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Congenitally corrected transposition and Ebstein's anomaly of the systemic atrioventricular valve: Association with aortic arch obstruction
Corrected Transposition and E1Dstein’s Anomaly of The Systemic Atrioventricular Valve: Asso&ation With Aortic Arch Obstruction Congecita!!y
MB,BS,MSc,FRACP.SEAMUSCULLEN,MB,
DAVIDS.CELERMAJER. JOHN
E. DEANFIELD,
MB. BCHIR. MRCP, IAN
D. SULLIVAN.
BCH.MRCPI,
BMEDSC, MB, CHB. FRACP
l.nn&II, Enplmd
Aortic arch abnormalities are unconnnon in palients wilh congenitally correcled transporiiion of Ihe great arteries. Over a ZO-year period,
10 patients with congenitally
corrected
transposition
and
corrected lmnqmsition and Et&in’s anomaly had mild left AV valveregurgitation; nwe of thesehadaartk arch obstruction. In nwnates
who have coexisting
Ehstein’s
anama~
of the
Ebrtein’s anomaly of the systemic alriovenlrictdar IAV) valve were identitied. Five neonates had sewre qrslemic AV valve regurgitation with were cnarclation orthe aorta tn = 4) oraorlic atreria tn = 1) and presented with heart failure. Four died in the neonatal period and one who had died 7 months pastoperatively. The remaining five patients wllh cor;genltally
systemic AV valve and congenitally cormtrd transposition, obstructionto aorticarchRowis cmnnmn.Severesystemic AV valve regurgitationwith B morphologic systemic ri@ht ventricle may have contributed to low anterograde thaw in the ascending aorta
Congenitally corrected transposition of the great arteries (disccrdant atrioventricular IAVI and vcntriculoarterial connection] is a rare abnormality, accounting for about 0.5% of clinically apparent congenital hean disease (I .Z). Ventricular reptal defect (60% to 70%) and pulmonary stenosis (30% to 50%) are common associations (3.4). but coarctation of the
correcwd transposition and Ebstein’r anomaly of the systemic AV valve. and these constituted the study group. Seven presented as neonates and three as children first seen between 2 and 7 years of age. Clinica! diagnosis. The diagnosis was made by angiocardiography (five patienW or by two-dimensionalechocardiog raphy (five patients presenting after 1980). The anatomy was confirmed in one patient at operation and in three patients at autopsy. All 10 patienis underwent angiocardiography at some stage. Ebstein’s anomaly of the left AV valve was defined as apical displacement of any part of the septal or mural leaflet attachment below the AV anulus. Severe AV valve regurgitation was defined angiograpbically as mpid and complete opacification of the atrium after contrast ventriculography, with failure to clear the contrast medium fmm the atrium After five beats. Mild AV valve regurgitation was defined as incomplete opacification of the atriurh, with clearing of contrast medium within three beats after completion of the injection. Statistics. Statistical cumpariron between groups was performed with use of the chi-square test.
coantatim repair
awta
is rarely
associated
(2.540).
Ten cases in which congenitally corrected transposition was associated with Ebstein’s anomaly of the systemic AV valve are described. In five neonales there was severe left AV valve regurgitation and aorlic arch hypoplasia with aortic atresia or cnarcration. All five died in heart failure in the 1st year of life. In the other live. there was only mild left AV valve regurgiration and no aortic arch abnormality.
Methods
Between
Study patients. 1968 and 1988. 78 patients with Ebstein’s anomaly of the tricwpid valve. 55 of whom were first seen as neonates. and 66 palients v.ith congenitally corrected transposition of rhe great arteries, of whom 33 were first seen as neonates. were examined at our institution. There were IO patients with coexisting congenitally
prwatally
and thereby to the amtIc
arch ahnormalIty.
