Guidelines on the Diagnosis and Treatment of Pulmonary Hypertension: Summary of Recommendations. / Guía de diagnóstico y tratamiento de la hipertensión pulmonar: resumen de recomendaciones.

Pulmonary hypertension is a hemodynamic disorder defined by abnormally high pulmonary artery pressure that can occur in numerous diseases and clinical situations. The causes of pulmonary hypertension are classified into 5 major groups: arterial, due to left heart disease, due to lung disease and/or hypoxemia, chronic thromboembolic, with unclear and/or multifactorial mechanisms. This is a brief summary of the Guidelines on the Diagnostic and Treatment of Pulmonary Hypertension of the Spanish Society of Pulmonology and Thoracic Surgery. These guidelines describe the current recommendations for the diagnosis and treatment of the different pulmonary hypertension groups.

Evaluation of humoral immunity profiles to identify heart recipients at risk for development of severe infections: A multicenter prospective study.

BACKGROUND: New biomarkers are necessary to improve detection of the risk of infection in heart transplantation. We performed a multicenter study to evaluate humoral immunity profiles that could better enable us to identify heart recipients at risk of severe infections. METHODS: We prospectively analyzed 170 adult heart recipients at 8 centers in Spain. Study points were before transplantation and 7 and 30 days after transplantation. Immune parameters included IgG, IgM, IgA and complement factors C3 and C4, and titers of specific antibody to pneumococcal polysaccharide antigens (anti-PPS) and to cytomegalovirus (CMV). To evaluate potential immunologic mechanisms leading to IgG hypogammaglobulinemia, before heart transplantation we assessed serum B-cell activating factor (BAFF) levels using enzyme-linked immunoassay. The clinical follow-up period lasted 6 months. Clinical outcome was need for intravenous anti-microbials for therapy of infection. RESULTS: During follow-up, 53 patients (31.2%) developed at least 1 severe infection. We confirmed that IgG hypogammaglobulinemia at Day 7 (defined as IgG <600 mg/dl) is a risk factor for infection in general, bacterial infections in particular, and CMV disease. At Day 7 after transplantation, the combination of IgG <600 mg/dl + C3 <80 mg/dl was more strongly associated with the outcome (adjusted odds ratio 7.40; 95% confidence interval 1.48 to 37.03; p = 0.014). We found that quantification of anti-CMV antibody titers and lower anti-PPS antibody concentrations were independent predictors of CMV disease and bacterial infections, respectively. Higher pre-transplant BAFF levels were a risk factor of acute cellular rejection. CONCLUSION: Early immunologic monitoring of humoral immunity profiles proved useful for the identification of heart recipients who are at risk of severe infection.

Long-Term Outcome of Patients With Chronic Thromboembolic Pulmonary Hypertension: Results From an International Prospective Registry.

BACKGROUND: Chronic thromboembolic pulmonary hypertension, a rare complication of acute pulmonary embolism, is characterized by fibrothrombotic obstructions of large pulmonary arteries combined with small-vessel arteriopathy. It can be cured by pulmonary endarterectomy, and can be clinically improved by medical therapy in inoperable patients. A European registry was set up in 27 centers to evaluate long-term outcome and outcome correlates in 2 distinct populations of operated and not-operated patients who have chronic thromboembolic pulmonary hypertension. METHODS AND RESULTS: A total of 679 patients newly diagnosed with chronic thromboembolic pulmonary hypertension were prospectively included over a 24-month period. Estimated survival at 1, 2, and 3 years was 93% (95% confidence interval [CI], 90-95), 91% (95% CI, 87-93), and 89% (95% CI, 86-92) in operated patients (n=404), and only 88% (95% CI, 83-91), 79% (95% CI, 74-83), and 70% (95% CI, 64-76) in not-operated patients (n=275). In both operated and not-operated patients, pulmonary arterial hypertension-targeted therapy did not affect survival estimates significantly. Mortality was associated with New York Heart Association functional class IV (hazard ratio [HR], 4.16; 95% CI, 1.49-11.62; P=0.0065 and HR, 4.76; 95% CI, 1.76-12.88; P=0.0021), increased right atrial pressure (HR, 1.34; 95% CI, 0.95-1.90; P=0.0992 and HR, 1.50; 95% CI, 1.20-1.88; P=0.0004), and a history of cancer (HR, 3.02; 95% CI, 1.36-6.69; P=0.0065 and HR, 2.15; 95% CI, 1.18-3.94; P=0.0129) in operated and not-operated patients, respectively. Additional correlates of mortality were bridging therapy with pulmonary arterial hypertension-targeted drugs, postoperative pulmonary hypertension, surgical complications, and additional cardiac procedures in operated patients, and comorbidities such as coronary disease, left heart failure, and chronic obstructive pulmonary disease in not-operated patients. CONCLUSIONS: The long-term prognosis of operated patients currently is excellent and better than the outcome of not-operated patients.

Updated clinical classification of pulmonary hypertension.

