JACC Vol. 3, No.2February 1984:437-41

CASE REPORTS

Origin of Both Coronary Arteries From the Pulmonary TrunkAssociated With Hypoplasia of the Aortic Tract Complex:A New Entity

SAROJA BHARATI, MD, FACC,*t ROBERT J. SZARNICKI, MD, FACC,:j:

ROBERT POPPER, MD, FACC,:j: ANDREW FRYER, MD,t MAURICE LEV, MD, FACC*t

Browns Mills. New Jersey

437

The first case of origin of both coronary arteries fromthe pulmonary trunk, associated with hypoplasia of theaortic tract complex is reported. Although this is a lethalanomaly, as both coronary arteries originate from the

Origin of the entire coronary artery supply from the pul­monary trunk is a rare anomaly. We have found 18 reportedcases (1-18) in which the anomaly was either isolated orassociated with other complexes. We recently studied onecase, associated with an atypical form of hypoplasia of theaortic tract complex and with aortic and mitral atresia. Thisis the first reported case in which this complex has beenassociated with the origin of both coronary arteries from thepulmonary trunk. This combination is especially intriguingbecause surgical intervention could conceivably make thisa viable entity.

Case ReportClinical History and Findings

This full-term infant was born to a 19 year old gravida3, para 0 mother after an uncomplicated del ivery. The babyweighed 2.74 kg at birth and appeared well with no cy­anosis, murmur or respiratory distress. However, low setears, a webbed neck, widely spread nipples and a single

*From the Congenital Heart Conduction System Laboratory. Depart­ment of Pathology, Deborah Heart and Lung Center, Browns Mills, NewJersey; tDepartment of Pathology, Temple University, Philadelphia, andPennsylvania State University, The Milton S. Hershey Medical Center,Hershey, Pennsylvania; and the :j:Departmenl of Cardiovascular Surgery,Presbyterian Hospital of Pacific Medical Center, San Francisco. California.This study was aided by Grant HL 30558-01 from the National Heart,Lung, and Blood Institute, National Institutes of Health, Bethesda, Mary­land. Manuscript received July 7, 1983; accepted August 2, 19X3.

Address for reprints: Saroja Bharati, MD, Congenital Heart and Con­duction System Laboratory, Department of Pathology. Deborah Heart andLung Center, Browns Mills, New Jersey 08015.

© 1984 by the AmericanCollege of Cardiology

pulmonary artery, it is conceivable that surgical inter­vention could make this unusual entity a viable one.Possible surgical techniques are discussed.

umbilical artery were noted. All peripheral pulses were strong.At this time, a complete blood count showed hemoglobinof 24 g, hematocrit of 71.5, white blood count of 28,900mrrr' with 63 polymorphonuclear leukocytes, 6% band cellsand 31% lymphocytes, Capillary blood gas determinationsin room air showed a pH of 7.25, partial pressure of carbondioxide (Pco-) of 38.4 and partial pressure of oxygen (Po 2)of 39,5. After supplementary oxygen, on forced inspiratoryoxygen (FI02) of 30%, repeat capillary blood gas deter­minations revealed pH 7.2, PC02 31.9, P02 42.3 and BE10.7. The baby then developed tachycardia and had diffi­culty in feeding.

Physical findings and treatment. On arrival of a trans­port team for Pacific Medical Center, the patient appeareddusky and in marked respiratory distress. The heart rate was130 beats/min and regular. The respiratory rate was 80/min,The blood pressure in the right arm was 70/40 mm Hg.Femoral pulses were normal. There was an increased rightventricular impulse; the first heart sound was increased inintensity and the second sound was single and loud. A grade2 to 3/6 musical early systolic murmur was heard along theleft sternal border. A lower pitched diastolic murmur washeard at the left sternal border. The liver edge was palpated1.5 cm below the right costal margin. Umbilical arterial andvenous lines were inserted. The patient was given 150 g ofcalcium gluconate by slow intravenous infusion and trans­ferred to Pacific Medical Center in San Francisco. On ar­rival, the patient was treated for metabolic acidosis andhypocalcemia.

An echocardiogram revealed a single ventricular cavitywith a muscular structure near the apex, There was a singleatrioventricular (AV) valve with somewhat redundant valvetissue, suggestive of endocardial cushion defect. The inter-

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atrial septum was deficient, although present, and there wasa single anteriorly placed great vessel with a normally func­tioning semilunar valve. An electrocardiogram showed anaxis of - 30° with extreme right atrial enlargement. AnX-ray film of the chest revealed a moderately enlarged glob­ular heart; the aortic knob was not seen. There was moderatepulmonary overcirculation.

Cardiac catheterization through the umbilical arterial andvenous lines revealed an oxygen step-up in the right atrium,elevated right atrial pressure and elevated ventricular end­diastolic pressure. Repeat cardiac catheterization revealedsystemic desaturation and a wide systemic arteriovenousoxygen difference suggesting low cardiac output. A largeoxygen step-up in the right atrium indicated a large pul­monary flow. Systemic pressure in the pulmonary arteries,equal to aortic pressure, was noted.

