Intracardiac thyroid heterotopia

Intracardiac thyroid heterotopia

Volume 125, Number 6 American Heart Journal and systolic movement echocardiographically. Therefore abnormalities such as perforation involving the an...

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Volume 125, Number 6 American Heart Journal

and systolic movement echocardiographically. Therefore abnormalities such as perforation involving the anterior mitral leaflet will be mobile and have excessive redundant tissue with chaotic motion. The color-flow Doppler regurgitant jet from perforation of the MAIVF is directed anteroposteriorly across the left atrium, somewhat oblique or almost parallel to the mitral anulus, whereas the regurgitant jet from the anterior mitral leaflet perforation is usually directed inferosuperiorly, somewhat perpendicular to the mitral anulus, as any other mitral regurgitant jet. TEE has been demonstrated to be highly valuable in recognizing complications of endocarditis involving the MAIVF. MAIVF involvement can result in hemodynamitally catastrophic consequences.3l l1 Previously published articles emphasize the importance of adequately evaluating this zone by TEE in patients with aortic valve replacement, especially prosthetic valve endocarditis. Our cases illustrate that this rare devastating complication can also occur in mitral valve endocarditis in the appropriate setting. Furthermore, fulminant septicemia can also cause seeding of the prosthetic ring, as seen in our second case, which has not been described previously. Therefore TEE should be considered in the evaluation of patients with isolated mitral valve endocarditis who demonstrate hemodynamic deterioration. REFERENCES

1. Schwartz DR, Belkin RN, Pucillo AL, Burleson PD, Fish BG, Pooley RW, Weiss MB, Herman MV. Aneurysm of the mitral-aortic intervalvular fibrosa complicating infective endocarditis: preoperative characterization by two-dimensional and color flow Doppler echocardiography, magnetic resonance imagine. and cineanpioaraahv. AM HEART J 1990:119:196-9. 2. Bansal%, GrahamB%I, j&y KR, Shakudo M; Shah PM. Left ventricular outflow tract to left atria1 communication secondary to rupture of mitral-aortic intervalvular fibrosa in infective endocarditis: diagnosis by transesophageal echocardiography and color flow imaging. J Am Co11 Cardiol 1990;15:499-504. 3. Karalis DG, Bansal RC, Hauck AJ, Ross JJ, Applegate PM, Jutzy KR, Mintz GS, Chandrasekaran K. Transesophageal echocardiographic recognition of subaortic complications in aortic valve endocarditis: clinical and surgical implications. Circulation 1992;86:353-62. 4. Gonzalez-Lavin L. Lise M. Ross D. The imnortance of the “iet lesion” in bacterial endocarditis involving the left heart: J Thorac Cardiovasc Surg 1970;59:185-92. 5. Edwards JE. Mitral insufficiency secondary to aortic valvular bacterial endocarditis. Circulation 1972;46:623-6. 6. Miyatake K, Yamamoto K, Park YD. Diagnosis of mitral valve perforation by real-time two-dimensional Doppler flow imaging technique. J Am Co11 Cardiol 1986;8:1235-9. 7. Fisher EA. Estioko MR. Stern EH. Goldman ME. Left ventricular to left atria1 communication secondary to a paraaortic abscess: color flow Doppler documentation. J Am Co11 Cardiol 1987;10:222-4. 8. Chesler E, Korns ME, Porter GE, Reyes CN, Edwards JE. False aneurysm of the left ventricle secondary to bacterial endocarditis with perforation of the mitral-aortic intervalvular fibrosa. Circulation 1968;37:518-23. 9. Jaffe WM, Morgan DE, Pearlman AS, Otto CM. Infective endocarditis, 1983-1988: echocardiographic findings and factors influencing morbidity and mortality. J Am Co11 Cardiol 1990;15:1227-33. 10. Sandler MA, Kotler MN, Bloom RD, Jacobson L. Pericardial

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abscess extending from mitral vegetation: an unusual complication of infective endocarditis. AM HEART J 1989:118:857-9. 11. Karalis DG, Chandrasekaran K, Wahl JM, Ross J,Mintz GS. Transesophageal echocardiographic recognition of mitral valve abnormalities associated with aortic valve endocarditis. AM HEART

J 1990;119:1209-11.

