Ann Thorac Surg 2006;81:1123-1126
© 2006 The Society of Thoracic Surgeons
Case report
Persistent Sinus Venosus Valve Mimicking Pulmonary Stenosis and Atrial Tumor
Felipe Moreno, MD
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,
Carmen Castro, MD,
Daniel Borches, MD,
María Jesús Cerro, MD,
Luis Garcia-Guereta, MD,
Dolores Rubio, MD
Department of Pediatric Cardiology and Pediatric Cardiac Surgery, Hospital Universitario La Paz, Madrid, Spain
Accepted for publication January 17, 2005.
* Address correspondence to Dr Moreno, Department of Pediatric Cardiology, Hospital Universitario La Paz, Paseo Castellana 261, Madrid, 28046 Spain (Email: fmoreno.hulp{at}salud.madrid.org).
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Abstract
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A case of giant remnant of the right sinus venosus valve simulating first a pulmonary stenosis and afterward a left atrial tumor is described. We believe that this is the first reported case in which a correct diagnosis was performed before surgery.
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Introduction
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Remnants of the embryologic sinus venosus valves are not uncommon. However, the persistence of a sinus venosus valve sufficiently large enough to protrude into the pulmonary artery and obstruct the blood flow through the pulmonary valve is extremely rare. We believe that only three cases have been reported to date [1–3]. We have diagnosed and successfully operated on a newborn with this anomaly simulating both pulmonary stenosis and left atrial tumor at different moments.
A 16-day-old girl was referred to our hospital with a diagnosis of pulmonary stenosis. The patient had been having cyanosis occasionally since birth, and a cardiac murmur had been noticed. On examination she was well developed and cyanosis was noted on crying. A grade 2/6 systolic ejection murmur was heard along the left sternal border. The electrocardiogram showed right ventricular hypertrophy. Chest roentgenograms disclosed cardiomegaly with normal vascular marks. Echocardiography suggested pulmonary stenosis with an eccentric jet and a 60 mm Hg systolic pressure gradient. A percutaneous valvuloplasty was indicated. Surprisingly catheterization revealed no transpulmonary gradient, and a pulmonary arteriography suggested the presence of a mobile "mass" within the left atrium (Fig 1A). An urgent echocardiogram was performed in the catheter lab and disclosed a mobile membranous structure anchored to the septal and lateral walls of the right atrium. No transpulmonary gradient was noted at this time. The "mass" entered the right ventricle and returned to the right atrium during the cardiac cycle (Figs 1B, 1C). The initial echocardiogram was then reviewed and a linear, mobile, echo-reflective structure was seen in the right ventricle entering the pulmonary trunk (Fig 2). The patient suffered two episodes of low cardiac output with hypotension and bradycardia during the catheterization that needed cardiopulmonary resuscitation maneuvers. She was taken to the operating room with the diagnosis of persistence of the sinus venosus valve obstructing the right ventricular outflow and eventually the left ventricular inflow. Under cardiopulmonary bypass and hypothermic arrest, the right atrium was opened and a thin membranous sack was seen anchored to the Eustachian valve, the lower border of the dilated foramen oval, and the tricuspid annulus (Fig 3). The entire saccular structure along with its attachments was removed and the foramen ovale was closed. The infant had an uncomplicated postoperative course and was discharged on the day 7. She remained acyanotic and 4 months later appeared entirely healthy.
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Fig 1. (A) Pulmonary arteriography. A round filling defect as a "mass" is seen in the left atrium (arrow). (B) Apical four-chamber view on echocardiography shows a membranous structure in the right atrium (RA) in systole (arrows). (C) In diastole, the membrane (arrow) entered the right ventricle (RV). (LA = left atrium.)
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Fig 2. Parasternal short axis view on echocardiography shows a linear echo-reflective structure (arrows) that entered the pulmonary artery (PA) from the right ventricle (RV). (Ao = aorta).
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Comment
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At the third fetal month the right sinus venosus valve attains a considerable size, dividing the atrium almost completely into two chambers. As the fetal heart develops, this valve normally regresses and its remnants persist as the crista terminalis and the thebesian and eustachian valves. Its persistence may produce a spectrum of anomalies that include a fenestrated membrane corresponding to Chiari's network, an almost complete septation of the right atrium (the so-called cor triatriatum dexter), or a membranous sack that can protrude into the right ventricle across the tricuspid valve [4–7] or even into the pulmonary artery as in the previously reported cases [1–3] and in our patient. In cor triatriatum dexter the entire right valve of the sinus venosus remains and causes a division between the sinus and primitive atrial portions of the right atrium. Aneurysmal dilatation of such a valve remnant can enter the right ventricle in diastole producing tricuspid valve obstruction [4–7] or more rarely pulmonary valve obstruction. Reviewing the literature, we have only found three cases of this anomaly with obstruction of the pulmonary artery [1–3], diagnosed either at necropsy [1] or at surgery [2, 3]. In two of them a cardiac tumor was suspected by echocardiography or angiocardiography [2, 3] because the "mass" was not obviously hollow. In another case a spinnaker-like structure entering the right ventricle and partially obstructing the right ventricular outflow tract was observed, and a cardiac tumor was suspected [4].
A correct diagnosis of these cases is difficult, and some have been misdiagnosed as myxoma or rhabdomyoma [2–4, 7] because the venous valve looked like a solid mass in echocardiography or angiocardiography. However, in a few cases as in our patient, the membranous nature of the "mass" could be determined enabling a correct diagnosis [5, 7]. Appreciation of this fact requires careful analysis of the echocardiogram. Failure to recognize this anomaly can lead to death [1]. On the other hand, successful resection of the membrane results in return to normal physiology [2, 3, 5].
In the present case, the disappearance of the transpulmonary gradient together with the appearance of a "mass" in the left atrium during angiography, suggests herniation of the membrane through the foramen ovale into the left atrium, a finding not previously reported. Obstruction of the left ventricular inflow could have been responsible for the episodes of low cardiac output that occurred during catheterization.
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References
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- Thomka I, Bending L, Arvay A. Cor triatriatum dextrum simulating right ventricular myxoma and pulmonary stenosis Thorac Cardiovasc Surgeon 1983;31:114-116.[Medline]
- Battle-Diaz J, Stanley P, Kratz C, Fouron JC, Guerin R, Davignon A. Echocardiographic manifestation of persistence of the right sinus venosus valve Am J Cardiol 1979;43:850-853.[Medline]
- Trento A, Zuberbuhler JR, Anderson RH, Park SC, Siewers RD. Divided right atrium (prominence of the eustachian and thebesian valves) J Thorac Cardiovasc Surg 1998;96:457-463.
- Lucas RV, Kimberley A, Krabill A. Abnormal systemic venous connectionsIn: Emmanouilides GC, Riemenschneider TA, Allen HD, Gutgessell HP, editors. Moss and Aadams Heart disease in infants, children and adolescents. 5th ed.. Baltimore: Williams and Wilkins; 1995. pp. 874-907.
- Smith NM, Byard RW, Vigneswara R, Bourne AJ, Knight B. Parachute-like sinus venosus remnantechocardiographic and pathological appearance. Pediatr Cardiol 1993;14:82-85.[Medline]
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Abstract
Introduction
