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Surgical management of patent ductus arteriosus associated with coarctation of the aorta
Surgical
EUGENE
Management of Patent Ductus Arteriosus Associated with Coarctation of the Aorta
DONG, JR., M.D., RICHARD R. LOSER, M.D., EDWARD J. HURLEY, M.D. AND NORMAN E. SHUMWAY, M.D., Palo Alto, Calijorniu
From tbe Department of Surgery, Stanford University School oJ Medicine, Palo Alto, CaliJomia. Tbis work was supported in part by Kesearcb Grant H-4658 of tbe Notional Heart Institute.
ITH continuing progress in the diagnosis and treatment of congenita1 cardiovascuIar disease, the lesion of coarctation of the aorta associated with patent ductus arteriosus is being reported more frequently in the surgical and pediatric literature. Prior to 1951 [1,10,1?,18,22,26,41] only a handful of cases had been reported. Since that time an increasing number of cases have been described from a few centers [y,r4-17,331. Forty-five to 70 per cent of these patients had additiona anomaIies, however, and surgical experience was limited. We believe it of interest and import, therefore, to report an additiona series of three successful surgical repairs of coarctation associated with patent ductus arteriosus.
W
CASE
REPORTS
A three months old white gir1 was CASE 1. deli\.cred two weeks prior to the estimated date because of a rising materna1 anti-rH titer. Her birth weight was 4 pounds, 14 ounces. She was we11 until the age of two months. At that time, she was hospitalized with anorexia, dyspnea and signs of congestive heart failure which responded to digitaIis therapy. One month Iater, anorexia, Ioose stools and easy fatigability again vomiting, developed. Signs of congestive faiIure were noted. She was readmitted to Palo AIto-Stanford Hospitals on November 17, 1960. Pulse was 146, bounding in the upper extremities, but barely paIpabIe in the Iower extremities. Recorded blood pressures arm, and 60 werr 175,/o mm. Hg in the right mm. Hg systolic in the right Ieg. The respiratory rate was 52. The heart was enlarged to the Ieft;
the second puImonic sound was spIit and louder than the second aortic sound; a grade 3 continuous murmur was heard over the entire upper Ieft part of the chest. The systolic component was heard over the precordium and back as well. The Iiver was enIarged, descending 4 to 5 cm. at the right costal margin; the spIeen was paIpabIe I cm. below the Ieft costal margin. There was no edema, clubbing or cyanosis. Chest roentgenograms revealed marked cardiomegaly, increased puImonary vascularity and enlargement of the left atrium. An eIectrocardiogram showed a normal sinus rhythm at a rate of 130: the P-R and QRS intervals were o. 12 and 0.06 second, respectively; the axis was plus 80 degrees; the S-T segments were sagging in Ieads I and aVL, and depressed in leads Vy to Vg; the T waves were inverted in IeacIs I, aVL., V,R and VI to Va; diphasic in Ieads VI and Vs. The diagnosis of coarctation of the aorta and patent ductus arteriosus was made on clinical grounds. The congestive failure responded to digitalis and diuretic therapy. At operation on November 23, 1960, a preducta1 coarctation of the aorta was resected and a patent ductus arteriosus divided. The coarcted segment was 0.5 cm. long; the ductus was 0.75 cm. in diameter and equal in size to the aorta. Reanastomosis of the aorta was accomplished without dificulty using a running cardiovascular suture posteriorly and interrupted evcrting sutures on the anterior row. Total time of aortir occlusion was thirty-seven minutes. The postoperative course was uncomplicated. The recorded blood pressures were 185.‘1 IO mm. Hg in the left arm on the first postoperative day, and 126’68 mm. Hg in the right arm and 130/7o mm. Hg in the left Ieg on discharge. One month postoperatively, the leg pulses wcrc easily palpablc; blood pressure in the arm was 144,‘73 mm. IIg. The chest roentgenogram sholvrd a normal puImonary vascularity, and the cardiac silhouette had diminished in size.
