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Eur J Cardiothorac Surg 2006;30:508-514
© 2006 Elsevier Science NL

Review

Transplantation for adults with congenital heart disease

Amir-Reza Hosseinpoura, Shay Cullenb, Victor T. Tsangb,*

a Birmingham Children's Hospital, Steelhouse Lane, Birmingham B4 6NH, UK
b UCLH Hospital and Great Ormond Street Hospital for Children, London, UK

Received 2 March 2006; received in revised form 31 May 2006; accepted 2 June 2006.

* Corresponding author. Tel.: +44 20 78138159; fax: +44 20 74301281. (Email: tsangv{at}gosh.nhs.uk).


   Abstract
Top
 Abstract
1. Introduction
 2. GUCH patients requiring...
 3. The challenges of...
 4. Outcome
 5. The availability of...
 6. Conclusion
 References
 
Heart transplantation is a recognised treatment for end-stage heart failure of any cause including congenital heart disease. Congenital heart disease has contributed relatively little to the adult heart transplant activities in the past two decades. However, this is likely to change as an increasing number of children with congenital heart disease reach adulthood because of the advances in paediatric cardiology and surgery. Some of these grown-ups with congenital heart disease (GUCH patients) will need transplantation for late myocardial dysfunction either secondary to uncorrected lesions, or despite previous repair or palliative surgery. These patients are managed along the same clinical principles as those with cardiac failure of other aetiologies, despite the lack of any evidence to support this approach. Nevertheless, they introduce new challenges. First, some may have pulmonary vascular disease and require heart–lung transplantation, or lung transplantation combined with repair of their cardiac defects. Second, those with failing Fontan circulation are usually much sicker than other transplant candidates, with protein-losing enteropathy along with renal and hepatic dysfunction. Third, a suitable donor organ may not be found due to elevated levels of antibodies in response to previous blood transfusions and possibly the previous implantation of homografts. Fourth, the operation may be technically difficult because of the presence of adhesions secondary to previous operations, collaterals, and unusual anatomy. Fifth, postoperative care may be complicated because of predisposition to bleeding, infection and pulmonary hypertension, and the presence of residual aortopulmonary collaterals resulting in a significant left-to-right shunt. Despite a higher early mortality, the overall results of heart transplantation so far have been encouraging with survivals similar to that of adults with acquired heart disease and that of the paediatric population. However, this may change as the proportion of high-risk patients (failing Fontans) increases. GUCH patients with Eisenmenger's syndrome may be offered lung transplantation with repair of the cardiac defect or heart–lung transplantation. However, because of the limited success of these approaches, and improved management of pulmonary hypertension, patient selection remains difficult.

Key Words: Congenital heart disease • GUCH • Transplantation


   1. Introduction
Top
 Abstract
 1. Introduction
2. GUCH patients requiring...
 3. The challenges of...
 4. Outcome
 5. The availability of...
 6. Conclusion
 References
 
Transplantation is an established treatment option for end-stage heart failure of any cause. The majority of such cases are due to acquired rather than congenital heart disease [1]. In fact, congenital heart disease was the primary diagnosis in only 2% of the heart transplants, 1% of isolated lung transplants, and a third of heart–lung transplants done in adults in the past two decades [1,2]. These patients with congenital heart disease are assessed and managed along the same clinical principles and guidelines as those with acquired heart disease, and the same indications, contraindications, short and long-term management and possible complications apply. Those clinical principles have been well described over the years and are beyond the scope of this review. This article aims to review only the additional issues and challenges encountered when considering transplantation for grown-ups with congenital heart disease (GUCH), the need for which may increase.

The need for transplantation in GUCH will increase as more children with congenital heart disease reach adulthood. Indeed, in the 1950s only 30% of babies with congenital heart disease survived beyond infancy [3]. Today, 75–85% survive into adolescence and adulthood [4,5]. This change in survival pattern has introduced new challenges. These challenges reflect on-going pathophysiological processes of residual or uncorrected lesions, or those imposed by previous procedures. Many of these sequalae may lead to late myocardial dysfunction, requiring transplantation [6]. In fact, it has been estimated that 10–20% of patients with congenital heart disease will require transplantation at some point in their life [7]. These patients form a heterogeneous group, which is best described if classified according to the underlying pathophysiology.


