Heart-lung-liver transplantation.

We report three patients with cystic fibrosis and one patient with primary biliary cirrhosis and plexogenic pulmonary hypertension who have undergone heart-lung-liver transplantation as a combined procedure. Liver transplantation was necessary in the three patients with cystic fibrosis because of portal hypertension secondary to either hepatic fibrosis or established cirrhosis in addition to their advanced lung disease. Three of the four patients were alive at 20, 50, and 100 months after transplantation (one patient with cystic fibrosis died on day 16 of pneumonia) with well-preserved pulmonary function (forced expiratory volume in 1 second 110%, 49%, and 100% predicted, respectively), normal hepatic function and New York Heart Association class 1 performance status. Heart-lung and concurrent liver transplantation is a feasible and successful procedure with a satisfactory long-term outcome in selected patients with advanced pulmonary and hepatic disease.

Airway complications after lung transplantation: treatment and long-term outcome.

BACKGROUND: Airway complications are a significant cause of morbidity after lung transplantation. Effective treatment reduces the impact of these complications. METHODS: Data from 123 lung (99 single, 24 bilateral) transplants were reviewed. Potential risk factors for airway complications were analyzed. Stenoses were treated with expanding metal (Gianturco) stents. RESULTS: Mean follow-up was 749 days. Thirty-five complications developed in 28 recipients (complication rate: 23.8%/anastomosis). Mean time to diagnosis was 47 days. Only Aspergillus infection and airway necrosis were significantly associated with development of complications (p < 0.00001 and p < 0.03, respectively). Stenosis was diagnosed an average of 42 days posttransplant. Average decline in forced expiratory volume in 1 second (FEV1) was 39%. Eighteen patients (13 single and 5 bilateral) required stent insertion. Mean increase in FEV1 poststenting was 87%. Two stent patients died from infectious complications. Six patients required further intervention. Long-term survival and FEV1 did not differ from nonstented patients. CONCLUSIONS: Aspergillus and airway necrosis are associated with the development of airway complications. Expanding metal stents are an effective long-term treatment.

Heart-lung transplantation for Eisenmenger syndrome: early and long-term results.

BACKGROUND: Heart-lung transplantation (HLT) for Eisenmenger syndrome (ES) provides superior early and intermediate survival when compared with other forms of transplantation. The early risk factors and long-term outcome of HLT for ES are less well defined. METHODS: We analyzed 263 patients who had undergone HLT at our institution during more than 15 years. Fifty-one consecutive patients with ES who underwent HLT, 33 (65%) of which had simple anatomy, were compared with 212 cases having HLT for other indications (non-ES). RESULTS: Female sex and previous thoracotomy were more prevalent in the ES group. Patients with ES had greater postoperative blood loss and returned more frequently to the operating room for control of bleeding. There were 8 (16%) early deaths in the ES group compared with 27 (13%) in non-ES (p = 0.65). One-, 5-, and 10-year survival rates for ES were 72.6%, 51.3%, and 27.6%, respectively, compared with non-ES of 74.1%, 48.1%, and 26.0%, respectively, and there was no difference in survival overall (p = 0.54). Among ES patients, previous thoracotomy was a risk factor for hospital death. A subgroup analysis based on simple versus complex type of ES did not show statistically significant differences in terms of postoperative course or early or late survival. CONCLUSIONS: Heart-lung transplantation is a successful procedure for ES. Despite a greater frequency of risk factors and a more difficult operative course, early and late outcome with HLT is comparable to non-ES recipients.

Resolution of severe ischemia-reperfusion injury post-lung transplantation after administration of endobronchial surfactant.

