Identification of cor triatriatum dexter by two-dimensional echocardiography

In contrast to the 2 patients described above, wbo responded, we have seen 2 other patients who were treated with prednisone after the Fontan procedure and neither responded: one with chylous pleural effusions 6 weeks after operation and the other with nonchylous pericardial effusion 12 weeks after surgery. In summary, our z successfully treated patients show that prednisone can be useful in the management of chronic pleural effusions in selected cases after modified Fontan procedures.

Identification of Cor Triatriatum Dexter by Two-Dimensional Echocardiography DAVID A. BURTON, MD ALVIN CHIN, MD PAUL M. WEINBERG, MD JOHN D. PIGOTT, MD

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or triatriatum dexter is an uncommon congenital heart defect that may cause cyanosis in infancy and mimic more complicated forms of congenital heart disease. This lesion occurs when the eustachian valve, or right venous valve of the inferior vena cava, is unusually large and causes obstruction to right ventricular filling. The eustachian valve is frequently seen by 2dimensional (Z-D] echocardiography and is a normal finding if the valve is smal1.l This report describes the From the Children’s Hospital of Philadelphia, 34th Street and Civic Center Boulevard, Philadelphia, Pennsylvania 19104. Manuscript reccivcd January 23, 1987; revised manuscript March 9,1987, accepted March 11,1987.

I. Serratto M. Miiler SA, Tatooies C, Ardekani R. iIemodynamic evuluutiu~i of Fontan operation in tricuspid atresio. Circulation ?976;54:suppl III:III-99111-101. 2. Laks H, Milliken JC, Perloff JK, Hellenbrand WE, George BL. Chin A, DiSessa TG, Williams RG. Experience with the Fcmtan procedure. 1 Thoruc Cordivasc Surg 1984;88:939-951. 3. Kreutzer GO, Vargas FJ. Schlichter AJ, Laura JP, Suarez JC, Rodriguez A, Kreutzer EA. Atriopufmonary onastomosis. J Thoroc Cordiovasc Surg 1982; 83:427-436. 4. Lowell JR. Pleural effusions: (I comprehensive review. Baltimore: University Park Press, 1977:1x 5. Engle MA, Zabriskie JB, Senterfit LB, Tay DJ, Ebert PA. Jmmunologic ond virologic studies in the postpericardiotomy syndrome [ Pediotr 1975:87:11031108.

echocardiographic identification of an obstructive stachian valve causing cyanosis.

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A 1%month-old boy was evaluated for cyanosis. Cardiac catheterization at age I month at another institution was interpreted as showing tricuspid atresia, atrial septal defect and ventricular septal defect. The infant failed to thrive, was cyanotic and was referred to our institution. He weighed 5.7 kg and was mildly cyanotic. The right ventricular impulse was increased. There were no precordial murmurs. An electrocardiogram revealed slightly diminished right ventricular forces. Chest x-ray showed mild cardiomegaly and normal vascular markings. A 2-D echocardiogram showed a large, discrete venous valve that appeared to separate the right atrium into 2 distinct chambers, establishing the diagnosis of car triatriatum dexter [Fig. 1). A secundum atrial septal defect also was present. The tricuspid valve was normal. Cardiac catheterization showed a linear density running vertically from the lateral margin of the inferior vena cava identical to the echocardiographic appearance. At surgery, the prominent venous valve was resected and the atrial septal defect was closed. The infant had an uncomplicated postoperative course.

FIGURE 1. Two-dimensional echocardiogram recorded from the subcostal sagittal view. Left, eustachian valve (arrows), which appears t0 partition the right atrium into 2 separate chambers: the right atrial appendage (RAA) portion, which contains the tricupsid valve (TV) and B venous portion, which receives superior vena cava (SVC), inferlor vena cava (not seen) and coronary sinus (not seen). R@if, tran~~u~~~ tilted slightly to the left separates the eustachian valve from the tricuspid valve. Note eustachian valve obstructing inflow to right v~ntr~ol~ (RV) while permitting caval flow across the atrial septal defect. a = anterior; i = inferior; LA = left atrium; p = ~~stor~~r; nary artery; s = superior.

