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Ann Thorac Surg 2002;74:1967-1978
© 2002 The Society of Thoracic Surgeons
a Department of Cardiac Surgery, Boston, Massachusetts, USA
b Biostatistics, Children’s Hospital Boston, Harvard Medical School, Boston, Massachusetts, USA
* Address reprint requests to Dr del Nido, Department of Cardiac Surgery, Children’s Hospital Boston, Harvard Medical School, 300 Longwood Ave, Boston, MA02115, USA.
e-mail: pedro.delnido{at}tch.harvard.edu
Presented at the Thirty-eighth Annual Meeting of The Society of Thoracic Surgeons, Fort Lauderdale, FL, Jan 28–30, 2002.
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Abstract |
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METHODS: A retrospective review of 135 patients with heterotaxy syndrome who underwent a Fontan operation between 1981 and 2000 was performed.
RESULTS: There were 93 patients with right isomerism and 42 with left isomerism. Anomalies of venous return included 25 patients with extracardiac pulmonary venous connection (19%) and 37 patients with an interrupted inferior vena cava (27%). Thirty-six patients (27%) had at least moderate atrioventricular valve regurgitation. The type of Fontan procedure included 17 patients with an atriopulmonary Fontan connection, 67 with a lateral tunnel modification, 19 with an intraatrial tube graft, 25 with an extracardiac tubegraft, and 7 with an intra-extra atrial tube graft. A fenestration was placed in 93 patients (78%). Early mortality was 19% before 1991, 3% since 1991, and no patient has died early since 1993. Ten-year survivals were 70% for Fontan operations before 1990 and 93% for Fontan operations after 1990. Thirty-two patients (23%) had prolonged pleural effusions. Risk factors for death included anomalous pulmonary venous connection (p = 0.02) and higher preoperative pulmonary vascular resistance (p = 0.002). Sixty-two patients (47%) had some form of early postoperative arrhythmia. At 10 years, freedom from late bradyarrhythmia and late tachyarrhythmia were 78% and 70%, respectively. Preoperative arrhythmias, older age at operation, and anatomic features were each independent predictors of late arrhythmia.
CONCLUSIONS: The Fontan operation can now be performed in patients with heterotaxy syndrome with excellent survival. However, morbidity in terms of postoperative arrhythmias and prolonged pleural effusions remains significant. Fontan staging, appropriate choice of Fontan modification, aggressive treatment of concomitant malformations, and use of a baffle fenestration contribute to improved outcome.
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Introduction |
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TopAbstractIntroductionMaterial and methodsResultsCommentDiscussionReferences |
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A variety of technical modifications have been developed to facilitate Fontan completion in patients with heterotaxy syndrome. Among them are the use of extracardiac conduits, intra-extracardiac conduits, intraatrial tube grafts, and intraatrial tunnels. However, few studies have investigated the impact of the various modifications on intermediate- and long-term results. When we analyzed the long-term results of the first 220 lateral tunnel Fontan operations at our institution we noted that the presence of heterotaxy syndrome was no longer an independent risk factor for early or late outcome, as it had been in our earlier experience [2, 8]. This finding prompted us to review our entire experience with the Fontan operation in patients with heterotaxy syndrome in an attempt to elucidate the impact of various surgical modifications on mortality, morbidity, and the incidence of supraventricular arrhythmia.
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Material and methods |
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TopAbstractIntroductionMaterial and methodsResultsCommentDiscussionReferences |
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Definitions
Visceroatrial heterotaxy syndrome was defined as the presence of a pattern of typical cardiac, vascular, and visceral abnormalities that indicated abnormal sidedness. Heterotaxy syndrome is largely synonymous with the syndromes known as left or right atrial isomerism or asplenia or polysplenia syndrome. However, since the features of each syndrome are not always consistent and often overlap, we have used the encompassing term heterotaxy syndrome in this study. Supraventricular tachyarrhythmia was defined as the presence of documented atrial fibrillation, atrial flutter, or atrioventricular reciprocating tachycardia, but not sinus tachycardia. Bradyarrhythmia included sinus bradycardia, abrupt and pronounced sinus pause or arrest, sick sinus syndrome, slow junctional escape rhythm, junctional rhythm at physiologic rates without evidence of sinus node activity, and second or third degree atrioventricular block. Sinus bradycardia was defined as a resting heart rate of more than two standard deviations below the age-adjusted mean. All arrhythmias that were reported in the charts were noted, without attempt to determine clinical impact. Late postoperative arrhythmia was defined as the occurrence of recurrent or permanent arrhythmia after the Fontan operation. This definition includes intraoperative and early postoperative arrhythmia that lasted into or recurred within the follow-up period, but excludes brief periods of arrhythmia intraoperative or early postoperative arrhythmia that resolved spontaneously and did not reoccur. The early postoperative period was defined as the time before discharge from the hospital or the first 30 postoperative days for patients who were hospitalized for more than 30 postoperative days.