Dong, Lower,
HurIey
CASE II. This seven year oId boy was first seen at PaIo Alto-Stanford HospitaIs on November 27, 1959, for cardiac evaluation. A murmur was noted in infancy, and except for easy fatigability, he had been asymptomatic. Past and family history was not pertinent. Physical examination reveaIed a we11 developed, we11 nourished boy. PuIse was g6 per minute, paIpabIe in the upper extremities and absent in the lower extremities. BIood pressures were 130/7o mm. Hg (arm) and 52 mm. Hg systoIic (leg). Respirations were 24 per minute. There was a funne1 chest deformity, more marked on the Ieft side. A right ventricmar heave was noted. The heart was enIarged to the left. The second puImonic sound was sIightIy spIit and much louder than the second aortic sound. A grade 2 rough systoIic murmur, with a “sea guI1” quaIity was heard aIong the upper Ieft sterna1 border and in the infracIavicuIar area. A grade 3 blowing, earIy diastolic, decreasing murmur was noted in the pulmonic area. A grade 3 short systolic murmur along the lower Ieft sterna1 border, and a grade 2 rumbling, apical, mid-diastoIic murmur were aIso heard. The murmur at the base at times seemed continuous. The lungs were clear. The liver and were paIpabIe at the costal margins. spIeen CIubbing of the toes was definite, but cyanosis and edema were absent. HemogIobin was 15 gm. per cent. An eIectrocardiogram showed a norma sinus rhythm at a rate of 82; the P-R and QRS intervals were 0.16 and 0.08 second, respectively; the axis was plus go degrees. There was a notched R wave with an upright T wave in Iead VaR with the R wave there was an aRs measuring 22 mm. in height; wave in Iead Vs with the R wave measuring I 6 mm. The interpretation was right ventricuIar hypertrophy. Roentgenograms reveaIed cardiomegaIy involving all four chambers and marked enlargement of the Ieft atrium; the ascending aorta was dilated; a bidirectiona1 shunt was seen at the IeveI of the ductus by means of cineangiography. A coarctation of the aorta was also noted. Cardiac catheterization reveaIed an oxygen step-up in the main pulmonary artery of 8 voIumes per cent; the oxygen saturation was 89 and 97 per cent in the abdomina1 aorta and brachia1 artery, respectiveIy. The pressures were 77/5g, 81/43 (mean 69) and 138/77 (mean 105) mm. Hg in the puImonary abdominal aorta and brachia1 artery, artery, respectively. The cIinica1 diagnosis was coarctation of the aorta, patent ductus arteriosus with a bidirectional shunt, and hyperkinetic puImonary hypertension. At operation the mean pressure of the puImonary artery dropped 15 mm. Hg when the ductus was clamped, and resection of the coarctation and division of the patent ductus arteriosus was then performed with the patient under genera1 anesthesia and hypothermia to
and Shumway
32’~. The ductus was 2 cm. in diameter, Iocated immediatcIy opposite the site of the coarctation. Duration of tota aortic occIusion was fifty-five minutes. PostoperativeIy, brachia1 bIood pressure rose to rljo/roo mm. Hg, and the patient compIained of abdominal pain. PhysicaI examination was noncontributory. He was afebriIe and had good bowe1 sounds. The white bIood ceI1 count and urine examination were within norma Iimits. Treatment was begun with analgesics and reserpine. He had marked side reactions to the reserpine which was therefore discontinued. The pain regressed in three days and he was discharged on the twelfth postoperative day. BIood pressure at discharge was 138/106 and 132/100 mm. Hg in the right arm and right Ieg, respectiveIy. Three months postoperatively, he was asymptomatic with norma bIood pressures and normal cardiac auscultatory findings. On roentgenogram the cardiac siIhouette was decreased in size, and evidence of puImonary overcircuIation had regressed.
CASE III. This two year oId gir1 had a murmur at six weeks of age. She tired easiIy and required frequent feedings, but she had no other difficulty. PhysicaI examination reveaIed that she was in the third percentiIe for her age in height and weight. BIood pressures were 124/82, 75/? and 75/? mm. Hg in the Ieft arm, right arm and Ieft Ieg, respectively. PuIses were absent in the femora1 and right radial arteries. The heart was enIarged to the left. A moderate heave was noted as was a systolic thrill at the base. A grade 2 harsh systolic murmur was heard at the second right interspace and aIong the left sterna1 border. A grade I apica diastoIic murmur couId be heard during periods of tachycardia. The Iiver was paIpabIe, descending two Iingerbreadths beIow the right Costa1 margin. The spIeen was paIpabIe one fingerbreadth below the Ieft Costa1 margin. Clubbing, cyanosis and edema were absent. HemogIobin was I I .6 gm. per cent. Roentgenograms were interpreted as showing biventricular enIargement, possibIe anomalous right subcIavian artery, and pulmonary overcircuIation. An electrocardiogram revealed a sinus rhythm at a rate of 130; the P-R and QRS intervaIs were 0.14 and 0.07 second, respectively; the axis was pIus go degrees. The P waves were peaked; the QRS compIexes in the chest Ieads were compatibIe with right ventricuIar hypertrophy. Cardiac catheterization data revealed an oxygen step-up in the main pulmonary artery of 5 voIumes per cent. The pulmonary artery, right ventricle and right puImonary artery wedge pressures were 64/26, 7g/4 and Hg, respectiveIy. Cineangiography re15 mm. veaIed coarctation of the aorta, possibIe subaortic stenosis, and aberrant or absent right subcIavian artery. The possibility of patent ductus arteriosus could not be ruIed out.
Patent
Ductus
Arteriosis
and Coarctation
of Aorta
5ubclavian
v
CI
arteriosus
FIG. I. This is a drawing of the anatomic situation in Case III. The coarctation is postductal, and the anomalous right subclavian artery departs from the aorta below the coarcted segment.
