Heart transplantation in the treatment of primary non-operable cardiac tumors

Address for correspondence: Dr. Siarhei G. Amelchanka, Department of Cardiac Surgery, Republican Scientific Practical Centre “Cardiology”, 110 R. Luxemburg St, 220036 Minsk, Belarus, phone: +37 5297848684, e-mail: omelchenkosergey2@gmail.com Received: 30.06. 2017, accepted: 21.08. 2017. Tumor resection within healthy tissues is the main treatment method for patients with primary benign cardiac tumors, which account for up to 90% of all primary cardiac tumors [1–3]. Short-term and long-term results after such interventions are generally good, and the 10-year recurrence-free survival is about 93–100% [3, 4]. Even in the case of incomplete resection of a benign cardiac tumor because of its size and localization (mostly in large intramural ventricle tumors), the prognosis is still good, because these tumors grow slowly and are not associated with a systemic adverse influence [5]. Patients with non-resectable benign cardiac tumors may be candidates for orthotopic heart transplantation (OHT) [6, 7]. Primary malignant tumors, of which 75% are sarcomas [8], are commonly diagnosed at an advanced stage, because of rapid growth; and only in 15% of patients is it possible to perform tumor resection, and this without significant prognosis improvement [8]. In the natural disease course average survival time in this category of patients is about 10 months after diagnosis [8, 9]. Polychemotherapy (PCT) and radiotherapy (RT) do not provide improvement of long-term survival [10]. Extended resections of tumorinvolved cardiac structures and reconstructive interventions with heart autotransplantation (HA) in combination with neo-adjuvant and adjuvant PCT and RT in selective cases may improve long-term survival in patients with primary malignant tumors of atria, and to a lesser extent in patients with involvement of ventricles [11]. Local tumor recurrence even after pathology-proven radical resection (R0) is the main cause of mortality in the early and midterm postoperative follow-up period [2, 3, 6]. Until now OHT has rarely been performed in patients with primary non-resectable malignant cardiac tumors because of controversial results achieved [6, 12, 13], because of donor-heart deficit and because of the legislation in several countries against performing such interventions to treat this pathology [3]. However, there is still a certain interest in this intervention as a method of treatment for primary malignant cardiac tumors, because of separate successive examples [14] and the absence of effective alternative treatment. In this report we present our experience in performing OHT for primary benign and malignant cardiac tumors. Two patients (1 male and 1 female) with non-resectable cardiac tumors were surgically treated at SI RSPC Cardiology of Republic of Belarus during the period from May 2011 to May 2015. In one case the tumor involving the right ventricle (RV) and interventricular septum (IVS) was benign, and in the remaining case the tumor was malignant, involving all cardiac chambers. In these 2 patients OHT was performed. Interventions were performed according to previously described biatrial and bicaval methods [15] under normothermic perfusion. Custodiol solution was used for cardioplegia. Mid-term results were obtained during repeated in-hospital examination of patients. Case 1: A 49-year-old female patient referred to the hospital in March, 2011 complaining of dyspnoea on moderate physical exertion and first appearance of syncopal condition. At the age of 24 she was diagnosed with a tumor of the IVS with a size of 15 mm according to transthoracic echocardiography (EchoCG) data. The patient was followed up with annual examinations and felt well until recently, despite slow tumor growth (Fig. 1). Non-invasive slow tumor growth, absence of signs of distant metastases according to computed tomography (CT), other clinical signs, and the patient’s feeling of well-being all suggested benign tumor. Multiple episodes of paroxysmal ventricular tachycardia were registered in hospital settings, and were successfully managed with medicinal treatment and cardioversion. Transthoracic and transoesophageal EchoCG revealed a neoplasm 47/27 mm in the middle and lower third of the IVS and RV containing calcification foci. The neoplasm restrained the right ventricular outflow tract with peak pressure gradient of 18 mm Hg. Right ventricle and left ventricle (LV) contractility, dimensions of cardiac cavities and funcHeart transplantation in the treatment of primary non-operable cardiac tumors