**11. Transplant outcomes**

Significant improvements have been achieved in the past two decades with organ preservation, surgical techniques, critical care and immunosuppression. At present over 80% of patients receiving a lung transplant for end-stage lung disease are alive at 1 year and half of them are at 5 years (33). There are differences in the survival for different etiology for the underlying end-stage lung disease (Figure 1). Primary graft dysfunction is an important cause of post-operative mortality (33). The major cause of early and late mortality is infectious complications and bronchiolitis obliterans, a condition of progressive airflow obstruction associated with chronic airway fibrosis a pathologic finding known as bronchiolitis obliterans (BO). Majority of the late mortality is directly or indirectly due to the development of bronchiolitis obliterans syndrome or OB (Table 2) (34,35). Although the pathological mechanism that lead to BO is not well understood there have been many associations reported. A significant predictor of OB is prior acute rejection. Other conditions are primary graft dysfunction, gastroesophageal reflux or infections (36). Currently there are few well established therapies available for prevention or treatment of OB. Ongoing research continues to advance our understanding of the pathogenesis which may lead to effective treatment strategies in the future.

Viral prophylaxis is most commonly targeted against cytomegalovirus (CMV). Aggressive prophylactic therapy is directed towards this organism because of its high virulence and association with mortality in the lung transplant population. Lung transplant recipients with either donor or recipient serology that is positive for CMV usually receive prophylactic therapy with valganciclovir anywhere between three months to lifelong therapy. CMV negative lung transplant recipients who received a CMV positive donor lung may also receive CMV immunoglobulin in addition to their current valganciclovir therapy. Unfortunately, while valganciclovir prophylaxis decreases the incidence of CMV infection during the time of administration, prophylaxis does not completely prevent the development of CMV infection especially after prophylaxis therapy is discontinued. The optimal duration and type of therapy are still a matter of debate. Acyclovir and its derivatives are given to CMV negative lung transplant recipients who receive CMV

Fungal prophylaxis varies among the different transplant centers depending upon prior colonization, mechanical airway complications and environmental factors. In general some centers provide general fungal prophylaxis while others consider preemptive therapy depending upon surveillance bronchoscopy findings. Lung transplant recipients are at increased risk for developing *Aspergillus spp.* colonization of the airways leading to anastomotic infections and ulcerative tracheobronchitis. Itraconazole (or other azole substitutes) and inhaled amphotericin are the most common fungal prophylactic agents that are currently used. The azoles will increase the levels of the calcineurin inhibitors (cyclosporine and tacrolimus) so that the doses of these immunosuppressive medications should be decreased by at least 1/3 of their original dose. Calcineurin levels should be checked approximately one week after starting an azole. Of note, voriconazole and sirolimus should not be used together due to the

Significant improvements have been achieved in the past two decades with organ preservation, surgical techniques, critical care and immunosuppression. At present over 80% of patients receiving a lung transplant for end-stage lung disease are alive at 1 year and half of them are at 5 years (33). There are differences in the survival for different etiology for the underlying end-stage lung disease (Figure 1). Primary graft dysfunction is an important cause of post-operative mortality (33). The major cause of early and late mortality is infectious complications and bronchiolitis obliterans, a condition of progressive airflow obstruction associated with chronic airway fibrosis a pathologic finding known as bronchiolitis obliterans (BO). Majority of the late mortality is directly or indirectly due to the development of bronchiolitis obliterans syndrome or OB (Table 2) (34,35). Although the pathological mechanism that lead to BO is not well understood there have been many associations reported. A significant predictor of OB is prior acute rejection. Other conditions are primary graft dysfunction, gastroesophageal reflux or infections (36). Currently there are few well established therapies available for prevention or treatment of OB. Ongoing research continues to advance our understanding of the pathogenesis which may lead to

negative donors in order to prevent the development of Herpes infections.

significant rise in sirolimus levels.

effective treatment strategies in the future.

**11. Transplant outcomes** 

Fig. 1. Kaplan-Meier survival by diagnosis for adult lung transplants performed between January 1990 and June 2008. AT Def, α1-antitrypsin deficiency emphysema; CF, cystic fibrosis; COPD, chronic obstructive pulmonary disease; IPAH, idiopathic pulmonary arterial hypertension; IPF, idiopathic pulmonary fibrosis. J Heart Lung Transplant. 10: 1083-1141, 2010


Table 2. Causes of Death Following Lung Transplantation in Adult Recipients. J Heart Lung Transplant. 10: 1083-1141, 2010

#### **12. Conclusion**

Lung transplant remains an effective treatment for selected patients with end-stage lung disease. The major rate limiting step for lung transplantation at present is the available donor organs. Chronic allograft dysfunction remains a major source of morbidity and mortality after lung transplantation. Investigations into improving donor lung availability, preventive and therapeutic approaches for OB and alternative for transplantation for end-

Lung Transplantation 307

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**13. References** 


**17** 

**Systematic Review of the Literature:** 

**Atresia with Tracheo-Esophageal** 

 *VU Medical Centre & Emma's children hospital of the AMC,* 

 **Fistula (EA-TEF)** 

*Pediatric Surgical Centre of Amsterdam,* 

M. W. N. Oomen

*The Netherlands* 

**Comparison of Open and Minimal Access** 

**Surgery (Thoracoscopic Repair) of Esophageal** 

Surgical correction of esophageal atresia with tracheo-esophageal fistula (EA-TEF) has been performed since 1943 (Cameron Haight) via postero-lateral thoracotomy using an extrapleural approach in most cases. This procedure can be considered as the standard treatment of EA. The pitfalls of the operation, the incidence of complications and the outcomes, both short term and long term, have been analysed and reported by many pediatric surgeons

Since 1999, minimal access surgery (MAS) has been practised for the correction of EA 1. The risk of complications and short term outcomes have been reported as equal to the open approach. MAS has been advocated because of a possibly reduced risk of impairment of shoulder function, and a possible reduction in occurrence of postoperative scoliosis. Next to

Several advantages and disadvantages of both procedures have been described. The open approach is well standardized and is resorted to in difficult cases. Disadvantages of the open approach are the presence of a scar, possible chest wall deformities and rib fusion. The occurrence of scoliosis and possible shoulder function impairment has been related to the

The thoracoscopic approach has the advantage of magnification of view. Next to that the chance on a postoperative scoliosis and impaired shoulder function may be reduced due to the small incisions which also might lead to better cosmesis. Technically, the thoracoscopic approach is more demanding than the open approach which has consequences for training

So far, it seems that there is no difference between open and MAS approach in the frequency

In 2005, Holcomb et al presented their results of MAS (thoracoscopic) EA-TEF repair in 104 patients in multiple centres. This landmark paper has been extensively discussed by leaders

of anastomotic leakages, strictures, recurrent fistulas, tracheomalacie or GERD.

that, it has been postulated the MAS repair might lead to a better cosmesis.

**1. Introduction** 

around the world.

open approach as well.

and education.