Because of an enlarging cardiac silhouette and cardiac catheterization data, surgery was recommended. Resection of a postductal coarctation and division of a Iarge ductus were performed on right subNovember 18, 1960. An anomalous clavian artery was Iocated distal to the coarctation. It was ligated and divided. Figure I shows the anatomy of the lesion complex. The bIood pressures postoperativcIy were I 34,’ I 24 and 148 153
mm. Ilg in the left arm and right leg, respec-
tively. A definite pulse was feIt in the right arm for the first time. On the second postoperative day fever and Ieukocytosis deveIoped. BIood, throat and urine cuItures were sterile. All wounds were cIean. Antibiotic therapy was begun empirically. On the seventh postoperative day, roentgenograms revealed the cardiac sifhouette to be greatly enlarged suggesting a pericardial fluid accumulation. The postpericardiotomy syndrome was diagnosed. She was afebriIe at the time of discharge. One month postoperativeIy she was doing
well. The auscuItatory findings of miId aortic stenosis persisted; howxver, blood pressures were 90,/30, 80/20 and 100/60 mm. Hg in the left arm right
arm and left leg, rcspectiveIy. COMMENTS
It is interesting to note the historic evolution of various aspects of this probIem. Isolated
case reports, casua1 discussion and classification of the combination of Iesions were recorded by Fawcett [I?], Abbott [II, Bonett 1~51 and others. The significance of various clinica findings, such as differential cyanosis, status of collaterals and the cIassification of coarctation into “aduIt” and “infantile” forms, was much emphasized. The development of a successful operation for coarctation alone allowed the study of coarctation of the aorta associated with patent ductus arteriosus to be more than of passing interest. Large series of cases of coarctation were then published, xvith the incidence of associated patent ductus arteriosus about IO to 15 per cent in the “adult” type [zIu,r9]. Because of the consideration of technical ease and the growth of anastomotic suture fines, it was recommended that operation for coarctation be delayed unti1 the second decade of life. ExtremeIy good results were noted in these older patients. Onls sporadic surgica1 cases were reported in infants [2,6,23J. The widely quoted series of ninety infants from the Toronto Hospital for Sick Children [?J] points out, however, that in approximately two thirds of all patients with coarctation, symptoms develop in infancy. Of these, 60 to 89 per
Dong,
Lower,
HurIey
cent wilI not survive the first year. The high mortaIity is owing to the frequent association with additiona Iesions. Of associated anomaIies, patent ductus arteriosus is the most frequent: seven of eIeven operative cases by Groves and Euler [20], fourteen of fourteen autopsy cases by GoIdring et a1. [r6J and sixty-nine of 108 consecutive cases reported by GIass et a1. [r5]. Hypertension in the systemic circuit and the consequences of Ieft ventricuIar hypertrophy, aneurysm and congestive heart failure were we11known. It aIso became evident with pathophysioIogic studies that puImonary hypertension [12] secondary to the patent ductus made a bad situation worse. MedicaI management was virtuaIIy hopeIess [3,8,10,15,38]. SurgicaI intervention in these chiIdren was recommended. With the operative approach for coarctation and patent ductus arteriosus in infants, a whoIe host of new probIems arises, such as growth of anastomosis, recoarctation and postoperative compIications. None of these present or past probIems has yet been definitiveIy investigated, and for this reason we present the resuIts of studies on these patients and add our commentaries. Many systems of cIassification have been advocated [22]. Because of the confusing nuances impIied in each author’s cIassification, we agree with those who simpIy describe the coarctation in relation to the ductus, i.e., preductal, juxtaducta1 and postducta1. The cardina1 sign of coarctation is of course the decreased femora1 puIse and bIood pressure. The use of the flush method for determining the systolic bIood pressure in infants has become standard. The armamentarium necessary to make the diagnosis depends on the cIinica1 findings. The first case required no more than the usua1 roentgenograms and an eIectrocardiogram. When cardiac catheterization is performed, as in the second and third incIuding cases, seIective angiocardiography cineangiography studies can be of great heIp in determining the direction of flow, the presence of septa1 defects, and other Iesions, such as subaortic stenosis. One cIinica1 point to emphasize is that the absence of girdIe cyanosis does not rule out a preductal coarctation. In fact girdIe cyanosis usuaIIy does not occur, as in fourteen of fourteen cases of preducta1 coarctation reported by GoIdring et a1. [16]. It is evident that the degree of oxygenation beIow the coarctation is dependent upon the direction of shunting across
and Shumway
the ductus. This, in turn, is reIated to (a) the presence and degree of puImonary hypertension, (6) the gradient of pressure across the coarcted segment, and (c) the gradient of pressures through the coIIatera1 system. The eIectrocardiogram is not diagnostic. Because of the frequency of additiona defects, it is diffIcuIt to anaIyze the reported cases. In juxta- and postducta1 coarctation Ieft ventricuIar hypertrophy appears to be the predominant pattern. Right ventricuIar hypertrophy is probabIy the most frequent pattern in preducta1 coarctation, but combined ventricuIar hypertrophy, as we11 as left ventricular hypertrophy has been noted. Hypertension in the puImonary circuit is an important physioIogic and prognostic consideration. Edwards [r2] in a series of papers emphasizes the vascuIar changes occurring with hypertension. Experimenta studies in adult dogs record puImonary hypertension in preducta1 coarctation and normotension in postducta1 coarctation [g,r7]. PubIished catheterization data in man is as yet scanty [r 1,401. Pulmonary hypertension is the ruIe in pre- or juxtaducta1 coarctation with a reversed or bidirectiona1 flow occurring commonIy, whereas hypertension is Iess common and the direction of Aow is Ieft to right in postducta1 coarctation. This was the situation in our cases. TECHNICS
The presence of the patent ductus apparentIy adds IittIe to the technica diffrcuIty of resecting the coarctation. Indeed, it is somewhat easier to repair the patent ductus arteriosus in the presence of the coarctation. Pott’s ductus cIamps are pIaced across the ductus on the puImonary artery side, and above and beIow the coarctation. The segment is then resected. The puImonary artery segment of the ductus is oversewn, and the aorta anastomosed. Moderate hypothermia to 30 degrees is empIoyed with the objective of proIonging the safe period of CrosscIamping of the aorta. We beIieve this to be of particular importance in patients with bidirectiona1 shunts, in whom the usua1 coIIatera1 system may not be we11 deveIoped due either to Iack or withdrawa of stimuIus. As previousIy suggested, when puImonary hypertension is present, the ductus is crosscIamped and the reaction of the right ventricle and puImonary artery pressure is 258
Patent
Ductus
Arteriosis
and
of Aorta
our patient, the side reactions with reserpine were so marked that it was discontinued. The Iong-term foIIow-up study on hypertension in coarctation has been reported by March et al. [JO]. Abdominal pain occurred in 31 per cent of their patients with coarctations, and in 16 per cent in the contro1 group of isolated patent ductus arteriosus. In follow-up examinations, from one to nine years postoperatively, only two patients (7 per cent) have sustained systoIic and diastoIic hypertension. ApparentIy no patient discharged with norma bIood pressures deveIoped hypertension in the foIIow-up period.
