130
Pulmonary Atresia with Intact Ventricular Septum: Results and Predictive Factors of Surgical Treatment U. Niederhauser, E. P. Bauer, L. K. von Segesser, T. Carrel, A. Laske, M. Schbnbe ck, and M. Turina Clinic for Cardiovascu lar Surgery, University Hosp ital, Zurich, Switzerla nd
Between 19 70 a nd 1989 26 ch ildren were ope rated for pulmo na ry at res ia wit h intact ventric ula r se ptum (PAlIVS). Accordi ng to the degree of right heart hypoplasia the patients we re d ivided into 3 groups of mild (5), mo derate (17) or severe (4) hypoplasia. Palliative operatio ns were perform ed in 25 ch ildre n (17 male, 8 female) at a mea n age of 10 days : 13 va lvotomies Ivalv.L 5 aortopulmo nary sh u nts , an d 7 valv . plus sh unt . One patient had total correction as pr ima ry procedu re . A total of 17 reoperatlons was necessary in 12 of 26 patie nts (lO pa lliat ions, 7 total correc tions ). Tota l corrections we re : 2 con du its and 5 patch es of the right-ventricula r outflow tr act (RYOn . Tota l mortali ty was 141 26 (54 %) ch ildre n (early 10/2 6 ~ 38 %. late 4/ 26 ~ 16 %). Afte r tota l cor rection mortality was 3/7 (43 %) pa tients . After a mean follow up of 10 .8 years after pall iation the 12 survivo rs are mostly in NYHA class I. Actua ria l surv ival after palliation was 60 %after 30 days a nd 44% after 5 an d 10 years. We a nalyzed 9 clinical an d he modynamic variables by u nivariate a nd multivariate analysis to assess the pred ictive fa ctor s of postop erative ou tco me . Multiva riat e analysis d isclosed the degree of rig ht-ventricular hy poplasia (p = 0.023) as an inde pende nt predicto r for dea th whereas the age at pa lliat ion only approache d significance (p = 0 .0 65). We recommend the followi ng s urgical st rategy. ai ming in th e firs t place at decom press ing the righ t ventricle : in m ild hypoplasia valvoto my a lone or com bine d wit h a shunt for pa lliatio n, in moderate hypo plas ia s hu nt plus pa tch of the RVOT and in severe hypopl asia sh unting alone. In case of a restrictive fora men ova le initial balloon sep tostomy is per formed . For tota l correction in a well deve lope d rig ht ventricle we prefer ASD-clos ure a nd pa tch or th e RYOT if possible with a homograft monocus p. In mo derate or severe hypoplasia a Fe ntan operation is perfor med w ith clos ure of th e ASD an d tr icuspi d or ifice wit h a single pa tch . Key words Pulmonary atresia wit h intact ven tric ula r se ptu m - Palliation Total cor rectio n - Right ventricula r hyp oplasia
Thora c. card iovasc. Surgeon 40 (1992) 130-1 34 © Georg Thieme Verlag Stuttgart · New York
Pulmonalatresi e be i in t aktem Ven trikel septem : Ergeb n isse und prognostische Faktoren der ch ir u rgisc he n Beh andlung Zwischen 1970 und 198 9 w urden 26 Kind er wege n Pulmonal a tres ie mit intaktem Ventrikelse ptum (PAlIYS) an un serer Klinik ope riert. Je n ach Hypoplasiegr ad des recht en Yentrike ls (RY) wurd en die Patienten in 3 Gru ppen eingeteilt mi t geringer (5). rnabiger (17) ode r sc hw ere r (4) Hypoplasie. Bei 25 Kinde m (17 Kna be n. 8 Madc he n) wurden folgen de palliative Oper atton cn im Durchsch nittsalter von 10 Tagen durchgeftlh rt : 13 Yalvoto mien (v alv.). 