Pulmonary hypertension is associated with higher mortality in cystic fibrosis patients awaiting lung transplantation.

AIM: Pulmonary hypertension (PH) is frequently found in patients with advanced parenchymal lung diseases. In advanced stages, cystic fibrosis (CF) patients can develop PH and eventually cor pulmonale. Little is known about the prevalence of PH in CF patients and its impact on outcome. METHODS: We retrospectively studied a large cohort of CF patients evaluated for lung transplantation between 1995 and 2010. All the patients underwent right heart catheterization as part of the evaluation. We included 179 unique consecutive adult CF patients. Age was 24±9 years and 45.8% were women. RESULTS: Eighty-seven patients were transplanted (48.6%) and 65 died (36.3%) while waiting for LT. By right heart catheterization, 38.5% of the patients had PH (mean ≥25 mm Hg). PaCO(2) (P=0.045) and forced vital capacity (P=0.023) were independent predictors of PH in CF patients. The median survival (free of lung transplantation) was 13.4 months. After adjusting for several covariates, the presence of PH significantly increased mortality (hazard ratio, HR) (P<0.001). Pulmonary vascular resistance was associated with mortality (P=0.03). When both PH and PVR were included in the model, only PH predicted mortality. CONCLUSION: Pulmonary hypertension of mild degree is frequently found in CF patients with advanced lung disease and its presence significantly worsens survival.

Pulmonary hypertension is associated with higher mortality in cystic fibrosis patients awaiting lung transplantation.

AIM:Pulmonary hypertension (PH) is frequently found in patients with advanced parenchymal lung diseases. In advanced stages, cystic fibrosis (CF) patients can develop PH and eventually cor pulmonale. Little is known about the prevalence of PH in CF patients and its impact on outcome. METHODS: We retrospectively studied a large cohort of CF patients evaluated for lung transplantation between 1995 and 2010. All the patients underwent right heart catheterization as part of the evaluation. We included 179 unique consecutive adult CF patients. Age was 24±9 years and 45.8% were women. RESULTS:Eighty-seven patients were transplanted (48.6%) and 65 died (36.3%) while waiting for LT. By right heart catheterization, 38.5% of the patients had PH (mean ≥25 mm Hg). PaCO2 (P=0.045) and forced vital capacity (P=0.023) were independent predictors of PH in CF patients. The median survival (free of lung transplantation) was 13.4 months. After adjusting for several covariates, the presence of PH significantly increased mortality (hazard ratio, HR) (P<0.001). Pulmonary vascular resistance was associated with mortality (P=0.03). When both PH and PVR were included in the model, only PH predicted mortality. CONCLUSION: Pulmonary hypertension of mild degree is frequently found in CF patients with advanced lung disease and its presence significantly worsens survival.

Treatment of complex airway lesions after lung transplantation with self-expandable nitinol stents: early experience.

Airway complications (AC) are considered a serious cause of morbidity after lung transplantation (LT). Mechanical dilatation, laser vaporization, and silicone stent placement usually solve it. However, the use of self-expandable metallic stents (SENS) may be indicated in selected cases. Ten lung transplant recipients with AC were treated with SENS. Six patients underwent LT for cystic fibrosis, 2 for idiopathic pulmonary fibrosis, 1 for bronchiectasis, and 1 for emphysema. All patients received at least 1 treatment attempt with dilatation and silicone stent placement. The indications for SENS placement were the presence of a tortuous airway axis with stenosis and malacia of the right main bronchus in 5 patients; a long stenosis of the main and intermediate right bronchus involving the upper lobe orifice in 3 patients; or malacia that could not be stabilized with silicone stents in 3 cases. In 1 patient the procedure was bilateral. Functional improvement was immediate with a mean forced expiratory volume at 1 second (FEV(1)) gain of 35%. No stent dislocation was observed. Symptoms did not occur again in 5 patients with previous recurrent episodes of pneumonia. One stenosis, which was due to the ingrowth of granulation tissue occurred at 6 months after the procedure, was successfully treated with mechanical dilatation and laser vaporization. The deployment of SENS in a selected group of patients with AC after LT was easy, safe, and effective.

Extracorporeal circulatory support for lung transplantation: institutional experience.

