[Role of bosentan in patients with chronic venous thromboembolic pulmonary hypertension]. / Efectividad del bosentán en el tratamiento de la hipertensión pulmonar debida a tromboembolia venosa crónica.

BACKGROUND AND OBJECTIVE: Chronic thromboembolic pulmonary hypertension (CTEPH) has a dismal prognosis when there are no central pulmonary thrombi amenable to surgical thromboendarterectomy. Pulmonary vasodilators could be useful in this setting. Initial experience with bosentan in a small group of patients with CTEPH has shown favourable results on the short term (3 to 6 months), but long-term effects remain unknown. PATIENTS AND METHOD: We retrospectively describe the effects of bosentan in 6 CTEPH patients with a mean follow-up period of 15 months (range, 8-26). RESULTS: At 3-month follow-up, all patients had experienced clinical improvement, with a statistical trend towards reduced pulmonary vascular resistance [1,008 (624) dyn/sec/cm-5 versus 768 (392), p = 0.07]. Clinical improvement persisted on the long-term, [baseline NYHA functional class 3.0 (0.4) versus 2.0 (0) at the last follow-up visit, p < 0.01]. Six-minute walk-test results [baseline 230 (124) meters versus a 313 (70) at 1 year] and NTproBNP [2,225 (2,079) pg/ml versus a 1,056 (1,104) at 1 year] were also consistent with persistent beneficial effect. CONCLUSIONS: Bosentan seemed to provide long-term benefits in this small series of patients with CTEPH.

[Heart failure: etiology and approach to diagnosis]. / Etiología y evaluación diagnóstica en la insuficiencia cardíaca.

In spite of its high prevalence and the huge burden it imposes on health care systems, heart failure is a clinical syndrome that has not yet been defined satisfactorily. In actual practice, diagnosis requires the presence of typical signs and symptoms along with data from complementary tests that indicate definite cardiac dysfunction. In this article we review current concepts of the disease, stages of development, common underlying causes, and the value of different diagnostic tests. Among these tests, measurement of B-type natriuretic peptide has proved useful for population screening and the differential diagnosis of heart failure. This indicator seems to be the ideal link between the large population of patients in whom heart failure is suspected and the subgroup for whom cardiac ultrasound, the most informative test in this disease, is warranted.

[Is heart transplantation for primary cardiac sarcoma a useful therapeutic option?]. / ¿Es útil el trasplante cardíaco como tratamiento del sarcoma cardíaco primario?

Primary cardiac sarcomas are rapidly progressive malignant tumors. No good therapeutic option is known. In recent years, heart transplantation has sometimes been performed in selected patients with cardiac sarcoma.We retrospectively analyzed 8 patients with primary cardiac sarcoma referred to our center to undergo assessment for heart transplantation. After an exhaustive study of the extension of the tumor, 6 patients were added to the waiting list for heart transplantation. Heart transplantation was not performed in 3 of these patients due to evidence of extracardiac extension, but the procedure was completed in the remaining 3 patients. The median survival in intention-to-treat analysis (transplantation or a frustrated transplantation attempt) was 8.5 months. Overall, the median survival of the 3 patients who underwent transplantation (12 months) was similar to that of the 5 patients who did not (11 months).

[Mechanical cardiac assist devices and heart transplantation. Treatment indications and the current situation in Spain]. / Asistencia mecánica circulatoria y trasplante cardiaco. Indicaciones y situación en España.

Despite advances in all areas of cardiology, the incidence of heart failure increases each year. Pharmacologic treatment, cardiac resynchronization therapy, and heart failure surgery have resulted in prolonged survival in these patients and in symptomatic improvement. However, in more advanced phases of the disease, these therapeutic options become less effective. This is when heart transplantation and cardiac assist devices are the only techniques that can lengthen survival and improve quality of life. For the past 25 years, heart transplantation has been the most effective therapeutic option for patients with end-stage heart failure. The scarcity of suitable donors and the development of alternative forms of treatment that are effective in less advanced disease stages have led to heart transplantation being reserved for young heart disease patients whose life-expectancy is short. Cardiac assist devices have been shown to be effective as temporary therapy, providing a bridge to heart transplantation or recovery, or as permanent support in patients with contraindications to heart transplantation. Currently both paracorporeal and intracorporeal assist devices are available, and there are even systems that can replace the heart completely. More widespread use of these devices depends on the establishment of appropriate indications, on an improvement in the devices' cost-benefit ratios, and on a reduction in the still high rate of complications.

Ámbito de actuación de la cardiología en los nuevos escenarios clínicos. Documento de consenso de la Sociedad Española de Cardiología / The scope of cardiological competence in new clinical settings. Spanish Society of Cardiology Consensus Document

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Efectividad del bosentán en el tratamiento de la hipertensión pulmonar debida a tromboembolia venosa crónica / Role of bosentan in patients with chronic venous thromboembolic pulmonary hypertension

Fundamento y objetivo: La hipertensión pulmonar crónica causada por tromboembolia (HTPTEC) sin trombos centrales que permitan la endarterectomía quirúrgica tiene un pésimo pronóstico. Los vasodilatadores pulmonares podrían ser de utilidad en esta entidad. El bosentán ha sido utilizado en un pequeño número de pacientes con HTPTEC con efectos iniciales (3-6 meses) favorables, pero los resultados a más largo plazo son desconocidos. Pacientes y método: Descripción retrospectiva de los efectos del bosentán en 6 pacientes con HTPTEC con un seguimiento medio de 15 (intervalo, 8-26) meses. Resultados: A los 3 meses se observó mejoría clínica en todos los pacientes, con tendencia a reducción de las resistencias pulmonares (media [desviación estándar], 1.008 [624] frente a 768 [392] din/s/cm­5; p = 0,07). A largo plazo, persistía la mejoría clínica (clase funcional de la New York Heart Association [NYHA] basal, 3,0 [0,4] frente a 2,0 [0] al final del seguimiento; p < 0,01) y en la prueba de 6 min (230 [124] m la basal frente a 313 [70] m 1 año después), y se mantenía la tendencia a la disminución del péptido escindido del péptido natriurético tipo B (NT-proBNP, 2.225 [2.079] frente a 1.056 [1.104] pg/ml 1 año después). Conclusiones: El bosentán parece una alternativa válida a largo plazo en este subgrupo de pacientes con HTPTEC

