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Right arrow Congenital - cyanotic
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Eur J Cardiothorac Surg 2003;24:502-510
© 2003 Elsevier Science NL

Orthotopic heart transplantation for failing single ventricle physiology

Guido Michielona*, Francesco Parisib, Duccio Di Carloa, Cosimo Squitieria, Adriano Carottia, Michaela Burattaa, Roberto M. Di Donatoa

a Department of Cardiac Surgery, Ospedale Pediatrico Bambino Gesù, Rome, Italy
b Department of Pediatric Cardiology, Ospedale Pediatrico Bambino Gesù, Rome, Italy

Received 5 November 2002; received in revised form 23 April 2003; accepted 26 April 2003.

* Corresponding author. Tel.: +39-06-6859-2333; fax: +39-06-6859-2257
e-mail: guido.michielon{at}tin.it


   Abstract
Top
 Abstract
1. Introduction
 2. Methods
 4. Results
 5. Discussion
 Appendix A. Conference...
 References
 
Objective: Evaluation of incremental risk factors for early mortality in children undergoing orthotopic heart transplantation (OHT) for failing single ventricle physiology. Methods: Between 1988 and 2002, 25 patients (mean age 9.3±7.1 years) underwent OHT for complex congenital heart disease (CHD) with a functional right (15 patients) or left (10 patients) single ventricle. Palliative staging towards Fontan completion had been previously accomplished in 22 patients (88%). Transition to OHT occurred from a shunt stage in 10, a bi-directional cavopulmonary anastomosis (BDG) stage in nine, and after Fontan failure in six patients. Results: Thirty-day survival was 68.0±9.3% with no additional mortality up to 14.1 years. OHT following BDG staging exhibited 100% long-term survival, as opposed to 66.7±15.7% for OHT after systemic-to-pulmonary shunt, and 33.3±19.2% for OHT following failing Fontan (p=0.032). Regression logistic modelling indicated failing Fontan circulation as predictor of higher mortality after OHT (p=0.041). Reintervention was necessary in four patients 40±11 months after OHT to address residual superior vena cava (two) and isthmic (two) stenosis. Overall freedom from reintervention was 88.3±8.1% at 5 years. Conclusions: OHT for structural CHD with single ventricle physiology entails substantial early mortality while BDG enables the best transition to heart transplant. OHT should be considered in the decision-making process as an alternative to Fontan completion in high-risk candidates, since rescue-OHT after failing Fontan seems unwarranted.

Key Words: Transplantation • Heart disease • Congenital


   1. Introduction
Top
 Abstract
 1. Introduction
2. Methods
 4. Results
 5. Discussion
 Appendix A. Conference...
 References
 
Parallel advancements in surgical technique, preoperative and postoperative care as well as better understanding of neonatal physiology in patients with duct-dependent pulmonary or systemic circulation and a functional single ventricle have led to superb results in staged long-term palliation of most complex congenital heart disease (CHD) [1]. The improved early survival, however, has not abolished late mortality secondary to myocardial failure, therefore, increasing the referrals for cardiac transplantation. Interstage attrition [2] is moreover expected in staged palliation towards completion of a Fontan-type circulation, while acute or late Fontan failure [3] represents a growing indication for heart transplantation. The annual report from the ISHLT [4] indicates that pediatric recipients received 10.9% of the reported heart transplants worldwide. However, little is known about the results of orthotopic heart transplantation (OHT) for failing single ventricle physiology. The focus of this report is a complete review of our experience with OHT in children with single ventricles. Specifically, we verified the anatomical predictors of poor outcome after OHT, estimating the impact of single ventricle staging on early hazard after OHT and questioning the choice of high-risk palliative surgery followed by rescue heart transplant as opposed to first-line transplantation.


   2. Methods
Top
 Abstract
 1. Introduction
 2. Methods
4. Results
 5. Discussion
 Appendix A. Conference...
 References
 
Between November 1988 and August 2002, 25 patients with single ventricle physiology underwent OHT for end-stage heart failure. There were 16 males and nine females. The mean age at OHT was 9.3±7.1 years. This cohort included three neonates (under 3 days of life at OHT) and two infants (under 6 months of age at OHT).

