Successful Aortocoronary Bypass in Osteogenesis Imperfecta

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CASE REPORTS

JOHN MURRAH PASSMORE, MD, WILLIAM EASTON WALKER, MD, PHD , FRANCISCO FUENTES, MD, FACC Houston, Texas

Cardiovascular abnormalities are infrequently docu anastomoses. Although successfulaortocoronary bypass mented in osteogenesis imperfecta, one of a group of surgery had not been previouslyreported in osteogenesis hereditary, generalized connective tissue disorders. A imperfecta, this patient received such surgery with ther patient with osteogenesis imperfecta is described with apeutic benefit. Therefore, coronary artery vascularl mitral valve prolapse, significantcoronary artery disease zation should be considered as a safe and effective treat and a coronary artery aneurysm. The latter two cardiac ment modality for patients with osteogenesis imperfecta defects are apparently rare in this disease. The option and coexistingcoronary atherosclerosis. of surgery was carefully considered with regard to tech (J Am Coll CardioI1987,'9:960-3) nical feasibility and potential deterioration of the graft

Osteogenesis imperfecta is one of the group of hereditary , disorders, including pseudoxanthoma elasticum (4) and Eh generalized disorders of connective tissue including Mar lers-Danlos syndrome (5). fan's syndrome, pseudoxanthoma elasticum, Ehlers-Danlos We present a patient with osteogenesis imperfecta who syndrome and Hurler's syndrome. Significant cardiovas had significant multivessel disease that was treated with cular abnormalities are a recognized feature of the latter four coronary artery bypass grafting. The results indicate that conditions (I ). Although the primary clinical manifestations such surgery is worthy of consideration and feasible in pa of osteogenesis imperfecta include skeletal, ocular, cuta tients with this connective tissue disorder. neous, otologic and dental abnormalities, serious defects of the cardiovascular system have also been recognized oc casionally. There are well documented cases of aortic and Case Report mitral valve regurgitation, and recently successful prosthetic Initial presentation. A 40 year old white man with aortic and mitral valve replacement has been reported in known type I osteogenesis imperfecta was referred to the several patients with osteogenesis imperfecta (2,3). How University cardiology clinic for the evaluation of chest dis ever , documentation of coronary artery disease has been comfort of recent onset. He had a past history of recurrent sparse . One patient who received a mitral valve prosthesis fractures including multiple rib fractures, long bone frac had significant stenosis in the proximal left anterior de tures and a hip fracture. One week before his clinic visit he scending coronary artery demonstrated by coronary angi had noted the onset of dull substernal pressure associated ography, but attempts at aortocoronary bypass grafting were with shortness of breath and light-headedness while walking unsucces sful (2). To our knowledge there are no previous the several blocks home from work. He described the feeling reports of coronary artery bypass surgery accomplished in in his chest as a sudden tightness that radiated down his patients with osteogenesis imperfect a. In contrast, success right arm; it was relieved after several minutes of rest but ful vascular bypass surgery has been reported in patients recurred with increasing frequenc y during the subsequent with coronary disease and other heritable connective tissue week with only minimal exertion. Sublingual nitroglyce rin usually alleviated the chest tightness in less than a minute . Positive risk factors for coronary artery disease included From the Divisions of Cardiology and Thoracic and Cardiovascular a family history of a maternal aunt dying at age 60 with a Surgery. the University of Texas Medical School at Houston , Houston . Texas. " heart attack ," hypercholesterolemia with a level of 34 1 Manuscript received November I I. 1985; revised manuscript received mg/dl and a 20 pack/year smoking history . The patient led June 2, 1986, accepted July 7, 1986. a fairly active life despite his propensity to fractures. Addre ss for reprints: Franc isco Fuentes, MD , Associate Professor of Medicine. Division of Cardiology. University of Texas Medical School. Clinical findings and noninvasive cardiac procedures. P.O. Box 20708, Houston, Texas 77225·0708. The patient was a short, white man with multiple long bone

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Figure I. Photograph of patient with osteogenesis irnperfccta demonstrating body habitus. The patient has distinctive hlue sclerae .

