Br Heart _r 1994;71:89-91 CASE REPOP ste--. Maldevelopment of conotruncal and aorto-pulmonary septum with absent left central pulmonary artery: anatomical and clinical implications Deutsches Herzzentrum, Abteilung fir Kinderkardiologiel Angeborene Herzfehler, Berlin, Germany I Schulze-Neick G Hausdorf P E Lange Correspondence to: Dr I Schulze-Neick, Abteilung Angeborene Herzfehler Kinderkardiologie, Deutsches Herzzentrum Berlin, Postfach 65 65 65, 1000 Berlin 65, Germany. A Bt I Schulze-Neick, G Hausdorf, P E Lange Abstract In a patient with pulmonary valve atresia with hypoplastic main pulmonary artery selective angiography showed absence of the central left pulmonary artery and a right pulmonary artery originating from the ascending aorta close to the left coro- nary artery. This unusual anatomical Figure I Angiograms in anterior-posterior views showing (A) the main aortopulmonary collateral artery arising from the upper descending aorta and supplying the circulation for the right upper lung lobe and (B) a modified left Blalock-Taussig anastomosis connected with the distal part of the left pulmonary artery. arrangement complicates interventional and surgical treatment. (Br HeartJ_ 1994;71:89-9 1) In patients with pulmonary valve atresia blood flows to the lung either through the ductus arteriousus or major aorto-pulmonary collateral arteries.' These collaterals usually arise from the descending aorta, but they can also originate from bronchial arteries, the subclavian artery, or other major branches of the aorta.2 Coronary fistulas feeding central or peripheral pulmonary or collateral arteries have also been described3 and can complicate surgical repair.4 An abnormal left or right pul- monary artery originating directly from the ascending aorta can cause unilateral or bilat- eral pulmonary artery hypertension with ensuing pulmonary vascular obstructive dis- ease.5 The occurrence of pulmonary valve atresia together with a right pulmonary artery with an abnormal origin and absence of the left central pulmonary artery has to the best of our knowledge not been reported before. Case report The patient was admitted to our institution when he was 13 days old with the diagnosis of pulmonary valve atresia with ventricular sep- tal defect with major aorto-pulmonary collat- eral arteries to the lung. At operation (age 16 days) no central communication was found between the left and right pulmonary artery and no ductal ligament or patent ductus was seen. A modified Blalock-Taussig anasto- mosis was established on the left side by connecting a 5 mm Gore-tex prothesis end- to-side with the left subclavian artery and end-to-end with the left pulmonary artery. The procedure was well tolerated. Postoperative re-catheterisation when the patient was 8 months old showed a major aorto-pulmonary collateral artery from the upper descending aorta supplying blood flow to the right upper lung (fig 1A). The shunt was connected to the distal part of the left pulmonary artery and the proximal part was missing (fig 1B). 89 on May 28, 2021 by guest. Protected by copyright. http://heart.bmj.com/ Br Heart J: first published as 10.1136/hrt.71.1.89 on 1 January 1994. Downloaded from
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Br Heart _r 1994;71:89-91
CASE REPOP ste--.
Maldevelopment of conotruncal andaorto-pulmonary septum with absent left centralpulmonary artery: anatomical and clinicalimplications
DeutschesHerzzentrum,Abteilung firKinderkardiologielAngeboreneHerzfehler, Berlin,GermanyI Schulze-NeickG HausdorfP E LangeCorrespondence to:Dr I Schulze-Neick,Abteilung AngeboreneHerzfehlerKinderkardiologie,Deutsches HerzzentrumBerlin, Postfach 65 65 65,1000 Berlin 65, Germany.
ABt
I Schulze-Neick, G Hausdorf, P E Lange
AbstractIn a patient with pulmonary valve atresiawith hypoplastic main pulmonary arteryselective angiography showed absence ofthe central left pulmonary artery and a
right pulmonary artery originating fromthe ascending aorta close to the left coro-nary artery. This unusual anatomical
Figure I Angiograms in anterior-posterior views showing (A) the main aortopulmonarycollateral artery arisingfrom the upper descending aorta and supplying the circulation forthe right upper lung lobe and (B) a modified left Blalock-Taussig anastomosis connected
with the distal part of the left pulmonary artery.
In patients with pulmonary valve atresiablood flows to the lung either through theductus arteriousus or major aorto-pulmonarycollateral arteries.' These collaterals usuallyarise from the descending aorta, but they canalso originate from bronchial arteries, thesubclavian artery, or other major branches ofthe aorta.2 Coronary fistulas feeding centralor peripheral pulmonary or collateral arterieshave also been described3 and can complicatesurgical repair.4 An abnormal left or right pul-monary artery originating directly from theascending aorta can cause unilateral or bilat-eral pulmonary artery hypertension withensuing pulmonary vascular obstructive dis-ease.5The occurrence of pulmonary valve atresia
together with a right pulmonary artery withan abnormal origin and absence of the leftcentral pulmonary artery has to the best ofour knowledge not been reported before.
Case reportThe patient was admitted to our institutionwhen he was 13 days old with the diagnosis ofpulmonary valve atresia with ventricular sep-tal defect with major aorto-pulmonary collat-eral arteries to the lung. At operation (age 16days) no central communication was foundbetween the left and right pulmonary arteryand no ductal ligament or patent ductus wasseen. A modified Blalock-Taussig anasto-mosis was established on the left side byconnecting a 5 mm Gore-tex prothesis end-to-side with the left subclavian artery andend-to-end with the left pulmonary artery.The procedure was well tolerated.
