The Outcomes of Superior Cavopulmonary Connection Operation: a Single Center Experience

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1 ORIGINAL ARTICLE Brz J Crdiovsc Surg 2017;32(6):503-7 The Outcomes of Superior Cvopulmonry Connection Opertion: Single Center Experience Alwleed Al-Diry 1, MD; Mzir Gholmpour Dehki 1, MD; Gholmrez Omrni 1, MD; Ali Sdeghpour 1, MD; Amir Hossein Jlli 1, MD; Rez Sdt Afjehi 1, MD; Mohmmd Mhdvi 2, MD; Mhmood Slesi 3, PhD DOI: / Abstrct Introduction: The superior cvopulmonry connection opertion is one of the stges of the pllitive surgicl mngement for ptients with functionlly single ventricle. After surviving this stge, the ptients re potentil cndidtes for the finl pllitive procedure: the Fontn opertion. Objectives: This study imed to nlyze the outcomes of superior cvopulmonry connection opertions in our center nd to identify fctors ffecting the survivl nd the progression to Fontn stge. Methods: The outcomes of 161 ptients were retrospectively nlyzed fter undergoing superior cvopulmonry connection opertion in our center between 2005 nd Results: The erly mortlity rte ws 2.5%. Five (3.1%) ptients underwent tkedown of the superior cvopulmonry connection. The rte of exclusion from the Fontn stge ws 8.3%. Sttisticl nlysis reveled tht elevted men pulmonry rtery pressure preopertively nd the prior pllition with pulmonry rtery bnding were risk fctors for both erly mortlity nd tkedown; however, the ge, the morphology of the single ventricle nd the type of opertion were not considered risk fctors. Conclusion: The superior cvopulmonry connection opertion cn be performed with low rte mortlity nd morbidity; however, the elevted men pulmonry rtery pressure preopertively nd the prior pulmonry rtery bnding re ssocited with poor outcomes. Keywords: Fontn Procedure. Hert Bypss, Right. Hert Ventricles/pthology. Hert Defects, Congenitl/surgery. Abbrevitions, cronyms & symbols CPB = Crdiopulmonry bypss CTA = Computed tomogrphic ngiogrphy LV = Left ventricle mpap = Men pulmonry rtery pressure PAB = Pulmonry rtery bnding PAP = Pulmonry rtery pressure RV = Right ventricle SCPC = Superior cvopulmonry connection TAPVC = Totl nomlous pulmonry venous connection TCPC = Totl cvopulmonry connection TTE = Trnsthorcic echocrdiogrphy INTRODUCTION The superior cvopulmonry connection (SCPC) opertion represents one of the stges for the surgicl pllition in ptients with functionlly univentriculr herts. This opertion my or my not be preceded by first stge pllition; however, it is well known tht this opertion results in more efficient oxygention thn the systemic pulmonry shunt with the dvntge of voiding the volume or pressure overlod of the single ventricle [1]. There re two bsic surgicl techniques for creting cvopulmonry connection, the bidirectionl superior cvopulmonry nstomosis (bidirectionl Glenn opertion) nd the Hemi-Fontn opertion. In those ptients who hve n interruption of the inferior ven cv with zygous or hemizygous continution, bilterl superior cvopulmonry connection opertion is performed with ll the systemic venous 1 Deprtment of Crdiovsculr Surgery, Division of Congenitl Crdic Surgery of Rjie Crdiovsculr Medicl nd Reserch Center, Irn University of Medicl Sciences, Tehrn, Irn. 2 Deprtment of Peditric Crdiology, Rjie Crdiovsculr Medicl nd Reserch Center, Irn University of Medicl Sciences, Tehrn, Irn. 3 Atherosclerosis Reserch Center, Bqiytllh University of Medicl Sciences, Tehrn, Irn. This study ws crried out t Rjie Crdiovsculr Medicl nd Reserch Center, Irn University of Medicl Sciences, Tehrn, Irn. No conflict of interest. No finncil support. Correspondence Address: Gholmrez Omrni Deprtment of Crdiovsculr Surgery Rjie Crdiovsculr Medicl nd Reserch Center, Tehrn 0098, Irn E-mil: gromrni@gmil.com Article received on Februry 3 rd, Article ccepted on My 18 th,