In 1998, a clinical classification of pulmonary hypertension (PH) was established, categorizing PH into groups which share similar pathological and hemodynamic characteristics and therapeutic approaches. During the 5th World Symposium held in Nice, France, in 2013, the consensus was reached to maintain the general scheme of previous clinical classifications. However, modifications and updates especially for Group 1 patients (pulmonary arterial hypertension [PAH]) were proposed. The main change was to withdraw persistent pulmonary hypertension of the newborn (PPHN) from Group 1 because this entity carries more differences than similarities with other PAH subgroups. In the current classification, PPHN is now designated number 1. Pulmonary hypertension associated with chronic hemolytic anemia has been moved from Group 1 PAH to Group 5, unclear/multifactorial mechanism. In addition, it was decided to add specific items related to pediatric pulmonary hypertension in order to create a comprehensive, common classification for both adults and children. Therefore, congenital or acquired left-heart inflow/outflow obstructive lesions and congenital cardiomyopathies have been added to Group 2, and segmental pulmonary hypertension has been added to Group 5. Last, there were no changes for Groups 2, 3, and 4.

Chronic thromboembolic pulmonary hypertension (CTEPH): results from an international prospective registry.

BACKGROUND: Chronic thromboembolic pulmonary hypertension (CTEPH) is often a sequel of venous thromboembolism with fatal natural history; however, many cases can be cured by pulmonary endarterectomy. The clinical characteristics and current management of patients enrolled in an international CTEPH registry was investigated. METHODS AND RESULTS: The international registry included 679 newly diagnosed (≤6 months) consecutive patients with CTEPH, from February 2007 until January 2009. Diagnosis was confirmed by right heart catheterization, ventilation-perfusion lung scintigraphy, computerized tomography, and/or pulmonary angiography. At diagnosis, a median of 14.1 months had passed since first symptoms; 427 patients (62.9%) were considered operable, 247 (36.4%) nonoperable, and 5 (0.7%) had no operability data; 386 patients (56.8%, ranging from 12.0%- 60.9% across countries) underwent surgery. Operable patients did not differ from nonoperable patients relative to symptoms, New York Heart Association class, and hemodynamics. A history of acute pulmonary embolism was reported for 74.8% of patients (77.5% operable, 70.0% nonoperable). Associated conditions included thrombophilic disorder in 31.9% (37.1% operable, 23.5% nonoperable) and splenectomy in 3.4% of patients (1.9% operable, 5.7% nonoperable). At the time of CTEPH diagnosis, 37.7% of patients initiated at least 1 pulmonary arterial hypertension-targeted therapy (28.3% operable, 53.8% nonoperable). Pulmonary endarterectomy was performed with a 4.7% documented mortality rate. CONCLUSIONS: Despite similarities in clinical presentation, operable and nonoperable CTEPH patients may have distinct associated medical conditions. Operability rates vary considerably across countries, and a substantial number of patients (operable and nonoperable) receive off-label pulmonary arterial hypertension-targeted treatments.

Exploración de la hemodinámica en la hipertensión pulmonar mediante cateterismo cardíaco derecho / Exploration of hemodynamics in pulmonary hipertension by right heart catherization

El diagnóstico final de hipertensión pulmonar se realizamediante cateterismo cardíaco derecho. Existe hipertensiónpulmonar cuando la presión media en la arteria pulmonarsupera los 25 mmHg en situación basal. Una adecuadaexploración hemodinámica permite encuadrar la hipertensiónpulmonar en algunos de los cinco grupos de la clasificaciónclínica actual. Se dan las recomendaciones para los diferentesespecialistas de cuándo efectuar el cateterismo derecho, quéparámetros se deben de registrar y como realizar e interpretaruna prueba aguda de vasorreactividad en la hipertensiónarterial pulmonar.

The final diagnosis of pulmonary hypertension is made byright heart catheterization. Pulmonary hypertension is presentwhen the average pressure in the pulmonary artery exceeds 25mm Hg at baseline. Proper hemodynamic assessment allowsclassification of pulmonary hypertension into one of five clinicalcategories. Recommendations are provided for specialists, whento conduct right heart catheterization, which parameters mustbe recorded and how to perform and interpret a test for acutevasoreactivity in pulmonary arterial hypertensio.

A randomized multicenter comparison of basiliximab and muromonab (OKT3) in heart transplantation: SIMCOR study.

BACKGROUND: Antilymphocytic antibodies have been long used for the prevention of acute rejection early after heart transplantation (HTx), but their adverse effects have limited their widespread use. Our aim was to evaluate the safety, tolerability, and efficacy of the novel anti-CD25 antibody basiliximab (BAS) compared with muromonab (OKT3). PATIENTS AND METHODS: In this multicenter study, 99 patients were randomly assigned to receive either BAS or OKT3 in the early post-HTx period. The primary endpoint was safety and tolerability. Specific safety variables were predefined for a better comparison of adverse effects. Secondary endpoints concerning anti-rejection efficacy were also evaluated. RESULTS: No adverse events related to study medication were found in the BAS group, whereas 23 were observed among patients receiving OKT3 (P<0.0001). The proportion of patients with predefined adverse events day 4 post-HTx was much higher with OKT3 than with BAS (43% vs. 4%; P<0.0001). Fever, acute pulmonary edema, hypotension, and other complications accounted for most of the difference. At 1-year follow-up, biopsy-proven rejection episodes grade>or=3A had occurred in 39.6% of BAS patients versus 40.4% of OKT3 patients (P=0.87). There were no differences in terms of severity and timing of acute rejection episodes. The number of infectious episodes, complications not related to study medication, and actuarial survival were similar in both groups. CONCLUSION: In this HTx study, induction therapy with BAS was safer and better tolerated than OKT3, without significant differences in efficacy outcomes.