Intracardiac thyroid heterotopia Lucia Ansani, MD: Gianfranco Percoco, MD,a Fabrizio Zanardi, MD,a Pierfranco Peranzoni, MD,b Gianluigi Gamba, MD,b and Gianenrico Antonioli, MD, PhD” Ferrara and Verona, Italy

Ectopic thyroid tissue in the cardiac chambers is extremely rare. Its incidence is unclarified. This abnormality seems to be the consequence of the intimate relationship between the thyroid primordium and the developing myocardium in the early period of morphogenesis.’ The few previous cases that have been reported2-l3 are either described at autopsy in patients dead of other causes or are clinically diagnosed and successfully excised during life. We describe a case of benign intracardiac thyroid mass that caused right ventricular outflow tract obstruction and mimicked a cardiac tumor; the mass was successfully treated surgically. In May 1975 a 60-year-old woman was diagnosed as having a congenital infundibular pulmonary stenosis. At that time cardiac catheterization showed a peak systolic pressure gradient of 80 mm Hg between the midright ventricle and its outflow tract. The mean right atria1 pressure was 8 mm Hg. Angiocardiography revealed a mildly dilated right atrium and a markedly hypertrophic right ventricle, with a prominent filling defect in the outflow tract and the passage of the contrast medium towards a small, subvalvular chamber. Left atriogram and ventriculogram were normal. She was then lost to follow-up until October 1990, when she was 75 years old. At that time she complained of irregular palpitations and dyspnea on effort. Her condition gradually worsened, and she was admitted to our hospital in December 1990. In the last few years she had had essential hypertension and was receiving digoxin, enalapril, and diuretics. Physical examination on admission showed an orthopneic patient with a heart rate of 75 beats/min and a blood pressure of 150/80 mm Hg. She was afebrile. Rales were present in both lower lung fields. A grade 416 holosystolic muimur was heard at the third left intercostal space near the sternal border. The liver was palpable 2 cm below

From the “Divisione di Cardiologia, Arcispedale S.Anna, Ferrara; ‘Divisione di Cardiochirurgia, Ospedale Borg&en@ Verona. Reprint S.Anna,

requests: Lucia Ansani, MD, Divisione di Cardiologia, c.so Giovecca 203, 44100 Ferrara, Italy.

AM HEART J 1993;125:1797-1801 Copyright ” 1993 by Mosby-Year Book, 0002.8703/93/$1.00 + .lO 4/4/45674

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Fig. 1. Bidimensional echocardiogram in right ventricular cavity.

Fig. 2. Right ventricular outflow tract.

angiogram

in the four-chamber

showing

large filling

the right costal margin. Slight leg edema was present. The thyroid gland was not enlarged. The electrocardiogram showed sinus rhythm and complete right bundle branch block. Chest x-ray film revealed moderate to severe cardiomegaly and interstitial pulmonary edema in both lower

subcostal

view showing

defect of mass protruding

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echodense

round mass

into right

ventricular

lung fields. Bidimensional echocardiography disclosed a huge ovoid mass in the right ventricular cavity (Fig. 1). The structure was approximately 4.5 x 4 cm and relatively fixed as a result of its dimension, subtotally obstructing the right ventricular outflow tract. Pulmonary and tricuspid

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Fig. 3. Gross appearance of resected intracardiac mass: cut-surface.

valves were apparently normal. At Doppler examination mild tricuspid regurgitation was observed. The left cardiac chambers and valves were normal. Cardiac catheterization was repeated; right pressure values were similar to those previously measured. Angiocardiography confirmed the presence of an enormous ovoid mass obstructing the right ventricular outflow tract (Fig. 2); a threadlike passage of the contrast medium was observed in systole. A primitive, progressively growing cardiac tumor was suspected. The patient underwent surgery. After a longitudinal median sternotomy a heart of normal morphologic structure and kinetics appeared. At cardiotomy an ovoid mass was found rising from the anterior papillary muscle, protruding into the right ventricular outflow tract, and apparently adhering to many surrounding structures. It was encapsulated and attached with a broad base. The tumor was cut from the papillary muscle; it measured approximately 5.5 x 5 cm and weighed about 60 gm. The cut surface was yellowish and had a spongelike appearance with several small cysts and areas of hemorrhage, fibrosis, and calcific deposits (Fig. 3). Microscopically the mass was composed of thyroid tissue that was well encapsulated with a dense fibrous membrane in which some cardiac muscle was found. The thyroid tissue was made of differently sized, colloid-containing follicles that sometimes showed hematic content with macrophages filled with hemosiderin; calcific areas were found among follicles (Fig. 4). No areas of neoplastic degeneration were noted, and the mass was consistent with an intracardiac thyroid rest. The postoperative course was uneventful. Thyroid function tests (Ts, Th, thyroid-stimulating hormone, FTs, and FTh) immediately after the operation showed normal levels. A whole-body thyroid scan showed a normal thyroid gland in the neck and no more ectopic thyroid tissue. 12 months after the operation the patient is