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Cor triatriatum dexter represents persistence of the fetal eustachian valve. In embryonic development, the eustachian valve forms during incorporation of the sinus venosus into the primitive right atrium, temporarily separating these 2 components of the definitive right atrium. Normally, the valve involutes, and by 55 days after ovulation it has regressed to a small rim of tissue at the junction of the inferior vena cava and the right atrium. Frequently, these remnants are normal echocardiographic finding& and are discovered only at necropsy.z If the valve persists, a partition forms across the right atrium directing inferior vena caval blood across the foramen ovale with right-to-left shunting and cyanosis. Sometimes the tissue fenestrates but does not resorb, and a veil-like network or rete Chiari persists, usually with no hemodynamic consequence. If the entire venous valve persists without even fenestration, car triatriatum dexter occurs.3

Doppler Echocardiographic Confirmation of Recurrent Atrial Septal Defect Stenosis in Infants with Mitral Valve Atrbsia DEREK A. FYFE, MD, PhD ASHBY B. TAYLOR, MD PAUL C. GILLETTE, MD CHARLES H. KLINE, RDMS FRED A. CRAWFORD, Jr., MD

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hildren with complex congenital heart defects, including mitral valve atresia and ventricular septal defect, require free egress of blood from the left atrium through a nonrestrictive atria1 septal defect (ASD). If an adequate naturally occurring ASD is not present at birth, balloon atria1 septostomy may be required during the newborn period. Although the ASD so produced may initially decompress the left atrium, progressive stenosis may subsequently occur, producing left atria1 hypertension and clinical signs and symptoms of pulmonary venous obstruction. Such progressive obstruction appears to characterize the natural history of these lesions.1-3 We report 3 cases in which restenosis of an ASD occurred in patients with mitral valve atresia. In each case the stenotic ASD was demonstrated by 2-D echocardiography. Presence of a high-velocity jet from the left to the right atrium was detected by pulsed or continuous-wave Doppler echocardiography at the region From the Division of Pediatric Cardiology and Cardiothoracic Surgery, South Carolina Children’s Heart Center, Medical University of South Carolina, 171 Ashley Avenue, Charleston, South Carolina 29425-0682. Manuscript received January 21, 1987; revised manuscript received March 10, 1987, accepted March 11, 1987.

These valves are large enough to cause obstruction of blood flow across the tricuspid valve leading to underdevelopment of the right side of the hearta Identification of an obstructive venous valve is difficult by angiography.5 Subcostal2-D echocardiography can readily distinguish tricuspid valve from venous valve, thereby resulting in a rapid, accurate diagnosis. A benign postoperative course may be expected. 1. Limacher MC, Guttgeswell HP, Vick GW, Cohen MH, Huhta JH. Echocardiographic anatomy of the eustachian valve. Am J Cardiol 1986;57:363-365, 2. Yater WM. Variations and anomalies of the venous valves of the right atrium of the human heart. Arch PathoJ 1929:7:418-441. 3. Weinberg PW, Peyser K, Hackney JR. Fetal hydrops in a newborn with hypoplastic left heart syndrome: tricuspid valve “stopper.” JACC 1985; 6236%1369. 4. Hansing CE, Young WP, Rowe GG., Cor triatriatum dexter. Am J Cardiol 1972;30:559-564, 5. Hausdorf G, Gravinghoff L, Sieg K, Keck W. Pitfalls in the diagnosis of tricuspid atresia: report of a new angiocardiographic sign. Clin Cardiol 1985$:189-198.

of the visualized stenotic ASD. Quantitation of the interatrial gradient by Doppler was validated by simultaneous catheterization in 1 case. In the other 2 cases catheterization atria1 pressures could not be determined, and atria1 obstruction was confirmed at operation. Doppler echocardiography is a valuable additional noninvasive technique for diagnosis and quantitation of restrictive ASD in patients with mitral valve atresia.

Clinical summary: The clinical and echocardiographic details of the 3 cases are shown in Table I. Patients were symptomatic, with tachypnea and failure to thrive (1 case) or increasing cyanosis (2 cases). Two-dimensional echocardiography showed severe stenosis of the ASD in each case. Qualitative assessment of interatrial blood flow by pulsed Doppler showed abnormal spectral patterns with continuous transatrial velocities exceeding 1.7 m/s, causing aliasing in each case. In 2 cases, continuous-wave Doppler showed maximal instantaneous interatrial gradients of 2.2 (21 mm Hg) and 2.3 (25 mm Hg) m/s. This latter case was correlated during catheterization, in which a 25-mm Hg instantaneous and 28-mm Hg mean gradient were measured from left to right atrium. In the other 2 cases, catheterization was not performed before septectomy; in 1 case as IVC obstruction was present. After surgical septectomy, 2-D echocardiography showed the ASDs to be widely patent and transatria1 Doppler gradients ranged from 0.8 to 1 m/s only. In each case surgical observation confirmed the ASD obstruction detailed by echo. Development of obstruction and stenosis of an ASD in patients with mitral atresia appears to be a frequent and predictable complication that may develop despite absence of an interatrial pressure gradient at catheterization in the newborn,period.1-3 This obstruction, which may develop even after both balloon atria1 septostomy and surgical septectomy, progresses insidiously. It has been suggested that repeated catheterizations should be performed to monitor development of