- Email: [email protected]
Jehovah's witnesses requiring complex urgent cardiothoracic surgery
Ann Thorac Surg 2004;78:695–7
695
References 1. Kato H, Sugimura T, Akagi T, et al. Long term consequences of Kawasaki disease: a 10- to 21-year follow-up study of 594 patients. Circulation 1996;94:1379 –85. 2. Yanagawa H, Yashiro M, Nakamura Y, Kawasaki T, Kato H. Results of 12 nationwide epidemiological incidence surveys of Kawasaki disease in Japan. Arch Pediatr Adolesc Med 1995; 149:779 –83. 3. Ng YM, Sung R, So LY, et al. A 5 years surveillance of Kawasaki Disease in Hong Kong-1999. Hong Kong Kawasaki Disease study group. Presented at the 10th Asian Congress of Pediatrics, March 26 –30, 1994, Taipai, Taiwan. 4. Takahashi K, Oharaseki T, Wakayama M, Shibuya K, Naoe S. Pathological study of ruptured coronary artery aneurysms of Kawasaki disease. Abstract presented at the 2nd World Congress of Pediatric cardiology and Cardiac Surgery, May 11–15, 1997, Honolulu, HI. Abstract 202. 5. Nakano H, Ueda K, Saito A, Nojima K. Repeated quantitative angiograms in coronary arterial aneurysm in Kawasaki Disease. Am J Cardiol 1985;56:846 –51. 6. Kitamuran S, Kameda Y, Seki T, et al. Long-term outcome of myocardial revascularization in patients with Kawasaki coronary artery disease. J Thorac Cardiovasc Surg 1994;107:663–74.
Jehovah’s Witnesses Requiring Complex Urgent Cardiothoracic Surgery Richard L. Holt, Tomas D. Martin, MD, Philip J. Hess, MD, Thomas M. Beaver, MD, and Charles T. Klodell, MD Division of Thoracic and Cardiovascular Surgery, University of Florida College of Medicine Health Science Center, Gainesville, Florida
Blood-sparing surgical technique and perioperative medical management allows complex surgery in Jehovah’s Witnesses. The authors review four cases of urgent cardiothoracic surgery performed on Jehovah’s Witnesses. No lasting sequelae associated with either bleeding or end-organ ischemia were noted. Cardiopulmonary bypass management, meticulous hemostatic operative technique, and pharmacologic support of hemostasis and erythropoiesis permit this complex surgery. (Ann Thorac Surg 2004;78:695–7) © 2004 by The Society of Thoracic Surgeons
Table 1. Diagnostic Features of Kawasaki Disease Fever of at least 5 days’ duration not responsive to antibiotics or antipyretic agents Presence of at least four of the following clinical features: 1) Polymorphous exanthem involving face, trunk, extremities 2) Bilateral conjunctivitis 3) Changes in the lips, strawberry tongue, erythema of oropharynx 4) Erythema of palms and soles, edema of hands and feet, followed by desquamation of fingers and toes 5) Cervical lymphadenopathy Exclusion of other diseases with similar findings
© 2004 by The Society of Thoracic Surgeons Published by Elsevier Inc
T
he Jehovah’s Witnesses, numbering greater than 5 million in the United States, refuse blood component transfusion as part of their religion. This stems from interpretation of biblical passages, not from antipathy toward medicine. The following cases are presented to demonstrate the various strategies used to permit comAccepted for publication July 30, 2003. Address reprint requests to Dr Klodell, Division of Thoracic and Cardiovascular Surgery, University of Florida College of Medicine, Health Science Center, PO Box 100286, Gainesville, FL 32610-0286; e-mail: [email protected].