[Pulmonary hypertension during exercise in toxic oil syndrome]. / Hipertensión pulmonar con el ejercicio en el síndrome del aceite tóxico.

BACKGROUND AND OBJECTIVE: Toxic oil syndrome is a risk factor for pulmonary arterial hypertension (PAH) and new cases of this entity are emerging after more than 20 years since the initial toxic oil epidemic. Abnormal elevation of pulmonary systolic pressure with exercise may be considered an early marker of PAH in populations at risk. We aimed to analyze the pulmonary systolic pressure with exercise echocardiography in toxic oil syndrome patients. PATIENTS AND METHOD: 50 toxic oil syndrome patients (cases), and 20 healthy control subjects were submitted to rest and peak exercise echocardiography (semi supine cycloergometer) measuring pulmonary systolic pressure. In toxic oil syndrome patients, pulmonary carbon monoxide diffusion capacity was also analyzed. RESULTS: Peak exercise pulmonary systolic pressure was statistically similar in cases and controls. Nevertheless, 8% of cases reached a pulmonary systolic pressure > or = 80 mmHg and this fact was associated with mild pulmonary arterial hypertension, reduced right ventricular function and abnormal pulmonary diffusion capacity in the rest study. A rest pulmonary systolic pressure cut-off value > or = 27 mmHg had a 100% sensitivity and 71% specificity to predict a peak exercise systolic pulmonary pressure > or = 80 mmHg. CONCLUSIONS: A minority of toxic oil syndrome patients develop severe pulmonary arterial hypertension during exercise. This abnormal response is associated with other markers of pulmonary vasculopathy. Further studies are needed to elucidate the relation between these findings and the likelihood to develop pulmonary arterial hypertension in the future.

Prevalencia de asma bronquial a nivel de altura y al nivel del mar / Asthma prevalence at high altitude and at sea leavel

El objetivo del presente trabajo es determinar la prevalencia de asma bronquial en Barranca (160 metros sobre el nivel mar-msnm) y en Tarma (3050 msnm). Estudios descriptivos, transversales. Se escogió una muestra aleatoria mediante el método de conglomerados. Fueron evaluados los mayores de 5 años. Para los mayores de 14 años, se consideró asmático aquel que presentase sibilancias independiente de los resfríos y disnea a esfuerzos moderados. Para los menores de 14 años, se consideró asmático al que tenía tos matutina y sibilancia a esfuerzos moderados. También se consideró asmático a todos los que tenían el diagnóstico previo. Se estudiaron a 299 personas en Barranca (155 mayores de 14 años y 144 menores de 14 años). En Tarma participaron 302 personas (160 mayores de 14 años y 142 menores de 14 años). Hubo mayor sintomatología respiratoria como tos, disnea y sibilancia en Barranca respecto a Tarma, a excepción de sibilancias en niños que fue más frecuente en Tarma. En el caso de adultos, la prevalencia de asma bronquial fue de 10.41 por ciento y 4.63 por ciento para Barranca y Tarma, mientras que en niños, fue de 9.72 por ciento y 2.81 por ciento respectivamente, dando una prevalencia global de 10.06 por ciento en Barranca y 3.75 por ciento en Tarma. La prevalencia de asma bronquial es menor en Tarma (3.75 por ciento) que en Barranca (10.06 por ciento).

Experiencia en hemorroidectomías aplicado en la Clínica Fiori / Experience in haemmorrhoidectomy at the clinica Fiori

Objetivo: Evaluar la utilidad de la cirugía ambulatoria con anestesia local en pacientes con patología hemorroidal. Material y métodos: Se realizó un estudio prospectivo, transversal, entre abril de 1996 y marzo de 1999, en el servicio de cirugía de la Clínica Fiori. Se intervinieron 260 pacientes por patología hemorroidal, de ellos 229 pacientes fueron sometidos a hemorroidectomía con anestesia local, los que fueron incluidos en el estudio. La técnica quirúrgica utilizada fue la de Milligan y Morgan con un tiempo operatorio de 20 minutos; y control a los 3 días. resultados: El 69.4 por ciento de patología hemorroidal se observó entre los 31-60 años de edad. Sólo el 1.7 por ciento (4 pacientes) presentaron complicaciones post-operatorias, 2 pacientes con sangrado leve y 2 con hemorroides residuales, ninguno resultó ser de gravedad; y el 98.3 por ciento (225 pacientes) no presentaron ningún tipo de complicaciones. No hubo accidentes anestésicos. Conclusión: La hemorroidectomía con anestesia local es efectiva y segura.