Statistical analysis
Data are presented as mean ± standard deviation or median and range as appropriate. Estimated survival and freedom from supraventricular tachyarrhythmia and bradyarrhythmia were determined by the Kaplan-Meier method based on the product-limit estimator, and 95% confidence intervals (CI) were constructed around the curves according to Greenwood’s formula. Variables tested for influence on each outcome are listed in the Appendix. The three outcome factors were mortality, presence of postoperative tachyarrhythmia, or presence of postoperative bradyarrhythmia. Variables were evaluated using the log-rank test in the univariable analysis and by the likelihood ratio test in the Cox proportional-hazards regression model for multivariable analysis. Hazard ratios with 95% confidence intervals were constructed for the significant multivariable predictors. Final selection of models were determined by the forward stepwise procedure with variables having p less than 0.1 in the univariable analyses entered as candidates into the Cox regression model with a p value less than 0.05 required for a variable to be retained in the multivariable equation. Simple binomial proportions were compared by Fischer’s exact test. All reported P values are two-tailed. Statistical analysis was performed using the SPSS software package (Version 11.0; SPSS Inc, Chicago, IL).
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Results |
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Anatomy
Ninety-three patients (69%) had asplenia syndrome or right atrial isomerism, and 42 patients (31%) had polysplenia syndrome or left atrial isomerism. Dextrocardia was present in 87 patients (63%), and 121 patients (90%) had essentially a common atrium without a distinct atrial septum. A typical double outlet right ventricle with muscular outlet conus under each great vessel was present in 83 patients (61%) and was associated with pulmonary stenosis or atresia in most of the patients. In 75 patients (55%) there was a functionally single right ventricle supporting the systemic circulation. In 87 patients (64%) there was a common atrioventricular valve, and in most of the cases there was an unbalanced opening into the morphologic right ventricle. Moderate atrioventricular valve regurgitation was demonstrated by echocardiography or catheterization, or both, in 36 patients (27%). Anomalies of systemic or pulmonary venous return, or both, were present in all patients. Sixty-four patients (47%) had bilateral superior venae cava, usually without a connecting innominate vein, and 37 patients (27%) had an interrupted inferior vena cava with azygos continuation. Among the patients with a noninterrupted inferior vena cava, the hepatic veins entered the atrium separate from the entrance of the inferior vena cava (IVC) in 26 patients (26%) and drained into the IVC in 72 patients (74%). Abnormalities of the pulmonary venous connection were present in a total of 61 patients (45%). All pulmonary veins drained to an abnormal location in 42 patients (31%) (total anomalous pulmonary venous return), usually through a common retrocardiac connecting vessel. Partial anomalous pulmonary venous return was found in 19 patients (14%). Extracardiac pulmonary venous drainage was present in 25 patients (19%) and all of these patients were of the supracardiac type. Pulmonary venous return was diagnosed to be obstructed in 11 patients (8%). Distortion, hypoplasia, or discrete stenosis of one or both pulmonary arteries, either congenital or acquired, associated with a systemic-to-pulmonary artery after shunt, were diagnosed in a total of 43 patients. Seven patients had discontinuous branch pulmonary arteries, 3 of these had left atrial isomerism, and 4 had right atrial isomerism. Nineteen patients had major systemic-to-pulmonary artery collaterals; and 13 patients with a longstanding superior cavopulmonary anastomosis without additional systemic-to-pulmonary shunts had significant pulmonary arteriovenous malformations.