0003-4975/04/$30.00 doi:10.1016/S0003-4975(03)01494-2
CASE REPORTS
corresponding incidence in Hong Kong is reported to be 30 per 100,000 children [3]. In the untreated group, coronary aneurysm occurs in about 20% of all cases, whereas coronary aneurysm develops in only 4% to 8% of children who receive IV gamma immunoglobulin [2]. Based on autopsy data, the incidence of coronary aneurysm rupture is reported to occur in 5% of cases [4]. Thrombosis of such coronary aneurysm results in myocardial infarction, the leading cause of death for these children [1]. Despite the generally accepted standard approach to the management of this disease, controversies persist regarding certain aspects of treatment. How should patients with giant coronary aneurysm be managed? Giant coronary aneurysms (defined as aneurysms with an internal luminal diameter of 8 mm or more) are much less likely to reduce in size and will often thrombose or rupture [5]. To us, pending aneurysmal rupture in spite of maximal medical therapy, as in this case, is a clear indication for surgery as ruptured giant coronary aneurysms carries very high mortality. Coronary artery bypass grafting for coronary artery stenosis, ventricular aneurysmectomy, valve replacement, and cardiac transplantation has all been described for the treatment of coronary ischemic sequelae of Kawasaki disease, but no clear recommendation is available for the optimal timing of surgical intervention during the acute phase. We believe, as highlighted in this case, that patients with enlarging coronary aneurysms should be monitored closely and early surgical intervention should be considered when repeated assessments demonstrate a progression of the size of the aneurysm despite maximal medical therapy. In this case we successfully saved a child’s life with coronary bypass grafting using venous graft alone. Although the published data suggest that an arterial graft carries a better long-term patency rate and grows with the child [6], we chose the long saphenous vein because the anastomosis site for the posterior descending artery was very distal and required a long graft that could only be provided safely by the long saphenous vein in such an emergency situation.
CASE REPORT HOLT ET AL JEHOVAH’S WITNESSES AND AORTIC SURGERY
696
CASE REPORT HOLT ET AL JEHOVAH’S WITNESSES AND AORTIC SURGERY
CASE REPORTS
plex cardiothoracic surgery in Jehovah’s Witnesses. Perioperative pharmacologic management of erythrocyte mass and coagulation is of paramount importance. Aprotinin (Trasylol; Bayer Corp, West Haven, CT) has proven effective not only in reducing transfusion requirement, but also may counteract bypass-related inflammatory response [1]. The full Hammersmith regimen of 6 million total KIU may be considered acceptable, if not standard, in Jehovah’s Witness patients [2]. Technically, fastidious hemostatic operative technique, the limited use of laparotomy pads and discard suction, and reduced blood sampling for laboratory tests minimizes “lost” blood. The use of small-volume oxygenators and small-diameter tubing, retrograde autologous priming, and intraoperative autologous donation has become fairly standard [3]. Reclamation of autologous blood loss and infusion by means of continuous circuit of salvaged cells also aid in maintaining intraoperative hematocrit [4]. The liberal use of ultrafiltration limits hypervolemic dilutional anemia, and aids in the preservation of hemostatic blood components. The patients in the following cases all agreed to intraoperative hemodilution as well as the reinfusion of blood from a cell-saving device.
Case Reports Patient 1 A 38-year-old female Jehovah’s Witness with a 6-week history of shortness of breath was noted to have evolving heart failure secondary to left-sided Streptococcus viridans endocarditis. Antibiotic therapy had been initiated 3 weeks before referral, to which regimen erythropoietin and iron were added. Echocardiogram revealed a markedly regurgitant aortic valve and a flail anterior mitral leaflet. Medical management was continued to permit optimization of red blood cell mass before surgery. The patient’s hematocrit increased from 25% on admission to 39% during 2 weeks of high-dose erythropoietin (200 U/kg daily) and iron supplementation. Intraoperative blood conservation consisted of retrograde autologous priming, intraoperative autologous transfusion, and ultrafiltration. A total bypass time of 155 minutes with a cardiac ischemic time of 110 minutes was required. After closing, her hematocrit had fallen to 18%, and there was some suspicion of continued mediastinal bleeding; therefore the surgical site was reexplored to exclude the possibility of even minimal bleeding. No bleeding was identified, and complete hemostasis was verified before reclosure. The patient experienced no postoperative complications other than edema and shortness of breath that resolved with aggressive diuresis. No neurologic or renal sequelae were noted. She was discharged on postoperative day 12 on erythropoietin.
