Ebstein's malformation

Diagnostic

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Ebstein’s Malformation Discordant

Intracavitary

Electrocardiographic

and Pressure Relationship* STANLEY S. MOLES, M.D., WILLIAM J. JACOBY, JR., M.D. and HENRY D. MCINTOSH, M.D. Durham,

North Carolina

LTHOUGH described in 1866,’ the diagnosis of Ebstein’s malformation was made exSince clusively at the autopsy table until 1950.2 then a clinical picture has evolved so that the diagnosis may be strongly suspected on the basis It may be confirmed by of routine evaluation. cardiac catheterization. In 1954 Sodi-Pallares et al.3 suggested that the diagnosis could be confirmed by simultaneously recording the intracardiac electrical potential and pressure as the catheter is withdrawn from the right ventricle across the tricuspid Hernandez et a1.4 orifice into the right atrium. and Yim and Yu5 confirmed this hypothesis and reported a typical right ventricular intracavitary electrical pattern with a simultaneously obtained low atria1 pressure curve. They also suggested the production of right ventricular monophasic waves by the tip of the catheter impinging upon the endocardium (distal portion of the low pressure chamber) was indicative of the anomaly. A recent experience in our laboratory has demonstrated that a normal electrical and pressure relationship may be obtained when the catheter is withdrawn across the tricuspid valve Therefore, the in a patient with this anomaly. diagnosis should not be discarded solely on the basis of a normal “pull-through” tracing, but rather multiple “pull-throughs” across different areas of the tricuspid orifice should be obtained.

CASE

A

&.WORT

A 20 year old single man was referred from the Portsmouth Naval Hospital for evaluation. He gave a history of bouts of mild cyanosis and episodes of tachycardia occurring since infancy. He was mildly dyspneic on exertion but otherwise asymptomatic except during prolonged episodes of tachycardia when congestive failure developed. Three previous attempts at cardiac catheterization had been terminated prematureIy because of the development of such (supraventricular) tachycardia. Blood pressure was 120/70, Physical Examination: pulse 96 and regular and respirations 17. The patient was well developed and well nourished and had no overt evidence of specific cardiac chamber hyperactivity. A systolic thrill was present along the left sternal border. Ml was normal. Sz was split normally and louder over the pulmonic than the aortic area. A protodiastolic and presystolic gallop were A grade 4/G present at the lower left sternal margin. holosystoiic murmur, which increased with inspiration, was heard best in the same area as the thrill. Accessory Clinical Findings: Laboratory studies indi-

cated blood and urine were normal. Achest roentgenogram revealed a prominent right border of the heart and normal pulmonary vascularity. An electrocardiogram showed Type B Wolff-Parkinson-White conduction and tall, peaked P waves in leads I, II and V1 suggesting right atria1 hypertrophy. Hospital course: A Cardiac Catheterization Studies: right and left heart catheterization was performed. Almost simultaneously with the entry of the catheter into the right atrium, atria1 flutter with a 2: 1 block

* From the Cardiovascular Laboratory, Department of Medicine, Duke University Medical Center, Durham, N. C. This study was supported in part by Research Grants HE-07563-02 and H-4807 from the National Heart Institute of the National Institutes of Health, U. S. Public Health Service and Grant-in-Aid QH-174,402 of the Council for Tobacco Research. 720

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Ebstein’s

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FIG. 1. A, persistence of “right ventricular type” complexes in the intracardiac electrocardiogram as the electrode catheter is withdrawn across the tricuspid valve despite a simultaneous right atria1 pulse contour. B, the tracings are discordant with the diagnosis of Ebscein’s malformation. ?‘he intracardiac electrocardiogram changes abruptly at the same time as the pressure tracing.

and a ventricular response of 200 developed. As this rate was well tolerated, the study was continued until all necessary data had been collected. He was then given intravenous Brevital@ anesthesia and normal sinus rhythm was restored by D.C. cardioversion. Catheterization data revealed pressures (mm. Hg) of 10/l in the right atrium, 23/5 in the right ventricle, and 22/13 in the pulmonary artery. Dye curves and cineangiocardiography demonstrated a small atria1 septal defect with left to right shunting. The cineangiocardiograms also demonstrated a large right atrium and a small right ventricle. Oxygen data revealed a pulmonary to systemic flow ratio of 1.3 : 1. By using a unipolar electrode catheter, a simultaneous record of intracardiac eIectrica1 complexes and pressures was obtained when the catheter was withdrawn across the tricuspid valve. The typical findings in Ebstein’s anomaly of ventricular complexes and atria1 pressures were demonstrated (Fig. 1A). However, normal pull-back tracings could be similarly obtained as demonstrated by Figure 1 B.

