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Journal of the Royal Society of Medicine Supplement No. 18 Volume 84 1991
Heart-lung transplantation for cystic fibrosis
T W Higenbottam MD FlCP
B Whitehead MB Bs MRCP Papworth Hospital, Papworth Everard, Cambridge CB3 8RE and Hospital for Sick Children, Great Ormond Street, London WCIN 3JH
Keywords: cystic fibrosis; heart-lung transplantation
Introduction It is now just over 5 years since the first successful heart-lung transplant (HLT) for cystic fibrosis (CF) was performed in the UK'. At Papworth some 30 CF patients have now undergone this form of surgery, 14 children have received HLT at the Hospital for Sick Children, Great Ormond Street, and 45 patients at the Harefield and Brompton hospitals. Estimates from North America suggest that 27 operations have been performed and a similar number in the rest of Europe. We are now in a position to appraise rationally the value, the problems and potential of HLT as a treatment for patients who have developed respiratory failure from CF. What operation: heart-lung or double lung traplant? For a range of end-stage cardiopulmonary diseases HLT provides a means of extending- survival and improving quality of life2. For 95 adult IHLT patients our current actuarial survival is 78% at one year and 65% at two years. Many of the technical problems of surgery have been overcome. In particular the problem ofchest wall bleeding which has contributed to fatalities in CF patients after HLT3 has been lessened by the peri-operative use of aprotinin or trasylol4. The use of the heart-lung block from a suitable donor for a patient with CF precludes the use of the heart for cardiac transplantation. Single lung transplantation whilst suitable for a wide range of disease cannot be used in CF due to the risks of cross infection from the remaining infected lung to the transplant lung. As a result double lung transplantation without the heart has been explored5. The initial problems were of poor tracheal healing which appears to be a feature of the absence of mediastinal collateral blood supply to the carina which, whilst present in the heart-lung block6 is not present in the double lung. However, use of bilateral bronchial anastomoses with sequential single lung transplantation has overcome this problem by retaining the recipient's own lower tracheal blood supply7.
An alternative approach in the UK, pioneered by Professor Yacoub is the 'domino' operation. Here the CF patient's heart is donated to a cardiac donor, the CF patient receiving the heart-lung block. At Papworth, 28 domino operations have been performed, and 17 were from CF patients. There appears to be no deleterious effects on outcome of either the HLT patient or the heart transplant recipient from the domino operation. The possible occurrence of occlusive coronary vascular disease in the heart of the HLT patients,
which is common in heart transplant recipients8, or the long-term function of the double lung transplant remain concerns about these two surgical techniques. Long-term follow-up will hopefully provide the answers.
Which patient should receive a heart-lung transplant Initially there were a number of contra-indications to this form of surgery in CF patients3. Insulindependent diabetics were excluded but as many HLT patients developed temporary insulin-dependent diabetes during periods of augmented immunosuppression with steroids and had come to no harm this contra-indication was dropped. For similar reasons other contra-indications are no longer considered important, for example long-term but low dose oral steroids, enzyme abnormalities suggesting liver disease and aspergillus infections of the lung. However, mycetoma, because of inability to exclude pleural involvement, remains a contra-indication. Similarly although previous thoracotomy is no longer a contra-indication a previous pleurectomy or pleurodesis remains a contra-indication. The selection of a patient for HLT depends on judging that the patient's chances of survival are limited but not too short to preclude the finding of a suitable donor. In keeping with other chronic lung disease we have chosen as indicators of poor survival chance in CF values ofFEV < 25% predicted and the presence of hypercapnia with respiratory failure3. Recently we have been able to study a number of measurements of pulmonary function and fitness in the 64 CF patients accepted at Papworth for HLT for Table 1. Predictions of survival in non-transplanted CF patients (n=64)
P value
FEVy (% pred) 12 min distance walked Minimum sat. 0
0.02 0.2 0.001
Blood gases Paco2
0.03
[H+l
Pao2 Sat 02 % Weight/height ratio
0.7 0.10
0.10 0.16
Predictors of survival from measurements made at the time of assessment for HLT in the 64 patients who were accepted for surgery. Only FEV, (% pred) and Paco2 together with the minimum oxygen saturation during the 12 min walk test were of value. Weight/height ratio was not, nor was exercise tolerance as measured by 12 min walking distance
Journal of the Royal Society of Medicine Supplement No. 18 Volume 84 1991 Table 2. Serious pulmonary infection (PI) and acute rejection (AR) in CF and non CF patients (n=52)
1.00 4 S.
0@_m 0.75 -
S.
me.e
@0
*S..