Postoperative re-catheterisation when thepatient was 8 months old showed a majoraorto-pulmonary collateral artery from theupper descending aorta supplying blood flowto the right upper lung (fig 1A). The shuntwas connected to the distal part of the leftpulmonary artery and the proximal part wasmissing (fig 1B).
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Figure 2 (A) Angiogram in laid back view, showing selective angiography into the leftdorsal sinus of Valsalva. Separate orifices are visible for the left coronary artery (LCA)and the right pulmonary artery (RPA), and the RPA connects with the distal end of thehypoplastic main pulmonary artery (MPA). (B) A drawing to illustrate the arrangementseen in (A).
Additional selective angiograms showed anabnormal vessel (diameter 2-5 mm) originat-ing 3 mm above the left coronary artery ori-fice and connecting with the distal end of ahypoplastic pulmonary artery stem. This ves-sel (diameter 7 mm) provided the pulmonaryblood flow to the right middle and lower lungand was thus considered to be the right pul-monary artery. The left coronary artery origi-nated normally from the left dorsal sinus ofValsalva of the aortic root and its orifice layjust below the orifice of the right pulmonaryartery (fig 2A and B). No communicationbetween the coronary artery and the rightpulmonary artery was shown by repeatedselective injections.
Further simultaneous selective angiogramsinto the right pulmonary artery and into theinfundibulum of the right ventricle confirmedthe existence of a hypoplastic main pul-monary artery (fig 3A). The atretic pul-monary valve membrane was perforated witha specially designed perforation needle (0-014
inch). Repeated selective angiograms belowthe pulmonary valve then showed theanatomical continuity of the hypoplastic rightventricular outflow tract with the main pul-monary artery (fig 3B). Because the pul-monary artery, the left coronary artery, andthe abnormal origin of the right pulmonaryartery lay so close together further attempts toopen the atretic pulmonary valve were aban-doned.
DiscussionThis was an unusual case of pulmonary valveatresia with an hypoplastic main pulmonaryartery, an abnormal origin of the right pul-monary artery from the ascending aorta, andabsent left central pulmonary artery.The anatomical continuity from the right
ventricle to the hypoplastic main pulmonaryartery and the atretic membrane indicatecompleted aorto-pulmonary septation in thevalve area and exclude a truncus arteriosus.67Conotrucal malformations (truncus arterio-sus, tetralogy of Fallot, or pulmonary atresia)cannot occur together, but each can coexistwith malformations of the pulmonary arterialsystem because the pulmonary artery systemand the conotruncal part of the heart developindependently and at different times duringembryogenesis.89 In some forms of tetralogyof Fallot both abnormal origin and absence oratresia of the pulmonary arteries have beendescribed.2 1l2 When there is a right aorticarch the left pulmonary artery is generallyabnormally connected to the ascending aorta,and vice versa.5 13The origin of the right pulmonary artery
from the aorta could indicate that the compli-cated formation of the bifurcation was dis-turbed. The anomalous origin of a pulmonaryartery from the ascending aorta has a typicalmorphology13 and is explained by failure ofthe right sixth aortic arch to migrate withinthe wall of the embryonic truncus from rightto left towards the left sixth aortic arch andthe main pulmonary artery during embryonicformation of the bifurcation.8 The fact that inour case the right pulmonary artery originatedfrom the left side of the ascending aorta sug-gests that migration had occurred and wascomplete. The left pulmonary artery, how-ever, was missing. This suggests persistenceof the origin of the right pulmonary arteryfrom the truncus and, after aortopulmonaryseptation, from the aorta. Unlike the rightside, the left central pulmonary artery isformed by nonarch respiratory vessels fromthe lung buds. The proximal part of the leftsixth aortic branch connects these pulmonaryvessels to the right sixth aortic arch and laterlargely disappears.8 The extent to which theproximal part is involved in the formation ofthe bifurcation is uncertain. The distal part ofthe left sixth aortic arch becomes the ductusarteriosus. In our case, both parts of the leftsixth aortic arch-the proximal part as the leftcentral pulmonary artery with connection tothe bifurcation and the distal part as ductusarteriosus-could not be found either during
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Maldevelopment of conotruncal and aorto-pulmonary septum with absent left central pulmonary artery
P.q.e
P.
Figure 3 Angiograms in the lateral view showing the continuity of the hypoplastic rightventricular infundibulum, perforated atretic pulmonary valve, and hypoplastic mainpulmonary artery stem shown by selective angiography into the orifice of the rightpulmonary artery (A) and into the right ventricular infundibulum after needle perforationof the valve (B).
operation or at catheterisation. This evidencesuggests congenital absence of the left sixthaortic arch.We think that our case represents an
extreme form of tetralogy of Fallot with pul-monary valve atresia and a hypoplastic mainpulmonary artery that is associated with acomplex pulmonary artery malformation.This unusual anatomical arrangement, withthe origin of the abnormal right pulmonaryartery lying between where the aorta is crossclamped and the aortic valve, means that atoperation cardioplegia solution will enter the
coronary arteries and the pulmonary arterialbed. Continuing collateral arterial perfusionof the lungs during cardiopulmonary bypasswill maintain significant pulmonary venousreturn.As techniques for selective and supraselec-
tive angiography together with new methodsfor treatment, such as embolisation of collat-eral vessels or interventional transcatheteropening of pulmonary valve atresia'4 developthe precise anatomical delineation of complexcardiac malformations will become more rele-vant. In our patient further interventionaltranscatheter opening of the atretic pul-monary valve was impossible, however,because the valve lay close to the left coro-nary artery and because the connection withthe high pressure in the aorta would have ledto pulmonary regurgitation with unpredict-able short and long term results.
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