Ann Thorac Surg 2009;88:688-689. doi:10.1016/j.athoracsur.2008.11.063
© 2009 The Society of Thoracic Surgeons
How To Do It
Hybrid Ventricular Decompression in Pulmonary Atresia With Intact Septum
Redmond P. Burke, MDa,*,
Robert L. Hannan, MDa,
Jennifer A. Zabinsky, MEnga,
Christopher F. Tirotta, MDb,
Evan M. Zahn, MDc
a Department of Cardiovascular Surgery, Miami Children's Hospital, The Congenital Heart Institute at Miami Children's Hospital, Miami, Florida
b Department of Anesthesiology, Miami Children's Hospital, The Congenital Heart Institute at Miami Children's Hospital, Miami, Florida
c Department of Cardiology, Miami Children's Hospital, The Congenital Heart Institute at Miami Children's Hospital, Miami, and Arnold Palmer Hospital for Children, Orlando, Florida
Accepted for publication November 26, 2008.
* Address correspondence to Dr Burke, Miami Children's Hospital, Division of Cardiovascular Surgery, 3100 SW 62nd Ave, Miami, FL 33155 (Email: redmond111{at}aol.com).
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Abstract
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Initial palliation for pulmonary atresia with intact ventricular septum continues to evolve in the face of significant early and late morbidity. In patients with suitable anatomy, decompression of the right ventricle may be the first step in treatment. A hybrid approach to right ventricular decompression, combining surgery and interventional catheterization techniques is described. Direct access to the right ventricle through a subxiphoid incision with transventricular sheath placement is used to provide optimum catheter position for radiofrequency perforation of membranous pulmonary atresia followed by balloon dilation. The technique may address key limitations of the traditional surgical and interventional approaches.
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Introduction
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Pulmonary atresia with intact ventricular septum continues to be associated with high early and late mortality [1]. Patients with favorable anatomy, including membranous pulmonary atresia, tripartite right ventricle (RV), and adequate tricuspid valve size may benefit from decompression of the RV with the ultimate goal being two-ventricle repair. Efforts to reduce therapeutic trauma led to the development of transcatheter techniques for valve perforation and subsequent balloon valvuloplasty [2]. The transcatheter technique has a significant procedural failure rate and a persistent risk of right ventricular perforation leading to tamponade [3]. In this report we describe a synthesis of surgical and interventional techniques to treat pulmonary atresia with intact ventricular septum.
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Technique
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Our technique was derived from our experience treating cardiac perforation and pericardial tamponade after attempted Bayliss Nykanen radiofrequency wire perforation of membranous pulmonary atresia. In that circumstance, a median sternotomy was performed, and the perforation was repaired. A pursestring suture was placed in the RV free wall and a dilator was passed directly through the membranous atresia. The pulmonary valve was subsequently balloon dilated. The patient had an excellent outcome. The observation was made in the catheterization laboratory that the approach to the pulmonary valve from the RV offered a much more favorable angle than the angle from the tricuspid valve, and that the unfavorable angle from the tricuspid valve resulted in the cardiac perforation. Direct approach through the RV seemed to make the approach to the pulmonary valve much more straightforward and reduced the risk of cardiac perforation in selected patients.
Our current practice in patients with anatomy suitable for RV decompression is to proceed with cardiac catheterization and assess the angle of the transvenous catheter in relationship to the infundibulum and the membranous pulmonary atresia. If the angle seems to be favorable for radiofrequency perforation through the femoral vein and tricuspid valve, then we proceed with that approach. If the angle between the catheter and the valve plate seems to place the tip of the catheter in an unfavorable position (Fig 1), we believe the hybrid approach is indicated, providing the safest and least traumatic approach to RV decompression. A 2-cm longitudinal incision over the lower sternum is made and the xiphoid process is removed. The pericardium is opened and stay sutures are used to evert the pericardium over the skin edge, exposing the diaphragmatic surface of the RV. A pursestring suture of 6-0 Prolene (Ethicon, Somerville, NJ) is placed in the RV epicardium, and a needle is advanced through the center of the pursestring suture. A guidewire is passed through the needle under fluoroscopic guidance and a sheath is passed over the guidewire into the RV chamber. The angle of approach from the base of the heart allows excellent alignment of the radiofrequency catheter with the infundibulum and valve plate (Fig 2a). After ensuring perfect alignment, radiofrequency energy is used to perforate the valve plate followed by serial balloon dilatations of the pulmonary valve through the RV sheath (Fig 2b). After completion angiography and pressure measurements, the sheath is removed, the pursestring suture is tied, and the incision is closed.

