Hipertensión pulmonar tromboembólica crónica en España: una década de cambio / Chronic thromboembolic pulmonary hypertension in Spain: a decade of change

Introducción y objetivos El tratamiento de la hipertensión pulmonar tromboembólica crónica (HTPTEC) ha evolucionado en la última década. Sin embargo, apenas se dispone de información sobre el impacto de estos logros en la población general a escala nacional. Este estudio se diseñó para describir las características de los pacientes con HTPTEC en España en la última década. Métodos Se recogieron prospectivamente datos epidemiológicos, clínicos y pronósticos de los pacientes con HTPTEC consecutivos incluidos en el registro español REHAP desde el 1 de enero de 2007 al 31 de diciembre de 2018. Se evaluaron las diferencias entre diferentes periodos de tiempo, estableciendo 2013 como fecha de referencia para el análisis. Se calculó la puntuación de propensión para la intervención mediante un modelo multivariable de regresión logística. Resultados Se incluyó a 1.019 pacientes; se remitió a 659 (64,4%) a un centro nacional de referencia en HTPTEC. Del total, se seleccionó a 350 (34,3%) para cirugía y a 97 (9,6%) para tratamiento percutáneo. Entre los pacientes diagnosticados entre 2007 y 2012 hubo más frecuencia de muerte que entre los diagnosticados de 2013 en adelante (HR=1,83; IC95%, 1,07-3,15; p=0,027). En el grupo de pacientes ajustado por el modelo de puntuación de propensión, las resistencias vasculares pulmonares basales y la distancia recorrida en el test de 6 min de marcha también fueron determinantes del pronóstico (respectivamente, HR=1,24; IC95%, 1,15-1,33; p=0,011, y HR=0,93; IC95%, 0,90-0,97; p=0,001). Las tasas de supervivencia de los pacientes que se sometieron a un procedimiento intervencionista (trombendarterectomía pulmonar o angioplastia con balón de arterias pulmonares) resultaron llamativamente altas. Conclusiones Durante la última década, el diagnóstico y el pronóstico de la HTPTEC han mejorado de manera considerable. La gravedad de la enfermedad al diagnóstico determinó el perfil de riesgo. ... (AU)

Introduction and objectives Chronic thromboembolic pulmonary hypertension (CTEPH) treatment has evolved in the last decade. However, there is scarce information on the long-term impact of this progress in a real-life population at a national level. This study was designed to analyze the characteristics of CTEPH patients in Spain over the last decade. Methods We prospectively collected epidemiological, clinical, and prognostic data from CTEPH patients consecutively included in the Spanish REHAP registry from January 1, 2007, to December 31, 2018. We evaluated differences over time, establishing 2013 as the reference date for analysis. Propensity scores for interventional treatment were calculated using a multivariable logistic regression model. Results A total of 1019 patients were included; 659 (64.4%) were evaluated at a national CTEPH center. Overall, 350 patients (34.3%) were selected for surgery and 97 (9.6%) for percutaneous treatment. Patients diagnosed between 2007 and 2012 died more frequently than those diagnosed from 2013 onward (HR, 1.83; 95%CI, 1.07-3.15; P=.027). Within the subgroup of patients adjusted by propensity score, baseline pulmonary vascular resistance and the 6-minute walk test distance also determined the outcome (HR, 1.24; 95%CI, 1.15-1.33; P=.011; and HR, 0.93; 95%CI, 0.90-0.97; P=.001, respectively). High survival rates were found in patients who underwent an invasive procedure (pulmonary endarterectomy or balloon pulmonary angioplasty). Conclusions CTEPH diagnosis and prognosis have consistently improved in the last decade. Baseline disease severity determines the risk profile. Patients who undergo pulmonary endarterectomy or balloon pulmonary angioplasty have better outcomes. (AU)

Chronic thromboembolic pulmonary hypertension in Spain: a decade of change.

INTRODUCTION AND OBJECTIVES: Chronic thromboembolic pulmonary hypertension (CTEPH) treatment has evolved in the last decade. However, there is scarce information on the long-term impact of this progress in a real-life population at a national level. This study was designed to analyze the characteristics of CTEPH patients in Spain over the last decade. METHODS: We prospectively collected epidemiological, clinical, and prognostic data from CTEPH patients consecutively included in the Spanish REHAP registry from January 1, 2007, to December 31, 2018. We evaluated differences over time, establishing 2013 as the reference date for analysis. Propensity scores for interventional treatment were calculated using a multivariable logistic regression model. RESULTS: A total of 1019 patients were included; 659 (64.4%) were evaluated at a national CTEPH center. Overall, 350 patients (34.3%) were selected for surgery and 97 (9.6%) for percutaneous treatment. Patients diagnosed between 2007 and 2012 died more frequently than those diagnosed from 2013 onward (HR, 1.83; 95%CI, 1.07-3.15; P=.027). Within the subgroup of patients adjusted by propensity score, baseline pulmonary vascular resistance and the 6-minute walk test distance also determined the outcome (HR, 1.24; 95%CI, 1.15-1.33; P=.011; and HR, 0.93; 95%CI, 0.90-0.97; P=.001, respectively). High survival rates were found in patients who underwent an invasive procedure (pulmonary endarterectomy or balloon pulmonary angioplasty). CONCLUSIONS: CTEPH diagnosis and prognosis have consistently improved in the last decade. Baseline disease severity determines the risk profile. Patients who undergo pulmonary endarterectomy or balloon pulmonary angioplasty have better outcomes.

