Ann Thorac Surg 2002;73:1274-1280
© 2002 The Society of Thoracic Surgeons
Original article: cardiovascular
Individualized total cavopulmonary connection technique for patients with Asplenia syndrome
Ryo Aeba, MDa*,
Toshiyuki Katogi, MDa,
Kenichi Hashizume, MDa,
Yoshimi Iino, MDa,
Shiaki Kawada, MDa,
Yuji Yuasa, MDa
a Division of Cardiovascular Surgery and Department of Radiology, Keio University, Tokyo, Japan
Accepted for publication November 28, 2001.
* Address reprint requests to Dr Aeba, The Division of Cardiovascular Surgery, Keio University, 35 Shinanomachi, Shinjuku, Tokyo 160-8582, Japan
e-mail: aeba{at}sc.itc.keio.ac.jp
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Abstract
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Background. Outcomes after univentricular repair for patients with asplenia syndrome remain unsatisfactory, not only because of clinical difficulties in patient selection, but also secondary to technical difficulties in the separation of the systemic and pulmonary circulations, particularly with the rerouting technique for the inferior systemic veins.
Methods. Between February 1995 and May 2000, a total of 14 consecutive patients with asplenia syndrome underwent bidirectional cavopulmonary connection with obliteration of additional pulmonary blood flow, followed by a total cavopulmonary connection. The rerouting technique for inferior systemic venous blood flow was individualized to optimize laminar nonturbulent flow characteristics in the pathway, and to minimize prosthetic load and suture load on the atrial wall. The lateral tunnel or tube conduit technique was used in an extraatrial, intra-extraatrial, or intraatrial fashion. No fenestration was applied.
Results. No hospital mortality was observed. Systemic venous flow was evaluated using magnetic resonance angiography, revealing no signs of obstruction, turbulence, or stasis either in or near the reconstructed pathways, irrespective of the rerouting technique. Postoperative catheterization revealed favorable hemodynamics including an inferior vena cava pressure of 13 ± 2 mm Hg and arterial oxygen saturation of 93.4% ± 3.5% at room air. All patients have remained free of symptoms, although 1 patient died of acute septic complications 3.5 years after the procedure.
Conclusions. The complexity of cardiac anomalies in asplenia syndrome warrants individualization of the total cavopulmonary connection technique used in reconstruction of the inferior systemic venous pathway. Optimizing flow characteristics in the pathway should be a priority. A staging approach allows suitable selection of candidates for univentricular repair.
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Introduction
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Patients with congenital cardiac anomalies as a part of asplenia syndrome, which are treated in clinical practice as synonyms for right atrial isomerism or bilateral right-sidedness of the heart, continue to demonstrate poor prognosis, even those patients who survive beyond the neonatal and early infantile period with or without palliative surgical interventions [1]. These severe, heterogeneous, complex anomalies are often unsuitable for biventricular repair. Hence, univentricular repair is the ultimate surgical procedure of choice.
Since Fontan and Baudet [2] reported the first successful univentricular repair (with a complete right heart bypass operation), a number of technical modifications have been proposed for patients with single ventricular physiology. With these changes, both early and late results have improved. The presence of asplenia syndrome, however, continues to be a risk factor in postoperative mortality and morbidity. This increased risk has been attributed to difficulties in proper patient selection, repair of associated lesions, and separation of the systemic from the pulmonary circulation, especially in the rerouting technique of inferior systemic veins including the inferior vena cava (IVC) and hepatic vein [1, 3–7]. A method for optimal patient selection and a technique for univentricular repair need to be determined.
The aim of this study was to retrospectively review the results for a cohort of patients who underwent univentricular repair under a single management strategy, with regard to the following: (1) precedence of bidirectional cavopulmonary shunt (BCPS) with obliteration of additional pulmonary blood flow; (2) repair of associated lesions at or before BCPS; and (3) application of total cavopulmonary connection (TCPC). An individualized technique for inferior systemic venous flow rerouting was used to optimize laminar and nonturbulent flow characteristics, which were evaluated postoperatively using catheterization and magnetic resonance angiography (MRA).
