María Teresa Subirana

Estándares asistenciales en hipertensión pulmonar* Documento de consenso elaborado por la Sociedad Española de Neumología y Cirugía Torácica (SEPAR) y la Sociedad Española de Cardiología (SEC)

En los ultimos anos se han producido importantes avances en el diagnostico y tratamiento de la hipertension pulmonar que han logrado una mejoria significativa en la supervivencia de esta enfermedad. Estas innovaciones se han recogido en guias de practica clinica basadas en la evidencia elaboradas por las sociedades cientificas. Sin embargo, no se incluyen en ellas, por falta de evidencia cientifica concluyente, algunos aspectos que inciden en la practica asistencial. Conscientes de ello, la Sociedad Espanola de Neumologia y Cirugia Toracica (SEPAR) y la Sociedad Espanola de Cardiologia (SEC) han promovido la elaboracion de un documento de consenso para definir en nuestro medio los estandares de calidad adecuados para el diagnostico y tratamiento de la hipertension pulmonar en sus diversas formas de presentacion, asi como la via clinica y las directrices basicas de la organizacion asistencial del cuidado de estos pacientes, haciendo especial hincapie en los requisitos y funciones de las unidades de referencia. Para su redaccion la SEPAR y la SEC designaron a un grupo de trabajo formado por expertos en los distintos aspectos de la enfermedad. Para la elaboracion del documento se han utilizado las guias clinicas internacionales existentes, la revision de la evidencia cientifica disponible y el debate en panel entre los expertos. El documento final, aprobado por todos los participantes, ha sido evaluado por revisores externos.

Guías de práctica clínica de la Sociedad Española de Cardiología en pruebas de esfuerzo

La mayor parte de las pruebas de esfuerzo se realizan a pacientes adultos con cardiopatia isquemica en estudio o ya conocida. En los ultimos anos se ha producido la incorporacion de las tecnicas de imagen en este campo, mejorando asi la informacion aportada por la prueba de esfuerzo convencional. Pero cada vez existen mas situaciones que escapan a esta norma general, tanto en sujetos sanos (asintomaticos, atletas, discapacitados, etc.) como en pacientes con cardiopatias diferentes de la isquemica (insuficiencia cardiaca congestiva avanzada, hipertension, trastornos del ritmo, cardiopatias congenitas etc.). Todos estos aspectos justifican un documento de consenso en Espana, necesariamente multidisciplinario. Este documento revisa en profundidad la metodologia de las pruebas de esfuerzo convencionales, sin olvidar las realizadas con determinacion de consumo de oxigeno. El papel de esta exploracion en el manejo de la cardiopatia isquemica, asi como las aplicaciones de las tecnicas de imagen al campo del estres, ocupan un lugar fundamental en esta revision. Por ultimo, se analiza la utilidad de las pruebas de esfuerzo en diversas cardiopatias no isquemicas y en diferentes poblaciones de sujetos sanos.

Standards of care in pulmonary hypertension.

Substantial progress in the diagnosis and treatment of patients with pulmonary hypertension in recent years has led to significant improvement in survival. Evidence-based clinical practice guidelines issued by scientific societies reflect these new developments. However, certain clinically relevant issues have not been covered in consensus guidelines because of the lack of conclusive scientific evidence. Therefore, the Spanish Society of Pulmonology and Thoracic Surgery (SEPAR) and the Spanish Society of Cardiology (SEC) have promoted the present consensus statement in order to define national standards of care in the evaluation and management of pulmonary hypertension in its various forms, as well as to outline a clinical pathway and the basic principles for organizing health care in this clinical setting, with special emphasis on the requirements for and functions of specialized referral units. To prepare the statement, SEPAR and SEC formed a task force composed of national experts in various aspects of pulmonary hypertension. The resulting consensus is based on international clinical guidelines, a review of available scientific evidence, and panel discussion among the task force members. The final statement, approved by all participants, underwent external review.