fJ Am Coil Cm$io11991;18:1056-8)
ReSUltS Incidence. An aortic arch abnormalitv war seen in five 172%) of the seven neonates with congenitally corrected transposhion of the greal artcries and Ebslein’s anomaly. In contrast. an aortic arch abnormality was seen in only 2 (4%) of the 48 neonates with Ebstein’s anomaly of the tricuspid valve and normal cardiac connections (p < O.tX:i and in none of the neonates with congenitatly corrected transposi-
Table 1. Case Details of Five Neonares Wnh Caneenitallv Corrcctcd Tranbpusitionol the Great .Artener. Ehkm’s’ Anomaly of the Syslern~ Alnoventncular Valve And Aartic
tion who had a morphologically
“normal” left AV valve (tricuspid) (p < 0.0011. An abnormality of right ventricular outflow (pulmonary stenosis or atrcsia) was found in 16 (33%) of the 48 neonatcs with Ebstein’s anomaly and normtal cardiac connections The association of congenitally corrected transposition and Ebstein’s anomaly of the left AV valve was found in three patients first seen after the neonatal period. None of these had aortic coarctation or atresia. All five children (Iwo neonates. three older) with congen&ally corrected transposition. Ebstein’s attonx!y and no abnormality of the aortic arch had mild regurgitation of the system;. AV valve only. Clinical features at the five neonates with an aortk arch abnormalily (Table I). All had severe regurgitation of the systemic AV valve. Four (80%) of the five also had a ventricular septal defect. None had pulmonary cu!Row iracl obstruction. In four neonates. angiography revealed a long hypoplastic segment of the aortic arch and isthmus with severe coarctation (Fig. and 21. In one neonate. there was aortic atresia with a hypoplastic aortic arch and isthmus. and the arch was filled retrogradely through an arterial duct. Treatment. In four moribund neonates, the condition was considered inoperable and all died of heart failure in the neonatal period. In the other infant.the aortx coarctation was satisfactorily repaired (Dacron parch aonoplasty) bu1
(1I).
I
the postoperative course was difficuh and artificial ventila-
tion was required for 2 weeks. This infant subsequently had persistent heart failure and died at home 7 months later. All five children with congenitally corrected hxnsposition and Ebstein’s anomaly without an annic arLh abnormality are alive at age 3 to 19 years. One required closure of a ventricular septal d&c” and none required surgical repair of the lefi AV valve.
Discussion are uncommon in congenirally corrected transposition of the great nneries (5-10). Allwork et al. (5) found no case ofaortic coarctation in 32 patients. but did note “hypoplasio of the aortic isthmus in 4 subjects who died in infancy.” Other than these. only I5 Incidence.Amtic
arch abnormahtler
Ftire
1. A~rtomdm in the left anterior nroirclion lrom a ne~nalt congenitaiy corrected tnnrparitik hf the great arteries. Eb~tcin’s anumaly and severe left arrioven~ricularvalve regur&Zion. showinghypoplasiaof the aortic arch and cuarctation. AoA =
&h
xending aorta: AoD = descendingaorta. cases of aortic arch abnormality in 330 cases of congenitally correckd transposition (S-91 have been reported. LundWorn et al. reviewed I cases ofcongenitally corrected
(IO)
II
tnnspowion from IWO institutions; each of the three patients wth Ebstein’s anomaly. sonic coarcfation and nconatal heart failure in that study is included in our patient group.
Only I of Ihe remaining I08 patients had an aorlic arch abnormality Icoarctatiott). The patient with congenrrally corrected transposition and aortic atresia was the subject of a case report (12). Association of aorlk arch abnormalities and sewre left AV valve regurgitation. Our data demonslratc that the combination of congenitally corrected transposition and Ebstein’s anomaly is associated with obstrttctioll of the sonic arch. The arch abnormalities were speckally and predictably related to early presentation with heart failure and severe Ieft AV valve regurgitation. In contrast. the aortic arch was normal m patients with congemtally corrected transposition and Ebstcin‘s anomaly hut only mild systemic AV valve regurgnauon.
Conclusions, We have described five neonates with congenitally corrected transposition and Ebsrein’s anomaly of the systemic AV valve. which was also severely regurgitant. All had associated hvuodasia of the aortic arch with sonic a&a orcoarctation &h all died iil the 1st year of life. This intracardiac anatomy may result in low flow in the ascending aorta in utero and lead to abnormal aortic development. Thus. although aortic arch abnormalities are rare in congenitally corrected transposition, they are common in neonates who have congenitally corrected transposition in combination with Ebstein’s anomaly of the left AV valve. The poor prognosis in this situation is relevant for counseling if fetal echocardiography providesa prenatal diagnosis. We thank Melirra Fulford.Kirby far her prepYalion of Ihe man~r~tipt.
References I. Kidd,
Figure 2. Postmortem specimen from the patient whose aortogram is shown in Figure 1. Note the hypertrophied left atrium ILA) consistent wilh severe regurgitation of the left atrioventricular valve
(LAVV).
The proximal attachments of this valve are displaced
i&ally from the atrioventricular CAV) ring: the probe indicates the reptal leaflet attachment. PV = pulmonary ventricle Imorphologic IeD venlriclci: RA = right atrium: RAVV = right AV YBIYC: SV = syslemic ventricle (morphologic right ventricle).