observed carefuhy before any further procedure is performed. In mobihzing the aorta, the intercostaks are aIso mobilized, but onIy a few are divided, with the resuIt that more pathologic aorta can be resected with primary anastomosis. SURGICAL
Coarctation
PROGNOSIS
SuIIavan et aI. [40] cohected forty cases of preducta1 coarctation from the Iiterature with an over-a11 mortaIity of 53 per cent. In patients under one year of age this rises to 68 per cent. Burford et a1. [9] reported a mortality of 44 per cent in sixteen patients under two years of age with patent ductus arteriosus and coarctation, some of whom had additiona defects. It wiI1 be recalled that in one of our patients the coarctation was preducta1 in location and that the other two had puImonary hypertension. AI1 three are doing weI1. With the poor prognosis for a11 cases of patent ductus arteriosus and coarctation, surgery in infants is a Iifesaving procedure. The consideration of suture line growth is secondary at present. Experimenta studies in dogs [8,2 1,391 and angiograms in human subjects [30,42] show a definite lag in growth in the area of the anastomosis, but it appears that the obstruction is not physioIogicaIIy significant. Indeed, the stenosis may be reIated to pathologic aortic tissue Ieft behind. One case of recoarctation reported by MuIder and Linde [32] occurred five weeks after surgery. The second resected segment reveaIed marked proliferative changes in the aortic waI1 identica1 to those in the original specimen. The high mortaIity reported in previous series of patients with patent ductus arteriosus and coarctation included may patients with still additiona defects. If in the Iong-term follow-up study, troubIe with re-stenosis does occur, it may we11 be that surgery couId be deIayed in a selected group, such as those with isolated postducta1 coarctation. Another major consideration is reIated to the occurrence of systemic hypertension. The syndrome of post-coarctation abdominal pain and hypertension is now we11 known, first reported by SeaIy f37J and emphasized by Ring and Lewis [34] and others [5,28]. Only one of our patients manifested this complication. Therap?- with autonomic bIocking agents has been suggested. AIthough it was attempted in
ANOMALOUS
RIGHT
SUBCLAVIAN
ARTERY
Case III presented a remarkabIe combination of Iesions. In 1794, the term, dysphagia Iusoria, was coined to describe the fact that an anomaIous right subcIavian artery arising beIow the Ieft subclavian, formed a vascular ring about the esophagus [35], and may give rise to symptoms of dysphagia. This lesion occurs in 0.4 to 0.16 per cent of autopsies. The radiologic diagnosis may be made by finding a diagonaI impression in the esophagus on barium swallow. SeaIy [37] reported the first case of anomaIous right subcIavian artery taking off beIow the coarctation. A number of simiIar cases has been recorded since. In Case III such a vessel was ligated and divided. It was immediately noted that the dista1 segment of subcIavian artery dilated, and a p&e was detected in the arm. This wouId imply that the direction of 110~ was from the subcIavian artery into the aorta, the subclavian artery acting as a coIIatera1 to the postcoarcted aorta. Hence, the source of bIood into the right radia1 artery is from anastomotic channels and not from the aorta. Indeed, it can be deduced that the aorta acted as a runoff drain, and when the subctavian was interrupted, the entire anastomotic flow was diverted into the arm with the appearance of the pulse and bIood pressure. A study of this phenomenon was recorded by the Mayo CIinic [24]. SUMMARY
Three successfu1 surgica1 cases of coarctation of the aorta associated with patent ductus arteriosus are presented; representing one of each anatomica type: preductal, juxtaducta1 and postductal. The additiona anomaIy of the right subcIavian artery arising below the coarctation is aIso discussed. 239
Dong,
Lower,
Hurley
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and Shumway
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260
I
Patent 38.
SI..AZIEI\,
the 39.
10.
h?.
aorta
B.
;rnd
with
Ductus
Arteriosis
GOLDKING, D. &uxWtion patent
ductus
artcriosus.
and Coarctation
of J.
41. TAYLOR, B. E., hUTSON, J. B., Ur HC~HEI.,., II.B., DAI'GIIERTY, G. LX'.and \?‘oon, E:. II. P:ltcnt ductus arteriosus with coarctntion: report of two cases studied before and after surgicxl trentmrnt. I’ TOC. Staff Meet. hfa~~~oClin., 25 : 62, rc)qo.
f’ediul., 47: 588, 1955. SFII;MACKEK, I I. B., FKEEMAK, L. W., HUTCIIINGS, L. II.and I<,\DIc;AY, L. Stud& in vnscular repair. Angiolog.y, 2: 263, 195 I. SC LLLAVA~, J. J. and MANGIARDI, J. L. Preductaf coarctation of the aorta and patent ductus. Ann. -pure.,
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42.