4. Histologic appearance of resected mass showing colloid-containing follicles resembling thyroid adjacent to myocardial tissue. Fig.

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Fig. 5. Bidimensional echocardiogram in four-chamber apical view after removal of mass. Moderately dilated right chambers (right) are seen.

in good general condition; echocardiography shows moderately dilated right chambers (Fig. 5) and a residual, mildto-moderate tricuspid regurgitation resulting from the intraoperative need to resect and subsequently reattach the anterior papillary muscle to reconstruct the integrity of the valve apparatus. Thyroid function tests remain normal. Ectopic thyroid location in the heart is a very rare occurrence that may result from the aberrant migration of the gland mechanically influenced by the developing heart.l The thyroid takes its origin from the entoderm of the pharyngeal floor. The entodermic downgrowth migrates caudally, following the primitive heart migration to its definitive site in the neck. In the presence of an abnormal cardiac influence, thyroid rests may be tracked to different sites along the heart way, ectopic thyroid tissue being found in cervical sites, mediastinum, aortic arch, pericardium, myocardium, and diaphragm. To our knowledge only 11 reports of cardiac thyroid have been published.2-13 In three the findings were made at postmortem examination.2-4s l3 In the others the clinical diagnosis and surgical treatment were made in living patients. 5-12Our case is the twelfth on the whole, the ninth in vivo report of cardiac thyroid. Some analogous features can be noted in most reports: (1) The frequent occurrence of this developmental abnormality in women and its clinical appearance in middle-age indicates the slow growth of the rests over time; (2) the frequent location of the ectopic thyroid tissue in the right ventricle-the tissue originates mostly from the right side of the ventricular septum and less frequently from the tricuspid valve apparatus and papillary muscle, and its growth toward the right ventricular cavity and outflow tract results in obstruction symptoms, systolic murmur, and right ventricular hypertrophy; and (3) the absence of reported malignant transformation, although potentially

possible, portends a good prognosis and makes the condition surgically curable. Nevertheless, some further considerations are necessary. First, the primitive neoplastic or metastatic nature of the mass should be carefully excluded. The right ventricular outflow tract is a frequent site of metastasis; in particular, thyroid carcinoma frequently metastasizes to the heart. It has been suggestedl” that a preoperative cardiac thyroid scan might be useful in patients with echocardiographic finding of right ventricular mass partially obstructing the outflow tract; a complete resection of the mass seems indeed to be indicated in most cases.Finally, echocardiography has largely improved the recognition of intracardiac tumors, sometimes allowing surgery without cardiac catheterization. 6.lo. la This is particularly true now for the transesophageal technique.14 REFERENCES

1. Werner SC. Normal and anomalous development of the thyroid. In: Werner SC, ed. The thyroid. New York: Harper and Row, 1962:249-65. 2. Dosch F. Uber einen fall von glandula thyroidea accessoria intracardialis. Beitr Path01 Anat 1941;105:244-55. 3. Rogers WM. Kesten HD. Cardiac thvroid. Embrvoloaic basis for-thyroid ‘tissue in the heart [Abstract]. Anat Ret 1962; 142:323. 4. Rogers WM, Kesten HD. A thyroid mass in the ventricular septum obstructing the right ventricular outflow tract and producing a murmur. J Cardiovasc Surg 1963;4:175-80. 5. Lo HM, Tseng YZ, Tseng CD, Chu SH, Chuang SM, Wu TL. Intracardiac goiter: a cause of right ventricular outflow obstruction and successful operative therapy. Am J Cardiol 1984;53:976-8. 6. Shemin RJ, Marsh JD, Schoen FJ. Benign intracardiac thyroid mass causing right ventricular outflow tract obstruction. Am J Cardiol 198$56:828-g. 7. Pollice L, Caruso G. Struma cordis: ectopic thyroid goiter in the right ventricle. Arch Path01 Lab Med 1986;110:452-5.