Figure 2. (A) Rest electrocardiogram of patient and (B) stress deformities. kyphosis. a triangular-shaped head and blue electrocardiogramat 6 minutes of the Bruce protocol. demonstrat sclerae (Fig. I). The blood pressure in the right arm was ingsignifi cant ST depression intheinferior andanterolateral leads. 134/80 mm Hg supine and 132/82 mm Hg standing. The heart rate was 90 beats/min and the respiratory rate :20/min. Cardiac examination revealed a hyperactive point of max 3 to 4 mm of ST segment depression in the inferior and imal impulse with a left ventricular impulse displaced 2 em anterolateral leads along with :2 mm of ST elevation in leads lateral to the midclavicular line in the fifth intercostal space. aVR and aVL which persisted for 5 minutes into recovery. A sternal impulse was present. There was a regular rhythm In addition. the blood pressure decreased from 134/82 mm with a normal first heart sound. physiologically split second Hg at rest to 104/68 mm Hg at peak exercise. suggesting sound and an audible fourth sound. A systolic click was exercised-induced left ventricular dysfunction. Thallium heard along with a grade 2/6 holosystolic high-pitched mur perfusion imaging with stress was abnormal with a large mur at the left lower sternal border without radiation. The anterior and septal perfusion defect that filled in at redis lungs were clear to auscultation and percussion. Physical tribution. These findings strongly suggested significant mul examination otherwise was normal. tivessel coronary artery disease. M-mode and two-dimen The chest radiograph showed a normal cardiovascular sional echocardiography was normal except for moderate silhouette. a calcified granuloma in the right lung and a systolic posterior displacement of the anterior and posterior healed left scapular fracture along with numerous healed rib mitral leatlets. fractures. The rest electrocardiogram was within normal Cardiac catheterization and angiographic findings. limits. During a thallium stress test (Fig. 2) the patient The right- and left-sided heart pressures were normal. Coro exercised for 6 minutes on the Bruce protocol to a maximal nary angiography demonstrated a 95% tubular stenosis of heart rate of 152 beats/min (X4% of predicted maximal heart the proximal left anterior descending coronary artery in rate). At peak exercise. the electrocardiogram demonstrated volving the origin of the first diagonal artery (Fig. 3). There 962 PASSMOREET AL. lACC Vol. 9. No.4 CORONARY BYPASS IN OSTEOGENESIS IMPERFECTA April 1987:960-3

Cardiac surgery and postoperative course. After care ful consideration, surgical therapy was recommended over percutaneous transluminal coronary angioplasty because of the theoretical risk of coronary artery dissection during bal loon dilation ofcoronary vessels in a patient with an inherent connective tissue defect. Subsequently, the patient received saphenous vein aortocoronary bypass grafts to the left an terior descending, obtuse marginal and right coronary ar teries. He tolerated the procedure well, and the immediate postoperative course was uneventful. Several months after discharge, the patient complained of vague neurologic symptoms including some recent loss of memory. A comprehensive neurologic evaluation was essentially negative, and soon thereafter this syndrome re solved. The patient has had extensive follow-up by a car diologist for 15 months after surgery, and he is currently free of cardiac symptoms on minimal antianginal medica tion. A repeat stress test performed I year after surgery demonstrated insignificant ST changes, normal hemody namic response and no symptoms with essentially the same A rate-pressure product achieved as before.

Discussion Although distinct cardiovascular abnormalities have been noted in all of the hereditary, generalized connective tissue disorders, the incidence of cardiac defects appears to be least common in osteogenesis imperfecta. Valvular heart disease has occasionally been documented by cardiac cath eterization in such patients. Despite the anticipated diffi culties with tissue friability, bleeding diathesis and poor wound healing, successful and uncomplicated prosthetic aortic and mitral valve replacement has been demonstrated in pa tients with osteogenesis imperfecta (2,3). In each of these patients, the authors described careful placement and but tressing of sutures to insure maximal support. Coronary artery disease. The incidence of coronary artery disease in osteogenesis imperfecta is seemingly rarer than the incidence of valvular heart disease. This may relate to a true infrequency of associated coronary atherosclerosis, to decreased expression or perception of ischemia due to limited exercise tolerance in patients with osteogenesis im perfecta because of orthopedic disability, or both. (Our pa Figure 3. Coronary angiograms demonstrating (A) 95% stenosis tient was quite active for someone with such a disabling of the proximal left anterior descending artery and 70% stenosis of the proximal circumflex artery and (B) aneurysmal dilation of disease.) Stein and Kloster (2) described a patient with os the proximal right coronary artery. teogenesis imperfecta with occlusion of the left anterior descending coronary artery who had successful mitral valve replacement, but aortocoronary bypass grafting was not ac complished because of anatomic considerations. was a 70% stenosis of the proximal circumflex artery before Our case is unique in that the patient had coronary artery the origin of the first obtuse marginal artery. Aneurysmal atherosclerosis, a coronary artery aneurysm and mitral valve dilation of the proximal right coronary artery was noted. prolapse. This constellation of cardiac findings has not been No regional wall abnormalities were observed on ventric previously reported in osteogenesis imperfecta. With mitral ulography, and the ejection fraction was 0.87. valve prolapse shown by physical examination and echo- lACC Vol. 9, No.4 PASSMORE ET AL. 963 April 1987:960- 3 CORONARY BYPASS IN OSTEOGENESIS IMPERFECTA

