic arch. TAC 3. 3.
Cases with wiith t interrupted int nteerru rupt ru pted pt ed oorr hy hypo hypoplastic p pl po plaastiic aaortic ortic ic arch arcch (I/HAA) (I//HAA (I /HAA AA)) with with h orr without with ithout houtt coarctation cooar oarctaation tionn ooff
the he aorta ao ort r a (COA) (COA (C O ) were OA were grouped grou rouped e together. ed tog oget ethe et heer. Aortic Aortic or ic arch archh hypoplasia hypo hy popl po plassia pl i w was as ddefined efiined d aaccording c or cc ordding ding g too Moulaert et al al. l.10 4.
Patients with simple or complex transposition of the great arteries (TGA) and
biventricular anatomy were grouped together. 5.
Atrioventricular septal defect (AVSD) included both the incomplete and complete forms.
6.
Patients with totally anomalous pulmonary venous drainage (TAPVD) were assigned to
this group if this was their dominant defect. 7.
Cases with pulmonary atresia (PA), with or without coexisting ventricular septal defect
(VSD), were assigned PA as their primary diagnosis if biventricular correction was performed. 8.
Tetralogy of Fallot (TOF) was defined according to convention. Double outlet right
5 Downloaded from http://circ.ahajournals.org/ at Tulane University on January 26, 2015
DOI: 10.1161/CIRCULATIONAHA.114.012033
ventricle (DORV) included both simple and complex forms.11 According to the diagnosis hierarchy in table 1, patients e.g. with both UVH and TAPVD were classified as UVH. Simple defects VSD and COA were defined conventionally. Valvular aortic stenosis was classified as AS, valvular pulmonary stenosis as PS, and stenosis or insufficiency of the mitral valve as MV. Cases with drainage to the right atrium from one or more – but not all – pulmonary veins, were classified as PAPVD. ASD included ASD secundum and superior and inferior sinus venosus defect, but not partial AVSD. PDA was diagnosed in the presence of an open ductus > 2 weeks after birth. Miscellaneous defects Th he MI MISC SC ggroup roupp ((n=369) n=369) included DORV (n=95 95)), subvalvular aand 95 n sup nd up pra ravalvular aortic stenosis The (n=95), supravalvular n=65 and 21, respectively), resppeccti re t veely) ly), ), Ebstein’s Ebste bsteein n’s anomaly anom mally (n=11), (n n=11)), congenitally conngen co ngeniitallly ccorrected orrect orre ctted T GA ((n=4), n=4) n=4) 4),, (n=65 TGA co oro rona nary na ry aartery rteery rt ery anom aanomalies nom o aliies (n n=1 =18) 8), cor 8) cor tr tria iatr ia triiatu tr tum tu m (n n=1 11)), and and va vasc sccul ular arr ri ingss (n=48). ings (n= n 48 48)). ). A total tootal otal off 97 9 coronary (n=18), triatriatum (n=11), vascular rings patients (26.4 4% off aall l 3369 ll 6 ppatients 69 atie at ient ntss in the nt he M ISC IS C ggroup ro oup aand nd 11.4% . % of aall .4 lll 77038 0388 pa 03 ati tien ents en ts iin n the study)) (26.4% MISC patients had defects lacking dominant features that justified classification into any of the previously mentioned categories. Only patients who had their first operation before their 16th birthday were included because this is the age threshold of the pediatric population in Norway. The term “early mortality” is used for all deaths that occurred < 30 days after the first operation, and “late mortality” denotes deaths occurring later. Reoperations Dates and descriptions of all operations were recorded prospectively. The first operation was
6 Downloaded from http://circ.ahajournals.org/ at Tulane University on January 26, 2015
DOI: 10.1161/CIRCULATIONAHA.114.012033