The underlying structural cardiac malformation and the description of previous surgical repair or palliation are depicted in Table 1. Ventricular morphology was right-dominant in 15 patients and left dominant in 10 patients. Visceral heterotaxy was diagnosed in three patients.


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  		Table 1. Anatomic details and surgical history of children with single ventricle physiology in chronological order of OHT

 
A mean of 2.2 previous palliative procedures (Table 1) had been accomplished prior to OHT, including Norwood first stage palliation or Damus–Kaye–Stansel with systemic-to-pulmonary shunt in three, coarctation repair in four, systemic-to-pulmonary shunting or pulmonary artery banding (PAB) in 17, bi-directional cavopulmonary anastomosis (BDG) in 14, Fontan completion in six, stent placement in three, and other procedures in 10.

For the purpose of a meaningful analysis, we combined under the term ‘shunt stage’ all non-palliated hypoplastic left heart syndrome (HLHS), Norwood first stage palliation, systemic-to-pulmonary shunting possibly associated with a Damus–Kaye–Stansel operation, and PAB, since they all reproduced a similar physiologic pattern. OHT was, therefore, performed at the shunt stage in 10 patients, BDG stage in nine patients and Fontan stage in six patients. Fontan completion was systematically prepared by intermediate staging with BDG, irrespective of ventricular morphology. Technically, all but one Fontan completion were performed according to our institutional protocol of extracardiac conduit interposition between the inferior vena cava (IVC) (four patients) or the hepatic veins (one patient), and the pulmonary arterial tree. Intracardiac conduit interposition between the IVC and the right pulmonary artery had been accomplished prior to OHT in one patient at another institution. Mean age at Fontan completion was 8.6±4.2 years. None of the patients undergoing Fontan completion could have been defined as ideal candidates at the time of Fontan evaluation, because of heterotaxy syndrome (three patients), AV valve incompetence (two patients) or mean PA pressure exceeding 15 mmHg (one patient). The mean time interval between Fontan completion and OHT for Fontan failure was 4.7±3.9 years. Fontan failure occurred within 12 months from surgery in two patients. Technical complications at the time of Fontan operation occurred in only one patient, who developed myoglobinura and acute renal failure secondary to lower limb ischemia and posterior compartment syndrome during prolonged femoro-femoral bypass. Substantial contribution to the deterioration of Fontan circulation was otherwise secondary to the onset of protein-losing enteropathy (two patients), progressive AV valve incompetence after attempted valve plasty (one patient), and atrial arrhythmias (two patients). Effective stent placement at the level of the left pulmonary artery branch (one patient) or the junction between the conduit and the pulmonary artery confluence (two patients) was achieved prior to OHT to solve even minor-grade stenosis of the systemic venous pathway. All patients were accepted and listed for OHT (24) or combined OHT and renal transplant (one patients) after careful in-house evaluation. At the time of OHT, substantial inotropic support (exceeding 6 µg/kg/ min i.v. dopamine infusion) was delivered to five patients. Acute renal failure requiring dialysis did not controindicate OHT in one patient.

2.1. Surgical management
Slightly oversized donors were preferred (mean donor-to-recipient weight ratio=1.3±0.6). According to the planned reconstruction, extensive native tissue was harvested from the donor, including the entire aortic arch, the pulmonary artery bifurcation, the pulmonary artery branches, the superior vena cava (SVC), and innominate vein. Graft preservation technique included a single-dose cold crystalloid cardioplegic infusion in the aortic root, soon after aortic cross-clamping. Cold Celsior® infusion has been preferred as cardioplegic and solid organ preservation solution in the last five patients. Heart transplant was performed under hypothermic cardiopulmonary bypass. Short periods of deep hypothermic circulatory arrest were necessary in 10 patients. Conventional orthotopic transplantation was accomplished according to the technique described by Lower and Shumway in six patients. Bicaval technique was recently preferred in the last 19 OHT. Associated procedures were necessary in 20 patients (80%) and depicted in Table 1.

