Lung Transplant in Children

0021-7557/02/78-Supl.2/S113 Jornal de Pediatria - Vol. 78, Supl.2, 2002 S113 Jornal de Pediatria Copyright © 2002 by Sociedade Brasileira de Pediatria REVIEW ARTICLE Lung transplant in children José J. Camargo*, Grupo de Transplante Pulmonar da Santa Casa de Porto Alegre, RS, Brazil Abstract Objective: this article presents a review of the main aspects related to lung transplant in children and shows the experience of the first medical team to perform this procedure in Latin America. Sources: literature review of scientific articles, using the Medline and Lilacs databases. Summary of the findings: the article was organized into topics. Similarities and differences regarding lung transplant in adults were identified. Specific problems presented by children subjected to transplant are discussed, and special emphasis is given to a specific situation: lung transplant with living related donors. Conclusions: advances in adult lung transplant for the treatment of parenchymatous or vascular lung diseases have been successfully employed in the pediatric population during the last few years. J Pediatr (Rio J) 2002;78(Suppl. 2):S113-S122: lung transplant, infancy, indications and results. Introduction The first lung transplants for the treatment of children cardiac disorders caused by pulmonary hypertension with lung parenchyma disorders or problems related to its decreased with the replacement of the diseased lungs, vessels were indeed cardiopulmonary transplants, following cardiopulmonary transplants became less frequent and were a current trend in the 1980s, which considered only recommended in those cases of pulmonary hypertension cardiopulmonary transplant to be more appropriate to treat associated with heart defects that could not be surgically patients with end-stage lung diseases that had or could have corrected and, sporadically, in cases of vascular lung disease some cardiac involvement. with left ventricle failure (usually with previous heart With the advent of unilateral and bilateral lung transplant, surgery). which is undoubtedly simpler, and the demonstration that Nowadays, bilateral lung transplant is the most widely used in the pediatric population, partly because cystic fibrosis is the most frequent indication for transplants in children, but also because there is some concern with graft * Professor of Thoracic Surgery, Fundação Faculdade Federal de Medicina de Porto Alegre. Coordinator, Transplant Center, Santa Casa de Porto growth and with occasional underfunction of the organs Alegre. donated by extremely small individuals.1 S113 S114 Jornal de Pediatria - Vol. 78, Supl.2, 2002 Lung transplant in children - Camargo JJ et alii Other indications for lung transplant include idiopathic Relative contraindications pulmonary fibrosis, primary pulmonary hypertension or Continued use of corticoid (> 0.5 mg/kg/day of pulmonary hypertension secondary to congenital heart prednisone) results in difficult bronchial healing and is a defects and an array of less frequent parenchyma diseases relative contraindication, since the dose of corticoid can (Figure 1). usually be reduced way below this limit. Many transplant centers regard the need for mechanical ventilation as a relative contraindication; however, the experience of centers specialized in transplants in children younger than six months shows that most of them were on mechanical ventilation and that this did not influence the success of the transplant, even in the presence of such cardiorespiratory dysfunction.3 The experience of this group revealed that the prompter availability of a donor, given the need for mechanical ventilation, had some impact on the survival rate. Therefore, the use of lobes from living donors has allowed saving the lives of children with remote chances of survival who were waiting for organs from dead donors.4 Life expectancy without the transplant is difficult to determine in the pediatric population, but in general, the inclusion of potential candidates in waiting lists follows reasonably safe rules, which vary according to the underlying disease. Cystic fibrosis In cystic fibrosis, the rehabilitation programs, especially CF: cystic fibrosis, BO: bronchiolitis obliterans, ILF: idiopathic lung fibrosis, based on continued physical therapy, adequate antibiotic PPH: primary pulmonary hypertension. therapy and proper nutrition, have increased the mean age of cystic fibrosis patients. However, the following criteria Figure 1 - Analysis of 200 consecutive cases of transplantation have been used to indicate the best time for lung transplant: at Cleveland Clinic and at Barnes Hospital, em Saint Louis – Postbronchodilator FEV1 of less than 20% predicted for adults, and less than 30% predicted for children and women at any age; – Resting hypoxemia (PaO2 of