Aneurysm of the nonpatent ductus arteriosus

Aneurysm of the Nonpatent Ductus Arteriosus

M. WAYNE FALCONE, MD* JOSEPH K. PERLOFF, MD, FACC; WILLIAM C. ROBERTS, MD, FACC

Bethesda,

Maryland

Clinical and necropsy findings are described in a 6 week old infant with aneurysm of the ductus arteriosus. It was obliterated at the pulmonary arterial end and patent at the aortic end. Observations in this case and in 60 previously described cases of ductal aneurysm disclosed that 46 of the aneurysms occurred in infants less than 2 months old, 4 in children, and 11 in adults. The aortic end of the ductal aneurysm is always patent. The pulmonary arterial end may or may not be patent. Since complications (rupture, embolism or infection) tend to develop in nearly half of the ductal aneurysms, operative resection appears indicated.

Persistent patency of the ductus arteriosus is a common congenital cardiovascular malformation. Aneurysmal dilatation is an occasional complication of patent ductus arteriosus in adults. Aneurysm of the ductus arteriosus in infancy-in contrast to simple patency-is rare. However, such was the case in an infant we recently studied. Clinical and necropsy observations in this patient with ductal aneurysm and in those previously reported by others are described in this communication.

Report of Patient

From the Departments of Medicine and Pediatrics, Georgetown University School Division of Cardiology, of Medicine, Georgetown University Hospital, Washington, D. C., and the Section of Pathology, National Heart and Lung Institute, National Institutes of Health, Bethesda, Md. Manuscript received January 8, 1971, accepted March 25, 1971. * Clinical fellow of the Washington Heart Association, Washington, D. C. t Holder of Research Career Program Award HE 14,009 from the U. S. Public Health Service, Bethesda, Md. Address for reprints: William C. Roberts, MD, Section of Pathology, National Heart and Lung Institute, National Institutes of Health, 9000 Rockville Pike, Bethesda, Md. 20014.

422

A 52 day old white boy, the first-born of twins, delivered at a referring hospital, weighed 2.2 kg after a term gestation. At birth he showed flaccidity. cyanosis and regurgitation; Apgar scores were 2 and 8 at 1 and 5 minutes, respectively. Positive pressure respirator and intubation were required initially with spontaneous onset of respirations at 5 minutes. He had multiple congenital abnormalities including an elongated neck, cloudy cornea, low-set and posteriorly rotated ears, complete cleft palate, long thumbs, fetal hypospadius and undescended testes. No precordial murmur was ever heard. Chest roentgenogram (Fig. 1) showed cardiomegaly. A large convex shadow at the left base was initially thought to be thymus. On day 1, the hematocrit was 33 vol percent; leukocyte and platelet counts and serum electrolytes, including calcium and phosphorus, were normal. Chromosome pattern was 46 XY without any structural abnormality. Initially the infant was hypotonic, fed poorly and had respiratory difficulties. Barium swallow on day 12 ruled out tracheoesophageal fistula. The cardiac silhouette was smaller (Fig. 1). The shadow in the angle between the aortic knob and pulmonary trunk was still interpreted as representing thymus. The baby continued to lose weight and by day 22 he began to vomit almost all feedings, necessitating parenteral fluids to maintain electrolyte and caloric balance. The next day a mass was palpated in the right upper quadrant of the abdomen, and upper gastrointestinal series demonstrated pyloric stenosis (Fig. 1). On the same day, he was trans-

The

American

Journal

of

CARDIOLOGY

ANEURYSM

OF DUCTUS

ARTERIOSUS

Figure 1. Chest roentgenograms. The arrows designate the ductal aneurysm. The cardiac size decreased from day 1 to day 23. Roentgenogram on the right demonstrates, in addition, pyloric stenosis with complete retention of contrast material 45 minutes after instillation of barium into the esophagus.

Figure 2. Unopened and opened views of the ductal aneurysm demonstrating its relation to surrounding structures. D.A. = ductal aneurysm; Inn. = innominate; L.P.A. = left main pulmonary artery; L.V. = left ventricle; P.T. = pulmonary trunk; R.V. = right ventricle: T. z trachea.

Photomicrographs of ductal anFigure 3. eurysm (a), and of portion of wal’l of aorta (b), ductal aneurysm (c) and left main pulmonary artery (L.P.A.) (d). The sites at which photomicrographs b, c and d were taken are shown by small blocks in a. The configuration of the elastic fibrils in the aorta (b) and left main pulmonary artery (d) are virtually identical. In contrast, fewer elastic fibrils are present in the wall of the ductal aneurysm (c), and the fibrils at this site have various spatial orientations. Movat stain, x 6 (a); elastic Van Gieson stains, each x 250 (b to d), all reduced by 43 percent.

