- Email: [email protected]
Failure of Percutaneous Closure of Prosthetic, Aortic Paravalvular Leak
CASE REPORT CASTEDO ET AL FAILED PERCUTANEOUS CLOSURE OF AORTIC PARAVALVULAR LEAK
in the right atrium at the right atrial appendage being careful to prevent breakage of the tumor mass. The patient was placed on cardiopulmonary bypass and was cooled down to 21.5°C. With a flat line electroencephalogram, thiopental and steroids were given. Her head was packed in ice and placed in a trendelenburg position, circulation was stopped, and the venous blood was withdrawn into the pump reservoir. Under circulatory arrest, a right artiotomy was made toward the inferior vena cava, and then at the kidney level, the cuff of the inferior vena cava at the attachment of the right renal vein was cut. The right kidney and the tumor were removed. The vena cava and the right atrium were repaired with 5-0 and 4-0 polypropylene sutures, respectively. Cardiopulmonary bypass was resumed after 19 minutes of circulatory arrest. Warming continued to 36°C. The patient was weaned from cardiopulmonary bypass without the need for any inotropic support. During the course of the operation, all shed blood was suctioned and recirculated back into the patient. Blood products included packed red blood cells, fresh frozen plasma, and platelets for hemostasis. Recovery occurred in the intensive care unit, and the patient was discharged on postoperative day 4.
Comment Renal cell carcinoma extending into the renal vein and the inferior vena cava as a tumor thrombus occurs in approximately 6% of all patients [2]. This tumor is not responsive to radiation and chemotherapeutics making radical surgical resection the definitive treatment for patients with no metastatic disease [3]. Patient symptoms may be completely absent, but when clinical manifestations do occur, symptoms are diverse and dependent on the tumor size, type, location, and caval-cardiac extension [4]. Severe paroxysmal dyspnea, syncope, diminished pulses during symptomatic paroxysms, changing heart murmurs, severe diuretic resistance, lower extremity edema, markedly dilated abdominal veins, and abdominal masses are highly characteristic and suggestive of this syndrome [4, 5]. Many surgical techniques have been described for this type of tumor, but DHCA provides a bloodless field and prevents tumor embolization [2– 4]. An operative mortality of 0% to 10% is reported with DHCA [4]. When surgery is performed without the use of cardiopulmonary bypass a mortality rate of as much as 30% can be expected with bleeding being the highest complication [4]. This case was further complicated by a known diagnosis of CA. The presence of cold agglutinins leads to hemagglutination at low temperatures followed by complement fixation and hemolysis when rewarming after cardiopulmonary bypass [6]. If the diagnosis of CA is unknown, the perfusionist may notice a separation of red blood cells and plasma, incomplete cardioplegic delivery or high pressures in the cardiopulmonary bypass circuit [6]. Further evidence of complications from CA includes hemolysis at the surgical site, microvascular occlusion, and decreased organ perfusion [1, 7]. Fortunately, this patient was diagnosed with CA preoperatively. All patients undergoing DHCA should have © 2009 by The Society of Thoracic Surgeons Published by Elsevier Inc
1327
screening for CA to prevent life-threatening complications from CA, such as cerebral or myocardial infarction, hepatic or renal failure, and hemolysis [6, 7]. The plasma titer of CAs and the thermal amplitude at which hemagglutination occurs is critical to planning a successful surgery for a patient requiring DHCA [6]. Patients are considered positive for CA at a titer of 1:70 or higher [7]. Prescreening should include queries about acrocyanosis, Reynaud’s phenomenon, hemoglobinuria, jaundice, and pallor [1]. This patient’s CA titer was 1:512, and the thermal amplitude was 17°C. Preoperative treatment was aimed at lowering the titer, and intraoperative management focused on not cooling lower than 20°C.
