Ann Thorac Surg 2008;85:2125-2126. doi:10.1016/j.athoracsur.2007.11.080
© 2008 The Society of Thoracic Surgeons
Case Reports
Novel Strategies for Recurrent Cardiac Myxoma
Kaushalendra Singh Rathore, MCh,
Saleem Hussenbocus, MBBS,
Robert Stuklis, FRACS,
James Edwards, FRACS*
Department of Cardiothoracic Surgery, Royal Adelaide Hospital, Adelaide, Southern Australia, Australia
Accepted for publication November 29, 2007.
* Address correspondence to Mr Edwards, Department of Cardiothoracic Surgery, Royal Adelaide Hospital, North Terrace, Adelaide, Southern Australia, 5000, Australia (Email: patricia.smith{at}health.sa.gov.au).
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Abstract
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A 21-year-old man presented with a stroke. Subsequent investigations revealed cardiac tumors in his left atrium and right ventricle. These were removed by conventional techniques. Histopathology confirmed them as myxomas. He had a complete neurologic recovery, but presented 2 years later with recurrence of a cardiac myxoma in his left ventricle and left atrium. This time, however, endoscopically assisted techniques were used. The tumor was excised with supplementary cryoablation. The patient made a quick recovery from this second procedure and has had no recurrence after a 1-year follow-up.
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Introduction
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The incidence of primary cardiac tumors varies from 0.02% to 0.28% [1], and they are one of the least common causes of cerebrovascular accidents [2]. Almost all of these tumors are sporadic benign myxomas, of which 7% are recurrent tumors [2]. The case presented here is of a young patient with recurrent myxomas who underwent endoscopically assisted excision of the recurrent tumors with supplementary cryoablation.
A 21-year-old man with no significant past medical history presented to the emergency department after experiencing a sudden collapse. A head computed tomography (CT) scan revealed a right middle cerebral artery territory infarction, which recovered partially after thrombolytic therapy. He was admitted, and subsequent investigations revealed a high erythrocyte sedimentation rate of 80 mm/h and raised level of C-reactive protein of 260 IU.
A thoracic CT scan revealed an 8.3- x 8.1-cm mass in the left atrium and 4.9- x 3.1-cm mass in the right ventricle. Echocardiography showed the origin of the left atrial mass was from the interatrial septum and the right ventricle mass was from the free wall. Chest roentgenogram and electrocardiography were unremarkable. A whole-body CT scan showed no other tumor. Although the patient had multiple nevi and lentigines on his face and shoulders, none of his family members had any pigmented cutaneous lesions or history of cardiac or extracardiac tumors.
Surgical excision was performed through median sternotomy, standard aortobicaval cardiopulmonary bypass, moderate hypothermia, and cold blood cardioplegia. The tumor was approached through the right atrium. The tumor was a pedunculated mass sitting on interatrial septum. The whole tumor was excised with the interatrial septum, and the interatrial septal defect was closed with autologous pericardial patch. The right ventricular tumor was excised through the tricuspid valve. Intraoperative transesophageal echocardiography (TEE) revealed no residual tumor. Histopathology confirmed benign myxoma.
The patient remained under routine surveillance. After 2 years, echocardiography revealed a 3- x 3-cm mass in his left ventricular apex, which was subsequently confirmed on magnetic resonance imaging (Fig 1). The patient was asymptomatic and had recovered completely from his past stroke.
The approach for the redo surgery was through a right anterolateral minithoracotomy (4.5-cm incision) using long-shafted instruments (Geister Medizintechnik GmbH Tuttlingen, Leipzig, Germany). The patient was supine, with the sand bag under the right side of the chest. Cardiopulmonary bypass was established by femoral artery–femoral vein–internal jugular vein cannulation. The right femoral artery and vein was cannulated with the Seldinger technique under TEE supervision using a 20F Fem-Flex II arterial cannula and 28F multistage venous cannulas (Edwards Lifesciences LLC, Irvine, CA). The right internal jugular vein was cannulated with 17F Fem-Flex II arterial cannula. One 10-mm port was created in the third intercostal space, the video thoracoscope (Karl Storz GmbH, Tuttlingen, Germany) was inserted, and carbon dioxide delivery was started at 4 L/min through the port.
Owing to extensive adhesions resulting from the first operation, the ascending aorta was not dissected and not cross-clamped. The heart was left perfused and beating throughout the procedure, with the operating table in a downward head-tilt position to minimize the risk of air embolism. The left ventricle was accessed through a 2-cm incision just inferior to the interatrial groove, and a thorascopic video system (Storz) was passed through the mitral valve. A 3.5- x 2.5-cm tumor was present at the apex, which was excised. A small recurrence was noted in the left atrium and was similarly excised. No other tumor mass was found anywhere in the cardiac chambers.
The base of the left ventricle and left atrium tumors was cryoablated using the SurgiFrost 10-cm surgical cryoablation system (CryoCath, Montreal, Quebec, Canada). A cryoablation probe was placed for 1 minute in contact with the tumor base, and the local temperature dropped to –110°C.
Intraoperative TEE revealed no residual tumor. Histology revealed benign myxomas. The patient's postoperative period was uneventful, and the tumor has not recurred to date at 12 months' follow-up.
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Comment
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Seven percent of all myxomas exhibit a familial pattern of disease as part of a complex neoplastic syndrome, known as Swiss syndrome; Lentigines, Atrial myxomas, Mucocutaneous myxomas, and Blue nevi (LAMB), or Carney complex, that is caused by a mutation in the gene encoding the R1-
regulatory subunit of cyclic adenosine monophosphate-dependent protein kinase A (PRKAR1), and located on the chromosome 17q2. These are transmitted in an autosomal dominant pattern. These patients present with spotty pigmentation (LAMB), nonmyxomatous extracardiac tumors like pituitary adenomas, Sertoli cell tumors, and breast fibroadenomas. Although Carney complex myxomas exhibit a predilection for the left atrial aspect of the fossa ovalis, nearly one-third of these tumors occur in ventricles or other atrial sites. They usually recur in the initial 4 years after excision and up to five recurrences over a lifetime are described [3]. In our patient, genetic testing of the tumor for the Carney complex was negative, which raises the possibility of a spontaneous mutation in a gene other than the PRKAR1 gene.
Endoscopically assisted surgery is an exciting technique that is now being used for mitral valve repair as well as for coronary artery procedures [4]. In addition, there are few case reports in the literature of its use for tumor resection [5]. Although the use of cryoablation in hepatic and gastrointestinal tumors is well documented, its use for cardiac tumors is still in its infancy [6]. We used it as an adjunct to the resection.
Minimally invasive endoscopically assisted surgery offers a new angle to approach the heart. Its advantages include less cardiac trauma, leading to fewer adhesions and faster recovery [7]. The Seldinger technique for arterial and venous cannulation under echocardiographic guidance leads to better placement of cannulas and results in optimal bypass conditions. Although endoscopically assisted cardiac surgery is still in its infancy, it has become standard at some centers for isolating mitral valve lesions, pulmonary vein isolation for atrial fibrillation, and simple congenital defects [8]. We describe its use to deal with a group of cardiac tumors noted for their recurrence. More work needs to be done to prove the reliability and safety of these techniques.
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References
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Abstract
Introduction
