Utilidad de la ergoespirometría en el diagnóstico precoz de hipertensión pulmonar en pacientes con esclerodermia / Role of cardiopulmonary exercise test in early diagnosis of pulmonary hypertension in scleroderma patients

Introducción: La hipertensión arterial pulmonar (HAP) es una enfermedad grave y progresiva, con elevada mortalidad. El diagnóstico precoz e inicio de tratamiento temprano mejora el pronóstico. Los pacientes con esclerodermia tienen riesgo elevado de desarrollar HAP. La única estrategia de cribado establecida y validada (ecocardiograma y/o algoritmo DETECT) reconoce la enfermedad cuando ya está avanzada, a pesar de encontrarse en una fase preclínica. La ergoespirometría (CPET) detecta afectación vascular pulmonar en fases más precoces.MétodosEstudio prospectivo de los 52 pacientes consecutivos diagnosticados de esclerodermia en nuestra área sanitaria, durante 2 años (2018 y 2019). A todos se les realizó CPET, además del cribado sistemático anual. Se comparó la sensibilidad para detectar HAP del cribado actual frente a la CPET. Para confirmar la presencia de HAP se realizó un cateterismo cardiaco derecho (CCD). En caso de mantener la sospecha de HAP en CPET, pero con CCD en reposo no confirmatorio, se continuó con CCD de esfuerzo.ResultadosSe realizaron 52 CPET, de las cuales 16 indicaron HAP. El CCD de reposo confirmó HAP en 5 pacientes y el CCD de esfuerzo en otros 7 (sensibilidad diagnóstica de CPET junto con el cateterismo de reposo y esfuerzo del 100%). De esos 16 pacientes, DETECT habría identificado 10, de los cuales el CCD de reposo confirmó HAP en 3 y el de esfuerzo en otros 2 (sensibilidad del algoritmo diagnóstico establecido según guías del 70%).ConclusionesLa CPET y el CCD de esfuerzo podrían reconocer HAP más precozmente que el cribado establecido en pacientes con esclerodermia, permitiendo adelantar el diagnóstico. (AU)

Introduction: Pulmonary arterial hypertension (PAH) is a severe, high mortality and progressive disease. Early diagnosis and treatment improves the prognosis. Patients with scleroderma disease presents high risk of developing PAH. Established screening strategies – echocardiogram and DETECT algorithm – recognize the disease when it is already advanced. Cardiopulmonary exercise testing (CPET) detects pulmonary vascular injury in earlier stages.MethodsProspective study of 52 consecutive patients diagnosed of scleroderma in our health area, during 2 years (2018 and 2019). All of them undergo CPET, in addition to the annual systematic screening. Sensitivity of current PAH screening is compared to CPET. To confirm the presence of PAH, right heart catheterization (RHC) is performed. In case of suspected PAH in CPET, but non-confirmatory right heart catheterization at rest, patients carried out exercise RHC.ResultsFifty-two CPET were performed, of which 16 suggested PAH. Resting RHC confirmed PAH in 5 patients and exercise RHC in 7 (diagnostic sensitivity of CPET together with rest and exercise catheterization of 100%). Of these 16 patients, DETECT had identified 10, of whom resting RHC confirmed PAH in 3 and exercise RHC in 2 (guideline-based diagnostic algorithm sensitivity 70%).ConclusionsCPET and exercise RHC could detect PAH earlier than established screening in patients with scleroderma disease, allowing early diagnosis. (AU)

[Role of cardiopulmonary exercise test in early diagnosis of pulmonary hypertension in scleroderma patients]. / Utilidad de la ergoespirometría en el diagnóstico precoz de hipertensión pulmonar en pacientes con esclerodermia.

INTRODUCTION: Pulmonary arterial hypertension (PAH) is a severe, high mortality and progressive disease. Early diagnosis and treatment improves the prognosis. Patients with scleroderma disease presents high risk of developing PAH. Established screening strategies - echocardiogram and DETECT algorithm - recognize the disease when it is already advanced. Cardiopulmonary exercise testing (CPET) detects pulmonary vascular injury in earlier stages. METHODS: Prospective study of 52 consecutive patients diagnosed of scleroderma in our health area, during 2 years (2018 and 2019). All of them undergo CPET, in addition to the annual systematic screening. Sensitivity of current PAH screening is compared to CPET. To confirm the presence of PAH, right heart catheterization (RHC) is performed. In case of suspected PAH in CPET, but non-confirmatory right heart catheterization at rest, patients carried out exercise RHC. RESULTS: Fifty-two CPET were performed, of which 16 suggested PAH. Resting RHC confirmed PAH in 5 patients and exercise RHC in 7 (diagnostic sensitivity of CPET together with rest and exercise catheterization of 100%). Of these 16 patients, DETECT had identified 10, of whom resting RHC confirmed PAH in 3 and exercise RHC in 2 (guideline-based diagnostic algorithm sensitivity 70%). CONCLUSIONS: CPET and exercise RHC could detect PAH earlier than established screening in patients with scleroderma disease, allowing early diagnosis.

