Eur J Cardiothorac Surg 2001;20:598-602
© 2001 Elsevier Science NL
Risk factors influencing early and late mortality after total cavopulmonary connection
Naoki Yoshimuraa,
Masahiro Yamaguchia,
Yoshihiro Oshimaa,
Shigeteru Okaa,
Yoshio Ootakia,
Teruo Teib,
Sachiko Kidob
a Department of Cardiothoracic Surgery, Kobe Children's Hospital, Kobe, Japan
b Department of Cardiology, Kobe Children's Hospital, Kobe, Japan
Received 23 October 2000;
received in revised form 21 May 2001;
accepted 13 June 2001.
Corresponding author. Department of Cardiothoracic Surgery, Kobe Children’s Hospital, 1-1-1, Takakura-dai, Suma-Ku, Kobe, 654-0081, Japan. Tel.: +81-78-732-6961; fax: +81-78-735-0910
e-mail: y-naoki{at}za2.so-net.ne.jp
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Abstract
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Objective: Among the later modifications of the Fontan type procedure, a significant alteration was introduced by de Leval and associates when they described the total cavopulmonary connection (TCPC). Although current results of TCPC have encouraged us to extend this procedure to high risk patients, risk factors influencing surgical outcome after TCPC have not been evaluated. We review our experiences with TCPC to identify which risk factors may have had a considerable impact on the outcome of patients undergoing TCPC and to clarify selection criteria of high-risk Fontan candidates for TCPC. Patients and methods: We retrospectively reviewed the medical and surgical records of all 76 patients who underwent TCPC between July 1988 and August 2000. A cross-sectional review of these patients was undertaken. Results: There were eight early deaths and four late deaths. In a Fisher's exact test, the following variables were associated with an increased early mortality after TCPC: systemic ventricular morphology (right ventricle), ejection fraction of the systemic ventricle less than 60%, and prolonged cardiopulmonary bypass time (240 min or longer). The log-rank test demonstrated that heterotaxy syndrome, moderate to severe atrioventricular valve regurgitation, prolonged cardiopulmonary bypass time (240 min or longer), and prolonged aortic cross clamp time (70 min or longer) were associated with late mortality after TCPC. Six deaths occurred in eight (75%) patients who had six or more risk factors, whereas six deaths (9%) occurred in those who had five or fewer. Conclusions: Patients with complex cardiac anomalies who have six or more risk factors should be excluded from TCPC candidates.
Key Words: Fontan operation • Total cavopulmonary connection • Risk factors
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1. Introduction
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Since Fontan and Baudet [1] first described their procedure for the correction of tricuspid atresia in 1971, the principles of the Fontan procedure have been applied to all forms of functional univentricular congenital heart defects. During the past two decades, several modifications of this operation and advances in postoperative management have improved surgical results despite application of the Fontan approach to patients with complex cardiac anomalies [2–6]. The total cavopulmonary connection (TCPC), first described by deLeval and associates [7] in 1988, is the most widely used in the later modifications of the Fontan operation [8,9]. It is well known that TCPC maintains laminar flow within the venous pathway and minimizes energy loss within the Fontan circuit [7,10,11]. Although operative indications of TCPC have now been extended to high risk patients, risk factors influencing early and late mortality after TCPC have not been evaluated. Therefore, we review our experiences with TCPC to identify which risk factors may have had a considerable impact on the outcome of patients undergoing TCPC and to clarify selection criteria of high-risk Fontan candidates for TCPC.
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2. Patients and methods
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2.1. Patients
We retrospectively reviewed the medical and surgical records of all 76 patients who underwent TCPC at Kobe Children’s Hospital between July 1988 and August 2000. The mean age at operation was 6.8±3.4 years, ranging from 1 to 18 years. The primary malformation was univentricular heart in 34, tricuspid atresia in 12, mitral atresia in 11, double outlet right ventricle in eight, transposition of the great arteries in 4, pulmonary atresia with intact ventricular septum in four, and other complex cardiac defects in the remaining three (Table 1). The main causes of univentricular repair in double outlet right ventricle were hypoplastic left ventricle, straddling atrioventricular valve, or multiple ventricular septal defects. The mean follow-up period was 43.6 months (range, 1–146 months) and the end of the follow-up study was September 30th, 2000.
2.2. Surgical procedures
The operation was performed with a cardiopulmonary bypass in all but three cases who underwent off pump TCPC. Unless a concomitant intracardiac procedure was to be performed, aortic cross clamping was avoided. Every effort was made to minimize the duration of cardiopulmonary bypass. The bidirectional Glenn shunt was made by direct end-to-side anastomosis between the superior vena cava and the pulmonary artery. Intraatrial conduit TCPC was performed in 44 patients, and extracardiac TCPC was performed in the 32 others. A 16-to 22-mm expanded polytetrafluoroethylene tube graft was interposed between the inferior vena cava and pulmonary artery in all but one patient who underwent extracardiac TCPC with direct anastomosis between the pulmonary trunk and the inferior vena cava. Additional procedures were performed in many cases, such as plasty of the pulmonary artery, repair of the atrioventricular valve, repair of anomalous venous return, and so on (Table 2). Later in this series, a fenestration [6,12] was created between the conduit and the right atrium in 26 high risk patients.
