Predictors of pulmonary hypertension in patients with end-stage heart failure.

Pulmonary hypertension (PH) is associated with a greater mortality rate in patients with heart failure (HF) and it is a risk factor for right ventricular failure after heart transplantation. This study was designed to explore risk factors for PH development in patients with advanced heart failure and left ventricular dysfunction. In a retrospective observational study of 419 patients evaluated for heart transplantation due to end stage HF, different variables were analyzed to find predictors of PH (defined as a mean pulmonary pressure >25 mmHg), reactive PH (defined as a transpulmonary gradient >12 mmHg) and severe PH (defined as a mean pulmonary pressure >40 mmHg and/or pulmonary vascular ressistance >3 WU) using a multivariate stepwise logistic regression analysis. Prevalence of PH, out of proportion and severe PH was 62.2%, 23.8%, and 18.8% respectively. The presence of moderate-severe mitral regurgitation [2.1 (1.2-3.7); P=0.006], moderate-severe tricuspid regurgitation [OR 2.9 (1.3-6.4); P=0.005] and a duration of disease >3 years [OR 1.7 (1.1-2.7); P=0.03] were independent risk factors associated with PH. Moreover, the presence of a moderate-severe mitral regurgitation and a duration of disease greater than 3 years, were independent predictors of out of proportion and severe PH.

Implante de desfibrilador como prevención primaria de muerte súbita en pacientes en lista de espera de trasplante cardiaco: experiencia de un centro / Defibrillator implantation for the primary prevention of sudden death in patients awaiting cardiac transplantation: one center’s experience

Los pacientes incluidos en lista de espera de trasplante cardiaco frecuentemente presentan un perfil acorde a las recomendaciones actuales en cuanto al implante de desfibrilador automático implantable como prevención primaria de muerte súbita. El eventual trasplante a corto-medio plazo hace dudar de la efectividad de dicha terapia. Analizamos la incidencia de terapias administradas por el desfibrilador implantado como prevención primaria en pacientes en lista, así como la evolución histórica en la frecuencia de muerte súbita en nuestro centro. Se revisaron los 308 pacientes incluidos en lista desde 1998 hasta 2008. En 17 pacientes se indicó desfibrilador automático implantable como prevención primaria al momento de la inclusión. El 53% de éstos recibió terapias adecuadas, habiendo portado el dispositivo una media de 7,8 meses (±4,8). Sólo 1 paciente presentó terapias inadecuadas y ninguno sufrió complicaciones asociadas al dispositivo. La frecuencia de muerte súbita se ha reducido a lo largo de los últimos años(AU)

Patients who are on a waiting list for cardiac transplantation often have a clinical profile that satisfies current recommendations for the implantation of an implantable cardioverter–defibrillator for the primary prevention of sudden death. The prospect that transplantation may take place within the shortto- medium term puts the effectiveness of this therapy in doubt. We investigated the incidence of therapy delivered by implantable cardioverter–defibrillators implanted for primary prevention in patients awaiting cardiac transplantation. Recent changes in the incidence of sudden death at our center were also investigated. Data on 308 patients listed for heart transplantation between 1998 and 2008 were reviewed. An implantable cardioverter–defibrillator was indicated for primary prevention at initial evaluation in 17 patients. Of these, 53% received appropriate implantable cardioverter–defibrillator therapy while carrying an implantable cardioverter–defibrillator for amean period of 7.8months (+/-4.8). Only one patient received inappropriate therapy and none had any complications associated with device use. The frequency of sudden death has decreased over the course of recent years(AU)

Defibrillator implantation for the primary prevention of sudden death in patients awaiting cardiac transplantation: one center's experience.

