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Heart-lung transplantation in a child with Cystic Fibrosis: first case in Argentina
Burgos Claudio; Siccardi María Alejandra; Mugianessi Oscar; Lemos Cecilia
Cardiovascular Service, Italian Hospital
Material and Methods
Introduction: Cystic Fibrosis (CF) is a genetic disease common in white people. It affects 1 per 2000 to 3000 live birth and is autosomic recessive transmitted. It's associated with generalized epithelial dysfunction. In respiratory tract more viscous secretion produces progressive airway obstruction with recurrent infections, respiratory insufficiency and parenchyma lung destruction. According to CF Foundation the mean of survival in these patients is 28 years and the first cause of death is respiratory failure. With actual medical cares, news antibiotics and gene therapy in investigation, heart-lung transplantation is in the last years an important option in several centers in the world.Top
Objectives: We report the first heart-lung transplantation in a pediatric patient with CF in our country.
Material and Methods: Patient male, 5 and half years old, Caucasian. The diagnosis of CF was made at 5º months of live. His disease evolution was characterized for frequents reactivation in pneumonia form with frequents admissions at hospital. He had Staphylococcus aureus MR and Pseudomona aeruginosa colonization. Two years before he presented congestive heart failure, desaturation and very important physical activity limitation, requiring O2 supply at home. In the first visit patient was regular, weight- height index 88%, pO2 68, pCO2 37 and saturation 93%. Catheterism informed pulmonary vascular hypertension, disease type B (severe media hypertrophy) with detriment of both ventricular function. Radioisotope imaging showed multiples defects in ventilation/ perfusion relation like chronic obstructive pulmonary disease. CMV serology was positive and cross-match 0%. After 8 months in waiting list the transplantation was performed with a 5 years old donor with ABO and 2 HLA, DrDQ, similarities. Physical compatibility of 95 %. Receptor and donor wherein same city but different hospitals. Multiorganic ablation was made and isquemic time was 180 minutes. Saint-Thomas cardioplegic solution was used for myocardial protection and modified Euro-Collins solution and Dextran 40 for lung preservation. Carefully dissection was performed for potential contamination. Tracheal suture was made without telescopage, with PDS (Ethicon) 5/0 and 6/0 in cartilaginous tracheal part and membranous part, respectively. Monophylament was used in vascular and atrial sutures. Posterior mediastine was carefully cover with Argon laser and biological adhesives (Beriplast) and tracheal suture was reinforced with autologous pericardio. Exhaustive hemostasy control with Aprotinine and platelets. Pleural and mediastinal drainage was placed. Cross Clamp Time was 82 minutes. Cardiopulmonary by-pass time was 148 minutes. Saturation was 99% and we didn't observe reperfusion injury. Intraoperative bronchoscopy showed an excellent trachea- tracheal adaptation. The total time of surgery was 4 Hs.
Results: Early postoperative period was successful, with extubation day 2. Inothropic support was administrated first 48 Hs. and negative fluid balance was maintained. Teycoplanine, Ceftazidime and Fluconazol were initiated based in previous cultures. Immunosupression was started with Azathioprine 2 mg/kg (day 0), intraoperatively methylprednisolone 30 mg/kg and postoperatively for 24 Hs 10 mg/kg. Oral steroid therapy was continued with prednisone tapering doses until 0,5 mg/kg. FK 506 was added the second day by gastrostomy 0,25 mg /kg, maintaining blood levels between 10 to 15 ng/ml. Amphotericin B aerosolized, Aciclovir and Trimetoprim- sulfamethoxazole (TMS) were used for prophylaxis. The 11º day after transplantation first myocardial biopsy was performed and presented rejection 1 "a". Patient left the hospital the day 24º. Leucopenia and neutropenia with CMV reactivation appeared in the 5º week after transplantation ( CMV buffy-coat was positive). Ganciclovir and CMV hyperimmune globulin were started and Azathioprine and TMS stopped. At the same time N. Carinii pneumonia was confirmed by broncho alveolar lavage (BAL) and was successfully treated with TMS IV. Ganciclovir oral prophylaxis was manteined for 4 months and 3 negatives buffy-coat were confirmed. Two pulmonary infections (bacterial and fungal) in the second six months after transplantation, were diagnosed by BAL and specifically treated with excellent results. At present patient is healthy, weight- height index 108%, pO2 97, pCO2 32, saturation 98%. Liver and renal functions are normal. Triple regimen Immunosupression is maintained with FK 506, Azathiprine and Prednisone. Prophylaxis with TMS was established He goes to elementary school normally.
Discussion: We report the first pediatric heart-lung transplantation in a child with CF in our country. The patient has previous natural disease history like others with CF. Life expectancy is very short when respiratory failure is established and some centers suggest performing the transplant before FEV1 falls bellow 20% predicted and while pCO2 remains normal. In our case pCO2 was normal and we couldn't measure VEF1. Nutritional status was acceptable. The presence of weight deficit below 20% is a bad prognosis factor for survival. Liver compromise is present in some CF patients. Our patient had normal liver function, type A Child criteria, and it tends to provide better survival. Donor selection, organ preservation and operative technique have a direct relation with a successful early postoperative period. Less bleeding has a direct relation with intraoperative use of Aprotinine. CF patients are colonized with SA MR and Pseudomonas and antibiotic therapy must be based in previous cultures and antibiograms. It has been reported that Amphotericin B and Aciclovir prophylaxis for fungal (Aspergillus, Candida) and viral infections, respectively, are effective and with a few adverse effects. In our service we had not documented N. Carinii infection in transplant recipients, but we initiated TMS prophylaxis with strict renal and hematologyc control. We would have to discontinue TMS for neutropenia and we suspect that this factor and lower immunosupression doses caused N. Carinii infection. CMV infection is very common in positive receptors. We did not t use prophylaxis but early diagnosis with antigenemia lead us to begin with therapy with Ganciclovir IV and CMV hyperimmune globulin as quickly as possible, and results were excellent. About Immunosupression we use similar protocols to other major centers. We chose FK 506 because it's more powerful, better than ciclosporine in early post transplantation period, presents better absorption in CF patients and it's rescue therapy in obliterative brochiolitis syndrome (OB). OB is anatomopathologic manifestation for airway chronic rejection of the lung allograft. The risk factors for OB include greater number of episodes of rejection in the first 6 months after transplantation, pulmonary infection, CMV and N.Carinii specially, and individual recipient compliance. Our case has presented only one acute rejection episode. Pulmonary infections, bacterial, viral and fungal, are very common and have longer evolution in these patients, requiring invasive diagnosis by broncho alveolar lavage. About adverse effects of Fk, sometimes drug should be discontinued and switched to ciclosporine. . In this case it has not happened at the moment. We have noted a very important improvement in nutritional status, and it has been shown to be an indicator of survival.
Conclusions: Heart- lung transplantation for end-stage respiratory disease in patients with CF provides improvement in quality of life. There's not significant difference in evolution and infection incidence whit non CF patients. Survival estimates at 1 and 3 years are 80% and 65% respectively. The transplantation improves life expectancy in CF patients.
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