VOLUME

29, MARCH 1972

423

FALCONE ET AL.

TABLE I

TABLE

Aneurysm

II

Spontaneous*

of the Ductus Arteriosus

Aneurysm

of the Ductus Arteriosus

I. Patent aortic and pulmonary arterial ends A. Fusiform* (with or without aneurysmal thrombus) 1. ,nfe~ted’,‘3,19,~o,23,zs 2

Anatomic Classification

Noninfected3,6-8,22,2*,2”,n.31,38,10

B. Tubular dilatationtl6Jsvz* C. Dissection10,12,91,er II. Nonpatent pulmonary arterial end A. Fusiform* (with or without aneurysmal 1. lnfected33J4 2 Nonjnfe~tedl~l4,27~20~~6-37,42~43

InRup- Embo- feeture lism tion

Age Range

no.

Assoc Mal

Patent ductal aneurysm Fu siform

19

2 days-51 yr

2

5

5

3t

Tubular dilatation Dissection

3 7

3 days-27 days 12 hr-1 mo

0 3

1 0

0 0

0 l$

* Both aortic and pulmonary arterial openings are small in comparison to the diameterof the aneurysm. topenings into aorta and pulmonary artery are of similar diameterto thatof the aneurysm. $ After operative ligation of a patentductusarteriosus.

Nonpatent ductal aneurysm Fusiform Totals

13 421

13 days-66 yr 12 hr-66 yr

4 9

2 8

2 7

60 10

ferred to Georgetown University Hospital, and pyloromyotomy under local anesthesia was performed immediately. Postoperatively, he had several apneic spells and continued to feed poorly; poor Moro and suck reflexes and generalized hypotonicity persisted. He died without an obvious precipitating cause. At necropsg, a 1.5 by 2 by 1 cm ductal aneurysm containing thrombus was found (Fig. 2). The pulmonary arterial end of the ductus was closed, and the aortic end was open. The wall of the aneurysm was composed primarily of smooth muscle and elastic fibrils; young

* Excludes postoperative patients. incompetent patent foramen t Extrophie vesicale,8 valvular ovale 31aortic coarctation.41 $ Hidrocele.27 8 Exomphaloqz double pelvis and ureter,33 atresia of left

III.

thrombus)

Postoperative$62s63

pulmonary artery, stenosis of right pulmonary artery and aneurysm of pulmonary trunk,36 right aortic arch,3Qcoarctation of aorta.42 7 Autopsy in 39. Assoc Mat = associated

congenital

malformations

connective tissue with focal calcific deposits was present at the junction of intraaneurysmal thrombus and ductal wall (Fig. 3). The right. atrium was dilated, and the valve over the fossa ovale was incompetent. No evidence of rupture, embolism or infection of the aneurysm was present.

Discussion Ductal aneurysm was described originally by Martin* in 182’7 and by Billard2 in 1828. Thore3 in 1850 reported 8 cases of ductal aneurysm, and Hammerschlag4 in 1925 reviewed the literature and excluded cases of aortic aneurysms involving the site of ductal insertion. Cruickshank and Marquis5 in 1958 summarized the previous literature and added the tenth adult case. At least 60 cases of ductal aneurysm have been reported.lA3 The anatomic types are summarized in Table I and Figures 4 and 5. Exact anatomic classification is not possible in 19 cases3~8,9,11,14.15.1~,27.29,32 because of insufficient information regarding the status of the pulmonary arterial end of the ductus. Six cases44m49previously considered ductal aneurysm were excluded because of inadequate anatomic detail, Observations in the remaining 42 cases are summarized in Table II. Ductal aneurysm also may be classified by ageinfantile, childhood and adult. Complications of the aneurysm in the 61 patients grouped by age are summarized in Table III and Figure 4. Infantile ductal aneurysm : Nonpatent ductal aneurysm has been reported in 13 patients, includ424

liFECTI6 Figure 4. Diagram of aneurysm of the nonpatent ductus arteriosus and its complications. LPA = left main pulmonary artery; PT = pulmonary trunk; RPA = right main pulmonary artery.

