Circulation. 2003;108:II-140-II-149
doi: 10.1161/01.cir.0000087442.82569.51
(Circulation. 2003;108:II-140.)
© 2003 American Heart Association, Inc.
Surgery for Congenital Heart Disease |
Orthotopic Heart Transplantation for Congenital Heart Disease: An Alternative for High-Risk Fontan Candidates?
Guido Michielon, MD;
Francesco Parisi, MD;
Cosimo Squitieri, MD;
Adriano Carotti, MD;
Giulia Gagliardi, MD;
Luciano Pasquini, MD;
Roberto M. Di Donato, MD
From the Dipartimento Medico-Chirurgico di Cardiochirurgia e Cardiologia Pediatrica, DMCCP, Ospedale Pediatrico Bambino Gesù, Roma, Italy.
Correspondence to Guido Michielon, MD, Dipartimento Medico Chirurgico di Cardiologia Pediatrica, Ospedale Pediatrico Bambino Gesù, P.zza S.Onofrio 4, 00165 Roma, Italy. Phone: 06-6859-2333; Fax: 06-6859-2257; E-mail: guido.michielon{at}tin.it
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Abstract
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Objective— Evaluation of incremental risk factors for
early mortality in children undergoing orthotopic heart transplantation
(OHT) for congenital heart disease.
Methods— Between 1988 and 2002, 43 patients (mean age 9.1±7.2 years) underwent 44 OHT for complex TGA (6), DORV (4), single ventricle (21), and other end-stage structural heart disease (11). Two discernible ventricular chambers were present in 18 pts (41.8%). Previous reconstructive or palliative procedures had been previously accomplished in 35 pts (83.3%), including atrial switch (5), systemic-to-pulmonary shunts (10), cavopulmonary anastomosis (9), Fontan completion (6), and others (5).
Results— 30-day survival for the 2-ventricle subgroup was 94.4±5.4% compared with 67.2±9.5% for the single ventricle subgroup (P=0.04) (overall 78.6%±3.3%). OHT following single ventricle staging to bi-directional cavopulmonary anastomosis exhibited 100% early survival, as opposed to 62.5±17.1% for OHT after systemic-to-pulmonary shunts, and 33.3±19.2% for OHT following failing Fontan (P=0.010). HLHS diagnosis (0.0085) and failing Fontan (P=0.003) were identified as independent predictors of early mortality by regression logistic modeling, while Fontan stage represented the only predictor of overall mortality by Cox proportional hazard. Overall 10-year survival was 54.3±11%.
Conclusions— OHT for structural congenital heart disease with single ventricle physiology entails substantial early mortality and bi-directional cavopulmonary anastomosis 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 • surgical aspects • pulmonary circulation • heart disease • congenital
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Introduction
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Neonatal primary repair or staged long-term palliation is currently
recommended for most complex congenital heart disease (CHD).
The improved early survival, however, has not abolished late
mortality secondary to myocardial failure, therefore increasing
referrals for cardiac transplantation.
1 Interstage attrition
2 is expected in staged palliation of single ventricles (SV),
while acute or late Fontan failure
3 represents a growing indication
for heart transplantation (OHT). The focus of this report is
a complete review of our experience with OHT in children with
CHD. 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.