Lung transplantation (LT) represents the only available therapy for selected patients affected by end-stage pulmonary disease. Cardiopulmonary bypass (CPBP) is used, when required, during single and sequential double lung transplantation; however, it increases the risk of bleeding, early graft dysfunction, failure, and other potential side effects. We report our experience with 145 patients who underwent lung transplantations, among whom 34 required intraoperative CPBP. The indications for LT among these 34 patients were cystic fibrosis (n = 22), chronic obstructive pulmonary disease (n = 3), bronchiectasis (n = 2), primary pulmonary hypertension (n = 1), fibrosis (n = 2), pulmonary microlithiasis (n = 1), and retransplantation for obliterative bronchilitis (n = 3). CPBP was planned in 12 cases (group I) and unplanned in 22 (group II). The main reason for planning CPBP was primary and secondary pulmonary hypertension (mean pulmonary artery pressure >or=25 mm Hg). Acute right ventricular failure, hemodynamic instability, arterial desaturation, and increased pulmonary artery pressure were mandatory for unplanned CPBP. Among the 34 CPBP patients, the 30-day mortality rate was 35% (12/34) including 9 (70%) in group II (unplanned CPBP). The leading cause of death was multiorgan failure. The 1-year survival rates were 67% and 36%, and the 3-year survival rates were 47% and 18% for groups I and II, respectively. In conclusion, even if it represents a useful tool in the management of critical events, the use of unscheduled CPBP during LT procedures is associated with an increased postoperative morbidity and mortality.

Surgical treatment of redundancy after retrosternal esophagocoloplasty.

Redundancy is a well-recognized complication of esophageal replacement with colonic interposition, occurring several years after surgery. In a small number of patients, symptoms are disabling and might require reoperation. This article describes the surgical treatment of a 54-year-old male presenting with severe dysphagia, malnutrition and recurrent aspiration pneumonia, progressively developed 30 years after esophageal replacement with retrosternal ileocolonic interposition for caustic strictures.

Extracorporeal removal CO2 using a venovenous, low-flow system (Decapsmart) in a lung transplanted patient: a case report.

BACKGROUND: Primary graft dysfunction (PGD) is a syndrome that may occur after lung transplantation. In some cases of severe PGD, conventional therapies like ventilatory support, administration of inhaled nitric oxide (iNO), and surfactant and intravenous prostacyclins are inadequate to achieve adequate gas exchange. The only lifesaving option is to use an extracorporeal membrane oxygenator. The Decapsmart is a new venovenous, low-flow extracorporeal device to removal carbon dioxide (CO(2)). It does not need a specialized staff. Herein we have presented a case report of a patient who underwent single lung transplantation and experienced respiratory failure. METHODS: On November 2007, a 52-year-old woman underwent a single right lung transplantation, and developed severe PGD in the postoperative period. After institution of conventional treatments, including ventilatory and hemodynamic support, iNO, and prostaglandine E1, we started treatment with Decapsmart to remove CO(2). Hemodynamic and respiratory parameters were assessed at baseline and after 3, 12, 24, and 48 hours. RESULTS: No adverse events occurred. From baseline to 48 hours, pH values increased and partial pressure of CO(2) reduced. At the same time ventilatory support was reduced, thereby mitigating barotrauma and risk of overdistension. CONCLUSION: The use of Decapsmart may be an important aid for patients with severe respiratory acidosis in association with conventional therapy during the perioperative period after lung transplantation.

Lung transplantation for cystic fibrosis: ten years of experience.

Lung transplantation represents the only therapeutic option for patients affected by end-stage cystic fibrosis (CF). We performed 76 lung transplantations in 73 patients from 1996-2007. The mean time on the waiting list was 10+/-6 months. The median follow-up after the transplantation was 69.3 months. Twenty-one transplants (27.6%) were performed under cardiopulmonary bypass. Perioperative mortality, excluding retransplants, was 16.4% (12 patients) and the causes of death were sepsis, primary graft failure, and myocardial infarction. The overall survival was 74.5%+/-5%, 62.9%+/-5%, 54.1%+/-6%, and 43.4%+/-6% at 1, 3, 5, and 10 years, respectively. The accurate selection of potential recipients and the correct timing of referral and transplantation are factors that play crucial roles to obtain satisfactory results in term of improvement of quality of life and long-term survival.

