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Repeat cardiac operation in a patient with osteogenesis imperfecta
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CORRESPONDENCE
References 1. Pathi V, Guererro R, MacArthur KJD, Jamieson MPG, Pollock JCS. Sinus venosus defect: single-patch repair with caval enlargement. Ann Thorac Surg 1995;59:1588-9. 2. Victor S. Butterfly Gore-Tex patch for repair of sinus venosus ASD [Abstract]. Indian Heart J 1983;35:271. 3. Victor S. Progress in thoracic and cardiovascular surgery. In: Rao MM, ed. Progress in surgery. Madras: The Association of Surgeons of India, 1984:65-8. 4. Victor S. New surgical techniques in congenital heart disease. In: Shetty KR, Parulkar GB, eds. Proceedings of the World Conference on Open Heart Surgery, 1985, Bombay. New Delhi: Tata McGraw-Hill Publishing Co Ltd, 1987:52. 5. Victor S, Nayak VM. Transcaval repair of sinus venosus defect using butterfly shaped patch. Tex Heart Inst J 1995;22:304-7.
Repeat Cardiac Operation in a Patient With Osteogenesis Imperfecta 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 m a n with OI had an aortic valve replacement for aortic regurgitation in 1981 with a no. 23 BjSrk-Shiley valve. His course was complicated by bleeding, a n d 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 h a d 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.
© 1996 by The Society of Thoracic Surgeons Published by Elsevier Science Inc
Ann Thorac Surg 1996;61:1288-94
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 h a d 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 h a d 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 /3-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 ]3-blockers to try to reduce future episodes of dissection. He has h a d no sternal healing problems.
Robert James Cusimano, MD Division of Cardiovascular Surgery The Toronto Hospital EN13-226 200 Elizabeth St Toronto, ON M5G 2C4 Canada References 1. Almassi GH, Hughes GR, Bartlett J. Combined valve replacement and coronary bypass grafting in osteogenesis imperfecta. Ann Thorac Surg 1995;60:1395-7. 2. Moriyama Y, Nishida T, Toyohira H, et al. Acute aortic dissection in a patient with osteogenesis imperfecta. A n n Thorac Surg 1995;60:1397-9. 3. Wong RS, Follis FM, Shively BK, Wernly JA. Osteogenesis imperfecta and cardiovascular diseases. A n n Thorac Surg 1995;60:1439-43. 4. Shores J, Berger KR, Murphy EA, Pyeritz RE. Progression of aortic dilation a n d the benefit of long-term (beta)-adrenergic blockade in Marfan's syndrome. N Engl J Med 1994;330: 1335-45.
0003-4975/96/$15.00
CORRESPONDENCE
References 1. Pathi V, Guererro R, MacArthur KJD, Jamieson MPG, Pollock JCS. Sinus venosus defect: single-patch repair with caval enlargement. Ann Thorac Surg 1995;59:1588-9. 2. Victor S. Butterfly Gore-Tex patch for repair of sinus venosus ASD [Abstract]. Indian Heart J 1983;35:271. 3. Victor S. Progress in thoracic and cardiovascular surgery. In: Rao MM, ed. Progress in surgery. Madras: The Association of Surgeons of India, 1984:65-8. 4. Victor S. New surgical techniques in congenital heart disease. In: Shetty KR, Parulkar GB, eds. Proceedings of the World Conference on Open Heart Surgery, 1985, Bombay. New Delhi: Tata McGraw-Hill Publishing Co Ltd, 1987:52. 5. Victor S, Nayak VM. Transcaval repair of sinus venosus defect using butterfly shaped patch. Tex Heart Inst J 1995;22:304-7.
Repeat Cardiac Operation in a Patient With Osteogenesis Imperfecta 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 m a n with OI had an aortic valve replacement for aortic regurgitation in 1981 with a no. 23 BjSrk-Shiley valve. His course was complicated by bleeding, a n d 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 h a d 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.
© 1996 by The Society of Thoracic Surgeons Published by Elsevier Science Inc
Ann Thorac Surg 1996;61:1288-94
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 h a d 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 h a d 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 /3-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 ]3-blockers to try to reduce future episodes of dissection. He has h a d no sternal healing problems.
Robert James Cusimano, MD Division of Cardiovascular Surgery The Toronto Hospital EN13-226 200 Elizabeth St Toronto, ON M5G 2C4 Canada References 1. Almassi GH, Hughes GR, Bartlett J. Combined valve replacement and coronary bypass grafting in osteogenesis imperfecta. Ann Thorac Surg 1995;60:1395-7. 2. Moriyama Y, Nishida T, Toyohira H, et al. Acute aortic dissection in a patient with osteogenesis imperfecta. A n n Thorac Surg 1995;60:1397-9. 3. Wong RS, Follis FM, Shively BK, Wernly JA. Osteogenesis imperfecta and cardiovascular diseases. A n n Thorac Surg 1995;60:1439-43. 4. Shores J, Berger KR, Murphy EA, Pyeritz RE. Progression of aortic dilation a n d the benefit of long-term (beta)-adrenergic blockade in Marfan's syndrome. N Engl J Med 1994;330: 1335-45.
0003-4975/96/$15.00