View metadata, citation and similar papers at core.ac.uk brought to you by CORE 960 provided bylACC Elsevier Vol. - Publisher 9. No.4 Connector April 1987:960-3 CASE REPORTS Successful Aortocoronary Bypass in Osteogenesis Imperfecta 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 ©1987 by the American Collegeof Cardiology 0735-1097/87/$3.50 l ACC Vol. 9. No.4 PASSMORE ET AL. %1 April 1987:960--) CORONARY BY PASS IN OSTEOGENESIS IMPERFECTA A B --'-"--,-, 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