Patient 2 A 70-year-old female Jehovah’s Witness with known aortic and mitral insufficiency was admitted for progressive dyspnea. Her past cardiac surgical history included coronary artery bypass grafting 4 years prior, mitral valve repair 3 years prior, and pacemaker implantation with AV nodal ablation for poorly controlled chronic atrial
Ann Thorac Surg 2004;78:695–7
fibrillation 9 months before the current admission. She also had experienced at least five cerebrovascular accidents during the past several years. Other medical comorbidities included hypertension, diabetes, hypercholesterolemia, and peptic ulcer disease. The patient had been deemed a prohibitive surgical risk at multiple institutions. Unfortunately, the rapidly progressive nature of her heart failure necessitated surgical intervention, and it was decided to proceed with aortic valve replacement, mitral valve replacement, and tricuspid annuloplasty. The standard pharmacologic and technical measures were applied. The operation proceeded uneventfully until protamine administration, at which point severe distention of the right atrium with concomitant right heart failure consistent with protamine reaction developed in the patient. After reheparinization, cardiopulmonary bypass was reinitiated, and after the administration of multiple inotropic agents the patient was ultimately weaned from bypass with near baseline ventricular function. Reversal of systemic heparinization was accomplished by slowly administering protamine through the left atrial catheter. The preoperative hematocrit of 45% had fallen to 34% on postoperative evaluation. The patient was transferred from the cardiac intensive care unit 8 days later with stable hematocrit, intact renal function, and no new clinically evident neurologic deficits. On postoperative day 12 she was discharged to a subacute care facility.
Patient 3 A 45-year-old church elder in the Jehovah’s Witnesses was transferred emergently with acute ascending aortic dissection, aortic insufficiency, and an expanding pericardial effusion with early tamponade. The patient arrived in extremis but was sufficiently capacitated to confirm both his consent to surgical treatment and his refusal of any transfusion of blood products. Before sternotomy the right femoral artery was expeditiously cannulated. Once the mediastinum was exposed, an adventitial tear at the root of the aorta was identified as the origin of the tamponade. The defect was controlled with digital pressure while the ascending aorta was dissected free. In this circumstance, we elected to proceed with aortic root replacement rather than a more protracted valve salvage procedure as described by David [5]. After the valve and ascending aorta were resected and sized, the patient was placed in deep Trendelenburg position and anterograde cerebral perfusion was initiated with balloon-tip perfusion catheters. During a 12-minute antegrade cerebral perfusion period the ascending aorta and proximal arch were resected back until normal tissue was revealed at the origin of the innominate artery, and a polyethylene terephthalate fiber (Dacron; Intervascular, Montvale, NJ) graft was placed. Total body perfusion was then reestablished. The remaining anastomoses were performed and reinforced with BioGlue (Cryolife Inc, Kennesaw, GA), air was removed from the heart, and the aortic cross-clamp was removed. The patient was extubated 43 hours postoperatively and spent 4 days in the cardiac intensive care unit. The preoperative hematocrit of 42% had fallen to 34%. Al-
CASE REPORT PIKE ET AL PULMONARY ARTERIOVENOUS MALFORMATION REGRESSION
though the patient experienced minor elevations in serum creatinine, wavering hematocrit, and transient fever while hospitalized, all resolved without further complication, and the patient was discharged on postoperative day 20.
Patient 4 A 48-year-old male Jehovah’s Witness with chronic anemia, renal insufficiency, and hypertension had been receiving erythropoietin in preparation for elective repair of a 7-cm descending thoracic aneurysm. He presented to an outside hospital with severe chest and back pain and was transferred emergently to our institution. On arrival to the operating room, a standard left posterolateral thoracotomy incision revealed no rupture, but rather what appeared to be an acute expansion of the aneurysm that had led to the patient’s symptoms. Although plaque was noted at the proximal extent of the aneurysm between the subclavian and carotid arteries, adequate control between these vessels was achieved. The subclavian artery was clamped separately, as was the thoracic aorta at the distal extent of what was discovered intraoperatively to be a type III dissection. The remainder of the operation was uncomplicated. The patient’s preoperative hematocrit of 39% had fallen to 28%. During postsurgical hospitalization the only complication encountered was significant hypertension that was difficult to control. No deterioration in renal function was encountered, nor were any obvious neurologic defects noted. The patient was discharged home on postoperative day number 7.
Comment There is considerable experience with elective coronary surgery with blood conservation [6 – 8]; however, more complex surgeries are less common. These cases demonstrate that even complex problems such as protamine reaction, acute dissection of the aorta, multiple redo sternotomy, or emergent aneurysm repair, do not pose insurmountable problems in Jehovah’s Witnesses. Appropriate preoperative planning and subsequent management make it possible to offer these patients complex operations.
