Successful explantation of ventricular assist device for systemic ventricular assistance in a patient with congenitally corrected transposit
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《血管的通路杂志》
Department of Cardiovascular Surgery, The Heart Institute of Japan, Tokyo Women's Medical University, 8-1, Kawada, Shinjuku, Tokyo 162-8666, Japan
Abstract
We report a case of a 13-year-old boy with congenitally corrected transposition of the great arteries after conventional repair who underwent an implantation of ventricular assist device (VAD) due to right (systemic) ventricular failure after tricuspid valve replacement. The anatomical right ventricle (systemic ventricle) was completely unloaded and the function improved over time under LVAD. He had an explantation of the VAD due to bacteremia 43 days after implantation, and his clinical condition improved significantly.
Key Words: Circulatory assist devices; Great vessel anomalies; Left ventricular assist device
1. Introduction
Anatomical right ventricular dysfunction as a systemic ventricle in patients with congenitally corrected transposition of the great arteries (cTGA), and in patients after Senning or Mustard operation for transposition of the great arteries (TGA), is a serious problem. Transplantation is still considered as a definitive therapy for refractory heart failure. Williams et al. reported that 10% of patients after the atrial switch repair for TGA had been found to have serious right ventricular dysfunction [1]. Siebenmann et al. described that systemic ventricular failure had been found in 16 out of 220 patients after atrial corrections of TGA and that heart transplantation had been performed in two patients out of the same patient group [2]. Carrel et al. showed that two patients had undergone orthotopic heart transplantation out of 189 operative survivors after the Mustard or Senning operation for TGA [3].
2. Case report
The patient was a 13-year-old boy who was diagnosed with double aortic arch, congenitally corrected transposition of the great arteries (cTGA), dextrocardia, ventricular septal defect (VSD), and supravalvar pulmonary stenosis at birth. He had undergone a division of the left aortic arch at 4 days of age, and patch closure of VSD, patch reconstruction of pulmonary artery, and mitral valve plasty at 7 years of age. Cardiac catheterization revealed tricuspid regurgitation (TR) grade III at the age of 9 years. He developed congestive heart failure due to severe right (systemic) ventricular dysfunction at 13 years of age, which resulted in the admission to our hospital.
On admission the patient's condition was assessed to be functional class II according to the New York Heart Association (NYHA) criteria. Chest X-ray showed a cardiothoracic ratio (CTR) of 73% with no congestion. Electrocardiogram revealed normal sinus rhythm, normal axis deviation, and no significant sign of myocardial damage. On the echocardiogram, right ventricular wall motion was severely reduced and TR was mild. Gated myocardial single-photon emission tomography (SPECT) revealed right ventricular end-diastolic volume (RVEDV) of 234 ml and right ventricular ejection fraction (RVEF) of 32%. Brain natriuretic peptide (BNP) was 537.2 pg/ml.
After admission, continuous infusion of milrinone was started. However, RVEDV and RVEF did not improve as assessed by Gated SPECT performed one month after admission. Despite treatment, congestive heart failure deteriorated even with administration of catecholamines. Two and a half months after admission, CTR and BNP increased to 88% and 1452 pg/ml, respectively. Medication was no longer thought to be effective and implantation of a ventricular assisted device (VAD) was recommended as a bridge to heart transplantation.
A median sternotomy was performed. After establishing extracorporeal circulation and clamping the ascending aorta, the heart was stopped using cold crystalloid cardioplegic solution. Tricuspid valve (systemic atrial-ventricular valve) replacement was performed with a Carpentier–Edwards bovine pericardial valve of 29 mm (Edwards Lifesciences LLC, Irvine, CA). He was weaned from cardiopulmonary bypass after 52 min of extracorporeal circulation. However, 8 min later, his hemodynamic state could not be maintained and a Toyobo VAD was implanted with insertion of an inflow cannula to the right ventricular apex and an outflow cannula to the ascending aorta.