Prior procedures
All but 11 patients had at least one operation before establishment of the Fontan circulation. Initial palliation operation included 90 patients with a previous systemic-to-pulmonary artery shunt, 16 with pulmonary artery banding, 7 with an atrial septectomy, 4 with repair of aortic coarctation, and 2 with a Damus-Kaye-Stansel or Norwood I-type anastomosis of the main pulmonary artery to the aorta. The completion of the Fontan circulation was preceded by a cavopulmonary shunt in 89 patients (66%), and 86 of 100 patients (86%) operated on after 1990 had a previous cavopulmonary shunt. Five patients had a classic, unilateral Glenn shunt, and 84 patients had a bidirectional Glenn shunt. In 9 of these patients with bidirectional Glenn shunts, a Hemifontan operation was performed with closure of the atrial superior vena cava (SVC) junction with a patch. In 24 patients with interrupted inferior vena cava and azygos continuation, a Kawashima-type operation was performed leaving only the hepatic veins and pulmonary veins draining to the atrium. In 56 patients with bilateral SVC and no connecting vein, bilateral superior cavopulmonary anastomoses were constructed, either at the staging operation or at the time of Fontan completion, and in 5 patients a redundant left superior vena cava was ligated. Thirty patients (22%) had undergone surgical pulmonary arterioplasty before the Fontan operation; 21 patients (16%) had an operation for partial or total extracardiac or obstructed intracardiac pulmonary venous return; 5 patients underwent an atrioventricular valve plasty for severe atrioventricular valve regurgitation; and 2 patients underwent atrioventricular valve replacement. Seventeen patients had previously undergone coil occlusion of aortopulmonary collateral vessels, and 3 had coil occlusion of pulmonary arteriovenous malformations.
Preoperative hemodynamics
Preoperative arterial oxygen saturation was 80% ± 6%, hemoglobin was 16 ± 1.6 mg/dL, and mean atrial pressure was 7.5 ± 3.5 mm Hg. Mean pulmonary artery pressure was 13.3 ± 3.7 mm Hg (range, 7 to 26 mm Hg) and was greater than 20 mm Hg in 2 patients. Median pulmonary vascular resistance was 2.2 ± 1.5 Wood units (range, 0.6 to 11 Wood units). Mean pulmonary vascular resistance was greater than 3 Wood units but reactive to oxygen ventilation in 10 patients. Ventricular end-diastolic pressure was higher than 10 mm Hg in 14 patients (median, 9.5 ± 3.7 mm Hg; range, 4 to 18 mm Hg).
Preoperative arrhythmia
Information on the presence or absence of preoperative arrhythmias was available for all patients. The prevalence of any documented temporary or permanent supraventricular arrhythmia before establishment of the Fontan circulation was 24% (32 of 135 patients). Twelve patients (9%) had a known history of supraventricular tachyarrhythmia, and 20 patients (15%) had temporary or permanent bradyarrhythmia. Six patients had a permanent pacemaker implanted before the Fontan operation.
Surgical technique
After median sternotomy, hypothermic cardiopulmonary bypass was used in all patients. The ascending aorta was cannulated, and up to four venous cannulas were inserted in superior vena cava, inferior vena cava, and hepatic vein when these did not drain into the IVC. Aortic cross clamping and cardioplegic arrest was performed in all but 8 patients. Depending on the preference of the surgeon and the type of concomitant procedure performed, a period of hypothermic circulatory arrest was used in 38 patients.