2.2. Immunosuppression
Non-invasive rejection surveillance was preferred in most cases and endomyocardial biopsy was used in selected cases. Immunosuppression was cyclosporine-based. Intravenous methylprednisolone was used for the first 48 h. Maintenance therapy included cyclosporine, azathioprine, and low-dose prednisone (0.1 mg/kg). Prednisone therapy was possibly discontinued within the first 3–6 months, while azathioprine was tapered off within the first 2 postoperative years and discontinued according to rejection-freedom. After the sixth postoperative month, a two-drug therapy regimen including MMF and cyclosporine was recently preferred in patients with repeated episodes of rejection.

Total follow-up was 76.2 patient-years, ranging from 1 day to 14.1 years (mean 36 months); it was 100% complete and updated. All survivors attended the local follow-up patient clinic. Routine ECG and 2D echocardiography were performed every 3 months or earlier when indicated. Endomyocardial biopsy was routinely performed at least on a yearly basis for the first 5 years after OHT, while cardiac catheterisation and coronary angiography were always performed 12 months after OHT and every second year thereafter.

2.3. Statistical analysis
Statistical analysis was conducted with the SAS-Statview-1998 statistical software, SAS Institute Inc. Cary, NC. Survival analysis and reoperation freedom after OHT were conducted according to the Kaplan–Meier technique; Mantel–Cox log–rank testing was utilized to compare survival among subgroups. Early mortality was defined as death within 30 days from surgery. Potential predictors for early mortality were assessed by univariate logistic regression analysis. A secondary analysis was conducted to verify the impact of SV staging on early transplant survival. Fisher's exact testing was used to verify any association between SV staging and favourable OHT outcome. Survival probability was then estimated and stratified by SV staging; log–rank testing was used to allow survival comparison among strata.


   4. Results
Top
 Abstract
 1. Introduction
 2. Methods
 4. Results
5. Discussion
 Appendix A. Conference...
 References
 
4.1. Mortality
There were eight early deaths after OHT. Causes of death included elevated PVR (one patient), necrotising enterocolitis (two patients), bleeding (there patients), residual right ventricular outflow tract obstruction (one patient), and cerebral embolism (one patient). No lethal event could be attributed to inadequate graft performance, although acute Fontan failure forced acceptance of a borderline graft (EF 35%) in one patient with polysplenia syndrome. A wrong indication to OHT was the cause of early graft failure in one patient, at the beginning of our experience with OHT in CHD. This child, born with {S,L,L} segmental anatomy, double inlet left ventricle, and subaortic stenosis developed neo-aortic valve incompetence after a Damus–Keye–Stansel operation with combined systemic-to-pulmonary shunting. Presence of borderline elevation in pulmonary vascular resistance index (3.8 units/m2) was attributed to diffuse hypoplasia of left pulmonary artery, with non-equal distribution of pulmonary blood flow. OHT was performed with a match-sized donor and a large, unrestrictive pulmonary artery reconstruction was accomplished. Nevertheless, persistent elevation in pulmonary vascular resistance resulted in pulmonary hypertension and right ventricular failure, while mechanical support devices and refined pulmonary vasodilation agents were not available.