Portopulmonary hypertension: prognosis and management in the current treatment era - results from the REHAP registry.

BACKGROUND: Portopulmonary hypertension (PoPH) is a rare condition with poorer survival compared to idiopathic/familial pulmonary arterial hypertension (IPAH/FPAH). AIMS: To compare the characteristics, survival, prognostic factors and management of PoPH and IPAH/FPAH patients and to assess the impact of treatment on survival of PoPH patients. METHODS: Analysis of data of prevalent and incident PoPH patients enrolled in the Spanish registry of PAH (REHAP) from January 1998 to December 2017 and comparison with IPAH/FPAH patients. Variables analysed: patient and disease (PAH and liver) characteristics, first-line PAH-targeted therapy, causes of death, prognostic factors and survival (according to aetiology and treatment in PoPH patients). RESULTS: Compared to IPAH/FPAH patients (n = 678), patients with PoPH (n = 237) were predominantly men, older and had better functional class and higher prevalence of ascites. Haemodynamics were better. Biomarkers for heart failure were worse. Age- and sex-adjusted 5-year survival rate from diagnosis was 49.3% for PoPH patients and 68.7% for IPAH patients (P < 0.001). Treated PoPH had better survival than non-treated. PAH- and liver-related causes accounted for 30.2% and 24.7% of deaths in PoPH patients. PoPH patients were less likely to receive first-line PAH-targeted therapy and this was associated with greater mortality. Increasing age, worse exercise capacity and ascites were independent prognostic factors of poorer survival; first-line oral monotherapy was associated with improved survival. Eight (3.4%) PoPH patients underwent liver transplantation. CONCLUSIONS: PoPH patients are undertreated and show poorer survival than IPAH/FPAH patients. First-line treatment with PAH-targeted therapy was associated with better survival. Presence of ascites was a predictor of mortality.

Right Heart Catheterization Further Confirms Successful Transition from Parenteral Prostanoid to Oral Selexipag / El cateterismo cardíaco derecho confirma con éxito el cambio de prostanoides parenterales a selexipag vía oral

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Living With Severe Pulmonary Arterial Hypertension Without an Infusion Pump? Selexipag has a Role to Play / ¿Viviendo con hipertensión arterial pulmonar grave sin una bomba de infusión? Selexipag tiene un papel

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Real-life experience of inhaled iloprost for patients with pulmonary arterial hypertension: Insights from the Spanish REHAP registry.

INTRODUCTION: REHAP is a voluntary, observational Spanish registry of patients with pulmonary arterial hypertension. We analyzed the experience (use and effectiveness) with inhaled iloprost (inh-ILO) in real-life conditions during a 3-year period. METHODS: Patients included were those with PAH ≥14 years recruited during 1998-2016 who had received inh-ILO. Variables were collected at the beginning of treatment (0 ±â€¯3 months) and 12 ±â€¯3/36 ±â€¯6 months follow-up. Effectiveness was assessed in the intent-to-treat population as changes in functional class and/or physical performance and transplant-free survival from the beginning of treatment. Stopping inh-ILO-related survival was also assessed. Subanalyses included treatment strategy (first-line therapy -monotherapy or upfront combination- or sequential therapy) and risk of clinical worsening/death. RESULTS: Inh-ILO was the most frequently used prostanoid in Spain, rendering 267 patients eligible for analysis. Median age was 54 years; 61% were WHO FC III. Sixty (23%) patients started inh-ILO as monotherapy, 27 (10%) as upfront combination and 180 (67%) sequentially. At 3-year follow-up significant clinical improvements were observed; however, transplant-free survival rate was 54%, being poorer in patients at high risk (63% vs. 85% in low risk patients; P < 0.001) and similar in the three treatment strategies. Only 25% patients remained on inh-ILO. Three-year after stopping inh-ILO-related survival rate was 24.7%. CONCLUSION: Data from the REHAP collected during 3 years shows that inh-ILO has low effectiveness independently of the treatment strategy used, with a 3-year survival rate of 54% despite significant clinical improvements, probably due to the use in high-risk patients. Discontinuation rate was as high as 75%.