In patients without a prior Glenn shunt, a unilateral or bilateral superior cavopulmonary anastomosis was constructed; the pulmonary artery was divided if necessary, any systemic-to-pulmonary arterial shunts were taken down, and a pulmonary arterioplasty was performed when necessary. The atrium was then opened in all patients, and the anatomy of systemic and pulmonary connections was inspected. The type of Fontan connection was chosen so as to obtain unobstructed flow of IVC or hepatic vein blood to the pulmonary artery without compromising pulmonary venous blood flow, or both. In earlier years an atriopulmonary anastomosis was fashioned and the atrium was septated or a complex patch inserted that directed the pulmonary venous blood to the atrioventricular valve (n = 17; 13%). After the introduction of the lateral tunnel Fontan modification in 1987, an intracardiac Fontan connection that incorporated the native atrial wall to allow for growth was the preferred technique, but it was not always applicable. In a total of 67 patients (50%) some form of lateral tunnel Fontan modification was used: either the typical tunnel along the lateral atrial wall connecting the IVC orifice with the SVC or right atrial roof (n = 43); a complex tunnel incorporating separate entrance sites of IVC and hepatic veins (n = 12); a tunnel traversing the back of the atrium to connect the IVC with a contralateral SVC (n = 9); or a tunnel guiding IVC blood to the entrance of an atrial appendage, which was then end-to-side anastomosed to the pulmonary artery (n = 6). In 2 patients an additional GoreTex tube graft (W.L. Gore & Assoc, Flagstaff, AZ) was used to connect separate hepatic veins with the lateral tunnel (within the atrium in 1 patient and in an extracardiac position between hepatic veins and IVC in the other patient). Entirely intracardiac tube grafts measuring between 14 and 22 mm in diameter were used to connect IVC or hepatic veins with the SVC or right atrial roof in 19 patients (14%). In 52 patients (58%) who had an intracardiac Fontan connection, the SVC–right atrium junction was incised and the roof of the right atrium was opened to enlarge the atriopulmonary anastomosis. Remnants of the interatrial septum were excised in 14 patients.
However, in 25 patients (18%) the atrial and venous anatomy did not allow for an intraatrial Fontan pathway, and a total extracardiac Fontan was constructed using a ring-enforced 14 to 22 mm GoreTex tube graft (W.L. Gore & Assoc) anastomosed to the IVC and the ipsilateral pulmonary artery. In patients with an absent IVC, the hepatic veins were usually excised including a cuff of atrial wall to facilitate anastomosis with the conduit. In 7 patients the tube graft was anastomosed with the IVC or hepatic vein orifice within the atrium, then guided through the atrial free wall and anastomosed with the SVC outside the heart (intraextracardiac Fontan). A 3.5 to 6 mm fenestration was placed in 98 patients (73%). Among the 32 patients operated on before 1991, 8 (25%) were fenestrated compared to 90 of 103 patients (87%) operated on after 1990 (p < 0.001; Fischer’s exact test). Together with the Fontan operation, a surgical pulmonary arterioplasty was performed in 14 patients. Twelve patients (9%) underwent relief of pulmonary vein stenosis or correction of anomalous extracardiac pulmonary vein connection, or both, at the time of the Fontan operation. In 10 additional patients (7%), a significantly regurgitant atrioventricular valve was repaired at the time of the Fontan completion. Mean cardiopulmonary bypass time was 137 ± 30 minutes, and mean aortic cross-clamp time was 70 ± 19 minutes.
Postoperative hemodynamics
On the first postoperative day, the average systemic venous pressure was 13.8 ± 2.6 mm Hg (range, 8 to 20 mm Hg), and pulmonary venous atrial pressure was 7.3 ± 2.3 mm Hg (range, 3 to 13 mm Hg). Mean arterial oxygen saturation at discharge was 84% ± 7% (range, 63% to 97%). The median mechanical ventilation time was 1 day (range, 1 to 25 days), median intensive care unit stay was 3 days (range, 1 to 38 days), and median time to discharge was 13 days (range, 4 to 48 days). Thirty-two patients (23%) had pleural effusions that drained for longer than 10 days, and 8 patients also had pericardial effusions that necessitated drainage.
At most recent follow-up, the average systemic venous pressure was 15.6 ± 4.8 mm Hg (range, 8 to 32 mm Hg), average pulmonary venous atrial pressure was 10.5 ± 4.7 mm Hg (range, 3 to 23 mm Hg), and the average arterial oxygen saturation was 90.3% ± 4.4%. Ventricular function was known to be significantly depressed in 3 patients, and 33 patients (25%) had mild-to-moderate or moderate atrioventricular valve regurgitation diagnosed by echocardiography. Nine patients had one or more pulmonary venous obstructions or occlusions at follow-up, and 5 of these patients had discrete stenosis of one pulmonary vein orifice. In 2 patients the pulmonary veins were externally compressed by a significantly enlarged atrium; in 1 patient the intracardiac baffle had obstructed pulmonary venous return; and in another patient the extracardiac conduit compressed the atrium resulting in obstructed pulmonary venous flow.