References 1. Murkin JM. Attenuation of neurologic injury during cardiac surgery. Ann Thorac Surg 2001;72(Suppl):S1838 –44. 2. Rosengart TK, Helm RE, Klemperer J, Krieger KH, Isom OW. Combined aprotinin and erythropoietin use for blood conservation: results with Jehovah’s Witnesses. Ann Thorac Surg 1994;58:1397–403. 3. Rosengart TK. Cardiac surgery in Jehovah’s Witnesses. In: Krieger KH, Isom OW, eds. Blood conservation in cardiac surgery. New York: Springer, 1998:159 –68. 4. Malan TP Jr, Whitmore J, Maddi R. Reoperative cardiac surgery in a Jehovah’s Witness: role of continuous cell salvage and in line reinfusion. J Cardiothorac Anesth 1989;3:211–4. 5. David TE. Aortic valve-sparing operations for aortic root aneurysms. Semin Thorac Cardiovasc Surg. 2001 Jul;13:291– 6. 6. Ott DA, Cooley DA. Cardiovascular surgery in Jehovah’s Witnesses: report of 542 operations without blood transfusion. JAMA 1977;238:1256 –8. 7. Helm RE, Rosengart TK, Gomez M, et al. Comprehensive © 2004 by The Society of Thoracic Surgeons Published by Elsevier Inc
697
multimodality blood conservation: 100 consecutive CABG operations without transfusion. Ann Thorac Surg 1998;65:125– 36. 8. Aldea GS, Shapira OM, Treanor PR, Lazar HL, Shemin RJ. Effective use of heparin-bonded circuits and lower anticoagulation for coronary artery bypass grafting in Jehovah’s Witnesses. J Card Surg 1996;11:12–7.
Regression of Severe Pulmonary Arteriovenous Malformations After Fontan Revision and “Hepatic Factor” Rerouting Nancy A. Pike, RN, FNP, Luca A. Vricella, MD, Jeffrey A. Feinstein, MD, Michael D. Black, MD, and Bruce A. Reitz, MD Division of Cardiothoracic Surgery, David Geffen School of Medicine at University of California Los Angeles, Los Angeles, California, and Division of Cardiothoracic Surgery, Pediatric Cardiology, Lucile Packard Children’s Hospital at Stanford, Stanford University School of Medicine, Stanford, California
Although previously described in patients undergoing staged palliation for univentricular heart disease, the mechanism by which hepatic venous flow prevents development of pulmonary arteriovenous malformations is still not completely understood. We present a case in which successful H-type Fontan revision with rerouting of hepatic venous flow through a hemiazygous vein successfully reversed the progression of severe left pulmonary arteriovenous malformations. (Ann Thorac Surg 2004;78:697–9) © 2004 by The Society of Thoracic Surgeons
P
ulmonary venous malformations (AVM) have been known to develop after a classic or bidirectional Glenn anastomosis owing to the exclusion of hepatic venous blood or “hepatic factor” to the lungs. This malformation can develop in one or both lungs if preferential blood flow is present. Surgical palliation to incorporate the inferior vena cava and hepatic veins into the pulmonary circulation can vary depending on the single ventricle anatomy. The patient is a 6-year-old girl born with left dominant atrioventricular canal, situs inversus, and inferior vena cava (IVC) interruption with hemiazygous continuation to the left superior vena cava (SVC). The hepatic vein confluence drained into the common atrium. The child underwent pulmonary artery banding as a newborn. At 6 months of age, a left-sided bidirectional Glenn anastomosis was performed, and the pulmonary valve was oversewn. A nonfenestrated extracardiac Fontan procedure was performed at 4 years of age utilizing a 16-mm Gore-Tex conduit (W.L. Gore, Flagstaff, AZ), connecting
Accepted for publication Oct 2, 2003. Address reprint requests to Ms Pike, Cardiothoracic Surgery, David Geffen School of Medicine, UCLA, Box 951741, Los Angeles, CA 900951741; e-mail: [email protected].