References 1. Hoffman JW, Gilbert TB, Hyder ML. Cold agglutinins complicating repair of aortic dissection using cardiopulmonary bypass and hypothermic circulatory arrest: case report and review. Perfusion 2002;17:391– 4. 2. Nesbitt JC, Soltero ER, Dinney CPN, et al. Surgical management of renal cell carcinoma with inferior vena cava tumor thrombus. Ann Thor Surg 1997;63:1592– 600. 3. Wotkowicz C, Libertino JA, Sorcini A, Mourtzinos A. Management of renal cell carcinoma with vena cava and atrial thrombus: minimal access vs. median sternotomy with circulatory arrest. BJU International 2006;98:289 –97. 4. Almassi GH. Surgery for tumors with cavoatrial extension. Semin Thorac Cardiovasc Surg 2000;12:111– 8. 5. Weinberg BA, Concen JD, Jr Waller BF. Cardiac manifestations of noncardiac tumors. Part II Direct effects. Clin Cardiol 1989;12:347–54. 6. Atkinson VP, Soeding P, Horne G, Taoulis J. Cold agglutinins in cardiac surgery: Management of myocardial protection and cardiopulmonary bypass. Ann Thor Surg 2008;85:311–3. 7. Madershahian N, Franke UFW, Jutte, H, Wippermann J, Berz D, Wahlers T. Cold agglutinins in on-pump cardiac surgery: a rare but potentially lethal problem. Int J Perfusionists 2004;1:1– 4.
Failure of Percutaneous Closure of Prosthetic, Aortic Paravalvular Leak Evaristo Castedo, MD, Santiago Serrano-Fiz, MD, Juan F. Oteo, MD, Sebastian Ramis, MD, Paloma Martinez, MD, and Juan Ugarte, MD Department of Cardiothoracic Surgery, Hospital Universitario Puerta de Hierro Majadahonda, Universidad Autonoma de Madrid, Madrid, Spain
We report the progression of aortic insufficiency after percutaneous closure of an aortic prosthesis paravalvular leak with the Amplatzer vascular plug (AGA Inc, Golden Valley, MN). Removal of the device and replacement of the aortic prosthesis was successfully performed. Based on operative findings, we hypothesize that shape mismatch between the occluder system and the leak might promote tearing at the end of slanted defects further enhancing the regurgitant area. (Ann Thorac Surg 2009;88:1327–9) © 2009 by The Society of Thoracic Surgeons Accepted for publication Feb 17, 2009. Address correspondence to Dr Castedo, Department of Cardiothoracic Surgery, Hospital Universitario Puerta de Hierro Majadahonda, Manuel de Falla 1, Majadahonda, 28222, Spain; e-mail: [email protected].
0003-4975/09/$36.00 doi:10.1016/j.athoracsur.2009.02.054
FEATURE ARTICLES
Ann Thorac Surg 2009;88:1327–9
1328
CASE REPORT CASTEDO ET AL FAILED PERCUTANEOUS CLOSURE OF AORTIC PARAVALVULAR LEAK
P
araprosthetic leak is a relatively common complication after heart valve replacement. Redoing the operation in these cases is a difficult procedure with significant associated morbidity and mortality. Transcatheter closure has recently emerged as a promising alternative to open heart surgery. However, although complete leak correction can be achieved, the outcome of a percutaneous method is varied and complications such as device embolization, valve obstruction, arrhythmias, and thromboembolism have been reported [1, 2].
FEATURE ARTICLES
A 79-year-old man was referred to our department from a foreign institution with an aortic paraprosthetic leak. Six years ago he had undergone Hancock II aortic valve replacement for aortic stenosis and insufficiency. The aortic root and annulus were reported to be hugely calcified at the time of surgery. A paravalvular leak that caused moderate aortic regurgitation was detected in the early postoperative period, but as the patient was asymptomatic he was discharged and followed-up. Five years later, he presented with dyspnea and the aortic insufficiency had progressed to severity, pressure half-time was less than 200 msec, and the regurgitant jet area and left ventricular outflow tract area was 50%. An attempt at percutaneous transcatheter closure was then recommended. The device selected was a selfexpandable nitinol 16-mm Amplatzer vascular plug (AGA Inc, Golden Valley, MN) that was successfully implanted in the leak. Transesophageal echocardiogram soon after deployment showed only a tiny residual leak, but a pre-
Fig 1. (A) Intraoperative photograph showing partial detachment of the bioprosthesis (in mosquito) beneath the right coronary sinus. The Amplatzer vascular plug (AGA Inc, Golden Valley, MN) is well positioned within the leak. (B) The tear is progressing through the commissure with the left coronary sinus (arrow). Shape mismatch between the slant-eyed defect and the cylindrical occluder device. (C) The Amplatzer (AGA Inc) (in forceps) being removed.
