Ann Thorac Surg CASE REPORT KENNEDY ET AL 1221 1995;59:1221-3 AORTIC VALVE MYXOMA

6. Sievers HH, Leyh R, Loose IL Guha M, Petry A, Bernhard A. Time course of dimension and function of the autologous pulmonary root in the aortic position. J Thorac Cardiovasc Surg 1993;105:775-80.

7. Ross DN, Jackson M, Davies J. The pulmonary autograft. A permanent aortic valve. Eur J Cardiothorac Surg 1992;6:113-7.

8. Daenen W, Gewillig M. Extended aortic root replacement with pulmonary autografts. Eur J Cardiothorac Surg 1993;7: 42-6.

Myxoma of the Aortic Valve Peter Kennedy, MA, Andrew J. Parry, FRCS, Dinah

Parums, FRCPath, and Ravi Pillai, FRCS

Department of Cardiothoracic Surgery, Oxford Heart Centre, John Radcliffe Hospital, Oxford, England

Myxomas are one of the most c o m m o n cardiac tumors but usual ly are confined to the atria. Al though described in all cardiac chambers and on all cardiac valves, only one postmortem case of an aortic valve myxoma has been publ i shed. We present the case of a young man wi th an aortic valve myxoma wi th peripheral embol izat ion. The other pathologic condit ions wi th which valvular myxo- mas can be confused are compared and discussed.

(Ann Thorac Surg 1995;59:1221-3)

A l though myxomas are the most commonly described tumors of the heart, they usually are confined to the

atria, more commonly the left atrium, or the ventricles. Myxomas of the cardiac valves "are rarely, if ever, found" [1]. Indeed, we have found only one postmortem report of an aortic valve myxoma [2]. Here we report the case of a patient with aortic valve myxoma that presented as acute claudication of the lower l imb and was successfully treated surgically.

A 23-year-old man had sudden onset of crippling calf pain. The pain disappeared after 4 hours of rest but after this episode, he complained of bilateral intermit tent claudication at 180 m. This was associated with tingling in the thighs but no buttock pain. There was no relevant medical history. Examination showed absent pulses dis- tal to the femoral arteries. In addition, he was found to have a to-and-fro m u r m u r at the left sternal edge radi- ating to the neck. He had no stigmata of bacterial endo- carditis, and all blood cultures and markers of acute inflammation were negative.

Peripheral arteriography demonstrated complete oc- clusion of both common femoral arteries with filling of the distal vessels by way of collaterals. Echocardiography confirmed the presence of moderate aortic regurgitation with a large mass on the right coronary cusp of the aortic valve (Fig 1). Hematologic and immunologic test results for au to immune disease were all negative except an

Accepted for publication Oct 12, 1994.

Address reprint requests to Mr Pillai, Oxford Heart Centre, John Radcliffe Hospital Headley Way, Headington, Oxford OX3 9DU, England.

B

Fig 1. (A, B) Preoperative echocardiograms clearly show the large mass (arrow) attached to the right coronary cusp of the aortic valve.

incidental f inding of ant i thrombin III deficiency with a titer of 56% of normal.

After administrat ion of ant i thrombin |II immediately prior to operation, the patient underwent an uneventful aortic valve replacement using a mechanical prosthesis. At operation, a myxomatous mass, which fused the right coronary and noncoronary leaflets of the aortic valve, was found (Fig 2). The valve was excised and sent for histo- logic examination.

The patient had an uneventful postoperative course and was anticoagulated with warfarin sodium. He also made an excellent recovery symptomatically from the peripheral vascular perspective. Because he felt so we l l despite advice that further intervent ion was essential, he declined operation to treat the femoral emboli.

Histologic examination of the mass confirmed a myx- oma of the aortic valve (Fig 3). The valve leaflet studied measured 4 × 1 cm and was noted to have a central soft nodule measur ing 1.5 cm in diameter. On histologic study, the valve leaflet was th inned and partially re- placed by granulat ion tissue elements. The large nodule seen macroscopically was acute thrombus. Special stains revealed no evidence of fungal or bacterial organisms.

© 1995 by The Society of Thoracic Surgeons 0003-4975/95/$9.50 0003-4975(94)00969-E

1222 CASE REPORT KENNEDY ET AL Ann Thorac Surg AORTIC VALVE MYXOMA 1995;59:1221-3

Fig 2. Macroscopic view of the excised valve. The large myxomatous mass can be seen bridging the right and noncoronary leaflets of the valve. The mass measured 4 x 1 cm.

Within the inf lamed valve tissue were focal myxoid areas, though the bulk of the myxoma had become detached.

C o m m e n t

The clinical diagnosis of cardiac myxoma is often difficult because of the many and various ways in which these tumors can present . Often they are discovered after serious complicat ions such as per iphera l embolizat ion have occurred, even though consti tutional symptoms may have been present for a period. Consequent ly the diagnosis is often made only post mortem.