The combination of severe systemic AV valve regurgitation and a morphologic systemic right ventricle may compromise forward systemic flow. This effect may contribute to hypoplk. of the systemic outflow Iracl. a eiluation lhat is analogous to the common association between severe Ebstein’s acomaly of the right AV valve and hypoplasia ur atka of lne pulmonary outflow tract (pulmonary stenosis or alresia) in a heart with normal connections. In each of our cases, there was hypoplaria of the aortic arch, a finding consistent with low flow in the ascending aorta of the developing fetus. Previous studies. The relation belween hypoplasia of the aortic isthmus and low ascending aortic Row in the fetus was suggested by Rudolph et al. (13). Shinebourne and Elseed (14) analyzed 162 patients with aortic caarctation and concluded that low aortic flow in the Fetus contributed lo development of the coa,&tion. Although Allan et al. (15) in 1984 reported a case in which Fetal coarctation preceded aortic isthmus hypoplasiaand used this case to argue againsi the “feral flow theory.” the morphology in our patients suppons the concept that low Row in the fetal aorta may contribute to ibthnlal hypoplasia and sonic coarctation.
BSL.C~“ngenilally comcled 1ransposilionofIhegrratanencs.I": Keith IA. Rowe RD. Vlad P. eds. Hean Diseasein Infancy am+ Childhood. 3rd cd. New York: Macmillan. 1978:61~27. 2. Bmrke BB. K!dd BSL. Coogenitally caneaed tranrporilion of the peat snrries: a climcal sludr of 101 cases. Acta Paedialr Stand 1976:65zlS& 60. 3. Mullins CE. Vantriculv inwsian. In: Garson A Jr. Uricker JT. McNamara DG. cdr. Pediatric Cardiology. Philadelphia: Lea & Febiger. 1490: 1233-45. 4. Losekom TG. Becker AE. Discordant utrioventricular connecuon and coogenilally con&d tranrpoiitian. In: Andcrron RH. Maurrtnq Fl, Shinrbourne EA.Tynao hl. edr; Paedu.lrieCardiol@y. ill Livir@one. 1987:861-88. 5. Allwark SP. Bemall HH. Becker AE, CI al. Congenitally comct~~l tm”:~~ilio” of Ihc grerr .~rt.ricr. m~rpbotogi~ sredy of 32 ~86~s. Am Cardiol t976:38:9lo-31. 6. SchieblerGL. EdwardsJE. Bumhell HB. Dushanc JW. On& PA, Wood EH. Congenital corrected Irampwition of Ihe art vessels: a study of33 cases. Pedialrics 1%1;27(suppl 111:8Sl-88. 7. FriedbergDZ. Nadas AS. Clinical pra!ile of patients wilh camgenilally corrected transpwtion 0rthc gra arteries:a study ol60cascs. N Eagl J Med 1970:282:1053-9. 8. Anderson RC. Lillehei CW. Lerlcr RG. Corrected lranspwibon of the gre~l VESS& oflhe hcan. Pedirwics 1956:2&626..46. 9. CraigBG. Smallhorn JF. Rowe RD. Williams WC. TruslerGA. Fnedom RF, Severe obslructmn lo systemic blood Row in cdagnilally corrected oansposidon (discordant alrioventricular and veuriculo-arterial convexion& lnt J Cnrdiol 1986:11:209-17. IO. Lundstrom U, Bull C. Wyre RKH. Somerville J. The natural and “u~~nduntl“ history aicongenimllyeonecled tr;m,posllion. AmJ Cardial Ip40’6~~1,,7~9 II. Cclermajrr DS. Cullen S. Sullivan ID. Wyse RKH. Deanfield JE. Whal bappcns 10 wonales with Ebaeini anomaly? Br Hean J 1991:66:52-3. 12. Deanficld JE, Anderson RH. Macarmey FJ. Aortic dresia with “FOP ncted hansposition of IMP great anenes” latriovenoicular and venlriculoaflcrisl discordance,. Br “es, J ,981;46:683& 13. Rudolph AM. Heymann MA. Spetznar U. Hemcdynamic cossiderations inlhe development ornarrowingotthePORB.Am J CardiolI972:3o:Sll25. II. Shinebourne EA. Elwd AM. Relation belwep letal Row patterns. coairtalion of the awm. and pulmontlry blood Row. Br Hean J 1974:363 491-F. IS. Allan LD. Crawford DC. Tynan M. Evolutian ofcoarctabon aftbe wna In lnlmuterine life. Br Heart J 1984,52:471-3.
