Coarctation of the aorta and cerebrovascular accident: A proposal for early corrective surgery

1004

December, 1970 T h e Journal of P E D I A T R I C S

Coarctation of the aorta and cerebrovascular accident: A proposal for early corrective surgery A 9-year-old boy was hospitalized because o[ an acute onset of right hemiplegia, right homonymous hemianopsia, and aphasia. Diagnostic studies revealed coarctation of the aorta distal to the origin of the left subclavian artery and a cerebrovascular accident involving the left cerebral hemisphere. Repair of isolated coarctation of the aorta at an early age, i.e., 8 years or younger, might possibly prevent eerebrovascular complications and relieve the continued stress upon the left ventricular muscle.

William T. Shearer, Ph.D., Joel Y. Rutman, M.D.,* Warren A. Welnberg, M.D., and David Goldring, M.D. ** ST.

LOUIS, MO.

T H ~ P ~ J R P O S E S of this c o m m u n i c a t i o n are to r e p o r t a case of isolated postductal c o a r c t a t i o n of the a o r t a with a cerebrovascular complication, to review the literature on coarctation of the a o r t a c o m p l i c a t e d by cerebrovascular disease, a n d to propose t h a t surgical correction of the c o a r c t a t i o n should preferably be done at the age of 8 years or younger.

CASE REPORT A 9-year-old Negro boy (M. H.) had been in apparent good health until he suddenly colFrom the Edward Mallinckrodt Department of Pediatrics, Washington University School of Medicine, and the Divisions of Pediatric Cardiology and Neurology, St. Louis Children's Hospital. Aided in part by the Arthur Fund, St. Louis Children's Hospital Heart Mothers' Fund, Scott Gentsch Memorial Fund, William T. Beauchamp Memorial Fund, John Clay Seier Fund, and the Allen P. and Josephine B. Green Foundation. *Clinical fellow in neurology. ~*Address: 500 ,7. Kingshighway Blvd., St. Louis, Mo. 63110.

Vol. 77, No. 6, pp. 1004-1009

lapsed, was unable to move his right side, and became aphasic. At a local hospital a traumatic lumbar puncture was performed, producing "slightly" xanthochromic bloody fluid with 85,000 red blood cells per cubic millimeter. A left carot-. id arteriogram was done and interpreted as normal. Upon transfer to the St. Louis Children's Hospital he could make crying noises, was lethargic but rousable, and tended to gaze to the left. He did not respond to verbal commands but would occasionally respond to gestures. Blood pressure was 200/120 mm. Hg in his right arm and 180/118 mm. Hg in the left arm; no blood pressure could be obtained by auscultation or palpation in the lower extremities. A grade I I / V I systolic ejection murmur was heard along the left sternal border and was transmitted to the carotid vessels; a thrill was palpated in the supraclavicular region. The neck was supple. The fundi showed diminished pulsations but normal optic disc margins; there was pronounced myopia bilaterally. A right homonymous hemianopsia was present; the left pupil was slightly smaller than the right, but both reacted normally to light. Full extraocular move-