   2. GUCH patients requiring transplantation
Top
 Abstract
 1. Introduction
 2. GUCH patients requiring...
3. The challenges of...
 4. Outcome
 5. The availability of...
 6. Conclusion
 References
 
Three pathophysiological groups may be defined:

(1) Those with uncorrected defects.
(2) Those with previous reparative surgery but with deteriorating ventricular function.
(3) Those with previous palliative surgery which is now failing.

2.1 Uncorrected defects
These can be the result of late presentation, misdiagnosis, or the previous decision not to intervene. Late presentation does not necessarily rule out conventional surgery, provided ventricular function is not severely impaired and pulmonary vascular disease has not developed. These parameters must be carefully assessed since uncorrected defects are often complicated with pulmonary hypertension (see below). In severe cases, pulmonary hypertension may be irreversible (Eisenmenger syndrome), in which case isolated heart transplantation would be contraindicated, and the patient may need heart–lung transplantation, or lung transplantation combined with repair of the cardiac defects [8–10].

A previous decision not to intervene may have been taken in asymptomatic patients with conditions, which could only be repaired with complex high-risk operations. Examples include Ebstein's anomaly and congenitally corrected transposition of the great arteries with intact ventricular septum. Some of these patients will need heart transplantation later in life as ventricular function deteriorates.

2.2 Previous reparative surgery
Any repair that leaves the right ventricle as the systemic pump may lead to late ventricular failure requiring transplantation [11–13]. Examples include the ‘classic’ repair of congenitally corrected transposition of the great arteries where only the associated lesions (e.g. ventricular septal defects) are repaired [11], and the Senning and Mustard procedures for transposition of the great arteries [12,13].

Repair of tetralogy of Fallot may also be followed by late right ventricular failure because of the resultant regurgitant pulmonary valve. Such pulmonary regurgitation is well tolerated initially, but causes right ventricular volume overload and late right ventricular failure [14,15]. This can be managed by reoperation for pulmonary valve replacement or percutaneous transcatheter pulmonary valve implantation provided ventricular function is not severely impaired [16]. Otherwise transplantation may be the only option.

2.3 Failing palliation
Hearts with only one well-formed functional ventricle are likely to need transplantation eventually. However, this may be delayed by various palliative measures. The palliated state depends on either a systemic-to-pulmonary artery shunt, or a circuit to bypass the right heart (a Fontan circulation). Unfortunately, neither of these two measures are good long-term solutions. The former represents a volume overload, and the latter a systemic venous pressure overload. These issues may explain less-than-ideal the survival figures. Survival after a Fontan operation was found to be only 86% at 5 years and 74% at 15 years, even in the most suitable candidates [17]. Although the results have improved by the various modifications of the Fontan procedure, the relentless attrition rate after this procedure has not been eliminated [18–20]. In fact, many of these patients may require transplantation even before reaching adulthood [21–25].


   3. The challenges of transplantation for GUCH patients
Top
 Abstract
 1. Introduction
 2. GUCH patients requiring...
 3. The challenges of...
4. Outcome
 5. The availability of...
 6. Conclusion
 References
 
The overall management of any patient requiring transplantation involves the following six considerations:

(1) The indications for listing, and its timing.
(2) The formal assessment of suitability for transplantation.
(3) The pre-transplant care.
(4) The operation.
(5) The postoperative care.
(6) The long-term care.
These considerations apply equally to those with congenital heart disease requiring transplantation. However, these GUCH patients present the following additional challenges with respect to each of the above considerations.