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261
T~vro~, 1~. G., Dow, J. D. and BKOCK, K. Aortogram after resection of coarctation of the aorta and insertion of a homograft. Gu,r’.s F-los~~. Kelp., 108: 388, 1959.
EUGENE
Management of Patent Ductus Arteriosus Associated with Coarctation of the Aorta
DONG, JR., M.D., RICHARD R. LOSER, M.D., EDWARD J. HURLEY, M.D. AND NORMAN E. SHUMWAY, M.D., Palo Alto, Calijorniu
From tbe Department of Surgery, Stanford University School oJ Medicine, Palo Alto, CaliJomia. Tbis work was supported in part by Kesearcb Grant H-4658 of tbe Notional Heart Institute.
ITH continuing progress in the diagnosis and treatment of congenita1 cardiovascuIar disease, the lesion of coarctation of the aorta associated with patent ductus arteriosus is being reported more frequently in the surgical and pediatric literature. Prior to 1951 [1,10,1?,18,22,26,41] only a handful of cases had been reported. Since that time an increasing number of cases have been described from a few centers [y,r4-17,331. Forty-five to 70 per cent of these patients had additiona anomaIies, however, and surgical experience was limited. We believe it of interest and import, therefore, to report an additiona series of three successful surgical repairs of coarctation associated with patent ductus arteriosus.
W
CASE
REPORTS
A three months old white gir1 was CASE 1. deli\.cred two weeks prior to the estimated date because of a rising materna1 anti-rH titer. Her birth weight was 4 pounds, 14 ounces. She was we11 until the age of two months. At that time, she was hospitalized with anorexia, dyspnea and signs of congestive heart failure which responded to digitaIis therapy. One month Iater, anorexia, Ioose stools and easy fatigability again vomiting, developed. Signs of congestive faiIure were noted. She was readmitted to Palo AIto-Stanford Hospitals on November 17, 1960. Pulse was 146, bounding in the upper extremities, but barely paIpabIe in the Iower extremities. Recorded blood pressures arm, and 60 werr 175,/o mm. Hg in the right mm. Hg systolic in the right Ieg. The respiratory rate was 52. The heart was enlarged to the Ieft;
the second puImonic sound was spIit and louder than the second aortic sound; a grade 3 continuous murmur was heard over the entire upper Ieft part of the chest. The systolic component was heard over the precordium and back as well. The Iiver was enIarged, descending 4 to 5 cm. at the right costal margin; the spIeen was paIpabIe I cm. below the Ieft costal margin. There was no edema, clubbing or cyanosis. Chest roentgenograms revealed marked cardiomegaly, increased puImonary vascularity and enlargement of the left atrium. An eIectrocardiogram showed a normal sinus rhythm at a rate of 130: the P-R and QRS intervals were o. 12 and 0.06 second, respectively; the axis was plus 80 degrees; the S-T segments were sagging in Ieads I and aVL, and depressed in leads Vy to Vg; the T waves were inverted in IeacIs I, aVL., V,R and VI to Va; diphasic in Ieads VI and Vs. The diagnosis of coarctation of the aorta and patent ductus arteriosus was made on clinical grounds. The congestive failure responded to digitalis and diuretic therapy. At operation on November 23, 1960, a preducta1 coarctation of the aorta was resected and a patent ductus arteriosus divided. The coarcted segment was 0.5 cm. long; the ductus was 0.75 cm. in diameter and equal in size to the aorta. Reanastomosis of the aorta was accomplished without dificulty using a running cardiovascular suture posteriorly and interrupted evcrting sutures on the anterior row. Total time of aortir occlusion was thirty-seven minutes. The postoperative course was uncomplicated. The recorded blood pressures were 185.‘1 IO mm. Hg in the left arm on the first postoperative day, and 126’68 mm. Hg in the right arm and 130/7o mm. Hg in the left Ieg on discharge. One month postoperatively, the leg pulses wcrc easily palpablc; blood pressure in the arm was 144,‘73 mm. IIg. The chest roentgenogram sholvrd a normal puImonary vascularity, and the cardiac silhouette had diminished in size.