Ann Thorac Surg 2009;88:1327–9
discharge transthoracic echocardiography revealed that the aortic regurgitation had progressed; the regurgitant jet area and left ventricular outflow tract area was then 80%, and diastolic reversal of flow in the descending thoracic aorta was detected by color flow Doppler. In view of these findings, the patient was submitted for open heart surgery. On admission to our hospital, the patient was symptomatic for recurrent dyspnea and hemolytic anaemia. He was operated on under standard cardiopulmonary bypass. Access to the valve was complicated due to a porcelain aorta, but a slant-eyed leak was observed just beneath the right coronary sinus (Fig 1A). The occluder device was correctly positioned within the leak cavity, but the dehiscence had progressed through the commissure between the left and right coronary sinus and half of the defect was actually not excluded (Fig 1B). The device was easily taken out (Fig 1C) and the bioprosthesis was replaced with a 21-mm St. Jude Medical mechanical valve (St. Jude Medical, Minneapolis, MN). The decision to implant a mechanical valve was based on the surgeon’s preference in cases of porcelain aorta to facilitate the aortic closure. The postoperative course was uneventful. The patient was discharged 7 days later in good hemodynamic condition, with no residual leak on the echocardiography.
Comment Despite evolving technology, the percutaneous management of paraprosthetic leaks is far from ideal nowadays.
CASE REPORT SANDERS ET AL TREATMENT IN A RARE CASE OF CONGENITAL HYPOFIBRINOGENEMIA
The reported series of the most experienced teams include only a few mitral procedures, and publications describing aortic leaks are rare [1]. The main reason is the current lack of a dedicated closure system. For example, one of the most widely used closure systems in the aortic position, the Amplatzer vascular plug (AGA Inc), was approved by the United States Food and Drug Administration for the occlusion of peripheral vessels [3]. The perfect fit of the occluder device within the leak cavity seems to be a major factor of success. Unfortunately, most dehiscences between the sewing ring and aortic annulus have an irregular crescent shape, and none of the available devices perfectly fit the leak defect. A cylinder-shaped device, such as the Amplatzer vascular plug may not only imperfectly occlude a slant-eyed leak, but may also promote tearing at both ends of the dehiscence while being deployed, further increasing the degree of regurgitation. This seems to be the case in our patient, in whom the aortic regurgitation jet width increased soon after the percutaneous closure attempt and a rip was observed through the left coronary sinus commissure. We should also be concerned about the surprisingly easy removal of the device and the possibility of dislodgement and catastrophic embolization. A dedicated device based on leak morphology assessed by previous operative findings is necessary to enhance the likelihood of success and should reduce the incidence of recurrent valve regurgitation and the need for reoperation.
References 1. Pate Ge, Al Zubaidi A, Chandavimol M, Thompson CR, Munt BI, Webb JG. Percutaneous closure of prosthetic paravalvular leaks: case series and review. Catheter Cardiovasc Interv 2006;68:528 –33. 2. Aranzulla TC, Cosgrave J, La Canna G, et al. Percutaneous treatment of periprosthetic mitral valve leaks: is it just a futile exercise? Ann Thorac Surg 2008;86:996 – 8. 3. Hill SL, Hijazi ZM, Hellenbrand WE, Cheatham JP. Evaluation of the Amplatzer vascular plug for embolization of peripheral vascular malformations associated with congenital heart disease. Catheter Cardiovasc Interv 2006;67:113–9.