Since the first successful resection of an atrial myxoma by Crawford in 1954 [3], numerous reports of successful operat ive removal have been publ ished. Most f requent ly these have involved the hear t cavities, but t r icuspid and mit ra l valve myxomas have also been successful ly t rea ted surgically [4]. It is c la imed that valvular and nonvalvular myxomas are histologically different, but any rnorphologic variat ions be tween them can also occur among myxomas originat ing in the atria [2]. However, it can general ly be said that valvular myxomas tend to have fewer cell clusters, fewer syncytial tumor giant cells, and

Fig 3. Histologic section through the tumor. Note the stellate cells arranged singly and in clusters embedded in a mucoid matrix. (He- matoxylin and eosin; xs0.)

less f requent per ivascular cuffing of tumor cells than atrial myxomas.

Various claims regarding aortic valve myxomas have been made, but these are usual ly confusions with three other pathologic conditions. In children, a group of valvular deformit ies that have er roneously been labe led myxomas is recognized. They represent the pers is tence of a deve lopmenta l stage when the valve tissue is com- pletely composed of myxoid tissue, and histologically the valve has general ized myxomatous thickenings ra ther than discrete tumors [5]. They thus represent hamar- tomatous malformat ions ra ther than true myxomas.

After the first year of life, the aortic valve can develop small tags known as Lambl 's excrescences caused by the organizat ion of fibrin deposi ts on the valve surface. Histologically they resemble myxomas, as they have a central fibrous connective tissue core with profuse elastin fibers, and an endothel ia l cell covering cont inuous with that of the cusp. However , they are usual ly asymptomat ic and are found as pos tmor tem findings I6]. They are normal ly 0.1 to 0.3 cm in d iameter and rarely exceed I cm in length. They therefore form a very specific group of tumors that can be precisely recognized by their macro- scopic and microscopic appearance . They bear a close resemblance to papil l i ferous tumors but appear to be a dist inct group, though a matura t ion from one to the other is certainly possible.

Papil l iferous tumors are well recognized as occurring on the hear t valves, especial ly the aortic valve. Though usual ly asymptomatic , these tumors can cause symptoms when large and mobile and in a posi t ion to occlude the coronary ostia [7]. Their histologic appearance , however, is classic. They are papil l i ferous and peduncu la ted tu- mors, the villi of which consist of a core of dense fibrous tissue (containing elastic fibers) with sur rounding myxo- matous tissue. The myxomatous t issue is loose and cel- lular with a mucopolysacchar ide ground substance, but it contains no iron. A flat tened epithel ial cell layer covers the surface. This differs from myxomas, which have an abundan t fibrillary mucoid matrix with stellate or poly- hedra l cells a r ranged singly or in small clusters that may mimic g landular s tructures [2]. Usually, syncytial cells can be seen. Evidence of hemorrhage is often present with hemos ider in - l aden macrophages . The surface is a single layer or focally mul t ip le layers of endothel ia l cells. There is no fibrous core, but in some cellular myxomas, inter l inking r ibbons of closely packed myxoma cells can produce a superficial resemblance to papi l l i ferous tu- mors. The origin of papil l i ferous tumors remains ob- scure; some favor an inf lammatory origin, whereas others claim they are unusual ly large Larnbl 's excrescences. However, these tumors bear no resemblance ei ther mac- roscopically or microscopical ly to cardiac myxomas, or more specifically to the tumor in our patient.

It is c la imed that after embolizat ion, there is a risk that the lodged tumor may start growing as a hematogenous metastasis and for this reason, the emboli should be removed. Local recurrence is well documented , but this may reflect a biologically more aggressive subgroup of tumors [8]. Distal embol izat ion is common, but there is d i sagreement whether true atrial myxomas can start

Ann Thorac Surg CASE REPORT YONAN ET AL 1223 1995;59:1223-6 LUNG HERNIATION AFTER HLT

growing at the site of impaction. Rather, it has been argued that all "metas tas iz ing myxomas" are, in fact, mal ignant tumors with such histologic characterist ics present in the pr imary tumor. Our pat ient had such a good symptomat ic recovery from the per iphera l emboli- zation that, though the risks of leaving the embol i in situ were explained, fur ther surgical in tervent ion was de- clined. Three years after the initial presentat ion, there has been no evidence of per iphera l recurrence. The case of this pat ient raises the quest ion whether fur ther oper- ation is requi red if the histology of the p r imary tumor is beyond question.

References

1. Becker AE, Anderson RH, eds. Tumours of the heart and pericardium. In: Cardiac pathology: an integrated text and color atlas. Edinburgh: Churchill Livingstone, 1983:8.3.

2. Wold LE, Lie JT. Cardiac myxomas. Am J Pathol 1980;101: 219-40.

3. Crawford C. Discussion on late results of mitral commissur- otomy. In: Lain CR, ed. International symposium on cardio- vascular surgery. Philadelphia: Saunders, 1955:202-11.