La evolución de la hipertensión arterial pulmonar a lo largo de 30 años: experiencia de un centro de referencia / Trends in pulmonary hypertension over a period of 30 years: experience from a single referral centre

Introducción y objetivos: La hipertensión arterial pulmonar (HAP) se caracteriza por aumento de resistencias vasculares pulmonares, disfunción progresiva del ventrículo derecho y muerte. A pesar de los avances, sigue asociada a alta morbimortalidad. El objetivo del estudio es describir el tratamiento de esta enfermedad y determinar factores pronósticos de pacientes con HAP tratados en un centro de referencia nacional a lo largo de 30 años. Métodos: Se estudió a 379 pacientes consecutivos diagnosticados de HAP (enero de 1984-diciembre de 2014). Se los distribuyó en 3 intervalos de tiempo: previo a 2004, 2004-2009 y 2010-2014, y se analizaron los factores pronósticos de deterioro clínico. Resultados: La mediana de edad de los pacientes es 44 años (el 68,6% eran mujeres) y estaban en clase funcional III-IV el 72%. Se observó un incremento en etiologías más complejas: enfermedad venooclusiva e hipertensión portopulmonar en el último periodo. La terapia combinada de inicio aumentó (el 5% previo a 2004 frente al 27% posterior a 2010; p < 0,05). El análisis multivariable mostró como factores independientes de deterioro clínico edad, sexo, etiología y variables combinadas (p < 0,05). La supervivencia libre de muerte o trasplante al primero, el tercero y el quinto año fueron del 92,2, el 80,6 y el 68,5% respectivamente. La mediana de supervivencia fue 9 años (intervalo de confianza del 95%, 7,532-11,959). Conclusiones: La HAP es una enfermedad heterogénea y compleja. La mediana de supervivencia libre de muerte o trasplante en nuestra serie es 9 años. La estructura de una unidad multidisciplinaria de HAP debe adaptarse con rapidez a los cambios que se producen en el tiempo incorporando nuevas técnicas diagnósticas y terapéuticas (AU)

Introduction and objectives: Pulmonary arterial hypertension (PAH) is characterized by increased pulmonary vascular resistance, right ventricular dysfunction and death. Despite scientific advances, is still associated with high morbidity and mortality. The aim is to describe the clinical approach and determine the prognostic factors of patients with PAH treated in a national reference center over 30 years. Methods: Three hundred and seventy nine consecutive patients with PAH (January 1984 to December 2014) were studied. Were divided into 3 periods of time: before 2004, 2004-2009 and 2010-2014. Prognostic factors (multivariate analysis) were analyzed for clinical deterioration. Results: Median age was 44 years (68.6% women), functional class III-IV: 72%. An increase was observed in more complex etiologies in the last period of time: Pulmonary venooclusive disease and portopulmonary hypertension. Upfront combination therapy significantly increased (5% before 2004 vs 27% after 2010; P < .05). Multivariate analysis showed prognostic significance in age, sex, etiology and combined clinical variables as they are independent predictors of clinical deterioration (P < .05). Survival free from death or transplantation for the 1st, 3rd and 5th year was 92.2%, 80.6% and 68.5% respectively. The median survival was 9 years (95% confidence interval, 7.532-11.959) Conclusions: The PAH is a heterogeneous and complex disease, the median survival free from death or transplantation in our series is 9 years after diagnosis. The structure of a multidisciplinary unit PAH must adapt quickly to changes that occur over time incorporating new diagnostic and therapeutic techniques (AU)

Trends in Pulmonary Hypertension Over a Period of 30 Years: Experience From a Single Referral Centre.

INTRODUCTION AND OBJECTIVES: Pulmonary arterial hypertension (PAH) is characterized by increased pulmonary vascular resistance, right ventricular dysfunction and death. Despite scientific advances, is still associated with high morbidity and mortality. The aim is to describe the clinical approach and determine the prognostic factors of patients with PAH treated in a national reference center over 30 years. METHODS: Three hundred and seventy nine consecutive patients with PAH (January 1984 to December 2014) were studied. Were divided into 3 periods of time: before 2004, 2004-2009 and 2010-2014. Prognostic factors (multivariate analysis) were analyzed for clinical deterioration. RESULTS: Median age was 44 years (68.6% women), functional class III-IV: 72%. An increase was observed in more complex etiologies in the last period of time: Pulmonary venooclusive disease and portopulmonary hypertension. Upfront combination therapy significantly increased (5% before 2004 vs 27% after 2010; P < .05). Multivariate analysis showed prognostic significance in age, sex, etiology and combined clinical variables as they are independent predictors of clinical deterioration (P < .05). Survival free from death or transplantation for the 1st, 3rd and 5th year was 92.2%, 80.6% and 68.5% respectively. The median survival was 9 years (95% confidence interval, 7.532-11.959) CONCLUSIONS: The PAH is a heterogeneous and complex disease, the median survival free from death or transplantation in our series is 9 years after diagnosis. The structure of a multidisciplinary unit PAH must adapt quickly to changes that occur over time incorporating new diagnostic and therapeutic techniques.

