Gen Thorac Cardiovasc Surg DOI 10.1007/s11748-015-0573-4

CASE REPORT

Orthotropic heart transplantation for adult congenital heart disease: a case with heterotaxy and dextrocardia Hikaru Matsuda1,3 • Norihide Fukushima1 • Hajime Ichikawa1,2 • Yoshiki Sawa1

Received: 1 April 2015 / Accepted: 29 June 2015 Ó The Japanese Association for Thoracic Surgery 2015

Abstract A 41-year-old male with heterotaxy (left isomerism) and dextrocardia composed by single ventricle, absent inferior vena cava, bilateral superior vena cava (SVC), common atrioventricular valve has received orthotopic heart transplantation (HTx) after long waiting period as Status-1. Reconstructions of bilateral SVC and hepatic vein route were successful without use of prosthetic material, and the donor heart was placed in the left mediastinum. In spite of satisfactory early recovery, the patient expired 4 months after transplantation mainly from fungal infection which developed following humoral rejection. HTx for adult patients with complex congenital heart disease is demanding in technical as well as pre- and posttransplant management, and indication should be critically determined.

Introduction

Keywords Adult congenital heart disease  Heart transplantation  Heterotaxy  Dextrocardia  Humoral rejection

Patient was a 41-year-old male and the diagnosis was left isomerism, single right ventricle, single atrium, common atrioventricular valve (CAVV), bilateral superior vena cava (SVC), inferior vena cava (IVC) interruption with azygos connection, pulmonary stenosis (PS), dextrocardia, and s/p relief of PS and CAVV replacement. The patient received right ventricular outflow reconstruction for PS at age of 2 years and had been followed to the adult age as inoperable because of suspected pulmonary hypertension. At 37 years of age (August, 2000), the patient started to require intravenous inotropes and was referred to our institution for possible HTx in March 2001. CAVV regurgitation was found as severe and CAVV replacement was done by porcine valve on March 14, 2001. The heart failure was temporarily improved, but again returned to inotropes dependent. Indication of HTx was carefully examined and medical data were compatible with recipient criteria. He was listed to the Japan Organ Transplantation Network in July 17, 2001 as Status-1. The

H. Matsuda: Professor Emeritus Osaka University. & Hikaru Matsuda [email protected] 1

Department of Cardiovascular Surgery, Osaka University Graduate School of Medicine, Yamadaoka 2-2, Suita, Osaka 565-0871, Japan

2

Present Address: National Cerebral and Cardiovascular Center, Osaka, Japan

3

Higashi Takarazuka Satoh Hospital, Nagaochou 2-1, Takarazuka, Hyogo 665-0873, Japan

The management of adult patients with congenital heart disease (ACHD) has been more demanding in recent practices of heart failure [1]. The heart transplantation (HTx) for those with ACHD has relatively high operative risk, but rather favorable long-term outcome has been reported [2, 3]. In ACHDs, surgical strategies for orthotropic HTx have been challenging especially for those with complex anatomy such as heterotaxy syndrome [4, 5]. In this report, we describe a case with left isomerism and dextrocardia who underwent orthotopic HTx.

Case

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clinical data at this point showed oxygen saturation of 80 % (room air), left ventricular EF 32.8 % (by angio), PVR 1.68 Unit, serum creatinine 1.7 mg/dl, total bilirubin 1.4 mg/dl, serum albumin 4.2 g/dl, and BNP 207 pg/ml, respectively. PRA (panel reactive antibodies) was 55 %. CBC showed white blood cell count as 3.67 9 103 with 21.5 % of lymphocyte. BMI was 14.4, and BSA was 1.30 m2. The myocardial biopsy showed the finding similar to cardiomyopathy with mild fibrosis. Preoperative chest X-ray showed dextrocardia with cardiothoracic ratio of 75 % (in Fig. 1a).