London: ChurchI
..____
MB,BS,MSc,FRACP.SEAMUSCULLEN,MB,
DAVIDS.CELERMAJER. JOHN
E. DEANFIELD,
MB. BCHIR. MRCP, IAN
D. SULLIVAN.
BCH.MRCPI,
BMEDSC, MB, CHB. FRACP
l.nn&II, Enplmd
Aortic arch abnormalities are unconnnon in palients wilh congenitally correcled transporiiion of Ihe great arteries. Over a ZO-year period,
10 patients with congenitally
corrected
transposition
and
corrected lmnqmsition and Et&in’s anomaly had mild left AV valveregurgitation; nwe of thesehadaartk arch obstruction. In nwnates
who have coexisting
Ehstein’s
anama~
of the
Ebrtein’s anomaly of the systemic alriovenlrictdar IAV) valve were identitied. Five neonates had sewre qrslemic AV valve regurgitation with were cnarclation orthe aorta tn = 4) oraorlic atreria tn = 1) and presented with heart failure. Four died in the neonatal period and one who had died 7 months pastoperatively. The remaining five patients wllh cor;genltally
systemic AV valve and congenitally cormtrd transposition, obstructionto aorticarchRowis cmnnmn.Severesystemic AV valve regurgitationwith B morphologic systemic ri@ht ventricle may have contributed to low anterograde thaw in the ascending aorta
Congenitally corrected transposition of the great arteries (disccrdant atrioventricular IAVI and vcntriculoarterial connection] is a rare abnormality, accounting for about 0.5% of clinically apparent congenital hean disease (I .Z). Ventricular reptal defect (60% to 70%) and pulmonary stenosis (30% to 50%) are common associations (3.4). but coarctation of the
correcwd transposition and Ebstein’r anomaly of the systemic AV valve. and these constituted the study group. Seven presented as neonates and three as children first seen between 2 and 7 years of age. Clinica! diagnosis. The diagnosis was made by angiocardiography (five patienW or by two-dimensionalechocardiog raphy (five patients presenting after 1980). The anatomy was confirmed in one patient at operation and in three patients at autopsy. All 10 patienis underwent angiocardiography at some stage. Ebstein’s anomaly of the left AV valve was defined as apical displacement of any part of the septal or mural leaflet attachment below the AV anulus. Severe AV valve regurgitation was defined angiograpbically as mpid and complete opacification of the atrium after contrast ventriculography, with failure to clear the contrast medium fmm the atrium After five beats. Mild AV valve regurgitation was defined as incomplete opacification of the atriurh, with clearing of contrast medium within three beats after completion of the injection. Statistics. Statistical cumpariron between groups was performed with use of the chi-square test.
coantatim repair
awta
is rarely
associated
(2.540).
Ten cases in which congenitally corrected transposition was associated with Ebstein’s anomaly of the systemic AV valve are described. In five neonales there was severe left AV valve regurgitation and aorlic arch hypoplasia with aortic atresia or cnarcration. All five died in heart failure in the 1st year of life. In the other live. there was only mild left AV valve regurgiration and no aortic arch abnormality.
Methods
Between
Study patients. 1968 and 1988. 78 patients with Ebstein’s anomaly of the tricwpid valve. 55 of whom were first seen as neonates. and 66 palients v.ith congenitally corrected transposition of rhe great arteries, of whom 33 were first seen as neonates. were examined at our institution. There were IO patients with coexisting congenitally
prwatally
and thereby to the amtIc
arch ahnormalIty.