Fontan failure
There were a total of 9 early deaths (7%) and 7 late deaths (6%) (Table 1).
Kaplan-Meier estimated survival for the entire cohort was 90% at 1 year and 87% at 10 years. Six of the early deaths occurred in patients operated on before 1991 (early mortality, 19%); 3 early deaths occurred after 1991 (early mortality, 3%). Kaplan-Meier survival curves for patients according to the era of operation (before 1991 and since 1991) are presented in Figure 1A. Survival rates were 70% (95% CI, 55%–84%) at 10 years for patients operated on before 1991, and 93% (95% CI, 88%–98%) for those since 1991. Two other patients underwent early postoperative takedown of the Fontan circulation to a bi-directional Glenn shunt for low cardiac output with systemic acidosis and renal failure. One patient underwent heart transplantation 7 years after the Fontan operation for protein losing enteropathy. Two more patients were hospitalized with a failing Fontan circulation 7 months and 6 years postoperatively, respectively, but were medically stabilized, and no further intervention has been performed to date.
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2 test). Survival for patients with preoperative atrioventricular valve regurgitation was not different from patients with a competent atrioventricular valve (81% vs 88% at 10 years; p = 0.53; log-rank test) (Fig 3).
There was no significant difference in survival between the different technical modifications used (Fig 4).
Univariable analysis further indicated that a prior bidirectional Glenn shunt had a positive impact, and late atrioventricular valve regurgitation had a negative impact on survival. Multivariable analysis identified only a higher pulmonary vascular resistance as an independent risk factor for death (hazard ratio = 1.6; p = 0.007) (Table 2).
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During follow-up, aortopulmonary collateral vessels or pulmonary arteriovenous malformations were coil-occluded in 11 patients. Two patients underwent pulmonary arterioplasty, and 1 patient underwent pericardiectomy for restrictive pericarditis. One patient with severe atrioventricular valve regurgitation underwent atrioventricular valve replacement and another patient atrioventricular valve plasty. One patient with a 14-mm intraatrial GoreTex tube (W.L. Gore & Assoc) had obstruction of IVC flow developed and this patient underwent replacement of the conduit with a 19-mm tube graft 3.2 years postoperatively. A total of 36 patients had device closure of the baffle fenestration, and the fenestration is known to have closed spontaneously in 5. In 9 patients, a small baffle leak was detected and interventionally closed in 7.
Postoperative arrhythmia
During the early postoperative period, a total of 62 patients (47%) had some form of transient or permanent arrhythmia, 31 in the form of supraventricular tachyarrhythmia and 31 as bradyarrhythmia. Twenty-eight patients were temporarily paced, and 11 underwent early postoperative implantation of a permanent pacemaker. At the time of discharge, sinus rhythm was documented in 81 of 126 patients (65%).
A current electrocardiogram was available in 95 patients (80%), and the mean interval from the Fontan operation to the most recent electrocardiogram was 6.5 ± 3.4 years. Nineteen (16%) of the surviving patients had documented bradyarrhythmia at follow-up, 11 of whom were permanently paced. Overall Kaplan-Meier freedom from late bradyarrhythmia was 83% at 5 years and 78% at 10 years, and this was lower for patients with left isomerism compared with patients with right isomerism (66% [95% CI, 57%–64%] vs 83% [95% CI, 78%–87%]; p = 0.019, log-rank test) (Fig 5). Late postoperative tachyarrhythmia was documented in 25 patients (21%), and was new in 19 (16%). Kaplan-Meier freedom from late tachyarrhythmia was 82% at 5 years and 70% at 10 years, and 4 patients underwent radiofrequency ablation. Although freedom from late tachyarrhythmia at 10 years appeared lower in patients with left isomerism than those with right isomerism (44% [95% CI, 28%–60%] vs 79% [95% CI, 73%–85%], the difference did not reach statistical significance (p = 0.2; log-rank test) (Fig 6).