2.3. Potential predictors
Although there are still no clearly accepted criteria regarding the risk factors for TCPC, we consider the following factors to be potential risk factors of mortality after TCPC according to previous reports [3–6] and our clinical impression. (1) Young age at operation (less than 2 years) (2) Systemic ventricular morphology (right ventricle). (3) Heterotaxy syndrome. (4) Moderate or severe atrioventricular valve regurgitation. (5) Four or more previous palliative procedure. (6) Deformity of the pulmonary artery (distortion, coarctation, unbalanced pulmonary arteries etc.). (7) Hypoplastic pulmonary artery (Nakata index less than 200 mm2/m2). (8) Mean pulmonary arterial pressure more than 20 mmHg. (9) Pulmonary vascular resistance more than 3 Wood unit. (10) Ejection fraction of the systemic ventricle less than 60%. (11) Arrhythmia. (12) Prolonged cardiopulmonary bypass time (240 min or longer). (13) Prolonged aortic cross clamp time (70 min or longer). (14) The presence of anomalous pulmonary venous return. (15) The presence of anomalous systemic venous return. (16) The presence of systemic obstruction. We also analyzed the following predictors. (1) The period of operation (1988–1996 versus 1997–2000). (2) One stage versus two stage TCPC. (3) Extra versus intracardiac TCPC. (4) Fenestrated versus non-fenestrated TCPC.
2.4. Statistical analysis
Relationships between early mortality and the potential predictors were examined with Fisher’ s exact test. The log-rank test was used for the analysis of late results. A P value less than 0.05 was considered significant.
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3. Results
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3.1. Early and late mortality after TCPC
There were eight early deaths due to low cardiac output syndrome in five, thrombosis in two, and tracheal bleeding in one. Preoperative diagnoses of these patients were univentricular heart in five, mitral atresia in two, double outlet right ventricle in one. A patient who had undergone five palliative procedures died of thrombosis within the central pulmonary artery. In this patient, the central pulmonary artery had been reconstructed with the use of an e-PTFE graft (Fig. 1)
. There were four late deaths due to congestive heart failure in two patients, arrhythmia in one, and cerebral infarction in one. Three of these four patients had heterotaxy syndrome. Deaths occurred from 4 to 18 months after TCPC.
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Fig. 1. Pulmonary arteriography of the patient who died of thrombosis within the central pulmonary artery. He had undergone five palliative procedures including a reconstruction of the central pulmonary artery with the use of an e-PTFE graft. He had six risk factors including multiple prior palliative operations and severe deformity of the pulmonary artery. He should have been excluded from TCPC candidates. rt.PA: right pulmonary artery, lt.PA: left pulmonary artery.
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3.2. Predictors of mortality
In a Fisher's exact test, the following variables were associated with an increased early mortality after TCPC: systemic ventricular morphology (right ventricle) (P<0.05), ejection fraction of the systemic ventricle less than 60% (P<0.05), and prolonged cardiopulmonary bypass time (240 min or longer) (P<0.05) (Table 3).
The log-rank test demonstrated that heterotaxy syndrome (P<0.05), atrioventricular valve regurgitation (P<0.05), prolonged cardiopulmonary bypass time (240 min or longer) (P<0.01), and prolonged aortic cross clamp time (70 min or longer) (P<0.05) were associated with late mortality after TCPC.
3.3. Relationship between mortality and the number of risk factors
The relationship between the number of risk factors and mortality after TCPC is shown in Fig. 2
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Fig. 2. Relationship between mortality and the number of risk factors. Six deaths occurred in eight (75%) patients who had six or more risk factors, whereas six deaths (9%) occurred in those who had five or fewer (P<0.0001).
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Six deaths occurred in eight (75%) patients who had six or more risk factors, whereas six deaths (9%) occurred in those who had five or fewer (P<0.0001).
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4. Discussion
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Among the later modifications of the Fontan type procedure, a significant alteration was introduced by de Leval and associates [7] when they described the total cavopulmonary connection (TCPC). There was improvement in the overall hemodynamic profile of TCPC in terms of energy conservation, mainly caused by a decrease in turbulence and energy dissipation. Moreover, this technique offers the advantages of being easily reproducible, suitable for all types of atrioventricular valve arrangements, and avoids the ill effect of high pressure in the coronary sinus by leaving it in the low-pressure chamber [7]. Although current results of TCPC have encouraged us to extend this procedure to high risk patients [8–10] who exceeded several factors of selection criteria described by Choussat and Fontan [13], risk factors influencing surgical mortality after TCPC have not been evaluated. In this study, we have analyzed early and late outcomes after TCPC to determine which factors predispose to failure of TCPC.
