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Ann Thorac Surg 1996;61:1294
© 1996 The Society of Thoracic Surgeons
Division of Cardiovascular Surgery, The Toronto Hospital, EN13-226, 200 Elizabeth St, Toronto, ON M5G 2C4, Canada
To the Editor:
I was interested to read the articles on the cardiovascular effects of osteogenesis imperfecta (OI) in the November 1995 issue of The Annals of Thoracic Surgery [1–3]. I would like to add my patient to the reported series, so that knowledge can be increased.
A 54-year-old man with OI had an aortic valve replacement for aortic regurgitation in 1981 with a no. 23 Björk-Shiley valve. His course was complicated by bleeding, and he required reexploration. Twelve hours before his second operation, sudden left neck pain developed, and he presented to another hospital with dyspnea. Other than recent-onset hypertension and multiple recurrent bone fractures (and an active stress fracture of the tibia), he was well. Computed tomographic scan performed at the first hospital revealed a type A ascending aortic dissection. He was then transferred to our hospital in congestive heart failure. Examination showed very distended jugular veins and hypotension consistent with cardiac tamponade. His international normalized ratio was 5 on admission. He had been taking warfarin for his mechanical valve.
He was emergently taken to the operating room, where he underwent an uneventful resternotomy. Femoral-arterial right atrial bypass was commenced, and the ascending aorta was replaced with a no. 26 Hemashield graft (Meadox Medical, Oakland, NJ). He had sustained a dissection extending into the aortic arch, and the aorta had actually freely ruptured at the noncoronary sinus. The graft was sutured onto the valve ring, which was left in place as it was functioning well, and the coronary arteries were reattached. Circulatory arrest time was 32 minutes at 20°C using retrograde cerebral perfusion via the superior vena cava.
The patient had no hemodynamic problems weaning from cardiopulmonary bypass. However, he suffered from a severe coagulopathy that required multiple blood, platelet, and coagulation factor replacement. He had few problems after leaving the operating room and was discharged 11 days postoperatively on a regimen of digoxin, warfarin, metoprolol, and enalapril. He is alive and well 8 months postoperatively and has had no sternal healing problems. Repeat computed tomographic scan 6 months later showed no residual dissection.
This case is unusual in that we report a patient with OI who underwent a redo cardiac operation, namely, repair of a ruptured ascending aortic dissection. He was sent home on ß-blocker therapy, both for a midventricular obstruction noted on postoperative echocardiography and to hopefully reduce the risk of future dissection [4].
Cardiac problems in OI are rare, and despite the fact that OI is part of the same family as Marfan's syndrome, with similar cardiovascular manifestations [1, 3], aortic dissection has not commonly been reported. In our patient, as in many others, coagulation problems developed after cardiopulmonary bypass on both occasions, confirming the tendency for these patients to bleed. As in our other cases of Marfan's syndrome or dissections, we will continue to give our patient ß-blockers to try to reduce future episodes of dissection. He has had no sternal healing problems.
References
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