2 Al-Diry A, et l. -Superior Cvopulmonry Connection Opertion Brz J Crdiovsc Surg 2017;32(6):503-7 return is directed to the pulmonry circultion except for the portl venous return, this opertion is clled Kwshim opertion [2]. However, the development of pulmonry rteriovenous mlformtions nd pulmonry rteriovenous fistule remins potentil compliction following Kwshim opertion [2-6]. The ptients who survive the SCPC opertion re potentil cndidtes for the finl pllitive procedure: the Fontn opertion [7]. This study imed to nlyze the outcomes of SCPC opertions in our center nd to identify fctors ffecting the survivl nd the progression to Fontn stge. METHODS Study Protocol nd Popultion Between 2005 nd 2015, 161 ptients with single ventricle physiology due to vrible congenitl hert defects underwent SCPC in our center, Rjie Crdiovsculr Medicl nd Reserch Center. In retrospective study, the outcomes of these ptients concerning the clinicl conditions, the survivl rtes, nd the progression to the finl pllitive stge were nlyzed (Fontn stge). Bseline demogrphics, preopertive, nd intropertive dt were collected from their chrts. This study protocol ws pproved by the locl ethics committee in our institution. Ptients Follow-Up The ptients were regulrly followed up in the outptient clinic (1 week nd 1 month fter surgery, then every 3 months), with complete physicl exmintion nd trnsthorcic echocrdiogrphy (TTE). The follow-up dt were obtined Tble 1. The underlying congenitl hert defects. Congenitl hert defect Vlues TA 60 (37.3%) PS or PA with or without VSD 23 (14.3%) TGA 22(13.7%) cc-tga 11 (6.8%) DILV 10 (6.2%) Mitrl tresi 9 (5.6%) DORV or DOLV with upstirs downstirs ventricles 8 (5%) Heterotxy syndrome 7 (4.3%) Unblnced CAVSD 6 (3.7%) Lrge multiple VSDs 5 (3.1%) All vlues re presented s number (%). CAVSD=complete trioventriculr septl defect; cc-tga=congenitlly corrected trnsposition of gret rteries; DILV= double inlet left ventricle; DOLV=double outlet left ventricle; DORV=double outlet right ventricle; PA=pulmonry tresi; PS=pulmonry stenosis; TA=tricuspid tresi; TGA=trnsposition of gret rteries; VSD=ventriculr septl defect from chrt review, with specil ttention to survivl nd the completeness of the finl pllitive stge. Dignostic Evlutions The min dignostic device ws the TTE for both preopertive dignosis nd postopertive follow-up. For further ntomicl evlution nd especilly for mesuring the men pulmonry rtery pressure (mpap), crdic ctheteriztion ws performed preopertively in 113 (70%) ptients. For those ptients who hd not undergone crdic ctheteriztion, the PAP ws mesured intropertively. Additionlly, computed tomogrphic ngiogrphy (CTA) ws performed in 90 (55.9%) ptients. Sttisticl Anlysis Continuous vrible were presented s men ± SD or medin (interqurtile rnge) s pproprite. Qulittive vribles were presented s frequency nd percentge. Mnn Whitney U test ws used to compre two groups mens nd P vlue < 0.05 ws considered sttisticlly significnt. All sttisticl nlyses were performed using SPSS 20 for windows (IBM Inc., Somers, NY, USA). RESULTS Bseline Chrcteristics Medin ge t SCPC opertion ws 5±4.9 yers (rnge 9 months to 24.5 yers), nd 54% of the ptients were mle (87 ptients). Men mpap preopertively ws 13±3.6 mmhg (rnge 7-27 mmhg). The most common congenitl hert defect in our ptients ws tricuspid tresi (60 ptients, 37.3%). The underlying congenitl hert defects re summrized in Tble 