0003-4975/04/$30.00 doi:10.1016/j.athoracsur.2004.02.003
CASE REPORTS
Ann Thorac Surg 2004;78:697–9
695
References 1. Kato H, Sugimura T, Akagi T, et al. Long term consequences of Kawasaki disease: a 10- to 21-year follow-up study of 594 patients. Circulation 1996;94:1379 –85. 2. Yanagawa H, Yashiro M, Nakamura Y, Kawasaki T, Kato H. Results of 12 nationwide epidemiological incidence surveys of Kawasaki disease in Japan. Arch Pediatr Adolesc Med 1995; 149:779 –83. 3. Ng YM, Sung R, So LY, et al. A 5 years surveillance of Kawasaki Disease in Hong Kong-1999. Hong Kong Kawasaki Disease study group. Presented at the 10th Asian Congress of Pediatrics, March 26 –30, 1994, Taipai, Taiwan. 4. Takahashi K, Oharaseki T, Wakayama M, Shibuya K, Naoe S. Pathological study of ruptured coronary artery aneurysms of Kawasaki disease. Abstract presented at the 2nd World Congress of Pediatric cardiology and Cardiac Surgery, May 11–15, 1997, Honolulu, HI. Abstract 202. 5. Nakano H, Ueda K, Saito A, Nojima K. Repeated quantitative angiograms in coronary arterial aneurysm in Kawasaki Disease. Am J Cardiol 1985;56:846 –51. 6. Kitamuran S, Kameda Y, Seki T, et al. Long-term outcome of myocardial revascularization in patients with Kawasaki coronary artery disease. J Thorac Cardiovasc Surg 1994;107:663–74.
Jehovah’s Witnesses Requiring Complex Urgent Cardiothoracic Surgery Richard L. Holt, Tomas D. Martin, MD, Philip J. Hess, MD, Thomas M. Beaver, MD, and Charles T. Klodell, MD Division of Thoracic and Cardiovascular Surgery, University of Florida College of Medicine Health Science Center, Gainesville, Florida
Blood-sparing surgical technique and perioperative medical management allows complex surgery in Jehovah’s Witnesses. The authors review four cases of urgent cardiothoracic surgery performed on Jehovah’s Witnesses. No lasting sequelae associated with either bleeding or end-organ ischemia were noted. Cardiopulmonary bypass management, meticulous hemostatic operative technique, and pharmacologic support of hemostasis and erythropoiesis permit this complex surgery. (Ann Thorac Surg 2004;78:695–7) © 2004 by The Society of Thoracic Surgeons
Table 1. Diagnostic Features of Kawasaki Disease Fever of at least 5 days’ duration not responsive to antibiotics or antipyretic agents Presence of at least four of the following clinical features: 1) Polymorphous exanthem involving face, trunk, extremities 2) Bilateral conjunctivitis 3) Changes in the lips, strawberry tongue, erythema of oropharynx 4) Erythema of palms and soles, edema of hands and feet, followed by desquamation of fingers and toes 5) Cervical lymphadenopathy Exclusion of other diseases with similar findings
© 2004 by The Society of Thoracic Surgeons Published by Elsevier Inc
T
he Jehovah’s Witnesses, numbering greater than 5 million in the United States, refuse blood component transfusion as part of their religion. This stems from interpretation of biblical passages, not from antipathy toward medicine. The following cases are presented to demonstrate the various strategies used to permit comAccepted for publication July 30, 2003. Address reprint requests to Dr Klodell, Division of Thoracic and Cardiovascular Surgery, University of Florida College of Medicine, Health Science Center, PO Box 100286, Gainesville, FL 32610-0286; e-mail: [email protected].