Non-Hodgkin's lymphoma, presenting as an isolated endobronchial mass after bilateral lung transplantation: a case report.

BACKGROUND: Lymphoma is a serious complication following solid organ transplantation. Endobronchial involvement of non-Hodgkin's lymphoma (NHL) is rare usually occurring in the setting of widely disseminated disease. Only a few cases of isolated endobronchial NHL have been reported. They were mostly confined to the main bronchi. METHODS: Herein we have described a case of an obstructive endobronchial NHL lesion in a young patient, which developed 6 years after bilateral lung transplantation for cystic fibrosis. RESULTS: Successful treatment was obtained with endoscopic resection of the lesion followed by chemotherapy. CONCLUSIONS: In rare cases NHL may present as an isolated endobronchial mass in the main bronchi. Because in these cases the prognosis seems to be better, early diagnosis is essential. Rigid bronchoscopy offers the opportunity to safely obtain large amounts of tissue for complete histological diagnosis and to de-obstruct the airway to relieve the symptoms.

Preoperative anaemia does not affect the early postoperative outcome in patients with lung cancer.

AIM: Several prognostic factors like age, gender, histology, stage, type of operation, associated disorders and administration of induction therapy have been evaluated to assess the risk of postoperative complications and outcome in patients with resectable lung cancer. Anemia is a frequent condition in this subset of patients being estimated up to 50%. The aim of this retrospective study was to evaluate the effect of preoperative anemia on early outcome after lung cancer resection. METHODS: One-hundred thirty nine consecutive patients undergoing surgery for non small cell lung cancer were retrospectively considered. The mean age was 64.8+/-11.6 years. No patient received blood transfusions or administration of erythropoetin preoperatively. Overall, we performed 96 lobectomies, 14 pneumonectomies, 2 bilobectomies and 27 atypical resections. A subset of 27 patients (19.4%) (group I) had a preoperative value of Hb less than 12 g/dl (10.4+/-1.9 g/dL). Seven patients of them were stage IA (26%), 9 stage IB (33.3%), 2 stage IIA (7.4%), 6 stage IIB (22.2%), 2 stage IIIA (7.4%) and 1 stage IIIB (3.7%). Age, gender, stage, type of operation, induction chemotherapy, comorbidities were evaluated by univariate analysis comparing patients with and without preoperative anaemia. The two groups were homogenous regarding demographic characteristics. RESULTS: Three patients (11.1%) in group I and 2 (1.8%) in group II required blood transfusions after surgery (P=0.01); 4 of them received pneumonectomy (P<0.0001). The overall morbidity was 17.9% (25/139); the most frequent complication was persistent air leakage, followed by retention of secretions. No statistically significant difference was observed between the 2 groups about early mortality (1 patient-3.7% in group I and 2 patients-1.8% in group II) and postoperative complications (5 patients-18.5% in group I and 20 patients-17.9% in group II). CONCLUSION: Preoperative anaemia is not a risk factor for an increased rate of postoperative complications and should not be considered a contraindication to surgery.

Malignancies following lung transplantation.

During the last 2 decades, long-term survival after lung transplantation has significantly improved. However, among the complications related to the continuous administration of immunosuppressive drugs, malignancy plays an important role. We retrospectively revisited our series of patients to report our experience. From January 1991 we performed 134 lung transplantations in 128 recipients (mean age, 33.4 +/- 13.5 years). In all patients the first-line immunosuppressive regimen was based on a calcineurin inhibitor (cyclosporine or tacrolimus), an antimetabolic agent (azathioprine), and steroids. Five patients (4.2%) developed malignancy and the mean time of occurrence after the transplantation was 46.4+/-23 months. The mean age was 41 +/- 16 years (P = not significant [ns]). The tumors were as follows: laryngeal cancer (radiotherapy), colon cancer (surgery plus adjuvant chemotherapy), gastric cancer (surgery plus adjuvant chemotherapy), endobronchial non-Hodgkin lymphoma (NHL) (endoscopic resection plus chemoradiotherapy), and cutaneous and visceral Kaposi's sarcoma (KS) (chemotherapy). All patients have reduced the dose of immunosuppressive drugs; in 1 of them, tacrolimus was changed to rapamycin. Two patients died because of neoplastic dissemination, another 1 due to obliterans bronchiolitis. The 2 patients with NHL and KS are alive at 6 and 9 months, respectively, without signs of recurrence. Malignancies after lung transplantation represent an important problem. A multidisciplinary approach is mandatory to obtain satisfactory results in terms of improved quality of life and long-term survival.