fJ Am Coil Cm$io11991;18:1056-8)
ReSUltS Incidence. An aortic arch abnormalitv war seen in five 172%) of the seven neonates with congenitally corrected transposhion of the greal artcries and Ebslein’s anomaly. In contrast. an aortic arch abnormality was seen in only 2 (4%) of the 48 neonates with Ebstein’s anomaly of the tricuspid valve and normal cardiac connections (p < O.tX:i and in none of the neonates with congenitatly corrected transposi-
Table 1. Case Details of Five Neonares Wnh Caneenitallv Corrcctcd Tranbpusitionol the Great .Artener. Ehkm’s’ Anomaly of the Syslern~ Alnoventncular Valve And Aartic
tion who had a morphologically
“normal” left AV valve (tricuspid) (p < 0.0011. An abnormality of right ventricular outflow (pulmonary stenosis or atrcsia) was found in 16 (33%) of the 48 neonatcs with Ebstein’s anomaly and normtal cardiac connections The association of congenitally corrected transposition and Ebstein’s anomaly of the left AV valve was found in three patients first seen after the neonatal period. None of these had aortic coarctation or atresia. All five children (Iwo neonates. three older) with congen&ally corrected transposition. Ebstein’s attonx!y and no abnormality of the aortic arch had mild regurgitation of the system;. AV valve only. Clinical features at the five neonates with an aortk arch abnormalily (Table I). All had severe regurgitation of the systemic AV valve. Four (80%) of the five also had a ventricular septal defect. None had pulmonary cu!Row iracl obstruction. In four neonates. angiography revealed a long hypoplastic segment of the aortic arch and isthmus with severe coarctation (Fig. and 21. In one neonate. there was aortic atresia with a hypoplastic aortic arch and isthmus. and the arch was filled retrogradely through an arterial duct. Treatment. In four moribund neonates, the condition was considered inoperable and all died of heart failure in the neonatal period. In the other infant.the aortx coarctation was satisfactorily repaired (Dacron parch aonoplasty) bu1
(1I).
I
the postoperative course was difficuh and artificial ventila-
tion was required for 2 weeks. This infant subsequently had persistent heart failure and died at home 7 months later. All five children with congenitally corrected hxnsposition and Ebstein’s anomaly without an annic arLh abnormality are alive at age 3 to 19 years. One required closure of a ventricular septal d&c” and none required surgical repair of the lefi AV valve.
Discussion are uncommon in congenirally corrected transposition of the great nneries (5-10). Allwork et al. (5) found no case ofaortic coarctation in 32 patients. but did note “hypoplasio of the aortic isthmus in 4 subjects who died in infancy.” Other than these. only I5 Incidence.Amtic
arch abnormahtler
Ftire
1. A~rtomdm in the left anterior nroirclion lrom a ne~nalt congenitaiy corrected tnnrparitik hf the great arteries. Eb~tcin’s anumaly and severe left arrioven~ricularvalve regur&Zion. showinghypoplasiaof the aortic arch and cuarctation. AoA =
&h
xending aorta: AoD = descendingaorta. cases of aortic arch abnormality in 330 cases of congenitally correckd transposition (S-91 have been reported. LundWorn et al. reviewed I cases ofcongenitally corrected
(IO)
II
tnnspowion from IWO institutions; each of the three patients wth Ebstein’s anomaly. sonic coarcfation and nconatal heart failure in that study is included in our patient group.
Only I of Ihe remaining I08 patients had an aorlic arch abnormality Icoarctatiott). The patient with congenrrally corrected transposition and aortic atresia was the subject of a case report (12). Association of aorlk arch abnormalities and sewre left AV valve regurgitation. Our data demonslratc that the combination of congenitally corrected transposition and Ebstein’s anomaly is associated with obstrttctioll of the sonic arch. The arch abnormalities were speckally and predictably related to early presentation with heart failure and severe Ieft AV valve regurgitation. In contrast. the aortic arch was normal m patients with congemtally corrected transposition and Ebstcin‘s anomaly hut only mild systemic AV valve regurgnauon.
Conclusions, We have described five neonates with congenitally corrected transposition and Ebsrein’s anomaly of the systemic AV valve. which was also severely regurgitant. All had associated hvuodasia of the aortic arch with sonic a&a orcoarctation &h all died iil the 1st year of life. This intracardiac anatomy may result in low flow in the ascending aorta in utero and lead to abnormal aortic development. Thus. although aortic arch abnormalities are rare in congenitally corrected transposition, they are common in neonates who have congenitally corrected transposition in combination with Ebstein’s anomaly of the left AV valve. The poor prognosis in this situation is relevant for counseling if fetal echocardiography providesa prenatal diagnosis. We thank Melirra Fulford.Kirby far her prepYalion of Ihe man~r~tipt.
References I. Kidd,
Figure 2. Postmortem specimen from the patient whose aortogram is shown in Figure 1. Note the hypertrophied left atrium ILA) consistent wilh severe regurgitation of the left atrioventricular valve
(LAVV).
The proximal attachments of this valve are displaced
i&ally from the atrioventricular CAV) ring: the probe indicates the reptal leaflet attachment. PV = pulmonary ventricle Imorphologic IeD venlriclci: RA = right atrium: RAVV = right AV YBIYC: SV = syslemic ventricle (morphologic right ventricle).