0003-4975/04/$30.00 doi:10.1016/S0003-4975(03)01494-2
CASE REPORTS
corresponding incidence in Hong Kong is reported to be 30 per 100,000 children [3]. In the untreated group, coronary aneurysm occurs in about 20% of all cases, whereas coronary aneurysm develops in only 4% to 8% of children who receive IV gamma immunoglobulin [2]. Based on autopsy data, the incidence of coronary aneurysm rupture is reported to occur in 5% of cases [4]. Thrombosis of such coronary aneurysm results in myocardial infarction, the leading cause of death for these children [1]. Despite the generally accepted standard approach to the management of this disease, controversies persist regarding certain aspects of treatment. How should patients with giant coronary aneurysm be managed? Giant coronary aneurysms (defined as aneurysms with an internal luminal diameter of 8 mm or more) are much less likely to reduce in size and will often thrombose or rupture [5]. To us, pending aneurysmal rupture in spite of maximal medical therapy, as in this case, is a clear indication for surgery as ruptured giant coronary aneurysms carries very high mortality. Coronary artery bypass grafting for coronary artery stenosis, ventricular aneurysmectomy, valve replacement, and cardiac transplantation has all been described for the treatment of coronary ischemic sequelae of Kawasaki disease, but no clear recommendation is available for the optimal timing of surgical intervention during the acute phase. We believe, as highlighted in this case, that patients with enlarging coronary aneurysms should be monitored closely and early surgical intervention should be considered when repeated assessments demonstrate a progression of the size of the aneurysm despite maximal medical therapy. In this case we successfully saved a child’s life with coronary bypass grafting using venous graft alone. Although the published data suggest that an arterial graft carries a better long-term patency rate and grows with the child [6], we chose the long saphenous vein because the anastomosis site for the posterior descending artery was very distal and required a long graft that could only be provided safely by the long saphenous vein in such an emergency situation.
CASE REPORT HOLT ET AL JEHOVAH’S WITNESSES AND AORTIC SURGERY
696
CASE REPORT HOLT ET AL JEHOVAH’S WITNESSES AND AORTIC SURGERY
CASE REPORTS
plex cardiothoracic surgery in Jehovah’s Witnesses. Perioperative pharmacologic management of erythrocyte mass and coagulation is of paramount importance. Aprotinin (Trasylol; Bayer Corp, West Haven, CT) has proven effective not only in reducing transfusion requirement, but also may counteract bypass-related inflammatory response [1]. The full Hammersmith regimen of 6 million total KIU may be considered acceptable, if not standard, in Jehovah’s Witness patients [2]. Technically, fastidious hemostatic operative technique, the limited use of laparotomy pads and discard suction, and reduced blood sampling for laboratory tests minimizes “lost” blood. The use of small-volume oxygenators and small-diameter tubing, retrograde autologous priming, and intraoperative autologous donation has become fairly standard [3]. Reclamation of autologous blood loss and infusion by means of continuous circuit of salvaged cells also aid in maintaining intraoperative hematocrit [4]. The liberal use of ultrafiltration limits hypervolemic dilutional anemia, and aids in the preservation of hemostatic blood components. The patients in the following cases all agreed to intraoperative hemodilution as well as the reinfusion of blood from a cell-saving device.
Case Reports Patient 1 A 38-year-old female Jehovah’s Witness with a 6-week history of shortness of breath was noted to have evolving heart failure secondary to left-sided Streptococcus viridans endocarditis. Antibiotic therapy had been initiated 3 weeks before referral, to which regimen erythropoietin and iron were added. Echocardiogram revealed a markedly regurgitant aortic valve and a flail anterior mitral leaflet. Medical management was continued to permit optimization of red blood cell mass before surgery. The patient’s hematocrit increased from 25% on admission to 39% during 2 weeks of high-dose erythropoietin (200 U/kg daily) and iron supplementation. Intraoperative blood conservation consisted of retrograde autologous priming, intraoperative autologous transfusion, and ultrafiltration. A total bypass time of 155 minutes with a cardiac ischemic time of 110 minutes was required. After closing, her hematocrit had fallen to 18%, and there was some suspicion of continued mediastinal bleeding; therefore the surgical site was reexplored to exclude the possibility of even minimal bleeding. No bleeding was identified, and complete hemostasis was verified before reclosure. The patient experienced no postoperative complications other than edema and shortness of breath that resolved with aggressive diuresis. No neurologic or renal sequelae were noted. She was discharged on postoperative day 12 on erythropoietin.