Volume 77 Number 6

ments were present with the oculocephaiic reflex. Central right facial paresis and a decreased right corneal reflex were noted; the tongue deviated to the right on protrusion. Response to pain was decreased On the right side. Flaccid paresis of the right arm and leg was evident. Reflexes in both legs were hyperactive, with bilateral extensor plantar responses. He remained afebrile throughout his hospital stay. Brain scan was interpreted as normal. An electroencephalogram showed persistent slow waves with no asymmetry. Urinalyses were free of red blood cells. Sickle cell trait was detected; the hemoglobin concentration was 13 Gin. per 100 ml. of blood. Lumbar puncture revealed an opening pressure of 240 mm. of water. The fluid was initially blood tinged but gradually cleared. There were 3,100 red blood cells per cubic millimeter in the first tube and 710 in the third tube; supernatant fluid was colorless, total protein concentration was 39 mg. per 100 ml., and that of glucose was 75 mg. per 100 ml. Chest roentgenograms showed cardiomegaly with left ventricular prominence. An electrocardiogram showed evidence of left ventricular hypertrophy. On the second hospital day angiocardiography and cardiac catheterization were performed and revealed severe coarctation of the aorta just distal to the origin of the left subclavian artery. The pressure in the ascending aorta was 180/100 mm. Hg, and distal to the coarctation the pressure was 80/66 mm. Hg. The iutracardiac oxygen saturations were all within normal limits. Left carotid arteriogram revealed an indistinct filling defect distal to the trifurcation of t h e left middle cerebral artery, suggesting a resolving erabolus or possible mycotic aneurysm. There was a 5 mm. midline shift of the internal cerebral vein from left to right. Narrowing of the supraclinoid portion of the left internal carotid artery was noted and was consistent with arterial spasm. Right carotid and right axillary arteriograms revealed no additional abnormalities. Blood pressure was difficult to control despite administration of hydralazine. The patient had no significant change in the neurologic findings. Resection of the coarctation was carried out uneventfully 2 weeks after admittance. The diameter of the aorta at the site of the coarctation was 3 mm. The resected aortic segment showed no evidence of inflammation or source for emboli. Immediately postoperatively, systemic blood pressure was 150/110 mm. Hg in both arms;

Coarctation o[ aorta

1 005

femoral pulses were full. Seven weeks postoperatively, he was alert, in good spirits, and walked unaided. His blood pressure in the upper extremities was 100/60 mm. I-Ig and in the lower limbs it was 120/80 mm. Hg. Eight months postoperatively, he stiI1 had a dense right homonymous hemianopsia, severe spastic right hemiparesis, and a severe residual aphasia. The clinical course and neuroradiographic findings were consistent with an acute cerebrovascular accident involving the left middle cerebral artery, type indeterminate, associated with significant structural damage to the left cerebral hemisphere. DISCUSSION T h e neurologic accident which this patient suffered is a rare b u t i m p o r t a n t complication of coarctation of the aorta. T a b l e I is a survey of the literature T M of patients u n d e r 21 years of age who h a d coarctation of the aorta and cerebrovascular disease. (Because of insufficient details in the reports, assessment of the n u m b e r of cases of isolated coarctation was not possible.) T h e acute onset of neurologic signs a n d the often fatal outcome are apparent. W h e n etiology was determined, the cerebrovascular disease was most often associated with r u p t u r e of intracerebral aneurysm(s) a n d rarely caused by thrombosis or embolization of the intracerebral vessels. I n 27 instances i n t r a c r a n i a l disease was demonstrated by arteriogram, surgery, or autopsy. I n 8 cases i n t r a c r a n l a l vascular disease was suggested by the clinical findings but was n o t demonstrated. T h e prevalence of i n t r a c r a n i a l aneurysms associated with coarctation of the aorta varies from 2.5 to 50 per cent of patients studi e d l , 4, ~, 15, 18, 19 whereas the prevalence in the general population varies from 0.65 to 2 per cent. 2~ 21 Most pediatric cardiologists agree u p o n the indications for surgical repair of isolated coarctation of the aorta. If a p a t i e n t has significant hypertension in the u p p e r extremities a n d a pressure gradient of at least 50 ram. H g between the u p p e r a n d lower extremities or p r o n o u n c e d demonstrable collateral circulation, surgical repair is indicated. However, there is difference of

10 0 6

Shearer et al.

The Journal of Pediatrics December 1970

T a b l e I. P a t i e n t s u n d e r 21 y e a r s of age w i t h c o a r c t a t i o n of t h e a o r t a a n d c e r e b r o v a s c u l a r disease Age (yr.) and sex

Re[erence Symptoms and findings A. Intracranial disease and coarctation o[ the aorta demonstrated by roentgenography, surgery, or autopsy 1 7 Cardiac symptoms and anginal pain for 3 years; admitted with signs of 1 M cerebral hemorrhage; autopsy revealed recent hemorrhage left occipital lobe No.

