Left Ventricular Outflow TractObstruction Due to Valve MyxomaIngeborg Keeling, MD, Peter Oberwalder, MD, HelmutSchuchlenz, MD, Michael Anelli-Monti, MD, and BrunoRigler, MD

Division of Cardiac Surgery, Departments of Surgery, andMedicine, Karl Franzens University of Graz, Graz, Austria

A life-threatening left ventricular outflow tract obstruc-tion developed in a 26-year-old man because of a veryuncommon myxoma emerging from the ventricular sideof the mitral valve. Immediate surgical treatment wasindicated because of high-risk factors reported herein.After transaortic resection of a single myxoma, theprogress of this patient has been excellent, especially asa familial myxoma could be excluded.

(Ann Thorac Surg 2000;69:1590–1)© 2000 by The Society of Thoracic Surgeons

Intracardiac myxoma is the most common tumor of theheart, with an estimated incidence of 0.5 per million

population per year [1]. The symptoms vary greatly,depending on the size and localization of the tumor. Upto 80% of myxomas are localized in the left atrium,whereas myxomas emerging from valve tissue are rarelyfound [2, 3]. A case of a mitral valve myxoma is discussed.

A 26-year-old man was admitted after a cardiac myxomahad been detected during preventive medical checkup.The patient was completely asymptomatic. Physical ex-

amination showed a variable diastolic murmur that de-creased in the supine position. Laboratory investigationsshowed normal levels with respect to coagulation vari-ables, antinuclear factors, and cardiolipin antibodies.Electrocardiogram showed normal sinus rhythm at 70beats/minute, indifference type, and a negative T wave inlead III. The result of a chest roentgenogram study wasnormal. Transesophageal echocardiography revealed anintracardiac tumor at the ventricular-sided basis of theanterior mitral valve leaflet resulting in a systolic pro-lapse through the aortic valve and a 50% obstruction ofthe left ventricular outflow tract (Fig 1). Magnetic reso-nance imaging of the neurocranium excluded asymptom-atic microembolisms of the brain. A familial occurrenceof the disease was excluded in first-grade relatives of thepatient.

A transaortic resection of a mitral valve myxoma,emerging from the anterior mitral valve leaflet, wasperformed under extracorporeal circulation and moder-ate hypothermia by way of a median sternotomy. Intra-operatively, after dissection of the left atrium and trans-verse aortotomy, the huge broad-based myxoma, 4 cm indiameter, became visible, as it nearly reached the supra-valvular area of the ascending aorta. Transaortic inspec-tion revealed that the relatively soft tumor arose with abroad basis from parts of the anterior papillary muscleand from the backside of the anterior mitral valve leaflet.The myxoma of this patient arose from the ventricularside of the mitral valve leaflet, which is extremely rare,and not as usual from the left atrial wall. Macroscopically,the gelatinous tissue appeared as yellow-brownish toyellow-greenish myxoid stroma. Tumor resection wasfollowed by inspection of both atria for more myxomatissue. Histopathologic examination revealed prolifera-tions of capillaries, blood extravasations, and dissemi-nated fibrin depositions. These findings were consistentwith the diagnosis of a myxoma. Intraoperative echocar-diography showed normal mitral valve function and ahemodynamically insignificant aortic insufficiency grade

Accepted for publication Sep 29, 1999.

Address reprint requests to Dr Keeling, Division of Cardiac Surgery,Department of Surgery, Karl Franzens University of Graz, Auenbrugger-platz 1, 8010 Graz, Austria; e-mail: ingeborg.keeling@kfunigraz.ac.at.

Fig 1. (A, B) Preoperative transesophageal echocardiograms of a mitral valve myxoma at the ventricular-sided basis of the anterior mitralvalve leaflet resulting in obstruction of the left ventricular outflow tract.

1590 CASE REPORT KEELING ET AL Ann Thorac SurgMITRAL VALVE MYXOMA 2000;69:1590–1

© 2000 by The Society of Thoracic Surgeons 0003-4975/00/$20.00Published by Elsevier Science Inc PII S0003-4975(00)01182-6

I. The postoperative course of the patient was uneventful,and after 13 months of follow-up he was free fromevidence of recurrence of disease.

