Complete interruption of the aorta∗

Case Reports Complete Interruption of the Aorta*

M . GONCEPCHSN Ruiz VILLALOROS, M .D ., DELFINA P . DE BALDERRAMA, M .D ., JUAN LOPEZ Y LOPEZ, M .D . and MIGUEL CASTELLANOS, M .D . Guadalajara, Mexico

A

Physical examination revealed an underdeveloped boy who appeared younger than the stated age of 14 years . The carotid pulsations were regular and had a bounding quality . A moderately harsh systolic murmur accompanied by a thrill was present over the neck vessels . The pulse in these vessels and in the suprasternal and supraclavicular areas was bounding in type . The chest was asymmetrical due to a large bulge in the precordial region, A well-developed collateral circulation was both visible and palpable over the posterior aspect of the chest. A

RECENT REVIEW of the literature on complete interruption of the aorta at the level

of the isthmus revealed only forty reported _s The majority of these patients cases .t b 7had a limited lifespan ; only the one studied by Donzelot et a] . 6 reached the age of 11 years (Table I) . The case of a patient studied recently in the Hospital Civil of Guadalajara, Mexico, is of interest because there were some unusual clinical features and the patient reached the age of 14

diffuse prccordial heave was present, the greatest impulse being in the epigastrium . There was a systolic thrill palpable in the epigastrium and along the left sternal border, particularly in the fifth and sixth intercostal spaces . The apical impulse was diffuse and easily palpable in the seventh left intercostal space . Percussion revealed (in addition to cardiomegaly) an area of dullness extending 3 cm . to the left of the sternum in the second and third intercostal spaces. A systolic thrill was palpable in the same

years . The principal malformation was corrected successfuly by surgery . CAsE REPORT This 14 year old boy was the product of an uncomplicated, full term delivery . However, the mother had had hyperemesis and frequent respiratory infections during the first trimester . One sibling (the previous child) was cyanotic and died a few hours after birth . The patient has had frequent and severe infections of the lower respiratory tract, beginning with severe bronchopneumonia during the first months of life . Dyspnea, hemoptysis and profuse epistaxis have become prominent during recent infectious episodes . The patient has always had limited physical en-

area . The heart rate was 90 per minute and the rhythm was regular . A harsh systolic murmur was heard along the left sternal border with grade 4 intensity in the fifth and sixth left intercostal spaces . 'I his murmur extended toward the left axilla and the back . A soft, grade 1 to 2 systolic murmur was heard at the apex . The second heart sound was accentuated in the second and third intercostal spaces on both sides of the sternum . The blood pressure was 220/120 mm . Hg in both arms and 120/80 mm . Hg in the legs . There was slight cyanosis of the toes, increasing with physical exertion . Examination of the eve grounds disclosed moderate vascular arteriolar sclerosis . The hemoglobin and red blood cell counts were within normal limits . The roeragenogram of the chest in the posteroanterior view (Fig. IA) revealed moderate cardiomegaly . Both ventricles were enlarged . The pulmonary artery

durance, but it was not until he was 5 years old during one of the respiratory infections, that a heart murmur was discovered . At the age of 8 the patient complained of frequent frontooccipital headaches and at the age 13 years occasional fainting spells developed . At the age of 14 years the patient had a severe infectious condition, thought to be subacute bacterial endocarditis . This was not proved . Despite negative blood cultures 20 million units of penicillin was given daily for a 2 week period, the patient responded well .

* From the Hospital Civil, Guadalajara, Mexico . 664

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Complete Interruption of Aorta FABLE I

Summary of Twenty-Seven Reported Cases of Complete Interruption of the Aorta

Author Seidelx"' Letterer"s Abbott' Abbott' Abbott% Gaspar's Evans' Evan" EvansHamburger' Wiscman ct al .2 Taussigr" StewartR4 Jew et al .'s Kintner's Donzelot° Barger et al . Dorncy et al .° Merril et al . o Everts-Suarez et al." Espino-Vela et a1 . Abranrs° Fournier et al ." Noonan et al. (4 eases)%0 Nounan et al'° Quic et al . as Ruiz Villalobos et al .