11 M

Fell, developed left hemiplegia; died 3 days later; autopsy showed large recent hemorrhage in temporal lobe

1

13 M

Suddenly fell dead while playing football; autopsy showed small ruptured aneurysm at junction right sylvian artery

1

17 M

Frequent complaints of constriction of chest; unconscious 3 times before death; autopsy showed bean-sized aneurysms of both anterior cerebral arteries; right had ruptured and there was extravasated blood around left

1

17 F

Fainted, vomited, speech less distinct; 3 weeks later died after second "fainting fit"; autopsy showed recent hemorrhage anterior end of each hemisphere

1

17 M

Fell down after hard work, developed opisthotonus, then coma; bloody cerebrospinal fluid; autopsy revealed recent hemorrhage at base of brain

1

7

19 F

Left hemiplegia; death 6 weeks later; autopsy showed recent hemorrhage into right corpus striatum

1

8

19 M

Left hemiplegia; 7 months later "cardiac death"; autopsy showed thrombosis of left internal carotid and posterior communicating arteries

1

9

20 M

Left hemiplegia and coma; died same day; autopsy showed small recent right subcortical hemorrhage anterior to Rolandic fissure

1

I0

20 M

Fell, meningeal signs, right hemiparesis; died 5 weeks later; ruptured aneurysm left posterior communicating artery

1

11

13 M

Four-day history of headache and vomiting; nuchal rigidity and bloody cerebrospinal fluid; bIood pressure 230/140; died i7 days after admittance; autopsy revealed Iarge blood clot at base of brain, ruptured left anterior cerebral artery

2

12

17 M

Five-day history of vomiting and headache; nuchal rigidity; hemorrhagic cerebrospinal fluid, blood pressure 220/112 (arms); no femoral pulse felt; died 13 months later after resuming normal activity; autopsy revealed death due to dissecting aneurysm of aorta and aneurysm left middle cerebral artery with questionable rupture

3

?

Three patients between ages of 10 and 20 yr. who died of intracranial lesions

4

19 F

Collapsed and died; autopsy revealed ruptured aneurysm at origin !eft anterior cerebral artery; coarctation of aorta undiagnosed during life

5

History of congestive heart failure and hemiplegia; died prior to corrective surgery; autopsy revealed old infarct in left parietal lobe with a recanalized thrombus of the left middle cerebral artery

6

13-15 16 17

2~ ?

18

13 M

Coarctation resected; 3 hours later right hemiplegia; died 24 hours postoperatively; autopsy showed thrombosis in suture line of aorta; emboli left carotid and middle cerebral arteries

6, 15

19

I4 M

Two-day history of headache and vomiting; blood pressure 150/90; nuchal rigidity and bloody cerebrospinal fluid; died 15 days after admittance with florid papilledema; autopsy revealed hemorrhage from aneurysm of right middle cerebral artery, coarctation of aorta

7

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C o a r c t a t i o n o[ a o r t a

1 O0 7

T a b l e I. C o n t ' d No.

Age and sex

Symptoms and findings

Reference

20

7 M

Coarctation noted at age 5 years; blood pressure, 140/90 (arms); coarctation resected age 7 yr.; 1 week later became unconscious; cerebrospinal fluid bloody; died 4 days later; autopsy revealed extensive hemorrhage into left cerebral hemisphere of unknown origin

8

21

14 M

Coaretation diagnosed at age 6 years; age 14 years, confused, headache, drowsy, meningeal signs; bloody cerebrospinal fluid; carotid arteriography disclosed aneurysm at origin left middle cerebral artery; coaretation corrected and patient transiently improved; on thirtieth hospital day had further subarachnoid bleeding and died; no autopsy

9, 10

22

10 ?

Arteriographic demonstration of aneurysm left internal carotid at origin of posterior communicating artery; underwent ligation in neck followed by transient hemiparesis; investigation of subsequent headaches led to discovery of coarctation which was repaired

11

23

11 ?

