Ann Thorac Surg 2009;87:305-307. doi:10.1016/j.athoracsur.2008.06.017
© 2009 The Society of Thoracic Surgeons
Case Reports
Right Ventricular Plication in a Neonate With an Unguarded Tricuspid Valvar Orifice
Yukihiro Kaneko, MD, PhDa,*,
Tomohiro Takeda, MDb,
Hitoshi Yoda, MDb,
Jotaro Kobayashi, MD, PhDa,
Nobuyuki Takada, MDc,
Keiji Tsuchiya, MDc,
Robert H. Anderson, MD, FRCPathd
a Department of Cardiovascular Surgery, Japanese Red Cross Medical Center Tokyo, Japan
b Department of Neonatology, Japanese Red Cross Medical Center Tokyo, Japan
c Department of Pediatrics, Japanese Red Cross Medical Center Tokyo, Japan
d Cardiac Unit, Institute of Child Health, University College of London, London, United Kingdom
Accepted for publication June 4, 2008.
* Address correspondence to Dr Kaneko, Department of Cardiovascular Surgery, Japanese Red Cross Medical Center, 4-1-22 Hiroo, Shibuya-ku, Tokyo, 150-8935, Japan (Email: yukihirokaneko{at}hotmail.com).
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Abstract
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Unguarded tricuspid orifice is a congenital cardiac malformation characterized by absence of the tricuspid valvar leaflets in the setting of a normal atrioventricular junction and obligatory severe tricuspid regurgitation. A neonate with such a lesion presented with severe cardiac failure. The right atrium and ventricle were grossly dilated, and there were small muscular ventricular septal defects. We plicated the right-sided structures to create space for adequate expansion of the lungs and left ventricular filling, proceeding to staged conversion to the Fontan circulation. Our experience shows that neonates with cardiac failure due to dilated right-sided structures can be stabilized by surgical plication.
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Introduction
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Unguarded tricuspid orifice is an uncommon congenital cardiac malformation characterized by the absence of tricuspid valvar leaflets in the setting of a normally formed atrioventricular junction with obligatory resultant severe tricuspid regurgitation [1]. Previous reports have commented on the distinction between this lesion and Ebstein's malformation, the tricuspid valvar leaflets being absent when the orifice is unguarded, but dysplastic and tethered to the ventricular endocardium in the setting of Ebstein's malformation. The lesions are otherwise similar in their hemodynamic and clinical presentations [1–3], with the major problems being compression on the lungs and left ventricular dysfunction due to the dilation of the right-sided cardiac structure [4, 5]. We report here our experience in stabilizing a neonate with unguarded tricuspid orifice who presented with severe cardiac failure by plicating the walls of the dilated right ventricle. As far as we are aware, this is the first report of successful neonatal right ventricular plication for this rare lesion.
A full-term male neonate presented with cyanosis and cardiac failure. On chest roentgenogram, the patient had a cardiothoracic ratio of 0.85 and decreased pulmonary vascular markings. Echocardiography demonstrated dilation of the right atrium and ventricle, severe tricuspid regurgitation, pulmonary atresia, and multiple apical ventricular septal defects. The largest of these defects measured less than 3 mm in diameter. Doppler interrogation revealed a left-to-right shunt across the defects, with a pressure gradient of 30 mm Hg. The ventricular septum was nearly flat in short axis view at end-diastole, indicating left ventricular compression by the dilated right ventricle. The tricuspid orifice measured 18 mm on the four-chamber echocardiogram (Z = 2.5), but the valvar leaflets were not visualized. Hence, we made the diagnosis of unguarded tricuspid orifice (Fig 1A).
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Fig 1. Four-chamber views on echocardiography at end diastole. (A) Image at 1 day old (LV = left ventricle; RV = right ventricle) and (B) shortly after right ventricular plication.