Selección de lo mejor del año 2017 en hipertensión pulmonar / Selection of the best of 2017 in pulmonary hypertension

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Rotura aórtica de origen traumático / No disponible

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Importancia pronóstica de la frecuencia cardiaca en la población general y en pacientes con enfermedad arterial coronaria e hipertensión arterial. Posibles mecanismos / Prognostic significance of heart rate in the general population and in patients with coronary artery disease and hypertension. Possible mechanisms

Numerosos estudios epidemiológicos han demostrado que hay relación entre el aumento de la frecuencia cardiaca y el incremento de la mortalidad total y cardiovascular tras ajustar por otros factores de riesgo cardiovascular. La mayoría de los estudios ha analizado poblaciones generales, mientras que sólo unos pocos han considerado poblaciones de pacientes hipertensos o con enfermedad coronaria estable. Esta relación entre aumento de la frecuencia cardiaca y morbimortalidad es más evidente en los varones que en las mujeres. Aunque el aumento de la frecuencia cardiaca puede ser un marcador de alguna enfermedad subyacente, lo que podría explicar la asociación con incremento de la mortalidad, lo cierto es que ésta no parece ser la causa. Hay evidencias de que el aumento de la frecuencia cardiaca se asocia con aumento de la actividad del sistema simpático, con aumento de la presión arterial, con obesidad y síndrome metabólico y con progresión de la aterosclerosis. De igual forma, se ha observado asociación entre el incremento de la frecuencia cardiaca y la rotura de la placa aterosclerótica coronaria. Dada esta información, parece razonable que los clínicos comencemos a valorar la importancia de la frecuencia cardiaca como un factor de riesgo independiente. Futuros estudios nos indicarán si la reducción selectiva de la frecuencia cardiaca mejora el pronóstico de los pacientes con frecuencia cardiaca elevada (AU)

Numerous epidemiological studies have shown that a raised heart rate is associated with higher overall and cardiovascular mortality, once other cardiovascular risk factors have been adjusted for. Most of these studies have involved the general population; only a few concerned hypertensives and patients with stable coronary artery disease. The association between a raised heart rate and increased morbidity and mortality is more marked in men than women. Although an increase in heart rate could be a marker of underlying disease, which would explain the association with increased mortality, it is clear that this does not appear to be the case. On the contrary, there is evidence that a raised heart rate is linked to increased sympathetic activity, high blood pressure, obesity, metabolic syndrome, and atherosclerosis progression. Similarly, it has been shown that a raised heart rate is also associated with the disruption of coronary atherosclerotic plaque. Given these findings, it is reasonable that clinicians should now recognize heart rate as a useful independent cardiovascular risk factor. Future studies will tell us whether targeted reductions in heart rate improve prognosis in patients in whom it is raised (AU)

Pilot assessment of the response of several pulmonary hemodynamic variables to sublingual sildenafil in candidates for heart transplantation.

OBJECTIVE: To determine the acute vasodilator effect of sublingual sildenafil in heart transplant candidates with severe pulmonary hypertension due to severe left ventricular dysfunction (LVD). BACKGROUND: Pulmonary hypertension confers an increased risk of early graft failure. PATIENTS AND METHODS: Seven patients, (mean age of 53+/-8) with severe LVD (mean EF: 19+/-1.7%, functional class III-IV) due to coronary artery disease, dilated cardiomyopathy and valvulopathy were evaluated for heart transplant. All patients presented a mean transpulmonary gradient >12 mmHg and pulmonary vascular resistances >2.5 W.U., despite full treatment for advanced heart failure. The following hemodynamic data were obtained at basal state and then 15, 30 and 45 min after administration of 100 mg of sublingual sildenafil: right atrial, mean pulmonary artery pressure (mPAP), mean pulmonary capillary wedge pressures, mean transpulmonary gradient (mTPG), blood pressure, cardiac output, pulmonary vascular resistances (PVR) and systemic vascular resistances. Sublingual sildenafil was given without changing the previous treatment of heart failure. RESULTS: After 30 min of sublingual sildenafil, mPAP decreased from 37 (28-61) to 30 (16-42) mmHg and PVR decreased from 5.2 (1.9-13.8) to 2.5 (1.4-3.9) W.U. after 45 min. Mean TPG decreased from 19 (16-33) to 12 (8-14) mmHg at 45 min. Mean pulmonary capillary wedge pressure, cardiac output, systemic vascular resistances and mean blood pressure were unchanged. Sublingual sildenafil was well tolerated, with only transient facial flushing in 4 patients and mild headache in 2. CONCLUSIONS: Based on this initial study, sublingual sildenafil may be a useful alternative drug to perform acute vasodilator test in heart transplant candidates with significant pulmonary hypertension due to severe LVD. Nevertheless, further studies are warranted to confirm our results.

