CASE REPORT - VASCULAR
Interactive Cardiovascular and Thoracic Surgery 15 (2012) 301–303 doi:10.1093/icvts/ivs194 Advance Access publication 16 May 2012
False aneurysm of ascending aorta due to pericardial mesothelioma† Vladimir Uspenskiy*, Alexei Lavreshin, Alexei Osadchii and Michael Gordeev Almazov Federal Heart, Blood and Endocrinology Centre, St. Petersburg, Russian Federation
* Corresponding author. 6 Lanskoe avenue, apt. 163, 197342 St. Petersburg, Russian Federation. Tel: +7-921-7442289; e-mail:
[email protected] (V. Uspenskiy) Received 19 January 2012; received in revised form 20 March 2012; accepted 1 April 2012
Abstract Ascending aortic false aneurysm is a rare but serious complication of pericardial mesothelioma. We report a case of ascending aortic pseudoaneurysm due to spindle cell pericardial mesothelioma. In this case, the first symptoms of the disease appeared 18 months before surgery. The final diagnosis was determined only when severe late complications occurred. Palliative tumour excision, aortoplasty and aortic valve prosthesis were performed with subsequent adjuvant chemotherapy. Over 10 months after surgery, the patient is alive and a significant reduction of the tumour mass has been achieved. This case demonstrates that timely lifetime diagnosis of malignant pericardial tumour remains very difficult and effective adjuvant chemotherapy is needed to improve the results of surgery.
INTRODUCTION Primary malignant pericardial mesothelioma arises from pericardial mesothelial cells or from more primitive progenitor cells [1]. Typical signs of this disease are nodes or solid bulbs in the pericardium, sometimes extending to adjacent structures (ascending aorta or pulmonary trunk). Surgery can be effective in cases of localized pericardial mesothelioma [2]. Surgery alone is usually inefficient, due to large solid tumour masses and pericardial carcinomatosis. Chemotherapy and radiotherapy are questionable [3]. Prognosis is extremely poor: six-month survival does not exceed 50% [2]. We present a case of pericardial mesothelioma complicated with ascending aortic false aneurysm.
CASE REPORT A 26-year-old man was admitted to our clinic on 19th April 2011 with complaints of chest pain, dyspnoea and fever, which first appeared in November 2009. The computed tomography revealed pericardial effusion; no invasive diagnostic procedure was performed. The patient received non-steroidal antiinflammatory drugs and improved. In April 2011, echocardiography revealed the ascending aortic pseudoaneurysm and, due to this finding, the patient was admitted to our hospital. Laboratory measurements revealed increased white blood cell counts (11 000/μl) and serum levels of C-reactive protein (2.88 mg/dl) and fibrinogen (540 mg/dl). Transthoracic echocardiography, cardiac contrast magnetic resonance imaging (Fig. 1(A)) † This article was presented at the 17th All-Russian Congress of Cardiovascular Surgeons, Moscow, Russia, 27 November 2011 under the title “Spindle cell mesothelioma of pericardium complicated with ascending aortic false aneurysm”.
and computed tomography (Fig. 1(B)) revealed a saccular ascending aortic pseudoaneurysm, 110 mm in diameter, with partial thrombosis of the aneurysmal cavity. The residual cavity was connected to the ascending aorta by a 6-mm neck, very close to the aortic valve. There was no aortic insufficiency; the left ventricle function was normal. Also there was no evidence of any tumourous formation, mediastinal lymphadenopathy, pericardial carcinosis of effusion. The patient had no significant concomitant diseases. On 29th April 2011 the patient underwent surgery. After midline sternotomy, strong adhesions in the pericardial cavity were marked. The inferior vena cava and the pulmonary veins could not be visualized, so a small part of the superior vena cava and the right femoral vein were used to establish cardiopulmonary bypass. The aortic cannula was placed conventionally. The heart was exposed under parallel circulation. We marked several rounded, dense nodes 1–2 cm in diameter, located on the left antero-lateral surface of the heart: a biopsy of one of these formations was made. Also the involvement of the pseudoaneurysm into the anterior wall of the right ventricle was noted. No histological examination was performed during the operation. After cardioplegia by antegrade introduction of Custodiol® solution, the aortic root was revised. The aortic wall fenestration was located in the non-coronary sinus, just below the sinotubular junction, close to the commissure between the noncoronary and the right coronary cusps. The pseudoaneurysm was partially excised (Fig. 1(C)). The edges of aortic fenestration, also, were partially excised and the aortic wall defect was closed with a xenopericardial patch (Fig. 1(D)). Due to the close location of the fenestration to the aortic valve commissure, we were unable to close this defect whilst preserving the valve. We therefore performed aortic valve replacement with the MedEng ADM23–1In 23 mm bileaflet mechanical
© The Author 2012. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.