Necrotising enterocolitis was the cause of early death in two neonatal recipients with HLHS. Laparotomy was attempted in one, but the extension of small bowel necrosis prevented effective resection. Necropsy confirmed the clinical diagnosis and demonstrated IVC thrombosis in both, with right renal vein occlusion in one and bilateral acute tubular necrosis in the other. Bleeding was the leading cause of death in three patients with failing Fontan circulation. Elevated systemic venous pressures predisposed to splanchnic venous congestion and liver dysfunction, which contributed to postoperative coagulopathy. Postoperative ECMO support was attempted in one case without success. In each patient, aprotinine was not available or not allowed for epidemic restraints (Creutzfedt–Jakob encephalopathy). Previous stenting of the left pulmonary artery prevented adequate right ventricular outflow tract reconstruction in another child with failing Fontan circulation. Parallel valved xenograft interposition between the donor RVOT and the right pulmonary artery was attempted; nevertheless, residual outflow obstruction resulted in supra-systemic right ventricular pressure, which was not felt to be amenable to further correction. Mechanical support was not deemed indicated. The last early death occurred in a neonate, born with HLHS and transplanted on the 28th day of life. The postoperative course was complicated by left ventricular dysfunction and multiorgan failure. Fatal cerebral embolism occurred on the sixth postoperative day. Postmortem examination showed myocardial ischemia involving the anterolateral wall of the left ventricle, with no evidence of coronary embolism and no histological evidence of hyperacute rejection. Thrombus adhesion to the left atrial suture line was considered to be the source of the embolic event.

Kaplan–Meier survival was 68.0±9.3% at 1 month. No additional mortality was experienced after the first postoperative month, therefore, cumulative survival remained unchanged at 68.0±9.3% up to 14.1-year follow-up. Splitting in half the time span of our experience, cumulative survival has improved to 73.7±10.1% at 7 years since 1995, yet not statistically different from 50.0±20.4% 14.1-year survival of the early series (Fig. 1 ). OHT in failing single ventricle physiology entailed a substantial early risk phase even in recent years. Logistic regression indicates Fontan completion as the only potential predictor of early failure in our experience (Table 2). A secondary analysis was conducted to verify the impact of single ventricle staging on early transplant survival. Fisher's exact testing demonstrated an association between BDG staging and favourable OHT outcome (Table 3). Stratification of the risk of death showed that survival probability was significantly improved when OHT was performed after BDG staging, rather than shunt staging or worse, after Fontan completion (Fig. 2 ). Late survival for OHT after BDG transition is still 100% at 6-year follow-up.



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  		Fig. 1. Kaplan Meier survival according to year of OHT.

 

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  		Table 2. Univariate logistic model coefficient table for early mortality

 

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  		Table 3. Fisher's exact test for OHT survival according to single ventricle staging strategy

 


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  		Fig. 2. Survival probability stratified by SV staging strategy.

 
4.2. Complications and reoperations
Postoperative morbidity included acute renal failure requiring peritoneal dialysis in two patients, phrenic nerve dysfunction in one, respiratory insufficiency requiring reintubation in two, and systolic hypertension necessitating long-term medical therapy in four patients. The mean ICU stay was 27±14 days, lowering to 18±7 days since 1995. OHT and prolonged total parenteral nutrition allowed successful control of protein-losing enteropathy in one patient with failing Fontan circulation. He returned to full diet and recovered normal total serum protidemia and protein electrophoretic pattern.

Further surgery or interventional procedures were indicated in four patients 40±11months after OHT. At the beginning of our transplant experience and prior to the advent of mechanical assist devices, a cavopulmonary anastomosis was performed 6 h after OHT to unload a failing right ventricle. Recovery of right ventricular function occurred, allowing BDG take-down 2 days later. The child is currently alive and well 14 years after OHT. Other procedures included combined balloon dilation of the aortic isthmus and SVC stenting in one patient, combined aorto-plasty and SVC bypass with a 10 mm PTFE conduit in one patient, and isolated balloon dilation of the aortic isthmus in one patient. No patient underwent retransplantation for chronic rejection. Overall freedom from any type of reintervention is 88.3±8.1% at 5 years and 44.1±22.4% at 14.1 years (Fig. 3 ). There were 22 episodes of acute rejection in the 17 early survivors with no lethal event. The linearized rate of acute rejection episodes was 0.29 per patient-year, falling from 0.52 episodes per patient in the first postoperative month to 0.014 episodes per patient-month thereafter. Currently, 11 patients (65%) are free of steroids and three patients (18%) are on monotherapy (cyclosporine). The patients on triple drug regimen (azathioprine, cyclosporine, and prednisone) are the ones with shorter follow-up (mean 23 months). No lethal infections or lymphoproliferative disease occurred in this cohort. There were two Gram positive bacterial infections that resolved with antibiotic therapy, while CMV was the most common source of viral infection (four patients). Protozoal infection was rare (one patient). The linearized rate of infection was 0.11 per patient-year.



