CASE REPORTS
Heart Transplantation After Longest-Term Support With Ventricular Assist Devices Roland Hetzer, MD, PhD, Oliver Miera, MD, Joachim Photiadis, MD, PhD, Edwald Hennig, PhD, Christoph Knosalla, MD, PhD, and Eva Maria Delmo Walter, MD, PhD Departments of Cardiothoracic and Vascular Surgery, Pediatric Cardiology and Congenital Heart Diseases, and Pediatric Cardiac Surgery, Deutsches Herzzentrum, Berlin, Germany
The use of mechanical circulatory support devices to keep patients alive until transplantation has become essential in the face of an increasing organ shortage. We report successful heart transplantations after 841 days of left ventricular assist device (LVAD) support in a child with hypoplastic left heart syndrome, and after 547 days of biventricular assist device (BVAD) support in another child with cardiomyopathy. To our knowledge, this report is the first on the longest-term (841 days) LVAD and the longest-term (547 days) BVAD support in children who were mobile and awake during the support, as a most effective bridge to heart transplantation. (Ann Thorac Surg 2014;98:1814–5) Ó 2014 by The Society of Thoracic Surgeons FEATURE ARTICLES
M
echanical circulatory support as a bridge to transplantation was successfully introduced by our group in 1987 [1, 2]. The subsequent use of assist devices to keep patients alive until transplantation paved their way as an established treatment for end-stage heart failure. In 1990, we reported the first successful pediatric bridge to transplantation using a pulsatile paracorporeal left ventricular assist device (LVAD) in an 8-year-old boy [3]. Although he was supported with an adult-sized VAD, he was successfully bridged to heart transplantation. This experience prompted us to develop miniaturized pump systems for infants and children [2]. Further improvements [4] were made, and over a 20-year period we used the Berlin Heart EXCOR in children with profound heart failure as a bridge to transplantation or to myocardial recovery [5]. The longest duration of VAD support then was 420 days before transplantation became feasible. A 5-day-old boy with hypoplastic left heart syndrome, mitral valve dysplasia, and aortic atresia underwent a Norwood procedure. Balloon dilatation for aortic isthmus restenosis and stent implantation for shunt stenosis with coil embolization of the right aortopulmonary collaterals were performed 5 months later. A Glenn procedure was performed 1.6 years afterward. He was readmitted 1 year after (then 2.6 years old; weight, 11.8 kg; BSA, Accepted for publication Dec 2, 2013. Address correspondence to Dr Delmo Walter, Deutsches Herzzentrum Berlin, Augustenburger Platz 1, 13353 Berlin, Germany; e-mail: [email protected].
Ó 2014 by The Society of Thoracic Surgeons Published by Elsevier
0.57 kg/m2) because of persistent low cardiac output refractory to maximal medical therapy. A 25-mL Berlin Heart Excor Pediatric device was implanted with systemic ventricular and aortic cannulation as a bridge to transplantation. On postimplantation days 9 and 12, despite strict adherence to an anticoagulation protocol, thrombus formation in the systemic ventricle cannula necessitated pump exchanges. Despite a four-cycle regimen of fibrinolysis, further thrombus formation in the inflow cannula and in the cardiac chamber persisted. With worsening of pump function and deterioration of hemodynamic status, intracavitary thrombectomy and thrombus evacuation in the systemic ventricle cannula was deemed necessary. The thickened systemic ventricular myocardium was causing ventricular outflow tract obstruction, and myectomy was performed. Postoperatively, he exhibited decreased motor activity of the left upper arm. Computed tomography showed intracerebral hemorrhage. There had been no further pump problems until it was exchanged to a 30-mL pump to accommodate his somatic growth and hemodynamic demands 2 years later. He used an LVAD for 841 days until a suitable donor heart became available. Orthotopic heart transplantation (OHT) was performed with aortic and bicaval cannulation. Extensive adhesions from previous operations and scarring around the cannulae were dissected. The brachiocephalic trunk and both venae cavae were cannulated. The heart with the assist device cannulae was freed from the residual adhesions under cardiopulmonary bypass (CPB). Because of uncontrollable bleeding at the arterial cannulation site during preparation of the neoaorta, deep hypothermic circulatory arrest for 56 minutes was deemed necessary. At 16 C under cardiopulmonary bypass, after exposure of the aortic arch, and a long incision in the pulmonary artery made, with localization of the four pulmonary veins, the native heart was excised. The donor heart (ischemic