Figure 5. Diagram of aneurysm of the patent ductus arteriosus. In the fusiform aneurysm, the lumen contains thrombus which may or may not occlude blood flow. In the dissecting aneurysm a hematoma is present in the wall of the ductus but not in its lumen. Abbreviations as in Figure 4.

The American

Journal

of CARDIOLOGY

ANEURYSM

ing the infant described herein. The pulmonary arterial end was closed and the aortic end open in all 13. The pathogenesis of nonpatent ductal aneurysm is uncertain. Normally, the ductus arteriosus closes physiologically shortly after birth, but anatomic closure requires several days. By intimal proliferation50 the ductus becomes obliterated first at the pulmonary arterial end, then the aortic end.21v51 The walls of the ductus undergo involutional changes by a decrease in elastica in comparison with the pulmonary trunk and aorta, and by increase of fibrous connective tissue, giving rise to the ligamenturn arteriosum. If closure of the aortic end is delayed, the ductus in effect becomes an aortic diverticulum, subject to systemic pressure at a time when its walls are undergoing involution. This circumstance favors dilatation of the patent portion of the ductus with subsequent aneurysm formation. This mechanism is the most attractive explanation of nonpatent ductal aneurysm in the infants described, all of whom were less than 2 months old. Necrosis of the walls of the ductus was thought to have been etiologically important in several cases23,26J7 but was not present in our patient. In those cases in which the histologic features were presented, decrease in elastic tissue in the wall of the aneurysm was observed. Adult ductal aneurysm: The origin of nonpatent ductal aneurysms in adults is less certain. Documented persistence into adulthood of an infantile ductal aneurysm is lacking. However, the anatomic similarities between adult and infantile ductal aneurysms suggest that the adult ductal aneurysm may represent persistence of the infantile variety. Other mechanisms have been proposed to explain adult nonpatent ductal aneurysm including trauma to a persistently patent ductus,38-40 infective arteritis in a patient with subsequent inflammatory closure of the pulmonary arterial end,33 and delayed spontaneous closure with arrest at the stage of obliteration of the pulmonary arterial end. The latter mechanism, long considered the most likely one, was recently challenged by Bosman and Leoncini42 as not being responsible by itself for aneurysm formation. These authors described a 66 year old man with a ductal aneurysm located just proximal to an aortic coarctation. They reasoned that in addition to obliteration of the pulmonary arterial end there must be some distal aortic obstruction causing increased systemic pressures in the patent portion of the ductus in order to initiate an aneurysm; 2 similar previously described patients were cited.37,41 Perhaps this additional lesion is necessary in the genesis of aneurysm in some adults with arrest at the stage of obliteration of the pulmonary arterial end ; the ductus under these circumstances has been subject to systemic pressure for a considerable period of time and there would not appear to be any stimulus for the walls to undergo involution. In fact, VOLUME

29. MARCH 1972

TABLE

OF DUCTUS ARTERIOSUS

III

Spontaneous* Aneurysm of the Ductus Arteriosus Complications Classification

no.

Age Range

Infantile3+-31 Childhood32--36 Ad~lt”,W-“3

46 4 11

12 hr-2 mo 20 mo-15 yr 23 yr-66 yr

Totals

61t

12 hr-66 yr

* Excludes t Autopsy

postoperative in 57.

Rupture 5 3 3 11

Embo- Infeclism tion

Assoc Mal

6 1 11

5 1

5 1 4

8

7

10

patients.

in the majority of patients with isolated delayed spontaneous closure, the ductus appears as an inverted cone with the apex at the pulmonary arterial end and the base at the aortic end. Whether the pathogenesis of ductal aneurysm in childhood is the same as that operative in the infant or the adult remains uncertain. Diagnosis : Correct antemortem diagnosis of ductal aneurysm has not been established in any infant. Because of the lack of related symptoms or physical signs, attention has not been drawn to the cardiovascular system in this age group. A soft tissue density at the upper border of the left cardiac silhouette may be mistaken for thymus, as it was in our patient (Fig. 1), or hilar adenopathy, as in Scheef’s patient.20 A nonpatent ductal aneurysm was established by angiocardiography and successfully resected in 1 patient35 in the childhood group. The diagnosis has been made antemortem in only 1 adult patient.43 The soft tissue density seen on chest roentgenogram was believed to represent aortic aneurysm in 2 patients,5*42 and tumor of the mediastinum in a third.38 Grahama incised a ductal aneurysm, having considered it to be a solid mediastinal tumor, and emphasized the importance of the correct diagnosis. He pointed out that the differential diagnosis of a mediastinal mass at the level of the pulmonary trunk with displacement of the trachea rightward must include ductal aneurysm. of ductal aneuComplications : Complications rysm resemble those of aqrtic aneurysm: rupture, embolism and infection. Of the 13 nonpatent ductal aneurysms reported (Table I), 4 ruptured, 2 embolized and 3 were infected. Death was directly attributable to 1 of these complications in 5 patients 5,3*,33,34,42 (The coniplication rate of the entire 6i patients was 43 percent, causing death in 19 patients [31 percent].) Treatment: Tutassaura and Mustards5 demonstrated the feasibility of total resection of a nonpatent ductal aneurysm, and there has been sufficient experience with postoperative ductal aneurysm52g53 to encourage resection, Because of the high incidence of complications operative excision appears indicated. 425