denoted the “index operation”. Operations both with and without cardiopulmonary bypass (CPB) were included, and operations were classified according to whether they could be considered as “corrections” or “palliations”. Palliative procedures mainly included shunt operations or banding of the pulmonary artery – either as destination therapy or as steps in staged procedures aimed at final correction. Accordingly, e.g. the Norwood I operation and Glenn operations were classified as a palliative procedures. E.g. Fontan operations, on the other hand, were classified as corrections. The few reoperations due to acute surgical complications have not been included. Statistical analysis We used Kaplan-Meier analyses to study survival after first operation (< 30 days and until age 16 without reoperation years), and reoperation-free “survival”. In the latter case, patients who died witho hooutt reo eo ope pera rati ra tion ti o were censored at the time death. The log-rank test (Mantel-Cox) was used to test for differences inn ssurvival between urvi ur viva vi vaal be betw wee eenn eras. Kaplan-Meier were separately Kaplan n-M -Meeier er probabilities pro roba babi ba bili bi liti ties ess of of 5 yyears earrs reoperation-free reopperatiion ion-fr n-fr freee survival survviva vivall w eree comp er ccomputed omp put uted ed d se eparrattely epar for fo or all all complex c mp co mple lexx defects le defe defe f ctts in in patients pat atie ient ntss who nt w o had wh had their thei th eirr first ei firrst operation oper op erat er atio on in in 1990-1999 199 9 0-19 1 99 vs. 19 vs. 2000-2011. 2000 000-20 011 11. was operated 1990-1999 Five years w as cchosen hose ho s n so se ssomewhat mewh me w at aarbitrarily wh rbittra rb rari rily ri ly bbecause ecau ec ause au se ppatients attie ient n s op nt oper e at ated ed iin n 19 1990 900-1 199 9999 ha hhad d longer follow-up than patients operated in 2000-2011, and since the great majority of reoperations were performed during the first few years after the first operation in both eras (see Results). The Mann-Whitney test was used when comparing variables not necessarily normally distributed. The binomial test was used when testing for differences in numbers of various types of operations in different eras. Two-sided p-values
Achievements in Congenital Heart Defect Surgery: A Prospective, 40 Year Study of 7038 Patients
Running title: Erikssen et al.; Postoperative mortality and reoperations Gunnar Erikssen, MD1; Knut Liestøl, PhD2; Egil Seem, MD3; Sigurd Birkeland, MD3; Kjell Johan Saatvedt, MD3; Tom Nilsen Hoel, MD3; Gaute Døhlen, MD4; Helge Skulstad, MD1; Jan Ludvig Svennevig, MD3; Erik Thaulow, MD4; Harald Lauritz Lindb Lindberg, berg, g M g, MD D3
1
Dept De pt ooff Ca Car Cardiology, rdio olo loggy, Oslo University Hospital, Rik R Rikshospitalet; ikshospitalet; 2De Dept D pt of of Informatics, University
of Oslo; Oslo; 3Dept pt ooff Th Thoracic horaacic acic S Surgery, urgeery urge ry,, Osl O Oslo slo U University niiverrsi sitty H Hospital, ospitaal, R osp Rikshospitalet; ik ksh shoosppitaale lett; t; 4Dept Dept p ooff Pe Pedi Pediatric d attric di of Ca Cardiology, ard dio iollogy logy gy,, Oslo Oslo oU University nive ni veerssit ityy Hospital, Hosp Ho spiitaal, Rikshospitalet, sp Riks Riks kshhosp hosp spit ital a et al et,, Oslo, Osllo, Norway Norw No r ay rw
Address for Correspondence: Gunnar Erikssen, MD Department of Cardiology Oslo University Hospital, Rikshospitalet Sognsvannsveien 20 0372 Oslo, Norway Tel: +47 23 07 00 00/ +47 47 68 30 00 Fax: +47 23 07 19 50 E-mail: [email protected] Journal Subject Code: Cardiovascular (CV) surgery:[41] Pediatric and congenital heart disease, including cardiovascular surgery
1 Downloaded from http://circ.ahajournals.org/ at Tulane University on January 26, 2015
DOI: 10.1161/CIRCULATIONAHA.114.012033
Abstract