3.1 The indications and timing for listing
The indication for transplantation for congenital heart disease, as for any other aetiology, is end-stage single organ failure, having exhausted all other treatment modalities. However, determining the optimal time to consider a patient with congenital heart disease for transplantation can be quite difficult, especially for heart–lung transplantation. Classically, this decision is based on a predicted life expectancy of 2 years or less without transplantation, despite the lack of solid evidence-based data to support the choice of this arbitrary time frame. This can be difficult to assess in the context of congenital heart disease with Eisenmenger syndrome. In fact, many of these patients seen in adult life have been told by a variety of clinicians that they would not survive, first into late childhood, then into adolescence, and then into adulthood. However, they may survive for many years with a restricted quality of life. Therefore, the objective assessment of deteriorating quality of life and the need for additional hospital admissions and medication may be the key determining factors for consideration of transplantation, rather than predictions of survival.

3.2 Formal assessment of suitability for transplantation
This evaluation takes into account the following issues:

(a) Cardiac anatomy.
(b) Cardiac function and pulmonary vascular resistance.
(c) General health of the patient.
The assessment of cardiac anatomy must include situs, the patterns of the systemic and pulmonary venous connections, and the morphology of the great vessels. In addition, distortions imposed by previous surgery must be evaluated. All this information is crucial for the planning of the operation, but it is important to note that no anatomical variation would preclude transplantation. There are ways around every anomaly [26–31].

The assessment of pulmonary vascular resistance (by cardiac catheterisation) is particularly important in the context of congenital heart disease since these patients are prone to pulmonary hypertension, and this may rule out isolated heart transplantation. In general, a pulmonary vascular resistance of less than or equal to 5 Wood units, or a transpulmonary gradient of less than 12 mmHg would be considered acceptable for heart transplantation, although there may be slight variation in the exact cut-off levels in different centres [32–36]. If higher values are detected, their reversibility should be tested in response to high-inspired oxygen, prostacycline, or nitric oxide. A good response is one where there is at least a 20% fall in the mean pulmonary artery pressure without any decrease in cardiac index or any elevation of the ratio of the pulmonary to the systemic vascular resistances. If this test restores the desired parameters (pulmonary vascular resistance of less than or equal to 5 Wood units, or a transpulmonary gradient of less than 12 mmHg), one may proceed to heart transplantation. Higher values would increase the risk of heart transplantation. Pulmonary vascular resistances exceeding 9 Wood units, and transpulmonary gradients of 15 mmHg or more would rule out isolated heart transplantation.

Despite the importance of haemodynamic evaluation, such an assessment may be difficult in many patients with congenital heart disease because of both low pulmonary blood flow and anomalies within the pulmonary vasculature. This is particularly the case in those with a failing Fontan circulation because of the significant loss of hydrodynamic energy, possible microvascular sludging and thromboemboli, and arteriovenous malformations with possible differences in the distribution of blood to the right and left lungs [37,38]. Therefore, the estimation of pulmonary vascular resistance can be inaccurate in these patients, and an element of doubt would always exist in this regard. Consequently, heart transplantation in this context may occasionally be somewhat of a ‘leap of faith’.

The general health of the patient must be carefully assessed. The suitable heart transplant candidate suffers from ‘single organ failure’, i.e. end-stage cardiac failure. However, this may not be achievable in the context of congenital heart disease, especially with a failing Fontan circulation. A failing Fontan circulation leads to protein-losing-enteropathy [22,23]. This is a devastating condition resulting in oedema, ascites, cachexia, poor healing, and a predisposition to infection. Also, these patients may have a degree of hepatic and renal dysfunction. Unfortunately, none of these complications are likely to improve significantly without transplantation or even in the early post-transplant period. Therefore, from the outset, these patients do not fit in the model of ‘single organ failure’ and are not ideal transplant candidates.

3.3 Pre-transplant care
The care of patients while waiting for transplantation is usually provided on an outpatient basis and consists of the optimisation of medical therapy, psychological support and the preparation of the patient and his/her family for life after transplantation. In addition, GUCH patients may require various pre-transplant procedures in order to provide a ‘safer’ platform for complex transplantation [6]. These include coil embolisation of modified Blalock-Taussig shunts and aortopulmonary collateral vessels, and balloon angioplasty/stenting of distorted pulmonary arteries.

Finding an immunologically suitable donor organ may also be an additional challenge. This is because many GUCH patients have undergone several previous operations. Consequently, they may have elevated levels of lymphocytotoxic IgG class antibodies in response to previous multiple blood transfusions and possibly the previous implantation of homografts [24].