Incidence of fungal infections in a solid organ recipients dedicated intensive care unit.

UNLABELLED: Invasive fungal infections are a significant cause of morbidity and mortality for patients undergoing solid organ transplantation. Our aim was to evaluate the incidence of invasive fungal infections in solid organ recipients within a dedicated intensive care unit (ICU). MATERIALS AND METHODS: From May 2002 to May 2005, 278 patients undergoing solid organ transplantation (105 liver, 142 kidney, 20 lung, 2 combined liver-kidney, 9 combined pancreas-kidney) were admitted to our posttransplant intensive care unit. We retrospectively analyzed data obtained from the ICU stay. Fungal infection was defined by positivity of normally sterile biological samples and by elevated positivity of normally non sterile biological samples. We did not consider superficial fungal infections and asymptomatic colonizations. RESULTS: Forty-six patients (16.5%) developed a fungal infection; at least one mycotic agent was isolated from each patient. Candida albicans was the most common pathogen, isolated from 71 % of infected patients (33 of 46). Infected patients showed a mortality rate of 35%, while that for non infected recipients was 3.5%. Total length of ICU stay was the most significant risk factor among infected patients (30.26 days vs 5.04 days P < .0001). Mean time between transplantation and first positive samples was 6.17 days (SD 8.88). CONCLUSION: Fungal infections in solid organ transplant patients are a major issue because of their associated morbidity and mortality. Candida albicans was the most common pathogen and total length of ICU stay was the most important risk factor.

[Surgical treatment of iatrogenic perforations of the distal third of the esophagus. Personal experience]. / Trattamento chirurgico delle perforazioni iatrogene del terzo inferiore dell'esofago. Esperienza personale.

AIM: The esophageal perforations are associated with a high mortality and morbidity when they are not diagnosed and treated quickly. The aim of our study is to analyze the treatment and prognosis of the distal iatrogenic esophageal perforations on the basis of time of onset, concomitant disease and size of perforations. METHODS: The retrospective review was performed on 10 patients treated for distal iatrogenic esophageal perforations at our Institution from 1994 to 2003. The cause of perforations was: pneumatic dilation (7 patients) and esophageal endoprosthesis placing (3 patients). Seven patients presented within 24 h (Group A), and 3 patients presented after 24 h (Group B). In Group A, 4 patients underwent primary repair, 2 patients required esophagectomy and 1 patient was treated conservatively. In Group B, 2 patients were treated conservatively and 1 patient required an esophagectomy. RESULTS: Hospital morbidity was 20% and mortality was 30%. In Group A no patients died. In Group B hospital mortality was 100%. The most common cause of death was multiorgan failure resulting from sepsis. CONCLUSIONS: The prognosis for esophageal perforations is influenced by the time elapsed between diagnosis and treatment. Esophagectomy is indicated for patients with extensive perforation and necrosis of the esophagus when primary repair cannot be carried out. It is indicated also as treatment for the concomitant disease.

[Mechanical cervical esophagogastric anastomosis after esophagectomy for cancer]. / Anastomosi meccanica esofagogastrica dopo esofagectomia per neoplasia.

AIM: Fibrous stenosis of the esophagogastric cervical anastomosis remains a significant complication occurring in up to one third of cases. Trying to reduce the incidence of this complication, we describe our technique of cervical esophago-gastric anastomosis using endoscopic linear stapler which seems to reduce the incidence of fibrous stricture formation after resection of esophageal cancer. METHODS: Between March 2000 and December 2004, 34 patients (20 males and 14 females) underwent esophagectomy using tubulized stomach for reconstruction. Mean age was 57 years. Eight patients with advanced stage (5 T3 and 3 T4) underwent induction chemotherapy. The most of patients was affected by squamous cell carcinoma. In all cases we performed cervical esophagogastric anastomosis using linear endoscopic stapler. The occurrence of postoperative anastomotic leak and development of anastomotic stricture were recorded and analyzed. RESULTS: All patients survived esophagectomy and 30 of them (88%) were available for postoperative follow-up at 6 months. Anastomotic leak developed in 1 case. No patient developed fibrous stenosis that required dilatation therapy. CONCLUSIONS: Complete mechanical esophago-gastric anastomosis, using endoscopic linear stapler is effective and safe, even when a narrow gastric tube is used as esophageal substitute. These technique seems superior to other techniques to reduce the incidence of postoperative anastomotic complications.