5 ao rtcpulmonale Shu nts und 7 Valv. plu s Sh unt. Eine Patie nti n wu rde pr imar total korrigiert. Bel 12 von 26 Patienten wa rc n insgesa mt 17 Reoperat ionen n otwendi g (10 palli ativ. 7 Tota lkor rekturen). Als Total kor rekturen wurden 2 Con du its und 5 Patcherweiterungen des rec htsve nt rikularen Aus fluBstraktes {RYOn a usgefuhrt . Die Gesa mt morta lWit betru g 14/ 26 (54 %) Pat ien ten (Frii hmortalitat 10/ 26 = 38%. Spa tmcrtalitat 4/ 26 = 16 %). Nach Totalkorr ektu r be trug d ie Gesamtmcrtali tat 317 (43 °/c0 Patienten. Nach eine m mittler en La ngzeitverlauf von 10 .8 J ahr en . bekann t von allen 12 Uberlebend en. sin d die rneiste n Patienten in NYHA Klasse I. Die ak tua rielle Uber lebensrate nach Palliation betragt60 % nach 30 Tagen und 44 % n ach 5 un d 10 Jahren . Wir analysier te n 9 klinisc he un d h am odyn am isch e Var iab len mit uni- und multivaria te r Analyse zur Bes timm un g von Pradiktoren bezuglich Mortalit at. Aus der Multiva rianzanalyse resultie rte der Gra d der rec hts ve ntrik ularen Hypopla s ie (p = 0 ,02 3) als unabha ngiger Pradiktor. wa hre nd das Alter be ! de r Palliat ion Signifika nz nicht gan z erreichte (p = 0 .06 5). Als Palliation em pfehlen wir folgendes chirurgisc hes vorgehen . welc hes in erster Lin ie den rechte n Ventr ikel dek om p rim ier en soli: bei ge ringe r Hypoplasle cine alle inige Valvoto rnie eventuell kombinie rt mit ein em Sh unt , bei manlger Hypoplasie Sh unt plus Patch des RYOT und bei sc hwere r Hypoplasie nur ei n Sh unt. Bel restri ktivem For am en ovale wird eine initiale Bailonse ptostomie vorg en om men. Als Totalkorrektur bei gut entwik kelte m rec hten Ventrikel em pfehle n wir de n ASD-Verschl uB und Patch des RVOT - we nn moglich mit ein em Hom ogra ft monocusp . Bei mabiger oder schwe re r Hypoplasie fuhren wir eine Fc ntan-O peratio n d urch mit YerschluB des ASD und der Trikuspi dalOlTn ung mit eine m ei nzigen Patch .
Received for Publicalion: November 3. 1991
Downloaded by: Universite Laval. Copyrighted material.
Summary
Thorae. 1day)
0.0225 0.0648 0.253
RV • right ventricle
numbe r of factors suc h as th e deg ree of hypoplasia or righ tventricula r developme nt, es pe cia lly of the nVOT, tri cu spid valve function and size, presence of sinuso ids , and ASD ca paci ty. For this reason ou r pa tients we re divid ed into 3 groups of mild . mod erate. a nd seve re hypopl as ia of their righ t ven tr icle according to pre- a nd intra operati ve find ings. The most decisive factor was the globa l and surgica l relevance of the hyp oplasia of th e right ve ntricle and its suitab ility for a future de finitive co rrection . Othe r possible classification crite ria were described by Goor a nd l.illehei in 19 75 (8). who used the presence of the three right-ventricular components (in let-. tra bec ula r-, outlet pa rt), a nd by I.ewis who ca lculale d a right-ven tri cul ar index (141. These gro upings influen ced surgical technique and pro gnosis just as increasing hypopl asia wa s a significa nt risk factor for death in ou r patients .
