Case Report 92

Truncus Arteriosus Communis Associate d with Interrupted Aortic Arch:A Report on two Uncommon Cases J H. . Skalski. H. H. Sievers. J Funda, D. Regensburger. andA. Bernhard DepartmentsofCardiovascuJar Surgery andPediatric Cardiologyofthe UniversityofKiel. Kiel. Germany * now Departmentof'Cardtac Surgery.Silesian HeartCenter. Silesian"1edicalAcademy. Katowice. Poland

The paper presents two infants with the A·4 type of truncus arteriosus communis (according to Van Pra ugh's classification). One patient who survived a surgicalprocedure demonstrated a rare variant of aortic arch interruption to the left off the left subclavian artery (type A according to Celoria and Patton) . whereas the second presented an uncommon anomaly in which the right subclavian arteryoriginated fromthe descending aorta with associated severe truncal valve incompetency. The authors describe the clinical picture along with the surgial treatment of the first infant who being six days old was subjected to a correction employing the wide patent ductus arteriosus to reconstruct the aortic arch. following the method described by Gomes andMcGaan. Subsequently an aortichomograft was implanted in order to connect the right ventricle and the pulmonary artery.

Keywords

Truncus arteriosus communis mit eine r Atres ic des Aortenbogens - Bericht tiber l wei Sunde r falle Die vorliegende Arb eit stellt zwei Saugllngsfalle mit dem Truncus arteriosus communis-Typ A4 dar (Klassifizierung nach der VanPraagh-Typologie). Einerder Pat ienten. der die chirurgische Behandlung tiberlebte. hatte eine seltene Variante der Atresie des Aortenbogens und zwar links von der Arteria subclavia (Typ A nach der Cetaria- und Patton-Typologie). Der andere Patient zeigte cine ungewtih nliche Anomalie. der gestalt. daB die reehte Arteria subc1avia von der Aorta deseendens entspra ng. auBerdem lag eine schwere Truncus-Klappeninsuffizienz vor. Die Autoren stellen das klinische Bild zusammen mit der ehirurgischen Behandlung des einen Sa ugltngs dar. derim Alter von 6 Tagen mit Hilfe cines weiten Ductus arteriosus (nach der Gomes- und McGoan -Methode) operiertwurde. Zwecks Verbin dung des rechten Ventrikels mit der Pulmonalarterie wurde an schliebend ein aortaler Homograft implantiert.

Truncus arteriosus - Interruption of aortic arch - Aortic arch anomalies - Conotruncal anomalies - Congenital heart disease

Truncus arteriosus communis (TAl ass ociated with a severe additional lesian in the sh ap e of interrupted aorti c arch (lAA) is an uncomm on anomaly. However. it has bee n am enabl e to corrective surgery since 1971 (13). 1M was observed in 4-1 9 % ofTA (3. 4.10.25) being -together with ather types af aortic ar ch hypoplasia - the third most common form of TA (4, 25). The defect has been class ified by Van Praagh as A-4 (25). In addition. typically, the interruption of aa rtic arch occurs between the left common carotid artery and the left subclavian art ery (1M type B) (3, 4). Ta the best ofa ur knowledge, the present report is uniqu e as it conce rns the case of a successful total repair of TA associated with lAAdistal to the left sub clavian art ery (1M type A). The second pati ent presented is unique in that the right subclav ian arte ry originated from the descending aorta.

Case 1 A female newborn (birth weight 2930g) was admitted to our department an the day of birth because of the tentative diagnosis of truncus arteriosus comm unis . Two-dimens iona l echocardiography confirmed the diagno sis then ca rdiac

Thorac. cardiovasc. Surgeon 40 (992) 92-95 © GeorgThieme Verlag Stuttgart- New York

catheterization with angiog raphy determined the anomaly as type A-4 (according to the Van Praagh classificatian) and sh owed a large, high ventricular se ptal defect with overriding of the truncal valve. The truncus arteriosus was asso ciated with the interruption of the aa rtic a rch distalta the origin of the left sub clavian arte ry (Fig. 1). The pulmonary art erial compa nent of the truncus was domina nt. and the aortic portion was rath er narrow. The pulmonary art ery arose dir ectly from the poster ior as pect of the truncus in close proximity, type II by Collet an d Edwards (8). The descending aorta was supplied by a wide pat ent ductus arte rias us (Fig. 1C). Identical pressur e values were noted in both ventricles, whereas no pressure gradients wer e seen in the tricuspid. mitral. and trun cal valves. A slight regurgitation existed in the tricuspid and truncal valves. The pulmonary flow relatian Qp : Qs was greater than 3. the pulmonary resistan ce was normal. Permanent infusion of prostaglandin E, was instituted. On the 6th day oflife the ea rly corrective ap eration was perform ed via standard med ian sternoto my. The proximal common trunk was cannulated highly cranially and cardiopulmonary bypass was commenced along with the occlu-