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Material and methods
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Patients
A total of 14 consecutive patients with asplenia syndrome and single ventricle physiology undergoing univentricular repair with TCPC between February 6, 1995 and May 22, 2000 at the Keio University Hospital were studied. Seven other patients with asplenia syndrome presenting during this time period were excluded from Fontan-track patient management, and were either surviving (n = 2) or dead (n = 5) after less definitive palliations. Diagnosis of asplenia syndrome was established on the basis of the following: (1) peripheral blood smears showing Howell-Jolly bodies; (2) chest radiographs with evidence of bilateral eparterial bronchi; (3) bronchoscopy with visualization of bilateral trilobed bronchial pattern; (4) juxtaposition of the aorta and IVC; (5) constellation of cardiac defects including unroofed coronary sinus, total anomalous pulmonary venous return to a systemic vein, common atrioventricular canal, double-outlet right ventricle or transposition of the great arteries with bilateral or subaortic conus, and pulmonary valve stenosis or atresia [8]; and (6) several spleen-imaging modalities (including ultrasound abdominal scans, computed tomography, magnetic resonance imaging, and radionuclide scans) showing the absence of spleen. Morphologic and preoperative demographic characteristics are listed in Table 1.
Preoperative cardiac function and oxygenation data are shown in Table 2.
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Table 2. Changes in Cardiac Performance and Arterial Oxygenation Before and 1 Year After Total Cavopulmonary Connection
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All patients had undergone previous BCPS as an interim step in the staged protocol for univentricular repair. Before BCPS, 9 patients (64%) had undergone creation of systemic-to-pulmonary arterial shunts; 3 of these patients had undergone two shunting operations. At BCPS, all additional sources of pulmonary blood flow, including the native pulmonary valve, any previously created systemic-to-pulmonary artery shunts, patent ductus arteriosus, and major aortopulmonary collateral arteries were obliterated. Ten patients displayed bilateral superior vena cava (SVC); in 9 patients, each SVC had been individually connected to the pulmonary artery (bilateral BCPS), but in 1 patient with a bridging vein (the smaller SVCs were suture ligated). An aggressive strategy was adopted to concomitantly repair associated lesions at BCPS. These procedures at BCPS included atrioventricular valvuloplasty (n = 4), pulmonary arterioplasty (n = 3), total anomalous pulmonary venous connection repair (n = 1), and atrial septectomy for pulmonary venous reconstruction (n = 1). In 1 patient, the initial TCPC attempt failed because of undiagnosed interatrial baffle leakage and a take-down was required. Subsequent TCPC was performed 4.5 years after the initial TCPC.
Operative procedures
All TCPC procedures were performed through a midline sternotomy (n = 13) or a left lateral thoracotomy (n = 1), using cardiopulmonary bypass (average time 150 ± 31 minutes) and aortic cross-clamping with cardioplegic cardiac arrest (average time: 64 ± 20 minutes). The bypass technique included aortic, bicaval (or tricaval if necessary) and hepatic venous (if separately entered) cannulation, and moderate hypothermia (lowest rectal temperature 28°C). Conventional ultrafiltration was used during the entire period of cardiopulmonary bypass, and modified ultrafiltration was used in the 7 patients who underwent surgery after 1996.
The inferior systemic veins (IVC and hepatic vein if each separately entered the atrium) were rerouted to the pulmonary artery by interposition of either a cylindrical (n = 7, conduit repair) or hemi-cylindrical (n = 7, lateral tunnel repair) prosthesis of expanded polytetrafluoroethylene (Cardiovascular patch, W. L. Gore and Associates Co, Flagstaff, AZ). The reconstruction technique was selected according to the following prioritization policy. The first priority was to minimize any abrupt concavities or protuberances created by angulation or gap at the junction between the reconstruction pathway to the pulmonary artery and inferior systemic veins. The second priority was to minimize both prosthesis load and suture load on the atrial wall.