Palliat ion: In acco rdance with previous studies (6. 9) transventricular closed va lvotomv es tablished in our se ries also ad equate continu ity between the nVOT a nd the pulm onary arteries in a lmost a ll pa tient s of grou p I. This continu itv was demonstrated bv de Leral (13) and Lewis (141to be a pre req uisite for growth a nd development of th e right ventricle . II reduces the right- ve ntricula r afte rloa d and therefore right-ventricular hypertension which is responsi ble for hyper trop hy . Fu rth ermore in a decom pressed right ventricle sinusoidal-coronary shunting may decrease. These sinuso ids, developed during fetal life, pe rsist becau se of contin uing right-ventricula r hyper tension (12 ). After decomp ression by pu lmon ary va lvotomy alo ne th e right vent ricle how eve r ma y rem ain small and oblite ra ted by hype rt ro phied mus cle. with resu lting ina dequ at e pulmona ry flow as in patients of gro ups II a nd III. Despite se ve ra l cla ssificati on methods in the Iiteralu re (1,13,14. 17 ) th e preoperative assessment of the right-ventricular capaci ty remains difficult and its potential for maintaining an adequate posto perative output and pulmonary flow often ca nnot be predicted with high re lia bility. Addi tiona lly. pulmonarv blood flow can be insufficient due to sign ificant rightto-I~ft shu nti ng a t the a trial level. For thes e rea sons we a dvocate in acco rda nce wit h othe r a ut ho rs (2.10.12. 131 ao rto pulmo na ry sh un tin g in th e majority of pa tien ts with PNI VS. In gro up II with nVOT often obstructe d by muscula r hypertro phy we recomm end the a pplication of a n outflow patch beca use va lvotomy alone does not resu lt in com plete antegrade decompress ion as it only removes the valvar part of th e obs truction . In our se ries 4 patients had to und er go reop erati on with pa tchin g of an obs tructed IlVOT after insufficient initial closed valvotomy. For palliation in gro u p III ao rtopulmonary shunting is sufficient as th eir definitive co rrection cons ists ofa Fontan procedure. If there is seve re hypoplasia (group III) with conco mitant restrictive foramen ovale we prefer atrial balloon se ptostomy at the initia l catheterization wh en prostaglandi n infus ion (I'G1) is a lso instit uted to kee p the duct pa tent . Th is gives time to improve the patient's hemodynamic and metabolic cond ition . Early age a t palliation (,.; lday) wa s a significa nt risk factor for death in our patients . Total correc tion: For choos ing the appropriate definitive procedure a classification in mild. moderate. and seve re hypopla sia is also useful. Total correction is per form ed du ring a sho rt normothermic cardiopulmonary bypass and con sist s of ASD closure an d rem oval of the syste mic to pu lm onary shunt. In a number of pati en ts of gro up I tota l correction may not be indicated where growth of the ventricular cavity and tricuspid valve is sufficient and obstructio n of the nVOT is neglectable. Thi s wa s the case in 4 of our pati ents in gro up I wh o are all in goo d con dition an d NYHA class I afte r a mean interval of 14 .6 years. Th ey need only ASD clos ure and shunt ligat ion as de finitive correction . In a ll othe r pati en ts of gro up I with perma nent IlVOT obstruction transannula r enla rge ment is indica ted with a patch or if possi ble with a hom ogr aft monocusp to get a com petent pulmonary valve. If moderate or seve re hypoplasia persists a Fontan operation modified after Kreutz er is preferred, co nnec ting the right a trium with th e pulmonary a rte ry and closin g the ASD a nd tricusp id or ifice with a single patch (I , 3). The Fonta n proc ed ur e is a lso indicated in ca se of well developed sinu soids but suffi cient antegrade coronary perfusion.
133
Downloaded by: Universite Laval. Copyrighted material.
Thorac. cardim·as c. Surgeo fl 40 (1992)
Pulmonary Atresia with Intact Ventricular Septum
Thorac. cardioaasc. Surgeon 40 (1992)