1. Intr- nd Post-Opertive Outcomes Primry SCPC defined s SCPC opertion without ny previous pllitive opertions ws performed in 61 (37.9%) ptients, nd secondry SCPC (with prior pllition) in the reminder. The prior pllitive opertions included systemic pulmonry shunt in 63 (39.1%) ptients, pulmonry rtery bnding (PAB) in 25 (15.5%), PAB with tril septectomy in 5 (3.1%), systemic pulmonry shunt with tril septectomy in 5 (3.1%), nd tril septectomy in two (1.3%) (Figure 1). The predominnt ventricle ws with left ventricle (LV) morphology in 118 (73.3%) ptients, with right ventricle (RV) morphology in 41 (25.5%), nd with intermedite morphology in two (1.2%). The type of the SCPC opertion ws right SCPC in 128 (79.5%) ptients, left SCPC in seven (4.3%), bilterl SCPC in 18 (11.2%), hemi-fontn in two (1.2%), nd Kwshim opertion in six (3.8%) (Figure 2). The opertion ws crried out using crdiopulmonry bypss (CPB) except for 22 ptients in whom right SCPC ws performed without CPB (13.7% of the cohort). The zygous (or the hemizygous) vein ws ligted nd divided in 96 (59.6%) ptients. Previous systemic pulmonry shunt (if existed) ws tken down in 75% of the cses, without ny effect on the outcomes. Concomitnt opertions t the time of SCPC included: repir of pulmonry rtery brnches (n=8), trioventriculr vlve 504

3 Al-Diry A, et l. -Superior Cvopulmonry Connection Opertion Brz J Crdiovsc Surg 2017;32(6):503-7 none shunt PAB with or without PDA closure PDA + septectomy Stge 1 shunt + septectomy coa repir with or without PAB + PDA closure shunt twice septectomy Fig. 1 - The pllitive opertions performed s first stge pllition. Count is expressed s bsolute numbers. coa=corcttion of the ort; PAB=pulmonry rtery bnding; PDA=ptent ductus rteriosus repir (n=3), totl nomlous pulmonry venous connection (TAPVC) repir (n=2), nd tril septectomy (n=2). Four (2.5%) ptients died in the hospitl due to pulmonry infection (two ptients), filure of the SCPC which ws tken down (one ptient), nd low crdic output syndrome with disseminted intr vsculr cogultion (one ptient). The chrcteristics of these ptients re summrized in Tble 2. Men mpap in this group of ptients (in-hospitl mortlity) ws 20±1.63 mmhg, which ws significntly higher thn tht in the survived ptients (12.85±3.44 mmhg), (P=0.001). Two ptients underwent tkedown of the SCPC on the sme dy of opertion; one of them died in the hospitl nd the other ws live fter 2-yer follow-up period. Twelve (7.5%) ptients suffered from prolonged pleurl effusion (> 14 dys), with three of them hving chylothorx. Follow-Up Medin follow-up time fter the SCPC opertion ws 3.1±1.9 yers (rnge 6 months to 10 yers). Two (1.27%) lte deths occurred during the follow-up period, both of them due to hert filure. The rte of freedom from mortlity in the followup period ws 96.27%. Thirty-seven (23.57%) ptients underwent totl cvopulmonry connection (TCPC), nd 99 others (63%) re witing for TCPC. Thirteen (8.3%) ptients were not cndidtes for TCPC due to high PAP (7 ptients of whom three ptients underwent tkedown of the SCPC), poor development of pulmonry rteries (three ptients), ventriculr dysfunction (two ptients), nd virl heptitis (one ptient). No ptient (especilly from those who underwent Kwshim opertions) developed pulmonry rteriovenous fistuls during the period of this study. Fig. 2 - Type of superior cvopulmonry connection. SCPC=superior cvopulmonry connection. Right SCPC in 79.5% of the ptients, left SCPC in 4.3%, bilterl SCPC in 11.2%, hemi-fontn in 1.2%, nd Kwshim opertion in 3.8%. 505