less than 55-60 mmHg); – hypercapnia (PaCO2 of less than 50 mmHg); – relevant secondary pulmonary hypertension (PMAP of less than 35 mmHg or PSAP of less than 45 mmHg); – hard-to-control weight loss, despite aggressive Indications management (nasoenteral tube, jejunostomy, etc.); The indications for lung transplant in children are often – repeated hospital admissions due to frequent and similar to those observed in adults2, including: untreatable infections, suggesting loss of control over – severe respiratory insufficiency; the disease. – lack of response to other forms of clinical or surgical Cystic fibrosis is the main indication for lung transplant treatments; in industrialized countries given the higher prevalence of – life expectancy shorter than two years, compatible with the disease among Anglo-Saxons. Some exclusion criteria the length of time on the waiting list; for potential candidates are not a common sense, such as colonization by Burkholderia cepacia, for which some – no other comorbidities (especially hepatic, renal and services refuse to recommend transplantation alleging high CNS); risk, whereas other specialized centers do not see any – psychological and emotional profile compatible with significant difference in outcome as a result of this agent. the requirements of a highly complex procedure; An identical situation occurs in relation to the colonization – adequate socioeconomic and family support conditions. by Aspergillus. Lung transplant in children - Camargo JJ et alii Jornal de Pediatria - Vol. 78, Supl.2, 2002 S115 In a different way, the involvement of other organs such – presence of hypoxemia, aggravated by exercise; as the pancreas and liver may determines an increase in – vital capacity of less than 60% predicted; postoperative risk and sometimes requires the transplantation – gradual deterioration, despite clinical treatment; of both lung and liver, an exception procedure that has thrived in several specialized centers.5 – signs of secondary pulmonary hypertension. An important aspect in the preparation for transplantation The growing need for oxygen supply and the gradual due to cystic fibrosis is the aggressive treatment against weight loss are important so that the severity of the situation malnutrition, often present at the moment of inclusion in the is known and transplantation can be recommended. waiting list. Malnutrition is one aggravating factor that adds In patients with fibrosis in which the lungs are metabolic difficulties to the losses of chronic infections and symmetrically affected (which is usually common), there is to the energy consumption that results from the enhanced a preference for left lung transplant due to the easy spatial respiratory effort. A follow-up with an experienced adaptation of the new organ, placed in a retracted pleural nutritionist, the prescription of diets extremely rich in cavity, to match the reduction in size of the fibrotic organ. proteins and calories and the use of more aggressive methods, Without opposition of the liver, left lung transplantation such as nasoenteral tube and gastrostomy or jejunostomy (if allows a fast descent of the hemidiaphragm, with total nasoenteral intubation is not possible), have been currently expansion of the newly implanted organ. used with the aim of eliminating this severe condition, The selection of the side is always based on the lowest which is reason enough for exclusion from the waiting list functional participation of a given lung, established on the or could result in failure if transplantation is carried out. initial assessment by means of perfusion scintigraphy (Figure Another relevant aspect is the effective previous control 2). of sinus disease, often expressed in the form of pansinusitis. As expected, the greatest difficulty in carrying out a The colonization by the same bacteria found in the lower transplant in children is to have a donor with compatible airways is inevitable in this population; however, patients size. Such difficulty is exacerbated in fibrosis, and is with retention of secretions that might be an entry point for characterized by increased chest retraction caused by pulmonary sepsis in the postoperative period are not allowed parenchyma disease, which allows the use of donors 20% to be in the waiting list. Large sinusotomies have locally smaller than the recipient. controlled infection, minimizing the risk of aspiration of Approximately one third of transplanted fibrotic patients gross pus by the upper airways. Sinusotomy, albeit use extracorporeal membrane oxygenation (ECMO). This considered a small surgery, requires general anesthesia and necessity is predictable because of previous pulmonary the patient usually has difficulty resuming respiratory hypertension

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