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perior vena cava in a patient with Turner’s syndrome has not been previously described. Despite the frequent asso- ciation of Turner’s syndrome with coarctation of the aorta, bicuspid aortic valve, aortic valve stenosis, pulmonic valve disease, cystic medial degeneration of the aorta, aneurys- ma1 dilatation of the aortic sinuses, aortic dissection, and (less commonly) anomalous pulmonary venous connection, the finding of pulmonary hypertension in our patient is unexpected and unexplained. Anomalous pulmonary venous return as previously described in patients with Turner’s syndrome could result in pulmonary hyperten- sion secondary to increased pulmonary flow with reactive and elevated pulmonary vascular resistance. However, anomalous pulmonary vein connections were clearly ex- cluded, as all four pulmonary veins were seen attached to the left atrium. This unusual case demonstrates a hereto- fore undiagnosed congenital association that was easiIy identified with TEE. The diagnosis of persistent left supe- rior vena caval is important and should be considered when these patients require transvenous pacing,5 right heart catheterization, endomyocardial biopsy, or any procedure attempted from the right jugular or subclavian approach.

REFERENCES

1. Subramaniam P. Case report. Turner’s syndrome and cardio- vascular anomalies: a case report and review of the literature. Am J Med Sci 1989;297:260-2.

2. Van Wassenaer AG, Lubbers LJ, Losekoot G. Partial abnor- mal pulmonary venous return in Turner syndrome. Eur J Pe- diatr 1988;148:101-3.

3. Moore J, Kirby W, Rogers W, Poth M. Partial anomalous nul- monary venous drainage associated with 45,X Turner’s syn- drome. Pediatrics 1990:86:273-6.

4. Lacro R, Jones K, Be&schke K. Coarctation of the aorta in Turner syndrome: a pathologic study of fetuses with nuchal cystic hygromas, hydrops fetalis, and female genitalia. Pedi- atrics 1988;81:445-51.

5. Dosios T, Gorgogiannis D, Sakorafas G, Karampatsas K. Per- sistent left superior vena cava: a problem in the transvenous pacing of the heart. PACE 1991;14:389-90.

Table I. Correlation of transthoracic, transesophageal, and gross pathologic findings in St. Jude Medical and biopros- thetic valve endocarditis

Severe Vegetations regurgitation

Valve position No. TTE TEE Surgery TTE TEE Surgery

St. Jude mitral 4 0 4 4 0 4 4 Biopro mitral 2 0 1 o* 1 1 1 St. Jude aortic 3 0 3t 3 2 2 2 Biopro aortic 1 0 1 1 0 0 0

Biopro, Bioprosthetic valves; TEE, tramesophageal echocardiogram and color flow Doppler; TTE, transthoracic echocardiogram and color flow Doppler. *One patient did not have valve surgery performed. tone patient had additional findings of aortic root abscess.

dimensional (2-D) echocardiography is of limited value in diagnosing the presence of vegetations on prosthetic valves.2v 3 Recently, transesophageal echocardiography has been shown to be superior to transthoracic imaging in de- tecting vegetations on native and prosthetic heart val- ves.2,4-6 We report our transesophageal echocardiographic experience in patients with infective endocarditis involving St. Jude Medical and bioprosthetic valves.

Transesophageal echocardiography and transthoracic color flow Doppler studies were prospectively performed in seven St. Jude Medical (St. Jude Medical Inc., St. Paul, Minn.) and three bioprosthetic valves implanted in the mitral and the aortic positions (Table I). All patients pre- sented with fever with or without chills and had positive blood cultures for either Staphylococcus aureus, Staphy- lococcus epidermidis, or Streptococcus uiridans. The di- agnosis was confirmed by subsequent valve replacement surgery performed in nine patients. Six patients had sur- gery based on clinical and transesophageai echocardio- graphic findings without prior cardiac catheterization. The transthoracic and transesophageal ultrasound studies were performed on the same day using conventional techniques and instrumentation. After the completion of 2-D echocar- diographic images, the prosthetic valves were interrogated for the presence or absence of valve regurgitation by color flow Doppler. Prosthetic mitral and aortic valve regurgita- tions were semiquantated by previously described meth- 0ds.l. s

Transesophageal echocardiographic evaluation of St. Jude Medical and bioprosthetic valve endocarditis

Mohsin Alam, MD, Howard S. Rosman, MD, and Irene Sun, RN, RDMS. Detroit, Mich.

