Ann Thorac Surg 2003;75:586-587
© 2003 The Society of Thoracic Surgeons
Case report
Surgical management of double outlet right ventricle with intact ventricular septum
Carlo Pace Napoleone, MD*a,
Roberto Formigari, MDb,
Bruno Chiappini, MDa,
Guido Frascaroli, MDc,
Gaetano Gargiulo, MDa
a Department of Pediatric Cardiac Surgery, S. Orsola-Malpighi Hospital, Bologna, Italy
b Department of Pediatric Cardiology, S. Orsola-Malpighi Hospital, Bologna, Italy
c Department of Cardiac Anesthesiology, S. Orsola-Malpighi Hospital, Bologna, Italy
Accepted for publication August 19, 2002.
* Address reprint requests to Dr Napoleone, Pediatric Cardiac Surgery, S. Orsola-Malpighi Hospital, Via Massarenti 9, 40138 Bologna, Italy
e-mail: pace{at}orsola-malpighi.med.unibo.it
 |
Abstract
|
|---|
A very rare case of double outlet right ventricle with intact ventricular septum and unrestricted pulmonary flow was successfully palliated with pulmonary banding and delayed bidirectional cavopulmonary anastomosis and mitral avulsion. This is the only case of pulmonary banding with mitral avulsion reported in the literature for this type of heart defect.
|
Introduction
|
|---|
An intact ventricular septum in double outlet right ventricle is an exceedingly rare occurrence. Few cases have been described and only 3 patients did survive first-stage palliation in the form of percutaneous [1] or surgical [2] atrial septostomy or a systemic-to-pulmonary shunt [3]. Left ventricular and mitral valve hypoplasia were always present. Indeed only 1 patient so far has been reported as successfully treated by a bidirectional cavopulmonary shunt [4].
We report the case of an infant with double outlet right ventricle, intact ventricular septum, and unrestricted pulmonary flow who underwent pulmonary artery banding as a neonate and a successful bidirectional Glenn shunt at 6 months of age.
The patient was delivered after an uneventful pregnancy. Mild cyanosis was present with a marked heart enlargement on chest roentgenogram. Two-dimensional echocardiography showed situs solitus of the viscera and atria, levocardia, D-loop ventricles, double outlet right ventricle with intact interventricular septum, and normally related great arteries (posterior D-aorta). The left ventricle was mildly hypoplastic (19 mL/m2) with a hammock mitral valve that was severely incompetent. The right ventricular outflow tract was widely patent with a well-developed bilateral infundibulum. A levoatriocardinal vein was also present. Owing to a restrictive atrial septal defect, balloon septostomy was promptly needed. At 5 days of age surgical palliation by pulmonary arterial banding was carried out for the onset of heart failure. After a smooth postoperative course, the neonate was discharged in good clinical condition, 80% oxygen saturation on room air, and a 70 mm Hg systolic gradient through the pulmonary band at Doppler examination.
At 6 months of age cardiac catheterization and angiography (Fig 1) showed a systemic right ventricular pressure without subaortic stenosis and a well-positioned pulmonary band with a 60 mm Hg systolic gradient. A suprasystemic pressure (130 mm Hg systolic) was recorded in the left ventricle. The patient was referred for surgery and underwent a bidirectional cavopulmonary anastomosis with atrioseptectomy and mitral avulsion. At the end of the extracorporeal circulation, intraoperative recordings showed low left ventricular pressures (40 mm Hg systolic) with normal atrial pressures and moderately increased v waves. The pulmonary band was tightened to leave a small pulsatile flow into the pulmonary arteries. The child had an uneventful postoperative course and was discharged with 80% oxygen saturation. Follow-up evaluation after 8 months shows excellent clinical condition with 84% saturation on room air. Echocardiography (Fig 2)
shows fair right ventricular performance, the absence of systemic outflow tract obstruction, a low pressure left ventricle (by Doppler evaluation of the left ventricle-left atrium regurgitation), and a low velocity left-to-right shunt through an unrestrictive atrial communication.