BACKGROUND: Ischemia-reperfusion injury (IRI) is a prominent cause of primary graft failure after lung transplantation and is associated with an altered surfactant profile. Experimental animal studies have found that replacement with exogenous surfactant administered via fiber-optic bronchoscopy (FOB) enhanced recovery from IRI with improved pulmonary compliance and gas exchange after lung transplantation. We report our clinical experience with FOB instillation of surfactant in severe IRI after human lung transplantation. METHODS: This study is a retrospective review of 106 consecutive lung or heart-lung transplants performed at a single institution. Severe IRI was defined as diffuse roentgenographic alveolar infiltrates, worsening hypoxemia and decreased lung compliance within 72 hours of lung transplantation. One vial of surfactant (20 mg/ml phospholipid) was instilled into each segmental bronchus upon diagnosis of IRI. RESULTS: Six patients (5 bilateral sequential and 1 re-do heart-lung transplant), mean age 46 years, were diagnosed with IRI and surfactant was administered at a mean of 37 hours (range 2.3 to 98) post-transplant. Mean graft ischemia time was 376 minutes (range 187 to 625) and cardiopulmonary bypass time 174 minutes (range 0 to 210). Mean Pao(2) [mm Hg]/Fio(2) ratio before and 48 hours after surfactant instillation was 70 and 223, respectively. Significant resolution of radiologic infiltrates was evident in all cases within 24 hours. Successful extubation occurred at a mean of 13.5 days and survival is presently 100% at 19 months (range 3 to 54). CONCLUSIONS: Bronchoscopic instillation of surfactant improves oxygenation and prognosis after severe IRI in lung transplant recipients. It represents a cost-effective, relatively non-invasive therapeutic alternative to extracorporeal membrane oxygenation.

Endobronchial nocardial infection.

The rare presentation of nocardial infection as an endobronchial "tumour" is reported. Haematogenous dissemination occurred after fibreoptic bronchoscopy and biopsy, a phenomenon not previously described in nocardial infections. This case highlights the difficulties in diagnosing pulmonary nocardial infection and the potential for invasive procedures to disseminate the disease.

Amiodarone pulmonary toxicity--three unusual manifestations.

Amiodarone is very useful treatment for refractory arrhythmias. However, it has a wide profile of adverse effects involving a number of organ systems. Pulmonary toxicity is the most serious of these side effects and often limits its clinical use. Three patients with unusual forms of amiodarone pulmonary toxicity are described. One had extensive unilateral alveolar disease, another pleural effusion, and in a third the pulmonary infiltrate improved with oral corticosteroids despite continuation of amiodarone therapy. Extensive unilateral alveolar disease and resolution of the pulmonary toxicity with corticosteroids despite continuing with amiodarone have not been previously reported. Pleural effusion has been noted only rarely. With the increasing use of amiodarone, pulmonary toxicity is likely to occur more frequently and it is important to be aware of its less common manifestations.

Gorham's syndrome: a usually fatal cause of pleural effusion treated successfully with radiotherapy.

A 21 year old man presented with a right sided pleural effusion. Destruction of the 11th and 12th right ribs and adjacent vertebral bodies was noted on computed tomographic scanning. An open rib biopsy revealed the histopathological changes of Gorham's syndrome. In view of the progressive vertebral destruction and inevitable spinal cord compromise, he was treated with high dose radiotherapy. The process was arrested and he remains well with no signs of recurrence after four years. Pleural effusion and vertebral destruction complicating Gorham's syndrome carry a poor prognosis but, in this case, high dose radiotherapy has been effective in controlling both the effusion and the progressive bony destruction.

Asphyxia while swallowing solid food caused by bronchial compression: a variant of the pneumonectomy syndrome.

A 40 year old man presented with recurrent episodes of dyspnoea whilst swallowing solid food. He had undergone right pneumonectomy and thoracoplasty for recurrent pneumonias and empyema 23 years previously. Solid food boluses appeared to cause bronchial obstruction by compressing the surgically distorted left main bronchus. This is a new variant of the post pneumonectomy syndrome.

Combined transplantation of the heart, lung, and liver.

Combined transplantation of the heart, lung, and liver may be indicated in patients with either end-stage respiratory failure complicated by advanced liver disease or end-stage liver failure complicated by advanced lung disease. A retrospective review of nine patients who underwent combined heart-lung-liver transplantation in Cambridge (1986-99) was carried out. The 1-year and 5-year actuarial survival was 56% and 42%, respectively. Combined heart-lung-liver transplantation is a feasible option for a few patients and has a 5-year survival similar to heart-lung transplantation but with a lower incidence of acute and chronic rejection.