Estándares asistenciales en hipertensión pulmonar

En los ultimos anos se han producido importantes avances en el diagnostico y tratamiento de la hipertension pulmonar que han logrado una mejoria significativa en la supervivencia de esta enfermedad. Estas innovaciones se han recogido en guias de practica clinica basadas en la evidencia elaboradas por las sociedades cientificas. Sin embargo, no se incluyen en ellas, por falta de evidencia cientifica concluyente, algunos aspectos que inciden en la practica asistencial. Conscientes de ello, la Sociedad Espanola de Neumologia y Cirugia Toracica (SEPAR) y la Sociedad Espanola de Cardiologia (SEC) han promovido la elaboracion de un documento de consenso para definir en nuestro medio los estandares de calidad adecuados para el diagnostico y tratamiento de la hipertension pulmonar en sus diversas formas de presentacion, asi como la via clinica y las directrices basicas de la organizacion asistencial del cuidado de estos pacientes, haciendo especial hincapie en los requisitos y funciones de las unidades de referencia. Para su redaccion la SEPAR y la SEC designaron a un grupo de trabajo formado por expertos en los distintos aspectos de la enfermedad. Para la elaboracion del documento se han utilizado las guias clinicas internacionales existentes, la revision de la evidencia cientifica disponible y el debate en panel entre los expertos. El documento final, aprobado por todos los participantes, ha sido evaluado por revisores externos.Consensus Statement of the Spanish Society of Pulmonology and Thoracic Surgery (SEPAR) and the Spanish Society of Cardiology (SEC). Substantial progress in the diagnosis and treatment of patients with pulmonary hypertension in recent years has led to significant improvement in survival. Evidence-based clinical practice guidelines issued by scientific societies reflect these new developments. However, certain clinically relevant issues have not been covered in consensus guidelines because of the lack of conclusive scientific evidence. Therefore, the Spanish Society of Pulmonology and Thoracic Surgery (SEPAR) and the Spanish Society of Cardiology (SEC) have promoted the present consensus statement in order to define national standards of care in the evaluation and management of pulmonary hypertension in its various forms, as well as to outline a clinical pathway and the basic principles for organizing health care in this clinical setting, with special emphasis on the requirements for and functions of specialized referral units. To prepare the statement, SEPAR and SEC formed a task force composed of national experts in various aspects of pulmonary hypertension. The resulting consensus is based on international clinical guidelines, a review of available scientific evidence, and panel discussion among the task force members. The final statement, approved by all participants, underwent external review.

Long term outcome of mechanical valve prosthesis in the pulmonary position

OBJECTIVES Assessment of the long term outcome of mechanical valve prosthesis at pulmonary position in a population of grown-up congenital heart disease patients from a tertiary referral center. METHODS From 1977 to 2007, 22 consecutive patients underwent a total of 25 pulmonary valve replacements with mechanical prosthesis. The most frequent underlying cardiac condition was tetralogy of Fallot (n=16, 64%) and the mean age at the time of pulmonary valve replacement was 32 ± 11 years (range 14-50 years). RESULTS The postoperative mortality rate was 4% (n=1) with no late deaths documented after a mean follow-up of 7.6 ± 7.6 years (range 0.29-24 years). No major bleeding episodes occurred. Three patients presented with valve thrombosis in the setting of long term anticoagulation withdrawal and required valve re-replacement. Two of these patients, both with poor right ventricular function and overt clinical signs of right heart failure at the time of valve re-replacement, experienced further episodes of thrombosis despite correct anticoagulation. All episodes resolved with thrombolysis. After addition of antiplatelet treatment in one case and anticoagulation self-control, in the other, no further thrombosis has been documented. CONCLUSIONS Mechanical valve prosthesis may be an alternative to tissue valve prosthesis in patients with congenital heart disease requiring pulmonary valve replacement. Optimal anticoagulation is crucial and additional antiplatelet treatment should be considered. Our data also suggest that patients with severe right ventricular dysfunction and congestive heart failure might be at particular risk for valve thrombosis.

Correlation between myocardial fibrosis and the occurrence of atrial fibrillation in hypertrophic cardiomyopathy: a cardiac magnetic resonance imaging study.

Cardiac magnetic resonance imaging (CMR) in hypertrophic cardiomyopathy (HCM) often shows delayed contrast enhancement (DE) representing regions of focal myocardial fibrosis. Atrial fibrillation (AF) is a commonly reported complication of HCM. We determined the relationship between the presence of left ventricular myocardial fibrosis (LVMF) detected by DE-CMR and the occurrence AF in a series of patients with HCM. 67 patients with HCM (47 males; mean age 50.1+/-18.5 years) were studied by CMR measuring mass of LVMF, left ventricular mass, volume and function, and left atrial (LA) area. AF was present in 17 (25%) patients. LVMF was observed in 57% of patients. AF was significantly more frequent in patients who also showed LVMF, compared with the group without LVMF (42.1% vs. 3.4%, respectively; p<0.0001). LA size was larger in patients showing DE (LA area: 37.4+/-11.1 vs. 25.9+/-6.8 cm(2); respectively, p=0.0001). AF in HCM is related with myocardial fibrosis detected by DE-CMR and dilatation of the LA. This fact adds to the proven adverse prognostic value of myocardial fibrosis in HCM, thus, reinforcing the usefulness of this technique in the assessment of these patients.