Dong, Lower,
HurIey
CASE II. This seven year oId boy was first seen at PaIo Alto-Stanford HospitaIs on November 27, 1959, for cardiac evaluation. A murmur was noted in infancy, and except for easy fatigability, he had been asymptomatic. Past and family history was not pertinent. Physical examination reveaIed a we11 developed, we11 nourished boy. PuIse was g6 per minute, paIpabIe in the upper extremities and absent in the lower extremities. BIood pressures were 130/7o mm. Hg (arm) and 52 mm. Hg systoIic (leg). Respirations were 24 per minute. There was a funne1 chest deformity, more marked on the Ieft side. A right ventricmar heave was noted. The heart was enIarged to the left. The second puImonic sound was sIightIy spIit and much louder than the second aortic sound. A grade 2 rough systoIic murmur, with a “sea guI1” quaIity was heard aIong the upper Ieft sterna1 border and in the infracIavicuIar area. A grade 3 blowing, earIy diastolic, decreasing murmur was noted in the pulmonic area. A grade 3 short systolic murmur along the lower Ieft sterna1 border, and a grade 2 rumbling, apical, mid-diastoIic murmur were aIso heard. The murmur at the base at times seemed continuous. The lungs were clear. The liver and were paIpabIe at the costal margins. spIeen CIubbing of the toes was definite, but cyanosis and edema were absent. HemogIobin was 15 gm. per cent. An eIectrocardiogram showed a norma sinus rhythm at a rate of 82; the P-R and QRS intervals were 0.16 and 0.08 second, respectively; the axis was plus go degrees. There was a notched R wave with an upright T wave in Iead VaR with the R wave there was an aRs measuring 22 mm. in height; wave in Iead Vs with the R wave measuring I 6 mm. The interpretation was right ventricuIar hypertrophy. Roentgenograms reveaIed cardiomegaIy involving all four chambers and marked enlargement of the Ieft atrium; the ascending aorta was dilated; a bidirectiona1 shunt was seen at the IeveI of the ductus by means of cineangiography. A coarctation of the aorta was also noted. Cardiac catheterization reveaIed an oxygen step-up in the main pulmonary artery of 8 voIumes per cent; the oxygen saturation was 89 and 97 per cent in the abdomina1 aorta and brachia1 artery, respectiveIy. The pressures were 77/5g, 81/43 (mean 69) and 138/77 (mean 105) mm. Hg in the puImonary abdominal aorta and brachia1 artery, artery, respectively. The cIinica1 diagnosis was coarctation of the aorta, patent ductus arteriosus with a bidirectional shunt, and hyperkinetic puImonary hypertension. At operation the mean pressure of the puImonary artery dropped 15 mm. Hg when the ductus was clamped, and resection of the coarctation and division of the patent ductus arteriosus was then performed with the patient under genera1 anesthesia and hypothermia to
and Shumway
32’~. The ductus was 2 cm. in diameter, Iocated immediatcIy opposite the site of the coarctation. Duration of tota aortic occIusion was fifty-five minutes. PostoperativeIy, brachia1 bIood pressure rose to rljo/roo mm. Hg, and the patient compIained of abdominal pain. PhysicaI examination was noncontributory. He was afebriIe and had good bowe1 sounds. The white bIood ceI1 count and urine examination were within norma Iimits. Treatment was begun with analgesics and reserpine. He had marked side reactions to the reserpine which was therefore discontinued. The pain regressed in three days and he was discharged on the twelfth postoperative day. BIood pressure at discharge was 138/106 and 132/100 mm. Hg in the right arm and right Ieg, respectiveIy. Three months postoperatively, he was asymptomatic with norma bIood pressures and normal cardiac auscultatory findings. On roentgenogram the cardiac siIhouette was decreased in size, and evidence of puImonary overcircuIation had regressed.
CASE III. This two year oId gir1 had a murmur at six weeks of age. She tired easiIy and required frequent feedings, but she had no other difficulty. PhysicaI examination reveaIed that she was in the third percentiIe for her age in height and weight. BIood pressures were 124/82, 75/? and 75/? mm. Hg in the Ieft arm, right arm and Ieft Ieg, respectively. PuIses were absent in the femora1 and right radial arteries. The heart was enIarged to the left. A moderate heave was noted as was a systolic thrill at the base. A grade 2 harsh systolic murmur was heard at the second right interspace and aIong the left sterna1 border. A grade I apica diastoIic murmur couId be heard during periods of tachycardia. The Iiver was paIpabIe, descending two Iingerbreadths beIow the right Costa1 margin. The spIeen was paIpabIe one fingerbreadth below the Ieft Costa1 margin. Clubbing, cyanosis and edema were absent. HemogIobin was I I .6 gm. per cent. Roentgenograms were interpreted as showing biventricular enIargement, possibIe anomalous right subcIavian artery, and pulmonary overcircuIation. An electrocardiogram revealed a sinus rhythm at a rate of 130; the P-R and QRS intervaIs were 0.14 and 0.07 second, respectively; the axis was pIus go degrees. The P waves were peaked; the QRS compIexes in the chest Ieads were compatibIe with right ventricuIar hypertrophy. Cardiac catheterization data revealed an oxygen step-up in the main pulmonary artery of 5 voIumes per cent. The pulmonary artery, right ventricle and right puImonary artery wedge pressures were 64/26, 7g/4 and Hg, respectiveIy. Cineangiography re15 mm. veaIed coarctation of the aorta, possibIe subaortic stenosis, and aberrant or absent right subcIavian artery. The possibility of patent ductus arteriosus could not be ruIed out.
Patent
Ductus
Arteriosis
and Coarctation
of Aorta
5ubclavian
v
CI
arteriosus
FIG. I. This is a drawing of the anatomic situation in Case III. The coarctation is postductal, and the anomalous right subclavian artery departs from the aorta below the coarcted segment.