Patients who are on a waiting list for cardiac transplantation often have a clinical profile that satisfies current recommendations for the implantation of an implantable cardioverter-defibrillator for the primary prevention of sudden death. The prospect that transplantation may take place within the short-to-medium term puts the effectiveness of this therapy in doubt. We investigated the incidence of therapy delivered by implantable cardioverter-defibrillators implanted for primary prevention in patients awaiting cardiac transplantation. Recent changes in the incidence of sudden death at our center were also investigated. Data on 308 patients listed for heart transplantation between 1998 and 2008 were reviewed. An implantable cardioverter-defibrillator was indicated for primary prevention at initial evaluation in 17 patients. Of these, 53% received appropriate implantable cardioverter-defibrillator therapy while carrying an implantable cardioverter-defibrillator for a mean period of 7.8 months (±4.8). Only one patient received inappropriate therapy and none had any complications associated with device use. The frequency of sudden death has decreased over the course of recent years.

Comparison of baseline characteristics and survival between patients with idiopathic and connective tissue disease-related pulmonary arterial hypertension.

BACKGROUND: Both idiopathic pulmonary arterial hypertension (IPAH) and pulmonary arterial hypertension (PAH) related to connective tissue diseases (CPAH) are classified in the group of PAH disorders. However, CPAH has a particularly worse prognosis than IPAH. Few studies have compared the clinical, functional and hemodynamic profiles of IPAH and CPAH. METHODS: We performed a retrospective cohort study of patients with IPAH or CPAH. Demographic characteristics, functional status (FE), pulmonary function test and hemodynamic values at the time of diagnosis were compared between the two etiologies. Global cumulative survival rates free from transplantation (SFT) and survival according to date of diagnosis were analyzed. RESULTS: Despite similar PAH severity, patients with CPAH showed a more severe baseline impairment of 6-minute walking test (6MWT) (307 +/- 116 m vs 378 +/- 101 m) and diffusion capacity of the lung for carbon monoxide (DLCO) (57 +/- 25% vs 75 +/- 30% of predicted) than IPAH (p < 0.01). Survival rates at 1, 3 and 5 years of follow-up were 87%, 71% and 63% for IPAH, and 70%, 53% and 42% for CPAH, respectively (p < 0.05). IPAH showed better survival when treatment was started after Year 2000 (p = 0.01). However, CPAH showed a poorer prognosis than IPAH in the more recent era (p < 0.05). CPAH (hazard ratio [HR] = 2.03), DLCO <80% (HR = 1.98) and treatment before Year 2000 (HR = 2.27) were associated with an independent increased risk of death or transplantation. CONCLUSIONS: Despite similar functional and hemodynamic severity, patients with CPAH showed a more severe baseline impairment of 6MWT and DLCO and worse overall prognosis than IPAH. Both IPAH and CPAH survival improved in the current era. Nevertheless, CPAH still showed a poorer prognosis than IPAH.

Management of primary pulmonary artery sarcoma: experience of a single center.

Primary sarcoma of the pulmonary artery (PSPA) is extremely rare. Many cases are misdiagnosed as pulmonary arterial hypertension (PAH) because of chronic thromboembolic disease (CTD). Four cases of PSPA with the initial misdiagnosis are reported. The presence of a unique mass in the main pulmonary artery or proximal branches, rapidly progressive dyspnea, and constitutional symptoms should raise the suspicion of PSPA. The pathological diagnosis is usually confirmed during surgery, which is done along with adjuvant chemotherapy, the treatment of choice.

[Clinical and Preclinical Heart Failure Research Network (REDINSCOR). Instituto de Salud Carlos III Cooperative Special Topic Research Networks]. / Red de investigación clínica y básica en insuficiencia cardiaca (REDINSCOR). Redes temáticas de investigación cooperativa del Instituto de Salud Carlos III.