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  		Fig. 3. Freedom from reoperation for OHT early survivors.

 

   5. Discussion
Top
 Abstract
 1. Introduction
 2. Methods
 4. Results
 5. Discussion
Appendix A. Conference...
 References
 
Neonatal palliation and staged reconstruction towards a Fontan-type circulation is currently feasible in most patients with complex CHD and single ventricle physiology. The concept of Fontan staging through a hemifontan or a BDG has reduced the early mortality for Fontan completion, preventing the onset of diastolic ventricular dysfunction [5]. Increased experience, relaxed criteria, and the introduction of the fenestration concept have resulted in extended indication for the modified Fontan operation, nevertheless, interstage attrition prior to Fontan completion and early failure of a Fontan circuit are expected and accepted in some otherwise lethal CHD. Aside from the experience of Bailey et al. [6], heart transplantation is usually considered as ‘the last resource’ in the decision-making process, with a strong preference for high-risk palliative procedures focused on the preservation of the native myocardium. Even in ideal Fontan candidates, however, the instantaneous risk of death from cardiac failure progressively increases 2 years from surgery, with an 86% 5-year and 74% 15-year survival, respectively [7]. The attrition rate is known to be highest in the first 6 months after Fontan completion [8], but arrhythmias, protein-losing enteropathy [9], and progressive cardiac failure can occur as late and possibly fatal complications of Fontan procedures. Single ventricle circulation appears to have a limited durability per se and myocardial dysfunction or anatomic features that preclude conservative therapy following single or multi-staged repair, represent a growing indication for OHT [3]. The worldwide number of pediatric heart transplants has remained stable since the mid-90s at approximately 350 procedures per year, yet limited donor supply remains the major drawback, especially for small size patients. The fourth official pediatric report of the ISHLT [10] demonstrated a consistent and substantial improvement in actuarial survival over the last 14 years, nevertheless CHD represents the leading independent predictor of 1-year mortality after pediatric heart transplantation [10]. This occurs while 76.5% of OHT performed under the first year of life are accomplished on patients with CHD [10]. Surgical creativity [11–15], innovative solutions, and careful surgical planning have largely overcome the technical dilemmas encountered in OHT for CHD, like abnormalities of atrial situs, cardiac position, systemic and pulmonary venous connections, or great arteries position, therefore, adapting the complex recipient anatomy to the normal donor anatomy. Moreover, deficiencies or post-surgical distortions in recipient anatomy can be compensated by extensive harvesting of arterial, venous, and pericardial donor tissues. Although innovative technical contributions can solve most anatomical puzzles in OHT, all transplant candidates compete for the same donor pool, forcing the ethical issue of making the best use of a limited resource. Our results on OHT for CHD have steadily improved in recent years, with an overall 7-year survival of 79.9% after 1995. This report, however, elucidates the sub-optimal early results of OHT in single ventricle physiology. What is new in this analysis is the evidence that the stratification of the risk of early failure within the single ventricle subgroup is different according to the stage of palliation towards Fontan completion. Since the mid-80s, we adopted a strategy of single ventricle staging by systematic interposition of a BDG prior to Fontan completion, irrespective of ventricular morphology [16]. We verified that BDG ensures a progressive change in ventricular geometry, largely related to regression of ventricular hypertrophy, secondary to abolishment of volume overload. The ventricular remodeling minimizes the risk of diastolic dysfunction after Fontan completion, ultimately reducing the overall risk of early Fontan failure. Provided that both Fontan completion and OHT are palliative in nature, we now demonstrated that BDG staging is, at the same time, a limbo and a cross-road in the decision-making process between Fontan completion versus OHT. Ultimately, OHT appears to be an excellent form of palliation, with no early or late mortality for OHT after BDG staging, with an 88.3±8.1% overall freedom from any type of surgical or interventional procedure at 5-year follow-up and with two-third of the patients being free from steroid therapy. This finding is valuable since our experience refers to a homogeneous cohort with a systematic BDG staging prior to extracardiac Fontan completion. Survival of OHT after BDG staging is comparable or even superior to the gratifying experience of OHT in CHD with two discernible ventricular chambers and pediatric OHT for cardiomyopathy [10]. This can be explained by the peculiar tolerance of a cavopulmonary shunt even in patients with borderline hemodynamic patterns, which allows for precise transplant timing and appropriate donor selection. On the other hand, both Fontan take-down and rescue-OHT are not an easy task to reach in failing Fontan patients [8]. The decision to undertake a high-risk repair versus heart replacement must be tailored to the individual patient, but our provocative inference is that high-risk Fontan completion may hazard a future transplant option. Moreover, the charges for heart transplantation usually exceed that for a Fontan procedure, nevertheless, Fontan palliation in high-risk patients is ultimately more costly and increases postoperative morbidity [17], delivering only a brief respite prior to transplantation referral. Defining a patient as a high-risk Fontan candidate is not a straightforward judgement and should account for both early mortality and short-term failure. Knott-Craig et al. [8] reviewed the Mayo Clinic experience on over 700 modified Fontan procedures performed over a 16-year interval. Hospital mortality was 14%, while Fontan take-down for early failure carried a worrisome 62.5% mortality rate. Higher preoperative mean PA pressure, asplenia syndrome, higher post-Fontan PA pressure, and longer aortic cross-clamp time were predictive of poor early outcome or Fontan take-down, while systemic AV valve incompetence reached borderline significance. A recent review of the Toronto experience showed a 56% mortality for pre-Fontan palliation in right atrial isomerism and single ventricle physiology [18]. Fontan completion was achieved in 22% patients with 25% mortality, while two patients underwent heart transplant with one death. Results were surprisingly worse for patients presenting after 1989, with a 32% 1-year actuarial survival, therefore, implying that improvements in surgical techniques did not improve early outcome in this difficult group of patients. Results were somewhat better for patients with left isomerism presenting at the same institution [19]. Single ventricle palliation in left isomerism was associated with 54% survival at 1 year, 45% at 5 years, and 39% at 10 years. Nevertheless, Fontan completion by extracardiac rerouting of the hepatic veins to the pulmonary arterial tree was associated with a 50% mortality in that experience. Conversely, heart transplantation is a valid alternative in children with visceral heterotaxy. Larsen et al. [20] from the Loma Linda group recently reported a 68% 5-year and 50% 10-year survival after OHT in 29 patients with visceral heterotaxy who were at high risk for standard palliation because of AV valve insufficiency, ventricular dysfunction, or aortic atresia.