Gentles and associates [6] reported that patients with left-sided atrioventricular valve atresia or stenosis, left ventricular hypoplasia, and obstruction of the systemic outflow tract were associated with a higher risk of early and late failure of the Fontan circulation. Uemura and associates [14] reported the unsuitability of the morphologically right ventricle for the systemic circulation after a Fontan-type operation. We found that the systemic ventricle of all patients who died after TCPC was morphologically right ventricle. Univariate analysis demonstrated a significant association between early mortality after TCPC and systemic ventricular morphology. For Fontan circulation, pulmonary vascular resistance and the compliance of the systemic ventricle are the most important factors in venous blood flowing through the pulmonary vasculature. We do consider ventricular dysfunction to be important concerns, but it may be difficult to predict ventricular function after ventricular volume load is reduced by the Fontan procedure. In the present study, ejection fraction of the systemic ventricle less than 60% was associated with an increased early mortality after TCPC.
Early operative survivors with heterotaxy syndrome had a significantly higher likelihood of late failure, as has been previously reported [5,6]. In the present study, there were 4 (20%) early deaths and 3 (15%) late deaths in 20 patients with heterotaxy syndrome. The log-rank test demonstrated that heterotaxy syndrome was one of the significant predictors of late outcomes of TCPC. Reasons for the poorer outcome in this anatomic subgroup are speculative and probably multiple. Patients with heterotaxy syndrome have a complex single ventricle anatomy. Anomalies of pulmonary and systemic venous return are prevalent and almost all had a common atrioventricular valve [15]. Although these factors were not associated with an increased mortality after TCPC by statistical analysis, atrioventricular valve regurgitation is likely to occur frequently across a common atrioventricular valve. Furthermore, concomitant procedures such as the repair of anomalous pulmonary or systemic venous return and plasty of an atrioventricular valve frequently require prolonged cardiopulmonary bypass and prolonged myocardial ischemia.
Deformity of the pulmonary artery, elevated pulmonary artery pressure, and elevated pulmonary vascular resistance are widely recognized risk factors for the outcome after Fontan type procedure. Deformity of the pulmonary artery, elevated pulmonary artery pressure and pulmonary vascular resistance were not independent predictors of mortality after TCPC in this series. However, the number of patients with elevated pulmonary artery pressure or pulmonary vascular resistance was too small to assess these factors in a meaningful way. Also, we did not consider patients who were not offered TCPC because of elevated pulmonary artery pressure or pulmonary vascular resistance. We certainly consider elevated pulmonary artery pressure or pulmonary vascular resistance important because blood should pass through the pulmonary vascular bed with low resistance in Fontan circulation.
In recent years, indications of the Fontan type operation have been extended to complex cardiac anomalies and high risk Fontan candidates [3,4]. To reduce the risks and to decrease the operative mortality and morbidity of high risk Fontan candidates, two-staged strategy [16] and baffle fenestration [6,12] were introduced. In the present study, a fenestration was placed in 26 patients since 1997. The indication for fenestration mainly depended on the preoperative risk factors, and the patients performed fenestrated TCPC had at least one risk factor related to pulmonary artery or ventricular function. The practice of allowing an atrial-level right-to-left shunt in a modified Fontan operation has gained widespread acceptance. In the immediate postoperative period, cardiac output can be maintained with lower systemic venous pressure that may modulate other risk factors such as residual pulmonary artery distortion or hypoplasia, bypass-related increase in pulmonary vascular resistance, and ventricular dysfunction [6,12]. This technical modification was associated with a decline in the mortality rate from 18.0% (9/50) in patients without fenestration to 11.5% (3/26) in patients with fenestration in our series. Although it is apparent that a fenestration is effective in reducing the mortality after Fontan type operation, there were three deaths in patients who underwent fenestrated TCPC. These three patients had nine [17], eight, and seven risk factors, which suggested that fenestration does not overcome overwhelming negative odds (Fig. 2). In the present study, we have analyzed the relationship between the number of risk factors and the mortality after TCPC and revealed that the mortality of the patients who had six or more risk factors was much higher than that of the patients who had five or fewer risk factors. These findings demonstrated that patient selection remains an important factor in the outcome of TCPC.
In summary, the results of the statistical analyses demonstrated that systemic ventricular morphology (right ventricle), dysfunction of the systemic ventricle, heterotaxy syndrome, atrioventricular valve regurgitation, and prolonged cardiopulmonary bypass or cardiac ischemia have had a significant impact on the outcome of patients undergoing TCPC. In our experience, the mortality of the patients who had six or more risk factors was much higher than that of the patients who had five or fewer risk factors. Thus we conclude that patients with complex cardiac anomalies who have six or more risk factors should be excluded from TCPC candidates.
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Footnotes
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Presented at the 24th World Congress of International Society for Cardiovascular Surgery, Melbourne, Australia, September 12–16, 1999.
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Top
Abstract
1. Introduction