In 8 patients, the pulmonary vein was either abnormally connected to the left atrium with a single orifice or was surgically rerouted to the atrium. The drainage location varied significantly among cases. In 3 patients, the dominant ventricular inflow (including the atrioventricular valve) was located on the opposite side of the orifice(s) of the pulmonary vein, from the perspective of the IVC orifice. An intraatrial conduit was used in these patients so that the systemic and pulmonary venous flows criss-crossed in the atrium (Fig 1).
In 4 patients, the hepatic vein directly entered the atrium. Three patients underwent an "intra-extraatrial" rerouting, in which the orifices of the IVC and the hepatic vein were incorporated with placement of a small expanded polytetrafluoroethylene baffle through a small atriotomy or an atrial wall flap before conduit or lateral tunnel repair (Figs 2 and 3).
The remaining patient underwent intraatrial conduit repair because of unfavorable position of the pulmonary vein orifice, as described earlier. In 6 patients, the inferior systemic venous blood was rerouted using a conventional technique with either intraatrial lateral tunnel [9] or extraatrial conduit [10]. In total, reconstruction technique was intraatrial, lateral tunnel (n = 6), intra-extraatrial, lateral tunnel (n = 1), intraatrial, conduit (n = 3), extraatrial, conduit (n = 2), and intra-extraatrial, conduit (n = 2). Single coronary sinus was not identified by operative inspection in any patients.
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Fig 1. Total cavopulmonary connection technique using intraatrial conduit for patient with dextrocardia, single orifice of pulmonary veins, and bilateral superior vena cavae. Anterior hemisphere of heart is omitted for convenience. Note that ventricular inflow is located on the opposite side of the single orifice of the pulmonary vein, from the perspective of the inferior vena cava.
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Fig 2. Operative schema of total cavopulmonary connection using an intra-extraatrial conduit for a patient with dextrocardia, single left superior vena cava, and separate entry of the hepatic vein. A single baffle was placed for partition of the atrium. Venous cannulas were placed in the abnormally entered hepatic vein along with the superior and inferior vena cavae. Arrows indicate locations of suture anastomosis.
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Fig 3. Operative schema of total cavopulmonary connection using an intra-extraatrial lateral tunnel technique for a patient with dextrocardia, bilateral superior vena cavae, and separate entry of the hepatic vein. A U-shaped atrial incision was made to create an atrial wall flap, which was sutured to the posterior wall of the atrium. The left branch pulmonary artery was horizontally incised and the inferior rim of the incision was sutured to the cranial epicardial surface of the atrium. A single baffle for lateral tunnel was positioned on the epicardium of the atrium to drain the inferior systemic vein to the pulmonary artery partially outside of the atrium. Arrows indicate locations of suture anastomosis.
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Temporary atrial and ventricular pacing were performed as necessary. Despite the maximum effort that went into repairing associated lesions at the previous BCPS, residual atrioventricular valve regurgitation of moderate or massive degree was noted in 2 patients. A mechanical valve was placed at the atrioventricular position for 1 patient whereas the other patient was treated with annuloplasty. A third patient underwent repair for correction of the mild valve regurgitation by partial closure of the valve orifice at the time of atriotomy for lateral tunnel TCPC. Cryoablation of the atrioventricular node with permanent pacemaker implantation was used in two patients with histories of junctional ectopic tachyarrhythmia.
Data acquisition and statistical analysis
Medical and operation records were reviewed for all patients. Follow-up information regarding current activity level, medications, and perceived complications was obtained from the consulting physicians or from the patients’ parents. Postoperative evaluation of the cardiac status included echocardiography, cardiac catheterization, Holter electrocardiographic monitoring, and MRA with gradient-echo and phase-contrast techniques [11].
Statistical analysis was performed using the SPSS program for Windows (SPSS Inc, Chicago, IL). Quantitative variables with and without normal distribution were expressed as the mean ± the standard deviation of the mean, and as the median and range, respectively. Comparisons between conditions before TCPC and after TCPC for quantitative variables with and without normal distribution were performed using the paired Student’s t test and the Wilcoxon rank test, respectively. The level of statistical significance was set at a p value less than 0.05.
