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Ann Thorac Surg 1997;63:746-750
© 1997 The Society of Thoracic Surgeons

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Original Article: Cardiovascular

Bidirectional Cavopulmonary Anastomosis With Intracardiac Repair

Section of Cardiology, Department of Pediatrics, and Department of Cardiovascular Surgery, Children's Hospital of Michigan, Wayne State University, Detroit, Michigan

Accepted for publication October 21, 1996.

	Abstract

Top Footnotes Abstract Introduction Material and Methods Results Comment References

 
Background. Patients with hypoplasia of the right ventricle and tricuspid valve have historically undergone a total cavopulmonary connection or a two-ventricle repair with atrial fenestration.

Methods. We reviewed our experience with 9 patients with hypoplasia of the right ventricle and tricuspid valve who underwent a bidirectional cavopulmonary anastomosis with intracardiac repair. Patient diagnoses included pulmonary atresia with intact ventricular septum (n = 3); hypoplastic right ventricle and tricuspid valve with atrial septal defect, ventricular septal defect, and right ventricular outflow tract obstruction (n = 3); unbalanced atrioventricular canal defect (n = 1); inlet ventricular septal defect with coarctation (n = 1); and tricuspid stenosis with atrial septal defect (n = 1).

Results. The median age at operation was 36 months. There was hypoplasia of the right ventricle and tricuspid valve in all patients. The tricuspid valve measured 56.5% of normal (range, 43.6% to 70.4%) by echocardiography, and the median ratio of the tricuspid valve to the mitral valve was 0.67 (range, 0.54 to 0.82). At operation, the median tricuspid valve annulus diameter was 65.6% of published autopsy normals (range, 57.8% to 78.5%) with a median Z value of -3.8 (range, -6.6 to -2.1). All patients survived operation. At a median follow-up of 16 months, 5 patients are asymptomatic, and 2 have occasional early-morning periorbital edema. Two patients are on a regimen of diuretics, 1 of whom is also taking an unloading agent. The patient with unbalanced atrioventricular canal died suddenly at home 6 months postoperatively.

Conclusions. Bidirectional cavopulmonary anastomosis with intracardiac repair may avoid the long-term complications associated with the Fontan modifications and eliminates the need of atrial fenestration in most instances. This operation should be considered for select patients with hypoplasia of the right ventricle and tricuspid valve.

	Introduction

Top Footnotes Abstract Introduction Material and Methods Results Comment References

 
A variable degree of right ventricular (RV) hypoplasia occurs in patients with pulmonary atresia and intact ventricular septum. Palliative procedures that establish flow from the RV to the pulmonary artery in these patients encourage RV and tricuspid valve growth [1, 2]. Despite their growth, persistent hypoplasia of the two structures may preclude a standard two-ventricle repair in these patients [1–3]. Right ventricular hypoplasia can also occur in patients with unbalanced forms of complete atrioventricular canal defect and with other uncommon congenital cardiac anomalies. Initial palliative procedures in these patients are directed at balancing systemic and pulmonary blood flows and protecting the pulmonary vascular bed. Preliminary procedures typically include systemic–pulmonary artery shunts for hypoxemia and pulmonary artery banding for excessive pulmonary blood flow. Standard two-ventricle repair may not be feasible in these patients because the RV and the tricuspid valve usually remain hypoplastic.

Hypoplasia of the RV and tricuspid valve is a spectrum of anatomic variations requiring individualized management. The long-term management of patients with more severe hypoplasia of these two structures has historically included performing various forms of cavopulmonary anastomoses: Glenn's procedure, bidirectional cavopulmonary shunt (BCPS), and the Fontan procedure (direct atriopulmonary connection). Most recently, the total cavopulmonary connection has been the operation of choice. In patients whose RV and tricuspid valve are larger but still hypoplastic, biventricular repair with RV muscular resection and atrial fenestration has been performed. In patients with a nearly normal RV and tricuspid valve, a more standard two-ventricle repair with or without atrial fenestration can be done. The Glenn shunt, BCPS, and total cavopulmonary connection have all been associated with the formation of arteriovenous fistulas [4–6]. Patients with a two-ventricle repair who require atrial fenestration may have hypoxia and the problems of chronic cyanosis. It is not clear whether they may also have a slightly increased risk for paradoxical emboli [7].