Received for Publicat ion : March 15. 1991

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Summar)'

Truncus Art eriosus Communis Associa ted with Interrupted Aortic Arch Fig. 1 Case I . Anteroposteriorviewof the heart- a)leftventricular cineangiogram showingthe common trunk,itsbranches and pulmonary arteries withnoobstructions,

Thom e. ea rdiovasc. Surgeon 40 (1992)

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Fig. 2 Case I . Operative procedure hg-homograft (aortic), LPA-left pulmonary artery, RPA-right pulmonaryartery, LS-Ieft subclavianartery, Pknitted Dacron conduit patch. Note- VSDisoccluded, pulmonary artery detachedandthe createdaperturerepaired by anautologuepericardial patch.

Fig. 1 a

sion of the pulmonary arteri es to avoid an overflow of th e lungs. Subsequently th e pati ent was cooled down to 20' C. The ao rta was clamped and the cold cardioplegic solu tion wa s infused into the truncal artery to indu ce cardiac arrest.

Fig.lb

c) diagramatic representation ofthegreat arteries. LS-Ieftsubclavianartery. LPA-Ieftpulmonary artery,RPA-right pulmonaryartery, PDApatent ductus arteriosus,Tr-commontrunk, VS D-ventricularsepta! defect.DA = descendingaorta.

Pulmonary arteri es wer e detached from the tru ncus and th e defect In the trun cal artery wall was repaired by an autologue pericardia! patch. Then. after closure of th e VSD by a Dacron-Velour patch usin g vertical ventriculotomy, the continuity between th e right ventricle and pulmonary arteries was established. employing an 11 mm aortic homograft extend ed proximally with a knitt ed Dacron conduit pat ch placed in its lower part. The wid e pat ent ductus arte riosus was used for the aortic arch reconstruction, thus making continuit y between th e truncus and th e descending aorta possible (Fig. 2). Becau se of hemod ynami cally effective compression of the conduit the middl e part of the sternum had to be resec ted and the superior and inferi or parts we re fixed using excised bony fragments. The postoperative course then was complicated only by a chylothorax, which required ches t dr ain age up to the 21th postoperative da y as well as medium- chain-triglicerid e (MCT)diet. No furt he r effusion was obse rved in the following period. After extubat ion on th e 22n d da y, respiration was only slightly disturbed by thoracic instabilit y. The echocardiogra phic exploration showe d a good result of correction: the right ventricular outflow tract

and the conduit we re wide without regurgation or sten osis of the homo gr aft and the trun cal valves. The function of th e left and right ventricles were satisfactory. On discharge, seve n weeks postop eratively, the condition of th e patient was rem ark ably good. Dyspnea due to moderat e instabilit y of th e chest was not observed. Case 2 A one-da y-old newb orn girl (b. w. 3500 g) was admitted in poor gene ral condition, with central cyanosis and tac hypnea as w ell as systolic and diastolic murmurs . The echoca r-

diographic finding s were confirme d by angiocardiography: a truncus ar teriosus communis of the type I by Collet and Edwards (8) combined with an aortic arch int erruption,

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b)diagramshowing anatomyof the heart andgreat arte ries.

Thome. cardiovasc. Surgeon 40 (1992)

J. H. Ska lski, H. H. Siev ers, J. Funda, D. Regensburger, and A. Bernhard Fig. 3 case 2.Anteroposteriorview ofthe heart - a)leftventricular clneangiogram demonstratingtruncal branch relations. " arteria lusoria.

ant erosuperiorly located VSD, pat ency of ductus arteriosus, and the right subcla vian artery aberrantly originating from the descending aorta (arte ria lusori a)-(Fig. 3). The most limiting factor was a severe regurgitation of the quadricuspid truncal valve. The smaller aortic part of the truncus led to both carotids. The dominant pulmonary part of the tru ncus was continu ed by a wide patent ductus arteriosus supplying the descendin g aorta wherein two subclavian arteries origi-

nat ed , the right one being most distal. No pressur e gradient was found in the truncal, tricuspid an d mitral valves. The pulmonary flow relation Qp : Qs was more than 3 and normal pulmonary resistan ce could be conflrmed. During severe bron chopn eumoni a the child died due to cardio-respira tory failure - before surgery could be performed . Fig.3a