time of 300 minutes) was implanted with left atrial anastomosis and end-to-end anastomosis with the inferior vena cava. The inferior vena caval cannula was transferred to the donor heart. Anastomoses to the recipient pulmonary artery and ascending aorta were performed, and the aortic cannula was transferred to the donor aorta. After right pulmonary artery reconstruction, an end-to-end anastomosis of the superior vena cava to the donor heart was done. Upon reaching systemic temperature, the heart beat in sinus rhythm, and the child was weaned off CPB. He was transferred to the intensive care unit in stable condition. He remained well without neurologic sequelae, and he went home on the 35th day. The second patient was a 14-month old boy (77 cm long, weighing 9 kg, BSA of 0.43 kg/cm2, with acute decompensated dilated cardiomyopathy and a left ventricular ejection fraction of 15%. A 25-mL Berlin Heart Excor LVAD was implanted. Weaning off CPB failed because of high pulmonary vascular resistance and right ventricular (RV) failure. An RV assist device (RVAD; Levitronix, Levitronix GmbH, Switzerland) was subsequently implanted. With improvement of RV function, an 0003-4975/$36.00 http://dx.doi.org/10.1016/j.athoracsur.2013.12.052
Ann Thorac Surg 2014;98:1815–8
Comment The optimal strategy for bridging children with failing single ventricle is by VAD support, which is appropriate for long-term assistance, given the shortage of donor organs. ECMO could certainly have improved hypoxemia and low cardiac output, albeit only short-term. VAD allowed a more effective problem being ventricular failure. The parallel circulation provided by the systemic ventricular inflow and aortic outflow cannulation requires increased pump flow rates (200 mL/kg/min) to meet the needs of pulmonary and systemic perfusion and maintain adequate oxygenation, in a patient with a single ventricle physiology and shunt-dependent circulation. The Glenn physiology inherently uploads the hepatic, renal, and splanchnic circulations by reducing venous hypertension, obviating the risk of multiorgan failure. In long-term VAD support, a hemorrhagic complication is obviously related to systemic anticoagulation, and to the fact that assisted cannulae prevent surface contact of the heart to other intrathoracic structures. Although heparin coating of the blood pumps and cannulae has significantly reduced the density and severity of thrombotic deposits, it has not completely resolved these problems. Likewise, repeated thrombi formation was Ó 2014 by The Society of Thoracic Surgeons Published by Elsevier
1815
presumably caused by liver dysfunction and coagulation factor alterations. The second patient had far fewer thrombotic complications, despite having two VADs, mainly because the etiology of heart failure is of a different mechanism and physiology than that of the first patient. Episodes of neurologic injuries were particularly frustrating because they occurred in the setting of full anticoagulation and antiplatelet therapy. We surmised that these morbidities might have occurred and subsequently healed because of the plasticity of the juvenile central nervous system. Precautions must be taken to avoid injury to the inflow and outflow cannulae while dissecting off extensive adhesions in preparation for excision of the native heart. Transplantation techniques are decided preoperatively based on the underlying etiology of the ventricular failure. Based on our experience, ischemic time of the donor heart of up to 300 minutes is well tolerated. Given the shortage of donor organs, this report emphasizes that mechanical circulatory support with a ventricular assist device is a feasible option for long-term therapy.
References 1. Hetzer R, Hennig E, Schiessler A, Friedel N, Warnecke H, Adt M. Mechanical circulatory support and heart transplantation. J Heart Lung Transplant 1992;11:S175–81. 2. Hennig E. Design criteria for pediatric mechanical circulatory support systems (PMCSS). In: Ferrazzi P, Parenzan L, editors. Annals of the concerted action HEART 1991-1992. Commission of the European Communities, Bergamo (Italy); 1991; 39–43. 3. Warnecke H, Berdjis F, Hennig E, et al. Mechanical left ventricular support as a bridge to transplantation in childhood. Eur J Cardiothorac Surg 1991;5:330–3. 4. Hetzer R, Potapov EV, Stiller B, et al. Improvement in survival after mechanical circulatory support with pneumatic pulsatile ventricular assist devices in pediatric patients. Ann Thorac Surg 2006;82:917–24. 5. Hetzer R, Alexi-Meskishvili V, Weng Y, et al. Mechanical cardiac support in the young with the Berlin Heart EXCOR pulsatile ventricular assist device: 15 years’ experience. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2006;9: 99–108.