Infective endocarditis is a serious complication of pros- thetic cardiac valves which, if untreated, is usually fatal1 Unlike its usefulness in native valves, transthoracic two-

From the Heart and Vascular Institute, Division of Cardiovascular Medi- tine, Henry Ford Hospital. Reprint requests: Mohsin Alam, MD, Heart and Vascular Institute, Division of Cardiovascular Medicine, Henry Ford Hospital, 2799 West Grand Blvd., Detroit MI 48202.

Transthoracic 2-D echocardiography demonstrated no vegetations in any of the 10 patients who had clinical ev- idence of endocarditis (Table I). Transesophageal 2-D echocardiography demonstrated vegetations in nine of these individuals (Figs. 1 to 4). The patient with no vege- tations by transesophageal study had no vegetations at the time of surgery, but did have a partially dehiscent valve at its suture ring, caused by infection. One additional patient had aortic root abscess (Fig. 2) demonstrable by the transesophageal study only. These findings were subse- quently confirmed at the time of surgery (Table I). In four

414133328 patients with St. Jude Medical mitral valves, transthoracic

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Number 1 Brief Communications 237

co10 ther

was valv logic

Fig. 1. Transesophageal two-dimensional echocardiogram of a patient with St. Jude Medical mitral valve endocarditis. Note the vegetations (arrow) on the atrial aspect of the valve. LA, Left atrium; LV, left ven- tricle; MV, mitral valve prosthesis.

Fig. 2. Transesophageal two-dimensional echocardiogram of a patient with St. Jude Medical aortic valve endocarditis. Note vegetation protruding in the left ventricular outflow tract (arrow). Also note increased echoes in the aortic root (arrowhead), compatible with an abscess. A, Aorta; LA, left atrium; LV, left ven- tricle.

r flow Doppler estimated mitral regurgitation to be ei- of the left atrial chamber. Two infected bioprosthese 5s in mild or absent. All four patients had severe regurgita- the mitral positions had severe and mild regurgitation n by by transesophageal color flow Doppler (Fig: 5) and this both the transesophageal and the transthoracic study I. In confirmed by surgery. In all St. Jude Medical mitral four patients with St. Jude Medical and bioprosthetic aor- es there were three additional smaller jets of “physio- tic valves, both transthoracic and transesophageal c :olor :“mitral regurgitation that encompassed less than 50 5% flow Doppler demonstrated severe regurgitation in twc I pa-

238 Brief Communications January 1992

American Heart Journal

Fig. 3. Transesophageal two-dimensional echocardiogram of a patient with a bioprosthetic mitral valve vegetation. Note the increased echoes on the cusps, compatible with vegetation (arrow). LA, Left atrium; ST, valve stents.

Fig. 4. Transesophageal two-dimensional echocardiogram of a patient with bioprosthetic aortic valve vegetations (arrow). A, Aorta; LA, left atrium; LV, left ventricle; ST, valve stents.

tients and mild or no regurgitation in the other two patients (Table I).

This study confirms our hypothesis that the transesoph- ageal approach for 2-D echocardiography is far superior to the transthoracic method for observing vegetations in St. Jude Medical and bioprosthetic valves. Transesophageal studies should be performed in all patients with suspected infective endocarditis, even in those with a normal tran- sthoracic study. However, reverberations from the metal portion of the mechanical valves could pose difficulties in diagnosing vegetations even with the transesophageal ap-

proach. This is especially true for mitral and aortic vege- tations that do not protrude into the left atrium or ventricular outflow tract, respectively. In the case of bioprosthetic valves, the vegetations could not be differen- tiated from valve thickening/calcification because of cusp degeneration. The echocardiographic findings of valve vegetations should therefore always be correlated with the clinical features and presentation. In the patient with aor- tic ring abscess, the transesophageal study influenced our decision to operate early despite hemodynamically mild aortic regurgitation.