Background—To update results achieved by modern surgery in congenital heart defects (CHD) over the last 40 years regarding survival and need for reoperations, especially focusing on the results from the two last decades. Methods and Results—From 1971 to 2011, all 7038 patients under age 16 years undergoing surgical treatment for CHD at Rikshospitalet (Oslo, Norway) were enrolled prospectively. CHDdiagnosis, date and type of all operations were recorded, as was all-cause mortality until December 31.2012. CHDs were classified as “simple” (3751/7038 = 53.2%), “complex” (2918/7038 marked, sequential 2918/7038 = 41.5%) or “miscellaneous” (369/7037 = 5.2%). Parallell to a mark ked, ed, se equ quen enti en tial ti al increase ncrease in operations for complex defects, median age at first operation decreased from 1.6 years year ye arss in 11971-1979 ar 9711-19979 to 0.19 years in 2000-2011. IIn 97 n total, total, 1033 died ed d prior priior tto o January 1, 2013. Cumulative 1990-2011 C um mulative survival sur urvvivall until unti till age ti age 16 years years ea s in in complex co omplexx CHD CH HD operated opperrat ateed ed in in 1971-1989 19771-1 19 -1198 9899 vs vs. 19 9900-20 -22011 was vs. 2005-11, wa as 62.4% 62.4 62 .4% % vs. v . 86.9% vs 86.99% (p< (p p< 0.0001). 0.00 0. 0 01 00 01). ). Comparing Com mpa pari ringg patients ri patie at entts operated opeerat op erat ated e inn 2000-04 ed 2 00-0 20 00-044 vs s. 20 005-1 05 11,, oone ne ne 90.7% vs. cumulative 88.8% year survivall wa wass 90 90.7 .7 7% vs s. 96 996.5% .5% .5 % (p ((p=0.003), =0.0 =0 .0 003 03)), aand nd fi five ve yyear earr cu ea umu m la lati tiive ssurvival urvi ur viva vall 88 va 88.8 .88% vs. 95.0% (p=0.0003). In simple vs. complex defects, 434 (11.6%) vs. 985 (33.8%) needed at least one reoperation before age 16 years. In complex defects, five years cumulative freedom of reoperation among patients operated in 1990-1999 vs. 2000-2011 was 66% vs. 73% (p=0.0001). Conclusions—Highly significant, sequential improvements in survival and reductions in reoperations after CHD surgery were seen. A future challenge is to find methods to reduce the need for reoperations and further reduce long term mortality.
Key words: congenital heart disease, surgery, survival, reoperations
2 Downloaded from http://circ.ahajournals.org/ at Tulane University on January 26, 2015
DOI: 10.1161/CIRCULATIONAHA.114.012033
Introduction Following the surgical closures of patent ductus arteriosus (PDA) in 19381 and atrial septal defect (ASD) in 1953,2 major treatment advances have made surgical palliation or correction3 of nearly all congenital heart defects (CHDs) possible. Still, 232.000 deaths globally were attributed to CHD in 2010,4 and most victims were young. Trends in the western countries indicate, however, that this number may be substantially reduced. Nieminen et al reported major improvements in survival of children in Finland undergoing CHD surgery up to 1989.5 Later, few population based studies have specifically targeted long-term survival after CHD surgery.6,7 In Norway, CHD surgery was introduced at Oslo University Hospital, Rikshospitalet, and the he number of patients operated for CHD increased rapidly during the early 1970 1970-ies. 70-i -ies e . Up uuntil es ntil nt il 2003, about 80% of all Norwegian CHD surgery was carried out at this hospital, and after 2003 Riks Ri Rikshospitalet ksho ks hosp ho spit ital it aleet has al has been been responsible for all operations. operaatio tions. All CHD operations ope p raati tioons on in patients aged < 16 registered prospectively 1971, yyears ear ars rs were reg gis istteredd pr pros ospe os pect pe ctiivel ct ivelly fr ffrom om 19 9711, providing prrovid din ingg a basis basiss for basi for a population poopul opul ulaatio ationn based base ba sed study stud st udy dy of the Based the he changes ch han a ge gess in survival survviva vivaal after after su afte ssurgery, rger rg eryy, er y, aage ge at at operation opper e atio io on and and rreoperations. eo ope p rati rati tion o s. B on assed oon n th he 703 77038 038 38 2011, mortality Dec wee wa want patients operated opera raate tedd on from fro rom m 19 1971 7 tto o 20 2011 1, an andd mo mort rtal rt a it al ityy da data taa uuntil ntil nt il D ec 331, 1, 22012, 0 2, w 01 w nt to update these trends for different types of CHDs, especially focusing on the last two decades.