Giant Right Atrium: An Extreme Case of Idiopathic Dilation of the Right Atrium Michael A. Long, MMed Department of Cardiothoracic Surgery, Free State University, Bloemfontein, South Africa
An adult with a grossly dilated right atrium of unknown etiology is presented. The right atrial volume was Accepted for publication Dec 30, 2013. Address correspondence to Dr Long, Department of Cardiothoracic Surgery, Faculty of Health Sciences, University of the Free State, PO Box 339, Bloemfontein 9300, South Africa; e-mail: [email protected].
0003-4975/$36.00 http://dx.doi.org/10.1016/j.athoracsur.2013.12.082
FEATURE ARTICLES
RVAD was explanted 5 days later. Frequent episodes of tachycardia and evidence of RV failure prompted the implantation of a 25-mL Berlin Heart Excor RVAD. Thrombin coating the pump prompted its exchange on the 17th postoperative day, and he was listed for highurgency heart transplantation. On the 19th day after LVAD implantation day, he exhibited right eye deviation to the left and left upper arm hemiparesis. Computed tomography showed right medial parietooccipital area infarction. He was extubated on the 30th postoperative day. He remained stable with a BVAD for 547 days, until a suitable donor heart came. After dissecting off the extensive adhesions, the BVAD cannulae were isolated, and residual adhesions were dissected off under CPB. Upon clamping the aorta, the assist cannula was divided and the heart was excised. The donor heart (ischemic time of 279 minutes) was anastomosed biatrially, followed by anastomoses of the pulmonary artery and aorta. During rewarming and reperfusion, he required high doses of catecholamines. In his immediate postoperative course, an extracoporeal membrane oxygenator (ECMO) was implanted, which stabilized the child until its explantation 7 days later. On the 28th day after OHT, an acute cellular and humoral rejection occurred; it was managed successfully with daily plasmapheresis for 4 days. He was eventually discharged home. Both children received continuous low-molecular-weight heparin with anti-Xa activity monitoring. After starting oral feeding and chest tube removal, acetylsalicylic acid (0.5 to 3 mg/kg/day) and dipyridamole (2 to 15 mg/kg/day) were started. Platelet aggregation tests were performed weekly, with a target activation of 30%.
CASE REPORT LONG GIANT RIGHT ATRIUM
Heart Transplantation After Longest-Term Support With Ventricular Assist Devices Roland Hetzer, MD, PhD, Oliver Miera, MD, Joachim Photiadis, MD, PhD, Edwald Hennig, PhD, Christoph Knosalla, MD, PhD, and Eva Maria Delmo Walter, MD, PhD Departments of Cardiothoracic and Vascular Surgery, Pediatric Cardiology and Congenital Heart Diseases, and Pediatric Cardiac Surgery, Deutsches Herzzentrum, Berlin, Germany
The use of mechanical circulatory support devices to keep patients alive until transplantation has become essential in the face of an increasing organ shortage. We report successful heart transplantations after 841 days of left ventricular assist device (LVAD) support in a child with hypoplastic left heart syndrome, and after 547 days of biventricular assist device (BVAD) support in another child with cardiomyopathy. To our knowledge, this report is the first on the longest-term (841 days) LVAD and the longest-term (547 days) BVAD support in children who were mobile and awake during the support, as a most effective bridge to heart transplantation. (Ann Thorac Surg 2014;98:1814–5) Ó 2014 by The Society of Thoracic Surgeons FEATURE ARTICLES
M
echanical circulatory support as a bridge to transplantation was successfully introduced by our group in 1987 [1, 2]. The subsequent use of assist devices to keep patients alive until transplantation paved their way as an established treatment for end-stage heart failure. In 1990, we reported the first successful pediatric bridge to transplantation using a pulsatile paracorporeal left ventricular assist device (LVAD) in an 8-year-old boy [3]. Although he was supported with an adult-sized VAD, he was successfully bridged to heart transplantation. This experience prompted us to develop miniaturized pump systems for infants and children [2]. Further improvements [4] were made, and over a 20-year period we used the Berlin Heart EXCOR in children with profound heart failure as a bridge to transplantation or to myocardial recovery [5]. The longest duration of VAD support then was 420 days before transplantation became feasible. A 5-day-old boy with hypoplastic left heart syndrome, mitral valve dysplasia, and aortic atresia underwent a Norwood procedure. Balloon dilatation for aortic isthmus restenosis and stent implantation for shunt stenosis with coil embolization of the right aortopulmonary collaterals were performed 5 months later. A Glenn procedure was performed 1.6 years afterward. He was readmitted 1 year after (then 2.6 years old; weight, 11.8 kg; BSA, Accepted for publication Dec 2, 2013. Address correspondence to Dr Delmo Walter, Deutsches Herzzentrum Berlin, Augustenburger Platz 1, 13353 Berlin, Germany; e-mail: [email protected].