Since 1990 at Children's Hospital of Michigan, we have performed a BCPS with intracardiac repair in select patients with hypoplasia of the RV and tricuspid valve. Our experience describes a satisfactory strategy for the management of patients whose RV hypoplasia would otherwise have precluded biventricular repair.

	Material and Methods

Top Footnotes Abstract Introduction Material and Methods Results Comment References

 
Patients
Nine patients with hypoplastic RV as a part of their congenital heart disease underwent a BCPS with intracardiac repair at Children's Hospital between January 1, 1990, and December 31, 1995. Diagnoses included pulmonary atresia with intact ventricular septum (n = 3); hypoplastic RV with atrial septal defect (ASD), ventricular septal defect, and RV outflow tract obstruction (n = 3); left-dominant atrioventricular canal defect with hypoplastic RV (n = 1); secundum ASD with inlet ventricular septal defect, hypoplastic RV, and juxtaductal aortic coarctation (n = 1); and tricuspid stenosis with hypoplastic RV, ASD, and partial anomalous pulmonary venous return (n = 1). Preliminary palliative operations included systemic–pulmonary artery shunts with transventricular pulmonary valvotomy in patients with pulmonary atresia, and pulmonary artery banding in patients with excessive pulmonary blood flow. Patient diagnosis, type of palliative operation, and age at intervention are found in Table 1.

Echocardiographic Assessment
The echocardiograms obtained prior to BCPS with intracardiac repair were retrospectively reviewed. In all patients, the RV appeared hypoplastic, although this was difficult to quantify. In each patient, the tricuspid valve was measured, and the largest diameter obtained from the apical four-chamber view was indexed for body surface area and compared with the normal values of King and colleagues [8]. The mitral valve diameter was measured for each patient on the same echocardiogram, and a ratio of the tricuspid valve diameter to the mitral valve diameter was obtained. Tricuspid valve Z values were determined by comparison with published normal data [8].

Intraoperative Assessment
At the time of BCPS with intracardiac repair, the RV was confirmed to be hypoplastic in each patient by direct observation, and the tricuspid valve hypoplasia was quantified by direct measurement with Hegar's dilators. Intraoperative tricuspid valve diameters were indexed for body surface area and compared with the published autopsy data of Rowlatt and colleagues [9]. The Z values for the tricuspid valve measurements were determined retrospectively using the data tables published by the same authors [9].

Surgical Procedure
Through a median sternotomy, cardiopulmonary bypass was instituted. In the 3 patients with pulmonary atresia and intact ventricular septum, the shunt was first ligated. Through a right atriotomy in all patients, the tricuspid valve and the RV were inspected. The intracardiac lesions were then repaired. The RV was enlarged in each patient as much as possible by myotomy and myectomy. Right ventricular outflow tract enlargement and pulmonary artery angioplasties were performed as necessary. To avoid any possibility that cannulation would distort the anatomy, total circulatory arrest was instituted for construction of the bidirectional cavopulmonary anastomosis. Circulatory arrest times ranged from 14 to 38 minutes (mean time, 25.8 minutes). The first and smallest patient also had a 4-mm atrial fenestration placed.

Follow-up
Postoperative echocardiography was performed in all patients at the time of discharge from the hospital and as indicated clinically during follow-up. Each patient's clinical status at the time of the last office visit was reviewed for symptoms, physical findings, medications, and overall clinical status.

	Results

Top Footnotes Abstract Introduction Material and Methods Results Comment References

 
The data from the echocardiograms preceding definitive repair are summarized in Table 2. The median tricuspid valve diameter, expressed as percent of normal, was 56.5% (range, 43.6% to 70.4%). The median ratio of the tricuspid valve diameter to the mitral valve diameter was 0.67 (range, 0.54 to 0.82). The median Z value for the tricuspid valve by echocardiography was -3 (range, -5.4 to -1.4).

