RESUMEN
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.
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Hipertensión Pulmonar , Esclerodermia Localizada , Humanos , Hipertensión Pulmonar/diagnóstico , Hipertensión Pulmonar/etiología , Prueba de Esfuerzo , Estudios Prospectivos , Ecocardiografía , Cateterismo Cardíaco , Diagnóstico PrecozRESUMEN
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.
Asunto(s)
Hipertensión Pulmonar , Hepatopatías , Hipertensión Arterial Pulmonar , Humanos , Hipertensión Pulmonar/diagnóstico , Hipertensión Pulmonar/epidemiología , Hipertensión Pulmonar/terapia , Masculino , Pronóstico , Sistema de RegistrosAsunto(s)
Acetamidas/uso terapéutico , Cateterismo Cardíaco , Epoprostenol/análogos & derivados , Hipertensión Pulmonar/tratamiento farmacológico , Pirazinas/uso terapéutico , Acetamidas/administración & dosificación , Administración Oral , Sustitución de Medicamentos , Epoprostenol/administración & dosificación , Epoprostenol/uso terapéutico , Femenino , Estudios de Seguimiento , Humanos , Inyecciones Subcutáneas/efectos adversos , Pirazinas/administración & dosificaciónRESUMEN
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 ≥14â¯years recruited during 1998-2016 who had received inh-ILO. Variables were collected at the beginning of treatment (0⯱â¯3â¯months) and 12⯱â¯3/36⯱â¯6â¯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 54â¯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; Pâ¯<â¯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 3â¯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%.
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Hipertensión Pulmonar/tratamiento farmacológico , Iloprost/administración & dosificación , Presión Esfenoidal Pulmonar/efectos de los fármacos , Sistema de Registros , Resistencia Vascular/efectos de los fármacos , Administración por Inhalación , Adulto , Anciano , Causas de Muerte/tendencias , Relación Dosis-Respuesta a Droga , Femenino , Estudios de Seguimiento , Volumen Espiratorio Forzado , Humanos , Hipertensión Pulmonar/mortalidad , Hipertensión Pulmonar/fisiopatología , Masculino , Persona de Mediana Edad , Estudios Prospectivos , Estudios Retrospectivos , España/epidemiología , Factores de Tiempo , Resultado del Tratamiento , Vasodilatadores/administración & dosificación , Función Ventricular Derecha/fisiologíaAsunto(s)
Acetamidas/uso terapéutico , Antihipertensivos/uso terapéutico , Hipertensión Arterial Pulmonar/tratamiento farmacológico , Pirazinas/uso terapéutico , Sustitución de Medicamentos , Quimioterapia Combinada , Epoprostenol/análogos & derivados , Epoprostenol/uso terapéutico , Femenino , Humanos , Bombas de Infusión , Persona de Mediana Edad , Fenilpropionatos/uso terapéutico , Piridazinas/uso terapéutico , Citrato de Sildenafil/uso terapéutico , Tadalafilo/uso terapéutico , Vasodilatadores/uso terapéuticoRESUMEN
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.
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Trasplante de Corazón , Hipertensión Pulmonar/fisiopatología , Piperazinas/farmacología , Vasodilatadores/farmacología , Administración Sublingual , Presión Sanguínea/efectos de los fármacos , Humanos , Hipertensión Pulmonar/cirugía , Masculino , Persona de Mediana Edad , Proyectos Piloto , Piperazinas/administración & dosificación , Presión Esfenoidal Pulmonar/efectos de los fármacos , Purinas , Citrato de Sildenafil , Sulfonas , Resistencia Vascular/efectos de los fármacos , Vasodilatadores/administración & dosificación , Disfunción Ventricular Izquierda/fisiopatología , Disfunción Ventricular Izquierda/cirugíaRESUMEN
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.
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Antihipertensivos/uso terapéutico , Epoprostenol/uso terapéutico , Infecciones por VIH/complicaciones , Hipertensión Pulmonar/tratamiento farmacológico , Adulto , Epoprostenol/análogos & derivados , Femenino , Hemodinámica/efectos de los fármacos , Hemodinámica/fisiología , Humanos , Hipertensión Pulmonar/etiología , Bombas de Infusión , Infusiones Intravenosas , Masculino , Resultado del Tratamiento , Vasodilatadores/uso terapéuticoRESUMEN
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.