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Results
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No hospital mortality after TCPC was observed. The peak in the mean pulmonary arterial pressure during the early postoperative period averaged 18.1 ± 2.0 mm Hg. Pleural fluid drainage was required for a median of 12 days (range 2 to 60 days) after TCPC. Chylous leak collection was noted in 2 patients. Ascites collection with oliguria was treated with peritoneal dialysis in 3 patients. None of the patients exhibited hepatic injury during the early postoperative period, as measured by serum levels of aspartate aminotransferase and alanine aminotransferase of 200 U/L or greater.
Early postoperative arrhythmias were documented in 12 patients (86%). Atrioventricular reciprocating or junctional ectopic tachycardia (6 patients), and paroxysmal atrial tachycardia (4 patients) developed on 13 occasions. All were either self-limited or treated successfully with pharmacologic intervention within 30 minutes after development. Most arrhythmias were associated with temporary hypotension. Sinus node dysfunction with escaped junctional activity developed in 5 patients; all were self-limited or treated using temporary pacing. These rhythm disturbances contributed greatly to postoperative ventilator support (median 4 days, range 20 hours to 21 days). At the time of hospital discharge, normal sinus node activity was observed in all patients with the exception of 2 with nontachycardial junctional rhythm.
Follow-up was completed in all patients, with a period of 1.4 to 6.7 years (average 4.4 ± 1.7 years). During the follow-up period, all patients have remained asymptomatic and in New York Heart Association class I. One patient, who had been asymptomatic for 3.5 years after TCPC, rapidly became moribund and died of fulminant septic complications; the source of sepsis in this patient was not identified. In another patient, asymptomatic, systemic-to-pulmonary venous collateral vessels developed and were obliterated in a reoperation 2.6 years after TCPC. No thromboembolic complications, episodes of pleural fluid or ascites collection, or documented protein-losing enteropathies were reported. Outpatient medications included antiplatelet-aggregation drugs (n = 13), diuretics (n = 6), digoxin (n = 5), and warfarin (n = 1, for the patient with the mechanical valve insertion).
The 1-year follow-up results of echocardiography and cardiac catheterization are listed in Table 2. Improvement of cyanosis was represented by a decrease in the hemoglobin concentration in blood and an increase in the arterial oxygen saturation. Ventricular wall motion remained good or fair in all patients. Atrioventricular valve regurgitation was graded as mild, trivial, or none, as preexisting moderate or massive regurgitation was treated successfully at TCPC. The cardiothoracic ratio on chest roentgenogram revealed similar values before and after TCPC. At cardiac catheterization the mean pulmonary arterial pressure was found to be marginally increased after TCPC (p = 0.07), although no measurements exceeded 16 mm Hg. The systemic vein pressure was 13 ± 2 mm Hg in the IVC, 13 ± 2 mm Hg in the SVC, and 12 ± 2 mm Hg in the pulmonary artery. The pressure difference between the IVC and pulmonary artery was 1 ± 1 mm Hg (range 1 to 3 mm Hg). The ventricular end-diastolic pressure and pulmonary arterial cross-sectional index did not change after TCPC. There were no patients in whom any episodes of tachycardia were documented, although late postoperative electrocardiographic monitoring was not comprehensive in this series.
Magnetic resonance angiography with gradient-echo and phase-contrast techniques revealed that the inferior systemic venous flow in and around the reconstructed pathway was free from obstruction, turbulence, or stasis, irrespective of rerouting technique.
