Ann Thorac Surg 2003;76:1935-1938
© 2003 The Society of Thoracic Surgeons
Original article: cardiovascular
Changing management of cardiac myxoma based on a series of 40 cases with long-term follow-up
Chekir Selkane, MDa,
Brahim Amahzoune, MDa,
Nicolas Chavanis, MDa,
Olivier Raisky, MDa,
Jacques Robin, MD, PhDa,
Jean Ninet, MDa,
Jean F. Obadia, MD, PhDa*
a Hôpital Cardiothoracique, Lyon, France
Accepted for publication May 2, 2003.
* Address reprint requests to Dr Obadia, Hôpital Cardiothoracique, BP Lyon-Monchat, 69394 Lyon, Cedex 03, France.
e-mail: jf.o{at}chu-lyon.fr
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Abstract
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BACKGROUND: Cardiac myxoma is generally considered to be a surgical emergency. However, as a result of progress in echocardiography and the increasing age of the patients presenting with this disease, the clinical presentation has changed and the management of cardiac myxoma now needs to be reviewed.
METHODS: Between 1978 and 2001, 40 patients (16 men and 24 women) between the ages of 6 months and 82 years (mean age, 55.6 years) were operated on for cardiac myxoma. Signs of heart failure with pulmonary congestion (22%) or pulmonary embolism (20%) indicated a high-risk emergency situation in some cases, whereas, in other cases (58%), the patient's condition was stable and the clinical presentation was less worrying. However, the tumor was always removed within 24 hours of admission. Most cases of cardiac myxoma observed over the last decade correspond to stable forms, as echocardiography has revealed smaller tumors in generally elderly patients.
RESULTS: The postoperative mortality was 7.5% (3 patients). No patients were lost to follow-up, and the mean follow-up was 13.6 years. One patient was reoperated for recurrence 3 years postoperatively. Five patients required further cardiac surgery: three mitral valve replacements, one coronary artery bypass graft, and one angioplasty. The 15-year survival rate was 69%.
CONCLUSIONS: Myxoma tends to be observed in a more elderly and higher risk population, often at an early stage. The classic approach of emergency surgery is not always appropriate in these stable forms, allowing more thorough preoperative assessment of these patients.
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Introduction
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Cardiac myxoma is usually considered to be a definite indication for urgent surgery. This is obviously true in certain symptomatic forms, such as cases presenting with embolism or heart failure. However, the development of echocardiography now allows more precise diagnosis, so that these severe forms are in now less frequently observed. We therefore wanted to determine whether, on the basis of our own experience and a review of the literature, this aggressive attitude is still appropriate.
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Material and methods
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Between September 1978 and September 2001, 40 patients (16 men and 24 women) between the ages of 6 months and 82 years (mean age, 55.6 years) were operated on for cardiac myxoma. Signs of heart failure with severe dyspnoea due to pulmonary congestion (22%) or pulmonary embolism (20%) indicated a high-risk emergency situation. Five of the eight cases with embolism presented cerebral emboli with minor stroke and no hemorrhagic complications on computed tomography (CT) scan and surgery was performed without delay. However, the other cases (58%) presented less worrying clinical features with progressively deteriorating breathlessness on exertion (26%), cardiac arrhythmia (15%), general signs (11%), or angina (6%). The distribution of these various acute and subacute clinical forms changed over time, as shown in Table 1. None of the patients was completely asymptomatic and none of them had a family history of myxoma.
Diagnosis
Transthoracic echocardiography indicated a diagnosis of myxoma in every case and allowed morphologic examination of the whole heart. The myxoma was situated in the left atrium in 34 cases, the right atrium in four cases, involved both atria in one case and the left ventricle in one case.
The tumor was implanted septally in 34 cases, on the right side in four cases, in the mitral valve in three cases, in the tricuspid valve in one case, in the pulmonary vein in one case, and on the posterior papillary muscle in one case.