Comment

We report on a case of an asymptomatic patient with amyxoma that originated from the left-ventricular-sidedaspect of the mitral valve and that required immediateresection. Furthermore, the myxoma required immediateresection as with future tumor growth this myxomathreatened valve destruction and a left ventricular out-flow tract obstruction. The patient was not only at anincreased risk for sudden cardiac death [4], but also at ahigher risk of embolization, which occurs in 30% to 43%of these patients [2, 3]. This higher risk of embolizationcan be due to the motion of the valve leaflets or from thehigh pressure within the left ventricle during systole [3].Cardiac myxomas seem to recur more often in youngmen, in patients with multifocal origins, and in up to 40%of patients who have a family history of the tumor [2, 5].The prognosis for patients with solitary myxomas aftersurgical resection is excellent as evidenced by the lowhospital mortality rate [2]. Late recurrences have beenreported to occur in 0.4% to 5% of surgically treatedpatients 3 months to 22 years after operation [2]. In thoserare instances in which the tumor arises from an atrio-ventricular valve, the valve occasionally requires valvu-loplasty or even replacement. We decided to remove thetumor through the aortic root to avoid the higher risk ofintraoperative embolization. This approach facilitatedthe exposure of the left-ventricular-sided aspect of themitral valve apparatus. Despite the broad basis by whichthe myxoma originated from the mitral valve, the valvecould be completely preserved. The transesophagealechocardiographic follow-up at 12 months revealed triv-ial mitral valve dysfunction without evidence of tumorrecurrence. The course of our patient indicates that themany risks inherent in this disease may be kept low byaiming at early diagnosis with immediate surgical treat-ment. As this disease may mimic a great variety of othercardiac diseases, its possible presence should always beconsidered, as relatively low-risk cardiac surgical treat-ment can be performed with an excellent postoperativeprognosis for most patients.

References

1. MacGowan SW, Sidhu P, Aherne T, et al. Atrial myxoma:national incidence, diagnosis and surgical management. IrJ Med Sci 1993;162:223–6.

2. Castells E, Ferran V, Octavio de Toledo MC, et al. Cardiacmyxomas: surgical treatment, long-term results and recur-rence. J Cardiovasc Surg 1993;34:49–53.

3. Chakfe N, Kretz JG, Valentin P, et al. Clinical presentationand treatment options for mitral valve myxoma. Ann ThoracSurg 1997;64:872–7.

4. Vassiliadis N, Vassiliadis K, Karkavelas G. Sudden death dueto cardiac myxoma. Med Sci Law 1997;37:76–8.

5. McCarthy PM, Schaff HV, Winkler HZ, Lieber MM, CarneyJA. Desoxyribonucleic acid ploidy pattern of cardiac myxo-mas. J Thorac Cardiovasc Surg 1989;98:1083–6.

Emergency Ligation of AnomalousLeft Coronary Artery Arising Fromthe Pulmonary ArteryChristian Kreutzer, MD, Andres J. Schlichter, MD,Maria Ines Roman, MD, and Guillermo O. Kreutzer,MD

Division of Cardiovascular Surgery, Ricardo GutierrezChildren’s Hospital, Buenos Aires, Argentina

We report two cases of successful emergency ligation ofanomalous left coronary artery arising from the pulmo-nary artery (ALCAPA) in patients with previous cardiacarrest. Both patients had regained marginal cardiac out-put after cardiopulmonary resuscitation and had maxi-mal doses of inotropic support. The ALCAPA ligationwas then performed as a life-saving procedure in theabsence of any kind of mechanical circulatory support.

(Ann Thorac Surg 2000;69:1591–2)© 2000 by The Society of Thoracic Surgeons

Anomalous left coronary artery arising from the pul-monary artery (ALCAPA) is a rare anomaly, with an

estimated incidence of 1 in 300,000 live births. Thisanomaly produces a coronary steal phenomenon, left-to-right shunt, and commonly, significant left ventriculardysfunction with mitral regurgitation from abnormalperfusion of the left ventricle. Different surgical ap-proaches have been reported including ligation of theanomalous left coronary artery in its origin [1], subclavi-an-left coronary anastomosis [2], transpulmonary bafflingor Takeuchi procedure [3], and direct reimplantation ofthe coronary artery in the aorta [4]. The use of leftventricular assist device (LVAD) in this anomaly had atremendous impact on surgical mortality. In recent seriespostoperative LVAD was used with significant reductionin mortality [5, 6]. We describe two cases in whichemergency ligation of the anomalous coronary artery wasperformed in the intensive care unit (ICU) in 2 patients incardiac arrest or severe low output and hypotensionwhen extracorporeal circulatory support (extracorporealmembrane oxygenation [ECMO] or LVAD) wasunavailable.

Case Reports

Patient 1In June 1979, a 5-month-old girl was admitted because ofsevere congestive heart failure with dilated cardiomyo-pathy. The diagnosis of ALCAPA was suspected with the

Accepted for publication Oct 1, 1999.

Address reprint requests to Dr Christian Kreutzer, Ricardo GutierrezChildren’s Hospital, Gallo 1330 (1425) Buenos Aires, Argentina.

1591Ann Thorac Surg CASE REPORT KREUTZER ET AL2000;69:1591–2 LIFE-SAVING ALCAPA LIGATION

© 2000 by The Society of Thoracic Surgeons 0003-4975/00/$20.00Published by Elsevier Science Inc PII S0003-4975(00)01179-6