Eight of the 9 patients have been followed up for a median of 16 months (range, 4 months to 5.8 years). Five are asymptomatic and free from medications. Two have occasional early-morning periorbital edema; 1 of these 2 remains on a regimen of diuretics and captopril. One patient has had development of paroxysmal supraventricular tachycardia late postoperatively and is being treated with a selective ß blocker. His identical twin, who underwent ASD and ventricular septal defect closure but did not require a BCPS, also has paroxysmal supraventricular tachycardia. There has been one late death. The patient had Down's syndrome and left-dominant atrioventricular canal, and the family failed to keep follow-up visits and maintain medical regimens. The child died suddenly at home of unknown causes 6 months after operation.

Echocardiography in all patients at the time of hospital discharge after operation confirmed unobstructed flow through the BCPS to the lungs and pulsatile flow from the RV to the pulmonary arteries. Routine echocardiograms made at the time of follow-up visits have confirmed forward flow through the RV and patency of the cavopulmonary anastomosis. In the patient with the atrial fenestration, the ASD measures 1 to 2 mm and has bidirectional flow by echocardiography 5.8 years postoperatively. This oxygen saturation of this patient is 93% and that of his identical twin is 97%.

	Comment

Top Footnotes Abstract Introduction Material and Methods Results Comment References

 
In 1989, Billingsly and colleagues [10] described a superior vena cava (SVC)–pulmonary artery anastomosis combined with RV enlargement and an adjustable ASD snare in 4 of their patients with pulmonary atresia and intact ventricular septum. In 1991, Kobayashi and colleagues [11] reported their results with bidirectional cavopulmonary anastomosis to augment pulmonary flow in 10 patients. Five of those patients had continuous pulsatile flow provided by a contralateral Blalock-Taussig shunt, 4 had additional pulmonary flow through a stenotic pulmonary valve, and 1 had both. Oxygen saturations were improved after the BCPS in these patients, and atrioventricular valve regurgitation improved in 5 of the 10. Angiography in some of the patients confirmed flow from the cavopulmonary anastomosis to both lungs despite pulsatile flow through the shunt or the pulmonary valve. Kobayashi and colleagues [11] concluded that bidirectional cavopulmonary anastomosis can augment pulmonary flow from the ventricle or through a Blalock-Taussig shunt with favorable results.

Patients with a hypoplastic RV and tricuspid valve present a spectrum of anatomic variations, and the surgical management requires individualization. Despite having a pulmonary valvotomy or a transannular patch, patients with pulmonary atresia and intact ventricular septum often have persistent RV hypoplasia [1–3]. A hypoplastic but potentially usable RV also occurs not uncommonly with left-dominant atrioventricular canal and with inlet ventricular septal defect and straddling tricuspid valve. Tricuspid stenosis and RV hypoplasia can be seen as an isolated lesion, as in our patient 9. It has also been reported with tetralogy of Fallot [12].

Treatment strategies for this population of patients with RV hypoplasia have most recently included the total cavopulmonary anastomosis (modified Fontan) or a two-ventricle repair with atrial fenestration. The BCPS with intracardiac repair described here is ideal for patients whose RV is too small for two-ventricle repair with fenestration, but large enough to accommodate the systemic venous return from the inferior vena cava. The combination of SVC flow and pulsatile RV flow to the pulmonary arteries as described in our patients appears to be well tolerated and can be accomplished with very satisfactory results. This operation recruits the hypoplastic RV, thereby providing pulsatile pulmonary blood flow, and the cavopulmonary connection passively augments pulmonary blood flow and effectively reduces the volume load on the RV. Longer follow-up is required to determine whether any arteriovenous fistulas will develop as seen with BCPS alone, but we remain optimistic that the pulsatile flow entering the pulmonary arteries from the RV will prevent fistula formation.

We have no definite absolute criteria in the spectrum of RV and tricuspid valve hypoplasia where BCPS with intracardiac repair is preferred over total cavopulmonary connection. We performed successful BCPS with intracardiac repair in patients whose tricuspid valves were as small as -5.4 Z values below normal. Muster and colleagues [12] in 1993 described 5 patients in whom they carried out a cavopulmonary connection in addition to the intracardiac repair. In these 5 patients, the mean diastolic volume of the RV was 48.4% of predicted and the mean stroke volume, 40.2%. The authors noted that absolute sizes of the RV and the tricuspid valve may not be the only factors that influence the success of this operation. Right ventricular compliance, tricuspid regurgitation, RV outflow tract, relative hypoplasia of the pulmonary arterial system, and pulmonary vascular resistance are important additional considerations in these patients. The presence of a competent pulmonary valve does not appear to be essential, as we performed extensive transannular patching in most of our patients.