U. Ni ederhauser. et a i.
Referen ces A /boliras. E. T.• P. R. Julsrud; G. K. Danielson. F. J. Puga. H. V. Sc haff. D. C. McGoon. D. J. Hagler. W D. Ed wards. and D. J. Driscoll: Definitive ope ration for pulm onary a tre sia with in ta ct ven tr icular septum. J. Thor ae. Cardiovasc. Surg . 93 (1987) 454 -464 2 Bar ragry. T. P.• W. S. Ring. J. W. Blat chford. andJ. E. Foker:Central aorta -pu lmona ry arte ry shunts in neonates with compl ex cyanotic congenital heart diseas e. J. Thora e. Cardiovasc . Surg. 93 (1987) 767 -774 3 Billingsley, A. M.• H. Laks. S. W. Boyce . B. George. T. Santulli. and R. G. William s: Definitive repa ir in pa tients wit h pulmon a ry at res ia and intact vent ricular septum. J . Thorae. Cardiovasc. Surg. 97 0 9891746 -7 54 .. Bu ll. C . M. R. de Leoal. C. M ercanti. F. 1. Ma cartney. R. H. Anderson. and M. R. C. Path : Titel titel. Circulation 66 , (1982) 266- 271 5 Cutler. S. J.. and F. Ed erer: Maximum utilizati on of the life tab le meth od in analyzing survival. J. Chron . Dis. 8 (1958) 699 -712 6 Dobel, A. R. c.. and A. Grignon: Ear ly and late results in pulmonary atresia. Ann . Thor ac. Surg. 24 (1977) 264 7 Freed om. R. M.: Pulmonary atresia with inta ct ventricular septum. Futu ra Publishing Company 1989 8 Goor. D. A.. and C. at. Lilleh ei.- Congenital malform at ions of th e heart. New York. Gro ne & Stratton 197 5 9 Grah am. T. P.. H. at. Bender. G. F Atwood. D. L. Page. and C. G. R. S ell: Incre as e in right ventri cular volume following valvulotomy for pu lmonary atresia or sten osis with intact ventricular septum . Circulation 50 (1974) Suppl III: 11- 69 10 Kanter. K. R.. D. G. Pennington. S. Nouri. S. Chen. and S. Jureidini: Concomita nt valvotomy and subclavi an-m ain pulmonary artery I
11 12
Il
14
15
16
17
sh unt in neon ates with pulmonary atresia and intact ventricular septum . Ann. Thorac. Surg . 43 (1987 ) 490-494 Keith . J . D.• R. D. Rowe. and P. Vlad: Heart disease in infancy and childhood. 3 nl ed. London. Macmillan, 1978 Leks. H.• and A. M. Billingsley : Advanc es in th e treatment of pulmona ry atresia with intact ven tricular septum : palliativ e and definitive repair . Cardiology Clinics 7 (1989 ) 387- 398 De Leoa t. M.• C. Bull. J. S tark. R. n. And erson. F. R. C. Path. J. F. N. Taylor. and F J . Macartney : Pulmonary atresia and intact ventricu lar septum: Surgica l management base d on a revised classification . Circulation 66 (1982) 272-280 Lewis. A. B.. W Wells. and G. G. Lindesmith: Right ventricular growth potential in neonater s with pulmona ry atresia and intact ven tricular septum. J . Thorac . Cardiovasc . Su rg . 9 1 119R6) 835-R40 Lewis, A. B.• W. Wells. and G. G. Lindesm ith : Evaluat ion and surgical treatment of pulmonary atresia and intact vent ricular septum in infan cy. Circulation 67 (1983) 1318 - 1323 Patel. R. G.. R. M. Freedom. a nd C. A. F. Moes: Right ven tricular volume dete rminations in 18 patients with pulmonary at resia and intact ventricular sep tum . Circulation 67 (1983 ) Zuberbuhler. J. R.. and R. H. A nde rson: Morpholo gical varia tions in pulm onary atresia with intact vent ricular se ptum. Br. Heart J . 41 (1979)281
Dr. U. Nie de rhauser
Klinik fiir Herzgefaflchiru rgie Universitatsspital CH·8091 Zurich Switzerland
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134
Pulmonary Atresia with Intact Ventricular Septum: Results and Predictive Factors of Surgical Treatment U. Niederhauser, E. P. Bauer, L. K. von Segesser, T. Carrel, A. Laske, M. Schbnbe ck, and M. Turina Clinic for Cardiovascu lar Surgery, University Hosp ital, Zurich, Switzerla nd
Between 19 70 a nd 1989 26 ch ildren were ope rated for pulmo na ry at res ia wit h intact ventric ula r se ptum (PAlIVS). Accordi ng to the degree of right heart hypoplasia the patients we re d ivided into 3 groups of mild (5), mo derate (17) or severe (4) hypoplasia. Palliative operatio ns were perform ed in 25 ch ildre n (17 male, 8 female) at a mea n age of 10 days : 13 va lvotomies Ivalv.L 5 aortopulmo nary sh u nts , an d 7 valv . plus sh unt . One patient had total correction as pr ima ry procedu re . A total of 17 reoperatlons was necessary in 12 of 26 patie nts (lO pa lliat ions, 7 total correc tions ). Tota l corrections