CASE REPORT
Keywords: Aortic aneurysm • False aneurysm • Mesothelioma • Pericardium
302
V. Uspenskiy et al. / Interactive CardioVascular and Thoracic Surgery
Figure 1: (A) Contrast magnetic resonance imaging. (B) Computed tomography. (C) Sectioned false aneurysm: note thrombus in aneurysmal cavity. (D) Closure of aortic fenestration with xenopericardial patch (arrow).
prosthetic valve. The resulting defect of the right ventricle wall was closed with several U-type sutures (Ethibond 2/0). The postoperative period was uneventful. Immunohistochemical analysis of the pericardial nodes revealed spindle cell mesothelioma with high proliferative potency. Also malignant cells were discovered in the edges of the aortic defect. On 16th May 2011, the patient was discharged but then admitted to the N.N. Petrov Scientific and Research Institute of Oncology (St. Petersburg, Russian Federation). To date, he has completed several courses of combined adjuvant chemotherapy and is doing well. The repeated computed tomography scan showed a significant (more than 50%) reduction of the tumour burden and no effusion.
DISCUSSION Primary malignant cardiac tumours are rare but timely lifetime diagnosis remains very difficult. Pericardial mesothelioma is one such rare but fatal disease. Commonly, mesothelioma has a pleural origin (98%) and, in 0.7% of instances, it has pericardial origin. Three histological types of pericardial malignant mesothelioma are known: epithelial, spindle cell and mixed types [4]. This tumour is very hard to detect due to non-specific manifestations including dyspnoea, fever and weight loss. It usually manifests itself through cardiac tamponade or constrictive
pericarditis. These complications may lead to cardiac arrest because of cardiac compression or involvement of malignant cells into the myocardium. Possible complications are brain ischaemia, myocardial infarction and superior vena cava syndrome [1]. In view of the features of this tumour, the patient faces surgery only when late complications occur. In the presented case, after the onset of non-specific symptoms, the young man did not receive the required diagnostic procedures, nor did he receive specific medical care for a long time. It took a complication as severe as ascending aortic pseudoaneurysm to make it clear that surgery was required. Due to the diffuse pericardial and epicardial carcinosis, surgical options were seriously limited and radical operation was impossible. The heart was profoundly involved with the tumourous nodes and the pseudoaneurysm. Complete removal of the neoplasm was impossible and results of the histological examination could not influence the mode of surgery. Due to localization of the aortic fenestration near the commissure, adequate repair of the fenestration without distortion of the aortic valve structures was similarly impossible. Detection of tumour cells in the edges of the aortic wall fenestration proved the tumourous nature of the pseudoaneurysm. Another discussed question was the necessity of pericardectomy. In palliative surgery, pericardectomy is usually performed to prevent cardiac tamponade and relieve constriction. Another
V. Uspenskiy et al. / Interactive CardioVascular and Thoracic Surgery
REFERENCES [1] Turk J, Kenda M, Kranjec I. Cardiac tamponade caused by primary pericardial mesothelioma. N Engl J Med 1991;325:814. [2] Suman S, Schofield P, Large S. Primary pericardial mesothelioma presenting as pericardial constriction: a case report. Heart 2004;90:e4. [3] Eren NT, Akar AR. Primary pericardial mesothelioma. Curr Treat Options Oncol 2002;3:369–73. [4] Yilling FP, Schlant RC, Hertzler GL, Krziniak R. Pericardial mesothelioma. Chest 1982;81:520–23. [5] Piwowarska W, Nessler B, Pietrzak I, Gajos G, Sadowski J, Krzemieniecki K. Diagnostic difficulties in a 32-year old patient with cardiac tamponade. Kardiol Pol 1993;38:209–12.
CASE REPORT
purpose is to create a window for introduction of chemotherapy agents [5]. In the presented case there were no signs of cardiac constriction or pericardial effusion, so we decided not to perform the fenestration or partial pericardectomy. Fortunately, 10 months after surgery, the patient is still alive. No doubt effective chemotherapy has contributed to the good result in this case. We can conclude that palliative resection of malignant pericardial tumours and repair of complications are both possible, but radical surgery is imperative and prognosis remains poor. Effective adjuvant chemotherapy is needed to improve results of surgery.
303