Extracorporeal circulation with low systemic heparinization during lung transplantation.

BACKGROUND: Some lung transplantation (LT) recipients suffer from pulmonary hypertension and right ventricular dysfunction or failure requiring extracorporeal circulation (ECC) to avoid catastrophic complications during surgery. The extracorporeal support usually requires systemic heparinization which is potentially associated with important side effects. We performed eight LT using preheparinized ECC circuits and an oxygenator associated with a lower level of systemic heparinization without evidence of perioperative complications. PATIENTS AND METHODS: From May 2002 to May 2005, 8 patients (5 men and 3 women) of mean age 22.5 +/- 9.5 years underwent bilateral sequential lung transplantation (BSLT) for cystic fibrosis (n = 6) or idiopathic pulmonary fibrosis (n = 2). All procedures were performed with ECC through a femoro-femoral veno-arterial bypass with preheparinized circuits and an oxygenator. RESULTS: No intraoperative mortality occurred. The mean ECC time was 147.8 +/- 31.3 minutes and the mean heparin administered was 3525 +/- 969.16 UI. No coagulopathy or thrombotic events were observed perioperatively. CONCLUSIONS: Our study confirmed the efficacy and safety of prehepanized circuits and oxygenator for femoro-femoral veno-arterial bypass during LT for patients with severe pulmonary hypertension requiring ECC.

Lung cancer after treatment for non-Hodgkin lymphoma.

AIM: Because of the improvement in treatment and survival of patients with lymphoma, late sequelae, including secondary cancers have been extensively studied. Lung cancer is one of the two most common solid tumors after Hodgkin's disease but fewer studies have been published about lung cancer after non-Hodgkin lymphoma (NHL). METHODS: Over the last five years at our Institution we have observed 16 patients, 13 male and 3 female, with a mean age of 61 years, previously treated for NHL and lung cancer. Median latency between NHL and lung cancer was 7 years. In 6 patients (37.5%) the latency period was shorter than 5 years and 3 of them developed lung cancer within 2 years after the end of NHL therapy. RESULTS: Ten patients underwent lung complete resection. Two, 3 and 5 year survival rate was respectively 52.7%, 26.3% and 13%. In contrast, the median survival of non surgical patients was 9 months. Comparison of survival between surgical and non-surgical group demonstrated a statistically significant better survival for surgically treated patients (P<0.04). CONCLUSIONS: Surgery can improve survival in patients with history of NHL and lung cancer. Early diagnosis and treatment is crucial. NHL survivors should undergo careful follow-up and surveillance for secondary malignancy.

Evolving techniques and perspectives in lung transplantation.

Lung transplantation is currently a suitable option for patients with end-stage lung disease. Since the early 1980s the surgical technique and immunosuppressive protocols have been progressively modified to improve results and favor long-term survival. The original heart-lung transplantation under cardiopulmonary bypass is now rarely performed and single or bilateral lung transplantation is the procedure of choice. Bilateral transplantation is performed with two single lung transplants performed in sequence. Extracorporeal support is rarely employed and in most cases it is instituted through the femoral approach. Also, the surgical approach has been modified and the original clam shell incision has been replaced by two small anterior thoracotomies. The use of marginal donors has been increasingly proposed to enlarge the number of organs potentially available for transplantation. Immunosuppressive protocols have evolved to patient-specific regimens that can be quickly modified if required by the clinical status. Induction is now more aggressive and also rescue protocols for obliterative bronchiolitis can contribute to improved outcomes. Overall, lung transplantation is now performed with encouraging long-term results.

[Mini-invasive treatment of pectus excavatum in adolescence. Initial experience]. / Trattamento mini-invasivo del petto escavato in età adolescenziale. Esperienza iniziale.