4. Lazzara RR, Park SB, Magovern GJ. Cardiac myxomas: results of surgical treatment. J Cardiovasc Surg (Torino) 1991;32: 824 -7.

5. Craig J. Congenital endocardial sclerosis. Bull Int Assoc Med Mus 1949;30:15-67.

6. Pomerance A. Papillary 'tumours' of the heart valves. J Pathol Bacteriol 1961;81:135-40.

7. Heath D, Best PV, Davis BT. Papilliferous tumours of the heart valves. Br Heart J 1961;23:20-4.

8. Read RC, White HJ, Murphy ML, Williams D, Sun CN, Flanagan WH. The malignant potentiality of left atrial myxo- mas. J Thorac Cardiovasc Surg 1974;68:857-68.

Right Lower Lobe Herniation After Domino Heart-Lung Transplantation Nizar A. Yonan, FRCS, Jim Egan, MRCP, Abdul K. Deiraniya, FRCS, and Ali N. Rahman, FRCS

Department of Cardiac Surgery and North West Lung Centre, Wythenshawe Hospital Manchester, England

We report 2 cases of trapping and incarceration of the right lower lobe in the left hemithorax after heart-lung transplantation with bicaval anastamoses (domino do- nors). This occurred despite confirmation of the normal anatomy at the time of implantation, before lung infla- tion. In I case this complication resulted in a right lower lobectomy 7 days after transplantation due to infarction and infection of the right lower lobe. These cases illus- trate the importance of reexamining the anatomy after lung inflation, before chest closure.

(Ann Thorac Surg 1995;59:1223-6)

Accepted for publication Sep 12, 1994.

Address reprint requests to Mr Yonan, The Transplant Centre, Wythen- shawe Hospital, Southmoor Rd, Manchester M23 9 LT, England.

H ear t - lung t ransplanta t ion (HLT) is an es tabl ished t rea tment for end-s tage lung disease [1]. A total of

26 HLT operat ions were per formed at our inst i tut ion be tween January 1990 and Apri l 1993. In 2 of the long- term survivors a complicat ion deve loped dur ing opera- tion wherein the right lower lobe hern ia ted beh ind the hear t and was par t ia l ly inflated in the left hemithorax. In 1 case, the complicat ion was recognized and corrected before chest closure. In the other case a lobectomy was pe r fo rmed on the seventh postopera t ive day. Both pa- t ients were domino donors.

Case Reports

Patient 1

A 23-year-old woman with cystic fibrosis underwent domino hear t and lung t ransplanta t ion in March 1991. Normal ana tomy was observed at the t ime of implanta- tion. There was no difficulty in weaning from bypass. However, before chest closure the surgeon not iced that the volume of the right lung was reduced and on further examinat ion, three lobes were observed in the left hemi- thorax. The right lower lobe had hern ia ted through a hiatus in the recipient pe r ica rd ium poster ior to the r ight phrenic nerve pedicle. The lobe had passed under the inferior vena cava and had become sandwiched be tween the recipient pe r ica rd ium poster iorly and the donor hear t anteriorly. The rest of the lobe was inflated in the lower left hemi thorax (Figs 1A, 1C). Normal ana tomy was res tored by deflating the hern ia ted lobe and manipula t - ing it back to its normal anatomic position. The lobe was reinflated when in the normal posi t ion with no further problems. This pat ient made an otherwise uneventful recovery. She is now alive and well 31/2 years after t ransplantat ion.

Patient 2

A 33-year-old woman with pr imary pu lmonary hyper ten- sion underwent domino HLT in June 1992. The normal ana tomy of each lung was observed at the t ime of p lacement of the hear t - lung block. The pat ient weaned from bypass easily, with satisfactory gases and stable hemodynamics . In the intensive care unit the pat ient b l ed excessively (200 to 400 mL/h) despi te correction of a coagulat ion abnormal i ty and the adminis t ra t ion of apro- tinin (Trasylol; Bayer AG, Leverkusen, Germany). The pat ient was re tu rned to the operat ing room 8 hours later because of excessive dra inage and hemodynamic insta- bil i ty with signs of cardiac tamponade . Throughout this per iod in the intensive care unit, b lood gases were satisfactory. Her first pos topera t ive chest roen tgenogram demons t ra t ed a reduct ion in the volume of the right lung compared with the left lung (Fig 2).

Upon reexp lo ra t ion the h e m o d y n a m i c s s tab i l ized quickly due to the release of the cardiac tamponade . No clots or re ta ined b lood was found in the pe r ica rd ium or in the pleural spaces. Whi le searching for the source of bleeding, we noted that the right lower lobe was inflated in the left hemithorax and had herniated, as in the first

© 1995 by The Society of Thoracic Surgeons 0003-4975/95159.50 0003 -4975(94)00883-9