Year of Publication

I

I

1818 1923 1927 1927 1927 1929 1933 1933 1933 1937 1948 1960 1948 1952 1953 1954 1954 1955 1957 1959 1959 1958 1960 1958 1958 1959 1961

was prominent; the hilar vascular shadows were increased and there were a few notchings at the lower margin of some ribs . The electrocardiogram (Fig . 2) revealed an unusual axis deviation with deep S waves in leads t, u, at and aVF, and prominent R in aVR . There were Rsr's' complexes in lead V e and diphasic deflections (RS) in V, to V 4 . The morphology of the yen-

Age and Sex

Deceased

Hours, M 5 days, F 15 days, _ . 1 day, . . . 6 me ., 6 mo ., F 5 days, M 2 days, F 11 days, Kf 1 mo ., M 71 hours, M

Yes Yes Yes Yes Yes Yes Yes Yes Yes Yes Yes Yes Yes Yes Yes Yes Yes Yes

45 hours, F 53 hours, M 14 hours, M 11 yr 9 days F 5 yr ., F 3 yr ., F 3 days, M 3 yr ., F 2 mo ., F 20 days ; . . 3 mo 14 yr.,

Yes Yes Yes Yes Yes Yes Yes No

Underwent Operation No No No No No No No No NO

No No No No No No No No No Yes No No No No No No Yes Yes

tricular complexes in leads i, V 5 and V 6 was qRS, with positive peaked T waves . The T waves were inverted in V, to V s . The tracing was interpreted as suggestive of incomplete right bundle branch block, biventricular hypertrophy, left ventricular diastolic overloading and right ventricular diastolic and systolic overloading . Cardiac Catheterization : These data led to the clinical

B A Fro . 1 . Roentgenograms in P-A view . A, preoperative film . Note the enlargement of the heart and the profuse vascularity of the lungs . B, postoperative film . Note the smaller heart size and the diminished vascularity of the lungs. NOVEMBER 1961



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Ruiz Villalobos et al .

FIG . 3 . A catheter is shown within the heart and in the aorta, through a "patent ductus arteriosus" ; actually it is entering the initial portion of the descending aorta which arose from the pulmonary artery .

pairs were dilated and tortuous . The aorta arose from the left ventricle in a normal fashion, ascended normally and gave origin to the right innominate artery, the left carotid artery and the left subclavian artery . These vessels were normally distributed . The pulmonary artery was approximately three times normal size and larger than the aorta, It arose in the usual fashion from the right ventricle . The right pulmonary artery was not remarkable, the left pulmonary artery arose at a sharp angle and was extremely dilated . The descending aorta emerged from this branch at the site where the ductus artcriosus is usually found, when patent. The first portion of this descending aorta was narrow in comparison to the remainder of the TABLE 11

Catheterization Findings

I

He . 2 . Electrocardiogram suggestive of partial right bundle branch block and biventricular hypertrophy .

Site

Oxygen Content (vol . %)

diagnosis of coarctation of the aorta, patent ductus arteriosus and ventricular septal defect, Cardiac catheterization2s disclosed the presence of a patent ductus arteriosus which was traversed by the catheter (Fig . 3) . Data from this study are shown in Table

Superior vena cava Right atrium Right Ventricle

12 .87 12 .75 12 .87

74 .73 74 .04 74 .73

Pulmonary artery Right Left Aorta

12_87

74 .73

12 .75 12 .99

74 .04 75 .43

Femoral artery Brachial artery Pulmonary capillary

13 .04 14 .89

75 .60 86 .48

H.

The patient was referred for surgery with the diagnosis of patent ductus arteriosus and coaretation of the aorta . Ventricular septal defect was not entirely excluded as an additional possibility . Surgical Findings : The heart was considerably enlarged . A thrill was felt over its entire surface . The intercostal arteries, especially the first two or three

Oxygen Saturation (%)

Blood Pressure rnrn . Hg) (7) » 90/1 .6 (38) 90/36 .3 (51) 117/74 (88) (18)

Numbers in parentheses indicate mean pressures . IRE AMERICAN JOURNAL OF CARDIOLOGY



Complete Interruption of Aorta

d .sp

Fu; . 4 . Drawing showing the aorta and its branches before the operation . vessel. In fact, this first part was actually the patent ductus arteriosus connecting the two vessels without a definite line of demarcation . these relationships are diagrammed in Figure 4 . 1 he distribution of the