4 Al-Diry A, et l. -Superior Cvopulmonry Connection Opertion Brz J Crdiovsc Surg 2017;32(6):503-7 Tble 2. Chrcteristics of the in-hospitl mortlity ptients. Ptient Age Dignosis Prior pllition Type of SCPC mpap b Associted procedures 1 3 PS without VSD Shunt Right SCPC without pump 18 None 2 6 Unblnced CAVSD PAB Right SCPC with pump 20 None TA+PS None Right SCPC with pump 20 PA brnch repir c 4 1 Heterotxy syndrome None Right SCPC with pump 22 TAPVC repir ge t opertion in yers, b men pulmonry rtery pressure in mmhg preopertively, c pulmonry rtery brnch repir CAVSD=complete trioventriculr septl defect; mpap=men pulmonry rtery pressure; PA=pulmonry tresi; PAB=pulmonry rtery bnding; PS=pulmonry stenosis; SCPC=superior cvopulmonry connection; VSD=ventriculr septl defect; TA=tricuspid tresi; TAPVC=totl nomlous pulmonry venous connection Tkedown of the SCPC Five ptients underwent tkedown of the SCPC (two on the sme dy of SCPC opertion of whom one died, nd three during follow-up). The common denomintor mong these ptients ws the prior pllition with PAB. Furthermore, their men mpap preopertively (17.4±3.29 mmhg) ws significntly elevted when compred with tht of the other ptients (12.87±3.5 mmhg), (P=0.01). All the ptients who survived the tkedown of the SCPC were excluded from the completeness of TCPC due to elevted mpap. The chrcteristics of the ptients who underwent tkedown of the SCPC re summrized in Tble 3. DISCUSSION The erly mortlity rte fter SCPC opertion in our study ws 2.5%. Five (3.1%) ptients underwent tkedown of the SCPC of whom two t the sme opertion dy nd three lter during the follow-up period. The rte of exclusion from the TCPC ws 8.3%. Sttisticl nlysis reveled tht elevted mpap preopertively nd the prior pllition with PAB were risk fctors for both erly mortlity nd tkedown of the SCPC; however, the ge, the morphology of the single ventricle, nd the type of SCPC were not considered risk fctors. The dignosis of lrge multiple ventriculr septl defects or the upstirs downstirs ventricles with double outlet right ventricle or double outlet left ventricle ws ssocited with poor outcomes but due to the smll number of ptients sttisticlly significnt correltion could not be found. Preopertive mpap hs been reported s risk fctor for deth fter the Glenn procedure [8], nd mortlity in those receiving pulmonry rtery bnding ws high [9], nd these findings were comptible with ours. From our perspective it is essentil to protect the pulmonry vsculrity in ptients with single ventricle nd unrestricted pulmonry blood flow since tht the PAP importntly ffects the results of the surgicl pllition in these ptients. There is no consensus regrding the idel time for performing the SCPC in ptients with single ventricle [10]. Age did not seem to influence the outcomes; however, we recommend surgery s erlier s possible, lthough other logistic fctors such s the vilbility of specilized centers nd physicins my ffect the trend to perform the SCPC erlier. The elimintion of n ccessory pulmonry blood flow (prior systemic pulmonry shunt) t the time of SCPC opertion did not ffect the outcomes. On the other hnd, some studies suggested tht this my be dvntgeous on long-term bsis [11]. Pulmonry rteriovenous mlformtions nd pulmonry rteriovenous fistuls did not develop during the follow-up period in this study in ptients who underwent Kwshim opertion. In one study, this compliction rose in 58% of the ptients in medin follow-up period of 5 yers fter Kwshim opertion [6]. Tble 3. Chrcteristics of the tkedown ptients. Ptient Age Dignosis Prior pllition mpap b Time of tkedown Follow-up Lrge multiple VSDs PAB 12 The sme opertion dy 2 yers 2 6 Unblnced CAVSD PAB 20 The sme opertion dy Died in the hospitl 3 3 