Disc ussion b)diagram showing anatomyoftheheart andgreat arteries,

Truncus arteriosus communis with ass ociated interrupted aortic arch is an extremely uncommon defect, and each new communication on this subject contributes to our know-

ledge on tr eatme nt of this rare anomaly. The flrst case ofTA with as sociated 1M was described in 1831 (ace, to 23). Subsequently, in 1965 Van Praagh classifled the defect as A4 type of TA (25). Others do not subdi vide types of the truncus arteriosus by their associated lesions (6). In 1962 the anomaly was described for the flrst time in its form consisting ofinte rruption ofl he aortic arch located to the left ofT the left subclavian artery (21) [Type A-1AA according to Celoria and Patton (7)]. This combination has been even more infrequently present ed in literature (1, 4, I S, 19, 21) and the clinical reports have been extremely scarce (15,1 9, 21). The A-4 TA type was observed over 60 times (12) and the majority of papers were base d upon autop sies. It is difficult to determine pr ecisely the numb er of cases noted so far, since the authors tended to repeat dat a concern ing

RPA

patients from their own and others' series , some times quoting the same patients twice.

Fig.3b

Clinical description of patients after success ful surgeries are few; so far only ten docum ented survivals have been c) diagramaticrepresentation ofthegreat arteries. LS-Ieftsubclavianartery, RS-right subclavian artery, LPA-left pulmonary artery, RPA-right pulmonaryartery, PDA-patent ductusarteriosus,Tr-common tru nk,VS D-ventricular septaldefect.

noted since the flrst successful operation perform ed in 1971 (9, 10, 12, 17, 18). To the best of our knowledge all described cases were the combination characterized by the B type of interrupted aortic arch. Such a combination is not acciden-

tal, and, as it has been emph asized from the embryological viewpoint, TA with 1M type B both belong to the conotru ncal anomalies, where TA repre sents the extreme form of conotru ncal malformations . Both defects are often cha rac terized by familial recurrence. The pr esent paper describes an infant with the Atype of 1M , and to the best of our knowledge is the flrst communication on the successful surgical outcome . Moreover, the

child in question belongs to the group of youngest patient s with A-4 TA subjected to surgery (12,1 7,1 8). The operation was performed according to the method described in 1971 by Gomes and McGoon (13), employing the wide patent ductus arteriosus to reconstruct the aortic arch. This techni -

que is readily used (9, 10), nevertheless, it ha s been recently criticized (12, 17) for the involved theoretical danger of future closure or stricture of th e operated seg me nt, as usua l-

ly happ ens with patent ductus arteriosus following a nat ural tendency. However , the literature present s only one case of such complication (10),which - as it has been emph asized could have been avoided by leaving a cufT of the pulmonary artery wall (9) and employing sutures located outside of the

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Pp Awa ll. Such pre cautions were adopted in our case. Moreover. in view of the fact that a homog raft valve condui t (aortic) was employed in a very young newbo rn the possibi lity of a reoperation exists (II), during which, shou ld it become necessery, the aortic arc h plastic operation might be undertaken . Moreover the long distan ce between the ascend ing a nd descending aorta in our pati ent rend er ed direct an astomosis difficult. The difficulties we encountered following the correction of the defect consisted of volume disproportion of the pericardial sac and the heart size. Due to compression and low cardia c output we were forced to perform the plastic surgery of the sternum. The procedu re consisted of the excision of the midd le segment of the sternum and the em ployment of the bony fragm ents thus obta ined in the superior and infer ior parts of the stern um in orde r to achieve the stability of the chest. The surgery resulted in widening the gap between stern al margins. increasing the tra nsverse diameter of the pericardial sac, maint aining an adequate thorax sta bility. From this expe rience such a procedure might be recommended in infan ts presenting similar technical problems upon chest closure. Alterna tively oth er techniqu es can be used to avoid conduit compression (22). In our second pati ent we encountered a similarly rare defect of TA ass ociated with 1M type B with a variant consisting of both the subclavi an arte ries atypically originating from the descen ding aorta. The right subclavian art ery originated below the left on e and surrounding the tr achea. Such a varia nt was described in 1961 (20) and ther eafter infrequently (I , 3, 21, 25). Our patient additiona lly manifested seve re truncal valve regurgitation and died pri or to surgery . The autho rs have not found a single case of successful surgical ma nagement in late r communic atio ns. It shou ld be emphasized that in both presented cases we did not observe the frequently reported and stress ed monoventricular origin oftruncus arteri osus mostly or complete ly from the right ventricle (I , 3, 9, 20 23, 24, 25),which in theory is embryologically related to the A-4 type of TA (24), but has never been described in relation to type A-4 with 1M type A.

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M. D. Janusz H. S ka lski I Department of Cardiac Surgery Silesia n Heart Cente r Silesia n Mediacal Academy Ziolowa Str. 47 PL-40-64 9 Katowice Poland

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Thorac. cardiovasc. Su rgeon 40 (I 992)

Trun cus Arteri osu s Commu nis Associa te d with Int errupted A ort ic A rch