The tumor diameter was between 2 and 5 cm in 58% of cases, between 5 and 10 cm in 29% of cases, and less than 2 cm in 13% of cases. Transthoracic echocardiography also demonstrated left ventricle function and absence of myxoma in the other cavities. Transoesophageal echocardiography was also performed in eight cases, particularly when there was a doubt about endocarditis or thrombus. Four patients underwent coronary angiography. Nine patients presented signs of mitral valve insufficiency, classified as grade III in four cases.
Surgery was indicated with varying degrees of urgency according to the clinical features, but was always performed within 24 hours of admission, even in the least severe clinical forms.
Dynamic interleukin 6 (IL-6) assay was performed only in the last patient of this series. In this patient, presenting with general symptoms, plasma IL-6 level was abnormally elevated before surgery (22 pg/L) and returned to normal values (< 5 pg/L) 2 days after surgery.
Surgical technique
A thoracic approach was used: 37 medial sternotomies and three right video-assisted mini-thoracotomies. Surgery was performed under normothermia (13 cases) or moderate hypothermia (27 cases), with fibrillating heart in four cases, beating heart in two cases, and under cardioplegia in 34 cases. Mean aortic cross-clamp time was 21 minutes (range, 9 to 75 minutes). Access to the heart was obtained by left atriotomy through the atrial groove in 31 cases, right atriotomy in two cases, and a transseptal approach in seven cases. Simple tumor resection with removal of the tumor and its pedicle was performed in 32 cases. In eight cases, tumor resection was extended to the tumor implantation base, requiring partial resection of the atrial septum followed by reconstruction using a Dacron patch. The posterior leaflet had to be temporarily detached to provide access to a left ventricle tumor situated on the posterior papillary muscle and tucked underneath the posterior leaflet between the chordae. Associated procedures consisted of one case of repair of atrial septal defect by simple suture, one mitral valve annuloplasty, one commissurotomy, and four prosthetic mitral valve replacements.
Data analysis
Survivors were followed by means of questionnaire and by telephone. Causes and dates of death were ascertained from official French registers. Survival rates were calculated using the Kaplan-Meier method [1].
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Results
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Histologic examination of the specimen confirmed the diagnosis of myxoma in every case. There were three immediate postoperative deaths (7.5%): two on day 1 and one on day 10 (one mitral ring rupture in a 6-month-old infant, one cerebral tumor embolism, and one case of septic shock in a patient with preoperative bacterial meningitis). Immediate results were favorable in the other 37 patients. The immediate follow-up was particularly favorable in terms of comfort and hospital stay in the 3 patients operated through a minimally invasive approach, although no conclusions can be drawn from this small series with short follow-up. No patients were lost to follow-up. Mean follow-up was 13.6 years, with a range of 1 to 34 years and a cumulative total of 465 years. Only 1 patient required surgery for recurrence, 3 years after the first operation; this patient did not present any subsequent recurrence and is still alive 13 years after the second operation. Five other patients required further cardiac surgery: three mitral valve replacements, one coronary artery bypass graft, and one coronary angioplasty. The 3 patients undergoing mitral valve replacement already presented grade 2 regurgitation soon after surgery. Unfortunately, intraoperative transoesophageal echocardiography was not performed in any of these patients in this old series. Eight patients subsequently died from noncardiological causes; 2 at 1 year, 1 at 2 years, 4 at 5 years, and 1 at 11 years. The other 29 patients are still alive and asymptomatic at last follow-up. The overall 15-year survival rate was 69% (Fig 1).
In a subgroup of 14 patients over the age of 65, the 5-year survival rate was only 47%.
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Fig 1. Survival rate (solid line) and freedom from reoperation (dotted line). There are 25 patients at risk at 5 years, 20 at 10 years, and 11 at 15 years.
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Comment
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A large number of papers on cardiac myxoma have been published in the literature, with 1,202 references in Pub Med between 1965 and 2002. Most of these publications have been case reports [2–5]. The histologic diagnosis is straightforward [6, 7], but the subject of discussion concerns the risk of recurrence after surgery [8]. This risk has now been clearly identified and is correlated with young age, a family history of myxoma [9, 10], and multifocal lesions. More recently, IL-6 [11–13] and endothelial growth factor [14] have been identified as markers for these tumors. Our own series comprised few of these risk factors. Relatively noninvasive surgical techniques were used, with simple resection in 87% of patients (ie, avoiding large resection of the atrial septum below the tumor implantation). Extensive resection therefore appears to be mainly justified in high-risk cases [15]. Our one case of recurrence, not presenting any particular risk factors, was probably due to incomplete resection.