667

vagus and the recurrent laryngeal nerves was that usually seen in cases of patent ductus arteriosus . Figure 5 shows the aorta arising from the left pulmonary artery, pulled back slightly with a tape . After the anatomy of the vessels was understood clearly, the descending aorta was clamped for 15 minutes and intravascular pressure readings were obtained by a needle inserted into the pulmonary artery . Because there was no significant alteration in pressure, the aorta was severed front the pulmonary artery . A Teflon graft, 15(m . long and 2 cm. in diarneter, was anastomosed end to side with the left subclavian artery ; the lower end of the graft was anastomosed end to end with the distal portion of the descending aorta . Figure 6 shows the portion of this graft, which functioned perfectly . Pressures and blood samples were obtained in the aorta, in the graft and in the pulmonary artery at the end of the procedure . Oxygen saturation was identical in the graft and in the aorta and equal to 89 .07 per cent . Both had equal pressures of 135180 mm . Hg . Oxygen saturation in the pulmonary artery was 755 per cent . 'I he pressure in this vessel decreased from 90,'36 to 75 ;30 mm . Hg .

5 6 Fm . 5 . Photograph showing the pulmonary artery (PA) to the left (the largest vessel) and the aorta (AO) pulled with a tape, arising from the pulmonary artery . Fm . 6. The Teflon graft in place, sutured to the descending aorta below and the suhelavian artery above . NOVEMBER

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Ruiz Villalobos et al .

Systemic blood pressure 24 hours after surgery was 130/90 mm . Hg in both arms and legs . At the end of the third day mild symptoms of cardiac failure developed but were promptly alleviated with digitalis therapy . Postoperative Results : The remainder of the postoperative course was uneventful . One month after surgery the pressure in the legs increased, reaching 150/80 mm . Hg ; the pressure in the arms remained 135/75 mm . Hg. The blood pressures have been stable for 7 months postoperatively . Radiologie improvement was noted-the size of the heart was considerably reduced and both ventricles decreased in size (Fig . 1B) . The profuse vascularity of the bungs was also reduced ; however, the prominence of the pulmonary artery remained unchanged . The shadow of the graft is easily seen above the pulmonary artery arch (Fig . 1B) . 'the electrocardiogram taken 1 month after surgery revealed few changes when compared to the preoperative tracing . Systolic overloading of the right ventricle diminished . Physical examination disclosed vigorous arterial pulsations in the neck and suprasternal region, a grade 2 systolic thrill over the right carotid artery and a grade 1 Systolic thrill over the left carotid artery . The apical impulse was now at the fifth left intercostal space and its impulse was about 2 cm, in area . At this site there was a grade 1 systolic murmur transmitted to the left axilla . Nothing abnormal was noted in the epigastrium . A basal systolic thrill, maximal over the third right intercostal space, and a grade 1 systolic murmur in the midprecordial area remained . COMMENT In reviewing the medical literature in relation to the occurrence of this congenital anomaly, 10-Is,17,211,21,25 it was found that only two other patients had undergone surgery . The patient described by Merril et al .'in 1955 survived 6 months . The second surgical case was reported by Quie et al :12 the patient died 2 days after operation . The patient reported herein underwent surgery on February 18, 1960, and at the present time is in excellent general condition . SUMMARY We have studied the case of a 14 year old boy with complete interruption of the aortic isthmus associated with patent ductus arteriosus and probable ventricular septal defect . Surgical correction of the interruption of the aorta was achieved by anastomosing a Teflon prosthesis from the left suhclavian artery to the distal aorta . As far as we know, this patient has had the