The postoperative course was uneventful until high fever appeared on post-operative day (POD) 37. Then Methicillin-Resistant Staphylococcus aureus was isolated from blood culture. An on-off test of the VAD was performed to assess the possibility of weaning from the VAD. It showed borderline hemodynamics with inotropic support. Since it was concluded that there was no cure but explantation of the VAD, VAD explantation was performed on POD 43. The postoperative course was, fortunately, not complicated. Six months later, CTR, BNP, and RVEDV decreased to 65%, 115.9 pg/ml, and 145 ml, respectively. Decrease of valve regurgitation caused significant reduction of CTR and RVEDV; however, right ventricular function did not improve. He receives medical treatment with digoxin, diuretics, angiotensin receptor blocker, and -blocker. His functional status recovered to NYHA II.
3. Discussion
There have been nine reported cases of VAD implantation to support the right (systemic) ventricle in patients with TGA following atrial switch operation or in patients with cTGA [4–7] (Table 1). Five patients died during VAD support, one patient died after VAD explantation, and three patients underwent heart transplantation after VAD support. There have been no reports of successful explantation of VAD from patients with d-TGA following atrial switch operation or patients with cTGA who had an implantation of VAD to support the right ventricle.
Dandel et al. reported that 32 idiopathic dilated cardiomyopathy patients who had had VAD explanted showed a survival rate of 78.3%±8.1 at 5 years after VAD explantation [8]. Heart failure recurred during the first 3 years after weaning in 31.3%. Two patients died because of heart failure after weaning.
In this operation, we inserted an inflow cannula to the right ventricular apex, and an outflow cannula to the ascending aorta. The inflow cannula, unlike the usual arrangement, was placed to the right side of the outflow cannula, because the patient had dextrocardia. Therefore, the VAD pump was placed with a flipped-over position. However, this did not cause any problems.
In summary, VAD provided a good right ventricular support in a patient with cTGA and it provided even recovery from refractory right ventricular dysfunction. With a lack of donor hearts, supporting the right ventricle with VAD in patients with cTGA, or in patients after Senning or Mustard operation for TGA who have right ventricular failure, is feasible as a bridge to transplantation or as a heart failure therapy.
References
Williams WG, Trusler GA, Kirklin JW, Blackstone EH, Coles JG, Izukawa T, Freedom RM. Early and late results of a protocol for simple transposition leading to an atrial switch (Mustard) repair. J Thorac Cardiovasc Surg 1988; 95:717–726.
Siebenmann R, von Segesser L, Schneider K, Schneider J, Senning A, Turina M. Late failure of systemic ventricle after atrial correction for transposition of great arteries. Eur J Cardiothorac Surg 1989; 3:119–123.
Carrel T, Pfammatter JP. Complete transposition of the great arteries: surgical concepts for patients with systemic right ventricular failure following intraatrial repair. Thorac Cardiovasc Surg 2000; 48:224–227.
Okabayashi H, Hirose H, Ogino H, Jinno K, Hanada M, Nishimura K, Hirata K, Nomoto S, Okamoto Y, Ban T. Changes in the immune response in postoperative patients while running the ventricular assist device (VAD). Nippon Kyobu Geka Gakkai Zasshi 1990; 38:10–15.
Ishino K, Loebe M, Uhlemann F, Weng Y, Hennig E, Hetzer R. Circulatory support with paracorporeal pneumatic ventricular assist device (VAD) in infants and children. Eur J Cardiothorac Surg 1997; 11:965–972.
Reinhartz O, Keith FM, El-Banayosy A, McBride LR, Robbins RC, Copeland JG, Farrar DJ. Multicenter experience with the thoratec ventricular assist device in children and adolescents. J Heart Lung Transplant 2001; 20:439–448.
Stewart AS, Gorman RC, Pocchetino A, Rosengard BR, Acker MA. Left ventricular assist device for right side assistance in patients with transposition. Ann Thorac Surg 2002; 74:912–914.