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Fig. 5. Transesophageal color flow Doppler echocardiogram of same patient imaged in Fig. 1 with St. Jude Medical mitral valve endocarditis and severe paravalvular regurgitation (arrow). LA, Left atrium; LV, left ventricle.

In all of our patients with St. Jude Medical mitral valves, the regurgitation was severely underestimated by the transthoracic color flow Doppler when compared with the findings of the transesophageal study. This is probably be- cause of flow masking of the left atrium from the transtho- racic ultrasound window, which is overcome by the trans- esophageal approach. Both transthoracic and transesoph- ageal color flow Doppler were of equal value demonstrating and estimating St. Jude Medical and bioprosthetic aortic valve regurgitation. In conclusion, in patients with sus- pected St. Jude Medical and bioprosthetic valve en- docarditis, transesophageal 2-D echocardiography with color flow Doppler is more sensitive than transthoracic im- aging in demonstrating valve vegetations and in estimating the severity of St. Jude Medical valve mitral regurgitation.

REFERENCES

1. Ivert TSA, Dismukes WE, Cobbs CG, Blackstone EH, Kirklin J, Bergdahl LAL. Prosthetic valve endocarditis. Circulation 1984;69:223-32.

2. Mugge A, Daniel WG, Frank G, Lichtlen PR. Echocardio- graphy in infective endocarditis: reassessment of prognostic implications of vegetation size determined by the transtho- racic and the transesophageal approach. J Am Co11 Cardiol 1989;14:631-8.

3. Sprecher DL, Adamick A, Adams D, Kisslo J. In vitro color flow pulsed and continuous wave Doppler ultrasound masking of flow by prosthetic valves. J Am Co11 Cardiol1987;9:1306-10.

4. Gussenhoven EJ, Meindert TA, Roelandt JRTC, Ligtvoet KM, McGhie J, VanHerwerden LA. Calahan MK. Transeso- phageal two-dimensional echocardiography: its role in solving clinical problems. J Am Co11 Cardiol 1986;8:975-9.

5. Polak PE, Gussenhoven WJ, Roelandt JRTC. Transesoph- ageal cross-sectional echocardiographic recognition of an aor- tic valve ring abscess and a subannular mycotic aneurysm. Eur Heart J 1987;8:664-6.

6. Erbel R, Rohmann S, Drexler M, Mohr-Kahaly S, Gerharz CD,

1.

8.

Iverson S, Oelert H, Meyer J. Improved diagnostic value of echocardiography in patients with infective endocarditis by transesophageal annroach: a urosuective studv. Eur Heart J 1988;1:43-53: - - - - Helmcke F, Nanda MC, Hsiung MC, Soto B, Adey CK, Goyal RG, Gatewood RP. Color Donnler assessment of mitral re- gurgitation with orthogonal pianes. Circulation 1987;75:175- 83. Perry GJ, Helmcke F, Nanda NC, Byard C, Soto B. Evalua- tion of aortic insticiency by Doppler color Aow mapping. J Am Co11 Cardiol 1987;9:952-9.

Development of obstruction to ventricular outflow and impairment of inflow in glycogen storage disease of the heart: Serial echocardiographic studies from birth to death at 6 months

Barbara L. Seifert, MD, Michael S. Snyder, MD, Arthur A. Klein, MD, John E. O’Loughlin, MD, Margaret S. Magid, MD,a and Mary Allen Engle, MD. New York, N.Y.

We report the earliest presentation and diagnosis of glyco- gen storage disease of the myocardium (Pompe’s disease), together with echocardiographic evidence of development

From the Division of Pediatric Cardiology and %he Department of Pathol- ogy, The New York Hospital-Cornell University Medical Center.

Reprint requests: Mary Allen Engle, MD, Division of Pediatric Cardiology, The New York Hospital, 525 E. 68th St., New York, NY 10021.

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