Methods Data collection The study was conducted at the Departments of Thoracic Surgery, Pediatric Cardiology, and Cardiology at Oslo University Hospital (OUS), Rikshospitalet. All operations from June 21, 1971, to December 31, 2011, were registered prospectively using a hand-written protocol from 1971 to 1989 (transferred to our electronic Datacor® database in 1990) and directly into
3 Downloaded from http://circ.ahajournals.org/ at Tulane University on January 26, 2015
DOI: 10.1161/CIRCULATIONAHA.114.012033
Datacor® thereafter. All-cause mortality until December 31, 2012, was obtained from our files and from the Norwegian population registry in Statistics Norway. The study was approved by the Data Protection Officer at OUS. Data handling was facilitated by using each patient’s unique eleven digit social security number, which includes individual birth date. CHD diagnoses were based on ICD-9 until 1998, and ICD-10 thereafter. The date of the first operation, number of operations, and type(s) of surgical correction(s) were recorded. A maximum of three diagnoses were given, and each patient was classified by the most severe diagnosis according to a diagnosis hierarchy as applied previously. 5 Data on the number of operations performed at other Norwegian hospitals lss ffrom r m 19 ro 1994 94 tto o 2003 were obtained from the Norwegian Cardiovascular Surgery Registry (operative from 1994) revealing eveeal alin ingg that in that 80.1% 80. 0 1% 0. 1% of all CHD operations in Norway Norw No way were perf performed for o meed at at Rikshospitalet during this hiss period. period. After Aft fter err 2003, 200 03, 3 all all CHD CHD H operations ope pera raatiions have haave been beenn performed perffor perf orme medd at Rikshospitalet. Rik i shos shosspi pita tale leet. From through 1998, left Fr om 11987 9887 th thr roug ughh 19 998 98, 51 ppatients a ie at ient ntss wi nt with th h hhypoplastic ypop yp opla op lassti la ticc le eft hheart e rt ssyndrome ea yndr yn d om dr o e (HLH ((HLHS) HLH LHS) S) hhad ad d their heir Fontan circulation cir ircu cula cu lati la t on ti o completed com om mpl p etted at at a surgical surg su rgic rg ical ic al centre cen entr tree in tr n tthe he U United nite ni t d St Stat States ates at es (25 (255 survivors). sur urvi v vo vi v rs).8 Thesee operations have not been included in our database. Data on mortality in all congenital heart defects in Norway from 1971 to 2011 were obtained through open access to official Norwegian mortality statistics at Statistics Norway. Rapid development occurred in the availability of diagnostic tools during the long study period. In the 1970-ies, primary pre-operative CHD diagnoses necessary for planning the operations were mainly based on clinical findings and invasive studies, but from the early 1980ies diagnostics increasingly relied on echo-Doppler techniques and computer tomography, and from the late 1980-ies also on magnetic resonance imaging. Significant refinements in all these
4 Downloaded from http://circ.ahajournals.org/ at Tulane University on January 26, 2015
DOI: 10.1161/CIRCULATIONAHA.114.012033
techniques are still taking place. However, final diagnoses (i.e. applied in this paper) were ultimately obtained during surgery. Complex defects A CHD was defined as a defect in the structure of the heart and/or the great vessels that was present at birth. If more than one CHD was present, patients were classified by the most severe diagnosis according to a diagnosis hierarchy as applied previously.5 The following diagnostic criteria were used for “complex defects” (table 1): 1.
Univentricular hearts (UVH) were characterized by the presence of one rudimentary and
one dominant ventricle (left or right), regardless of whether it had a double-inlet left or right t ventricle or a single atrioventricular inlet. 2.
Truncus arteriosus communis (TAC) was diagnosed according to Van Praagh, 9 including
TA AC associated asso as sooci ciat ated at d with wit i h an interrupted aortic arch. TAC 3. 3.
Cases with wiith t interrupted int nteerru rupt ru pted pt ed oorr hy hypo hypoplastic p pl po plaastiic aaortic ortic ic arch arcch (I/HAA) (I//HAA (I /HAA AA)) with with h orr without with ithout houtt coarctation cooar oarctaation tionn ooff
the he aorta ao ort r a (COA) (COA (C O ) were OA were grouped grou rouped e together. ed tog oget ethe et heer. Aortic Aortic or ic arch archh hypoplasia hypo hy popl po plassia pl i w was as ddefined efiined d aaccording c or cc ordding ding g too Moulaert et al al. l.10 4.
Patients with simple or complex transposition of the great arteries (TGA) and
biventricular anatomy were grouped together. 5.
Atrioventricular septal defect (AVSD) included both the incomplete and complete forms.
6.
Patients with totally anomalous pulmonary venous drainage (TAPVD) were assigned to
this group if this was their dominant defect. 7.
Cases with pulmonary atresia (PA), with or without coexisting ventricular septal defect
(VSD), were assigned PA as their primary diagnosis if biventricular correction was performed. 8.