Ó 2014 by The Society of Thoracic Surgeons Published by Elsevier
0.57 kg/m2) because of persistent low cardiac output refractory to maximal medical therapy. A 25-mL Berlin Heart Excor Pediatric device was implanted with systemic ventricular and aortic cannulation as a bridge to transplantation. On postimplantation days 9 and 12, despite strict adherence to an anticoagulation protocol, thrombus formation in the systemic ventricle cannula necessitated pump exchanges. Despite a four-cycle regimen of fibrinolysis, further thrombus formation in the inflow cannula and in the cardiac chamber persisted. With worsening of pump function and deterioration of hemodynamic status, intracavitary thrombectomy and thrombus evacuation in the systemic ventricle cannula was deemed necessary. The thickened systemic ventricular myocardium was causing ventricular outflow tract obstruction, and myectomy was performed. Postoperatively, he exhibited decreased motor activity of the left upper arm. Computed tomography showed intracerebral hemorrhage. There had been no further pump problems until it was exchanged to a 30-mL pump to accommodate his somatic growth and hemodynamic demands 2 years later. He used an LVAD for 841 days until a suitable donor heart became available. Orthotopic heart transplantation (OHT) was performed with aortic and bicaval cannulation. Extensive adhesions from previous operations and scarring around the cannulae were dissected. The brachiocephalic trunk and both venae cavae were cannulated. The heart with the assist device cannulae was freed from the residual adhesions under cardiopulmonary bypass (CPB). Because of uncontrollable bleeding at the arterial cannulation site during preparation of the neoaorta, deep hypothermic circulatory arrest for 56 minutes was deemed necessary. At 16 C under cardiopulmonary bypass, after exposure of the aortic arch, and a long incision in the pulmonary artery made, with localization of the four pulmonary veins, the native heart was excised. The donor heart (ischemic time of 300 minutes) was implanted with left atrial anastomosis and end-to-end anastomosis with the inferior vena cava. The inferior vena caval cannula was transferred to the donor heart. Anastomoses to the recipient pulmonary artery and ascending aorta were performed, and the aortic cannula was transferred to the donor aorta. After right pulmonary artery reconstruction, an end-to-end anastomosis of the superior vena cava to the donor heart was done. Upon reaching systemic temperature, the heart beat in sinus rhythm, and the child was weaned off CPB. He was transferred to the intensive care unit in stable condition. He remained well without neurologic sequelae, and he went home on the 35th day. The second patient was a 14-month old boy (77 cm long, weighing 9 kg, BSA of 0.43 kg/cm2, with acute decompensated dilated cardiomyopathy and a left ventricular ejection fraction of 15%. A 25-mL Berlin Heart Excor LVAD was implanted. Weaning off CPB failed because of high pulmonary vascular resistance and right ventricular (RV) failure. An RV assist device (RVAD; Levitronix, Levitronix GmbH, Switzerland) was subsequently implanted. With improvement of RV function, an 0003-4975/$36.00 http://dx.doi.org/10.1016/j.athoracsur.2013.12.052
Ann Thorac Surg 2014;98:1815–8
Comment The optimal strategy for bridging children with failing single ventricle is by VAD support, which is appropriate for long-term assistance, given the shortage of donor organs. ECMO could certainly have improved hypoxemia and low cardiac output, albeit only short-term. VAD allowed a more effective problem being ventricular failure. The parallel circulation provided by the systemic ventricular inflow and aortic outflow cannulation requires increased pump flow rates (200 mL/kg/min) to meet the needs of pulmonary and systemic perfusion and maintain adequate oxygenation, in a patient with a single ventricle physiology and shunt-dependent circulation. The Glenn physiology inherently uploads the hepatic, renal, and splanchnic circulations by reducing venous hypertension, obviating the risk of multiorgan failure. In long-term VAD support, a hemorrhagic complication is obviously related to systemic anticoagulation, and to the fact that assisted cannulae prevent surface contact of the heart to other intrathoracic structures. Although heparin coating of the blood pumps and cannulae has significantly reduced the density and severity of thrombotic deposits, it has not completely resolved these problems. Likewise, repeated thrombi formation was Ó 2014 by The Society of Thoracic Surgeons Published by Elsevier
1815
presumably caused by liver dysfunction and coagulation factor alterations. The second patient had far fewer thrombotic complications, despite having two VADs, mainly because the etiology of heart failure is of a different mechanism and physiology than that of the first patient. Episodes of neurologic injuries were particularly frustrating because they occurred in the setting of full anticoagulation and antiplatelet therapy. We surmised that these morbidities might have occurred and subsequently healed because of the plasticity of the juvenile central nervous system. Precautions must be taken to avoid injury to the inflow and outflow cannulae while dissecting off extensive adhesions in preparation for excision of the native heart. Transplantation techniques are decided preoperatively based on the underlying etiology of the ventricular failure. Based on our experience, ischemic time of the donor heart of up to 300 minutes is well tolerated. Given the shortage of donor organs, this report emphasizes that mechanical circulatory support with a ventricular assist device is a feasible option for long-term therapy.