Cardiopatías congénitas: presente y futuro

Rev Esp Cardiol. 2005;58(12):1381-4 1381 De todos es conocido el importante desarrollo que el mundo de la cardiologia ha experimentado en los ultimos anos pero, sin duda, ello ha tenido especial repercusion en el mundo de las cardiopatias congenitas. Desde que, en el ano 1938, Robert Gross practico la primera ligadura de un conducto arterioso persistente, en 1944, William Blalock, bajo la presion de Helen Taussig, llevo a cabo la creacion de la primera fistula sistemico-pulmonar en un nino con una cardiopatia congenita cianotica (tetralogia de Fallot), y en 1945, Gross y Crafoord, trabajando de forma independiente, repararon una coartacion aortica, el tratamiento de los pacientes con una cardiopatia congenita ha evolucionado de manera espectacular. Ha nacido una nueva poblacion de adolescentes y adultos con una cardiopatia congenita mas o menos reparada (practicamente nunca curada) y con necesidad de un control cardiologico especializado. Tenemos delante un nuevo reto, que prevemos de no facil solucion, sobre todo si tenemos en cuenta que en los ultimos anos, y puesto que las cardiopatias congenitas eran un problema basicamente pediatrico, este tipo de enfermedades se ha concentrado en los servicios de pediatria, y la mayoria de los cardiologos de adultos se han olvidado de su existencia. Es necesario reestructurar en profundidad el sistema sanitario, para lo que es imprescindible la creacion de unidades y/o programas destinados al control y el manejo de estos pacientes, la formacion de especialistas en dicha materia que trabajen en estrecha colaboracion con los cardiologos y cirujanos cardiacos pediatricos y, sobre todo, es necesario dotar a los servicios de cardiologia de los recursos adecuados para afrontar esta nueva tarea. Pero ?cuantos cardiologos es necesaED I TO R I A L E S

Unusual case of new‐onset heart failure due to cor triatriatum sinister

We report the case of a 30‐year old man who came to the emergency department of our hospital with acute left heart failure, and was diagnosed with a rare congenital anomaly (cor triatriatrum sinister), which can mimic a severe mitral stenosis. Cor triatriatum sinister is a rare anomaly (0.1% of all cases of congenital heart disease) that is seldom diagnosed in adult patients. The hallmark of this congenital defect is the presence of a fibromuscular membrane that divides the left atrium (LA) into two chambers: a postero‐superior chamber into which the pulmonary veins drain and an infero‐anterior chamber (true LA) containing the mitral valve and atrial appendage. Both chambers communicate through a membrane in which one or more drain holes can be found. When the hole is significantly obstructive, it results in increased venous and arterial pressures. Even though the definitive treatment of cor triatriatum is the surgical excision of the membrane, we present a balloon dilatation case with a good response to percutaneous therapy, both initially and in the ensuing months.

Afectación valvular tricuspídea y pulmonar en un caso de síndrome carcinoide

En el tumor carcinoide la presencia de metastasis hepaticas y de serotonina en plasma son los responsables del sindrome carcionide. Presentamos un caso de afectacion tricuspidea y pulmonar secundaria a este sindrome. El ecocardiograma oriento el diagnostico al observar en la proyeccion subcostal la presencia de nodulos hepaticos hiperrefringentes anadidos a la valvulopatia descrita.

Detection and Quantification of Myocardial Fibrosis in Hypertrophic Cardiomyopathy by Contrast-Enhanced Cardiovascular Magnetic Resonance

INTRODUCTION AND OBJECTIVES Severity of hypertrophic cardiomyopathy has been associated with the amount of myocardial fibrosis in autopsy studies. Cardio-vascular magnetic resonance allows, by means of the delayed contrast-enhancement technique, an in vivo detection of focal myocardial fibrosis. Our aim was to study myocardial fibrosis in patients with hypertrophic cardiomyopathy by means of contrast-enhance cardio-vascular magnetic resonance. METHODS 43 patients (30 males; mean age 47 [18] years) were studied by cardio-vascular magnetic resonance. In all patients left ventricular function and mass was analyzed. Total mass of myocardial fibrosis, as identified by delayed contrast-enhancement, was also calculated. RESULTS In 63% of patients some degree of myocardial delayed contrast-enhancement was observed, total mass of myocardial fibrosis ranging between 1 and 59 g (mean: 17 g). There was a positive correlation between the amount of myocardial fibrosis and the degree of hypertrophy. Maximal wall thickness was higher in patients with myocardial fibrosis (23 [7] vs 18 [4] mm, respectively, P=.04). Familial cases were also more prevalent among this group (48% vs 13%, respectively), as well as conventional clinical risk factors. CONCLUSIONS Myocardial fibrosis as detected by contrast-enhanced cardio-vascular magnetic resonance is highly prevalent in hypertrophic cardiomyopathy patients, particularly in familial cases with severe hypertrophy and associated risk factors.