Background and objective: Chronic thromboembolic pulmonary hypertension (CTEPH) has a dismal prognosis when there are no central pulmonary thrombi amenable to surgical thromboendarterectomy. Pulmonary vasodilators could be useful in this setting. Initial experience with bosentan in a small group of patients with CTEPH has shown favourable results on the short term (3 to 6 months), but long-term effects remain unknown. Patients and method: We retrospectively describe the effects of bosentan in 6 CTEPH patients with a mean follow-up period of 15 months (range, 8-26). Results: At 3-month follow-up, all patients had experienced clinical improvement, with a statistical trend towards reduced pulmonary vascular resistance [1,008 (624) dyn/sec/cm­5 versus 768 (392), p = 0.07]. Clinical improvement persisted on the long-term, [baseline NYHA functional class 3.0 (0.4) versus 2.0 (0) at the last follow-up visit, p < 0.01]. Six-minute walk-test results [baseline 230 (124) meters versus a 313 (70) at 1 year] and NTproBNP [2,225 (2,079) pg/ml versus a 1,056 (1,104) at 1 year] were also consistent with persistent beneficial effect. Conclusions: Bosentan seemed to provide long-term benefits in this small series of patients with CTEPH

Asistencia mecánica circulatoria y trasplante cardiaco. Indicaciones y situación en España / Mechanical cardiac assist devices and heart transplantation. Treatment indications and the current situation in Spain

A pesar de los avances en todos los campos de la cardiología, la incidencia de insuficiencia cardiaca aumenta cada año. El tratamiento farmacológico, los dispositivos de resincronización y la cirugía de la insuficiencia cardiaca han conseguido prolongar la supervivencia de estos pacientes y mejorar sus síntomas. Sin embargo, en las fases más avanzadas de la enfermedad, estas opciones terapéuticas llegan a ser ineficaces, y son el trasplante cardiaco (TXC) y los dispositivos de asistencia circulatoria (DAC) los que pueden permitir prolongar la supervivencia y mejorar la calidad de vida. El TXC es, desde hace 25 años, la opción terapéutica más efectiva para pacientes con insuficiencia cardiaca en fases terminales. La escasez de donantes apropiados y el desarrollo de otras alternativas terapéuticas eficaces en estadios menos avanzados hacen que este tratamiento se reserve para pacientes jóvenes que presentan cardiopatías con poca expectativa de vida a corto plazo. Los DAC han demostrado su efectividad como terapia temporal, ya sea como puente al trasplante o como puente a la recuperación, y también como soporte permanente en pacientes con contraindicaciones para TXC. Actualmente disponemos de asistencias paracorpóreas, intracorpóreas e incluso sistemas que permiten la completa sustitución del corazón. La generalización de su uso dependerá del establecimiento de unas indicaciones apropiadas, de la mejora en su relación coste-beneficio, y de la disminución de la aún importante tasa de complicaciones asociadas (AU)

Despite advances in all areas of cardiology, the incidence of heart failure increases each year. Pharmacologic treatment, cardiac resynchronization therapy, and heart failure surgery have resulted in prolonged survival in these patients and in symptomatic improvement. However, in more advanced phases of the disease, these therapeutic options become less effective. This is when heart transplantation and cardiac assist devices are the only techniques that can lengthen survival and improve quality of life. For the past 25 years, heart transplantation has been the most effective therapeutic option for patients with end-stage heart failure. The scarcity of suitable donors and the development of alternative forms of treatment that are effective in less advanced disease stages have led to heart transplantation being reserved for young heart disease patients whose life-expectancy is short. Cardiac assist devices have been shown to be effective as temporary therapy, providing a bridge to heart transplantation or recovery, or as permanent support in patients with contraindications to heart transplantation. Currently both paracorporeal and intracorporeal assist devices are available, and there are even systems that can replace the heart completely. More widespread use of these devices depends on the establishment of appropriate indications, on an improvement in the devices’ costbenefit ratios, and on a reduction in the still high rate of complications (AU)

¿Es útil el trasplante cardíaco como tratamiento del sarcoma cardíaco primario? / Is heart transplantation for primary cardiac sarcoma a useful therapeutic option?

El sarcoma cardíaco primario es un tumor maligno de evolución rápida y fatal. Se desconoce en la actualidad cuál debe ser la aproximación terapéutica ideal a esta enfermedad. El trasplante cardíaco se ha utilizado ocasionalmente como tratamiento definitivo de esta entidad. Presentamos a 8 pacientes diagnosticados de sarcoma cardíaco primario que fueron valorados para trasplante. Después del estudio de extensión, 6 pacientes fueron incluidos en lista de espera para trasplante cardíaco. En 3 pacientes, la intervención no se pudo completar al demostrarse intraoperatoriamente la extensión extracardíaca del sarcoma, y en tres pacientes el procedimiento se llevó a término. La mediana de supervivencia por intención de tratar (trasplante o intento de trasplante) fue de 8,5 meses. La mediana de supervivencia de los 3 pacientes trasplantados (12 meses) fue similar a la de los 5 pacientes no trasplantados (11 meses) (AU)