Aortic Valve Replacement and Coronary Artery Bypass Grafting in a Rare Case of Congenital Hypofibrinogenemia Lucas H. A. Sanders, MD, FRACS, Ben J. Anderson, MD, FRACS, Jaffar Shehatha, MD, FRCS, Mara Clarson, MD, and Julie A. Mundy, MD, FRACS Department of Cardiothoracic Surgery, Princess Alexandra Hospital, Woolloongabba, Australia
Congenital hypofibrinogenemia is a rare condition. We believe that cardiac surgery using cardiopulmonary bypass Accepted for publication Jan 20, 2009. Address correspondence to Dr Sanders, Department of Cardiothoracic Surgery, Princess Alexandra Hospital, Ipswich Rd, Woolloongabba, 4012, Australia; e-mail: [email protected].
© 2009 by The Society of Thoracic Surgeons Published by Elsevier Inc
1329
in a patient with congenital hypofibrinogenemia has not been reported before. We discuss the management in a patient with this condition who successfully underwent an aortic valve replacement and coronary artery bypass grafting. (Ann Thorac Surg 2009;88:1329 –31) © 2009 by The Society of Thoracic Surgeons
C
ongenital hypofibrinogenemia is a rare condition associated with bleeding episodes during trauma or surgery. Rarely thromboembolic complications occur, and paradoxically these are negatively associated with the fibrinogen levels. We believe that the perioperative course and management for cardiac surgery using cardiopulmonary bypass of a patient with congenital hypofibrinogenemia has not been reported before. A 69-year-old man, known to have congenital hypofibrinogenemia, presented with symptoms of angina Canadian Cardiovascular Society class II-III, occasional dizziness, and palpitations. Echocardiogram revealed severe calcific aortic stenosis with an aortic valve area of 0.7 cm2, mean/peak aortic gradient of 51/89 mm Hg, normal left ventricular systolic function, and mild left ventricular hypertrophy. Coronary angiogram showed severe triple-vessel disease with multiple 60% to 80% stenoses. Six years previously he had been refused for coronary artery bypass grafting because of the risk of bleeding associated with hypofibrinogenemia. Medical management had then been continued. From an early age on he had prolonged bleeding after injuries, but no prior episodes of spontaneous bleeding. After a near asphyxiation event due to pharyngeal bleeding after spine surgery 20 years ago, he was diagnosed with hypofibrinogenemia. At times he needed blood transfusions after tooth extractions. Concomitant medical history included dyslipidemia, hypertension, past heavy smoking (50 pack/year), constipation, obstructive sleep apnea, and symptomatic spinal stenosis awaiting repeat cervical spine surgery. Preoperative coagulation profile showed an international normalized ratio of 1.2 (reference range [ref], 0.9 –1.2), prothrombin time of 12 seconds (ref, 9 –12), activated partial thromboplastin time of 30 seconds (ref, 24 –39), thrombin time of 15 seconds (ref, 11–16), reptilase time of 18 seconds (ref ⬍ 20), fibrinogen (derived) 1.2 g/L (ref, 1.7– 4.5), fibrinogen (clottable) 1.1g/L (ref, 2.0 – 4.5), and platelets 227 ⫻ 109/L (ref, 140 – 400). In preparation for cardiac surgery platelet inhibitors (aspirin and nonsteroidal antiinflammatory drugs) were ceased a week before surgery. At surgery the aortic valve was replaced with a size 23 Perimount bioprosthesis (Edwards Lifesciences, Irvine, CA) and four coronary artery bypass grafts were performed (single reversed, long saphenous vein to left anterior descending coronary artery and sequential saphenous vein to diagonal, obtuse marginal and posterolateral artery). Aortic cross clamp and bypass times were 132 and 188 minutes, respectively. Before induction of anesthesia, 5 units of cryoprecipitate were administered. Another 5 units were administered after reversal of heparin with protamine at completion of surgery. He received 2 million units of 0003-4975/09/$36.00 doi:10.1016/j.athoracsur.2009.01.062
FEATURE ARTICLES