Decerebrate rigidity with bloody cerebrospinal fluid; aneurysm of hifurcation of basilar artery by angiogram; no operation; died 2 days after bleeding episode

11

24

13 M

Aneurysm of anterior communicating artery ligated; alive and well 6 years later (coarctation repaired?)

11

25

19 M

Headache, bloody cerebrospinal fluid; angiographic demonstration of aneurysm at bifurcation of internal carotid artery and second aneurysm at origin of left anterior cerebral artery; surgery not performed; subarachnoid bleeding occurred and patient died 2 weeks after initial presentation; no autopsy

12

26

4 M

Sudden left hemiplegia; blood pressure 145/100 (arm); pneumoencephalogram revealed expanding lesion right island of Reil; coarctation repaired; 8 years later had residual left spastic hemiparesis

13

27

15 M

Suddenly unconscious; blood pressure 150/100 (arm); bloody cerebrospinal fluid; left pericallosal artery aneurysm clipped and coarctatiun repaired; 1 year later asymptomatic

13

B. Cases of coarctation of aorta in which intracranial vascular disease was suspected clinically but not demonstrated by any other means

28

14 M

Sudden onset right hemiplegia; coma 8 hours later; absent central pulses; brain not examined

1

29

ll M

Headaches, suddenly became aphasic with right-sided hemiplegia; eerebrospinal fluid clear; bilateral carotid angiography normal; eoarctation surgically corrected; 3 years later walked freely but rarely used right hand; spontaneous speech limited to a few words

8

30

12 F

Two months prior to admittance became faint, vomited, febrile; 1 month history of dragging left leg and weakness left arm; blood pressure 210/170 (arm), 0/0 (legs); retinal hemorrhage noted but no signs of increased intracranial pressure; 1 ~ years later still could not use left arm and leg well

14

, 31

5 Iv[

Pneumonia age 9 months; large heart noted and coarctation diagnosed; noted to have left hemiplegia during an episode of cardiac decompensation associated with pneumonia; hemiplegia still present age 5 years

15

32

I1 M

Headaches, coarctation repaired; 1 month later became ill, vertiginous, and vomited; had minimal residual cerebellar signs 2 years later

15

33

8 M

Child died of ruptured cerebral aneurysm (not documented) 3 years following uneventful repair of coarctation

16

34

9 ?

Subarachnoid hemorrhage prior to repair of coarctation (ruptured intracranial aneurysm ?)

17

35

7 ?

Subarachnoid hemorrhage 7 years after repair of eoarctation

17

1008

Shearer et al.

opinion regarding the optimum age for repair of isolated coarctation of the aorta. McNamara and Rosenberg 22 advise surgicaI correction at a "timely age." Others23, 24 recommend surgical correction between 8 and 15 years of age or from 7 to 20 years of ageY 5 In contrast to the above authors, Keith and associates 2a state that if a child with coarctation of the aorta is free from complicating defects, he may be operated on at 7 or 8 years of age. Shumacker and associates 27 report that in their experience children who had their coarctations repaired while they were somewhat under 8 years of age did as well as those children who were repaired between 8 and 12 years of age. Tawes and associates 1~ perform elective surgery for coarctation of the aorta preferably at 4 to 7 years of age because of the elasticity of the aorta, the technical ease of the operation at this age, and the avoidance of complications, such as the rupture of an intracranial aneurysm. We suggest that the preferable age for repair is 8 years of age or younger. This would not only reduce the continued stress upon the left ventricle but might also reduce or prevent the threat of the neurologic complications. There is no evidence that surgical correction of the coarctation would prevent the development of cerebral aneurysms, but cerebrovascular accidents (thrombosis, embolus, or rupture of aneurysms) may possibly be prevented. Other indications for early surgery include congestive heart failure, sudden cardiomegaly, or significant sustained rise in the degree of hypertension of 20 ram. Hg or higher. A possible objection to repair of the coarctation at 8 years of age is that the aorta has not achieved adult proportions and that significant restenosis might develop by adulthood because the anastomotic site may not grow with the patient. This argument is not valid. Moss and associates 2s have shown that the aorta achieves 72 per cent of its adult diameter by the age of 8 years. A more pertinent comparison of the aorta of the child with that of the adult would be the cross-sectional area or the lumen of the