Patient 2 A 70-year-old female Jehovah’s Witness with known aortic and mitral insufficiency was admitted for progressive dyspnea. Her past cardiac surgical history included coronary artery bypass grafting 4 years prior, mitral valve repair 3 years prior, and pacemaker implantation with AV nodal ablation for poorly controlled chronic atrial
Ann Thorac Surg 2004;78:695–7
fibrillation 9 months before the current admission. She also had experienced at least five cerebrovascular accidents during the past several years. Other medical comorbidities included hypertension, diabetes, hypercholesterolemia, and peptic ulcer disease. The patient had been deemed a prohibitive surgical risk at multiple institutions. Unfortunately, the rapidly progressive nature of her heart failure necessitated surgical intervention, and it was decided to proceed with aortic valve replacement, mitral valve replacement, and tricuspid annuloplasty. The standard pharmacologic and technical measures were applied. The operation proceeded uneventfully until protamine administration, at which point severe distention of the right atrium with concomitant right heart failure consistent with protamine reaction developed in the patient. After reheparinization, cardiopulmonary bypass was reinitiated, and after the administration of multiple inotropic agents the patient was ultimately weaned from bypass with near baseline ventricular function. Reversal of systemic heparinization was accomplished by slowly administering protamine through the left atrial catheter. The preoperative hematocrit of 45% had fallen to 34% on postoperative evaluation. The patient was transferred from the cardiac intensive care unit 8 days later with stable hematocrit, intact renal function, and no new clinically evident neurologic deficits. On postoperative day 12 she was discharged to a subacute care facility.
Patient 3 A 45-year-old church elder in the Jehovah’s Witnesses was transferred emergently with acute ascending aortic dissection, aortic insufficiency, and an expanding pericardial effusion with early tamponade. The patient arrived in extremis but was sufficiently capacitated to confirm both his consent to surgical treatment and his refusal of any transfusion of blood products. Before sternotomy the right femoral artery was expeditiously cannulated. Once the mediastinum was exposed, an adventitial tear at the root of the aorta was identified as the origin of the tamponade. The defect was controlled with digital pressure while the ascending aorta was dissected free. In this circumstance, we elected to proceed with aortic root replacement rather than a more protracted valve salvage procedure as described by David [5]. After the valve and ascending aorta were resected and sized, the patient was placed in deep Trendelenburg position and anterograde cerebral perfusion was initiated with balloon-tip perfusion catheters. During a 12-minute antegrade cerebral perfusion period the ascending aorta and proximal arch were resected back until normal tissue was revealed at the origin of the innominate artery, and a polyethylene terephthalate fiber (Dacron; Intervascular, Montvale, NJ) graft was placed. Total body perfusion was then reestablished. The remaining anastomoses were performed and reinforced with BioGlue (Cryolife Inc, Kennesaw, GA), air was removed from the heart, and the aortic cross-clamp was removed. The patient was extubated 43 hours postoperatively and spent 4 days in the cardiac intensive care unit. The preoperative hematocrit of 42% had fallen to 34%. Al-
CASE REPORT PIKE ET AL PULMONARY ARTERIOVENOUS MALFORMATION REGRESSION
though the patient experienced minor elevations in serum creatinine, wavering hematocrit, and transient fever while hospitalized, all resolved without further complication, and the patient was discharged on postoperative day 20.
Patient 4 A 48-year-old male Jehovah’s Witness with chronic anemia, renal insufficiency, and hypertension had been receiving erythropoietin in preparation for elective repair of a 7-cm descending thoracic aneurysm. He presented to an outside hospital with severe chest and back pain and was transferred emergently to our institution. On arrival to the operating room, a standard left posterolateral thoracotomy incision revealed no rupture, but rather what appeared to be an acute expansion of the aneurysm that had led to the patient’s symptoms. Although plaque was noted at the proximal extent of the aneurysm between the subclavian and carotid arteries, adequate control between these vessels was achieved. The subclavian artery was clamped separately, as was the thoracic aorta at the distal extent of what was discovered intraoperatively to be a type III dissection. The remainder of the operation was uncomplicated. The patient’s preoperative hematocrit of 39% had fallen to 28%. During postsurgical hospitalization the only complication encountered was significant hypertension that was difficult to control. No deterioration in renal function was encountered, nor were any obvious neurologic defects noted. The patient was discharged home on postoperative day number 7.
Comment There is considerable experience with elective coronary surgery with blood conservation [6 – 8]; however, more complex surgeries are less common. These cases demonstrate that even complex problems such as protamine reaction, acute dissection of the aorta, multiple redo sternotomy, or emergent aneurysm repair, do not pose insurmountable problems in Jehovah’s Witnesses. Appropriate preoperative planning and subsequent management make it possible to offer these patients complex operations.