[Treatment of HIV-associated pulmonary hypertension with treprostinil]. / Tratamiento de la hipertensión pulmonar asociada a la infección por VIH con treprostinil.

BACKGROUND AND OBJECTIVE: The treatment of pulmonary hypertension associated with infection by human immunodeficiency virus has not been well defined. Treprostinil is a prostacyclin analogue that has recently been shown to be useful for the treatment of pulmonary hypertension, whether primary, secondary to congenital heart disease, or associated with collagen disease, in a 12-week, double-blind study. We report the results of a one-year follow-up of three patients with pulmonary hypertension associated with human immunodeficiency virus infection who are being treated with treprostinil at our center. PATIENTS AND METHOD: After secondary causes of pulmonary hypertension were excluded by a routine work-up, patients started treatment with subcutaneous prostacyclin (treprostinil) with progressive up-titration of the dose. Functional status and effort capacity were assessed every three months and an echocardiographic study was performed every six months. RESULTS: All patients showed improvement in clinical status, as shown by the NYHA functional class and the results of the six-minute walking test (increase of at least 75 meters). All the patients remain alive after one year of follow-up. Echocardiographic systolic pulmonary pressure decreased in two patients. No serious adverse events were observed. CONCLUSIONS: Subcutaneous prostacyclin (treprostinil) seems to be an effective and safe therapeutic option for the treatment of pulmonary hypertension associated with human immunodeficiency virus infection.

Tratamiento de la hipertensión pulmonar asociada a la infección por VIH con treprostinil / Treatment of HIV-associated pulmonary hypertension with treprostinil

Introducción y objetivo. El tratamiento de la hipertensión pulmonar asociada a infección por virus de la inmunodeficiencia humana no está bien definido. El treprostinil es un análogo de la prostaciclina que recientemente ha demostrado eficacia en el tratamiento de la hipertensión pulmonar primaria, secundaria a cardiopatías congénitas y asociada a colagenosis, en un estudio doble ciego de 12 semanas de duración. Describimos el seguimiento al año de los 3 pacientes con hipertensión pulmonar asociada a infección por virus de la inmunodeficiencia humana tratados con treprostinil en nuestro centro. Pacientes y método. Tras descartar hipertensión pulmonar secundaria a otra causa mediante el estudio rutinario, los 3 pacientes comenzaron tratamiento con prostaciclina subcutánea (treprostinil) a dosis progresivamente crecientes. Se realizó una evaluación trimestral de la clase funcional y de la capacidad de esfuerzo, así como una ecocardiografía semestral. Resultados. Los 3 pacientes mejoraron clínicamente según la clase funcional NYHA y la distancia caminada en el test de 6 min (aumento de al menos 75 metros), y permanecen vivos tras un año de seguimiento. En dos pacientes disminuyó la presión pulmonar sistólica estimada por ecocardiografía. No se observaron complicaciones graves asociadas. Conclusiones. La prostaciclina subcutánea (treprostinil) parece ser una opción de tratamiento segura y eficaz en pacientes con hipertensión pulmonar asociada a infección por virus de la inmunodeficiencia humana (AU)

[Ten years' experience in continuous intravenous epoprostenol therapy in severe pulmonary arterial hypertension]. / Experiencia de 10 años en el tratamiento con epoprostenol en perfusión intravenosa continua en hipertensión pulmonar arterial grave.

INTRODUCTION: Primary pulmonary hypertension and its associated forms is a progressive and often fatal disease, the course of which has been favourably modified by prostacyclin therapy in the last decade. OBJECTIVE: The aim of this study is to analize retrospectively the efficacy of continuous intravenous epoprostenol (synthetic prostacyclin) therapy in pulmonary arterial hypertension, and to compare it with conventional therapy (anticoagulants, digoxin and diuretics). METHODS: Between 1990-2000, 31 patients with severe precapillary pulmonary hypertension in functional class III or IV went on continuous intravenous epoprostenol therapy, administered by a portable infusion pump through a Hickman catheter. We compared their survival with a group of 16 patients treated with conventional therapy alone. RESULTS: Time of follow-up was 33.25 months in the prostacyclin group and 20 months in the conventional group. The one- three- and five- year survival rates were 86%, 50% and 38% respectively for patients treated with epoprostenol compared with 40%, 40% and 8% survival rates at idetical periods for patients treated conventionally (p = 0,02). Functional class and the mean distance walked in the 6 minutes test were improved in patients treated with prostacyclin (p < 0,01). Serious complications attributable to the delivery system included 3 deaths, mainly due to infection. CONCLUSION: Continuous intravenous epoprostenol therapy improves survival and exercise capacity in patients with severe pulmonary arterial hypertension despite potentially serious complications attributable to the delivery system.

