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Ann Thorac Surg 1995;60:1395-1397
© 1995 The Society of Thoracic Surgeons

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Case Reports

Combined Valve Replacement and Coronary Bypass Grafting in Osteogenesis Imperfecta

Medical College of Wisconsin and Columbia Hospital, Milwaukee, Wisconsin

Accepted for publication April 7, 1995.

	Abstract

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Open cardiac procedures in osteogenesis imperfecta have been associated with a high mortality rate. A patient with osteogenesis imperfecta underwent successful aortic valve replacement and coronary artery bypass grafting along with closure of a patent foramen ovale in preparation for a planned hip replacement.

	Introduction

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Osteogenesis imperfecta is a rare disease that can cause even rarer valvular heart disease. We could only find 1 case report of successful combined valve replacement and coronary bypass grafting in this disease entity in the English-language literature [1]. We are reporting a case of osteogenesis imperfecta that successfully underwent combined valvular-coronary bypass operation along with closure of a patent foramen ovale.

A 40-year-old man with osteogenesis imperfecta underwent a cardiac evaluation as part of a preoperative work-up for a planned total hip replacement. Echocardiography revealed moderately severe aortic insufficiency and a patent foramen ovale. Cardiac catheterization disclosed 80% stenosis of the left anterior descending and second diagonal coronary arteries. The patient was advised to have combined aortic valve operation and coronary artery bypass grafting.

Past medical history was positive for multiple bone and rib fractures resulting in severe kyphoscoliosis, a left total hip replacement, deafness in the right ear despite two operations for ossicular implants, a right lens implant forcataract, and a ventricular shunt for spontaneous cerebral hemorrhage. He was also a heavy cigarette smoker for 30 years.

See also 1397 and 1439.

Findings at operation were a small ascending aorta and a thin, dilated aortic root and annulus, with a myxomatous and very thin trileaflet aortic valve. The aortic valve was replaced with a 29-mm St. Jude aortic valve prosthesis (St. Jude Medical, St. Paul, MN); two saphenous vein aortocoronary bypass grafts and closure of a large patent foramen ovale also were performed. The sternum was approximated with Parnham bands (Zimmer Co, Warsaw, IN) to guard against cutting of stainless steel wires through the soft sternal bone.

The patient was discharged from the hospital on the fifth postoperative day; however, he was readmitted to the hospital a week later with an asthmalike bronchospastic disorder responding ultimately to systemic steroids and bronchodilator therapy. Five months later he underwent a successful total hip replacement and is doing well 1 years after his cardiac operation.

	Comment

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Osteogenesis imperfecta is a rare hereditary disease of mendelian dominant behavior with variable penetrance and is divided into four categories [2]. The severe form (Sillence type II) affects the fetus in utero or in early infancy and is associated with high early mortality. Sillence types I, III, and IV, also called osteogenesis imperfecta tarda in older literature, are compatible with long survival and even normal longevity. Type III osteogenesis imperfecta is mendelian recessive. Patients with this disease classically have a triangular ``elfin facies,'' and blue sclerae, and may be deaf. The majority of patients suffer from multiple fractures. The major body systems affected are skeletal, ocular, auditory, integument, and teeth, and to a much lesser extent cardiovascular [3].

Cardiovascular manifestations in osteogenesis imperfecta are virtually similar to those in Marfan's syndrome. The myxomatous degeneration of the heart valves in this condition affects aortic and mitral valves more than the right-sided valves [1]. Aneurysm of the sinuses of Valsalva and ascending aorta are the other manifestations of this disease [4].