we re : 2 con du its and 5 patch es of the right-ventricula r outflow tr act (RYOn . Tota l mortali ty was 141 26 (54 %) ch ildre n (early 10/2 6 ~ 38 %. late 4/ 26 ~ 16 %). Afte r tota l cor rection mortality was 3/7 (43 %) pa tients . After a mean follow up of 10 .8 years after pall iation the 12 survivo rs are mostly in NYHA class I. Actua ria l surv ival after palliation was 60 %after 30 days a nd 44% after 5 an d 10 years. We a nalyzed 9 clinical an d he modynamic variables by u nivariate a nd multivariate analysis to assess the pred ictive fa ctor s of postop erative ou tco me . Multiva riat e analysis d isclosed the degree of rig ht-ventricular hy poplasia (p = 0.023) as an inde pende nt predicto r for dea th whereas the age at pa lliat ion only approache d significance (p = 0 .0 65). We recommend the followi ng s urgical st rategy. ai ming in th e firs t place at decom press ing the righ t ventricle : in m ild hypoplasia valvoto my a lone or com bine d wit h a shunt for pa lliatio n, in moderate hypo plas ia s hu nt plus pa tch of the RVOT and in severe hypopl asia sh unting alone. In case of a restrictive fora men ova le initial balloon sep tostomy is per formed . For tota l correction in a well deve lope d rig ht ventricle we prefer ASD-clos ure a nd pa tch or th e RYOT if possible with a homograft monocus p. In mo derate or severe hypoplasia a Fe ntan operation is perfor med w ith clos ure of th e ASD an d tr icuspi d or ifice wit h a single pa tch . Key words Pulmonary atresia wit h intact ven tric ula r se ptu m - Palliation Total cor rectio n - Right ventricula r hyp oplasia
Thora c. card iovasc. Surgeon 40 (1992) 130-1 34 © Georg Thieme Verlag Stuttgart · New York
Pulmonalatresi e be i in t aktem Ven trikel septem : Ergeb n isse und prognostische Faktoren der ch ir u rgisc he n Beh andlung Zwischen 1970 und 198 9 w urden 26 Kind er wege n Pulmonal a tres ie mit intaktem Ventrikelse ptum (PAlIYS) an un serer Klinik ope riert. Je n ach Hypoplasiegr ad des recht en Yentrike ls (RY) wurd en die Patienten in 3 Gru ppen eingeteilt mi t geringer (5). rnabiger (17) ode r sc hw ere r (4) Hypoplasie. Bei 25 Kinde m (17 Kna be n. 8 Madc he n) wurden folgen de palliative Oper atton cn im Durchsch nittsalter von 10 Tagen durchgeftlh rt : 13 Yalvoto mien (v alv.). 5 ao rtcpulmonale Shu nts und 7 Valv. plu s Sh unt. Eine Patie nti n wu rde pr imar total korrigiert. Bel 12 von 26 Patienten wa rc n insgesa mt 17 Reoperat ionen n otwendi g (10 palli ativ. 7 Tota lkor rekturen). Als Total kor rekturen wurden 2 Con du its und 5 Patcherweiterungen des rec htsve nt rikularen Aus fluBstraktes {RYOn a usgefuhrt . Die Gesa mt morta lWit betru g 14/ 26 (54 %) Pat ien ten (Frii hmortalitat 10/ 26 = 38%. Spa tmcrtalitat 4/ 26 = 16 %). Nach Totalkorr ektu r be trug d ie Gesamtmcrtali tat 317 (43 °/c0 Patienten. Nach eine m mittler en La ngzeitverlauf von 10 .8 J ahr en . bekann t von allen 12 Uberlebend en. sin d die rneiste n Patienten in NYHA Klasse I. Die ak tua rielle Uber lebensrate nach Palliation betragt60 % nach 30 Tagen und 44 % n ach 5 un d 10 Jahren . Wir analysier te n 9 klinisc he un d h am odyn am isch e Var iab len mit uni- und multivaria te r Analyse zur Bes timm un g von Pradiktoren bezuglich Mortalit at. Aus der Multiva rianzanalyse resultie rte der Gra d der rec hts ve ntrik ularen Hypopla s ie (p = 0 ,02 3) als unabha ngiger Pradiktor. wa hre nd das Alter be ! de r Palliat ion Signifika nz nicht gan z erreichte (p = 0 .06 5). Als Palliation em pfehlen wir folgendes chirurgisc hes vorgehen . welc hes in erster Lin ie den rechte n Ventr ikel dek om p rim ier en soli: bei ge ringe r Hypoplasle cine alle inige Valvoto rnie eventuell kombinie rt mit ein em Sh unt , bei manlger Hypoplasie Sh unt plus Patch des RYOT und bei sc hwere r Hypoplasie nur ei n Sh unt. Bel restri ktivem For am en ovale wird eine initiale Bailonse ptostomie vorg en om men. Als Totalkorrektur bei gut entwik kelte m rec hten Ventrikel em pfehle n wir de n ASD-Verschl uB und Patch des RVOT - we nn moglich mit ein em Hom ogra ft monocusp . Bei mabiger oder schwe re r Hypoplasie fuhren wir eine Fc ntan-O peratio n d urch mit YerschluB des ASD und der Trikuspi dalOlTn ung mit eine m ei nzigen Patch .