View larger version (181K):
[in this window]
[in a new window]
|
Fig 1. Angiographic study of the right ventricle (RV) 6 months after pulmonary artery banding. (Ao = aorta; PA = pulmonary artery.)
|
|
View larger version (89K):
[in this window]
[in a new window]
|
Fig 2. End-systolic (panel A) and end-diastolic (panel B) echocardiographic four-chamber view showing the small left ventricle (LV), which is allowed to decompress into the left atrium (LA) after mitral valve avulsion. (RA = right atrium; RV = right ventricle.)
|
|
|
Comment
|
|---|
With respect to the cases reported in the literature this patient presented some challenging aspects. Indeed the presence of a subpulmonary infundibulum with various degrees of pulmonary stenosis, as previously reported in the literature [3, 4], is the most favorable anatomy in that it protects the lungs from high blood flow, thus permitting consideration of a cavopulmonary anastomosis later in life. If severe stenosis with low systemic saturation occurs, a systemic-to-pulmonary shunt can be accomplished. Conversely, it is not an easy task to perform a well-balanced pulmonary arterial banding in a significantly desaturated neonate, even with complete ligature of the pulmonary trunk with a modified Blalock shunt as a last ditch resort in case of a critically low arterial saturation. Moreover the presence of a bilateral infundibulum was a matter of concern as a risk factor for late subaortic obstruction. Our patient did tolerate pulmonary banding very well without developing systemic outflow tract obstruction so that a cavopulmonary shunt could be planned. We performed a complete mitral avulsion instead of simply patching the mitral orifice as reported by Troise and coworkers [4]. In our opinion this solution avoids leaving a small but highly hypertensive ventricle that may be the cause of late arrhythmias, similar to what is believed to be the case for the right ventricle in the setting of pulmonary atresia with an intact septum. Our patient is the only case of pulmonary banding with mitral avulsion reported in the literature for this type of heart defect.
This rare anatomic entity is still a difficult challenge, especially if there is an unrestricted pulmonary flow with a subaortic infundibulum. Nevertheless pulmonary banding should be attempted to provide the ground for a later cavopulmonary shunt even in the presence of a risk for potential subaortic obstruction. In our opinion the left ventricle should be allowed to collapse by mitral valve avulsion and retrograde decompression.
|
References
|
|---|
- Cheung Y.F., Yung T.C., Leug M.P. Left ventriculo-coronary communications in double-outlet right ventricle with an intact ventricular septum. Int J Cardiol 2000;74:227-229.[Medline]
- Accorsi F., Tiene G. Double-outlet right ventricle with intact interventricular septum. Case report, review of the literature and proposed pathogenetic interpretation. G Ital Cardiol 1985;15:232-237.[Medline]
- Vairo U., Tagliente M.R., Fasano M.L., Adurno G., Serino W. Double-outlet right ventricle with intact ventricular septum. Ital Heart J 2001;2:397-400.[Medline]
- Troise E.D., Ranieri L., Arciprete P.M. Surgical repair for double outlet right ventricle and intact ventricular septum. Ann Thorac Surg 2001;71:1018-1019.[Abstract/Free Full Text]
This article has been cited by other articles:
|
|
|
|
 
D. E. Troise, L. Ranieri, and P. M. Arciprete
Optimal Surgical Repair for Double Outlet Right Ventricle and Intact Ventricular Septum
Ann. Thorac. Surg.,
May 1, 2005;
79(5):
1824 - 1824.
[Full Text]
[PDF]
|
|
|
|
|
|
|
C. P. Napoleone
Optimal Surgical Repair for Double Outlet Right Ventricle and Intact Ventricular Septum: Reply
Ann. Thorac. Surg.,
May 1, 2005;
79(5):
1824 - 1824.
[Full Text]
[PDF]
|
|
|
Top
Abstract
Introduction