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Kantelip B, Lusson JR, De Riberolles C, Lamaison D, Bailly P. Intracardiac ectopic thyroid. Hum Path01 1986;17:1293-6. 9. Grigg LE, Downey W, Tatoulis J, Hunt D. Benign congenital intracardiac thyroid and polycystic tumor causing right ventricular outflow tract obstruction and conduction disturbance. J Am Co11 Cardiol 1987;9:225-7. P, Ottino GM, Avonto L, Carini G, Pozzi R, 10. Greco-Lucchina Emanuelli G. Ectopic thyroid remnants within the myocardium: an unusual case of right ventricular mass. AM HEART J 8.

1988;115:195-8.

Rose AG, Novitzky D, Price SK. Heterotopic thyroid tissue in the heart. Am J Cardiovasc Path01 1988;1:401-4. 12. Doria E, Agostoni P, Fiorentini C. Accessory thyroid tissue in the right ventricle. Chest 1989;96:424-5. 13. Richmond T, Whittaker JS, Deiraniya AK, Hassan R. Intracardiac ectopic thyroid: a case report and review of published cases. Thorax 1990;45(4):293-4. 14. Mugge A, Daniel WG, Haverich A, Lichtlen PR. Diagnosis of noninfective cardiac mass lesions by two-dimensional echocardiography. Comparison of the transthoracic and transesophageal approaches. Circulation 1991;83:70-8. 11.

Solitary left ventricular metastasis of renal cell carcinoma Toshikazu Sobue, MD: Mitsunori Iwase, MD,a Masatsugu Iwase, MD: Toshio Aoki, MD, Akiko Noda,b Minoru Tanaka, MDC and Mitsuhiro Yokota, MDb Nagoya, Japan From the BFirst Department of Internal Medicine, bDepartment of Clinical Laboratory Medicine, and eFirst Department of Surgery, Nagoya University Hospital. Reprint requests: Mitsuhiro Yokota, MD, Circulatory Physiology Section, Department of Clinical Laboratory Medicine, Nagoya University Hospital, 65 Tsurumai-cho, Shows-ku, Nagoya 466, Japan. AMHEARTJ 1993;125:1801-1802 Copyright 0 1993 by Mosby-Year Book, Inc. 0002-8703/93/$1.00 + .lO 4/4/45676

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Many metastatic cardiac lesions remain clinically silent only to be identified at necropsy.’ The following case represents a rare instance of antemortem diagnosis of a solitary left ventricular metastasis of a renal cell carcinoma. A 60-year-old woman was admitted to Nagoya University Hospital for cardiac catheterization on January 21, 1992. She had a history of renal cell carcinoma of the right kidney that extended to the right atria1 cavity via the inferior vena cava. The tumor was completely removed by nephrectomy in 1986. Interferon-a was administered until September 1991. On November 14,1991, the patient experienced a typical anterior myocardial infarction and was admitted to a regional hospital. At that time echocardiography identified only the asynergy of the left ventricular anterior wall. The patient was admitted to Nagoya University Hospital in 1992. Findings on physical examination were normal except for an ejection systolic murmur of grade 216 over the left sternal border. Electrocardiogram showed poor R-wave progression in precordial leads and inverted T waves in VI through V3. Chest x-ray film showed normal findings. The echocardiogram (Fig. 1) revealed a large mass in the left ventricle. The pericardial echo-free space was within normal limits. Coronary angiography showed normal findings except for the presence of faint fine vessels to the tumor. Left ventriculography and biopsy were not performed because of the risk of peripheral embolization caused by dislodgment of a tumor fragment or an associated thrombus. Magnetic resonance imaging scan (Fig. 2) confirmed a lobular mass that originated in the left ventricular posterior wall and protruded into the left ventricular cavity. No sign of local recurrence or distant metastases were found by abdominal ultrasonography and computed tomography scans of the abdomen and chest. Tumor markers (carcinoembryonic antigen, a-fetoprotein and CA 19-9) were within normal limits. Starting February 18 intramuscular interferon-a (3 X lo6 IU) was adminis-

Fig. 1. Long-axis (A) and four-chamber view(B) of two-dimensional left ventricle. LV, Left ventricle; LA, left atrium; RA, right atrium.

echocardiogram.

Note large mass in