Retrospectively, all our patients undergoing OHT after Fontan failure could have been defined as high-risk candidates at the time of Fontan evaluation. Based on this review, we infer that heterotaxia and especially asplenia syndrome, presence of moderate to severe systemic AV valve incompetence, or mean PA pressure exceeding 15 mmHg should probably suggest a careful evaluation before Fontan completion, since BDG staging and elective conversion to heart transplantation can probably achieve a better outcome for these patients. The second fundamental question refers to the justified concern of OHT candidacy for failing Fontan, since post-transplant morbidity and mortality in this subgroup is quite serious. The unsatisfactory results of OHT in failing Fontan is probably multifactorial [21–23]. Calculation of pulmonary vascular resistance in pre-transplant evaluation is hampered by low flow secondary to loss of hydrodynamic energy through the venous pathway or by non-equal blood-flow distribution in the pulmonary arterial tree. Microvascular sludging and thromboemboli can occur in the pulmonary vasculature, while arterio-venous malformations may false trans-pulmonary gradients. Therefore, we should anticipate a potential for right ventricular failure in all Fontan patients undergoing OHT. Moreover, perioperative bleeding is expected, due to either chronic anticoagulation or splanchnic venous congestion and associated liver dysfunction. Besides, previous stenting of either systemic venous pathway or pulmonary artery branches can puzzle the surgical course. Although current results and short donor supply do not justify routine heart–lung transplantation, acceptance of less-than-ideal heart donors should be avoided even under the pressure of deteriorating clinical status of the recipient. Successful outcome is largely related to the number of desirable donor criteria and donor–recipient matching, since poor recipients eventually require the best hearts, as we learned from at least one of our early OHT failures. Prospective cross-matching, early recipient referral for transplant assessment, optimal donor selection, and careful surgical planning with minimized operative time represent the keys of successful transplantation in patients with failing Fontan circulation.