DORV upstirs/downstirs ventricles PAB 20 After 3.5 yers 9 yers 4 1 DORV upstirs/downstirs ventricles PAB 17 After 3 yers 9 yers 5 2 DOLV upstirs/downstirs ventricles PAB 18 After 3 yers 7 yers ge t SCPC opertion in yers, b men pulmonry rtery pressure in mmhg preopertively. CAVSD=complete trioventriculr septl defect; DORV=double outlet right ventricle; mpap=men pulmonry rtery pressure; PA=pulmonry tresi; PAB=pulmonry rtery bnding; PS=pulmonry stenosis; SCPC=superior cvopulmonry connection; VSD=ventriculr septl defect; TA=tricuspid tresi; TAPVC=totl nomlous pulmonry venous connection 506

5 Al-Diry A, et l. -Superior Cvopulmonry Connection Opertion Brz J Crdiovsc Surg 2017;32(6):503-7 Limittion The retrospective nture of this study is one of its min limittions, nd the short follow-up period in some ptients ws nother considerble one. CONCLUSION The SCPC opertion is n essentil stge for the surgicl pllition in ptients with univentriculr hert nd cn be performed with low rte of mortlity nd morbidity; however, the elevted mpap nd the prior pllition by PAB remin n importnt risk fctors for poor outcomes. Authors roles & responsibilities AAD MGD GO AS AHJ RSA MM MS Substntil contributions to the conception or design of the work; finl pprovl of the version to be Finl pprovl of the version to be Finl pprovl of the version to be Drfting the work or revising it criticlly for importnt Drfting the work or revising it criticlly for importnt Drfting the work or revising it criticlly for importnt Finl pprovl of the version to be Acquisition, nlysis, or interprettion of dt for the work; finl pprovl of the version to be REFERENCES 1. Tnoue Y, Kdo H, Boku N, Ttewki H, Nkno T, Fuke K, et l. Three hundred nd thirty-three experiences with the bidirectionl Glenn procedure in single institute. Interct Crdiovsc Thorc Surg. 2007;6(1): Kwshim Y, Kitmur S, Mtsud H, Shimzki Y, Nkno S, Hirose H. Totl cvopulmonry shunt opertion in complex crdic nomlies: new opertion. J Thorc Crdiovsc Surg. 1984;87(1): Bruh SD, Mishr S, Mrwh A, Shrm R. Heptozygos venous shunt for Fontn completion fter Kwshim opertion. Ann Peditr Crdiol. 2016;9(3): Kvrn MN, Jones JA, Stroud RE, Brdley SM, Ikonomidis JS, Mukherjee R. Pulmonry rteriovenous mlformtions fter the superior cvopulmonry shunt: mechnisms nd clinicl implictions. Expert Rev Crdiovsc Ther. 2014;12(6): Srivstv D, Preminger T, Lock JE, Mndell V, Kene JF, Myer JE Jr, et l. Heptic venous blood nd the development of pulmonry rteriovenous mlformtions in congenitl hert disese. Circultion. 1995;92(5): Brown JW, Ruzmetov M, Vijy P, Rodefeld MD, Turrentine MW. Pulmonry rteriovenous mlformtions in children fter the Kwshim opertion. Ann Thorc Surg. 2005;80(5): Mendoz A, Albert L, Ruiz E, Boni L, Rmos V, Velsco JM, et l. Fontn opertion. Hemodynmic fctors ssocited with postopertive outcomes. Rev Esp Crdiol. 2012;65(4): Petrucci O, Khoury PR, Mnning PB, Eghtesdy P. Outcomes of the bidirectionl Glenn procedure in ptients less thn 3 months of ge. J Thorc Crdiovsc Surg. 2010;139(3): Alsoufi B, Mnlhiot C, Ehrlich A, Oster M, Kogon B, Mhle WT, et l. Results of pllition with n initil pulmonry rtery bnd in ptients with single ventricle ssocited with unrestricted pulmonry blood flow. Thorc Crdiovsc Surg. 2015;149(1): Cnot JF, Allen KR, Coln S, Covitz W, Grhm EM, Hehir DA, et l. Superior cvopulmonry nstomosis timing nd outcomes in infnts with single ventricle. J Thorc Crdiovsc Surg. 2013;145(5): Minwring RD, Lmberti JJ, Uzrk K, Spicer RL, Coclis MW, Moore JW. Effect of ccessory pulmonry blood flow on survivl fter the bidirectionl Glenn procedure. Circultion. 1999;100(19 Suppl):II