The Journal o[ Pediatrics December 1970

vessel. At 8 years of age the aortic crosssectional area is 52 per cent of that of the adult aorta. Gupta and Wiggers 29 have shown in experimental animals that the cross-sectional area of the aortic lumen has to be reduced by more than 50 per cent before the arterial pressures are affected in the upper and lower extremities. Clatworthy and associates 3~ found in dogs that collateral circulation did not develop until a coarctation greater than 75 per cent of the crosssectional area was produced. Also, Keith and associates 26 believe that the anastomotic site grows with the child. Moss and associates 2s reported upon 5 children with coarctation of the aorta operated upon at less than 2 years of age. At 2 to 4 years follow-up, 4 of 5 children had detectable constriction at the anastomotic site; 2 of these children had a constriction of 55 per cent of the diameter at the site of repair, but the brachial and femoral blood pressures were within normal limits. The rate of restenosis in patients operated on when over one year of age was less than 1 per cent in Tawes and associates '17 series. Finally, a follow-up study (1 to 15 years) 31 of 20 children with coarctation of the aorta from our hospital who were operated upon as infants less than a year of age showed that 8 patients had no significant difference in blood pressure between the arms and legs and had normal pressures in their arms at the time of the follow-up examination. Another 8 patients had moderate blood pressure differences between the upper and lower extremities. Further follow-up of this group will be needed to determine how many will need reoperation. Four patients, although asymptomatic, had recurrent severe coarctation and will require reoperation. Surgical repair of isolated coarctation of the aorta at the age o f 8 years, therefore, seems reasonable. The operation may be done at a younger age under the special circumstances mentioned previously. The age of 8 is suggested because the probability of developing recoarctation is remote, the continued stress upon the heart will be relieved, and the threat of catastrophic cerebrovascular accidents may be reduced,

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We wish to express our thanks to Dr. Homer E. Nash for permission to use the hospital data on his patient in this study. We also wish to thank Dr. Philip R. Dodge for his critical review of the manuscript and Dr. David O. Davis for his review and interpretation of the angiographic studies. REFERENCES 1. Abbott, M. E.: Coarctation of the aorta of the adult type. II. A statistical study and historical retrospect of 200 recorded cases with autopsy, of stenosis or obliteration of descending arch in subjects above age of two years, Amer. Heart J. 3: 392, 1928. 2. O'Reilly, J. N., and Chapman, O. W.: Coarctation of aorta with cerebral aneurysm, Arch. Dis. Child. 18: 109, 1943. 3. Davies, J. N. P., and Fischer, J. A.: Coarctation of the aorta, double mitral AV orifice and leaking cerebral aneurysm, Brit. Heart J. 5: 197, 1943. 4. Relfenstein, G. H., Levine, S. A., and Gross, R. E.: Coarctation of the aorta: A review of 104 autopsied cases of "adult type," 2 years of age or older, Amer. Heart J. 33: 146, 1947. 5. Wright, C. J. E.: Coarctation of the aorta with death from rupture of a cerebral aneurysm, Arch. Path. 48: 382, 1949. 6. Berthrong, M., and Sabiston, D. C.: Cerebral lesions in congenital heart disease. A review of autopsies of 162 cases, Bull. Hopkins Hosp. 89: 384, 1951. 7. Ostrum, H. W., Gyorgy, P., and Brody, J. I.: Clinical pathologic conference, Philadelphia General Hospital, Amer. J. Med. Sci. 229: 413, 1955. 8. Landtman, B., and Tuuteri, L.: Vascular complications in coarctation of the aorta, Acta Paediat. 48: 329, 1959. 9. Hoads, H. L., Stelnfeld, L., and BlumenthaI, S.: Congenital cerebral aneurysms and coarctation of the aorta, Arch. Pediat. 76: 28, 1959. 10. Schwartz, M. J., and Baronofsky, I. D.: Ruptured intracranial aneurysm associated with coarctation of the aorta, Amer. J. Cardiol. 6: 982, 1960. i1. Matson, D. D.: Intracranial aneurysms in children, J. Neurosurg. 23: 578, 1965. 12. Robinson, R. G.: Coarctation of the aorta and cerebral aneurysm, J. Neurosurg. 26: 527, 1967. 13. LeBlanc, F. E., Charrette, E. P., Dobell, A. R. C., and Branch, C. L.: Neurological complication or aortic coarctation, Canad. Med. Ass. J. 99: 299, 1968. 14. Lichtenburg, H. H., and Gallagher~ H. F.: Coarctation of the aorta--anomaly of great vessels of neck and intermittent leakage of cerebral aneurysm during life, Amer. J. Dis. Child. 45: 1253, 1933. 15. Tyler, H. R., and Clark, D. B.: Neurologic

