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STATE OF THE ART ARTICLE Initial Experience of Left Ventricular Assist Device Support for Adult Patients with Transposition of the Great Vessels Jonathan N. Menachem, MD,* Aparna C. Swaminathan, MD,† Thomas M. Bashore, MD,† Cary C. Ward, MD,† Joseph G. Rogers, MD,† Carmelo A. Milano, MD,† and Chetan B. Patel, MD† *Department of Internal Medicine, Division of Cardiology, Hospital of the University of Pennsylvania, Philadelphia, Pa, USA; †Department of Internal Medicine, Division of Cardiothoracic Surgery, Division of Cardiology, Duke University Medical Center, Durham, NC, USA

Key Words. LVAD; Transposition; Mechanical Support; Transplant

Introduction

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omplete transposition of the great arteries (TGA) is one of the most common cyanotic defects in newborns, and in most cases, requires surgical correction to redirect blood flow and maintain pulmonic and systemic circulation.1,2 The first repair of TGA (atrial switch or Senning procedure) involved creating an atrial baffle from autologous tissue to direct venous return to the contralateral atrioventricular (AV) valve and ventricle.3 Later, the Mustard procedure was developed in which the atrial septum was excised and a synthetic material was used to create the baffle.4 These have now been replaced by an arterial switch procedure, which reconnects the aorta to the morphologic left ventricle (LV). In comparison, congenitally corrected transposition of the great arteries (CCTGA) patients are less likely to be diagnosed at birth given the lack of cyanosis, but up to one-third of patients develop heart failure (HF) by the fifth decade.5 In CCTGA (as in TGA after an atrial switch), the morphologic right ventricle (RV) continues to function as the systemic ventricle, with systolic HF developing because of limitations of the RV to support the systemic circulation.6,7 The RV myocardium is spongy and trabeculated with a mechanically inefficient tricuspid subvalvular apparatus that frequently fails when facing systemic pressures.8 In addition, it is believed that the right coronary artery cannot meet the myocardial perfusion demands of a systemic ventricle, which results in myocardial ischemia.9 Ultimately, cardiac transplantation provides a definitive solution for Congenit Heart Periodicals, Dis. 2015;10:382–386 © 2015 Wiley Inc.

advanced HF symptoms caused by a failing systemic RV. However, because of a limited number of donor organs, allosensitization, complex anatomy, and risks associated with previous cardiac surgeries, this option cannot always be pursued. Further, mechanical circulatory support (MCS) devices are not always possible for these patients because of complex anatomy, biventricular HF, and risks associated with multiple surgical procedures. We report on three patients who had left ventricular assist device (LVAD) implantation into the systemic RV at Duke University Hospital. Patient 1

A 45-year-old male with TGA underwent a corrective Mustard procedure at 3.5 years of age. He later developed systemic ventricular dilation and dysfunction, tricuspid insufficiency, aortic insufficiency, and symptomatic infra-Hisian block requiring a dual chamber pacemaker. At age 45, he suffered a witnessed cardiac arrest. His postresuscitation course was complicated by recurrent ventricular arrhythmia and hemodynamic instability requiring venoarterial extracorporeal membrane oxygenation for stabilization and a partially successful ventricular tachycardia (VT) ablation. The patient was allosensitized with strongly positive class II anti–human leukocyte antigen antibodies, which precluded emergent transplantation, so a HeartMate II VAD (Thoratec Corp., Pleasanton, CA, USA) was placed into the systemic RV as a bridge to transplantation (Figure 1). C 2015 V Wiley Inc. Congenit Heart Dis.Periodicals, 2015;••:••–••

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Figure 1. (A) Chest radiograph of patient 1, 45-year-old male with transposition of the great arteries (TGA) who underwent a corrective Mustard procedure at age 3.5 years and underwent HeartMate II ventricular assist device (VAD) placement into the systemic right ventricle (RV) as a bridge to transplantation. (B) Illustration detailing the orientation of VAD placement and the direction of blood flow of the same patient. LA, left atrium; LV, left ventricle; PA, pulmonary artery; RA, right atrium.

