HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS		QUICK SEARCH:   [advanced] Author: Keyword(s): Year:  Vol:  Page:

This Article Abstract Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): Dario E. Troise Paolo M. Arciprete Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Troise, D. E. Articles by Arciprete, P. M. Search for Related Content PubMed PubMed Citation Articles by Troise, D. E. Articles by Arciprete, P. M. Related Collections Congenital - cyanotic

Ann Thorac Surg 2001;71:1018-1019
© 2001 The Society of Thoracic Surgeons

---

Case report

Surgical repair for double outlet right ventricle and intact ventricular septum

^a Department of Pediatric Cardiac Surgery, Giovanni XXIII Hospital, Bari, Italy

Accepted for publication September 14, 2000.

Address reprint requests to Dr Troise, I Trav via Leonida Bissolati 1, ZC 70124, Bari, Italy
e-mail: troisemd{at}tin.it

	Abstract

Top Abstract Introduction Comment References

 
We report the case of a neonate with a complex cardiac anomaly that consisted of a double outlet right ventricle, intact ventricular septum, small left ventricle, and pulmonary stenosis who underwent surgical repair with a successful outcome. We have not previously found a case report of a patient with double outlet right ventricle and intact ventricular septum who is still alive and in good health at an intermediate postoperative follow-up.

	Introduction

Top Abstract Introduction Comment References

 
Double outlet right ventricle (DORV) is a cardiac lesion in which both great arteries originate from the right ventricle. Most reported cases of DORV are associated with a ventricular septal defect that represents the only outlet from the left ventricle. Double outlet right ventricle with intact ventricular septum is extremely rare. A review of past case reports and a MEDLINE search from 1966 to June 1999 yielded only 13 clinically documented postnatal cases [1–6].

A 2-day-old female neonate was referred because of severe cyanosis. Arterial blood gas analysis revealed a metabolic acidosis with a systemic saturation of 56% in 100% oxygen. Echocardiography documented normal situs, concordant atrioventricular connection, DORV with intact ventricular septum, malposition of the great vessels with a right-sided anterior aorta, critical pulmonary outflow tract obstruction, hypoplastic left atrioventricular connection (Z value, –4.3) with a left ventricle not forming the apex of the heart and an incompetent mitral valve. Intravenous infusion of prostaglandin was promptly instituted. Once the neonate was stabilized hemodynamic evaluation was undertaken, confirming the diagnosis of DORV with intact ventricular septum. During the same procedure a significant gradient was recorded between both atria; then a Rashkind septostomy was performed to increase the output of the left ventricle. At the conclusion of the procedure no gradient was detected between the right and left atrium.

Five days later the neonate underwent a modified right Blalock-Taussig shunt using a 4-mm-diameter polythetrafluoroethylene graft. After this procedure, arterial blood gas analysis disclosed a pH of 7.35, a systemic saturation of 79%, and a PaCO₂ of 61 mm Hg. Color Doppler echocardiography confirmed a well functioning shunt but a significant gradient was detected across the interatrial septum. The neonate was then scheduled for a surgical septectomy. On cardiopulmonary bypass most of the interatrial septum was removed. The patient had an uneventful postoperative course and was discharged home 2 weeks later. At the time of discharge the arterial saturation was 91% on room air and PaCO₂ was within normal limits.

Fourteen months later the patient was electively admitted for a cardiac catheterization that demonstrated no pressure gradient between the atria. The left ventricular volume was severely reduced but did not appear hypoplastic. On cardiopulmonary bypass the Blalock-Taussig shunt was sectioned and a right atriotomy was performed after cardioplegia. The mitral valve was inspected; the anulus was clearly hypoplastic and its leaflets demonstrated restricted motion and absent coaptation under testing with a saline injection. The papillary muscles appeared hypoplastic. A ventricular exclusion was then performed by suturing the medial edges of both leaflets and applying a bovine pericardium patch in a supraannular position. Once the atriotomy was sutured the aortic clamp was released, and on a warm beating heart a bidirectional cavopulmonary anastomosis was carried out. A trivial forward flow through the pulmonary outflow tract remained as an additional source of pulmonary flow. The patient was discharged home after an uneventful postoperative course on hospital day 18. Echocardiography revealed no flow through the mitral valve and a perfect exclusion of the left ventricle, which was reduced to a crescent cavity. The right ventricle was mildly enlarged but contractility was satisfactory. Arterial saturation was 85% on room air and the patient remains symptom-free on minimal furosemide at 38 months of follow-up.