Cardiac operation in patients with osteogenesis imperfecta carries a high mortality (Table 1). In Criscitiello and associates' [5] report of 3 patients with valvular heart disease and osteogenesis imperfecta, only 1 patient underwent aortic valve reconstruction with Bahnson Teflon leaflets, and this patient died on the fourth postoperative day. Ohteki and associates' [6] patient died on the eighth postoperative day following aortic root replacement after bleeding for 5 days postoperatively. Koentges and colleagues [7] reported a patient who underwent aortic and mitral valve replacements. This patient required two interventions for mitral valve dehiscence and ultimately died. In a review of the literature, Koentges and colleagues came up with 30 cases with significant aortic and mitral valve regurgitation. Thirteen patients underwent valve operations; 4 of them died postoperatively. Stein and Kloster [8] reported a father and a daughter with valvular heart disease and osteogenesis imperfecta. The father also had coronary artery disease. He did well after mitral valve replacement, although coronary bypass grafting proved impossible. The daughter, however, died after mitral and aortic valve replacements. On the other hand, both patients reported by Wood and colleagues [9] survived after mitral valve replacement and mitral valve repair. Similarly, Weisinger and associates [10] reported aortic valve replacement with a Starr-Edwards valve in 2 patients with aortic insufficiency; both patients did very well postoperatively.

The incidence of coronary artery disease in osteogenesis imperfecta is even rarer than the incidence of valvular heart disease. Passmore and co-workers [3] reported on a patient with coronary artery disease and a successful outcome after three-vessel aortocoronary saphenous vein bypass grafting. Saphenous vein was used in preference to the internal thoracic artery for revascularization in our patient out of concern for sternal healing, despite the young age of this patient. For the same reason wide Parnham sternal bands were used for sternal closure to prevent any possible cutting of the usual stainless steel sternal wires through the soft sternal bone. We know of 1 report of a patient with osteogenesis imperfecta who underwent combined valve replacement and coronary bypass grafting with a successful outcome [1].

We remain uncertain as to the cause of the bronchospastic condition that developed in our patient postoperatively, requiring hospital readmission and ultimately systemic steroid therapy. This patient had been a heavy smoker since the age of 10 years, and this may have been an important contributory factor in the genesis of his bronchospastic disorder. Nonetheless, he recovered from this bronchospastic disorder and subsequently underwent his orthopedic operation. He has resumed his full-time job and is doing well 20 months after his cardiac operation.

	Footnotes

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Address reprint requests to Dr Almassi, Department of Cardiothoracic Surgery, Medical College of Wisconsin, 8700 W Wisconsin Ave, Milwaukee, WI 53226.

	References

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1. Thibault GE. Clinical problem-solving. The heart of the matter. N Engl J Med 1993;329:1406–10.[Free Full Text]
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9. Wood SJ, Thomas J, Braimbridge MV. Mitral valve disease and open heart surgery in osteogenesis imperfecta tarda. Br Heart J 1973;35:103–6.[Free Full Text]
10. Weisinger B, Glassman E, Spencer FC, Berger A. Successful aortic valve replacement for aortic regurgitation associated with osteogenesis imperfecta. Br Heart J 1975;37:475–7.[Abstract/Free Full Text]
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12. Melamed R, Aygen MM, Lowenstein A. Osteogenesis imperfecta with mitral insufficiency due to the ballooning of the mitral valve. A case report. Isr J Med Sci 1976;11:1325–8.
13. Waters DD, Clarke DW, Symbars PN, Schlant RC. Aortic and mitral valve replacement in a patient with osteogenesis imperfecta. Chest 1977;72:363–4.[Abstract/Free Full Text]
14. Gelach PA, Rosensweig J, Ramanathan KB. Successful aortic valve replacement in osteogenesis imperfecta: with special emphasis on peri-operative management. Can J Cardiol 1987;3:132–5.[Medline]
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				M. B. Izzat, S. Wan, I. Y.P. Wan, K. S. Khaw, and A. P.C. Yim Ministernotomy for aortic valve replacement in a patient with osteogenesis imperfecta Ann. Thorac. Surg., April 1, 1999; 67(4): 1171 - 1173. [Abstract] [Full Text] [PDF]

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This Article Abstract Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): G. Hossein Almassi Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Almassi, G. H. Articles by Bartlett, J. Search for Related Content PubMed PubMed Citation Articles by Almassi, G. H. Articles by Bartlett, J. Related Collections Related Articles