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Ann Thorac Surg 1998;66:678-680
© 1998 The Society of Thoracic Surgeons
ventricle repairs
a Division of Cardiac Surgery, Hospital for Sick Children, University of Toronto, Toronto, Canada
b Division of Cardiology, The Hospital for Sick Children, University of Toronto, Toronto, Canada
Address reprint requests to Dr Van Arsdell, Division of Cardiovascular Surgery, The Hospital for Sick Children, Rm 1525, 555 University Ave, Toronto, Ont, Canada M5G 1X8
Presented at the Workshop on "One and One-Half Ventricle Repairs," Gubbio, Italy, Dec 6–7, 1996.
Abstract
Background. Perioperative and long-term problems associated with the Fontan circulation are substantial. There has been an exploration of extending the limits of a biventricular ventricular repair by using a superior vena cava-to-pulmonary artery anastomosis. This type of repair is known as a 1
ventricle repair.
Methods. Patients having defects of the pulmonary ventricle in size or function have undergone 1 ventricle repairs with or without creation of an atrial septal defect. Repairs with tricuspid z values as small as -10 and predicted pulmonary ventricular volumes as low as 30% have been reported. The 1 ventricle repair technique has also been used in special situations associated with an arterial switch or double switch procedure.
Results. Mortality has ranged from 0% to 12%. Complications have included persistent elevation of superior vena cava pressure, intermittent periorbital edema, and 1 superior vena caval aneurysm requiring takedown. There appears to be an increased risk of perioperative pleural effusions and chylothorax. Protein-losing enteropathy and chronic atrial arrhythmias have not been present.
Conclusions. Successful 1 ventricle repairs have been reported for morphologically small or poorly functioning pulmonary ventricles and special situations. Intermediate-term follow-up is favorable when compared with reported outcomes for the Fontan circulation.
Patients with congenital cardiac defects are usually candidates for either a biventricular or univentricular repair. A subset of patients have ventricular anatomy or function that lies in between these categories [1–3]. Attempts at biventricular repair in these patients can lead to low cardiac output syndrome based on an inability of the right side to handle the entire systemic venous return. A partial diversion of systemic return directly to the pulmonary artery lessens the volume load on the pulmonary ventricle. This is achieved by performing a bidirectional cavopulmonary anastomosis in conjunction with a standard repair. Marginal ventricles may then become a useful pump in series. This type of repair is known as a 1 ventricle repair.
Rationale for a 1 ventricle repair
The ability of the pulmonary ventricle to generate appropriate indexed output is dependent on morphology, function, and pulmonary vascular resistance. In the perioperative period a 1 ventricle repair is useful only if morbidity and mortality are less than or equal to those of the corresponding biventricular or Fontan repair. Reported mortality has varied from 0% to 12% [1, 4–9], which compares favorably with the mortality of the Fontan procedure. Mortality for marginal situations and biventricular repair is not well delineated. In certain circumstances it is high [2].
Long-term outcomes will have to be compared with long-term Fontan outcomes [10]. Chronic atrial arrhythmias, cyanosis, or protein-losing enteropathies have not been identified in the short-term and intermediate-term follow-up of 1 ventricle repairs [1, 8].
New York Heart Association classification in these patients has been class I or II; however, no cohort data are available on measured exercise capacity and maximal oxygen consumption.
Limiting criteria for a 1 ventricle repair
The limits of size and function for biventricular repair are not known on a prospective basis. Experimental, anecdotal, and cohort reports provide useful guidelines for selection of patients.
Canine animal modeling by Ilbawi and associates [11] has demonstrated a continued favorable hemodynamic response in the pulmonary vasculature when the right ventricle is downsized in the acute setting to as small as 30% of normal. This same group has successfully performed 1 ventricle repairs in the clinical setting with predicted ventricular volumes as small as 30% [4].
De Leval and associates [2] have shown a high mortality with pulmonary atresia and intact ventricular septum when biventricular repair has been attempted with tricuspid z values [12] of less than -3. Conversely, a good outcome was achieved with z values greater than -3 and a tripartite right ventricle.