In 1994, Gentles and co-workers [13] described an anastomosis of the distal SVC to the superior aspect of the right pulmonary artery and an anastomosis of the SVC–right atrial junction to the inferior aspect of the right pulmonary artery in 8 patients whose RV stroke volumes were 9% to 25% of those of the left ventricle. In 5 of these 8 patients, the right pulmonary artery was ligated proximal to the cavopulmonary anastomoses. This operation permits inferior vena cava blood to either pass through the hypoplastic RV to the main pulmonary artery or flow directly to the pulmonary system through the inferior anastomosis of the cavoatrial junction to the right pulmonary artery. Time will tell if this operation is superior to the total cavopulmonary anastomosis for the severely hypoplastic RV.

Alvarado and associates [14] reported their experience in 9 patients who had inlet ventricular septal defect or complete atrioventricular canal with hypoplasia of the RV. The authors studied flow patterns by echocardiography after successful two-ventricle correction incorporating a bidirectional cavopulmonary anastomosis. They documented systolic reversal of flow in the SVC for the first 6 weeks postoperatively. Then the reversal of SVC flow disappeared, presumably because of improvement in left ventricular compliance and a fall in pulmonary vascular resistance. Late postoperatively, there was respiratory-dependent SVC flow to the right pulmonary artery and proximal left pulmonary artery in diastole and pulsatile flow from the RV to both lungs in systole. This flow pattern has been corroborated by cardiac catheterization in a limited number of patients postoperatively [11, 12]. These observations and ours confirm that the small but usable RV can provide systolic flow to both lungs, while the cavopulmonary anastomosis effectively reduces the volume load on the small right RV.

In conclusion, a cavopulmonary anastomosis can be performed in combination with intracardiac repair. The pulsatile systolic flow to the lungs from the RV may prevent the development of arteriovenous fistulas. Atrial fenestration is rarely necessary, thus obviating its possible complications. Bidirectional cavopulmonary anastomosis with intracardiac repair is a viable alternative for patients with RV hypoplasia.

	Footnotes

Top Footnotes Abstract Introduction Material and Methods Results Comment References

 
Address reprint requests to Dr Clapp, Section of Cardiology, Children's Hospital of Michigan, 3901 Beaubien Blvd, Detroit, MI 48201.

This article has been selected for the open discussion forum on the STS Web site: http://www.sts.org/annals

	References

Top Footnotes Abstract Introduction Material and Methods Results Comment References

 

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3. Patel RG, Freedom RM, Moes CAF, et al. Right ventricular volume determinations in 18 patients with pulmonary atresia and intact ventricular septum. Analysis of factors influencing right ventricular growth. Circulation 1980;61:428–40.[Abstract/Free Full Text]
4. Kopf GS, Laks H, Stansel HC, Hellenbrand WE, Kleinman CS, Talner NS. Thirty-year follow-up of superior vena cava–pulmonary artery (Glenn) shunts. J Thorac Cardiovasc Surg 1990;100:662–71.[Abstract]
5. Cloutier A, Ash JM, Smallhorn JF, et al. Abnormal distribution of pulmonary blood flow after the Glenn shunt or Fontan procedure: risk of development of arteriovenous fistulae. Circulation 1985;72:471–9.[Abstract/Free Full Text]
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8. King DH, Smith EO, Huhta JC, Gutgesell HP. Mitral and tricuspid valve annular diameter in normal children determined by two-dimensional echocardiography. Am J Cardiol 1985;55:787–93.[Medline]
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10. Billingsly AM, Laks H, Boyce SW, George B, Santulli T, Williams RG. Definitive repair in patients with pulmonary atresia and intact ventricular septum. J Thorac Cardiovasc Surg 1989;97:746–54.[Abstract]
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This Article Abstract Alert me when this article is cited Alert me if a correction is posted Citation Map 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): Henry L. Walters, III Kevin W. Lobdell Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Clapp, S. K. Articles by Hakimi, M. Search for Related Content PubMed PubMed Citation Articles by Clapp, S. K. Articles by Hakimi, M.