Ann Thorac Surg 2009;88:1329 –31
in the right atrium at the right atrial appendage being careful to prevent breakage of the tumor mass. The patient was placed on cardiopulmonary bypass and was cooled down to 21.5°C. With a flat line electroencephalogram, thiopental and steroids were given. Her head was packed in ice and placed in a trendelenburg position, circulation was stopped, and the venous blood was withdrawn into the pump reservoir. Under circulatory arrest, a right artiotomy was made toward the inferior vena cava, and then at the kidney level, the cuff of the inferior vena cava at the attachment of the right renal vein was cut. The right kidney and the tumor were removed. The vena cava and the right atrium were repaired with 5-0 and 4-0 polypropylene sutures, respectively. Cardiopulmonary bypass was resumed after 19 minutes of circulatory arrest. Warming continued to 36°C. The patient was weaned from cardiopulmonary bypass without the need for any inotropic support. During the course of the operation, all shed blood was suctioned and recirculated back into the patient. Blood products included packed red blood cells, fresh frozen plasma, and platelets for hemostasis. Recovery occurred in the intensive care unit, and the patient was discharged on postoperative day 4.
Comment Renal cell carcinoma extending into the renal vein and the inferior vena cava as a tumor thrombus occurs in approximately 6% of all patients [2]. This tumor is not responsive to radiation and chemotherapeutics making radical surgical resection the definitive treatment for patients with no metastatic disease [3]. Patient symptoms may be completely absent, but when clinical manifestations do occur, symptoms are diverse and dependent on the tumor size, type, location, and caval-cardiac extension [4]. Severe paroxysmal dyspnea, syncope, diminished pulses during symptomatic paroxysms, changing heart murmurs, severe diuretic resistance, lower extremity edema, markedly dilated abdominal veins, and abdominal masses are highly characteristic and suggestive of this syndrome [4, 5]. Many surgical techniques have been described for this type of tumor, but DHCA provides a bloodless field and prevents tumor embolization [2– 4]. An operative mortality of 0% to 10% is reported with DHCA [4]. When surgery is performed without the use of cardiopulmonary bypass a mortality rate of as much as 30% can be expected with bleeding being the highest complication [4]. This case was further complicated by a known diagnosis of CA. The presence of cold agglutinins leads to hemagglutination at low temperatures followed by complement fixation and hemolysis when rewarming after cardiopulmonary bypass [6]. If the diagnosis of CA is unknown, the perfusionist may notice a separation of red blood cells and plasma, incomplete cardioplegic delivery or high pressures in the cardiopulmonary bypass circuit [6]. Further evidence of complications from CA includes hemolysis at the surgical site, microvascular occlusion, and decreased organ perfusion [1, 7]. Fortunately, this patient was diagnosed with CA preoperatively. All patients undergoing DHCA should have © 2009 by The Society of Thoracic Surgeons Published by Elsevier Inc
1327
screening for CA to prevent life-threatening complications from CA, such as cerebral or myocardial infarction, hepatic or renal failure, and hemolysis [6, 7]. The plasma titer of CAs and the thermal amplitude at which hemagglutination occurs is critical to planning a successful surgery for a patient requiring DHCA [6]. Patients are considered positive for CA at a titer of 1:70 or higher [7]. Prescreening should include queries about acrocyanosis, Reynaud’s phenomenon, hemoglobinuria, jaundice, and pallor [1]. This patient’s CA titer was 1:512, and the thermal amplitude was 17°C. Preoperative treatment was aimed at lowering the titer, and intraoperative management focused on not cooling lower than 20°C.