Coronary artery perforation: don't rush, IVUS may be useful.

Coronary artery perforation (CAP) is a rare but potentially fatal complication of percutaneous coronary intervention. Polytetrafluoroethylene-covered stents prevent blood leakage between struts with a high rate of success. However, they lack elasticity and rapid and correct deployment is difficult. They have also a higher rate of stent restenosis and thrombosis. For these reasons, optimal deployment is essential. Although severe CAP needs an emergent solution, after stabilizing the patient, intracoronary imaging techniques may be useful to ensure correct expansion and reduce further adverse events. We present a case that shows the potential role of intravascular ultrasound in the resolution of a CAP.

Valor de la ecografía intracoronaria en la trombosis de stent / Role of intravascular ultrasound in stent thrombosis

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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.

Métodos de imagen en la hipertensión pulmonar tromboembólica crónica / Imaging tests in chronic thromboembolic pulmonary hypertension

Las técnicas de imagen son el pilar del diagnóstico de la hipertensión pulmonar trobmboembólica crónica (HPTEC). Actualmente, se dispone de una serie de técnicas de imagen de indudable utilidad: gammagrafía ventilación/perfusión, ecocardiografía, tomografía computarizada (TC) multicorte, resonancia magnética y arteriografía pulmonar, aunque no todas están indicadas de rutina en todos los pacientes. La gammagrafía y la ecografía se utilizan como método de cribado de HPTEC tras un tromboembolismo pulmonar agudo. La TC con multidetector (TCMD) es una herramienta eficaz en el estudio de la HPTBEC, ya que demuestra la localización, extensión y características de los trombos arteriales centrales y periféricos, además de ofrecer un estudio del vaso distal a la obstrucción. Permite valorar otros hallazgos que pueden condicionar el tratamiento médico o quirúrgico, como circulación sistémica colateral, patología parenquimatosa coincidente, patología coronaria o cardíaca. Es un método no invasivo y bien tolerado por los pacientes, por lo que puede emplearse en el seguimiento de la enfermedad o para valorar resultados posquirúrgicos. La arteriografía pulmonar ha precedido al resto de técnicas de imagen, siendo la técnica de elección para el diagnóstico de HPTEC durante muchos años. Un acceso limitado a esta técnica, un riesgo bajo pero existente, y el desarrollo de técnicas de diagnóstico no invasivas, fundamentalmente la TCMD, han hecho que en la actualidad su principal indicación sea la valoración de la accesibilidad quirúrgica en los pacientes en los que la cirugía de tromboendarterectomía pulmonar se considera una opción terapéutica. Realizada por hemodinamistas expertos, de forma estandarizada en el laboratorio de hemodinámica, es un procedimiento seguro, incluso en pacientes con hipertensión pulmonar severa (AU)

Imaging tests are the cornerstone of the diagnosis of chronic thromboembolic pulmonary hypertension (CTEPH). A series of imaging tests of unquestionable utility is currently available: ventilation-perfusion scintigraphy, echocardiography, multislice computed tomography (CT), magnetic resonance imaging, and pulmonary arteriography. However, not all of these techniques are routinely indicated in all patients. Scintigraphy and echography are used to screen for CTEPH after an acute pulmonary thromboembolism. Multidetector CT is effective in the study of CTEPH since this procedure reveals the localization, extension and characteristics of the central and peripheral arterial thrombi as well as providing data on the vessel distal to the obstruction. Other findings that can affect medical or surgical treatment can be assessed with this technique, such as collateral systemic circulation, concurrent parenchymatous abnormalities, and coronary or cardiac disease. Multidetector CT is a non-invasive method that is well tolerated by patients and consequently can be used in the follow-up of the disease or to evaluate postsurgical results. Pulmonary arteriography predated other imaging techniques and was the technique of choice for the diagnosis of CTEPH for many years. Because of limited access to this procedure, a low but nevertheless present risk, and the development of non-invasive diagnostic techniques, mainly multidetector CT, the main indication of pulmonary arteriography is currently assessment of surgical accessibility in candidates for pulmonary thromboendarterectomy. When performed by expert hemodynamists using a standardized technique in the hemodynamic laboratory, this procedure is safe even in patients with severe pulmonary hypertension (AU)