longest survival with good operative results of any other reported case . REFERENCES 1 . Aauo'rr, M . E . Atlas of Congenital Cardiac Disease, p. 20 . New York, 1936 . American Heart Association . 2 . ABBOTT, M . E ., OSIER, W. and McCREA, T . Modern Medicine, ed . 3, Vol . 4, Philadelphia, 1927 . 3 . AxRAms, H . L. Persistence of fetal ductus function after birth . Circulation, 28 : 206, 1958. 4 . BARGER, J. D ., CREASMAN, R . W. and EDWARDS, J . F . Bilateral ductus arteriosus associated with interruption of the aortic arch . Am, J . Cfin . Path ., 24 : 441, 1954. 5 . CURRARINO, G ., EDWARDS, F . K . and KAPLAN, S . Hypnplasia of the left heart complex . .t: I.s. Child., 97 : 843, 1959 . 6 . DONZELOT, E . 'l'raite des Carcliopathies Congenitales . Paris, 1954. Masson et tie. 7. DORNEY, E . R., FOWLER, N . 0 . and MANNAIX, E . P . Unilateral clubbing of the fingers due to absence of the aortic arch . Am- J . Med ., 18 : 150, 1955 . 8. EVANS, W . Congenital stenosis (coarctation), atresia, and interruption of the aortic arch (a study of 28 cases) . Quart, J. ., Med 2 : 1, 1933 . 9. ESPINO-VELA, J ., ACOSTA, R . A . AND DE LA CRUZ, M . V. Interruption completa del istmo a6rtico . Arch . Inst . Cardiol ., 19 : 53, 1959 . 10 . Esrtxo-VRI .A, J . Malformaciones Cardiovasculares Congenitas . 1959, Inst.NacionaldeCardiologia . 11 . EvERrs-SUAREZ, E . A . and CARSON, P . C . The triad of congenital absence of aortic arch, patent ductus arteriosus and interventricular septal defect, Ann . S'urg ., 150 : 153, 195912 . FOURNIER, P . and ZAmr, Z . H . Congenital absence of the aortic arch . Am . Heart J ., 59 : 148, 1960 . 13. GASPAR, I . Two of the rarer congenital anomalies of the heart . Am . J. Path ., 5 ; 285, 1929 . 14. HAmxuEoRR, L . P. Congenital cardiac malformation with complete interruption of isthmus aortae and transposition of great arteries . Bull . Johns Hopkins Hosp., 61 : 421, 1937 . 15. JEW, E . W., JR . and CROSS, P . Aortic origin of the right pulmonary artery and absence of the transverse aortic arch associated with patency of interventricular septum and ductus arteriosus . Arch . Path ., 53 : 191, 1952 . 16. KDNreR, E . P . Congenital malformation of the heart : interruption of the aortic arch, mitral valve orifice atresia, and persistent left superior vena cava. Lab. Invest ., 2 : 388, 1953 . 17. KIELLBERG, S. R ., MANNHEIMER, E. . RuDtte, U . and Joassox, B . Diagnosis of Congenital Heart Disease . p . 448 . Chicago, 1955 . The Year Book Puhlishers . 18. LEETTRER, E . Kongenitaler Defekt des A ortenhogns . Centralbl . allg. Path . u . Path . Anne., 33 : 155 . 1923 . 19, MF.RRIL, D . L . ; WLBSmn, C, A . and SANSON, P . C . Congenital absence of the aortic isthmus . J. Thoracic Surg., 33 : 311, 1957 . 20 . NooNAN, J . A . and NADAS, A . S . The hypoplastic left heart syndrome . Pediat . Cfin . North America, 5 : 588, 1958 . 'UHF: AMERIcAN JOURNAL Our CARDIOLOGY



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A. S. Pediatric Cardiology, p . 378 . Philadelphia, 1957 . W. B . Saunders . 22 . Qumm, P . G . . Novncac, R ., ADAMS, P., JR., ANDERSON, R . C . and VARCO, R . L. Congenital interruption of the aortic arch . .T. Pedial., 54 : 87, 1959 . 23 . SLIDEL, J . F . Index Musei anatomici Kiliensis, p . 61 . Kilie, 1818 . C. F . Mohr. 24 . Sriewnxr, 9I . Congenital interruption of the aortic arch . Areh . Dis . Child ., 23 : 326, 1948 . 25 . Soruf, P . Cardiopathics Congenitales, p . 277 . NAD .AS,

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Paris, 1956 . L 'Expansion Scientifique Francaise H, B . Congenital Malformation of the Heart . p . 246 . New York, 1960 . The Commonwealth Fund . 27 . WEISMAN, D,, KESTLN, H, D . and PLAINS, W . Absence of transverse aortic arch with defects of cariliac scptums . Am . J . Dis . Child., 76 : 326, 1948 . 28 . ZIMMERMAN, H . A . Intravascular Cathetcrization, p . 178 . Springfield, Ill ., 1959 . Charles C Thomas . 26 .

TAussrs,