References 1. Hoffman JW, Gilbert TB, Hyder ML. Cold agglutinins complicating repair of aortic dissection using cardiopulmonary bypass and hypothermic circulatory arrest: case report and review. Perfusion 2002;17:391– 4. 2. Nesbitt JC, Soltero ER, Dinney CPN, et al. Surgical management of renal cell carcinoma with inferior vena cava tumor thrombus. Ann Thor Surg 1997;63:1592– 600. 3. Wotkowicz C, Libertino JA, Sorcini A, Mourtzinos A. Management of renal cell carcinoma with vena cava and atrial thrombus: minimal access vs. median sternotomy with circulatory arrest. BJU International 2006;98:289 –97. 4. Almassi GH. Surgery for tumors with cavoatrial extension. Semin Thorac Cardiovasc Surg 2000;12:111– 8. 5. Weinberg BA, Concen JD, Jr Waller BF. Cardiac manifestations of noncardiac tumors. Part II Direct effects. Clin Cardiol 1989;12:347–54. 6. Atkinson VP, Soeding P, Horne G, Taoulis J. Cold agglutinins in cardiac surgery: Management of myocardial protection and cardiopulmonary bypass. Ann Thor Surg 2008;85:311–3. 7. Madershahian N, Franke UFW, Jutte, H, Wippermann J, Berz D, Wahlers T. Cold agglutinins in on-pump cardiac surgery: a rare but potentially lethal problem. Int J Perfusionists 2004;1:1– 4.
Failure of Percutaneous Closure of Prosthetic, Aortic Paravalvular Leak Evaristo Castedo, MD, Santiago Serrano-Fiz, MD, Juan F. Oteo, MD, Sebastian Ramis, MD, Paloma Martinez, MD, and Juan Ugarte, MD Department of Cardiothoracic Surgery, Hospital Universitario Puerta de Hierro Majadahonda, Universidad Autonoma de Madrid, Madrid, Spain
We report the progression of aortic insufficiency after percutaneous closure of an aortic prosthesis paravalvular leak with the Amplatzer vascular plug (AGA Inc, Golden Valley, MN). Removal of the device and replacement of the aortic prosthesis was successfully performed. Based on operative findings, we hypothesize that shape mismatch between the occluder system and the leak might promote tearing at the end of slanted defects further enhancing the regurgitant area. (Ann Thorac Surg 2009;88:1327–9) © 2009 by The Society of Thoracic Surgeons Accepted for publication Feb 17, 2009. Address correspondence to Dr Castedo, Department of Cardiothoracic Surgery, Hospital Universitario Puerta de Hierro Majadahonda, Manuel de Falla 1, Majadahonda, 28222, Spain; e-mail: [email protected].
0003-4975/09/$36.00 doi:10.1016/j.athoracsur.2009.02.054
FEATURE ARTICLES
Ann Thorac Surg 2009;88:1327–9
1328
CASE REPORT CASTEDO ET AL FAILED PERCUTANEOUS CLOSURE OF AORTIC PARAVALVULAR LEAK
P
araprosthetic leak is a relatively common complication after heart valve replacement. Redoing the operation in these cases is a difficult procedure with significant associated morbidity and mortality. Transcatheter closure has recently emerged as a promising alternative to open heart surgery. However, although complete leak correction can be achieved, the outcome of a percutaneous method is varied and complications such as device embolization, valve obstruction, arrhythmias, and thromboembolism have been reported [1, 2].
FEATURE ARTICLES
A 79-year-old man was referred to our department from a foreign institution with an aortic paraprosthetic leak. Six years ago he had undergone Hancock II aortic valve replacement for aortic stenosis and insufficiency. The aortic root and annulus were reported to be hugely calcified at the time of surgery. A paravalvular leak that caused moderate aortic regurgitation was detected in the early postoperative period, but as the patient was asymptomatic he was discharged and followed-up. Five years later, he presented with dyspnea and the aortic insufficiency had progressed to severity, pressure half-time was less than 200 msec, and the regurgitant jet area and left ventricular outflow tract area was 50%. An attempt at percutaneous transcatheter closure was then recommended. The device selected was a selfexpandable nitinol 16-mm Amplatzer vascular plug (AGA Inc, Golden Valley, MN) that was successfully implanted in the leak. Transesophageal echocardiogram soon after deployment showed only a tiny residual leak, but a pre-
Fig 1. (A) Intraoperative photograph showing partial detachment of the bioprosthesis (in mosquito) beneath the right coronary sinus. The Amplatzer vascular plug (AGA Inc, Golden Valley, MN) is well positioned within the leak. (B) The tear is progressing through the commissure with the left coronary sinus (arrow). Shape mismatch between the slant-eyed defect and the cylindrical occluder device. (C) The Amplatzer (AGA Inc) (in forceps) being removed.