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31.

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complications in patients with coarctatlon of aorta, Neurology 8: 712, 1958. Haxton, H.: The late results of excision of aortic coarctation, J. Cardiovasc. Surg. 6: 346, 1965. Tawes, R. L., Aberdeen, E., Waterston, D. J., and Carter, R. E. B.: Coarctation of the aorta in infants and children. A review of 333 operative cases, including 179 infants, Circulation 39: 173, 1969. (Suppl.) Edwards, J. E., Carey, L. S., Neufeld, H. N., and Lester, R. G.: Congenital heart disease, vol. II, Philadelphia, 1965, W. B. Saunders Company, p. 692. Stehbens, W. E.: Cerebral aneurysms and congenital abnormalities, Aust. Ann. Med. 11: 102, 1962. Walton, J. N.: Subarachnoid hemorrhage, Edinburgh, 1956, E. & S. Livingstone, Ltd., p. 217. Merritt, H. H.: Textbook of neurology, ed. 4, Philadelphia, 1967, Lea & Febiger, Publishers, p. 205. McNamara, D., and Rosenberg, H. S.: Coarctation of the aorta, in Watson, H., editor: Paedlatric cardiology, London, 1968, LloydLuke, Ltd. (St. Louis, 1968, The C. V. Mosby Company), chap. 11. Schuster, S. R., and Gross, R. E.: Surgery for coarctation of the aorta: A review of 500 cases, J. Thorac. Cardiovasc. Surg. 43: 54, 1962. Nelson, W. E., Vaughan, V. C., III, and McKay, R. J.: Textbook of pediatrics, ed. 9, Philadelphia, 1969, W. B. Saunders Company, p. 1004. Nadas, A. S.: Pediatric cardiology, ed. 2, Philadelphia, 1963, W. B. Saunders Company, p. 530. Keith, J. D., Rowe, R. D., and Vlad, P.: Heart disease in infancy and childhood, ed. 2, New York, 1967, The Macmillan Company, chap. 11, p. 226. Shumacker, H. B., Nahrwold, D. L., King, H., and Waldhausen, J. A.: Coarctation of the aorta, Curt. Probl. Surg. Feb., 1968. Moss, A. J., Adams, F. H., O'Loughlin, B. J., and Dixon, W. J.: The growth of the normal aorta and of the anastomotic site in infants following surgical resection of coaretatlon of the aorta, Circulation 19: 338, 1959. Gupta, T. C., and Wiggers, C. J.: Basic hemodynamic changes produced by aortic coarctation of different degrees, Circulation 3: 17, 1951. Clatworthy, H. W., Jr., Sako, Y., Chisholm, T. C., Culmer, C., and Varco, R. L.: Thoracic aortic eoarctation: Its experimental production in dogs with special reference to technical methods capable of inducing significant intraluminal stenosis, Surgery 28: 245, 1950. Hartmann, A. F., Jr., Goldring, D., Hernandez, A., Behrer, M. R., Schad, N., Ferguson, T., and Burford, T.: Recurrent coarctation of the aorta after successful repair in infancy, Amer. J. Cardiol, 6: 405, 1970.