3.4 The operation
There are two main issues: the choice of the operation, and the additional technical difficulties imposed by congenital heart disease. The choice of the operation is determined by the state of the pulmonary vasculature. Ideally, isolated heart transplantation is aimed for. However, this may be contraindicated by an elevated pulmonary vascular resistance. Borderline cases may be managed with the domino operation.

The domino operation takes advantage of heart–lung transplantation being offered to a patient with isolated end-stage lung disease (e.g. cystic fibrosis) [39]. Such a donor heart is potentially in a very good condition because of a short ischaemic period and not having suffered the consequences of brainstem death (‘living donor’). In addition, with its right ventricle being hypertrophied, it may be able to cope with a relatively elevated pulmonary vascular resistance. However, this approach is rarely used, partly for logistic reasons, and partly because end-stage isolated lung disease is treated increasingly with double-lung rather than heart–lung transplantation.

Severe and irreversible elevation of pulmonary vascular resistance (pulmonary vascular disease) would rule out isolated heart transplantation and impose heart–lung transplantation. However, the poor outcomes of this procedure are of major concern. Alternatively, the combination of repair of the cardiac defects and lung transplantation has been advocated by some workers, provided there is enough myocardial functional reserve to permit this and the cardiac lesion is relatively simple [8–10]. This approach has the advantage of expanding the donor pool, but it adds to the complexity of the procedure and is associated with certain postoperative pitfalls (see below).

Regardless of which operation is chosen, transplantation for congenital heart disease is often technically more complex and challenging for several reasons. First, there may be dense adhesions because of multiple previous operations. Second, cyanotic patients are likely to have developed many collateral vessels, which, if damaged, may bleed profusely. This includes all the vascular adhesions, which need to be dissected. Also, the systemic-to-pulmonary collaterals in the posterior mediastinum result in an increased pulmonary venous return which may compromise exposure during surgery. These issues are even more problematic for heart–lung transplantation since more dissection of the pleural space and posterior mediastinum is required. In fact, for heart–lung transplantation, some workers advocate access by bilateral thoraco-sternotomy (clamshell incision) rather than sternotomy in order to improve access and facilitate haemostasis [8]. Third, the conventional technique of transplantation [40] may not be possible in the context of congenital heart disease because of the unusual anatomy, and the distortion of various structures by previous surgery. However, technical adjustments, including the cannulation techniques, have been described to deal with such possible distortions and malformations [26–31]. Therefore, no anatomic variation, nor any form of distortion caused by previous surgery, would preclude transplantation. Nevertheless, these anomalies do add to the duration and complexity of the operation.

These technical issues underscore two important points regarding transplantation for congenital heart disease. First, the operation may take significantly longer in terms of the re-sternotomy and taking down of adhesions, meticulous haemostasis, and the reconstruction of distorted structures. Second, extra donor tissue may be needed for the reconstruction. The donor team must provide as much donor tissue as is necessary for the reconstruction, e.g. extra length of donor aorta including the arch, or extra length of pulmonary artery including the right and left pulmonary arteries, or extra length of the superior vena cava with or without the innominate vein. These two points highlight the importance of close communication between the donor and recipient surgical teams. The donor team must allow time for the recipient team to prepare for the implantation of the donor organ. In particular, meticulous haemostasis must be achieved before implantation.

3.5 Postoperative care
The principles of postoperative care are also identical to those for transplantation for other aetiologies, but there are a few additional issues. First, even with meticulous haemostasis, bleeding could be a major problem. These patients are likely to be coagulopathic because of prolonged bypass and possibly hepatic dysfunction in those with a failing Fontan circulation. This problem can be addressed with the use of coagulation factors, aprotinin, tranexamic acid, and surface sealants. Recombinant factor VIIa may also be helpful, but it is not widely used yet [41].

Predisposition to infection is seen in those with a failing Fontan circulation and protein-losing-enteropathy [22,23]. This is aggravated by the sudden introduction of high doses of immunosuppressive agents, and could result in significant postoperative morbidity.