Ann Thorac Surg 2009;88:1327–9
discharge transthoracic echocardiography revealed that the aortic regurgitation had progressed; the regurgitant jet area and left ventricular outflow tract area was then 80%, and diastolic reversal of flow in the descending thoracic aorta was detected by color flow Doppler. In view of these findings, the patient was submitted for open heart surgery. On admission to our hospital, the patient was symptomatic for recurrent dyspnea and hemolytic anaemia. He was operated on under standard cardiopulmonary bypass. Access to the valve was complicated due to a porcelain aorta, but a slant-eyed leak was observed just beneath the right coronary sinus (Fig 1A). The occluder device was correctly positioned within the leak cavity, but the dehiscence had progressed through the commissure between the left and right coronary sinus and half of the defect was actually not excluded (Fig 1B). The device was easily taken out (Fig 1C) and the bioprosthesis was replaced with a 21-mm St. Jude Medical mechanical valve (St. Jude Medical, Minneapolis, MN). The decision to implant a mechanical valve was based on the surgeon’s preference in cases of porcelain aorta to facilitate the aortic closure. The postoperative course was uneventful. The patient was discharged 7 days later in good hemodynamic condition, with no residual leak on the echocardiography.
Comment Despite evolving technology, the percutaneous management of paraprosthetic leaks is far from ideal nowadays.
CASE REPORT SANDERS ET AL TREATMENT IN A RARE CASE OF CONGENITAL HYPOFIBRINOGENEMIA
The reported series of the most experienced teams include only a few mitral procedures, and publications describing aortic leaks are rare [1]. The main reason is the current lack of a dedicated closure system. For example, one of the most widely used closure systems in the aortic position, the Amplatzer vascular plug (AGA Inc), was approved by the United States Food and Drug Administration for the occlusion of peripheral vessels [3]. The perfect fit of the occluder device within the leak cavity seems to be a major factor of success. Unfortunately, most dehiscences between the sewing ring and aortic annulus have an irregular crescent shape, and none of the available devices perfectly fit the leak defect. A cylinder-shaped device, such as the Amplatzer vascular plug may not only imperfectly occlude a slant-eyed leak, but may also promote tearing at both ends of the dehiscence while being deployed, further increasing the degree of regurgitation. This seems to be the case in our patient, in whom the aortic regurgitation jet width increased soon after the percutaneous closure attempt and a rip was observed through the left coronary sinus commissure. We should also be concerned about the surprisingly easy removal of the device and the possibility of dislodgement and catastrophic embolization. A dedicated device based on leak morphology assessed by previous operative findings is necessary to enhance the likelihood of success and should reduce the incidence of recurrent valve regurgitation and the need for reoperation.
References 1. Pate Ge, Al Zubaidi A, Chandavimol M, Thompson CR, Munt BI, Webb JG. Percutaneous closure of prosthetic paravalvular leaks: case series and review. Catheter Cardiovasc Interv 2006;68:528 –33. 2. Aranzulla TC, Cosgrave J, La Canna G, et al. Percutaneous treatment of periprosthetic mitral valve leaks: is it just a futile exercise? Ann Thorac Surg 2008;86:996 – 8. 3. Hill SL, Hijazi ZM, Hellenbrand WE, Cheatham JP. Evaluation of the Amplatzer vascular plug for embolization of peripheral vascular malformations associated with congenital heart disease. Catheter Cardiovasc Interv 2006;67:113–9.