BACKGROUND: Personal preliminary experience with Minimally Invasive Repair of Pectus Excavatum (MIRPE), "Nuss" procedure, using VATS is reported. METHODS: From January 2001 to February 2002, MIRPE has been performed on 5 patients (age range 13-18 y; mean 14.8 y). Under general anesthesia, a curved steel bar is inserted into the retrosternal tunnel between 2 bilateral midaxillary line incisions. The tunnel passes initially under the pectoral muscles and enters the pleural space at level of the mammilary line. Under thoracoscopic vision, the bar is passed through the tunnel with the concavity facing the front and then is turned over thereby correcting deformity. An epidural catheter relieved perioperative pain successfully. RESULTS: In all patients the repair has been good. Mean hospital length of stay has been 6.8 d. Pneumothorax occurred in 1 patient requiring tube thoracostomy. After 45 d 1 patient had a bar displacement requiring a reoperation. All patients have a normal life. CONCLUSIONS: The Minimally Invasive Repair of Pectus Excavatum is an effective procedure even in adolescence. Thoracoscopic vision makes safer the creation of the retrosternal tunnel and the passage of the bar. Short-term results have been good. Further follow-up is necessary to determine long-term results.

Lung transplantation for cystic fibrosis.

BACKGROUND: Lung transplantation is a robust therapeutic option to treat patients with cystic fibrosis. PATIENTS AND METHODS: Since 1996, 109 patients with cystic fibrosis were accepted onto our waiting list with 58 bilateral sequential lung transplants performed in 56 patients and two patients retransplanted for obliterative bronchiolitis syndrome. RESULTS: Preoperative mean FEV(1) was 0.64 L/s, mean PaO(2) with supplemental oxygen was 56 mm Hg, and the mean 6-minute walking test was 320 m. Transplantation was performed through a "clam shell incision" in the first 29 patients and via bilateral anterolateral thoracotomies without sternal division in the remaining patients. Cardiopulmonary bypass was required in 14 patients. In 21 patients the donor lungs had to be trimmed by wedge resections with mechanical staplers and bovine pericardium buttressing to fit the recipient chest size. Eleven patients were extubated in the operating room immediately after the procedure. Hospital mortality of 13.8% was related to infection (n = 5), primary graft failure (n = 2), and myocardial infarction (n = 1). Acute rejection episodes occurred 1.6 times per patient/year; lower respiratory tract infections occurred 1.4 times per patient in the first year after transplantation. The mean FEV(1) increased to 82% at 1 year after operation. The 5-year survival rate was 61%. A cyclosporine-based immunosuppressive regimen was initially employed in all patients; 24 were subsequently switched to tacrolimus because of central nervous system toxicity, cyclosporine-related myopathy, or renal failure, obliterative bronchiolitis syndrome, gingival hyperplasia, or hypertrichosis. Ten patients were subsequently switched to sirolimus. Freedom from bronchiolitis obliterans at 5 years was 60%. CONCLUSIONS: Our results confirm that bilateral sequential lung transplantation is a robust therapeutic option for patients with cystic fibrosis.

[Thoracoscopic esophagectomy for esophageal cancer. Personal experience]. / Esofagectomia toracoscopica per cancro dell'esofago.

BACKGROUND: Esophageal surgery was recently modified by minimally-invasive approach. Personal experience with the thoracoscopic technique for esophagectomy in patients with early stage esophageal cancer is described. METHODS. From 1996 to 2000 at the Department of Thoracic Surgery of the University of Rome "La Sapienza", 10 patients, 7 male and 3 female, underwent video-thoracoscopic esophagectomy for esophageal cancer. Median age was 64 years (range 53-72). With the patient in left lateral decubitus 4 ports were positioned between the 4th and 8th intercostal space. The thoracic esophagus was mobilized in the entire length and circumference with the connective tissue and peri-esophageal nodal stations. A cervicotomy followed by a median laparotomy for tubulization of the stomach was performed. RESULTS: Nobody required conversion to thoracotomy. No complication or intraoperative death were observed. The median thoracic time was 110 minutes (range 55-165). No death within 30 days after discharge was recorded. One patient presented left vocal cord paralysis. In one case a recurrence in cervical anastomosis two months after the operation was observed. One patient died after 36 month for metastatic spread. Eight patients are alive with no evidence of disease, with median follow-up of 20 months. CONCLUSIONS: In our experience, the video-toracoscopic approach is a viable and safe option for the treatment of early stage esophageal cancer. Low incidence of complications and local recurrence should encourage a most frequent use of this procedure.