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Methods
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Between November 1988 and August 2002, 43 patients underwent
44 OHT for end-stage heart failure secondary to structural CHD,
not amenable to conservative surgical reconstruction or definitive
palliation. There were 28 males and 15 females. The mean age
at OHT was 9.27±7.2 years. This cohort included 3 neonates
(under 30 days of life at OHT) and 5 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. Two discernible ventricular chambers were present in 18 patients while 25 patients underwent OHT, falling out a staged palliation toward Fontan completion. Ventricular morphology in SV was right-dominant in 15 and left dominant in 10. Visceral heterotaxy was diagnosed in 3 patients with SV. A mean of 2.14 previous reconstructive or palliative procedures (Table 1) had been accomplished in 35 patients (83.3%) before OHT, including atrial switch operation in 5, REV operation in 1, Norwood first stage palliation or Damus-Kaye-Stansel with systemic-to-pulmonary shunt in 3, coarctation repair in 6, systemic-to-pulmonary shunting or pulmonary artery banding in 24, bi-directional cavopulmonary anastomosis (BDG) in 14, Fontan completion in 6, stent placement in 4, and other procedures in 33. For the purpose of a meaningful analysis in the SV subgroup, we defined as "shunt stage" all nonpalliated HLHS, Norwood first stage palliation, systemic-to-pulmonary shunting possibly associated with a Damus-Kaye-Stansel operation, and pulmonary artery banding, since they all reproduced similar physiology. Therefore, OHT in SV was performed at the shunt stage in 10 patients, at the BDG stage in 9 and at the Fontan stage in 6 patients (Table 2). Fontan completion was systematically prepared by intermediate staging with BDG, regardless ventricular morphology. Before Fontan completion, mean O2 saturation was 77.2%, with a mean PA pressure of 12.8 mm Hg and a mean LVEDP of 7.7 mm Hg. Preoperative mean creatinine level was 0.8 mg%. Mean age at Fontan completion was 8.6 years ±1.7, certainly unusual for normal Fontan candidates. However, none of them could have been defined as ideal candidates at the time of Fontan evaluation, because of heterotaxy syndrome (3 patients), AV valve incompetence (2 patients) or mean PA pressure exceeding 15 mm Hg (1 patient). AV valve plasty had been attempted one year prior to Fontan completion in 1 patient, while another patient had previously received a Kawashima operation. Anatomy of the pulmonary arteries at pre-Fontan evaluation was judged as adequate in 5 patients, while left pulmonary artery stenosis was present in 1. Technically, all but 1 Fontan completion were performed according to our institutional protocol of extracardiac conduit interposition between the inferior vena cava (4 patients) or hepatic veins (1 patient) and the pulmonary arterial tree. Intracardiac conduit interposition between the inferior vena cava and the right pulmonary artery had been accomplished elsewhere in 1 patient, before OHT. The mean time interval between Fontan completion and OHT for Fontan failure was 4.7 years ±1.6. Fontan failure occurred within 12 months from surgery in 2 patients. Technical complications at the time of Fontan operation resulting in myoglobinura and renal failure, forced the indication for OHT in one patient. Substantial contribution to the deterioration of Fontan circulation was otherwise related to the onset of protein-losing enteropathy (2 patients), to progressive AV valve incompetence after redo valve plasty (1 patient) and atrial arrhythmias (2 patients). Effective stent placement of the left pulmonary artery (1 patient) or at the junction between the conduit and the pulmonary artery confluence (2 patients) was achieved before OHT to solve even minor-grade stenosis of the systemic venous pathway. All patients were listed for OHT (42 patients) or combined OHT and renal transplant (2 patients) after careful in-house evaluation. At the time of OHT, mechanical circulatory support was active in 2 patients (IABP in 1 and ECMO in 1), while substantial inotropic support (exceeding 6 µg/kg/ min IV Dopamine infusion) was delivered to 5 additional patients. Acute (2 patients) and chronic (1 patient) renal failure requiring dialysis did not controindicate OHT.
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TABLE 2. Fisher’s Exact Test for OHT Early Survival According to Functional Anatomy (Single Ventricle Versus Two-ventricles) and Single Ventricle Breakdown According to Staging Strategy
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Surgical Management
Slightly oversized donors were preferred (mean donor-to-recipient weight ratio=1.58±0.11). According to the planned reconstruction, extensive native tissue was harvested from the donor. Graft preservation technique included single-dose cold crystalloid cardioplegic infusion in the aortic root, while Cold Celsior® infusion was preferred as cardioplegic and solid organ preservation solution in the last 6 patients. Heart transplant was performed under hypothermic cardiopulmonary by-pass. Short periods of deep hypothermic circulatory arrest were necessary in 13 patients. Conventional orthotopic transplantation was accomplished according to the technique described by Lower and Shumway in 12 patients. Bicaval technique was recently preferred in the last 32 OHT. Associated procedures were necessary in 37 patients (86%), mainly to address pulmonary artery (15 patients) or aortic arch (5 patients) reconstruction.