cardiography, one might easily have attributed the patient 's the cardiovascular surgical team . The chest wound healed symptoms to this disorder. Howe ver, because of the nature without difficult y. of the chest pain and his moderate coronary risk profile, the Conclusion. A patient with osteogenesis imperfecta who patient was advised to have cardiac catheterization with has mitral valve prolap se, significant coronary artery ath coronary angiography. After demonstration of critical coro erosclerosis and a coronary artery aneurysm has been de nary stenoses, consideration was given to utilizing percu scribed. The latter two cardiovascular abnormalities are ap taneou s transluminal coronary angioplasty. This idea was parently rare in this connective tissue disorder. A successful abandoned, howe ver, because of the fear that dilation of three vessel aortocoronary bypass procedure was performed, vessels with a collagen defect might result in coronary artery demon strating that such surgery represents a technically fea dissection or rupture. sible therapeutic option when coronary disease coexists with Coronary artery aneurysm. This was an unexpected osteogenesis imperfecta. finding . Ascending aortic aneur ysm commonly occurs in Marfan's syndrome (6). Aneurysms of the sinus of Valsalva We thank Mary Cholakian for her assistance in the preparation of this have been reported in patients with Marfan's syndrome (7,8) manuscript. and Ehlers-Danlos syndrome (9). An aneurysm of the in ternal carotid artery has been described in pseudoxanthoma elasticum (10). The recent Coronary Artery Surgery Study References (CASS ) (II) identified aneury smal coronary disease in 4.9% I. McKusick VA. Heritable Disorders of Connective Tissue. 2nd ed. St. of the total registry population, In this study, coronary risk Louis: CV Mosby. 1960;42-279. factor s did not differ significantly between the groups with 2. Stein D, Kloster FE. Valvular heart disease in osteogenesis imperfecta. and without aneurysm, sugge sting that aneurysmal coronary Am Heart J 1977:94:637-41. disease is usually a variant of occlusive coronary athero 3. Weisinger B, Glas sman E, Spencer FC, Berger A. Successful aortic valve replacement for aortic regurgitation associated with osteo genesis sclerosis. Presumably the coronary artery aneurysm in our irnperfecta. Br Heart J 1975;37:475-7. patient with osteogenesis imperfecta was a manifestation of 4. Bete J, Banas J. Moran J, Pinn V, Levine HJ. Coronary artery disease the inherent connective tissue disorder, although its coin in an 18-year-old girl with pseudoxanthoma elasticum: succes sful sur cidental presence cannot be excluded. gical therapy. Am J Cardiol 1975;36:515- 20. Mitral valve prolapse. Th is condition has previousl y 5. Redington A, McCue J, Lennox S. Coronary artery vein grafling in a case of Ehlers-Danlos syndrome. Br Heart J 1984;52:237-42. been infrequently reported in osteogenesis imperfecta. White 6 . Crawford ES. Marfan ' s syndrome. Broad spectral surgical treatment et al. (12) studied 20 patients with osteogenesis imperfecta of cardiovascular manife stations. Ann Surg 1983;198 :487-505. by cardiac ultrasound and found only one with echocardio 7. Tuna N. Tha i AP . Some unusual features of the Marfan syndrome . graphic evidence of mitral valve prolap se. This incidence Circulation 196\;24 :1154- 63. is probably consistent with the frequency of prolapse of the 8. Papaioannou AC, Agustsson MH . Gazul BM. Early manifestations mitral valve (5 to 18%) in the general population (13,14). of the cardiovascular disorders in Marian's syndrome. Pediatrics 1961;27: 255- 68. A mitral valve click, late systolic murmur and documen 9. Tucker DH, Miller DE. Jacoby WJ. Ehlers-Danlos syndrome with a tation of mitral valve prolapse by echocardiography are rec sinus of Valsalva aneurysm and aortic insufficiency simulating rheu ognized features of Marfan's syndrome, Ehlers-Danlos syn matic heart disease. Am J Med 1963:35:715-20. drome and pseudoxanthoma elasticum (15) . 10. Dixon JM . Angioid streak s with pseudoxanthom a elasticum. Am J Surgery. In this patient, end to side anastomoses of the Ophthalmol 1951;34: 1322-3. saphenou s vein to the distal coron ary vessels were sutured II . Swayc PS. Fisher LD, Litwin P, et al. Aneurysmal coronary artery disease. Circulation 1983;67:134-8. without difficult y. There was moderate bleeding during the 12. White NJ. Winearls CG. Smith R. Cardiovascular abnormalities in procedure, but adequate hemostatis was obtained and only osteogenesis imperfe cta. Am Heart J 1983;106 :1416-20. two units of blood were transfu sed intraoperatively. Wound 13. Procacci PM, Savran SV , Schreiter SL. Bryson AL. Prevalence of closure was easily accomplished , and the patient's post clinical mitral valve prolapse in 1169 young women. N Engl J Med operative recovery was benign . During the follow-up period 1976;294: 1086- 8. of 15 months, he has manife sted no cardiac symptoms while 14. Hickey AJ, Wilcken DEL. Prevalence of mitral valve prolapse in an Australian popul ation . Lancet 1980:I:1366 . returnin g to his preoperative moderately strenuous exerci se 15. Stollenna n GH . Rheum atic and heritabl e connective tissue diseases level. He had minor problems with wound healing in his of the cardiovascular system . In: Braunwald E. ed. Heart Disease. legs, but this resolved with careful outpatient follow-up by Philadelphia: WB Saunders, 1984:1641-75.