Experiencia de 10 años en el tratamiento con epoprostenol en perfusión intravenosa continua en hipertensión pulmonar arterial grave / Ten Years' Experience in Continuous Intravenous Epoprostenol Therapy in Severe Pulmonary Arterial Hypertension

Introducción. La hipertensión pulmonar (HTP) primaria y sus formas asociadas tienen un curso habitualmente progresivo y fatal, modificado en la última década por el tratamiento con prostaciclina. Objetivo. Analizar retrospectivamente la eficacia del tratamiento con epoprostenol (prostaciclina sintética) en perfusión intravenosa continua en HTP primaria y sus formas asociadas, comparándola con tratamiento convencional (anticoagulación, digoxina y diuréticos).Métodos. Entre 1990 y 2000, 31 pacientes con HTP arterial grave en clase funcional (CF) III/IV de la NYHA recibieron tratamiento con epoprostenol en perfusión intravenosa continua a través de catéter tipo Hickman y bomba de perfusión portátil. Se compara su supervivencia con un grupo de 16 pacientes tratados convencionalmente. Resultados. El tiempo de seguimiento en el grupo de prostaciclina fue de 33,25 meses y en el de tratamiento convencional 20 meses. La supervivencia a 1,3 y 5 años fue del 86, 50 y 38 por ciento, respectivamente, en los pacientes tratados con epoprostenol, del 40 por ciento al año y 3 años y del 8 por ciento a los 5 años en los pacientes tratados convencionalmente (p = 0,02). Se observó una mejora en la CF y en la distancia recorrida en el test de 6 min en los pacientes tratados con epoprostenol (p < 0,01). El sistema de infusión fue un problema grave y causó 3 muertes fundamentalmente por infección. Conclusión. El epoprostenol en perfusión intravenosa continua mejora la supervivencia y la capacidad funcional en los pacientes con HTP arterial, a pesar de las complicaciones graves relacionadas con el sistema de infusión (AU)

Guías de práctica clínica de la Sociedad Española de Cardiología en tromboembolismo e hipertensión pulmonar / Guidelines of the Spanish Society of Cardiology for Pulmonary Thromboembolism and Hypertension

La hipertensión pulmonar primaria es una enfermedad de carácter progresivo, más frecuente en mujeres jóvenes y de mediana edad. Su etiología se desconoce, aunque existe una predisposición familiar hasta en un 6 por ciento de los casos. Las teorías patogénicas actuales se centran en la existencia de disfunción endotelial y fallos en los canales iónicos de las fibras musculares lisas del vaso. Las pruebas diagnósticas se dirigen a descartar las causas secundarias y a evaluar la gravedad de la enfermedad. El test vasodilatador agudo es imprescindible para la elección del tratamiento más adecuado. La anticoagulación oral está indicada en todos los pacientes. El trasplante de pulmón queda reservado a aquellos casos en los que fracasa el tratamiento médico. La septostomía auricular es un procedimiento paliativo útil en casos seleccionados. La hipertensión pulmonar tromboembólica crónica es una forma especial de hipertensión pulmonar secundaria; aunque indistinguible clínicamente de la hipertensión pulmonar primaria, su diagnóstico resulta crucial, ya que es posible su curación mediante la realización de tromboendarterectomía pulmonar. El tromboembolismo pulmonar es frecuente en pacientes hospitalizados, presentando elevadas tasas de mortalidad (el 30 por ciento en pacientes no tratados). El diagnóstico es difícil, ya que puede acompañar o simular otras enfermedades cardiopulmonares. Las pruebas diagnósticas no invasivas tienen una baja sensibilidad y especificidad. Nuevas alternativas como la determinación de dímero D o la TAC helicoidal incrementan la precisión diagnóstica. El tratamiento estándar consiste en la administración de heparina durante 5-10 días y posteriormente anticoagulantes orales durante 3-6 meses. La prevención con heparinas o dextranos en los pacientes de alto riesgo ha demostrado claros beneficios (AU)