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Comment
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Of a series of modifications to Fontan operation, introduction of TCPC was an important breakthrough [9]. TCPC is currently the procedure of choice in many institutes. Recently, increased interest has been shown regarding the optimal rerouting technique for the inferior systemic venous blood [6, 10, 12]. An extraatrial conduit is advantageous in: (1) avoiding aortic cross-clamp and even cardiopulmonary bypass; (2) preventing baffle leakage; (3) creating perfectly laminar flow in the conduit; (4) minimizing suture-load on the atrial wall resulting in a decreased incidence in the development of late atrial tachyarrhythmia; and (5) allowing the entire atrium to have lower pressure thereby maintaining normal sinus node function. An intraatrial conduit allows: (1) the minimal length of prosthesis; and (2) the minimal orientation angle between the IVC and the conduit; in addition to (3) creating perfectly laminar flow. A lateral tunnel is advantageous in: (1) preserving the growth potential of the reconstructed systemic venous pathway (especially important for young patients); (2) more generosity in length and orientation of the prosthetic patch; (3) easier creation of fenestrations; (4) more facility in dealing with separate entry of the hepatic vein.
Asplenia syndrome represents a surgical challenge for univentricular repair, including the TCPC procedure, for a number of reasons. First, abnormalities of the systemic and pulmonary venous connection are commonly encountered in this entity [8], which complicate the TCPC in terms of separating the systemic from the pulmonary circulation [13]. The IVC orifice is often abnormally large, partly because of elevated pressure due to ventricular volume over-load and atrioventricular valve regurgitation. The hepatic vein often drains directly into the atrium rather than after joining the IVC; the orifices of the IVC and hepatic veins are apart from each other. This is a difficult situation for application of an extraatrial conduit repair, because several advantages of this repair may disappear; in such circumstances, placement of an intra-extraatrial conduit is indicated. We aggressively placed the venous cannula in this abnormal hepatic vein rather than temporarily cross-clamping to avoid liver congestion for even a short period, because liver dysfunction is a common complication both early and late after univentricular repair [14, 15]. Deep hypothermic circulatory arrest is an alternative adjunctive, but it carries a potential risk of brain injury. Coronary venous drainage is almost always abnormal. The coronary sinus is often absent, and only multiple small orifices for the coronary veins are identified. After lateral tunnel TCPC in such cases, it is possible that, unintentionally, the coronary vein drains into a high-pressure side chamber (as after the classic Fontan procedure), leading to suboptimal coronary perfusion. Furthermore, this coronary venous network is a potential source of leak (shunt) from the high-pressure systemic venous chamber to the low-pressure pulmonary venous chamber. Extraatrial conduit reconstruction is better than lateral tunnel repair in terms of avoiding these complications. Pulmonary venous rerouting can be another problem. The native or previously reconstructed orifice of the pulmonary veins is often located on the opposite side of the dominant ventricular inflow from the perspective of the IVC orifice. This makes a lateral tunnel or an extraatrial conduit repair awkward to perform. This anatomic configuration is the primary indication for creation of an intraatrial conduit.
Second, other associated cardiac lesions with asplenia syndrome almost always exist and may impair, if not erase, a patient’s candidacy for univentricular repair. These comorbidities include: (1) atrioventricular valve regurgitation; (2) pulmonary vein obstruction; (3) branch pulmonary arterial obstruction due to native pulmonary arterial coarctation or late distortion after previously placed systemic-to-pulmonary arterial shunt; and (4) subaortic obstruction at the conus or ventricular septal defect (bulbo-ventricular foramen) level. Comorbidities are frequently difficult to evaluate accurately, as their magnitude may be overestimated or masked by the volume-load and pulmonary flow status. The residue of these lesions at the time of univentricular repair can distort assessment of pulmonary vascular resistance and myocardial performance—two of the most important factors for validation and maintenance of Fontan physiology–and can impede adequate patient selection for this procedure. Our current strategy for management of a Fontan-track patient with asplenia syndrome, therefore, includes early ventricular volume-load eradication by means of BCPS with obliteration of additional pulmonary blood flow, and repair of associated lesions before or at BCPS rather than at TCPC. Although small, our experience may indicate that this strategy allows accurate assessment of pulmonary vascular resistance and myocardial performance, and that candidates selected by this assessment (even those with asplenia syndrome) can undergo univentricular repair with low risk of early and midterm failure.