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Other late complications
In 3 patients thrombus formation in the Fontan pathway was detected, and 1 of these patients also had a stroke. Another patient developed seizures for which anticonvulsive medication was prescribed. One patient had right hemidiaphragm paralysis with decreased exercise tolerance. Another patient developed hemoptysis which resolved after coil embolization of pulmonary arteriovenous malformations. Information on functional status was available for 104 patients; 73 patients (70%) met the criteria for the New York Heart Association functional class I, 28 (27%) met the criteria for functional class II, and 3 (3%) met the criteria for functional class III.
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Comment |
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TopAbstractIntroductionMaterial and methodsResultsCommentDiscussionReferences |
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However, it must be emphasized that our study is strictly limited to patients who were deemed to benefit from a Fontan-type operation and who reached the appropriate age. Reports on the overall-outcome of children with heterotaxy syndrome have generally shown a poor prognosis. Of 20 patients diagnosed with right isomerism, reported by Sadiq and colleagues [13], overall survival was 45%, and only 9 patients were potential candidates for a Fontan-type operation. Hashmi and colleagues [3] reported a mortality rate of 69% for children with right atrial isomerism, and Gilljam and colleagues [5] from the same institution described a mortality of 44% in children born with left atrial isomerism. These cohort studies include patients with normal functioning hearts, hearts amenable for a biventricular repair, as well as hearts with a single functioning ventricle. Of the many associated malformations found in heterotaxy syndrome with single ventricle, atrioventricular valve regurgitation and anomalous pulmonary venous connection are considered the most important contributors to increased mortality. As Gaynor and associates [4] have recently analyzed, many of the children with single ventricle physiology and total anomalous venous connection die before they reach the stage of a Fontan completion. Mortality was 53% before or at the initial palliative operation, and mortality risk for the subsequent cavopulmonary anastomosis was 38%. For patients who reach the stage of Fontan completion, however, the outlook is better, but historically it has still been worse than for other patients with single ventricle. Michelion and associates [14] reported a 10-year survival of patients who underwent a Fontan operation between 1975 and 1990. Five-year survival was greater than 70% for patients operated on after 1986. The authors attributed the improved outcome to the development of more effective Fontan modifications such as the lateral tunnel and intraatrial conduit. For a number of patients with anomalous location of the pulmonary vein orifices, extracardiac tube grafts have added a further option for direction of IVC or hepatic venous drainage to the pulmonary arteries [15], or both. Consequently, Azakie and colleagues [16] recently reported much improved results of the Fontan operation in patients with heterotaxy syndrome. An extracardiac conduit was used in 22 of 30 patients, but they still observed a high incidence of early postoperative supraventricular arrhythmia. Since 3 of 4 early deaths occurred in patients with concomitant repair of anomalous pulmonary venous connection (APVC), they emphasized the importance of early detection and repair of anomalous or obstructed APVC, or both. Similarly, Caldarone and associates [17] recently identified the presence of heterotaxy syndrome or univentricular physiology, or both, as a significant risk factor for death in patients undergoing surgery for total APVC. Although an earlier article from our institution stated that the presence of total AVPC did not represent a risk factor for newborns with heterotaxy syndrome, it must be remembered that the article focused on primary palliation in the neonate (ie, repair of obstructed APVC or initial shunt palliation, or both), and the article did not systematically study the outcome after completion of Fontan repair [18].
Although a clear cause and effect relationship cannot be proven from the data presented in this retrospective study, we attribute the improved outcome over the last decade to the combination of four important treatment concepts: (1) Aggressive surgical treatment of concomitant disease such as anomalous or obstructed pulmonary venous return, or both; atrioventricular valve regurgitation; and pulmonary artery stenosis, distortion, or discontinuity before or at the time of the Fontan operation; (2) a staged approach to the Fontan operation with bidirectional cavopulmonary shunts in the vast majority of patients; (3) the appropriate choice of Fontan modification according to the atrial and venous anatomy; and (4) the frequent use of a baffle or conduit fenestration. We briefly address each of these points as follows:
19 mm) conduit. Both extracardiac and intracardiac pathways are complicated when there are abnormalities of the hepatic venous blood flow. Some have argued that the risk of thromboembolism is elevated in patients with interrupted IVC when the extracardiac conduit carries only hepatic venous blood with relatively low flow. It is our policy to recommend warfarin anticoagulation for at least 3 months postoperatively with such cases. In patients with a continuous IVC, the hepatic veins can enter the atrium separately and at some distance from the IVC orifice, so that an extracardiac conduit must be anastomosed either to a cuff of atrial wall or separately to IVC and hepatic veins.