Heart failure is a prevalent condition that is associated with high morbidity and mortality and that places a substantial demand on healthcare resources. As the condition often affects older individuals with comorbidities, its treatment requires the implementation of an integrated model of care that can be applied in primary care, in hospitals, and in the patient's home. The use of heart failure units has improved quality of life in these patients and has reduced hospital admissions. However, in Spain integrated care has not yet been widely implemented. Clinical and preclinical research on heart failure is complex and, consequently, it is essential for investigators to work together in research networks. As a result, 16 Spanish centers have organized themselves into a heart failure research network (REDINSCOR). This network is considering the establishment of a national heart failure registry and is involved in seven research projects, whose subjects range from molecular biology and cell physiology to diagnosis, treatment, and the development of integrated healthcare models.

Desequilibrio entre oferta y necesidades de cardiólogos en España / The imbalance between supply and demand for cardiologists in Spain

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[Transition from prostacyclin to bosentan in five patients with severe pulmonary hypertension: the switch is possible]. / Transición de prostaciclina a bosentán en 5 pacientes con hipertensión pulmonar severa: el cambio es posible.

Prostacyclin improves symptoms, exercise tolerance, and survival in patients with pulmonary arterial hypertension. However, the difficulty of administration (whether intravenous, subcutaneous, or by inhalation) often causes side effects that can reduce the patient's quality of life and which may sometimes be serious. Bosentan, an orally active endothelin receptor antagonist, improves functional class and exercise tolerance in these patients. We describe the successful transition from prostacyclin to bosentan in five patients with severe pulmonary arterial hypertension who suffered serious side effects with prostacyclin treatment.

Transición de prostaciclina a bosentán en 5 pacientes con hipertensión pulmonar severa: el cambio es posible / Transition from prostacyclin to bosentan in five patients with severe pulmonary hypertension: the switch is possible

La prostaciclina mejora los síntomas, la capacidad de ejercicio y la supervivencia en los pacientes con hipertensión arterial pulmonar. Sin embargo, sus complejas vías de administración (intravenosa, inhalada, subcutánea) ocasionan frecuentes efectos adversos que disminuyen la calidad de vida y pueden ser graves. Bosentán, un antagonista oral de los receptores de la endotelina, mejora la clase funcional y la capacidad de ejercicio en estos pacientes. Describimos la transición de prostaciclina a bosentán en 5 pacientes con hipertensión arterial pulmonar severa e importantes complicaciones secundarias al tratamiento con prostaciclina

Prostacyclin improves symptoms, exercise tolerance, and survival in patients with pulmonary arterial hypertension. However, the difficulty of administration (whether intravenous, subcutaneous, or by inhalation) often causes side effects that can reduce the patient's quality of life and which may sometimes be serious. Bosentan, an orally active endothelin receptor antagonist, improves functional class and exercise tolerance in these patients. We describe the successful transition from prostacyclin to bosentan in five patients with severe pulmonary arterial hypertension who suffered serious side effects with prostacyclin treatment

[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.

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

Fundamento y objetivo: Los pacientes con síndrome del aceite tóxico son una población de riesgo de padecer hipertensión arterial pulmonar. La elevación anómala de la presión sistólica pulmonar (PSP) durante el ejercicio podría considerarse un marcador temprano de hipertensión pulmonar en poblaciones de riesgo. El objetivo del presente estudio ha sido analizar mediante ecocardiografía la respuesta de la PSP al ejercicio en pacientes con síndrome del aceite tóxico. Pacientes y método: Se estudió a 50 pacientes con síndrome del aceite tóxico y a 20 voluntarios sanos con ecocardiograma en reposo y en el máximo ejercicio (cicloergómetro semisupino) y se midió la PSP. En los pacientes se determinó la capacidad de difusión de monóxido de carbono. Resultados: No se observaron diferencias en el valor medio de la PSP en el ejercicio entre los pacientes y los voluntarios sanos. En un 8% de los primeros se observó un valor igual o superior a 80 mmHg, lo que se asoció a hipertensión pulmonar leve en reposo, disminución de la función ventricular derecha y trastornos de la difusión pulmonar. Un valor de corte de la PSP en reposo igual o superior a 27 mmHg tuvo una sensibilidad del 100% y una especificidad del 71% para predecir una PSP pico de 80 mmHg o superior. Conclusiones: Una minoría de pacientes con síndrome del aceite tóxico muestra hipertensión pulmonar grave con el ejercicio. Este tipo de respuesta anormal se asocia a otros marcadores de vasculopatía pulmonar. Futuros estudios han de esclarecer la relación entre estas anomalías y el riesgo de desarrollar ulteriormente hipertensión arterial pulmonar