Dandel M, Weng Y, Siniawski H, Potapov E, Lehmkuhl HB, Hetzer R. Long-term results in patients with idiopathic dilated cardiomyopathy after weaning from left ventricular assist devices. Circulation. 2005; 112:I37–I45.(Tadahisa Sugiura, Hiromi )
Abstract
We report a case of a 13-year-old boy with congenitally corrected transposition of the great arteries after conventional repair who underwent an implantation of ventricular assist device (VAD) due to right (systemic) ventricular failure after tricuspid valve replacement. The anatomical right ventricle (systemic ventricle) was completely unloaded and the function improved over time under LVAD. He had an explantation of the VAD due to bacteremia 43 days after implantation, and his clinical condition improved significantly.
Key Words: Circulatory assist devices; Great vessel anomalies; Left ventricular assist device
1. Introduction
Anatomical right ventricular dysfunction as a systemic ventricle in patients with congenitally corrected transposition of the great arteries (cTGA), and in patients after Senning or Mustard operation for transposition of the great arteries (TGA), is a serious problem. Transplantation is still considered as a definitive therapy for refractory heart failure. Williams et al. reported that 10% of patients after the atrial switch repair for TGA had been found to have serious right ventricular dysfunction [1]. Siebenmann et al. described that systemic ventricular failure had been found in 16 out of 220 patients after atrial corrections of TGA and that heart transplantation had been performed in two patients out of the same patient group [2]. Carrel et al. showed that two patients had undergone orthotopic heart transplantation out of 189 operative survivors after the Mustard or Senning operation for TGA [3].
2. Case report
The patient was a 13-year-old boy who was diagnosed with double aortic arch, congenitally corrected transposition of the great arteries (cTGA), dextrocardia, ventricular septal defect (VSD), and supravalvar pulmonary stenosis at birth. He had undergone a division of the left aortic arch at 4 days of age, and patch closure of VSD, patch reconstruction of pulmonary artery, and mitral valve plasty at 7 years of age. Cardiac catheterization revealed tricuspid regurgitation (TR) grade III at the age of 9 years. He developed congestive heart failure due to severe right (systemic) ventricular dysfunction at 13 years of age, which resulted in the admission to our hospital.
On admission the patient's condition was assessed to be functional class II according to the New York Heart Association (NYHA) criteria. Chest X-ray showed a cardiothoracic ratio (CTR) of 73% with no congestion. Electrocardiogram revealed normal sinus rhythm, normal axis deviation, and no significant sign of myocardial damage. On the echocardiogram, right ventricular wall motion was severely reduced and TR was mild. Gated myocardial single-photon emission tomography (SPECT) revealed right ventricular end-diastolic volume (RVEDV) of 234 ml and right ventricular ejection fraction (RVEF) of 32%. Brain natriuretic peptide (BNP) was 537.2 pg/ml.
After admission, continuous infusion of milrinone was started. However, RVEDV and RVEF did not improve as assessed by Gated SPECT performed one month after admission. Despite treatment, congestive heart failure deteriorated even with administration of catecholamines. Two and a half months after admission, CTR and BNP increased to 88% and 1452 pg/ml, respectively. Medication was no longer thought to be effective and implantation of a ventricular assisted device (VAD) was recommended as a bridge to heart transplantation.
A median sternotomy was performed. After establishing extracorporeal circulation and clamping the ascending aorta, the heart was stopped using cold crystalloid cardioplegic solution. Tricuspid valve (systemic atrial-ventricular valve) replacement was performed with a Carpentier–Edwards bovine pericardial valve of 29 mm (Edwards Lifesciences LLC, Irvine, CA). He was weaned from cardiopulmonary bypass after 52 min of extracorporeal circulation. However, 8 min later, his hemodynamic state could not be maintained and a Toyobo VAD was implanted with insertion of an inflow cannula to the right ventricular apex and an outflow cannula to the ascending aorta.