Study limitations
The two major limitations of the present study are its retrospective nature and the restriction to patients with heterotaxy syndrome who had reached the stage of a Fontan repair and were deemed promising candidates. Although we are confident regarding the outcomes of death and failure, the incidence of late arrhythmia is most likely underestimated. Our data represent any kind of a reported bradyarrhythmia or tachyarrhythmia, irrespective of duration or clinical significance. Regarding the pre-selection of Fontan candidates with heterotaxy syndrome, we have made reference to the high mortality of patients with single ventricle and anomalous pulmonary venous connection. However, the present article is not a cohort study, but rather serves to demonstrate that a Fontan operation in patients with such complex malformations can now be performed with excellent results once a patient has reached the appropriate stage.
Although morbidity in terms of postoperative arrhythmias and prolonged pleural effusions is significant, the Fontan operation can now be performed in patients with heterotaxy syndrome with excellent long-term survival. This does not imply that heterotaxy syndrome is no longer a risk factor for a Fontan operation, because our cohort was obviously pre-selected, and many patients with heterotaxy and severe associated malformations never reach the stage of Fontan completion. However, excellent long-term survival can be achieved in many of these patients when malformations such as extracardiac or obstructive pulmonary venous return and atrioventricular valve regurgitation are treated aggressively, the Fontan repair is staged, a fenestration is used liberally, and the technique is carefully chosen to minimize the risk of pulmonary or systemic venous obstruction.
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Appendix |
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Year of Fontan operation (continuous)
Gender
Double outlet right ventricle
Common atrioventricular valve
Single right ventricle
Right isomerism
Left isomerism
Interrupted inferior vena cava
Bilateral superior vena cava
Bilateral superior vena cava anastomosis
Atrioventricular valve regurgitation
Total anomalous pulmonary venous return
Anomalous systemic venous return
Pulmonary artery distortion
Prior Blalock–Taussig shunt
Prior pulmonary artery banding
Prior coarctation repair
Prior bidirectional Glenn
Prior Kawashima operation
Prior atrial septectomy
Prior pulmonary arterioplasty
Prior pulmonary vein operation
Prior atrioventricular valve plasty
Presence of aortopulmonary collaterals
Prior pacemaker implantation
Preoperative pulmonary artery pressure (continuous)
Preoperative pulmonary vascular resistance (continuous)
Preoperative left ventricular end-diastolic pressure (continuous)
Preoperative hemoglobin (continuous)
Atriopulmonary Fontan
Intraatrial tunnel Fontan
Intraatrial tube graft
Extracardiac tube graft
Intraextra-atrial tube graft
Fenestration
Superior vena cava–right atrium junction incised
Septectomy
Pulmonary arterioplasty
Pulmonary vein operation
Atrioventricular valve repair
Pleural effusion more than 10 days
Early postoperative arrhythmia
Early postoperative bradyarrhythmia
Early postoperative tachyarrhythmia
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Discussion |
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As others in the recent past, the authors today have made a very strong case that improved outcomes are in large part related to staging of Fontan operations with early removal of the volume load on the systemic ventricle by means of caval pulmonary anastomosis, and they have emphasized the evolution of techniques based on total caval pulmonary connection of either the intracardiac tunnel or extracardiac conduit variety. The Boston group gravitated toward total caval pulmonary connection of the lateral atrial tunnel type in 1987 and went largely to staging high-risk Fontans with a preliminary bidirectional Glenn anastomosis in 1989.
My first question, Dr Stamm, relates to the breakpoint in your analysis, which, as you indicated was in 1991. Are the outcomes analyzed in this fashion because of a specific shift in treatment protocol at that time, or did this result from a preliminary review of the outcome data?
My second question relates to the observation that although many patients had operations on the pulmonary veins and on the atrioventricular valves prior to the Fontan operation, you also reported that a comparable number, I believe 22 patients, had operations on the pulmonary veins or the atrioventricular valves at the time of the complete Fontan procedure. Was there any difference in eventual outcomes between those two patient groups? Should these factors be neutralized prior to undertaking the Fontan procedure as suggested by the Toronto group and others, or do the improving results which you have experienced give you the confidence to tackle atrioventricular valve regurgitation and pulmonary venous obstruction at the time of an eventual Fontan procedure?