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Right ventricular plication was performed at the age of 9 days [6]. Using cardiopulmonary bypass, the right ventricular free wall was plicated from outside with several buttressed sutures from the acute margin to the mid-portion of the anterior wall, taking care not to kink the right coronary artery. Under cardioplegic cardiac arrest, we also excised the redundant wall of the right atrium. Inspection of the interior aspect of the right ventricle confirmed the presence of a normal atrioventricular junction and complete absence of the tricuspid valvar leaflets. Probing of the ventricular septum revealed at least three defects within the apical muscular septum, each being smaller than 3 mm. Closure of these multiple defects appeared possible but time consuming, and because they were restrictive, we chose not to attempt closure. Using sutures placed from the free wall to the septum, we also obliterated the right ventricular cavity from apex to base, and sutured the anterior portion of the tricuspid orifice to the ventricular septum 5 mm downstream to the atrioventricular junction, taking care not to injure the atrioventricular node. We then created a shunt from the brachiocephalic artery to the pulmonary artery using a 3.5-mm expanded polytetraethylene graft (Gore-Tex vascular graft; W. L. Gore and Associates Inc, Flagstaff, AZ). The operation time, cardiopulmonary bypass time, and myocardial ischemic time were 230 minutes, 158 minutes, and 41 minutes, respectively. Postoperative echocardiography showed significant reduction of the right ventricular volume, normal left ventricular shape at end-diastole, and to-and-fro flow across the ventricular septal defects (Fig 1B). The cardiothoracic ratio decreased to 0.65. Residual cardiac failure meant that he required 25 days of postoperative intubation and mechanical ventilation. Thereafter, recovery proved uneventful. At the age of 4 months, he underwent a successful bidirectional superior cavopulmonary anastomosis. At the age of 1 year and 2 months, we created an extracardiac total cavopulmonary connection using a 16-mm expanded polytetraethylene graft. Postoperative echocardiography confirmed spontaneous closure of the ventricular septal defects. The cardiothoracic ratio had decreased further to 0.60. The patient now shows normal development and weight gain at the age of 1 year and 4 months.
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Comment
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By searching the literature, we found 35 previous reports of patients with unguarded tricuspid orifices, including 6 fetuses (all dying prior to birth), 4 neonates, and 4 infants at the time of diagnosis. All of the reported neonates and infants had cyanosis, and 4 of them had cardiac failure. Of those reported, 7 died during their initial hospitalization, 4 without undergoing cardiac surgery, and 3 after creation of a systemic-to-pulmonary arterial shunt. Only one patient survived the initial hospitalization and later underwent a bidirectional cavopulmonary shunt procedure [2]. Associated cardiac lesions have included pulmonary stenosis, pulmonary atresia, two-chambered right ventricle with intact ventricular septum, and atrial septal defect. Our patient had multiple apical ventricular septal defects, an association not previously reported.
The neonates with unguarded tricuspid orifices have much in common with those presenting in symptomatic fashion during the neonatal period with Ebstein's malformation. Survival of the latter cohort has greatly improved with early surgical intervention to correct right ventricular dilation by repair of the malformed tricuspid valve, exclusion of the right ventricle, or right ventricular plication [6, 7]. Therefore, we decided that the condition of our patient warranted early surgical intervention to correct the right ventricular dilation. We considered the option of excluding the right ventricle, but chose instead to plicate both the right atrium and right ventricle, minimizing right ventricular compliance. Closure of the ventricular septal defects would have been desirable, but was deemed unnecessary. Of course, if the defects had been larger and nonrestrictive, their concomitant closure would have been mandatory. Right ventricular plication created room for adequate expansion of the lungs and filling of the left ventricle [4, 5]. Our experience suggests that neonates with either unguarded tricuspid orifice or severe Ebstein's malformation can be stabilized by plicating the dilated right side of the heart, the space-occupying structures producing the major problems in the initial periods of life.
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References
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- Anderson RH, Silverman NH, Zuberbuhler JR. Congenitally unguarded tricuspid orifice: its differentiation from Ebstein's malformation in association with pulmonary atresia and intact ventricular septum Pediatr Cardiol 1990;11:86-90.[Medline]
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- Hornberger LK, Sahn DJ, Kleinman CS, Copel JA, Reed KL. Tricuspid valve disease with significant tricuspid insufficiency in the fetus: diagnosis and outcome J Am Coll Cardiol 1991;17:167-173.[Abstract]
- Lang D, Oberhoffer R, Cook A, et al. Pathologic spectrum of malformations of the tricuspid valve in prenatal and neonatal life J Am Coll Cardiol 1991;17:1161-1167.[Abstract]
- Takagaki M, Ishino K, Kawada M, et al. Total right ventricular exclusion improves left ventricular function in patients with end-stage congestive right ventricular failure Circulation 2003;108(Suppl 1):II226-II229.[Medline]
- Yun TJ, Lee SH, Ko JK. Neonatal stenotic Ebstein's anomaly: a novel technique of right ventricular exclusion J Thorac Cardiovasc Surg 2006;131:469-471.[Free Full Text]
- Reemtsen BL, Fagan BT, Wells WJ, Sternes VA. Current surgical therapy for Ebstein anomaly in neonates J Thorac Cardiovasc Surg 2006;132:1285-1290.[Abstract/Free Full Text]
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Abstract
Introduction