Because of an enlarging cardiac silhouette and cardiac catheterization data, surgery was recommended. Resection of a postductal coarctation and division of a Iarge ductus were performed on right subNovember 18, 1960. An anomalous clavian artery was Iocated distal to the coarctation. It was ligated and divided. Figure I shows the anatomy of the lesion complex. The bIood pressures postoperativcIy were I 34,’ I 24 and 148 153
mm. Ilg in the left arm and right leg, respec-
tively. A definite pulse was feIt in the right arm for the first time. On the second postoperative day fever and Ieukocytosis deveIoped. BIood, throat and urine cuItures were sterile. All wounds were cIean. Antibiotic therapy was begun empirically. On the seventh postoperative day, roentgenograms revealed the cardiac sifhouette to be greatly enlarged suggesting a pericardial fluid accumulation. The postpericardiotomy syndrome was diagnosed. She was afebriIe at the time of discharge. One month postoperativeIy she was doing
well. The auscuItatory findings of miId aortic stenosis persisted; howxver, blood pressures were 90,/30, 80/20 and 100/60 mm. Hg in the left arm right
arm and left leg, rcspectiveIy. COMMENTS
It is interesting to note the historic evolution of various aspects of this probIem. Isolated
case reports, casua1 discussion and classification of the combination of Iesions were recorded by Fawcett [I?], Abbott [II, Bonett 1~51 and others. The significance of various clinica findings, such as differential cyanosis, status of collaterals and the cIassification of coarctation into “aduIt” and “infantile” forms, was much emphasized. The development of a successful operation for coarctation alone allowed the study of coarctation of the aorta associated with patent ductus arteriosus to be more than of passing interest. Large series of cases of coarctation were then published, xvith the incidence of associated patent ductus arteriosus about IO to 15 per cent in the “adult” type [zIu,r9]. Because of the consideration of technical ease and the growth of anastomotic suture fines, it was recommended that operation for coarctation be delayed unti1 the second decade of life. ExtremeIy good results were noted in these older patients. Onls sporadic surgica1 cases were reported in infants [2,6,23J. The widely quoted series of ninety infants from the Toronto Hospital for Sick Children [?J] points out, however, that in approximately two thirds of all patients with coarctation, symptoms develop in infancy. Of these, 60 to 89 per
Dong,
Lower,
HurIey
cent wilI not survive the first year. The high mortaIity is owing to the frequent association with additiona Iesions. Of associated anomaIies, patent ductus arteriosus is the most frequent: seven of eIeven operative cases by Groves and Euler [20], fourteen of fourteen autopsy cases by GoIdring et a1. [r6J and sixty-nine of 108 consecutive cases reported by GIass et a1. [r5]. Hypertension in the systemic circuit and the consequences of Ieft ventricuIar hypertrophy, aneurysm and congestive heart failure were we11known. It aIso became evident with pathophysioIogic studies that puImonary hypertension [12] secondary to the patent ductus made a bad situation worse. MedicaI management was virtuaIIy hopeIess [3,8,10,15,38]. SurgicaI intervention in these chiIdren was recommended. With the operative approach for coarctation and patent ductus arteriosus in infants, a whoIe host of new probIems arises, such as growth of anastomosis, recoarctation and postoperative compIications. None of these present or past probIems has yet been definitiveIy investigated, and for this reason we present the resuIts of studies on these patients and add our commentaries. Many systems of cIassification have been advocated [22]. Because of the confusing nuances impIied in each author’s cIassification, we agree with those who simpIy describe the coarctation in relation to the ductus, i.e., preductal, juxtaducta1 and postducta1. The cardina1 sign of coarctation is of course the decreased femora1 puIse and bIood pressure. The use of the flush method for determining the systolic bIood pressure in infants has become standard. The armamentarium necessary to make the diagnosis depends on the cIinica1 findings. The first case required no more than the usua1 roentgenograms and an eIectrocardiogram. When cardiac catheterization is performed, as in the second and third incIuding cases, seIective angiocardiography cineangiography studies can be of great heIp in determining the direction of flow, the presence of septa1 defects, and other Iesions, such as subaortic stenosis. One cIinica1 point to emphasize is that the absence of girdIe cyanosis does not rule out a preductal coarctation. In fact girdIe cyanosis usuaIIy does not occur, as in fourteen of fourteen cases of preducta1 coarctation reported by GoIdring et a1. [16]. It is evident that the degree of oxygenation beIow the coarctation is dependent upon the direction of shunting across
and Shumway
the ductus. This, in turn, is reIated to (a) the presence and degree of puImonary hypertension, (6) the gradient of pressure across the coarcted segment, and (c) the gradient of pressures through the coIIatera1 system. The eIectrocardiogram is not diagnostic. Because of the frequency of additiona defects, it is diffIcuIt to anaIyze the reported cases. In juxta- and postducta1 coarctation Ieft ventricuIar hypertrophy appears to be the predominant pattern. Right ventricuIar hypertrophy is probabIy the most frequent pattern in preducta1 coarctation, but combined ventricuIar hypertrophy, as we11 as left ventricular hypertrophy has been noted. Hypertension in the puImonary circuit is an important physioIogic and prognostic consideration. Edwards [r2] in a series of papers emphasizes the vascuIar changes occurring with hypertension. Experimenta studies in adult dogs record puImonary hypertension in preducta1 coarctation and normotension in postducta1 coarctation [g,r7]. PubIished catheterization data in man is as yet scanty [r 1,401. Pulmonary hypertension is the ruIe in pre- or juxtaducta1 coarctation with a reversed or bidirectiona1 flow occurring commonIy, whereas hypertension is Iess common and the direction of Aow is Ieft to right in postducta1 coarctation. This was the situation in our cases. TECHNICS
The presence of the patent ductus apparentIy adds IittIe to the technica diffrcuIty of resecting the coarctation. Indeed, it is somewhat easier to repair the patent ductus arteriosus in the presence of the coarctation. Pott’s ductus cIamps are pIaced across the ductus on the puImonary artery side, and above and beIow the coarctation. The segment is then resected. The puImonary artery segment of the ductus is oversewn, and the aorta anastomosed. Moderate hypothermia to 30 degrees is empIoyed with the objective of proIonging the safe period of CrosscIamping of the aorta. We beIieve this to be of particular importance in patients with bidirectiona1 shunts, in whom the usua1 coIIatera1 system may not be we11 deveIoped due either to Iack or withdrawa of stimuIus. As previousIy suggested, when puImonary hypertension is present, the ductus is crosscIamped and the reaction of the right ventricle and puImonary artery pressure is 258
Patent
Ductus
Arteriosis
and
of Aorta
our patient, the side reactions with reserpine were so marked that it was discontinued. The Iong-term foIIow-up study on hypertension in coarctation has been reported by March et al. [JO]. Abdominal pain occurred in 31 per cent of their patients with coarctations, and in 16 per cent in the contro1 group of isolated patent ductus arteriosus. In follow-up examinations, from one to nine years postoperatively, only two patients (7 per cent) have sustained systoIic and diastoIic hypertension. ApparentIy no patient discharged with norma bIood pressures deveIoped hypertension in the foIIow-up period.