Immunosuppression
Noninvasive 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 hours. Maintenance therapy included cyclosporine, azathioprine, and low-dose prednisone (0.1 mg/kg). Prednisone therapy was possibly discontinued within the first 3 to 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 2-drug therapy regimen including MMF and cyclosporine was recently preferred in patients with repeated episodes of rejection.
Bidimensional echocardiography was performed every 3 months, or earlier when indicated. Endomyocardial biopsy was routinely performed on a yearly basis for the first 5 years after OHT, while cardiac catheterization and coronary angiography were always performed 12 months after OHT and every second year thereafter. Mean follow-up was 43.8 months (range 7 to 173 months) and was 100% complete
Statistical Analysis
Statistical analysis was conducted with the SAS-Statview-1998 statistical software, SAS Institute Inc, Cary, NC. Chi-square analysis was used to compare discrete variables between the single ventricle and the 2-ventricles subgroups, while continuous variables were compared by unpaired t-testing. Categorical analysis was conducted by chi square and Fisher exact testing.
Survival analysis after OHT was conducted according to the Kaplan-Meier technique; Wilcoxon and log-rank testing was utilized to compare survival between the single ventricle and 2-ventricles subgroup. Early mortality was defined as death within 30 days from surgery. Hazard modeling of survival was conducted according to the Blackstone method and stratified by surgical era and by functional anatomy. A secondary analysis was conducted on the SV subgroup, stratified by palliation staging; log-rank testing was again used to allow survival comparison among strata. Cox proportional hazard was used to identify independent predictors of mortality after OHT for CHD, while logistic regression was preferred to evaluate the risk factors for early mortality after OHT.
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Results
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Mortality
There were 9 early and 6 late deaths. Causes of early and late
mortality are outlined in
table 1. No early death 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 1 patient, at the beginning
of our experience with OHT in CHD. In this patient, a borderline
elevation in pulmonary vascular resistance index (3.8 U/m2)
was thought to be secondary to diffuse hypoplasia of the left
pulmonary artery after a Damus-Kaye-Stansel operation with combined
with systemic-to-pulmonary shunting. Despite unrestrictive pulmonary
artery reconstruction at the time of OHT, 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 hypoplastic left heart syndrome (HLHS). Fatal cerebral embolism occurred 6 days after OHT in another 28 days old neonate with HLHS. Postmortem examination showed myocardial ischemia, with no evidence of coronary embolism and no histological evidence of acute rejection. Thrombus adhesion to the left atrial suture-line was considered to be the source of the embolic event. Bleeding was the leading cause of death in three patients with failing Fontan circulation. Postoperative ECMO support was unsuccessful in one of them. Elevated systemic venous pressures predisposed to splanchnic venous congestion and liver dysfunction, which contributed to postoperative coaugulopathy. 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 reconstruction of the pulmonary arterial tree in another child with failing Fontan circulation. 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.
Causes of late death were secondary to transplant-related morbidity, including acute rejection 3 months after OHT in 1, chronic rejection 71 months after retransplantation in 1, chronic rejection 27 months after OHT in 1 and non compliance with immunosuppressive therapy in 3 patients.