FALCONE ET AL.

References

5. 6. 7.

8. 9.

10. 11. 12. 13. 14. 15. 16. 17. 18. 19. 20.

21. 22. 23. 24.

25.

Martin: Dilatation aneurysmal du canal atteriel. Bull Sot Anat Paris 22:17, 1827 Billard CM: Trait6 des Maladies des Enfans Nouveaux -N&s et I la Mamelle, Paris, JB Bailliere, 1828, p 567 Thore: De I’anevrisme du canal arteriel. Arch Gen Med (Paris) 23:30-39, 1850 [8 cases]+ Hammerschlag E: Ein Fall von wahrem Aneurysma des Ductus arteriosus Botalli. Virchow Arch Path Anat 258: l-8,1925 Cruickshank B, Marquis RM: Spontaneous aneurysm of the ductus arteriosus. Amer J Med 25:140-149, 1958 Parise: Dilatation fusiforme-anevrisme du canal arteriel. Bul,l Sot Anat Paris 12:95-96. 1837 Virchow R: Gesammette Abhandlungen zur Wissenchaftlchen Medicin, Frankfurt-a-Main, Meidinger Sohn, 1856, p 595 Renaut MJ: Deux cas d’anevrisme du canal atteriel. Bull Sot Anat Paris 45:238-240, 1870 [2 cases]* Roeder H: Die Ruptur des Ductus arteriosus Botalli: eine monographische Studie zur Pathologie des Ductus. Arch Kinderheilk 30:157-216, 1900 [l case]* Roeder H: Zwei FBlle von Ruptur des Ductus arteriosus BotaEli. Berl Klin Wschr 38:72-78, 1901 [l case]* Roeder il: Ein Fall eines solid thrombiesten Dilatationsaneurysma des Ductus arteriosus Botalli. Virchow Arch Path Anat 166:513-526; 1901 [l case]* Bednar: Quoted in Ref 7 Buhl: Quoted in Ref 49 [l case]* Gerard G: De I’aneurysme du cane1 arteriel. Etude anatomique. J Anat (Paris) 39:1-10, 1903 [2 cases]* Gruner: Quoted in R’ef 35 Weller CV: Cardiac anomalies. Two rare cardiac ma,lformations. Bull Int Ass Med Mus 5:122-125, 1915 [l case]* Schattman: Inaug-Dissertation, Breslau, 1919. Quoted in Ref 36 Dry DM: Congenital aneurysmal dilatation of ductus BotaEli. Amer J Dis Child 22:181-185, 1921 Kaufmann E: Lehbuch der spezielleh Pathologischen Anatomie fijr Studierende und Arzte. Erster Band. Berlin and Leipzig, Walter de Gruyter, 1922, p 71 Scheef S: Uber die Ruptur eines mykotischen Aneurysmas des Ductus Botalli und die rtintgenologische Darstellung des erweiterten Duktus beim Saugling. Arch Kinderheilk 117:234-243. 1939 Jager BV, Wollenmann d;J Jr: An anatomical study of the closure of the ductus atteriosus. Amer J Path 18: 595-613, 1942 [4 cases]* Gross RE: Arterial embolism and thrombosis in infancv. Amer J Dis Child 70:61-73. 1945 Pinninger JL: Aneutysm of the dbctus arteriosus. J Path Bact 61:458-460,1949 Kneidel JH: A case of aneurysm of the ductus arteriosus, with post mortem roentgenologic study after instlllation of barium paste. Amer J Roentgen 62:223-228, 1949 Fell EH, Davis CB: Surgical problems associated with treatment of patent ductus atteriosus. Arch Surg (Chi-

cago) 61:738-747, 1950 [l case]* 26. Lennox B, MacCarthy D: Aneurysm 27. 28.