Aortic Valve Replacement and Coronary Artery Bypass Grafting in a Rare Case of Congenital Hypofibrinogenemia Lucas H. A. Sanders, MD, FRACS, Ben J. Anderson, MD, FRACS, Jaffar Shehatha, MD, FRCS, Mara Clarson, MD, and Julie A. Mundy, MD, FRACS Department of Cardiothoracic Surgery, Princess Alexandra Hospital, Woolloongabba, Australia
Congenital hypofibrinogenemia is a rare condition. We believe that cardiac surgery using cardiopulmonary bypass Accepted for publication Jan 20, 2009. Address correspondence to Dr Sanders, Department of Cardiothoracic Surgery, Princess Alexandra Hospital, Ipswich Rd, Woolloongabba, 4012, Australia; e-mail: [email protected].
© 2009 by The Society of Thoracic Surgeons Published by Elsevier Inc
1329
in a patient with congenital hypofibrinogenemia has not been reported before. We discuss the management in a patient with this condition who successfully underwent an aortic valve replacement and coronary artery bypass grafting. (Ann Thorac Surg 2009;88:1329 –31) © 2009 by The Society of Thoracic Surgeons
C
ongenital hypofibrinogenemia is a rare condition associated with bleeding episodes during trauma or surgery. Rarely thromboembolic complications occur, and paradoxically these are negatively associated with the fibrinogen levels. We believe that the perioperative course and management for cardiac surgery using cardiopulmonary bypass of a patient with congenital hypofibrinogenemia has not been reported before. A 69-year-old man, known to have congenital hypofibrinogenemia, presented with symptoms of angina Canadian Cardiovascular Society class II-III, occasional dizziness, and palpitations. Echocardiogram revealed severe calcific aortic stenosis with an aortic valve area of 0.7 cm2, mean/peak aortic gradient of 51/89 mm Hg, normal left ventricular systolic function, and mild left ventricular hypertrophy. Coronary angiogram showed severe triple-vessel disease with multiple 60% to 80% stenoses. Six years previously he had been refused for coronary artery bypass grafting because of the risk of bleeding associated with hypofibrinogenemia. Medical management had then been continued. From an early age on he had prolonged bleeding after injuries, but no prior episodes of spontaneous bleeding. After a near asphyxiation event due to pharyngeal bleeding after spine surgery 20 years ago, he was diagnosed with hypofibrinogenemia. At times he needed blood transfusions after tooth extractions. Concomitant medical history included dyslipidemia, hypertension, past heavy smoking (50 pack/year), constipation, obstructive sleep apnea, and symptomatic spinal stenosis awaiting repeat cervical spine surgery. Preoperative coagulation profile showed an international normalized ratio of 1.2 (reference range [ref], 0.9 –1.2), prothrombin time of 12 seconds (ref, 9 –12), activated partial thromboplastin time of 30 seconds (ref, 24 –39), thrombin time of 15 seconds (ref, 11–16), reptilase time of 18 seconds (ref ⬍ 20), fibrinogen (derived) 1.2 g/L (ref, 1.7– 4.5), fibrinogen (clottable) 1.1g/L (ref, 2.0 – 4.5), and platelets 227 ⫻ 109/L (ref, 140 – 400). In preparation for cardiac surgery platelet inhibitors (aspirin and nonsteroidal antiinflammatory drugs) were ceased a week before surgery. At surgery the aortic valve was replaced with a size 23 Perimount bioprosthesis (Edwards Lifesciences, Irvine, CA) and four coronary artery bypass grafts were performed (single reversed, long saphenous vein to left anterior descending coronary artery and sequential saphenous vein to diagonal, obtuse marginal and posterolateral artery). Aortic cross clamp and bypass times were 132 and 188 minutes, respectively. Before induction of anesthesia, 5 units of cryoprecipitate were administered. Another 5 units were administered after reversal of heparin with protamine at completion of surgery. He received 2 million units of 0003-4975/09/$36.00 doi:10.1016/j.athoracsur.2009.01.062
FEATURE ARTICLES
Ann Thorac Surg 2009;88:1329 –31