In a series of 9 patients with unbalanced atrioventricular septal defects, Alvarado and associates [5] successfully used the 1 ventricle approach for right atrioventricular valve z values as small as -10. Billingsley and colleagues [13] also have used a 1 ventricle repair in patients with pulmonary atresia and intact ventricular septum. Others have also reported successful series of cavopulmonary anastomosis associated with biventricular repair [6–9, 14].
Thirty-eight patients were treated with a 1 ventricle repair over a 14-year period at the Hospital for Sick Children, Toronto [1]. Patients having tricuspid z values as small as -6 and right ventricular predicted volumes as small as 47% were successfully treated. Patients with chronic dilation and moderately depressed right ventricular function also had favorable outcomes. A small number of patients had conversion to a 1 ventricle repair when there was right ventricular failure in the intensive care unit after a standard biventricular repair. This approach was not successful.
Application of the 1 ventricle repair
Clinical experience and analysis of data in the Toronto experience of 1 ventricle repairs has led to selection criteria based on three categories: morphologically small pulmonary ventricles, functional deficits to the ventricle, and special circumstances.
Anatomic selection criteria for small pulmonary ventricles
The anatomic selection criteria are outlined in Table 1.
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ventricle repairs ventricle repairs when there is dilation to 120% or more and at least moderate reduction in ventricular function. Other situations where this approach is used is with borderline ventricles in patients demonstrating hemodynamic decompensation with catheter balloon occlusion of an atrial septal defect, or when there is a borderline-sized right ventricle and an elevated central venous pressure immediately after bypass following biventricular repair. In this situation one must make sure the repair is without right ventricular outflow tract obstruction.
Special circumstances
Patients with Ebstein’s anomaly and partial right ventricular outflow tract obstruction caused by the billowing Ebstein valve may benefit from a 1 ventricle repair as an adjunct to repair of Ebstein’s anomaly [1].
Rare conditions such as D-transposition of the great vessels, ventricular septal defect, and a small right ventricle may be treated initially with balloon atrial septostomy and a pulmonary artery band. An arterial switch, ventricular septal defect closure, and cavopulmonary anastomosis can then be performed when adequate maturation of the pulmonary vasculature has occurred. Transposition with a small left ventricle may be treated by early atrial septostomy followed by a delayed atrial switch procedure and a cavopulmonary anastomosis [1].
Reddy and colleagues [9] have performed 1 ventricle repairs for children with straddling tricuspid valves and avoided a Fontan procedure.
Many patients with corrected transposition of the great arteries have borderline-sized morphologic right ventricles. The 1 ventricle repair has also been a useful adjunct to the "double switch" procedure [15], in which the pulmonary ventricle is made anatomically and functionally smaller by the intracardiac baffle and ventriculotomy. The superior limb of the atrial switch is eliminated. This precludes a potential obstruction site, the atrial baffle is simplified, the quantity of atrial suture lines is diminished, and the aortic cross-clamp time is shortened [1].
In summary, the diagnoses for which 1 ventricle repairs may be appropriate are as follows:
Complications
Most of these children require staging procedures before and after the cavopulmonary anastomosis such as an atrial septostomy, pulmonary artery banding, systemic-to-pulmonary artery shunt, atrial septal defect closure, and pulmonary arterioplasty [1, 4, 8]. Complications thus include those of cardiac reoperation.
The incidence of low cardiac output syndrome after the procedure is low; however, should it develop an atrial fenestration may be required. Alternatively, an adjustable atrial septal defect can be created at the time of repair [13].
There is an increased incidence of chylothorax [16] and pleural effusions [17] when there is an alternate source of pulmonary blood flow to the pulmonary arteries in addition to the bidirectional cavopulmonary shunt. There is also a case report of a left superior vena caval aneurysm after correction of an atrioventricular septal defect in a patient who had the left superior vena cava connected to the pulmonary artery as a cavopulmonary shunt [18]. Clapp and colleagues [8] have reported early-morning periorbital edema in 2 patients. In 1 child having a 1 ventricle repair performed with a classic Glenn procedure, pulmonary arteriovenous fistulas developed [7].
