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Use of the total artificial heart as a bridge to transplant in a 13-year-old with congenitally corrected transposition of the great arteries

2015; Elsevier BV; Volume: 151; Issue: 4 Linguagem: Inglês

10.1016/j.jtcvs.2015.11.049

1097-685X

Ming‐Sing Si, Francis D. Pagani, Jonathan W. Haft,

Mechanical Circulatory Support Devices

Central MessageThe total artificial heart can be used in children who have undergone a failed double switch for congenitally corrected transposition.See Editorial Commentary page e73. The total artificial heart can be used in children who have undergone a failed double switch for congenitally corrected transposition. See Editorial Commentary page e73. Without anatomic correction, the systemic right ventricle (RV) and tricuspid valve are prone to failure in patients with congenitally corrected transposition of the great arteries (CCTGA).1Wallis G.A. Debich-Spicer D. Anderson R.H. Congenitally corrected transposition.Orphanet J Rare Dis. 2011; 6: 22Crossref PubMed Scopus (78) Google Scholar Left ventricular function may also be depressed in these patients, resulting in biventricular failure.2Giardini A. Lovato L. Donti A. Formigari R. Oppido G. Gargiulo G. et al.Relation between right ventricular structural alterations and markers of adverse clinical outcome in adults with systemic right ventricle and either congenital complete (after Senning operation) or congenitally corrected transposition of the great arteries.Am J Cardiol. 2006; 98: 1277-1282Abstract Full Text Full Text PDF PubMed Scopus (95) Google Scholar A 13-year-old boy (body surface area 1.8 m2) with dextrocardia, {S,L,L} CCTGA, ventricular septal defect, pulmonary stenosis, Ebsteinoid tricuspid valve with severe regurgitation, and pulmonary hypertension (Figures 1 and 2) was seen at the University of Michigan. Pulmonary hypertension was elevated (but reactive to inhaled nitric oxide from 8 to 4 Wood units), and a heart transplant was therefore considered to carry a higher risk than anatomic repair. The patient underwent a Senning-Rastelli procedure and RV–to–pulmonary artery conduit placement (Hancock; Medtronic, Inc, Minneapolis, Minn). RV pressures immediately after repair were half of systemic pressures. On postoperative day (POD) 0, the patient had biventricular failure as a result of diastolic dysfunction (central venous and left atrial pressures >20 mm Hg) and required venoarterial extracorporeal membrane oxygenation support. The patient was successfully weaned on POD 4; however reinstitution of extracorporeal membrane oxygenation was necessary 3 days later for worsening diastolic failure and was maintained for 8 more days, with multiple unsuccessful attempts at weaning.Figure 2A, Three-dimensional reconstruction of cardiac magnetic resonance image demonstrating congenitally corrected transposition of the great arteries and dextrocardia. B, Axial cardiac magnetic resonance image demonstrating cardiomegaly and anteroposterior mediastinal dimension of 12 cm at the level of the pulmonary veins. C, Preoperative chest radiograph demonstrating dextrocardia and cardiomegaly.View Large Image Figure ViewerDownload Hi-res image Download (PPT) Bridge to transplant with a total artificial heart (TAH; Syncardia Systems, Inc, Tuscon, Ariz) was undertaken (Figure 3). During TAH implantation, the Senning pathway was reversed. The 70-cc TAH ventricles were implanted with the pulmonary outflow connected to the RV–to–pulmonary artery conduit. TAH settings were as follows: left ventricular drive pressure, 190 mm Hg; RV drive pressure, 70 to 80 mm Hg; vacuum, 11 to 12 mm Hg; beat rate, 100 beats/min; and cardiac output, 5.3 L/min. A small decrease in RV drive pressure and vacuum were made on the basis of the appearance of the chest radiograph and fluid mobilization. Anticoagulation was accomplished with aspirin, heparin, warfarin sodium, and dipyridamole. Fibrinogen levels were 244 to 391 mg/dL, and platelet concentrations were 84 to 208 × 103 cells/μL. A suitable donor graft became available on POD 8, and the patient underwent explantation of the TAH and an orthotopic heart transplant. No thrombus was noted in the TAH at explantation. The ascending aorta and hemiarch were also replaced because of a deadventitializing injury that had occurred from cannulation at his original procedure. The patient was started on inhaled nitric oxide intraoperatively and was transitioned to sildenafil. Pulmonary vascular resistance was initially 15 Wood units/m2 but decreased to 4.5 Wood units/m2 by discharge. The patient was transferred to the ward on POD 16 after the transplant and was discharged on POD 24 (Figure 3). Mechanical circulatory support device options for pediatric patients are limited. Because of this patient's size, the TAH was a suitable option. Biventricular diastolic dysfunction prevented the use of a systemic ventricular assist device. This is the second report of TAH use in a pediatric patient with CCTGA. A 17-year-old with CCTGA who had undergone a physiologic repair with subsequent TAH implantation required modification because of anatomical considerations.3Morales D.L. Khan M.S. Gottlieb E.A. Krishnamurthy R. Dreyer W.J. Adachi I. Implantation of total artificial heart in congenital heart disease.Semin Thorac Cardiovasc Surg. 2012; 24: 142-143Abstract Full Text Full Text PDF PubMed Scopus (29) Google Scholar In our case, the only modification performed was that we left the distal portion of the RV–to–pulmonary artery conduit intact and anastomosed the right sided outflow graft to this. Further, we elected to reverse the Senning procedure because of the risk of obstruction of the pulmonary venous pathway after TAH implantation. Our patient tolerated the TAH without any adverse events. He did have a labile pre-transplant pulmonary vascular resistance; however, we did not consider this severe enough to outweigh the risk of postponing his transplant (increased time on the device and the potential development of severe adhesions). To our knowledge, Park and colleagues4Park S.S. Sanders D.B. Smith B.P. Ryan J. Plasencia J. Osborn M.B. et al.Total artificial heart in the pediatric patient with biventricular heart failure.Perfusion. 2014; 29: 82-88Crossref PubMed Scopus (25) Google Scholar have reported the only other successful case of pediatric TAH use, in a 14-year-old with dilated cardiomyopathy. With the advent of the 50-cc TAH, we anticipate that this device will benefit pediatric patients with end-stage, complex congenital heart disease.5Ryan T.D. Jefferies J.L. Zafar F. Lorts A. Morales D.L. The evolving role of the total artificial heart in the management of end-stage congenital heart disease and adolescents.ASAIO J. 2015; 61: 8-14Crossref PubMed Scopus (42) Google Scholar