Received for Publicalion: November 3. 1991
Downloaded by: Universite Laval. Copyrighted material.
Summary
Thorae. 1day)
0.0225 0.0648 0.253
RV • right ventricle
numbe r of factors suc h as th e deg ree of hypoplasia or righ tventricula r developme nt, es pe cia lly of the nVOT, tri cu spid valve function and size, presence of sinuso ids , and ASD ca paci ty. For this reason ou r pa tients we re divid ed into 3 groups of mild . mod erate. a nd seve re hypopl as ia of their righ t ven tr icle according to pre- a nd intra operati ve find ings. The most decisive factor was the globa l and surgica l relevance of the hyp oplasia of th e right ve ntricle and its suitab ility for a future de finitive co rrection . Othe r possible classification crite ria were described by Goor a nd l.illehei in 19 75 (8). who used the presence of the three right-ventricular components (in let-. tra bec ula r-, outlet pa rt), a nd by I.ewis who ca lculale d a right-ven tri cul ar index (141. These gro upings influen ced surgical technique and pro gnosis just as increasing hypopl asia wa s a significa nt risk factor for death in ou r patients .
Palliat ion: In acco rdance with previous studies (6. 9) transventricular closed va lvotomv es tablished in our se ries also ad equate continu ity between the nVOT a nd the pulm onary arteries in a lmost a ll pa tient s of grou p I. This continu itv was demonstrated bv de Leral (13) and Lewis (141to be a pre req uisite for growth a nd development of th e right ventricle . II reduces the right- ve ntricula r afte rloa d and therefore right-ventricular hypertension which is responsi ble for hyper trop hy . Fu rth ermore in a decom pressed right ventricle sinusoidal-coronary shunting may decrease. These sinuso ids, developed during fetal life, pe rsist becau se of contin uing right-ventricula r hyper tension (12 ). After decomp ression by pu lmon ary va lvotomy alo ne th e right vent ricle how eve r ma y rem ain small and oblite ra ted by hype rt ro phied mus cle. with resu lting ina dequ at e pulmona ry flow as in patients of gro ups II a nd III. Despite se ve ra l cla ssificati on methods in the Iiteralu re (1,13,14. 17 ) th e preoperative assessment of the right-ventricular capaci ty remains difficult and its potential for maintaining an adequate posto perative output and pulmonary flow often ca nnot be predicted with high re lia bility. Addi tiona lly. pulmonarv blood flow can be insufficient due to sign ificant rightto-I~ft shu nti ng a t the a trial level. For thes e rea sons we a dvocate in acco rda nce wit h othe r a ut ho rs (2.10.12. 131 ao rto pulmo na ry sh un tin g in th e majority of pa tien ts with PNI VS. In gro up II with nVOT often obstructe d by muscula r hypertro phy we recomm end the a pplication of a n outflow patch beca use va lvotomy alone does not resu lt in com plete antegrade decompress ion as it only removes the valvar part of th e obs truction . In our se ries 4 patients had to und er go reop erati on with pa tchin g of an obs tructed IlVOT after insufficient initial closed valvotomy. For palliation in gro u p III ao rtopulmonary shunting is sufficient as th eir definitive co rrection cons ists ofa Fontan procedure. If there is seve re hypoplasia (group III) with conco mitant restrictive foramen ovale we prefer atrial balloon se ptostomy at the initia l catheterization wh en prostaglandi n infus ion (I'G1) is a lso instit uted to kee p the duct pa tent . Th is gives time to improve the patient's hemodynamic and metabolic cond ition . Early age a t