The postoperative course was uneventful until high fever appeared on post-operative day (POD) 37. Then Methicillin-Resistant Staphylococcus aureus was isolated from blood culture. An on-off test of the VAD was performed to assess the possibility of weaning from the VAD. It showed borderline hemodynamics with inotropic support. Since it was concluded that there was no cure but explantation of the VAD, VAD explantation was performed on POD 43. The postoperative course was, fortunately, not complicated. Six months later, CTR, BNP, and RVEDV decreased to 65%, 115.9 pg/ml, and 145 ml, respectively. Decrease of valve regurgitation caused significant reduction of CTR and RVEDV; however, right ventricular function did not improve. He receives medical treatment with digoxin, diuretics, angiotensin receptor blocker, and -blocker. His functional status recovered to NYHA II.
3. Discussion
There have been nine reported cases of VAD implantation to support the right (systemic) ventricle in patients with TGA following atrial switch operation or in patients with cTGA [4–7] (Table 1). Five patients died during VAD support, one patient died after VAD explantation, and three patients underwent heart transplantation after VAD support. There have been no reports of successful explantation of VAD from patients with d-TGA following atrial switch operation or patients with cTGA who had an implantation of VAD to support the right ventricle.
Dandel et al. reported that 32 idiopathic dilated cardiomyopathy patients who had had VAD explanted showed a survival rate of 78.3%±8.1 at 5 years after VAD explantation [8]. Heart failure recurred during the first 3 years after weaning in 31.3%. Two patients died because of heart failure after weaning.
In this operation, we inserted an inflow cannula to the right ventricular apex, and an outflow cannula to the ascending aorta. The inflow cannula, unlike the usual arrangement, was placed to the right side of the outflow cannula, because the patient had dextrocardia. Therefore, the VAD pump was placed with a flipped-over position. However, this did not cause any problems.
In summary, VAD provided a good right ventricular support in a patient with cTGA and it provided even recovery from refractory right ventricular dysfunction. With a lack of donor hearts, supporting the right ventricle with VAD in patients with cTGA, or in patients after Senning or Mustard operation for TGA who have right ventricular failure, is feasible as a bridge to transplantation or as a heart failure therapy.
References
Williams WG, Trusler GA, Kirklin JW, Blackstone EH, Coles JG, Izukawa T, Freedom RM. Early and late results of a protocol for simple transposition leading to an atrial switch (Mustard) repair. J Thorac Cardiovasc Surg 1988; 95:717–726.
Siebenmann R, von Segesser L, Schneider K, Schneider J, Senning A, Turina M. Late failure of systemic ventricle after atrial correction for transposition of great arteries. Eur J Cardiothorac Surg 1989; 3:119–123.
Carrel T, Pfammatter JP. Complete transposition of the great arteries: surgical concepts for patients with systemic right ventricular failure following intraatrial repair. Thorac Cardiovasc Surg 2000; 48:224–227.
Okabayashi H, Hirose H, Ogino H, Jinno K, Hanada M, Nishimura K, Hirata K, Nomoto S, Okamoto Y, Ban T. Changes in the immune response in postoperative patients while running the ventricular assist device (VAD). Nippon Kyobu Geka Gakkai Zasshi 1990; 38:10–15.
Ishino K, Loebe M, Uhlemann F, Weng Y, Hennig E, Hetzer R. Circulatory support with paracorporeal pneumatic ventricular assist device (VAD) in infants and children. Eur J Cardiothorac Surg 1997; 11:965–972.
Reinhartz O, Keith FM, El-Banayosy A, McBride LR, Robbins RC, Copeland JG, Farrar DJ. Multicenter experience with the thoratec ventricular assist device in children and adolescents. J Heart Lung Transplant 2001; 20:439–448.
Stewart AS, Gorman RC, Pocchetino A, Rosengard BR, Acker MA. Left ventricular assist device for right side assistance in patients with transposition. Ann Thorac Surg 2002; 74:912–914.
Dandel M, Weng Y, Siniawski H, Potapov E, Lehmkuhl HB, Hetzer R. Long-term results in patients with idiopathic dilated cardiomyopathy after weaning from left ventricular assist devices. Circulation. 2005; 112:I37–I45.(Tadahisa Sugiura, Hiromi )