My third question is related to the potential for pulmonary venous obstruction given the nearly infinite variety of patterns of pulmonary venous connection in heterotaxy patients. With staging, after superior caval pulmonary anastomosis, the pulmonary-to-systemic blood flow ratio is routinely approximately 0.5 or 0.6 to 1. How then at the time of pre-Fontan hemodynamic evaluation do you ascertain the adequacy of the pulmonary venous connections, knowing that pulmonary blood flow will increase by as much as 100% as a consequence of the Fontan operation?
My final question is: Have you solved the technical challenges associated with reliable fenestration of extracardiac conduit Fontans, or does this difficult problem account in part for the higher incidence of effusions postoperatively in these challenging patients?
My compliments to you and your colleagues on your very fine work and my gratitude to the Society of Thoracic Surgeons for the privilege of discussing this article. Thank you.
DR STAMM: Regarding the cut-off point of the year 1991 in the survival analysis, I have to admit that this was chosen more or less arbitrarily. The rationale behind this for us was that the shift from older style Fontan modifications, such as the atriopulmonary anastomosis as well as the beginning of the fenestration area, was completed in 1991 at Children’s Hospital Boston, so that basically all patients were treated according to the more modern concepts. The final decision on what kind of Fontan modification to use in a patient is made by the individual surgeon, and 1991 was the time point when the shift in techniques was unified among all the surgeons that were operating on at the time. However, in the multivariable analysis we used the year of operation as a continuous variable, which means that whenever we found an earlier year of an operation as a risk factor for a particular outcome, this simply meant "an earlier time point" but not "before 1991."
With regard to atrioventricular valve operation before or at the time of Fontan, I believe that one of the reasons that there are a number of patients who had their AV valve repair only at the time of the Fontan and not before ie, at the time of the Glenn shunt is that in some patient with ventricular volume overload and atrioventricular valve regurgitation one hopes that the Glenn shunt will decrease the volume load and thereby lead to an improvement of the atrioventricular valve regurgitation. This does happen in many patients but not in all, and I think that these patients, who even after the Glenn, even with reduced ventricular volume load still had atrioventricular valve regurgitation, were the ones who had their atrioventricular valve repaired at the time of the Fontan.
With regard to your question about pulmonary venous obstruction, it is of course one of the most difficult diseases to be diagnosed in our specialty. It is a very good question that I cannot answer conclusively. I believe that Dr. John Mayer is in the audience. Maybe he would like to comment on the diagnosis of pulmonary vein obstruction after the Glenn.
DR JOHN E. MAYER (Boston, MA): I do think that Marshall has a raised a very important point about the diagnosis of pulmonary venous obstruction, particularly in situations of reduced pulmonary blood flow. I would say that our general approach is that we try to optimize the patients prior to the Fontan, operation, and so certainly, if one makes the diagnosis in the pre-Glenn time period, then one would try to repair the veins at that point. There is also the option of direct observation, at least of the entrance points, of the pulmonary veins into the atrium at the time of the Fontan.
One of the other reasons for delaying the time of either pulmonary vein or atrioventricular valve repair at the time of bidirectional caval pulmonary shunt is that one can clearly do the bidirectional caval pulmonary shunt with the beating heart without any period of cardiac ischemia. And so we frequently will, if you will, hedge our bets a bit by looking first for the effect of reducing the volume load on the regurgitant atrioventricular valve at the time of the Glenn procedure and will not necessarily have to address that until the time of the Fontan.
DR STAMM: Thank you very much, Dr Mayer. And with regard to Dr Jacobs’ final question about the extracardiac Fontan modification and fenestration, as far as I am informed, we make every effort to place a fenestration, even when we do an extracardiac Fontan operation. There is, of course, more than one way to do this. One can puncture a hole into the extracardiac graft and into the atrial wall and anastomose it directly, but sometimes one has to interpose a tube graft. What modification leads to the best patency rate of the fenestration is actually the subject of a study that we are doing right now, which deals exclusively with fenestration in patients with an extracardiac Fontan graft.
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