	Comment

Top Abstract Introduction Comment References

 
The pathophysiology of DORV and intact ventricular septum seems quite simple. When both great vessels originate from the right ventricle with no communication between the ventricles, a dangerous situation exists. It is not surprising that most of these patients die soon after birth [1–6].

The cardiac anatomy of the published cases of DORV with intact ventricular septum is rather repetitive: all cases with malposition of the great vessels were associated with a rather small left ventricle. Both cases reported with normally related great vessels [3, 5] were associated with a well developed left ventricle. We have not previously found a case report of a patient with DORV and intact ventricular septum who underwent surgical repair and is doing well. The "fate" that we assigned to the left ventricle was strongly debated preoperatively before our final decision was made.

We recommend prompt surgical septectomy as the initial surgical step because of a strong tendency of the foramen ovale to become critically restrictive in the condition of mitral hypoplasia. The efficacy of this approach is confirmed by the observation that all patients who survived surgery [4, 6] have undergone septectomy as part of their treatment.

The complete and long-lasting reduction of the small systemic ventricle to a vestigial cavity after closure of the mitral valve confirmed our preoperative impression that the retrograde flow through the mitral valve decompresses the left ventricle, allowing survival in the fetal stage but masking an "untraining" of the left ventricular myocardium. Retrospectively we speculate that the presence of malposition of the great vessels is a strong pathologic marker of an "untrained" systemic ventricular chamber and an indication for left ventricular exclusion in this condition.

We have reported a case of DORV and intact ventricular septum that is unusual in that the infant received permanent palliation with the result of optimal hemodynamic condition at 38 months of follow-up. A possible Fontan operation is anticipated within 5 years of age.

	References

Top Abstract Introduction Comment References

 

1. Descalzo A., Canadas M., Cintado C., Castillo J.A., Miralles J., Ariza S. Right ventricle with double outlet and intact interventricular septum. Report of 1 case and review of the literature. Arch Mal Coeur Vaiss 1979;72:899-903.[Medline]
2. Sridaramont S., Feldt R.H., Ritter D.G., Davis G.D., Edwards J.E. Double outlet right ventricle. Hemodynamic and anatomic correlations. Am J Cardiol 1976;38:85-94.[Medline]
3. Gerlis L.M., Dickinson D.F., Anderson R.H. Disadvantageous closure of the interventricular communication in double outlet right ventricle. Br Heart J 1984;51:670-673.[Abstract/Free Full Text]
4. Accorsi F., Thiene 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]
5. Marino B., Loperfido F., Sardi C.S. Spontaneous closure of ventricular septal defect in a case of double outlet right ventricle. Br Heart J 1983;49:608-611.[Abstract/Free Full Text]
6. Ikemoto Y., Nogi S., Teraguchi M., Imamura H., Kobayashi Y. Double-outlet right ventricle with intact ventricular septum. Acta Paediatr Jpn 1997;39:233-236.[Medline]

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, R. Formigari, B. Chiappini, G. Frascaroli, and G. Gargiulo Surgical management of double outlet right ventricle with intact ventricular septum Ann. Thorac. Surg., February 1, 2003; 75(2): 586 - 587. [Abstract] [Full Text] [PDF]

This Article Abstract Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): Dario E. Troise Paolo M. Arciprete Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Troise, D. E. Articles by Arciprete, P. M. Search for Related Content PubMed PubMed Citation Articles by Troise, D. E. Articles by Arciprete, P. M. Related Collections Congenital - cyanotic