Ann Thorac Surg 2012;93:1703-1704. doi:10.1016/j.athoracsur.2011.08.040
© 2012 The Society of Thoracic Surgeons
Case Reports
Starnes Procedure in a Neonate With Pulmonary Atresia and Intact Ventricular Septum
Alejandro R. Arevalo, MDa,
Umar S. Boston, MDb,
Steven P. Goldberg, MDb,*,
Judith A. Becker, MDa,
Christopher J. Knott-Craig, MDb
a Department of Pediatric Cardiology, University of Tennessee Health Science Center, Le Bonheur Children's Hospital, Memphis, Tennessee
b Department of Pediatric Cardiothoracic Surgery, University of Tennessee Health Science Center, Le Bonheur Children's Hospital, Memphis, Tennessee
Accepted for publication August 15, 2011.
* Address correspondence to Dr Goldberg, Pediatric Cardiothoracic Surgery, Le Bonheur Children's Medical Center, 50 N Dunlap St, Ste 2598, Memphis, TN 38103 (Email: steven.goldberg{at}lebonheur.org).
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Abstract
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We describe the case of 7-day-old neonate with pulmonary atresia, intact ventricular septum, and severe tricuspid valve (TV) dysplasia who underwent a Starnes right ventricular exclusion procedure (RVEP). The patient had severe tricuspid stenosis and regurgitation and right ventricular dysfunction after perforation and balloon dilation of the pulmonary valve.
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Introduction
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The Starnes right ventricular exclusion procedure (RVEP) has been used as a form of single-ventricle palliation for symptomatic neonates with Ebstein's anomaly. This was first described by Starnes and associates in 1991 [1]. We present another indication for the use of RVEP in a neonate with pulmonary atresia/intact septum (PA/IVS) with severe tricuspid regurgitation after a catheter-based pulmonary perforation and valvuloplasty.
The patient is a 7-day-old, 3.2-kg neonate born at 35 weeks' gestation to an insulin-dependent diabetic mother. The patient was diagnosed with PA/IVS and severe TV dysplasia by fetal echocardiography. On physical examination, the patient was afebrile with systemic saturation of 92% on room air, heart rate of 140 beats/minute, respiration rate of 40 breaths/minute, and a systemic blood pressure of 62/32 mm Hg. Cardiac examination revealed a II/VI early systolic murmur on the left sternal border and a single S2 sound. The postnatal echocardiogram confirmed the diagnosis. The pulmonary valve annulus measured 4.7 mm with fused commissures. There was moderate (2/4) tricuspid valve (TV) stenosis and moderate to severe (3/4) regurgitation. The effective TV orifice was 7 mm. The right ventricle was moderately hypoplastic and bipartite without a trabecular portion.
The patient was initially maintained on a prostaglandin infusion and then underwent palliation in the catheterization laboratory where perforation and balloon pulmonary valvuloplasty was performed at 4 days of life. This established a 6-mm pulmonary valve orifice with significant pulmonary regurgitation. Over the ensuing 3 days the patient had progressively worsening right ventricular failure with cardiomegaly (Fig 1), hepatomegaly, ascites, renal insufficiency, and elevated lactate levels despite optimal medical therapy. A second echocardiogram showed severe tricuspid regurgitation and severe pulmonary insufficiency with a leftward deviated ventricular septum (Fig 2).
The decision for surgical repair was made with a planned biventricular strategy. Intraoperatively the TV was not amenable to repair. Because of the small size of the right ventricular cavity, a bioprosthesis, even in a supraannular position, was considered too bulky and would have resulted in TV or right ventricular outflow tract obstruction, or both.
Single-ventricle palliation using an RVEP was decided on. This would provide stable systemic-to-pulmonary blood flow as well as eliminate the patient's right heart failure. RVEP included atrial septectomy, excision of TV leaflets, closure of the TV with a 3-mm fenestrated patch, subtotal ligation of the main pulmonary artery, reduction right atrioplasty, and a 4-mm systemic-to-pulmonary artery shunt from the aorta to the confluence of the pulmonary artery. The patient was weaned from cardiopulmonary bypass on minimal inotropic support with good left ventricular function and no right ventricular distention. The postoperative course was unremarkable, and the patient was discharged home on the 11th postoperative day.
As of this writing, the patient is now 9 months old and has since successfully undergone a bidirectional Glenn's anastomosis at 5 months of age and is thriving at home with appropriate weight gain and developmental milestones. Left ventricular function was normal on the most recent echocardiogram.
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Comment
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Management strategies for patients with PA/IVS and associated TV disease varies between institutions [2–4]. The goal is to provide stable pulmonary blood flow and alleviate the ductal dependent circulation. This can be achieved by establishing prograde flow either surgically or by balloon angioplasty. Critical to the decision making is the adequacy of the right ventricular volume, which can be difficult to quantify by echocardiography. This is because of its crescent shape and its cumbersome proximal relation to the sternum, which makes it difficult to image by echocardiography. The size of the TV has been used as a surrogate to estimate right ventricular volume [5]. If the right ventricular volume is estimated to be small, a systemic-to-pulmonary shunt will be required in addition to the prograde flow.
Unique to our case was the severe right heart failure secondary to the tricuspid regurgitation that was present. Adding a systemic-to-pulmonary shunt in the face of severe tricuspid regurgitation and pulmonary regurgitation would have resulted in either a circular shunt or worsening right ventricular function from the increased volume load, or both. In our patient, RVEP not only provided a means to alleviate the ductal dependent circulation but also was successful in relieving the degree of right ventricular failure.
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References
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- Starnes VA, Pitlick PT, Bernstein D, Griffin ML, Choy M, Shumway NE. Ebstein's anomaly appearing in the neonate: a new surgical approach J Thorac Cardiovasc Surg 1991;101:1082-1087.[Abstract]
- Knott-Craig CJ, Overholt ED, Ward KE, Razook JD. Neonatal repair of Ebstein's anomaly: indications, surgical technique, and medium term follow-up Ann Thorac Surg 2000;69:1505-1510.[Abstract/Free Full Text]
- Knott-Craig CJ, Overholt ED, Ward KE, Ringewald JM, Baker SS, Razook JD. Repair of Ebstein's anomaly in the symptomatic neonate: an evolution of technique with 7-year follow-up Ann Thorac Surg 2002;73:1786-1792.[Abstract/Free Full Text]
- Reemtsen BL, Fagan B, Wells W, Starnes VA. Current surgical therapy for Ebstein anomaly in neonates J Thorac Cardiovasc Surg 2006;132:1285-1289.[Abstract/Free Full Text]
- Salvin JW, Melhinney DB, Colan SD, et al. Fetal tricuspid valve size and growth as predictors of outcome in pulmonary atresia with intact ventricular septum Pediatrics 2006;118:e415-e420.[Abstract/Free Full Text]
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Abstract
Introduction