In order to implant this device in its usual orientation, the preperitoneal pocket was created in the right upper abdomen; the pump outflow graft was oriented in the right upper abdomen and coursed through the right chest prior to the aortic anastomosis. The patient recovered enough to return to work full time, but unfortunately continued to have recurrent VT despite maximal medical management. During episodes of VT, he experienced reduced VAD flows and fatigue but remained hemodynamically stable. This was likely due to the ability of the subpulmonic ventricle (morphologic LV) to tolerate VT and maintain flow across the pulmonary circuit and into the failing RV, which was supported by the VAD. Because of recurrent VT, he underwent desensitization and successful cardiac transplantation. Patient 2

A 75-year-old female with CCTGA and situs inversus and dextrocardia presented with cardiogenic shock. She was initially managed with inotrope therapy, but because of worsening end organ dysfunction, an intraaortic balloon pump was placed and then weaned. Her age and situs inverCongenit Heart Dis. 2015;••:••–••

sus precluded transplantation, so a HeartMate II LVAD was placed as destination therapy. The systemic ventricle’s (morphologic RV) apex was positioned at the right axillary line and therefore it was necessary to invert the VAD (the anterior surface was rotated 180 degrees to face posteriorly). Rotation of the pump resulted in the apical cannula being directed toward the systemic ventricle (morphologic RV) apex (Figure 2). The power source was brought out of the left (rather than right) upper abdomen. The pump functioned well in this novel orientation and the early postoperative course was unremarkable. Unfortunately, the patient expired secondary to a traumatic subdural and subarachnoid hemorrhage. Patient 3

A 49-year-old female with CCTGA, repaired VSD, and systemic mechanical AV valve regurgitation was evaluated because of advanced cardiomyopathy. She had frequent ventricular ectopy despite antiarrhythmic therapy. An ICD and later CRT were implanted. She later had a cardiac arrest secondary to refractory ventricular arrhythmias. Despite changes in antiarrhythmic therapy and implantation of an azygous vein coil, her funcCongenit Heart Dis. 2015;10:382–386

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Figure 2. (A) Chest radiograph of patient 2, 75-year-old female with congenitally corrected transposition of the great arteries (CCTGA) and situs inversus who underwent left ventricular assist device (LVAD) placement for cardiogenic shock. Note that the apex of the systemic right ventricle is rightwardly pointed and the drainage cannula is rightwardly placed. (B) Illustration detailing the orientation of VAD placement and the direction of blood flow of the same patient. IVC, inferior vena cava; LA, left atrium; LV, left ventricle; PA, pulmonary artery; RA, right atrium; RV, right ventricle; SVC, superior vena cava.

tional status declined and HF symptoms increased. The patient was listed for cardiac transplantation, but was highly allosensitized. Given her progressive symptoms and the need for inotropic support, she was considered for VAD therapy with a HeartWare HVAD (HeartWare Inc., Framingham, MA, USA). The procedure was performed via median sternotomy and the HVAD device was placed into the anterior systemic ventricle (morphologic RV). The aorta was oriented leftward and therefore the HVAD outflow was directed toward the left pleural space before attachment to the aorta (Figure 3). The patient’s postoperative course was interestingly complicated by subpulmonic ventricular (morphologic LV) failure requiring prolonged inotropic support. She was ultimately discharged with oral diuretic therapy with improved functional status. Discussion

The first atrial switch was first described by Senning in 1959, with the Mustard procedure folCongenit Heart Dis. 2015;10:382–386

lowing in 1964.3,4 In atrial switch patients, systemic ventricular failure typically begins in the second and third decades of life. Therefore, heart failure/transplant teams see both patients with CCTGA and those with TGA who had their operations prior to the first arterial switch procedure, which was described by Jatene in 1976. The arterial switch procedure has become the procedure of choice as it allows the systemic circulation by the morphologic LV.2 In patients with a failing systemic RV, implantation of an LVAD into the RV has been reported as a successful strategy. Wiklund and colleagues first described implantation of a HeartMate vented electric device into a teenager post-Mustard procedure 15 years prior.10 The pump was placed intraperitoneally and in a back-to-front position with the outflow cannula directed toward the left chest. The inflow cannula was placed in the diaphragmatic wall of the RV instead of the apex of the systemic ventricle.10 George et al. found that such rotation was not necessary and achieved optimal ventricular drainage by placing the device closer to midline.11 We utilized two different Congenit Heart Dis. 2015;••:••–••

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Figure 3. (A) Chest radiograph of patient 3, 49-year-old female with congenitally corrected transposition of the great arteries (CCTGA), ventricular septal defect (VSD), and systemic atrioventricular (AV) valve regurgitation who underwent placement of HVAD as a bridge to transplantation. (B) Illustration detailing the orientation of VAD placement and the direction of blood flow of the same patient. AO, aorta; CS, coronary sinus; IVC, inferior vena cava; LA, left atrium; LV, left ventricle; PA, pulmonary artery; RA, right atrium; RV, right ventricle.

