Pulmonary fibrosis lung transplantation

Nunley DR, Ohori P, Grgurich WF, lacono AT, Williams PA, Keenan RJ, Dauber JH (1998) Pulmonary aspergillosis in cystic fibrosis lung transplant recipients. Chest 114 1321-1329... 

The first combined heart-lung transplant was performed in 1981 at Stanford University. Combined heart-lung donors need to satisfy both the requirements already described separately. Combined heart-lung transplant is recommended in patients with congenital problems affecting these organs, pulmonary hypertension and/or cystic fibrosis. The recipients for the combined transplant are recommended to be less than 55 years old. Survival rates are 79, 66, and 54% at 1 month, 1 year and 3 years, respectively, after transplantation. 

A 51-year-old man underwent double lung transplantation for pulmonary fibrosis, accidentally received an infusion of ciclosporin 30 mg/hour instead of 3 mg/hour, and 3 hours later had bilateral reactive mydriasis and absence of tendon reflexes. A CT brain scan showed diffuse cerebral edema, and massive intracranial hjrpertension rapidly developed. He died 5 hours later from brainstem compression, and pathological examination showed diffuse cerebral edema with neuronal necrosis. 

Thabut G, Mai H et al. (2003) Survival benefit of lung transplantation for patients with idiopathic pulmonary fibrosis. J Thorac Cardiovasc Surg 126 469-475... 

Sweet MP, Patti MG, Leard LE, et al. Gastroesophageal reflux in patients with idiopathic pulmonary fibrosis referred for lung transplantation. J Thorac Caidiovasc... 

Venuta F, Rendina EA, Ciriaco P, et al. Efficacy of cyclosporine to reduce steroids in patients with idiopathic pulmonary fibrosis before lung transplantation. J Heart Lung Transplant 1993 12 909 914. 

Mogulkoc N, Brutsche MH, Bishop PW, et al. Pulmonary function in idiopathic pulmonary fibrosis and referral for lung transplantation. Am J Respir Crit Care Med 2001 164(1) 103-108. 

Meyer DM, Edwards LB, Torres F, et al. Impact of recipient age and procedure type on survival after lung transplantation for pulmonary fibrosis. Ann Thorac Surg 2005 79(3) 950-957 discussion 7-8. 

Whelan TP, Dunitz JM, Kelly RF, et al. Effect of preoperative pulmonary artery pressure on early survival after lung transplantation for idiopathic pulmonary fibrosis. J Heart Lung Transplant 2005 24(9) 1269-1274. 

Despite case reports of response to treatment with corticosteroids, larger studies have not shown any outcome benefit. Currently, accepted treatment options are limited to supportive care and prevention and treatment of infection. There is a report of three BMT recipients with IPS whose lung function improved following etanercept administration (13). Lung transplant may offer a therapeutic option for selected patients. Although the pneumonitis resolves in about 31%, the clinical course of IPS is often comphcated by viral and fungal infections, pneumothorax, pneumomediastinum, subcutaneous emphysema, pulmonary fibrosis, and autoimmune polyserositis (5). The case fatality of IPS is... 

Fig 2.2.9a-d. Bronchiolitis obliterans and pulmonary fibrosis. A 42-year-old man with a history of heart/lung transplantation for Eisenmenger syndrome 10 years ago, now with increasing shortness of breath, a, b Chest CT shows changes of end-stage bronchiolitis obliterans and pulmonary fibrosis secondary to chronic lung rejection. C, d Note typical anatomic configuration of heart transplantation performed with the atrial-atrial method. There is an increased space between the donor aorta and the recipient superior vena cava (arrow). Note the remnant of the donor superior vena cava (arrowhead)... 

In 1983 the Toronto Lung Transplant Group (1986) performed the first single-lung transplantations for patients with end-stage chronic obstructive pulmonary disease and advanced pulmonary fibrosis. Their technique was later expanded to bilateral sequential single-lung transplantation for patients with bronchiectasis and cystic fibrosis. 

Bronchogenic carcinoma develops in the native lung of transplant recipients with emphysema and pulmonary fibrosis at frequencies of 2% and 4%, respectively. The carcinomas most commonly manifest as a pulmonary nodule or mass on chest radiographs, with more nodules seen on CT scans (Collins et al. 2002). This rate is similar to that in other high-risk populations (e.g. elderly smokers with emphysema or other chronic lung disease). The majority of cancers are associated with a poor prognosis. The most common imaging manifestations are a solitary pulmonary nodule or mass. 

Toronto Lung Transplantation Group (1986) Unilateral lung transplantation for pulmonary fibrosis. N Engl J Med 314 1140-1145... 

Since the early experimental success with gene therapy in the 1990s, the clinical use of gene therapy has been proven to be difficult. Phase I clinical trials for pulmonary diseases have focused primarily on cystic fibrosis and more recently on lung cancer (109,110). Although gene therapy for lung transplantation has seen increased experimental success, no clinical trials have been conducted. 

Pneumocystis pneumonia occurs in patients with congenital or acquired immunodeficiency, for instance transplant patients and others who are receiving immunosuppressive therapy. Not infrequently, Pneumocystis pneumonia coexists with other pulmonary infections. The infection may be diffuse or focal, with a propensity to affect the lower lobes. In contrast with several other forms of pneumonia, there is no fibrinous pleurisy. The alveolar septa are increased in thickness and infiltrated with plasma cells and lymphocytes. The characteristic feature is the intra-alveolar exudate. This is foamy and strongly periodic acid-Schiff (PAS) positive, being composed of parasitic cysts. An intra-alveolar eellular reaction to the exudate is absent, and a few areas of hyaline membrane formation may be found. The diagnosis may be obtained by lung biopsy material. The organism may also be seen in sputum. In some instances, diffuse interstitial fibrosis may be the end result. 

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