29.

30.

1963 31. 32. 33. 34. 35.

36.

37.

38.

39.

40.

41.

42.

43.

44. 45. 46. 47. 48. 49.

50. of the ductus arteriin the new-born. Arch

osus and umbilical haemorrhage Dis Child 26:169-174, 1951 Birrell JHW: Three aneurysms of the ductus arteriosus in the new-born. Aust Ann Med 3:3743, 1954 [5 cases]* Lyon RA, Kaplan S: Patent ductus arteriosus in in-

51.

52. * Number of well documented cases of ductal aneurysm in this report. In the other cited references 1 case is described in each.

fancy. Pediatrics 13:3~57-362, 1954 [l case]* Cuccurullo L: Contributo allo studio delle dilatazioni aneurismatche del dotto di Botallo. Presentazione di un case e rassegna della litteratura. Riv Anat Pat Oncol 22: 422-438, 1962 Weisser E: Aneurysma des Ductus arteriosus Botalli und thrombotischer Verschlua der erkrankten Aorta abdominalis bei einem SBugling, eine anscheinend typische Kombination. Frankfurt Z Path 73:149-162,

53.

Payan H, Blaustein A: Aneurysm of the ductus atteriosus. Amer J Clin Path 44:449-452, 1965 Thorel C: Pathologie der Kreislauforgane. Ergebn Al&g Path 9:594, 1903 Hutchison R: A case of aneurysm of the ductus atteriosus. Brit J Child Dis 19:85-86, 1922 Guggenheim A: Aneurysma des Ductus arteriosus Botal’li mit Ruptur. Frankfurt Z Path 40:436-443, 1930 Tutassaura H, Goldman B, Moes CAF, et al: Spontaneous aneurysm of the ductus arteriosus in childhood. J Thorac Cardiovasc Surg 57:180-184.1969 Hebb RG: Aneurysm of ductus arteriosus and atheroma of pulmonary artery. Tr Path Sot London 44:45-47, 1893 Monckeburg JG: Die Missbildungen des Herzen. In, Handbuch der speziellen pathologischen Anatomie und Histolog,ie, Zweiter Band: Herz und Feflsse (Henke F, Lubarsch 0, ed). Berlin, Julius Springer, 1924, p 163165 Graham EA: Aneurysm of the ductus arteriosus, with consideration of its importance to the thoracic surgeon. Arch Surg 41:324-333, 1940 [2 cases] Mackler S, Graham EA: Aneurysm of the ductus Botalli as a surgical problem, with a review of the literature and report of an additional case diagnosed before operation. J Thorac Surg 12:719-727, 1943 Dvorak L, Schmittovia M: Aneurysma arteriove duceje. ylli$koanatomicka studie. Cas Lek Cesk 92:1171-1174,

Otto L:

TLjdliche Ruptur eines Aneurysmas des Ductus arteriosus Botalli mim Erwachsenenalter. Thoraxchirurgie 12:340-344, 1964 Bosman C, Leoncini B: On pathogenesis of a case of du’ctus atteriosus aneurysm. Acta Cardiol (Brux) 22:279288, 1967 Cheng TO: Aneurysm of a nonpatent ductus arteriosus. An unusual cause of mediastinal mass. Dis Chest 55: 497-500, 1969 Baron: Quoted in Ref 2 Westhoft: lnaug Dissertation, Gottingen, 1873. Quoted in Ref 18 Voss M: lnaug Dissertation, Kiel, 1900. Quoted in Ref 18 Buhl: Quoted in Ref 7 [2 cases] Schmorl: Quoted in Ref 7 Abbott ME: Congenital cardiac disease. In, Modern Medicine, third edition, vol 4, (Osler W, McCrae T, ed). London, Oxford University Press, 1927, p 770-772 Wilcox BR, Roberts WC, Carney EK: The effect of reduced atmospheric oxygen concentration on closure of the ductus arteriosus lin the dog. J Surg Res 2:312-316, 1962 Barclay AE, Barcroft J, BBrron DH, et al: X-ray studies of closing of ductus arteriosus. Brit J Radio1 11:5170585, 1938 Das JB, Chesterman, JT: Aneurysms of the patent ductus arteriosus. Thorax 11:295-302, 1956 Ross RS, Feder FP, Spencer FC: Aneurysms of the previously ligated patent ductus atteriosus. Circulation 23: 350-3,57, 1961

The American

Journal

of CARDIOLOGY