In the Toronto experience of 38 patients, the mean postoperative cavopulmonary shunt pressure was 14.9 mm Hg (standard deviation, 4.9 mm Hg). Six patients had early cavopulmonary shunt pressures of greater than 20 mm Hg. The cavopulmonary anastomosis was taken down and an atrial septal defect was created in 1 child who had a persistent elevation of the central venous pressure to 26 mm Hg [1]. An alternative might be to separate the right and left pulmonary arteries as in a classic Glenn procedure; however, the risks of pulmonary arteriovenous malformations would seem to make this inadvisable. We believe it is preferable to create an atrial septal defect, by using catheter techniques, as a first line of therapy for persistent elevation of superior vena caval pressures. Successful application of this therapy allows later conversion to a 1 ventricle repair with preoperative testing of hemodynamic tolerance by catheter balloon occlusion of the atrial septal defect. Some patients may require conversion to the Fontan algorithm.
Conclusions
Patients with borderline pulmonary ventricles based on either ventricular size or chronic poor function are candidates for a 1 ventricle repair. Pulmonary vascular resistance must be low and the pulmonary arteries of appropriate size. The 1 ventricle approach has successfully been employed for a wide variety of diagnoses with tricuspid z values as small as -10 and predicted pulmonary ventricular volumes as low as 30%. Staging by delayed atrial septal defect closure may be required. The 1 ventricle repair may also be successfully employed in patients having dilated right ventricles with moderate to severe reduction in ventricular function. This approach is useful to simplify the double switch procedure or to eliminate the need for an intracardiac baffle in certain complex situations with a left superior vena cava.
References
This article has been cited by other articles:
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  S. Kim, O. Al-Radi, M. K. Friedberg, C. A. Caldarone, J. G. Coles, E. Oechslin, W. G. Williams, and G. S. Van Arsdell Superior vena cava to pulmonary artery anastomosis as an adjunct to biventricular repair: 38-year follow-up. Ann. Thorac. Surg., May 1, 2009; 87(5): 1475 - 1482. [Abstract] [Full Text] [PDF]
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 J. Heggie, N. Poirer, W. G. Williams, and J. Karski Anesthetic Considerations for Adult Cardiac Surgery Patients with Congenital Heart Disease Seminars in Cardiothoracic and Vascular Anesthesia, June 1, 2003; 7(2): 141 - 152. [Abstract] [PDF]
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 G. Stellin, V.L. Vida, O. Milanesi, M. Rubino, M.A. Padalino, S. Secchieri, G. Pittarello, and D. Casarotto Surgical treatment of complex cardiac anomalies: the 'one and one half ventricle repair' Eur. J. Cardiothorac. Surg., December 1, 2002; 22(6): 1043 - 1049. [Abstract] [Full Text] [PDF]
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G. Stellin, V.L. Vida, O. Milanesi, M. Rubino, M.A. Padalino, S. Secchieri, G. Pittarello, and D. Casarotto Surgical treatment of complex cardiac anomalies: the 'one and one half ventricle repair' Eur. J. Cardiothorac. Surg., September 1, 2002; 22(3): 431 - 437. [Abstract] [Full Text] [PDF]
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R. Aeba, T. Katogi, S. Takeuchi, and S. Kawada Surgical management of the straddling mitral valve in the biventricular heart Ann. Thorac. Surg., January 1, 2000; 69(1): 130 - 134. [Abstract] [Full Text] [PDF]
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C. Mavroudis, C. L. Backer, L. M. Kohr, B. J. Deal, J. Stinios, A. J. Muster, and D. F. Wax Bidirectional Glenn shunt in association with congenital heart repairs: the 1 1/2 ventricular repair Ann. Thorac. Surg., September 1, 1999; 68(3): 976 - 981. [Abstract] [Full Text] [PDF]
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