Clinical characteristics and survival of patients with pulmonary hypertension: a 40-month mean follow-up.

BACKGROUND AND AIMS: The aim of our study was to describe the characteristics and clinical outcomes of pulmonary hypertension (PH) patients who underwent their follow-up at a single centre. METHODS: We included patients who fulfilled the criteria for the diagnosis of PH by right heart catheterisation. A detailed clinical history and complete physical examination were recorded for each patient. Demographic data, laboratory testing, haemodynamic values and treatment records were also collected. A 6-min walk test was used to evaluate exercise capacity. Survival analysis was performed using the Kaplan-Meier method, and a log-rank test was used to compare survival curves among groups. A multivariate Cox's proportional hazards model was used in order to identify independent predictors of mortality in the overall PH population. RESULTS: A total of 93 patients (64 women and 29 men) were studied. The patients were distributed across the groups as follows: 50.5% in group 1, 20.4% in group 2, 9.7% in group 3, 14% in group 4 and 5.4% in group 5. The most common type of PH in our population was portopulmonary hypertension followed by PH associated with left heart disease, and idiopathic and chronic thromboembolic disease. Nineteen deaths, constituting 20.5% of the sample population, were reported. The Kaplan-Meier estimates of the 1-, 2- and 3-year survival rates were 91%, 89% and 85%, respectively. There were no statistically significant differences in survival among the groups (P = 0.18). A multivariate analysis revealed that the cardiac index (CI) was an independent predictor of death. CONCLUSIONS: The survival rates of PH patients are higher than previously reported. In our study, the largest number of deaths occurred in groups 1 and 2, and CI was found to be an independent predictor of mortality.

Functional outcomes after lung transplant in chronic obstructive pulmonary disease.

INTRODUCTION: Lung transplantation (LT) is a therapeutic option with controversial results in chronic obstructive pulmonary disease (COPD). We aimed to analyze the outcomes of transplantation in terms of lung function and to identify prognostic factors. METHOD: A retrospective analysis of 107 patients with COPD receiving lung transplants in the La Fe Hospital between 1991 and 2008 was performed. Preoperative variables, pulmonary function tests before and after LT, surgical procedure variables and long-term monitoring, expressed as mean or percentage, as applicable, were analyzed. Spirometric results before and after LT were analyzed. Linear or logistic regression were used for multivariate analysis depending on the variable. RESULTS: Ninety-four men (87.9%) and 13 women (12.1%) were transplanted, with a mean age±standard deviation of 52.58±8.05 years; 71% of LTs were double-lung transplantations. Spirometric values improved after LT: FVC: +1.22L (+34.9%), FEV1: +1.66L (+56.7%) and FEF25-75: +1.85L (+50.8%); P=.001. This functional improvement was maintained after 5 years only in the group with BODE score >7 (P=.001). Recipient height, type of LT, use of extracorporeal circulation during the surgical procedure, presence of bronchiolitis obliterans syndrome and the age and cause of death of the donor significantly influenced lung function over time. CONCLUSIONS: LT improves lung function in COPD patients. This improvement was maintained at 5years only in patients with BODE>7. Double lung transplantation provides better functional results than single-lung transplantation.

Thrombotic microangiopathy associated with tacrolimus in lung transplantation.

Thrombotic microangiopathy (TMA) is a rare complication associated with the use of calcineurin inhibitors in lung transplantation, irrespective of the underlying disease of the graft recipient. It usually occurs in incomplete forms, complicating and delaying diagnosis until damage is already irreversible. It is unrelated to time from transplantation and often presents with concomitant infection, which tends to confound diagnosis. The cases discussed here have a common causative agent and all present with concomitant infection. Treatment recommendations have changed in recent years with the introduction of plasmapheresis or, more recently, the availability of the antibody eculizumab. Notwithstanding, the most cost-effective measure is withdrawal or switching of the calcineurin inhibitor. TMA is an underdiagnosed clinical entity that should be considered in the management of transplantation patients.

Tumor, node and metastasis classification of lung cancer--M1a versus M1b--analysis of M descriptors and other prognostic factors.

INTRODUCTION: The current edition of the tumor, node and metastasis (TNM) classification of lung cancer (LC) divides the presence of metastasis (M1) into two categories: M1a and M1b, depending on its anatomical location. To assess this new classification, the survival and the M descriptors of LC patients with metastatic disease registered by the Bronchogenic Carcinoma Cooperative Group of the Spanish Society of Pneumology and Thoracic Surgery II (GCCB-S-II), were analyzed. METHODS: Non-small cell lung cancer (NSCLC) patients, with M1a or M1b disease, included in the GCCB-S-II, from April 2009 to December 2010, staged in accordance with the prospective staging project protocol of the International Association for the Study of Lung Cancer (IASLC), and with complete TNM staging and follow-up data, were studied. The overall survival associated with each M1 category and each M descriptor, besides other prognostic factors (sex, age, performance status [PS] and others) were analyzed by univariate and multivariate models. RESULTS: 640 NSCLC patients (195 M1a and 445 M1b) were included. M1b tumors had significantly worse survival than M1a tumors (p < 0.001). The prognostic value of M1 category was independent from other prognostic variables such as PS, weight loss, and others. The number of metastatic sites (isolated versus multiple) and the number of lesions (single versus multiple) in patients with isolated metastasis showed prognostic value, especially in those with brain metastasis. CONCLUSION: The current division of the M1 category into two subsets (M1a and M1b) is warranted by their prognostic significance. The number of metastatic sites and the number of lesions in patients with isolated metastasis should be taken into account, because they also have prognostic relevance.