Third, it has been reported that the predominant or single ventricle supporting systemic and pulmonary flows is the morphologic right ventricle in the majority of asplenic hearts [8]; this was also the case in the present series. The adverse effects of right ventricular morphology on operative outcome are debatable [16, 17]. In our series, however, myocardial performance was unimpaired under a volume-overload eradicated status, as discussed earlier, which may infer that right ventricular morphology per se is not an independent risk factor for mortality and morbidity after univentricular repair.
Other early postoperative morbidities include tachyarrhythmias from ectopic atrial, paroxysmal atrial, or atrioventricular reciprocating activity. In asplenic hearts, there are double conduction systems including the sinus node, atrioventricular node, and bundle of His [18]. The atrioventricular conduction system forms a "sling." The prevalence of tachyarrhythmia early after TCPC may be attributed to a combination of these unique anatomical anomalies [19] in addition to provocation by suboptimal coronary perfusion during this period. Diastolic dysfunction has been reported after univentricular repair [20, 21]. Tachyarrhythmia, which shortens the diastolic phase, may decrease cardiac output to a critical level. In our series, this complication developed early after TCPC in 10 of 14 patients (71%), including those repaired using an extraatrial conduit. A limited suture-load and stretch on the atrial wall seems incapable of effectively coping with this problem. Therapeutic or even prophylactic procedures for tachycardia control (eg, Cox-Maze operation, radio- or cryoablation of the accessory sinus node or atrioventricular node at the time of TCPC [22]) may therefore be warranted.
No patients in our series underwent fenestration, despite the fact that several authors [6, 23] have reported the effectiveness of the technique, particularly in high-risk patients undergoing univentricular repair, including asplenic patients. Hypoxia secondary to residual right-to-left shunts is a potential complication following univentricular repair. Although shunts through the baffle suture line are avoidable by application of an extracardiac conduit TCPC, an abnormal connection between the systemic and pulmonary veins remains another potential shunt. In our series, arterial oxygenation after TCPC without fenestration was unpredictably low in several cases, which is not explained by desaturation with coronary venous flow. Fenestration may increase unpredictability of the arterial oxygenation level and complicate perioperative patient management.
Although the rerouting technique for the inferior systemic veins used in our series was heterogeneous, MRA identified flow in and near the reconstructed pathway as nonturbulent, nonobstructive, and nonstatic. Phase-contrast MRA is sensitive to both the presence and velocity of flow in three different directions during a specific cardiac phase without bias (as by using contrast material in roentgenogram angiography), and is inherently quantitative even during slow flow [11]. This modality, therefore, is both reliable and suitable for flow evaluation of the systemic venous route after TCPC [24]. The MRA results indicated equally efficient flow dynamics in our systemic venous route reconstructed using heterogeneous techniques. It is possible that this was achieved by our policy regarding selection of reconstruction technique: in our series, maximization of smooth flow (ie, minimization of energy loss due to obstruction, turbulence, or stasis) was the first priority. Energy loss during reconstructed pathway aggravates lower systemic venous hypertension, which is already inherent to Fontan physiology, and can therefore act as an attack to the "Achilles’ heel." The TCPC technique was individualized according to positions of several anatomic landmarks including the orifices of veins (IVC, hepatic vein, and pulmonary vein), the anastomotic site of the pulmonary artery, and the inflow of the dominant ventricle. The amount of atrial wall surface area may be another factor in selection. We infer that uniform application of single reconstruction technique (eg, extraatrial conduit repair) is not only unrealistic but also unnecessary.
In conclusion, myocardial performance and pulmonary vascular resistance in asplenic patients preparatory to univentricular repair should be assessed under normal volume-load status by using a prior BCPS, obliteration of additional pulmonary blood flow, and repair of associated lesions. Individualization of the technique for inferior systemic venous rerouting in TCPC should be performed with respect to the dictates of the anatomic landmarks. Either the lateral tunnel or the conduit technique can be applied in an extraatrial, intra-extraatrial, or intraatrial fashion.
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Invited commentary
- Christopher A. Caldarone
Ann. Thorac. Surg. 2002 73: 1280-1281.
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Abstract
Introduction
