Laurent Viard

Double-lung transplantation in mechanically ventilated patients with cystic fibrosis☆

Many lung transplant programs consider ventilator dependence as a contraindication for transplantation. Among 54 patients in whom bilateral lung transplantations for cystic fibrosis were performed by the Joint Marseille-Montreal Lung Transplant Program, 10 were ventilator dependent. Three of them died in the early postoperative period (30%): 2 as a result of cerebral anoxia and sepsis, 1 of Pseudomonas cepacia pneumonia. Two patients died at 15 and 19 months after transplantation of obliterative bronchiolitis and secondary bacterial pneumonitis. Another 2 patients in whom obliterative bronchiolitis developed underwent retransplantation with a heart-lung block; 1 of those was operated on at 12 months and is well at 29 months after his initial transplantation; the second was operated on at 34 months and died of primary graft failure. Three other patients are alive and well at 3, 11, and 14 months after transplantation. Actuarial survival at 1 year was 70%. The postoperative course and the infectious and rejection complications were no different from those in patients who underwent transplantation while spontaneously breathing. Obliterative bronchiolitis developed in 66% of patients at risk (2 of 6 patients surviving more than 6 months). We conclude that transplantation in mechanically ventilated patients with cystic fibrosis is not associated with an increase in morbidity or mortality after bilateral lung transplantation. Long-term survival, as in patients who undergo transplantation while spontaneously breathing, is limited by the development of obliterative bronchiolitis.

Lung infections in pediatric lung transplantation: experience in 49 cases

OBJECTIVES Pulmonary infections, and particularly cytomegalovirus (CMV) infections, are a major cause of morbidity after lung transplantation. We report here our results in 49 pediatric lung transplantations. METHODS Between may 1988 and 1997, we have done 49 lung transplantations in 42 children (en bloc double lung transplantation (DLT):10, HLTx:7, sequential bilateral sequential-lung transplantation (BSLT):31, single-lung transplantation (SLT): 1). In seven, it was a retransplantation. Among these, 34 were cystic fibrosis (CF) patients, all with multiresistant organisms (Pseudomonas aeruginosa, Burkholderia cepacia, Achromobacter xylososydans, Staphylococcus aureus). All patients were treated with multiantibiotic prophylaxy adapted to the preoperative cultures. Donor-recipient CMV matching was possible in only 31 cases. CMV prophylaxy and immunosuppression protocols have evolved with time, with a current protocol of IV Gancyclovir prophylaxy for 3 months and triple drug immunosuppression without post-operative rabbit anti-thymocyte globulin (RATG) induction. There was no perioperative mortality in the primary transplantations and three early deaths in the whole group (6.1%). RESULTS Only five patients had no pulmonary infection. The patients presented 3.2 infection episodes per year, 75% localized on the lungs, 41% during the first 3 months. Among the 13 deaths in the 1st year, 10 were directly related to infection, 60% due to CMV. After the 1st year, in all patients dying of pulmonary dysfunction or obliterative bronchiolitis (OB), bacterial infections were associated. There was no serious fungal infection. Actuarial survival at 3 months, 1, 3, 5 years were 85, 65.7, 47.5 and 28.5%, respectively. There was a significant difference in 3 year survival between patients receiving CMV negative organs (40%) and CMV positive organs (17%). CONCLUSION In our experience, as in others, pulmonary infection risk is important in lung transplantation. Bacterial infections were mainly an aggravating factor of secondary pulmonary dysfunction or OB, and were not the primary cause of death. CMV infections have been very severe and lead us, despite the scarcity of donors, to avoid positive donors in negative recipients, this leads to disastrous mid-term results in our experience, despite prophylaxis.

Double-lung transplantation in children: A report of 20 cases

In the last 3 1/2 years, we have performed 20 double-lung transplantations in children between 7 and 16 years old (mean age, 13 years). One patient had primitive bronchiolitis obliterans and the other 19, cystic fibrosis. Eight patients were operated on in an emergency situation, 7 of them requiring ventilator support before transplantation. The procedures were en bloc double-lung transplantation in the first 11 patients with separate bronchial anastomoses in 10, and sequential bilateral lung transplantation in the later 9 patients. There were no operative deaths. Two patients died in the hospital on postoperative days 37 and 73, and there were four late deaths, which were due to infection, rejection, and bronchiolitis obliterans. The acceptable incidence of airway complications, the improvement in lung function of survivors, and the acceptable midterm survival make double-lung transplantation an acceptable alternative to heart-lung transplantation in children. However, in very small children, heart-lung transplantation may be preferable because of the size of the airway anastomoses at risk.

Successful treatment of fungal right atrial thrombosis complicating central venous catheterization in a critically ill child.

A 9-year-old boy was admitted to our pediatric intensive care unit after multiple trauma. On the 17th day post trauma, he developed catheter-related sepsis with candidemia. After removal of the catheter and 6 days of unsuccessful intravenous antifungal therapy, conventional and transesophageal two-dimensional echocardiography was performed revealing a large right atrial thrombus. Surgical thrombectomy under cardiopulmonary bypass was performed and the patient recovered within a few days. Fungal right atrial thrombus is a rare, lifethreatening complication of central venous catheterization. Two-dimensional echocardiography is a simple and effective diagnostic technique that should be performed when candidemia is detected. The proper therapeutic response depends on the findings of this examination. For a symptomatic patient with a large, mobile thrombus, we strongly recommend thrombectomy. Surgery not only allows removal of the mass and thus elimination of the mechanical complication but is also a key to management of infection.

Une cause rare de défaillance cardiaque brutale chez le nourrisson : la cardiomyopathie histiocytoïde

Histiocytoid cardiomyopathy is a rare disease which occurs predominantly in the first two years of life, with a female preponderance. We report the cases of two girls (11 and 15-month-old) which were respectively referred to our institution for ventricular tachycardia and ventricular fibrillation without prodroma. Etiologic findings only showed mild cardiomyopathy. Autopsy and histologic examination led to the diagnosis of histiocytoid cardiomyopathy. Furthermore, in the first observation, agenesis of the corpus callosum was found.

Lung transplantation in cystic fibrosis

Until 1984, cystic fibrosis (CF) was considered as a contra-indication to lung transplantation for the following reasons: 1) The importance of the bronchopulmonary sepsis with polyresistant organisms inducing an increased post-operative risk. 2) The persistence of the native trachea and infected sinuses with a potential for mid-term additional septic risk in immunosuppressed patients. 3) The systemic aspect of the disease, with continuous pancreatic and hepatic evolving disease. 4) The unknown risk of constitution of the disease on the transplanted lung.