PEDIATRIC LUNG TRANSPLANTATION - 10/09/11
Riassunto |
Advances in lung transplantation for adults with end-stage lung and pulmonary vascular disease have been applied with increasing success in the pediatric age group in recent years (Figure 1)16, 27, 38, 40 This article reviews the pediatric experience with lung transplantation, identifies the similarities to and differences from adult transplantation, discusses special problems related to children, and highlights issues specific to cystic fibrosis (CF).
The initial transplant operation used for children with end-stage lung and pulmonary vascular disease was heart–lung transplantation. Since the technique of isolated lung transplantation became successful,30 heart–lung transplantation has been reserved for children with irreparable congenital heart disease and pulmonary vascular disease, and the occasional association of pulmonary vascular disease with left ventricular failure (usually after previous cardiac repair). In recent years, the number of heart–lung transplants has decreased as the numbers of single and double lung transplants have increased.16 The most common pediatric transplant is a double lung (Table 1), in part because CF is the most common disease leading to transplant in children. Other reasons for double lung transplant include concern about subsequent allograft growth and the lack of competition for the remaining lung from very small donors. Other disease entities leading to transplantation are pulmonary fibrosis, pulmonary hypertension with or without congenital heart disease, and a variety of other lung diseases (Figure 2)16 Most transplants in infants and toddlers have been for pulmonary vascular disease or severe parenchymal lung disease (Table 2 and Table 3)4, 27, 38, 40
The indications for childhood transplant are similar to adults in the generic sense—progressive respiratory disability with an expected survival of less than the projected waiting time for cadaveric organs, exhaustion of other medical options, absence of other serious illness, documented ability to manage a complex medical regimen, access to adequate resources (personal, social, and financial), and willingness to accept the risk of transplantation. The prediction of survival can be difficult, especially for rare conditions. Disease-specific guidelines exist based on survival data analysis. For CF, the listing guidelines are a forced expiratory volume in 1 second less than 30% of predicted for children and females of any age, and less than 20% predicted for adult men. Other physiologic markers of a waning prognosis are an arterial partial pressure of carbon dioxide of 50 mm Hg or greater, and a room air arterial partial pressure of oxygen of 50 mm Hg or less. Clinical indicators include frequent pulmonary exacerbations leading to hospitalization or intravenous antibiotic therapy, poor exercise tolerance, poor nutrition despite aggressive intervention, pulmonary hypertension, and increasing microbial resistances.21
The guidelines for adults with pulmonary fibrosis include a forced vital capacity or total lung capacity (TLC) less than 60% of predicted, hypoxia of the same degree noted for CF, and poor exercise tolerance. The guidelines for pulmonary fibrosis and other interstitial lung diseases in children have not been validated. The parameters for primary pulmonary hypertension are systemic pulmonary artery pressures, elevated right atrial pressures (mean > 10 mm Hg), and a decreased cardiac index (<2.5 L/minute/m2).The guidelines for primary pulmonary hypertension (PPH) are difficult to apply to patients with elevated pulmonary artery pressures secondary to structural heart disease (repaired or unrepaired) because the survival and quality of life are better for the patients with that form of pulmonary vascular disease than PPH.6 For rare conditions and when children are too young to perform pulmonary function testing, other objective measures (e.g., chest radiograph, resting respiratory rate, serial blood gas measurements, oximetry) and clinical assessment of the trajectory of disease progression are required to estimate survival. Because the wait for donor lungs may be considerably shorter for children fewer than 2 years of age (median of fewer than 2 months in St. Louis) the timing of the listing process may be postponed.4
The contraindications to transplantation have been discussed in the article by Dr. Smith in this issue, and are similar to adults. Many, but not all, transplant centers consider the need for mechanical ventilation a relative contraindication. The experience in St. Louis with children fewer than 6 months of age shows that the majority required mechanical ventilation at the time of transplantation and that transplantation can be successful even with that degree of cardiorespiratory impairment.4 The shorter waiting time in that age group facilitates survival during the period of ventilation before transplantation. The availability of living donor transplantation has also made rapid transplantation available to very ill children who have little hope of surviving while awaiting cadaveric organs. Many pediatric transplant centers therefore perform transplantation on children requiring mechanical ventilatory support.4, 27, 38, 40
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| Address reprint requests to Paul C. Stillwell, MD, Cleveland Clinic Foundation, Pediatrics A120, 9500 Euclid Avenue, Cleveland, OH 44195 |
Vol 18 - N° 2
P. 405-414 - giugno 1997 Ritorno al numero
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- THE COST OF LUNG TRANSPLANTATION AND THE QUALITY OF LIFE POST-TRANSPLANT
- Cynthia R. Gross, Ganesh Raghu
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