Surgical Treatment for Primary Cardiac Leiomyosarcoma Causing Right Ventricular Outflow Obstruction

Abstract

rimary cardiac leiomyosarcoma is extremely rare, constituting less than 0.25% of all cardiac tumors, 1 but it has a lethal prognosis even if surgical resection, with or without adjuvant therapy, is performed. 2,3 Only a few cases of surgical resection of cardiac leiomyosarcoma have been reported. 4 We present a case of surgical resection of a primary cardiac leiomyosarcoma involving the pulmonary valve, which was reconstructed with a bioprosthetic valve and Xenomedica patch. Case ReportA 55-year-man was admitted to another hospital because of dry cough and exertional breathlessness that had rapidly worsened over the previous 1-2 weeks. He had no remarkable medical history. A computed tomographic (CT) examination of the chest revealed a filling defect that almost completely obstructed the main pulmonary arterial trunk.A diagnosis of pulmonary thromboembolism was assumed, and the patient was treated with catheter-directed thrombolysis therapy over 3 days. However, his dyspnea and shortness of breath worsened despite an optimal regimen of thrombolytic and anticoagulation therapy. The patient was referred to us for further evaluation and possible surgical management of the presumed pulmonary thromboembolism.Upon admission, laboratory results showed elevated lactate dehydrogenase (785 U/L) and liver enzymes (AST Circulation Journal Vol.69, January 2005 49 U/L, ALT 95 U/L). Chest X-ray showed mild cardiomegaly with a cardiothoracic ratio of 52%. The patient underwent magnetic resonance imaging (MRI) of the chest, which revealed a solid mass at the pulmonary valve, measuring approximately 50×40×30 mm with low signal intensity on T1-weighted image and increased signal intensity on T2-weighted image, that appeared to be invading the main pulmonary arterial trunk (Fig 1a,b). There was no evidence of distal thromboembolism in either of the pulmonary arteries. Because the mass appeared to be a tumor rather than a thrombus, the possibility of malignancy was raised. Transthoracic echocardiography disclosed a large mass involving the pulmonary valve and extending to the main pulmonary arterial trunk, enlargement of the right ventricle (end-diastolic diameter, 52 mm), and displacement of the ventricular septum toward the left ventricle. The mass almost completely obstructed the main pulmonary arterial trunk (Fig 1c), and right ventricular failure was suspected. The patient was scheduled to undergo surgery for tumor extirpation and histological evaluation of the mass.At surgery, a hard mass was palpated in the slightly dilated main pulmonary arterial trunk. Conventional cardiopulmonary bypass was established with ascending aortic perfusion and bicaval drainage through a median sternotomy. After the heart was arrested, the main pulmonary arterial trunk was incised longitudinally from just above the pulmonary valve to the bifurcation, whereupon a whitish tumor originating from the pulmonary valve was found to have almost completely blocked the lumen of the main pulmonary arterial trunk and invaded its anterior wall. We also o...

Huge Primary Pleomorphic Leiomyosarcoma in the Right Ventricle With Impending Obstruction of Both Inflow and Outflow Tracts

“…4,7,9-11 TEE, CT, and CMR offer additional information about the size, location, attachment, and local spread of the tumor. 4,7,[9][10][11] However, it is not easy to discriminate between malignant and benign cardiac tumors before histological examination. 5,7 Recently, PET-CT has been a preferred method to use to evaluate the local invasion and distant metastasis of malignant cardiac tumors.…”

Primary sarcoma of the right ventricle: surgical and adjuvant therapy

“…Pulmonary valvular function has been reconstructed using a bioprosthetic valve. 11,12 We implanted a stentless valve instead of a mono-cusped biomaterial patch to obtain competent pulmonary valvular function because the patient needed suffi cient valvular function to maintain pulmonary blood fl ow against pulmonary hypertension resulting from distal tumor microembolization. Also, patch enlargement of the pulmonary arteries, which would delay pulmonary artery reobstruction by the tumor growth, seemed effective in lengthening the survival of our patient.…”