DISCUSSION The finding of a ventricular electrical complex with an atria1 pressure on “pull-through” across the tricuspid valve located to the left of the spine established the diagnosis of Ebstein’s malformation. It was shown, however, as in Figure lB, that normal atria1 electrical complexes and “pull-through” pressures could also be obtained if the catheter passed over the VOLUME

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1964

valve in the mid-atria1

region

rather

than

in the

low atrium.

In Ebstein’s malformation the anterior leaflet of the tricuspid valve is usually attached to the true annulus along its medial aspect, whereas the septal (medial) and posterior leaflets are displaced downward and do not insert into the true annulus. It is apparently possible to withdraw the catheter slowly over the anterior leaflet into the right atrium and record normal complexes. Therefore, it, becomes necessary to explore the tricuspid orifice with care in studying cases suspected of having such an anomaly. Gandhi and Datey2 have reported recording ventricular electrical complexes and atria1 pressures when the catheter was withdrawn from the right ventricle into the lower portion of the right atrium in three patients whom they considered to have normally developed and positioned tricuspid leaflets. Our experience and that of Gandhi and Datey indicate that both false negative and false positive data may be obtained by this technic. It is, therefore, important to observe the position of the tricuspid valve in relation to the spine and possibly define its position by cineangiocardiography in suspected cases of Ebstein’s malformation. Patients with Ebstein’s malformation have been considered extremely poor catheterization

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and surgical risks because of their tendency to develop supraventricular arrhythmias.8 The atria1 flutter which developed in this patient during catheterization responded satisfactorily to D.C. cardioversion. Since arrhythmias may lead to serious complications in such patients, cardiac catheterization should probably be performed only when means of electrical control of cardiac rhythm are available. SUMMARY

This case demonstrates that normal atria1 intracardiac electrical complexes and pressures can be obtained in a patient with Ebstein’s malformation if the tip of the electrode catheter is withdrawn through that portion of the valve attached to the true annulus of the tricuspid ring. False negative as well as previously reported false positive tracings may be obtained. Careful anatomic localization of the tricuspid valve is imperative in patients suspected of having Ebstein’s malformation. The diagnosis can also be confirmed by the production of ventricular extrasystoles or monophasic ventricular complexes when the electrode is pressed against “atrialized” ventricular endocardium. REFERENCES 1. EBSTEIN, W.

fiber einen sehr seltenen Fall van In-

and McIntosh sufficienz der Valvula trlcuspidalis, bedingt durch eine angeborene hochgradige Missbildung derselben. Arch. Anat. Physiol. u. wissensch. Med., 238, 1866. 2. REYNOLDS, G. Ebstein’s disease, a case diagnosed clinically. Guy’s Hosp. Rep., 99: 276, 1950. 3. SODI-PALLARES,D., ACEVEDO,.J. S., CISNEROS,F. and ALVANADE, A. Wolff-Parkinson-White syndrome in Ebstein’s disease; possibility of diagnosing this anomaly by means of intracavitary leads.In: Abstracts of Scientific Paoers. Second World Congress of Cardiology and 2?th Annual Scientific Sessions of the American Heart Association, Inc., p, 162. New York, 1956. Stuyvesant Press Corp. 4. HEIUIIANDEZ,F. A., ROCHIUND,R. and COOPER, H. R. The intracavitary electrocardiogram in the diagnosis of Ebstein’s anomaly. Am, J. Cardiol., 1 : 181, 1958. 5. YIM, B. J. B. and Yu, P. N. Value of an electrode catheter in diagnosis of Ebstein’s disease. Circulation, 17: 543, 1958. 6. EDWARDS, J. E. Symposium on cardiac catheterization; pathologic features of Ebstein’s malformation of the tricuspid valve. Proc. Staf Meet. Mayo Clin., 28: 89, 1953. 7. GANDHI, M. J. and DATEY, K. K. The value of electrophysiologic changes at the tricuspid valve in the diagnosis of Ebstein’s anomaly. Am. J, Cardiol., 12: 169,1963. 8. KJELLBERG, S. R., MANNHEIMER, E., RUDHE, U. and JONSSON,B. Diagnosis of Congenital Heart Disease, p. 690. Chicago, 1956. The Year Book Publishers, Inc.

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