Pulmonary hypertension post-transplantation can be treated with vasodilators such as phosphodiesterase inhibitors, inhaled nitric oxide and intravenous prostacycline. When pulmonary hypertension is sufficiently severe to lead to right ventricular dysfunction, right ventricular mechanical assistance may be considered for temporarily support.

Residual aortopulmonary collaterals may result in a significant left-to-right shunt and a consequent state of high output cardiac failure following transplantation [6,42]. This should be suspected if prolonged inotropic support is needed despite normal ventricular function and in the absence of rejection. Such collateral vessels may be treated by cardiac catheterisation and coil embolisation. This approach has been successfully employed after heart transplantation both in adults [6] and in children [42].

When lung transplantation is combined with repair of a cardiac defect, additional issues are introduced [8]. First, postoperative left ventricular function may be suboptimal due to its interactions with an abnormal hypertrophied right ventricle. Second, if single-lung (rather than double-lung) transplantation is offered, there may be significant postoperative ventilation/perfusion mismatch. Third, lung transplantation results in a sudden decrease in pulmonary vascular resistance (afterload), while concomitant repair of the cardiac defect results in a sudden decrease in volume load (preload). Such a sudden reduction in both preload and afterload in a hypertrophied right ventricle can initiate dynamic outflow tract obstruction. This is aggravated by inotropic agents, and ameliorated as the right ventricle remodels in response to the post-transplant low pulmonary vascular resistance. However, it is not clear to what extent and how quickly would this remodelling occur. In extreme cases, urgent reoperation may be needed for resection of muscle bundles from the right ventricular outflow tract, and augmentation of this area with a patch.

3.6 Long-term care
The main objectives, as for all transplantation, are the management of immunosuppression, and the surveillance and treatment of rejection and infection. The additional issue imposed by GUCH patients is related to the fact that these patients are usually younger than those from other aetiologies, and may still be relatively young when their transplanted organ fails from the effects of chronic rejection. Therefore, many of these patients may need re-transplantation. However, re-transplantation remains a difficult issue. This is partly because of the shortage of donor organs, and partly because the results of re-transplantation are significantly worse than those of first time transplantation, with only 50% of heart re-transplant recipients surviving 4 years [1].


   4. Outcome
Top
 Abstract
 1. Introduction
 2. GUCH patients requiring...
 3. The challenges of...
 4. Outcome
5. The availability of...
 6. Conclusion
 References
 
Data from the International Society of Heart and Lung Transplantation (ISHLT) shows that only 2% of heart transplants in adults in the past two decades have been performed for congenital heart disease [1]. This is in contrast to the paediatric population where about 40% of heart transplants have been for congenital heart disease [43]. The survival statistics show that 50% of GUCH heart transplant recipients survive 11 years. This is very similar to the survival statistics in the paediatric population and in adults with acquired heart disease.

Although these survival figures are encouraging, they are potentially misleading as there is a discrepancy between the early and late mortalities in the ISHLT data. In the ISHLT data, congenital heart disease is identified as one of the strongest risk factors for 1-year mortality after heart transplantation in adults [1]. In contrast, in those who survive 3 years, congenital heart disease has a marked 10-year survival advantage independently of age. This discrepancy may possibly be related to different underlying pathologies being associated with different mortality rates.

The individual published series of transplantation for GUCH describe variable outcomes but the overall pattern is in keeping with the ISHLT report in that the long-term survival is similar to that of heart transplantation for other aetiologies [6,8,44–48]. However, the underlying diagnoses in a large proportion of these patients were repaired or uncorrected defects with late ventricular dysfunction and no pulmonary vascular disease. For example, previous repair of tetralogy of Fallot, and Senning and Mustard procedures were common diagnoses, and these patients seem to do well. Less than half of the diagnoses were failure of palliation, and few of these were failing Fontans. Despite these low numbers, the raw data is suggestive that the percentage mortality may be higher amongst the failing Fontan cases. These findings are summarised in Table 1 .