References 1. Murkin JM. Attenuation of neurologic injury during cardiac surgery. Ann Thorac Surg 2001;72(Suppl):S1838 –44. 2. Rosengart TK, Helm RE, Klemperer J, Krieger KH, Isom OW. Combined aprotinin and erythropoietin use for blood conservation: results with Jehovah’s Witnesses. Ann Thorac Surg 1994;58:1397–403. 3. Rosengart TK. Cardiac surgery in Jehovah’s Witnesses. In: Krieger KH, Isom OW, eds. Blood conservation in cardiac surgery. New York: Springer, 1998:159 –68. 4. Malan TP Jr, Whitmore J, Maddi R. Reoperative cardiac surgery in a Jehovah’s Witness: role of continuous cell salvage and in line reinfusion. J Cardiothorac Anesth 1989;3:211–4. 5. David TE. Aortic valve-sparing operations for aortic root aneurysms. Semin Thorac Cardiovasc Surg. 2001 Jul;13:291– 6. 6. Ott DA, Cooley DA. Cardiovascular surgery in Jehovah’s Witnesses: report of 542 operations without blood transfusion. JAMA 1977;238:1256 –8. 7. Helm RE, Rosengart TK, Gomez M, et al. Comprehensive © 2004 by The Society of Thoracic Surgeons Published by Elsevier Inc
697
multimodality blood conservation: 100 consecutive CABG operations without transfusion. Ann Thorac Surg 1998;65:125– 36. 8. Aldea GS, Shapira OM, Treanor PR, Lazar HL, Shemin RJ. Effective use of heparin-bonded circuits and lower anticoagulation for coronary artery bypass grafting in Jehovah’s Witnesses. J Card Surg 1996;11:12–7.
Regression of Severe Pulmonary Arteriovenous Malformations After Fontan Revision and “Hepatic Factor” Rerouting Nancy A. Pike, RN, FNP, Luca A. Vricella, MD, Jeffrey A. Feinstein, MD, Michael D. Black, MD, and Bruce A. Reitz, MD Division of Cardiothoracic Surgery, David Geffen School of Medicine at University of California Los Angeles, Los Angeles, California, and Division of Cardiothoracic Surgery, Pediatric Cardiology, Lucile Packard Children’s Hospital at Stanford, Stanford University School of Medicine, Stanford, California
Although previously described in patients undergoing staged palliation for univentricular heart disease, the mechanism by which hepatic venous flow prevents development of pulmonary arteriovenous malformations is still not completely understood. We present a case in which successful H-type Fontan revision with rerouting of hepatic venous flow through a hemiazygous vein successfully reversed the progression of severe left pulmonary arteriovenous malformations. (Ann Thorac Surg 2004;78:697–9) © 2004 by The Society of Thoracic Surgeons
P
ulmonary venous malformations (AVM) have been known to develop after a classic or bidirectional Glenn anastomosis owing to the exclusion of hepatic venous blood or “hepatic factor” to the lungs. This malformation can develop in one or both lungs if preferential blood flow is present. Surgical palliation to incorporate the inferior vena cava and hepatic veins into the pulmonary circulation can vary depending on the single ventricle anatomy. The patient is a 6-year-old girl born with left dominant atrioventricular canal, situs inversus, and inferior vena cava (IVC) interruption with hemiazygous continuation to the left superior vena cava (SVC). The hepatic vein confluence drained into the common atrium. The child underwent pulmonary artery banding as a newborn. At 6 months of age, a left-sided bidirectional Glenn anastomosis was performed, and the pulmonary valve was oversewn. A nonfenestrated extracardiac Fontan procedure was performed at 4 years of age utilizing a 16-mm Gore-Tex conduit (W.L. Gore, Flagstaff, AZ), connecting
Accepted for publication Oct 2, 2003. Address reprint requests to Ms Pike, Cardiothoracic Surgery, David Geffen School of Medicine, UCLA, Box 951741, Los Angeles, CA 900951741; e-mail: [email protected].
0003-4975/04/$30.00 doi:10.1016/j.athoracsur.2004.02.003
CASE REPORTS
Ann Thorac Surg 2004;78:697–9