Overall survival was 79.1±6.2% at 1 month, 76.6±6.5% at 1 year, 69.6±7.5% at 5 years and 54.3±16.6% at 10 years (Figure 1). Splitting in half the time span of our experience, there has been a progressive improvement in surgical outcome. Since 1995, actuarial survival is 79.9±7.6% at seven years, yet not statistically different from the results of the early series (Figure 1). Comparing the instantaneous hazard of death of the first and second half of our experience (Figure 2), the early risk phase played a major role even in recent years, while transplant-related morbidity was predominant in the late risk phase. OHT in SV entailed a substantial early risk phase (Figure 3), which translated in a significantly worse early survival compared with the 2-ventricle subgroup (Figure 4). A 30-day survival for the 2-ventricle subgroup was 94.4±5.4% compared with 67.2±9.5% for the SV subgroup (P=0.04). Transplantation was technically more demanding in SV, as demonstrated by longer duration of cardiopulmonary by-pass (349 minutes ±36 in SV versus 206 minutes ±14 in the 2-ventricles subgroup) (P=0.0033). When the potential risk factors for early mortality, identified by univariate analysis (Table 3), were entered in a multivariate logistic regression model, only Fontan completion and HLHS diagnosis predicted a major risk of early failure, while need of preoperative mechanical and/or inotropic support reached borderline significance (Table 4). A secondary analysis was conducted to verify the impact of SV staging on early OHT mortality. Stratification of the risk of death in SV showed that both early and late survival was significantly improved when OHT was performed at a BDG stage, rather that at a shunt stage or, worse, after Fontan completion (Figure 5). Fisher exact testing confirmed BDG as a superior bridge to OHT in single ventricles, compared with non-BDG staging (Table 2). Late survival for BDG transition to OHT is still 100% at 6.5 years follow/up. Cox proportional risk multivariate analysis identified Fontan completion as the only independent predictor of overall mortality for OHT in CHD (coef: 1.38, OR: 4.01, 95% confidence limits: 1.23 to 13.05, P=0.024).
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Figure 1. Kaplan-Meier survival plot with 95% confidence limits. The plot shows the improved results for OHT in CHD in the most recent era, with a 79.9% survival at 7 years.
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Figure 2. Predicted instantaneous hazard for mortality with 70% confidence limits according to surgical era of OHT. The nomogram of hazard function for death in the most recent experience showed an early, rapidly declining phase of hazard with no appreciable late phase. The early risk phase for OHT performed between 1988 and 1995, is slowly declining and a late risk phase is evident at 10 years after OHT. This late phase of hazard is totally related to the effects of chronic immunosuppressive therapy.
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Figure 3. Cumulative hazard plot for mortality according to functional anatomy. OHT for CHD with single ventricle physiology entails a substantial and significantly higher early risk phase, compared with CHD with 2 ventricles. Late hazard was totally unrelated to functional anatomy of CHD, and, although predominant in CHD with 2 ventricles, it is expected to occur in OHT for failing single ventricles as well.
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Figure 4. Kaplan Meier survival plot with 95% confidence limits stratified by functional anatomy. Because most uncensored events occurred in the early postoperative period, the x axis depicting the follow-up in months is expressed on a logarithmic scale, which expands the early phase of risk. The vertical dotted line highlights the significant difference in survival at one month from OHT between single ventricle and 2 ventricles CHD.
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Figure 5. Kaplan Meier survival plot with 95% confidence limits, depicting OHT outcome for failing single ventricles, stratified by palliation staging to OHT. BDG transition to OHT showed 100% survival at 6.5 years follow-up.
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Complications and Reoperations
Postoperative morbidity included acute renal failure requiring peritoneal dialysis in 6, phrenic nerve dysfunction in 3, respiratory insufficiency requiring reintubation in 3 and systolic hypertension necessitating long-term medical therapy in 8. The mean ICU stay was 32.1 days ±6.1, lowering to 15.2 days ±1.7 since 1997. OHT and prolonged total parenteral nutrition allowed successful control of protein-losing enteropathy in 1 patient with failing Fontan circulation. He returned to full diet and recovered normal protein electrophoretic pattern.
Further surgery or interventional procedures were indicated in 7 patients 35.2 months ±5.8 after OHT. Before the advent of mechanical assist devices, a cavopulmonary anastomosis was performed 6 hours after OHT, to unload a failing right ventricle. Right ventricular function recovered, allowing BDG take-down 2 days later. The child is currently alive and well 14 years after OHT. Other reoperations included: main pulmonary artery plasty in 1 patient, superior vena cava stenting in 1 patient, combined aorto-plasty and SVC by-pass with a 10 mm PTFE conduit in 1 patient, balloon dilation of the aortic isthmus in 2 patients and take-down of an infected AV fistula, after combined renal and heart transplant in 1 patient. Only 1 patient underwent retransplantation for chronic rejection, 72 months after OHT. Left hemiplegia occurred after retransplantation, nevertheless the patient survived for additional 71 months before recurrence of fatal chronic rejection. Overall freedom from any type of reintervention is 72.1±0.13SE % at 14 years.