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  		Table 1. Summary of the published series of heart transplantation for GUCH patients
 
If the above explanation for the discrepancy between early and late mortality is correct, then the overall survival statistics may change in the near future. This is because of the changing strategies in paediatric cardiology and cardiac surgery. For example, Senning and Mustard procedures have been largely superseded by the arterial switch operation. Also, the long-term deleterious effects of pulmonary regurgitation after repair of tetralogy of Fallot are being addressed. On the other hand, the Fontan procedure has become increasingly popular since the late 1980s. These changing patterns of clinical practice are likely to result in a decrease in the number of relatively low risk transplant recipients, with a concomitant increase in a higher risk (failing Fontan) group. This may reflect on future survival statistics. However, this speculation is based on very small numbers, and is certainly not universally agreed upon.

For heart–lung transplantation, the results are much less encouraging, even if organ availability permits this procedure to be carried out. The ISHLT data shows that a third of heart–lung transplants in adults as well as in the paediatric age group are done for congenital heart disease [2]. Overall 50% of adults survive 3 years. The results are slightly better in the subgroup of congenital heart disease with Eisenmenger syndrome where 50% of recipients survive 5 years [2,49]. In those with other forms of congenital heart disease, 50% survive 1 year. In view of these disappointing survival figures, heart–lung transplantation may no longer be considered a realistic option for GUCH patients.

For lung transplantation, the ISHLT data shows that the overall survival for all diagnoses in adults appears to be slightly better than those of heart–lung transplantation, with 58% survival at 3 years and 47% at 5 years [2]. However, only a very small proportion of these (1%) were done for congenital heart disease, and the outcome for this small sub-group was not specifically analysed. Such an analysis was done in a small series where 30 GUCH patients were treated with lung transplantation, and 31 with heart–lung transplantation [8]. Of the 30 lung transplant cases, 7 had previously undergone repair of their cardiac lesions, and 23 underwent concomitant intracardiac repair at the time of transplantation. The results for both heart–lung and lung transplantation were very similar to those for other diagnoses. Survival at 3 years was 60% for lung transplantation, and 47% for heart–lung transplantation.


   5. The availability of donor organs
Top
 Abstract
 1. Introduction
 2. GUCH patients requiring...
 3. The challenges of...
 4. Outcome
 5. The availability of...
6. Conclusion
 References
 
The demand for donor organs has always far exceeded their availability. This problem is likely to be aggravated by GUCH patients for several reasons. First, this is an expanding group of patients. Second, in view of their relatively young age, they may become candidates for re-transplantation. Third, these patients are very prone to pulmonary vascular disease and may need heart–lung transplantation. This creates difficulties partly because of the poor results of this procedure, and partly because it draws from an even smaller pool of donors. In fact, only two heart–lung transplants were performed in the UK last year for congenital heart disease.

The problem of organ shortage may be ameliorated to some extent in selected cases by the combination of lung transplantation and repair of cardiac defects. However, this approach only applies to relatively simple lesions and has its own unique postoperative problems, as described above.


   6. Conclusion
Top
 Abstract
 1. Introduction
 2. GUCH patients requiring...
 3. The challenges of...
 4. Outcome
 5. The availability of...
 6. Conclusion
References
 
Transplantation is an established treatment for adults with end-stage congenital heart disease. These patients are challenging at every level of clinical management. So far, the total number of transplants done in this context has been small, and the overall results comparable to transplantation for other aetiologies. However, both these features may change. The requirement for transplantation for congenital heart disease is likely to increase, since the majority of affected children now survive into adulthood and some may develop late ventricular dysfunction. At the same time, the outcome may deteriorate since the proportion of high-risk candidates is likely to increase. These changes in clinical patterns and survival must be closely monitored and reported as they have significant repercussions on decision making, patient counselling, and service implications.


   Footnotes
 
{star} Presented at the postgraduate course at the joint 19th Annual Meeting of the European Association for Cardio-thoracic Surgery and the 13th Annual Meeting of the European Society of Thoracic Surgeons, Barcelona, Spain, September 25–28, 2005.


   References
Top
 Abstract
 1. Introduction
 2. GUCH patients requiring...
 3. The challenges of...
 4. Outcome
 5. The availability of...
 6. Conclusion
 References
 

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