References 1. Hetzer R, Hennig E, Schiessler A, Friedel N, Warnecke H, Adt M. Mechanical circulatory support and heart transplantation. J Heart Lung Transplant 1992;11:S175–81. 2. Hennig E. Design criteria for pediatric mechanical circulatory support systems (PMCSS). In: Ferrazzi P, Parenzan L, editors. Annals of the concerted action HEART 1991-1992. Commission of the European Communities, Bergamo (Italy); 1991; 39–43. 3. Warnecke H, Berdjis F, Hennig E, et al. Mechanical left ventricular support as a bridge to transplantation in childhood. Eur J Cardiothorac Surg 1991;5:330–3. 4. Hetzer R, Potapov EV, Stiller B, et al. Improvement in survival after mechanical circulatory support with pneumatic pulsatile ventricular assist devices in pediatric patients. Ann Thorac Surg 2006;82:917–24. 5. Hetzer R, Alexi-Meskishvili V, Weng Y, et al. Mechanical cardiac support in the young with the Berlin Heart EXCOR pulsatile ventricular assist device: 15 years’ experience. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2006;9: 99–108.
Giant Right Atrium: An Extreme Case of Idiopathic Dilation of the Right Atrium Michael A. Long, MMed Department of Cardiothoracic Surgery, Free State University, Bloemfontein, South Africa
An adult with a grossly dilated right atrium of unknown etiology is presented. The right atrial volume was Accepted for publication Dec 30, 2013. Address correspondence to Dr Long, Department of Cardiothoracic Surgery, Faculty of Health Sciences, University of the Free State, PO Box 339, Bloemfontein 9300, South Africa; e-mail: [email protected].
0003-4975/$36.00 http://dx.doi.org/10.1016/j.athoracsur.2013.12.082
FEATURE ARTICLES
RVAD was explanted 5 days later. Frequent episodes of tachycardia and evidence of RV failure prompted the implantation of a 25-mL Berlin Heart Excor RVAD. Thrombin coating the pump prompted its exchange on the 17th postoperative day, and he was listed for highurgency heart transplantation. On the 19th day after LVAD implantation day, he exhibited right eye deviation to the left and left upper arm hemiparesis. Computed tomography showed right medial parietooccipital area infarction. He was extubated on the 30th postoperative day. He remained stable with a BVAD for 547 days, until a suitable donor heart came. After dissecting off the extensive adhesions, the BVAD cannulae were isolated, and residual adhesions were dissected off under CPB. Upon clamping the aorta, the assist cannula was divided and the heart was excised. The donor heart (ischemic time of 279 minutes) was anastomosed biatrially, followed by anastomoses of the pulmonary artery and aorta. During rewarming and reperfusion, he required high doses of catecholamines. In his immediate postoperative course, an extracoporeal membrane oxygenator (ECMO) was implanted, which stabilized the child until its explantation 7 days later. On the 28th day after OHT, an acute cellular and humoral rejection occurred; it was managed successfully with daily plasmapheresis for 4 days. He was eventually discharged home. Both children received continuous low-molecular-weight heparin with anti-Xa activity monitoring. After starting oral feeding and chest tube removal, acetylsalicylic acid (0.5 to 3 mg/kg/day) and dipyridamole (2 to 15 mg/kg/day) were started. Platelet aggregation tests were performed weekly, with a target activation of 30%.
CASE REPORT LONG GIANT RIGHT ATRIUM