Angiography revealed a single heavily trabeculated ven­tricle and single AV valve, which gave rise to an anteriorlyplaced great vessel, which gave rise to the pulmonary ar­teries and the ductus arteriosus. On right venticular injec­tion, the coronary arteries were suspected to arise from thisvessel. The ductus led to the descending aorta near. or justdistal to, a coarctation. Above the coarctation. the aorticarch gave rise to the brachiocephalic vessels. The proximalportion of the arch ended abruptly and no ascending aortacould be seen. The impression was: I) a single ventricularchamber, probably of right ventricular type; 2) aortic atresiawith coarctation; 3) probable mitral atresia with hypoplasticor atretic ventricle; and 4) atrial septal defect.

Prostaglandins were administered with no significant im­provement. The patient was seen by a cardiac surgeon whoconsidered the defect inoperable. The patient remained cy­anotic and in congestive heart failure with progressive met­abolic acidosis and died on the third hospital day.

Pathologic Diagnosis

Extracardiac findings. Pathologic study revealed: I)Turner's syndrome, and 2) pulmonary atelectasis (bilateral).

Cardiac findings. The heart was somewhat enlarged.with the apex formed by the right ventricle (Fig. I). Fromthe base, two vessels emerged: a larger pulmonary trunksituated to the left and anterior, and a minute aorta to theright and posterior. Although it was possible to identify thedistal part of the anterior and posterior descending coronaryarteries. they were not well developed.

The right atrium was greatly enlarged and its wall wasthickened. There was a combined eustachian and thebesianvalve. The limbus was divided into several componentstoward its distal end. The septum primum was redundantand markedly fenestrated, forming an aneurysm that bulgedinto the tricuspid anulus at the junction of the septal andanterior leaflets. A defect in the atrial septum was presentin the region of the fossa ovalis, measuring 0.8 em in great-

Figure 1. Schematic representation of the coronaryarteries arisingfrom the pulmonary trunk. I == large right coronary artery: 2 ==small anterior descending coronary artery: 3 = small left circum­flex coronary artery: 4 = sling formation at the base of the pul­monary trunk between the right and the left coronary arteries.

est dimension. The endocardium of this chamber was con­siderably thickened and whitened.

The tricuspid orifice was enlarged. The leaflet tissue wasredundant. nodose and irregular, presenting a sheet of val­vular tissue connected by a few chordae directly to theposterior and anterior walls of the right ventricle withoutdistinct papillary muscles.

The right ventricle was enlarged and its wall was thick­ened (Fig. 2). The septal and parietal bands formed a shal­low arch. The endocardium of this chamber showed con-

Figure 2. External view of the heart. AO = ascending aorta; D= ductus arteriosus; DA = descending aorta; PT = pulmonarytrunk; T = transverse arch. Note the minute ascending aorta thatwidens out to form the transverse arch and the ductus forming thedescending aorta. Note also the two probes lifting the right andthe left coronary arteries as they form a sling around the base ofthe pulmonary trunk.

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siderable thickening and whitening, The pulmonary trunkemerged from this chamber and its orifice was enlarged,The two coronary ostia were small and emerged from thejunction of the right and left posterior sinuses of Valsalva.The right ostium formed the right coronary artery (Fig, 3),It gave off a large conal branch and ended in the posteriordescending artery, The left coronary artery was of good sizeat its origin, It wound around the left side of the pulmonarytrunk to enter the interventricular groove, where it becameindistinct. It formed a sling with the right coronary arteryon the anterior wall of the base of the pulmonary trunk.The ductus was widely patent, forming the descending aorta(Fig, I),

The left atrium was smaller and its wall was thinner thannormal (Fig. 4). It received the pulmonary veins in a normalmanner. The mitral orifice was a minute structure. Its valveconsisted of a sheet of valvular tissue with no distinct an­terior and posterior leaflets, connected by a few chordaedirectly to the ventricular wall.

The left ventricle was a minute chamber with a wallthinner than normal. Its endocardium was thickened andwhitened. A ventricular septal defect was present in theapical region, measuring 0.2 to 0.3 em in greatest dimen­sion. This entered the trabeculated part of the right ventricle

Figure3. Right ventricular outflow tract view showing the originof both coronary arteries from the posterior sinuses of Valsalva.D = ductus arteriosus; DA = descending aorta; PT = pulmonarytrunk; RV = right ventricle. Note the two strings that open upthe sinuses of Valsalva and the arrows pointing to the coronaryostia.

Figure 4. Left atrial view of the heart. LA = leftatrium; LV =left ventricle: mv = mitral valve.

beneath the septal leaflet of the tricuspid valve. No vesselarose from this chamber. A minute aorta proceeded to thebase of the heart without entering the latter. No coronaryarteries arose from the aorta. A minute vessel formed alarger transverse arch which gave off the brachiocephalicvessels in a normal manner. The crista reuniens formed aprominent circumferential ridge. The descending aorta wasnormal.