[Lung transplantation in sporadic lymphangioleiomyomatosis: study of 7 cases]. / Trasplante pulmonar en la linfangioleiomiomatosis esporádica. Estudio de 7 casos.

BACKGROUND AND OBJECTIVE: Sporadic lymphangioleiomyomatosis (S-LAM) is a rare disease that affects only women. It is characterized by an abnormal proliferation of immature smooth muscle cells (LAM cells) that grow in an aberrant manner in the airway, parenchymal lung lymph and blood vessels, determining the onset of pulmonary cystic lesions. The disease has no treatment, progressing to respiratory failure, and lung transplantation (LT) may be a treatment option at this stage. Our goal was to study 7 patients undergoing LT for S-LAM. MATERIAL AND METHOD: We studied a series of clinical and demographic characteristics, diagnostic modality and post-transplant outcomes. We performed a descriptive analysis of the series. The Kaplan-Meier method was used to estimate survival. RESULTS: The mean age of onset of symptoms was 35 years, the diagnosis of 37 years and that of LT 38 years. The most common symptom was dyspnea. Four patients had a history of pneumothorax and pleural effusion. The mean forced expiratory volume in one second was 32.7% and the diffusing capacity for carbon monoxide was 29%. All patients were subjected to LT and survival was 100, 85.7 and 57.1% at one, 3 and 5 years, respectively. Three died of bronchiolitis obliterans and 2 necropsies did not show evidence of disease recurrence. CONCLUSIONS: LT is a therapeutic option in patients with S-LAM with an advanced respiratory functional impairment.

[Sporadic lymphangioleiomyomatosis and pulmonary hypertension. Clinical and pathologic study in patients undergoing lung transplantation]. / Linfangioleiomiomatosis esporádica e hipertensión pulmonar. Estudio clínico-patológico en pacientes receptoras de un trasplante pulmonar.

BACKGROUND AND OBJECTIVE: Lymphangioleiomyomatosis (LAM) is a rare disease characterized by an aberrant immature smooth muscle cells proliferation. This excessive growth of LAM cells around airways, parenchyma, lymphatic ganglia and lung vessels, produces cystic damages. LAM affects mainly women and can lead to pulmonary hypertension (PH). The aim of this research is the clinical study, as well as the analysis of the morphological features, expressing PH, of the native lungs from lung transplant (LT) patients. PATIENTS AND METHODS: Several clinical variables were studied in 7 patients who had a LAM and who were subjected to LT. The variables analyzed using morphologic and immunohistochemistry techniques were: histological alterations in native lungs, their severity rate, the presence of haemosiderosis in the air spaces and correlation index between thickness of the middle layer and the pulmonary arterial vascular diameter, morphological expression of HP. RESULTS: The average age was 34 years old and the predominant symptom was dyspnea. The respiratory functional pattern was mixt, with decrease of DLCO% and KCO%, serious hypoxemia and normocapnia. In the cardiological study, subclinical signs of HP were found. The immunohistochemistry was positive for smooth muscle and melanic expression markers, estrogen receptor in 6 cases and progesterone receptor in 4. Vascular correlation index showed HP criteria at all. CONCLUSIONS: This research shows the presence of clinical and morphological HP criteria in advanced LAM patients. Therefore, cardiological assessment before LT with right catheterization will be justified. Future research should be addressed to clarify whether the HP should be criteria for LT in LAM patients.

Lymphangioleiomyomatosis.

Lymphangioleiomyomatosis (LAM) is a rare disease that mainly affects women, particularly at fertile age. It is sporadic or associated with tuberous sclerosis complex. It is characterised by an abnormal proliferation of immature smooth muscle cells (SMC), which grow aberrantly in the airway, parenchyma, lymphatics and pulmonary blood vessels and which can gradually lead to respiratory failure. It affects several systems, affecting the lymphatic ganglia and causing abdominal tumours. Given its very low prevalence, a difficult to establish early diagnosis, absence of curative treatment and the difficulty in obtaining information, places LAM under the heading of the so-called Rare Diseases. There is a growing interest in the study of this disease which has led to the setting up of patient registers and an exponential growth in LAM research, both at a clinical level and cellular level.