There were 53 episodes of acute rejection in the 34 early survivors with 1 lethal event. The linearized rate of acute rejection was 0.37 episodes/patient-year. Currently 20 patients (71.4%) are free of steroids and 4 patients (15%) are on monotherapy (cyclosporine). The patients on triple drug regimen (azathioprine, cyclosporine, and prednisone) are the ones with shorter follow-up (mean 14.7 months). Accelerated coronary artery disease occurred in 2 operative survivors. Both patients had been maintained on chronic steroid therapy for recurrent relapses of acute rejection, while CMV infection had been previously diagnosed in one. This patient died 27.7 months after transplant, while the other underwent successful retransplantation at 72.1 month from OHT.
No lethal infections or lymphoproliferative disease occurred in this cohort. One patient developed mitral valve endocarditis (staphylococcus aureus) which resolved with antibiotic therapy. There were 5 additional bacterial infection, while CMV was the most common source of viral infection (7 patients). Protozoal infection was rare (1 patient). The linearized rate of infection was 0.095 per patient-year.
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Discussion
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Neonatal repair or staged long-term palliation is currently
feasible in most patients with complex CHD. Evolution in surgical
strategies have opened new frontiers for selected patients.
Failing systemic right ventricles following atrial switch procedures
for transposition of the great arteries, for example, are now
considered for LV retraining and arterial switch operation.
4 Conversely, long-term results in SV physiology are less encouraging.
Even in ideal Fontan candidates, the instantaneous risk of death
from cardiac failure progressively increases 2 years from surgery,
with an 86% 5-year and 74% 15-year survival.
5 The attrition
rate is known to be highest in the first 6 months after Fontan
completion,
6 but arrhythmias, protein losing enteropathy
7 and
progressive cardiac failure can occur as late and possibly fatal
complications. Since SV circulation appears to have a limited
durability per se, increasing referrals for OHT can be predicted.
The fourth official pediatric report of the ISHLT
8 demonstrated
a substantial improvement in OHT survival over the last 14 years.
CHD diagnosis, however, represents the leading independent predictor
of 1-year mortality after pediatric OHT.
8 CHD currently accounts
for 76.5% of OHT performed in the first year of life.
8 This
implies that the early hazard for patients with complex CHD
needing a heart transplant under the first year of life remains
high.
Surgical creativity9–12 has largely overcome the technical dilemmas encountered in OHT for CHD, like abnormalities of cardiac position, atrial situs, venous connections, and great arteries position, therefore, adapting the complex recipient anatomy to the normal donor anatomy. Native atrial flaps can be preserved to redirect anomalous systemic venous return to a more convenient position for caval inflow. Moreover, deficiencies or postsurgical distortions in recipient anatomy can be compensated by extensive harvesting of donor tissue. Overcome the technical queries, donor shortage remains the major drawback of pediatric OHT. Because all candidates compete for the same donor pool, we endeavor to make the best use of a limited resource. Our results on OHT for CHD have steadily improved in recent years, with an overall seven-year survival close to 80% after 1995. This report, however, elucidates the sub-optimal early results of OHT in SV physiology, when compared with OHT in CHD with 2 discernible ventricular chambers. What is new in this analysis is the evidence that the risk stratification of early failure in SV is different according to the stage of palliation toward Fontan completion. Since the mid-1980s we endorsed a program of SV staging by systematic interposition of a BDG before Fontan completion, regardless the morphology of the dominant ventricular chamber.13,14 We verified that BDG ensures a progressive change in ventricular geometry, largely related to regression of ventricular hypertrophy, which minimizes the risk of diastolic dysfunction at Fontan completion. Provided that both Fontan completion and OHT are palliative in nature, we now demonstrated that BDG staging represents a crossroad in the decision making process between Fontan completion versus OHT. Ultimately OHT appears to be an excellent form of palliation, with no early nor late mortality for OHT after BDG staging, which favorably compares with the gratifying experience of OHT in CHD with two discernible ventricular chambers and with pediatric OHT for cardiomyopathy.15–17 This can be explained by the peculiar tolerance of a cavopulmonary shunt even in borderline hemodynamic patterns, which allows for precise transplant timing and appropriate donor selection. BDG can therefore be defined as the best bridge to OHT in failing SV.