CF
PPH
ES
P value
0.35 0.92
0.36 0.91
NS NS
0.19 0.24
0.06 0.14
Journal of the Royal Society of Medicine Supplement No. 18 Volume 84 1991
Heart-lung transplantation for cystic fibrosis
T W Higenbottam MD FlCP
B Whitehead MB Bs MRCP Papworth Hospital, Papworth Everard, Cambridge CB3 8RE and Hospital for Sick Children, Great Ormond Street, London WCIN 3JH
Keywords: cystic fibrosis; heart-lung transplantation
Introduction It is now just over 5 years since the first successful heart-lung transplant (HLT) for cystic fibrosis (CF) was performed in the UK'. At Papworth some 30 CF patients have now undergone this form of surgery, 14 children have received HLT at the Hospital for Sick Children, Great Ormond Street, and 45 patients at the Harefield and Brompton hospitals. Estimates from North America suggest that 27 operations have been performed and a similar number in the rest of Europe. We are now in a position to appraise rationally the value, the problems and potential of HLT as a treatment for patients who have developed respiratory failure from CF. What operation: heart-lung or double lung traplant? For a range of end-stage cardiopulmonary diseases HLT provides a means of extending- survival and improving quality of life2. For 95 adult IHLT patients our current actuarial survival is 78% at one year and 65% at two years. Many of the technical problems of surgery have been overcome. In particular the problem ofchest wall bleeding which has contributed to fatalities in CF patients after HLT3 has been lessened by the peri-operative use of aprotinin or trasylol4. The use of the heart-lung block from a suitable donor for a patient with CF precludes the use of the heart for cardiac transplantation. Single lung transplantation whilst suitable for a wide range of disease cannot be used in CF due to the risks of cross infection from the remaining infected lung to the transplant lung. As a result double lung transplantation without the heart has been explored5. The initial problems were of poor tracheal healing which appears to be a feature of the absence of mediastinal collateral blood supply to the carina which, whilst present in the heart-lung block6 is not present in the double lung. However, use of bilateral bronchial anastomoses with sequential single lung transplantation has overcome this problem by retaining the recipient's own lower tracheal blood supply7.
An alternative approach in the UK, pioneered by Professor Yacoub is the 'domino' operation. Here the CF patient's heart is donated to a cardiac donor, the CF patient receiving the heart-lung block. At Papworth, 28 domino operations have been performed, and 17 were from CF patients. There appears to be no deleterious effects on outcome of either the HLT patient or the heart transplant recipient from the domino operation. The possible occurrence of occlusive coronary vascular disease in the heart of the HLT patients,
which is common in heart transplant recipients8, or the long-term function of the double lung transplant remain concerns about these two surgical techniques. Long-term follow-up will hopefully provide the answers.
Which patient should receive a heart-lung transplant Initially there were a number of contra-indications to this form of surgery in CF patients3. Insulindependent diabetics were excluded but as many HLT patients developed temporary insulin-dependent diabetes during periods of augmented immunosuppression with steroids and had come to no harm this contra-indication was dropped. For similar reasons other contra-indications are no longer considered important, for example long-term but low dose oral steroids, enzyme abnormalities suggesting liver disease and aspergillus infections of the lung. However, mycetoma, because of inability to exclude pleural involvement, remains a contra-indication. Similarly although previous thoracotomy is no longer a contra-indication a previous pleurectomy or pleurodesis remains a contra-indication. The selection of a patient for HLT depends on judging that the patient's chances of survival are limited but not too short to preclude the finding of a suitable donor. In keeping with other chronic lung disease we have chosen as indicators of poor survival chance in CF values ofFEV < 25% predicted and the presence of hypercapnia with respiratory failure3. Recently we have been able to study a number of measurements of pulmonary function and fitness in the 64 CF patients accepted at Papworth for HLT for Table 1. Predictions of survival in non-transplanted CF patients (n=64)
P value
FEVy (% pred) 12 min distance walked Minimum sat. 0
0.02 0.2 0.001
Blood gases Paco2
0.03
[H+l
Pao2 Sat 02 % Weight/height ratio
0.7 0.10
0.10 0.16
Predictors of survival from measurements made at the time of assessment for HLT in the 64 patients who were accepted for surgery. Only FEV, (% pred) and Paco2 together with the minimum oxygen saturation during the 12 min walk test were of value. Weight/height ratio was not, nor was exercise tolerance as measured by 12 min walking distance
Journal of the Royal Society of Medicine Supplement No. 18 Volume 84 1991 Table 2. Serious pulmonary infection (PI) and acute rejection (AR) in CF and non CF patients (n=52)
1.00 4 S.
0@_m 0.75 -
S.
me.e
@0
*S..
CF
PPH
ES
P value
0.35 0.92
0.36 0.91
NS NS
0.19 0.24
0.06 0.14