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Fig 1. Percutaneous approach of a lateral angiogram performed in an atretic infundibulum using a standard percutaneous approach to attempt valve perforation. Note the anterior alignment of the radiofrequency wire (arrow) in relationship to the imperforate valve plate secondary to the course of the catheter from the anterior to the posterior (*) position through the tricuspid valve. This position carries a high risk of cardiac perforation and resultant tamponade.
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Fig 2. The hybrid approach of (a) a lateral angiogram in the infundibulum demonstrating the simple, straight catheter (*) course from a subxiphoid approach. Note the favorable posterior position along the valve plate that the radiofrequency wire was directed across (arrow). (b) Final angiographic result after perforation and valvuloplasty demonstrating a widely patent right ventricular outflow tract.
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We have used the hybrid approach on 3 patients, including the initial approach in the setting of the RV perforation. All 3 patients have done well without significant complications. One patient had repeat transcatheter pulmonary valve dilation and 1 patient underwent modified Blalock-Taussig shunt. All 3 patients remain in a two-ventricle pathway.
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Comment
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Although reports continue to focus on the relative superiority or inevitability of catheter versus surgical techniques for RV decompression via opening the right ventricular outflow tract [3], a combined approach might be optimal. Hamilton and colleagues [4] at Leeds first described operative balloon dilation [4], a cooperative effort between surgeons and interventional cardiologists, but the technique was limited by the requirement of a full sternotomy and large angioplasty catheters. Our hybrid technique mitigates the trauma of open surgery by avoiding cardiopulmonary bypass, a right ventriculotomy, and a full median sternotomy. Virtually all of the limitations of transcatheter techniques for valve perforation (ie, vascular injury, creating tricuspid insufficiency, arrhythmias, unstable catheter positions, failure to align the catheter with the pulmonary artery, cardiac perforation, and resultant tamponade) may be moderated by surgically establishing a short, straight, stable catheter course, with a transcardiac sheath. These advantages may be even more striking in premature newborns. Further investigation will determine whether procedure times, contrast, radiation exposure, morbidity, and mortality are reduced with this approach.
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References
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- Guleserian KJ, Armsby LB, Thiagarajan RR, del Nido PJ, Mayer Jr JE. Natural history of pulmonary atresia with intact ventricular septum and right-ventricle-dependent coronary circulation managed by the single-ventricle approach Ann Thorac Surg 2006;81:2250-2257.[Abstract/Free Full Text]
- Parsons JM, Rees MR, Gibbs JL. Percutaneous laser valvotomy with balloon dilatation of the pulmonary valve as primary treatment for pulmonary atresia Br Heart J 1991;66:36-38.[Abstract/Free Full Text]
- McLean KM, Pearl JM. Pulmonary atresia with intact ventricular septum: initial management Ann Thorac Surg 2006;82:2214-2219.[Abstract/Free Full Text]
- Hamilton JR, Fonseka SF, Wilson N, Dickinson DF, Walker DR. Operative balloon dilatation for pulmonary atresia with intact ventricular septum Br Heart J 1987;58:374-377.[Abstract/Free Full Text]
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S. Li, W. Chen, Y. Zhang, H. Zhang, Z. Hua, D. Wang, and S. Hu
Hybrid Therapy for Pulmonary Atresia With Intact Ventricular Septum
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May 1, 2011;
91(5):
1467 - 1471.
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