On the other hand, both Fontan take-down and rescue-OHT are not an easy task to reach in failing Fontan.6 Our provocative inference is that high-risk Fontan completion may hazard a future transplant option. Moreover, albeit the charges for OHT usually exceed that for a Fontan procedure, Fontan palliation in high-risk patients is ultimately more costly and increases postoperative morbidity,18 delivering only a brief delay before transplantation referral. Defining a patient as a high-risk Fontan candidate is not a straightforward judgment, and should account for both early mortality and short-term failure. Knott-Craig6 reviewing the Mayo Clinic experience on over 700 modified Fontan procedures performed over a 16-year interval, reported a 14% hospital mortality, while Fontan take-down for early failure carried a worrisome 62.5% mortality rate. Higher preoperative mean PA pressure, asplenia syndrome and higher post-Fontan pulmonary artery pressure, 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 that Fontan completion was achieved in only 22% patients with right atrial isomerism and single ventricle physiology19 with 25% mortality. Actuarial survival for patients presenting after 1989 was 32% at 1 year, therefore implying that improvements in surgical techniques did not ameliorate early outcome. Results were somewhat better for patients with left isomerism presenting at the same institution. Nevertheless Fontan completion by extracardiac rerouting of the hepatic veins to the pulmonary circulation was associated with a 50% mortality.20 Our experience confirms the suboptimal results of Fontan completion in this group of high-risk Fontan candidates. Out of 137 patients with asplenia/polysplenia syndrome seen at our Institution in the last 2 decades, Fontan completion was accomplished in 25 children with a 24% hospital mortality (6/25). Conversely heart transplantation is a valid alternative in children with visceral heterotaxy. Larsen and associates21 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. Lamour et al22 reported successful OHT in 3 cases with failed Fontan physiology. Development of pulmonary arteriovenous fistulae, mainly because of long-term presence of classic Glenn shunting, represented the leading indication for OHT, but no patient could be defined as acute Fontan failure. Free drainage of the hepatic veins in the low pressure atrial chamber was moreover present in 2 cases. This differs from our experience of OHT in failing Fontan, where the hepatic veins were always included in the pulmonary circulation, therefore contributing to liver congestion and dysfunction. Retrospectively, all our OHT after Fontan failure could have been defined as high-risk candidates at the time of Fontan evaluation. Based on our experience and the meta-analysis of Fontan outcome in high-risk candidates, we infer that heterotaxy and especially asplenia syndrome, presence of moderate to severe systemic AV valve incompetence or mean PA pressure exceeding 15 mm Hg should probably suggest a careful evaluation before Fontan completion, since BDG staging and elective bridge to OHT can probably achieve a more gratifying outcome. The second fundamental question refers to the justified concern of OHT candidacy for failing Fontan. The unsatisfactory results of OHT in failing Fontan find a multifactorial genesis.23,24 Calculation of pulmonary vascular resistance in pretransplant evaluation is hampered by low flow or by nonequal 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, anticipating a potential for right ventricular failure. Perioperative bleeding is expected, because of 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 not be considered even under the pressure of deteriorating clinical status of the recipient, 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, represent the cornerstones of successful transplantation in patients with failing Fontan circulation.
In conclusion, OHT for structural congenital heart disease with SV 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.
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Acknowledgments
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The author recognizes the technical assistance and expertise
of Dr. Marina Negri for the statistical review and data analysis
of this cohort.
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Footnotes
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Presented at the 2002 Meeting of the American Heart Association,
Chicago, November 17 to 20, 2002.
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References
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Abstract
Introduction