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 cicloergometer) 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 >= 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 >= 27 mmHg had a 100% sensitivity and 71% specificity to predict a peak exercise systolic pulmonary pressure >= 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

Hipertensión arterial pulmonar en el año 2004 / Pulmonary Arterial Hypertension in 2004

Recientemente se han producido importantes avances en el conocimiento de la biopatología de la hipertensión arterial pulmonar (HAP) que han cambiado la perspectiva de la enfermedad. La disfunción del endotelio vascular induce un predominio del tono vasoconstrictor, de la hipercoagulabilidad y, fundamentalmente, incrementa la proliferación celular, produciendo un remodelado vascular con obliteración progresiva de la luz y un incremento de las resistencias vasculares pulmonares. Los nuevos tratamientos médicos actuarán sobre el remodelado vascular y la disfunción endotelial. Asimismo, el número creciente de enfermedades asociadas a la HAP y la aparición de nuevas técnicas diagnósticas obligan a sistematizar el procedimiento diagnóstico y definir una clasificación. En esta revisión, se actualizan las principales novedades en la patobiología pulmonar, las modificaciones en la clasificación clínica, los cambios en las definiciones diagnósticas, las nuevas estrategias terapéuticas y las perspectivas futuras. Además, se exponen las recomendaciones de las principales sociedades científicas para el manejo del paciente con HAP (AU)

Recent advances in the understanding of biopathology in pulmonary arterial hypertension have changed the perspective of the disease. Endothelial dysfunction induces a vasocontrictive response, a hypercoagulability state and, more importantly, an increase of cellular proliferation with subsequent vascular remodeling. Pulmonary vascular resistance is therefore increased due to progressive vessel obliteration. New therapeutic approaches are focused on vascular remodeling inhibition and endothelial dysfunction. Pulmonary arterial hypertension is associated with an increasing number of different diseases, which makes necessary to establish a systematic diagnostic approach, and to define a classification. The main topics in this review will be the recent advances in pathobiology, the new clinical classification, the changes in diagnostic definitions and the last therapeutic approaches, pointing out the future directions. Finally, recent recommendations for diagnosis and treatment published by the main scientific societies are provided (AU)

[Efficacy of oral sildenafil as rescue therapy in patients with severe pulmonary arterial hypertension chronically treated with prostacyclin. Long-term results]. / Eficacia del sildenafilo por vía oral como terapia de rescate en pacientes con hipertensión arterial pulmonar severa en tratamiento crónico con prostaciclina. Resultados a largo plazo.

INTRODUCTION AND OBJECTIVE: Prostacyclin therapy is an effective treatment for severe pulmonary hypertension. Sildenafil, a selective phosphodiesterase type 5 inhibitor, induces selective vasodilatation of the pulmonary vessels. A synergistic effect has been described for these two drugs. The aim of this study was to evaluate the efficacy and safety of sildenafil as rescue therapy in patients with severe pulmonary hypertension on chronic treatment with prostacyclin whose clinical or functional course was unsatisfactory. PATIENTS AND METHOD: Observational study of 11 patients (7 men, 4 women, mean age 42 [8] years) diagnosed as having severe idiopathic pulmonary hypertension, who were receiving chronic prostacyclin therapy. Sildenafil was started after a worsening of their clinical or functional status. Baseline, 3-month and 12-month follow-up evaluations were based on functional status (NYHA functional class and 6-minute walking test), the presence of decompensated right heart failure and echocardiogram. RESULTS: Seven of the 11 patients showed significant improvements in exercise capacity (distance walked in 6 minutes) at 3 (+25 m) and 12 months' follow-up (+36 m). Improvements in functional class were seen, and heart failure disappeared. No significant adverse effects of sildenafil were detected. The echocardiographic parameters showed a significant reduction in right ventricular end-diastolic diameter and left ventricular diastolic eccentricity index. One patient died after 4 months of follow-up from sudden cardiac death. CONCLUSIONS: The addition of oral sildenafil to chronic prostacyclin treatment in patients with severe pulmonary hypertension improved functional capacity and reduced episodes of decompensated right heart failure, with good tolerance and no significant adverse effects.