Tetralogy of Fallot (TOF) was defined according to convention. Double outlet right
5 Downloaded from http://circ.ahajournals.org/ at Tulane University on January 26, 2015
DOI: 10.1161/CIRCULATIONAHA.114.012033
ventricle (DORV) included both simple and complex forms.11 According to the diagnosis hierarchy in table 1, patients e.g. with both UVH and TAPVD were classified as UVH. Simple defects VSD and COA were defined conventionally. Valvular aortic stenosis was classified as AS, valvular pulmonary stenosis as PS, and stenosis or insufficiency of the mitral valve as MV. Cases with drainage to the right atrium from one or more – but not all – pulmonary veins, were classified as PAPVD. ASD included ASD secundum and superior and inferior sinus venosus defect, but not partial AVSD. PDA was diagnosed in the presence of an open ductus > 2 weeks after birth. Miscellaneous defects Th he MI MISC SC ggroup roupp ((n=369) n=369) included DORV (n=95 95)), subvalvular aand 95 n sup nd up pra ravalvular aortic stenosis The (n=95), supravalvular n=65 and 21, respectively), resppeccti re t veely) ly), ), Ebstein’s Ebste bsteein n’s anomaly anom mally (n=11), (n n=11)), congenitally conngen co ngeniitallly ccorrected orrect orre ctted T GA ((n=4), n=4) n=4) 4),, (n=65 TGA co oro rona nary na ry aartery rteery rt ery anom aanomalies nom o aliies (n n=1 =18) 8), cor 8) cor tr tria iatr ia triiatu tr tum tu m (n n=1 11)), and and va vasc sccul ular arr ri ingss (n=48). ings (n= n 48 48)). ). A total tootal otal off 97 9 coronary (n=18), triatriatum (n=11), vascular rings patients (26.4 4% off aall l 3369 ll 6 ppatients 69 atie at ient ntss in the nt he M ISC IS C ggroup ro oup aand nd 11.4% . % of aall .4 lll 77038 0388 pa 03 ati tien ents en ts iin n the study)) (26.4% MISC patients had defects lacking dominant features that justified classification into any of the previously mentioned categories. Only patients who had their first operation before their 16th birthday were included because this is the age threshold of the pediatric population in Norway. The term “early mortality” is used for all deaths that occurred < 30 days after the first operation, and “late mortality” denotes deaths occurring later. Reoperations Dates and descriptions of all operations were recorded prospectively. The first operation was
6 Downloaded from http://circ.ahajournals.org/ at Tulane University on January 26, 2015
DOI: 10.1161/CIRCULATIONAHA.114.012033
denoted the “index operation”. Operations both with and without cardiopulmonary bypass (CPB) were included, and operations were classified according to whether they could be considered as “corrections” or “palliations”. Palliative procedures mainly included shunt operations or banding of the pulmonary artery – either as destination therapy or as steps in staged procedures aimed at final correction. Accordingly, e.g. the Norwood I operation and Glenn operations were classified as a palliative procedures. E.g. Fontan operations, on the other hand, were classified as corrections. The few reoperations due to acute surgical complications have not been included. Statistical analysis We used Kaplan-Meier analyses to study survival after first operation (< 30 days and until age 16 without reoperation years), and reoperation-free “survival”. In the latter case, patients who died witho hooutt reo eo ope pera rati ra tion ti o were censored at the time death. The log-rank test (Mantel-Cox) was used to test for differences inn ssurvival between urvi ur viva vi vaal be betw wee eenn eras. Kaplan-Meier were separately Kaplan n-M -Meeier er probabilities pro roba babi ba bili bi liti ties ess of of 5 yyears earrs reoperation-free reopperatiion ion-fr n-fr freee survival survviva vivall w eree comp er ccomputed omp put uted ed d se eparrattely epar for fo or all all complex c mp co mple lexx defects le defe defe f ctts in in patients pat atie ient ntss who nt w o had wh had their thei th eirr first ei firrst operation oper op erat er atio on in in 1990-1999 199 9 0-19 1 99 vs. 19 vs. 2000-2011. 2000 000-20 011 11. was operated 1990-1999 Five years w as cchosen hose ho s n so se ssomewhat mewh me w at aarbitrarily wh rbittra rb rari rily ri ly bbecause ecau ec ause au se ppatients attie ient n s op nt oper e at ated ed iin n 19 1990 900-1 199 9999 ha hhad d longer follow-up than patients operated in 2000-2011, and since the great majority of reoperations were performed during the first few years after the first operation in both eras (see Results). The Mann-Whitney test was used when comparing variables not necessarily normally distributed. The binomial test was used when testing for differences in numbers of various types of operations in different eras. Two-sided p-values