In conclusion, OHT for CHD with single ventricle physiology entails substantial early mortality, nevertheless, BDG enables the best transition to heart transplant. Beyond cavopulmonary shunt staging, OHT should be considered in the decision-making process as an alternative to Fontan completion in high-risk candidates, since rescue-OHT after failing Fontan seems unwarranted.


   Acknowledgments
 
The authors recognise the technical assistance and expertise of Dr Marina Negri for the statistical review and data analysis of this cohort.


   Footnotes
 
Presented at the 16th Annual Meeting of the European Association for Cardio-thoracic Surgery, Monte Carlo, Monaco, September 22–25, 2002.


   Appendix A. Conference discussion
Top
 Abstract
 1. Introduction
 2. Methods
 4. Results
 5. Discussion
 Appendix A. Conference...
References
 
Dr M. Turina (Zurich, Switzerland): Did you encounter any situs inversus anatomy and how did you solve it? I find it personally extremely difficult, especially if you try to use it without foreign material.

Dr Michielon: Yes, we did encounter one patient like this, a patient with polysplenia syndrome, situs inversus, hemiazygous continuation to a left SVC and hepatic veins draining independently to a common atrium. We tried to reroute the systemic venous return with the use of conduits. The procedure, however, was not successful. Nevertheless, excellent results are reported in the literature with the use of atrial flaps. I believe that that is the way to go.

Dr C. Yankah (Berlin, Germany): I would like to ask a question and to comment also on the anatomical risk factors for early mortality. Which anatomic variation revealed as a high risk for perioperative heart failure? Which mechanical circulatory support systems do you use for perioperative heart failure or postcardiotomy heart failure as a bridge to heart transplantation in order to decrease the early mortality? You have a high early mortality between the primary Fontan operation and orthotopic transplantation among the high risk patients which are not well defined. However, I believe that these candidates with a low cardiac output and organ dysfunction need the strategy of our Institution by applying earlier a ventricular assist system to decrease the early morbidity and mortality.

Dr Michielon: Congenital heart disease is the leading independent predictor of 1-year mortality after pediatric heart transplantation worldwide. Most of these events occur in the early postoperative period. The best mechanical support of a failing transplant is still ECMO, up to now. We have supported two patients prior to transplant and after transplant with IABP and ECMO. One patient survived to recovery. But when considering a transplant in a failing Fontan, elevated venous pressures, mesenteric venous stasis and liver dysfunction predispose to coagulopathy; beyond the fact that most of these patients are chronically anticoagulated. ECMO can be helpful, but morbidity in the very early phase is high and not unfrequently lethal.

Dr M. Turina: Did you ever enter the age of the patients as an independent variable? It does not appear in your statistics as a risk factor.

Dr Michielon: When age was entered as a continuous variable in both univariate and multivariate analysis, there has been no correlation between patient's age and risk of early death after transplantation.


   References
Top
 Abstract
 1. Introduction
 2. Methods
 4. Results
 5. Discussion
 Appendix A. Conference...
 References
 

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