Eficacia del sildenafilo por vía oral como terapia de rescate en pacientes con hipertensión arterial pulmonar severa en tratamiento crónico con prostaciclina. Resultados a largo plazo / Efficacy of oral sildenafil as rescue therapy in patients with severe pulmonary arterial hypertension chronically treated with prostacyclin. Long-term results

Introducción y objetivo. La prostaciclina es un tratamiento eficaz en la hipertensión arterial pulmonar severa. El sildenafilo, un inhibidor selectivo de la fosfodiesterasa (PDE) tipo 5, produce vasodilatación selectiva de los vasos pulmonares, y se ha descrito un efecto sinérgico con la prostaciclina. El objetivo es analizar la eficacia y seguridad del sildenafilo como terapia de rescate en pacientes con hipertensión pulmonar (HTP) en tratamiento crónico con prostaciclina que presentan mala evolución clínica y funcional. Pacientes y método. Estudio observacional de 11 pacientes (7 varones, edad 42 ñ 8 años) con hipertensión pulmonar idiopática (HTPI) en tratamiento crónico con prostaciclina. Se asoció sildenafilo oral cuando presentaban deterioro clínico o funcional. Basalmente, a los 3 y los 12 meses de tratamiento combinado, se evaluó la capacidad funcional (clase funcional [CF] de la New York Heart Association [NYHA] y prueba de los 6 min), la presencia de insuficiencia cardíaca derecha (ICD) descompensada y se realizó un ecocardiograma. Resultados. Se produjo una mejoría significativa de la capacidad de esfuerzo, con incremento de la distancia recorrida en 6 min a los 3 y los 12 meses (+25 y +36 m, respectivamente), mejoría de la CF y desaparición de la insuficiencia cardíaca en 7 de los 11 pacientes. No se observaron efectos adversos significativos. El ecocardiograma mostró una reducción significativa del diámetro diastólico del ventrículo derecho y del índice de excentricidad diastólico del ventrículo izquierdo. Un paciente falleció (muerte súbita) a los 4 meses. Conclusiones. La adición de sildenafilo al tratamiento crónico con prostaciclina en pacientes con HTP severa mejoró la capacidad funcional y disminuyó los episodios de ICD descompensada, con buena tolerancia al medicamento y sin efectos adversos graves (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.

Bosentán, ¿perspectiva o realidad en el tratamiento de la hipertensión arterial pulmonar? Respuesta / Bosentán, hope or reality for the treatment of pulmonary arterial hypertension. Reply

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Hipertensión arterial pulmonar: perspectivas terapéuticas / Primary pulmonary hypertension: prospects for treatment

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[Transition from intravenous to subcutaneous prostacyclin in pulmonary hypertension]. / Transition from intravenous to subcutaneous prostacyclin in pulmonary hypertension.

Treatment of arterial pulmonary hypertension with epoprostenol (intravenous prostacyclin) improves survival and quality of life, but the need for an implanted central venous catheter is associated with frequent complications, that often (as in the case of infection or dislodgment) are serious and require catheter replacement. Treprostinil is a prostacyclin analogue suitable for continuous subcutaneous administration. We report the successful transition from intravenous epoprostenol to subcutaneuos treprostinil in four patients with severe pulmonary hypertension who suffered from serious complications associated with the epoprostenol infusion system.