Fig. 1 Chest X-rays before transplantation (a) and post-transplant day 3 (b)

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Because of high PRA, desensitization therapy was conducted twice with cyclophosphamide and intravenous c-Globulin, and PRA was decreased to 29 % for Class-I and 0 % for Class-II before HTx. On November 20, 2004, a matched donor heart was obtained from a donor of age 50 s with 40 min of resuscitation before brain death. Because the waiting period reached to 1221 days, the indication for HTx was critically assessed and decided to accept this marginal donor heart. Direct cross match was reported as negative. Operative procedures: After redo median sternotomy, cardiopulmonary bypass (CPB) was established by arterial cannula to ascending aorta and three venous drainage cannulas, to the right and left SVCs and hepatic vein directly. After arrival of donor heart, the recipient’s heart was excised. Left mediastinal space was enlarged by dissecting left side pericardium and pleura preserving left phrenic nerve intact. Then remnant of posterior atrial wall above the orifices of hepatic veins was tailored constructing a straight conduit for hepatic vein to right atria connection. Donor heart was placed in the following sequences;  anastomosis of donor left atrium to the cuff of recipient’s left atrium, ` excision of recipient’s pulmonary artery (PA) trunk followed by transverse opening of the main PA facilitating a wide and un-angulated anastomosis between bifurcation of donor main PA, ´ anastomosis of donor aorta to recipient’s aorta, ˆ remove the aortic clamp resuming coronary perfusion with initial leukocyte-depleted blood cardioplegia, ˜ anastomosis between hepatic vein conduit to donor IVC, Þ right SVC reconstruction by end-to-end fashion, and þ left SVC reconstruction placing donor innominate vein behind the ascending aorta. These procedures are illustrated in the

Fig. 2 Schematic drawing of the gross appearance of the heart

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Figs. 2 and 3. CPB time was extended to 331 min, and total operating time was 9 h and 20 min. Donor heart ischemic time was 234 min. The ventricular function was well maintained and returned to ICU with a moderate dose of inotropes. The Chest X-ray on day 3 is shown in Fig. 1b demonstrating well-placed donor heart in the left side of mediastinum. The immunosuppression was started with steroid,

mycophenolate mofetil, and tacrolimus considering the impaired renal function. During the post-transplant week 2 , hemodynamic status started to deteriorate suggesting acute rejection. Myocardial biopsy demonstrated significant interstitial edema without apparent myocyte necrosis suggesting humoral rejection. Plasma exchange was started combined with anti-thymocyte globulin, and cyclophosphamide and rituximab were added (Fig. 4). The PRA level on post-transplant day 8 confirmed the acute rise as 100 % for Class-I and Class-II. The subsequent course was compromised with pneumonia from fungal infection and intensive treatment was required for over a month. However, the patient showed progressive recovery and transferred to the high-care ward at postoperative week 12 and started rehabilitation. Chest CT demonstrated no apparent obstruction of the constructed venous routes. The patient again developed respiratory distress with fungal pneumonia and septic condition. The organisms detected were Staphylococcus epidermidis from blood sample and Aspergillum sp. from the sputum. Also Pseudomonas fluorescence and putida were detected in the sputum. The patient expired from persisting fungal infection combined with bacterial septicemia and multiorgan failure on post-transplant day 121.

Discussion

Fig. 3 Outline of the operative procedures. a After explanting the native heart, hepatic vein conduit was first reconstructed using adjacent atrial wall. After left atrial anastomosis, pulmonary artery route was reconstructed to the left main pulmonary artery, and then aortic anastomosis was followed. b Transplantation was completed by three venous anastomoses after release of the aortic clamp

The heart transplantation from brain-dead donor under the legislation in this country was started in February 1999 [6] and until recently the total number has reached to over two hundred with significant increase after revise of the law in 2010 [7]. The case presented here was one of the early phases in Japan, and considering the complex cardiac anatomy, it took a long waiting period to get a suitable donor, but eventually we have accepted the marginal donor. The donor heart ischemic time was controlled within 4 h and ventricular function after transplantation was acceptable. Currently, the candidates for the HTx for those with congenital heart disease have been shifted toward the adult cohort [2]. However, adult patients with heterotaxy and complex systemic venous return as seen in left isomerism heart have various burdens toward the favorable outcome after HTx [8]. In the anatomical aspect of ventricle morphology, one ventricle has higher early mortality than those with two ventricle anatomy [9]. In the previous reports, including those with situs inversus, various technical attempts have been described [10, 11]. Technical modification of bi-caval anastomosis in patients with heterotaxy was demanding. In the recipient with absence of IVC, it is required to construct the hepatic venous route to donor right atrium (IVC) crossing the