implantation techniques with novel pump orientations in patients 1 and 2 to allow the HeartMate II device to perform effectively in these patients. Similar modifications in the implantation technique were not required with the HeartWare HVAD, where there was no need for the creation of a preperitoneal pocket to house the device. Special attention is required when implanting the LVAD into the RV apex as it is not as well developed as the LV apex. Additionally, the ventricular trabeculae and moderator band must be carefully resected to avoid obstruction of the inflow cannulae.11 The integrity of atrial baffles (in TGA patients) should be carefully assessed prior to implantation into the systemic ventricle. Any obstruction to flow through the baffle may result in inefficient flow from the pulmonary vasculature and inadequate resolution of pulmonary edema. Finally, the unconventional placement of the device on the right side may cause compression of the liver and create unfavorable angles for inflow and outflow cannulas.10 Despite technical challenges for implantation, there is presumably reduced risk of failure of the unsupported venCongenit Heart Dis. 2015;••:••–••

tricle. The morphologic LV has greater tolerance for the surgical procedure and may be less prone to negative structural changes that can occur after initiation of LVAD support including septal bowing toward the inflow cannula, AV valve insufficiency, and volume overload of the unsupported ventricle. Surprisingly, patient 3 appeared to manifest failure of the sub-pulmonic ventricle (morphologic LV) during the early postoperative period. We believe this was due to the decline in function of this ventricle despite escalating support in the weeks leading up to the operation. The number of patients with end-stage CCTGA and atrial switch TGA remains small given the low incidence of the disease and the transition to the arterial switch procedure. However, because of the challenges of cardiac transplantation, durable MCS may provide a therapeutic option given the favorable characteristics of the LV functioning as an unsupported pulmonic ventricle. Finally, MCS therapy may stabilize critically ill patients or improve certain patient characteristics to optimize candidacy for Congenit Heart Dis. 2015;10:382–386

386 Support for Adults with Transposition LVAD transplantation such as fixed pulmonary hypertension and perhaps even chronic hepatic congestion.

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Corresponding Author: Chetan B. Patel, MD, Department of Internal Medicine, Division of Cardiothoracic Surgery, Division of Cardiology, Duke University Medical Center, DUMC 3034, Durham NC 27710, USA. Tel: (+1) 919-681-3398; Fax: (+1) 919668-7078; E-mail: [email protected]

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Conflict of interest: CBP: Consulted for Thoratec Corp. and HeartWare Inc. All others: None.

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Accepted in final form: March 23, 2015. References

1 Levin DL, Paul MH, Muster AJ, Newfeld EA, Waldman JD. d-Transposition of the great vessels in the neonate. A clinical diagnosis. Arch Intern Med. 1977;137:1421–1425. 2 Warnes CA. Transposition of the great arteries. Circulation. 2006;114:2699–2709. 3 Senning A. Surgical correction of transposition of the great vessels. Surgery. 1959;45:966–980. 4 Mustard WT. Successful two-stage correction of transposition of the great vessels. Surgery. 1964;55:469–472. 5 Graham TP Jr, Bernard YD, Mellen BG, et al. Long-term outcome in congenitally corrected

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transposition of the great arteries: a multiinstitutional study. J Am Coll Cardiol. 2000;36:255– 261. Warnes CA, Somerville J. Transposition of the great arteries: late results in adolescents and adults after the Mustard procedure. Br Heart J. 1987;58:148– 155. Buch J, Wennevold A, Jacobsen JR, Hvid-Jacobsen K, Lauridsen P. Long-term follow-up of right ventricular function after Mustard operation for transposition of the great arteries. Scand J Thorac Cardiovasc Surg. 1988;22:197–202. Joyce DL, Crow SS, John R, et al. Mechanical circulatory support in patients with heart failure secondary to transposition of the great arteries. J Heart Lung Transplant. 2010;29:1302–1305. Hornung TS, Bernard EJ, Jaeggi ET, Howman-Giles RB, Celermajer DS, Hawker RE. Myocardial perfusion defects and associated systemic ventricular dysfunction in congenitally corrected transposition of the great arteries. Heart. 1998;80:322–326. Wiklund L, Svensson S, Berggren H. Implantation of a left ventricular assist device, back-to-front, in an adolescent with a failing mustard procedure. J Thorac Cardiovasc Surg. 1999;118:755–756. George RS, Birks EJ, Radley-Smith RC, Khaghani A, Yacoub M. Bridge to transplantation with a left ventricular assist device for systemic ventricular failure after Mustard procedure. Ann Thorac Surg. 2007;83:306–308.

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Initial Experience of Left Ventricular Assist Device Support for Adult Patients with Transposition of the Great Vessels.

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