DiscussionCoronary artery anatomy. When both coronary arter­

ies arise from the pulmonary trunk, the coronary ostia areusually located in the right and left sinuses of Valsalva, justto the right and left of the commissure separating the rightand left pulmonary valve leaflets (15). The peripheral dis­tribution in these cases is usually normal (15). In one case(II), both coronaryostia arose from the left pulmonary sinusof Valsalva. In this case, the right coronary artery proceededbetween the aorta and the pulmonary trunk to the right AVgroove. When there is a single coronary artery arising fromthe pulmonary trunk, it may arise from the right sinus ofValsalva (10) and bifurcate into right and left coronary ar­teries. The left coronary artery then passes between the aortaand the pulmonary trunk. The vessels then go to their usualdistribution. When a single coronary artery arises from theleft pulmonary sinus of Valsalva (15), it bifurcates almostimmediately and the component vessels go on to their usualdistribution. In our case, however, the right coronary artery

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passed behind the aorta and then proceeded in its usualmanner.

The walls ofthe coronary arteries are normal in structure(9) or vein-like in this anomaly (8) or show intimal prolif­eration (5). In one case, there was thickening of the arterialwalls due to intimal fibrous proliferation. If the child liveslong enough, myocardial infarction may occur (10) or theremay be myocardial changes, compatible with ischemia .

Associated anomalies. Origin of both coronary arteriesor a solitary, single coronary artery from the pulmonarytrunk may occur as an isolated anomaly (10-14). Morefrequently, it is associated with other anomalies, includingpatent ductus arteriosus (3,6-8) , ventricular septal defect(2, 12,17,18), truncus communis ( I), tetralogyof Fallot (4),tricuspid atresia complex (2) and coarctation of the aorta(7,18). Our case is the first case where the anomaly isassociated with hypoplasia of the aortic tract complex (hy­poplastic left heart syndrome).

Features affecting survival. Origin of both coronaryarteries from the pulmonary trunk usually does not permitsurvival beyond the first 2 weeks of life (15) because of theflow to the myocardium of anoxic blood at relatively lowpressure. However, ifthe anomaly is associatedwitha lesionthat increases the oxygen content or the right ventricularpressure, a longer survival may occur.

This takes us to our present case. In classic hypoplasiaof the aorta. The coronary supply is restricted because thedrome, there is aortic atresia with mitral stenosis or atresia(19,20). This is associated with an atrial septal defect, butusually no ventricular septal defect. The ascending aorta isminute, the transverse aorta is somewhat smaller than nor­mal and the descending aorta is normal in size. The ductusarteriosus is widely patent. When there is mitral atresia,there is muscle representing the left ventricle but no lumento this ventricular mass. When there is mitral stenosis, thenthere is a small left ventricle with endocardial fibroelastosis.Ourcase is atypical in that there is a ventricular septaldefect.

In this complex, the coronary arteries emerge at the baseof the aorta. The coronary supply is restricted because theblood flow is from the right ventricle through the ductus tothe transverse and descending aorta and only finally throughthe minute ascending aorta to the coronary arteries in aretrograde manner. If. however, the coronary arteries emergefrom the pulmonary trunk in this anomaly, as in our casewith a hypertrophied right ventricle and an atrial and ven­tricular septal defect, it is conceivable that suffic ient lon­gevity may occur to permit eventual surgery.

Surgery. Surgery in hypoplastic left heart syndromehasrecently been described by Norwood et al. (21,22). In thefirst stage, a valve conduit is placed between the right ven­tricle and the descending aorta, a large interatrial commu­nication is established, the ductus arteriosus is ligated andthe pulmonary artery is banded. In an alternate procedure,the main pulmonary artery is transected and the proximal

portion anastomosed to the ascending aorta and transversearch, while the right ventricle is anastomosed to the branch­ing pulmonary artery. Another alternate procedure involvesthe anastomosis of the proximal main pulmonary artery tothe ascending aorta and aortic arch. A central shunt is thenestablished between the newly constructed ascending aortaand the confluence of the pulmonary arteries. The secondand thirdprocedures are completedby the creation of a largeintraatrial communication and ligation of the ductus. Anyone of the three procedures is followed later by partitioningthe atria so that the left atrium is connected to the tricuspidvalve and the right atrium to the branching pulmonary ar­teries and the previous shunt is closed. In our case, withthe coronary arteries arising from the pulmonary trunk, wemight propose a similar but simpler procedure.

More recently Lang and Norwood (23) reported on twoinfants who were clinically well 9 and 12 months aftersurgery for hypoplastic left heart syndrome, in whom sur­gery had been done in two stages. In the first stage, thepulmonary arteries would each be banded and a conduitplaced between the pulmonary artery and the transverse ordescending aorta. This would be followed later by atrialseptation so that the left atrium was connected to the tri­cuspid valve, and a Fontan reconstruction of the right sideof the heart would be made with a bifurcating conduit tothe branching pulmonary artery. Thus, the main pulmonarytrunk with the coronary arteries and the right ventricle fun­damentally would become the systemic circuit.

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