palliation (,.; lday) wa s a significa nt risk factor for death in our patients . Total correc tion: For choos ing the appropriate definitive procedure a classification in mild. moderate. and seve re hypopla sia is also useful. Total correction is per form ed du ring a sho rt normothermic cardiopulmonary bypass and con sist s of ASD closure an d rem oval of the syste mic to pu lm onary shunt. In a number of pati en ts of gro up I tota l correction may not be indicated where growth of the ventricular cavity and tricuspid valve is sufficient and obstructio n of the nVOT is neglectable. Thi s wa s the case in 4 of our pati ents in gro up I wh o are all in goo d con dition an d NYHA class I afte r a mean interval of 14 .6 years. Th ey need only ASD clos ure and shunt ligat ion as de finitive correction . In a ll othe r pati en ts of gro up I with perma nent IlVOT obstruction transannula r enla rge ment is indica ted with a patch or if possi ble with a hom ogr aft monocusp to get a com petent pulmonary valve. If moderate or seve re hypoplasia persists a Fontan operation modified after Kreutz er is preferred, co nnec ting the right a trium with th e pulmonary a rte ry and closin g the ASD a nd tricusp id or ifice with a single patch (I , 3). The Fonta n proc ed ur e is a lso indicated in ca se of well developed sinu soids but suffi cient antegrade coronary perfusion.
133
Downloaded by: Universite Laval. Copyrighted material.
Thorac. cardim·as c. Surgeo fl 40 (1992)
Pulmonary Atresia with Intact Ventricular Septum
Thorac. cardioaasc. Surgeon 40 (1992)
U. Ni ederhauser. et a i.
Referen ces A /boliras. E. T.• P. R. Julsrud; G. K. Danielson. F. J. Puga. H. V. Sc haff. D. C. McGoon. D. J. Hagler. W D. Ed wards. and D. J. Driscoll: Definitive ope ration for pulm onary a tre sia with in ta ct ven tr icular septum. J. Thor ae. Cardiovasc. Surg . 93 (1987) 454 -464 2 Bar ragry. T. P.• W. S. Ring. J. W. Blat chford. andJ. E. Foker:Central aorta -pu lmona ry arte ry shunts in neonates with compl ex cyanotic congenital heart diseas e. J. Thora e. Cardiovasc . Surg. 93 (1987) 767 -774 3 Billingsley, A. M.• H. Laks. S. W. Boyce . B. George. T. Santulli. and R. G. William s: Definitive repa ir in pa tients wit h pulmon a ry at res ia and intact vent ricular septum. J . Thorae. Cardiovasc. Surg. 97 0 9891746 -7 54 .. Bu ll. C . M. R. de Leoal. C. M ercanti. F. 1. Ma cartney. R. H. Anderson. and M. R. C. Path : Titel titel. Circulation 66 , (1982) 266- 271 5 Cutler. S. J.. and F. Ed erer: Maximum utilizati on of the life tab le meth od in analyzing survival. J. Chron . Dis. 8 (1958) 699 -712 6 Dobel, A. R. c.. and A. Grignon: Ear ly and late results in pulmonary atresia. Ann . Thor ac. Surg. 24 (1977) 264 7 Freed om. R. M.: Pulmonary atresia with inta ct ventricular septum. Futu ra Publishing Company 1989 8 Goor. D. A.. and C. at. Lilleh ei.- Congenital malform at ions of th e heart. New York. Gro ne & Stratton 197 5 9 Grah am. T. P.. H. at. Bender. G. F Atwood. D. L. Page. and C. G. R. S ell: Incre as e in right ventri cular volume following valvulotomy for pu lmonary atresia or sten osis with intact ventricular septum . Circulation 50 (1974) Suppl III: 11- 69 10 Kanter. K. R.. D. G. Pennington. S. Nouri. S. Chen. and S. Jureidini: Concomita nt valvotomy and subclavi an-m ain pulmonary artery I
11 12
Il
14
15
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Dr. U. Nie de rhauser
Klinik fiir Herzgefaflchiru rgie Universitatsspital CH·8091 Zurich Switzerland
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