However, our series comprised a significant number (6/40) of concomitant mitral valve procedures, plus another three cases of secondary mitral valve replacement. Similar results were reported in another series of 22 patients [16] with two first-line and two secondary mitral valve replacements. Kabbani and associates[17] reported four mitral valve replacements in a series of 24 tumor resections and Semb [18] reported four cases of mitral valve insufficiency in a series of 11 patients and one tricuspid valve replacement in two cases of right cardiac myxoma. The valves should therefore be very thoroughly examined during initial surgery to lower the reoperation rate. A conservative mitral procedure, such as annuloplasty, would no doubt be feasible as part of the first step, and transoesophageal echocardiography should be systematically performed. In this abundant literature [15, 19], the classic attitude of emergency management of cardiac myxoma is rarely questioned [20]. This approach is perfectly logical in the case of acute symptomatic forms, such as heart failure with pulmonary edema, or embolism [21]. It is also justified in high-risk forms in stable patients presenting threatening echocardiographic images: a clapper-shaped tumor intermittently prolapsing into the mitral orifice, or a large, multilobed tumor suggesting a risk of embolism. However, emergency management appears to be much less clearly indicated in stable patients, in whom the only real risk is that of embolism [22], as the risk of embolism is probably low for tumors less than 2 cm in diameter. All 8 patients in our series presenting with embolism had tumors larger than 3 cm. There is little mention in the literature about the correlation between size and risk of embolism, but, to the best of our knowledge, no case of embolism has ever been reported for small tumors. Such small tumors are no longer rare with the development of echocardiography (Table 1), which questions the dogma of emergency surgery, proposed at a time when acute symptomatic forms were the most frequent. Moreover, cardiac myxoma, initially described as a disease of early adulthood or middle age, is now increasingly diagnosed in sometimes very elderly patients (Fig 2).
This trend also applies to cardiac surgery in general, which tends to concern an aging population with specific problems. In these older patients with a higher operative risk, more delayed surgery allows time for a more complete preoperative assessment, including more systematic coronary angiography. With the exception of real emergency situations, there is no reason why surgery for cardiac myxoma should not comply with the usual recommendations for preoperative coronary angiography before any form of cardiac surgery. Coronary angiography was performed in only 4 patients with a history of angina in this old series. In the 14 patients over the age of 65 years, two coronary revascularisations were performed, 6 months and at 2.5 years after tumor resection, but these cases could have been detected by preoperative coronary angiography. For all of these reasons, we therefore consider that emergency surgery is only indicated in patients with severe dyspnoea and presenting a high risk of embolism (ie, with a history of embolism or with large, mobile tumors on echocardiography). In all other cases, preoperative assessment should be performed to allow better patient preparation and to detect any associated coronary or valvular heart disease. This assessment should only take 1 or 2 days, which does not constitute a high risk for stable patients, but allows surgery to be performed under better conditions, particularly in elderly patients. Finally, in some exceptional cases in very debilitated patients, surgery may even be unnecessary, as illustrated by the case of an 86-year-old patient with a nonoperated small myxoma, who was followed for 4 years without incident [20].
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Fig 2. Comparative age distribution of 21 patients operated on between 1978 and 1989, and 19 patients operated on between 1990 and 2001.
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Conclusion
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The circumstances of discovery of cardiac myxoma have changed over recent decades. They are more often detected at an early stage, but also in a more elderly and higher-risk population. Emergency surgery is still appropriate in patients with severe dyspnoea or a high risk of embolism, but in all other cases, a more complete preoperative assessment is recommended to allow better patient preparation and to detect any associated coronary or valvular heart disease. Surgery could therefore be more frequently performed with a minimally invasive approach with video-thoracoscopy.
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Acknowledgments
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The authors thank François Gueffier for statistical analysis.
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Abstract
Introduction
