Journal of Organ Transplantation

Journal of Organ Transplantation

Current Issue Volume No: 1 Issue No: 1

Case-report Article Open Access
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  • Atypical Patterns Of Constrictive Pericarditis After Heart Transplantation: A Case Report

    1 Department of Cardiology, National Cerebral and Cardiovascular Center, Japan 

    2 Department of Transplantation, National Cerebral and Cardiovascular Center, Japan 

    3 Department of Cardiovascular Surgery, National Cerebral and Cardiovascular Center, Japan 

    Abstract

    Constrictive pericarditis (CP) represents a rare complication after heart transplantation (HTx), resulting from various postoperative events such as mediastinitis, pericardial effusion, or allograft rejection. We describe our recent experience with managing an HTx recipient who developed atypical patterns of CP predominantly involving the right ventricle. A 52-year-old male who had received heart transplantation 2.5 years before was admitted to our institution because of progressive symptoms of heart failure. The patient had experienced acute rejection twice post-HTx, both with International Society for Heart and Lung Transplantation grade 1R, undergoing an additional endomyocardial biopsy other than those performed during regular check-ups. On admission, echocardiography revealed paradoxical septal motion and a large cystic-like mass with a thick capsule in front of the right ventricle. Right heart catheterization revealed elevation of right atrial pressure, with severely reduced cardiac index. Magnetic resonance imaging revealed both seroma and a thick cystic-like capsule tightly adhered to the right ventricle. CP was suspected despite the atypical patterns of presentation. Seroma was removed through exploratory lateral thoracotomy, without improvement in symptoms, which was only achieved via subsequent pericardiectomy involving resection of the thickened parietal pericardium, removal of effusion fluid, and further excision of diffusely thickened visceral pericardium and epicardium. The patient is currently recovering uneventfully. The possibility of CP after HTx should be considered despite the rarity of this condition and HTx recipients should be closely monitored using various imaging modalities because CP typically demonstrates non-specific symptoms and physical findings of heart failure, with high mortality.
    Author Contributions
    Received May 15, 2017     Accepted Aug 16, 2017     Published Sep 01, 2017

    Copyright© 2017 Ueda Nobuhiko, et al.
    License
    Creative Commons License   This work is licensed under a Creative Commons Attribution 4.0 International License. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

    Competing interests

    The authors have declared that no competing interests exist.

    Funding Interests:

    Citation:

    Ueda Nobuhiko, Seguchi Osamu, Kuroda Kensuke, Nakajima Seiko, Sato Takuma et al. (2017) Atypical Patterns Of Constrictive Pericarditis After Heart Transplantation: A Case Report Journal of Organ Transplantation. - 1(1):32-37
    DOI 10.14302/issn.2576-9359.jot-17-1594

    Introduction

    Introduction

    Constrictive pericarditis (CP) has been reported in recipients of heart transplantation (HTx).1 Various post-transplant complications such as mediastinitis, pericardial effusion including hemorrhage, and allograft rejection are considered as causes of post-transplant CP.234 While pericardiectomy is recognized as the fundamental treatment for CP, one-third of patients with CP have recurrence of heart failure symptoms after pericardiectomy.5

    We describe herein our recent experience with managing a heart transplant recipient who had developed atypical patterns of CP predominantly involving the right ventricle.

    Discussion

    Discussion Constrictive Pericarditis and Epicarditis after HTx

    CP after HTx has been recognized as a rare disease, and moreover the diagnosis of CP is especially difficult in HTx recipients.3 A previous study reviewing 133 HTx recipients reported that 12% of patients developed postoperative complications involving the mediastinum and pericardium, and 2 patients (1.5%) developed signs of pericardial constriction.1 A more recent report reviewing 163 patients receiving pericardiectomy for CP reported that only 3 HTx recipients (1.8%) were found in this series.6 In our institution, 93 patients received HTx until 2016, and the patient described herein is the only one who underwent pericardiectomy for post-transplant CP (1.1%).

    With regard to diagnosis, since patients with CP typically demonstrate non-specific symptoms and non-specific physical findings of heart failure, both right heart catheterization and various imaging investigations such as echocardiography, computed tomography, and magnetic resonance imaging should be performed. 7 For definitive diagnosis of CP, constrictive hemodynamic physiology together with thickened pericardium adhered to the myocardium should be proven.8 However, constrictive or restrictive physiology are sometimes non-specifically and temporary observed in HTx recipients for other reasons. In particular, ACR, which is one of the major causes of death during the early post-HTx period, has been reported to be associated with constrictive-restrictive physiology of the cardiac allograft.9 Seacord et al. reported that a heart transplant recipient with abnormal hemodynamics indicative of CP had exhibited improvement in the hemodynamic abnormality after ACR treatment with antilymphocyte globulin.10 ACR might promote constrictive-restrictive myocardial physiology, which improves upon resolution of ACR.9 Therefore, when the constrictive-restrictive physiology is suspected in HTx recipients, ACR should be initially ruled out, and accurate evaluation of hemodynamics should be performed to verify whether the findings fulfill the diagnostic criteria of CP.

    In terms of etiology, various causes specific to HTx recipients should be taken into account in addition to conventional surgical matters. ACR with pericardial effusion and vasculopathy are characteristic causes of CP in HTx recipients.4,11 Further, administration of immunosuppressive agents in HTx recipients is known to double the incidence of mediastinitis or other intrathoracic infections postoperatively,12 which are thought to potentially cause CP in the early stage of after HTx in recipients with strong immunosuppression.2,3,13 In our patient, two episodes of ACR were detected before the development of CP, indicating that ACR might be the primary cause for CP in this patient. Since ACR is highly related to the development of pericardial effusion, repetitive ACR might have resulted in pericardial effusion followed by effusive CP. A previous study reviewing 15 patients with effusive CP reported that extensive epicardiectomy is the procedure of choice in patients requiring surgical intervention. Indeed, our patient also required not only parietal pericardiectomy but also visceral pericardiectomy including epicardiectomy.

    The following three clinical features were notable in our patient: 1) effusive constrictive pericarditis, 2) the need for not only parietal pericardiectomy but also visceral pericardiectomy including epicardiectomy for resolving the constrictive hemodynamic physiology, and 3) right ventricle involvement. Considering the former two findings, we believe the etiology of CP in this patient is primarily related to ACR. However, right ventricle involvement is suggestive of a different etiology such as minor perforation by repeated EMB. Furthermore, since everolimus administration has been reported to be associated with the development of pericardial effusion, it is possible that everolimus administration had an additive effect on the effusive pathophysiology of CP.14

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