The combination of severe systemic AV valve regurgitation and a morphologic systemic right ventricle may compromise forward systemic flow. This effect may contribute to hypoplk. of the systemic outflow Iracl. a eiluation lhat is analogous to the common association between severe Ebstein’s acomaly of the right AV valve and hypoplasia ur atka of lne pulmonary outflow tract (pulmonary stenosis or alresia) in a heart with normal connections. In each of our cases, there was hypoplaria of the aortic arch, a finding consistent with low flow in the ascending aorta of the developing fetus. Previous studies. The relation belween hypoplasia of the aortic isthmus and low ascending aortic Row in the fetus was suggested by Rudolph et al. (13). Shinebourne and Elseed (14) analyzed 162 patients with aortic caarctation and concluded that low aortic flow in the Fetus contributed lo development of the coa,&tion. Although Allan et al. (15) in 1984 reported a case in which Fetal coarctation preceded aortic isthmus hypoplasiaand used this case to argue againsi the “feral flow theory.” the morphology in our patients suppons the concept that low Row in the fetal aorta may contribute to ibthnlal hypoplasia and sonic coarctation.
BSL.C~“ngenilally comcled 1ransposilionofIhegrratanencs.I": Keith IA. Rowe RD. Vlad P. eds. Hean Diseasein Infancy am+ Childhood. 3rd cd. New York: Macmillan. 1978:61~27. 2. Bmrke BB. K!dd BSL. Coogenitally caneaed tranrporilion of the peat snrries: a climcal sludr of 101 cases. Acta Paedialr Stand 1976:65zlS& 60. 3. Mullins CE. Vantriculv inwsian. In: Garson A Jr. Uricker JT. McNamara DG. cdr. Pediatric Cardiology. Philadelphia: Lea & Febiger. 1490: 1233-45. 4. Losekom TG. Becker AE. Discordant utrioventricular connecuon and coogenilally con&d tranrpoiitian. In: Andcrron RH. Maurrtnq Fl, Shinrbourne EA.Tynao hl. edr; Paedu.lrieCardiol@y. ill Livir@one. 1987:861-88. 5. Allwark SP. Bemall HH. Becker AE, CI al. Congenitally comct~~l tm”:~~ilio” of Ihc grerr .~rt.ricr. m~rpbotogi~ sredy of 32 ~86~s. Am Cardiol t976:38:9lo-31. 6. SchieblerGL. EdwardsJE. Bumhell HB. Dushanc JW. On& PA, Wood EH. Congenital corrected Irampwition of Ihe art vessels: a study of33 cases. Pedialrics 1%1;27(suppl 111:8Sl-88. 7. FriedbergDZ. Nadas AS. Clinical pra!ile of patients wilh camgenilally corrected transpwtion 0rthc gra arteries:a study ol60cascs. N Eagl J Med 1970:282:1053-9. 8. Anderson RC. Lillehei CW. Lerlcr RG. Corrected lranspwibon of the gre~l VESS& oflhe hcan. Pedirwics 1956:2&626..46. 9. CraigBG. Smallhorn JF. Rowe RD. Williams WC. TruslerGA. Fnedom RF, Severe obslructmn lo systemic blood Row in cdagnilally corrected oansposidon (discordant alrioventricular and veuriculo-arterial convexion& lnt J Cnrdiol 1986:11:209-17. IO. Lundstrom U, Bull C. Wyre RKH. Somerville J. The natural and “u~~nduntl“ history aicongenimllyeonecled tr;m,posllion. AmJ Cardial Ip40’6~~1,,7~9 II. Cclermajrr DS. Cullen S. Sullivan ID. Wyse RKH. Deanfield JE. Whal bappcns 10 wonales with Ebaeini anomaly? Br Hean J 1991:66:52-3. 12. Deanficld JE, Anderson RH. Macarmey FJ. Aortic dresia with “FOP ncted hansposition of IMP great anenes” latriovenoicular and venlriculoaflcrisl discordance,. Br “es, J ,981;46:683& 13. Rudolph AM. Heymann MA. Spetznar U. Hemcdynamic cossiderations inlhe development ornarrowingotthePORB.Am J CardiolI972:3o:Sll25. II. Shinebourne EA. Elwd AM. Relation belwep letal Row patterns. coairtalion of the awm. and pulmontlry blood Row. Br Hean J 1974:363 491-F. IS. Allan LD. Crawford DC. Tynan M. Evolutian ofcoarctabon aftbe wna In lnlmuterine life. Br Heart J 1984,52:471-3.
London: ChurchI
..____