observed carefuhy before any further procedure is performed. In mobihzing the aorta, the intercostaks are aIso mobilized, but onIy a few are divided, with the resuIt that more pathologic aorta can be resected with primary anastomosis. SURGICAL
Coarctation
PROGNOSIS
SuIIavan et aI. [40] cohected forty cases of preducta1 coarctation from the Iiterature with an over-a11 mortaIity of 53 per cent. In patients under one year of age this rises to 68 per cent. Burford et a1. [9] reported a mortality of 44 per cent in sixteen patients under two years of age with patent ductus arteriosus and coarctation, some of whom had additiona defects. It wiI1 be recalled that in one of our patients the coarctation was preducta1 in location and that the other two had puImonary hypertension. AI1 three are doing weI1. With the poor prognosis for a11 cases of patent ductus arteriosus and coarctation, surgery in infants is a Iifesaving procedure. The consideration of suture line growth is secondary at present. Experimenta studies in dogs [8,2 1,391 and angiograms in human subjects [30,42] show a definite lag in growth in the area of the anastomosis, but it appears that the obstruction is not physioIogicaIIy significant. Indeed, the stenosis may be reIated to pathologic aortic tissue Ieft behind. One case of recoarctation reported by MuIder and Linde [32] occurred five weeks after surgery. The second resected segment reveaIed marked proliferative changes in the aortic waI1 identica1 to those in the original specimen. The high mortaIity reported in previous series of patients with patent ductus arteriosus and coarctation included may patients with still additiona defects. If in the Iong-term follow-up study, troubIe with re-stenosis does occur, it may we11 be that surgery couId be deIayed in a selected group, such as those with isolated postducta1 coarctation. Another major consideration is reIated to the occurrence of systemic hypertension. The syndrome of post-coarctation abdominal pain and hypertension is now we11 known, first reported by SeaIy f37J and emphasized by Ring and Lewis [34] and others [5,28]. Only one of our patients manifested this complication. Therap?- with autonomic bIocking agents has been suggested. AIthough it was attempted in
ANOMALOUS
RIGHT
SUBCLAVIAN
ARTERY
Case III presented a remarkabIe combination of Iesions. In 1794, the term, dysphagia Iusoria, was coined to describe the fact that an anomaIous right subcIavian artery arising beIow the Ieft subclavian, formed a vascular ring about the esophagus [35], and may give rise to symptoms of dysphagia. This lesion occurs in 0.4 to 0.16 per cent of autopsies. The radiologic diagnosis may be made by finding a diagonaI impression in the esophagus on barium swallow. SeaIy [37] reported the first case of anomaIous right subcIavian artery taking off beIow the coarctation. A number of simiIar cases has been recorded since. In Case III such a vessel was ligated and divided. It was immediately noted that the dista1 segment of subcIavian artery dilated, and a p&e was detected in the arm. This wouId imply that the direction of 110~ was from the subcIavian artery into the aorta, the subclavian artery acting as a coIIatera1 to the postcoarcted aorta. Hence, the source of bIood into the right radia1 artery is from anastomotic channels and not from the aorta. Indeed, it can be deduced that the aorta acted as a runoff drain, and when the subctavian was interrupted, the entire anastomotic flow was diverted into the arm with the appearance of the pulse and bIood pressure. A study of this phenomenon was recorded by the Mayo CIinic [24]. SUMMARY
Three successfu1 surgica1 cases of coarctation of the aorta associated with patent ductus arteriosus are presented; representing one of each anatomica type: preductal, juxtaducta1 and postductal. The additiona anomaIy of the right subcIavian artery arising below the coarctation is aIso discussed. 239
Dong,
Lower,
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and Shumway
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260
I
Patent 38.
SI..AZIEI\,
the 39.
10.
h?.
aorta
B.
;rnd
with
Ductus
Arteriosis
GOLDKING, D. &uxWtion patent
ductus
artcriosus.
and Coarctation
of J.
41. TAYLOR, B. E., hUTSON, J. B., Ur HC~HEI.,., II.B., DAI'GIIERTY, G. LX'.and \?‘oon, E:. II. P:ltcnt ductus arteriosus with coarctntion: report of two cases studied before and after surgicxl trentmrnt. I’ TOC. Staff Meet. hfa~~~oClin., 25 : 62, rc)qo.
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