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Gen Thorac Cardiovasc Surg Fig. 4 The course of humoral rejection. PEX plasma exchange, ATG anti-thymocyte globulin, CP cyclophosphamide, FK506 tacrolimus and PRD predonine

midline without use of prosthetic material. The conduit construction using redundant atrial wall was very suitable in this situation. Also, long preserved innominate vein of donor heart has facilitated the unobstructed anastomosis to the left SVC passing behind the new aorta. The occurrence of humoral rejection may be related to previous surgery, and in spite of lowered pre-transplant PRA after desensitization treatment, the post-transplant course showed the occurrence of humoral rejection. The immunofluorescence staining for IgGm and C3 was not apparently confirmative, but humoral rejection was diagnosed from myocardial biopsy finding and reactivation of PRA in this case. From this experience, it is reminded that humoral rejection can occur in patients with ACHD even when the preoperative desensitization therapy lowered the PRA level and direct cross match was negative. The intraoperative plasma exchange may be a matter of concern. Because the number of patients with ACHD requiring HTx is increasing [12], it should be reminded that the humoral rejection is of higher risk when assist device was implanted [13].

Conclusion This ACHD case with dextrocardia received HTx as the first case for CHD in Japan. Orthotopic HTx was technically feasible. Post-transplant course was compromised with humoral rejection and subsequent fungal infection caused hospital death 4 months after HTx. This experience can be helpful for current practices in the management of ACHD including HTx.

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Conflict of interest All the authors have declared no conflict of interest relating to this report.

References 1. Ryan TD, Jefferies JL, Wilmot I. Managing heart failure in adults with congenital heart disease. Curr Treat Options Cardiovasc Med. 2015;17:376. doi:10.1007/s11936-015-0376-z. 2. Davies RR, Russo MJ, Yang J, Quaegebeur JM, Mosca RS, Chen JM. Listing and transplanting adults with congenital heart disease. Circulation. 2011;123:759–67. 3. Bhama JK, Shulman J, Bermudez CA, Bansal A, Ramani R, Teuteberg JJ, et al. Heart transplantation for adults with congenital heart disease: results in the modern era. J Heart Lung Transplant. 2013;32:499–504. 4. Burchill LJ, Edwards LB, Dipchand AI, Stehlik J, Ross HJ. Impact of adult congenital heart disease in survival and morbidity after heart transplantation. J Heart Lung Transplant. 2014;33: 1157–63. 5. Lamour JM, Kanter KR, Naftel DC, Chrisant MR, Morrow WR, Clemson BS, et al. The effect of age, diagnosis, and previous surgery in children and adults undergoing heart transplantation for congenital heart disease. J Am Coll Cardiol. 2009;54:160–5. 6. Matsuda H, Fukushima N, Sawa Y, Nishimura M, Matsumiya G, Shirakura R. First brain dead donor heart transplantation under new legislation in Japan. Jpn J Thorac Cardiovasc Surg. 1999;47:499–505. 7. Nakatani T, Fukushima N, Ono M, Saiki Y, Matsuda H, Yozu R, et al. The registry report of heart transplantation in Japan (1999–2013). Circ J. 2014;78:2604–9. 8. Jacobs JP, Asante-Korang A, O’Brien SM, Chai PJ, Dadlani GH, et al. Lessons learned from 119 consecutive cardiac transplant for pediatric and congenital heart disease. Ann Thorac Surg. 2011;91:1248–55. 9. Karamlou T, Diggs BS, Welke K, Tibayan F, Gelow J, Guyton SW, et al. Impact of single-ventricle physiology on death after heart transplantation in adults with congenital heart disease. Ann Thorac Surg. 2012;94:1281–8.

Gen Thorac Cardiovasc Surg 10. Vricella LA, Razzouk AJ, Gundry AR, Larsen RL, Kuhn MA, Bailey LL. Heart transplantation in infants and children with situs inversus. J Thorac Cardiovasc Surg. 1998;116:82–9. 11. Michler RE, Sandhu AA. Novel approach for orthotropic heart transplantation in visceroatrial situs inversus. Ann Thorac Surg. 1995;60:194–7. 12. Karamlow T, Hirsch J, Welke K, Ohye RG, Bove EL, Devanney EJ, et al. A United Network for organ sharing analysis of heart

transplantation in adults with congenital heart disease: Outcome and factors associated with mortality and retransplantation. J Thorac Cardiovasc Surg. 2010;140:161–8. 13. Saito S, Matsumiya G, Fukushima N, Sakaguchi T, Fujita T, Ueno T, et al. Successful treatment of cardiogenic shock caused by humoral cardiac allograft rejection. Circ J. 2009;73:970–3.

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Orthotropic heart transplantation for adult congenital heart disease: a case with heterotaxy and dextrocardia.

A 41-year-old male with heterotaxy (left isomerism) and dextrocardia composed by single ventricle, absent inferior vena cava, bilateral superior vena ...
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