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BACKGROUND: Idiopathic pulmonary arterial hypertension is associated with high morbidity and mortality. In recent years, the use of targeted therapies has led to an improvement in prognosis. Prostacyclin analogues treprostinil and epoprostenol require continuous subcutaneous or intravenous infusion and are generally administered in a stepwise approach. However, there are no clear recommendations for transition in high-risk patients requiring high doses of prostacyclin analogues. CASE SUMMARY: In this report, we describe the case of a 20-year-old woman under combined treatment with sildenafil, macitentan, and treprostinil who required transition from subcutaneous treprostinil therapy to intravenous epoprostenol due to erratic drug absorption and functional class progression. The transition was performed over 48 h in a stepwise approach reducing treprostinil dose 4 ng/kg/min every 3 h while increasing epoprostenol infusion 2 ng/kg/min until achieving a maintenance dose of 32 ng/kg/min. There were no side effects requiring changes in the infusion rate. DISCUSSION: Patients with advanced pulmonary arterial hypertension may necessitate switching from subcutaneous treprostinil to epoprostenol. Although many protocols have been used to date, there are no guidelines to direct this process safely. This 48-h scheme based on the pharmacokinetic properties of each drug was successful and well-tolerated.
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Dengue virus can affect the heart, with complications as bradycardia, arrhythmias, and death. We present a case of a 15-year-old patient, diagnosed 4 years before with severe idiopathic pulmonary hypertension, confirmed by catheterism, with continuous follow up. At that time, she was living in Bogotá (2640 m above sea level). Sildenafil and Macitentan were started. She was recommended to live at low altitude and she moved. The patient was transferred back to Bogota, from that place, due to flu-like symptoms and fever. Immunoglobulin M for dengue was confirmed and second-degree atrioventricular block Mobitz I with bradycardia (40 beats/minute) was documented throughout the clinical course. She recovered.
Assuntos
Bloqueio Atrioventricular , Dengue , Hipertensão Pulmonar , Adolescente , Arritmias Cardíacas , Criança , Dengue/complicações , Dengue/diagnóstico , Hipertensão Pulmonar Primária Familiar , Feminino , Humanos , Hipertensão Pulmonar/diagnóstico , Hipertensão Pulmonar/etiologiaRESUMO
Introduction: Despite the prognostic importance of traditionally derived measurements, the significance of right heart catheterization (RHC) remains controversial. Thus, a continued search for hemodynamic markers that define better responsive patients is required. Since, right ventricular failure is the most fatal pathway, right (RVPO) and left (LVPO) ventricular power output are parameters that could provide input for a better understanding of the hemodynamics involved in idiopathic pulmonary artery hypertension (IPAH). Method: We retrospectively analyzed how demographics and outcome correlate with hemodynamics to identify responders among IPAH patients. Results: Ninety patients fulfilled the following criteria for inclusion in this study: (1) complete RHC at baseline; (2) an acute evaluation for vasodilators (AEFV, including a positive response, that is, an increase in CO, a decrease in both mPAP and pulmonary vascular resistance ≥ 20% from baseline, respectively); and (3) a long-term follow-up under accepted IPAH treatments. If RVPO decreased (p < 0.001) and LVPO increased (p < 0.012) during AEFV, it is considered that these findings reinforce our ability to identify responders; that is, patients that remained as responders after 6.4 ± 3 years under nifedipine treatment (37.7% of the studied IPAH population). After multivariate analysis, age, RVPO, and LVPO remained as independent variables (OR = 0.927, 95%CI: 0.87-0.98, p = 0.01; OR = 0.114, 95%CI: 0.00-0.91, p = 0.045; and OR = 171.5, 95% CI: 5.3-549, p = 0.004, respectively) when estimating the probability of being a responder. On this basis, an equation was derived to identify responders among IPAH patients, where the probability of being a responder = 1.0196-0.0631 (age) - 4.7693 (RVPO) + 3.8152 (LVPO), ROC: 0.76, 95% CI: 0.63-0.89; p = 0.001. Conclusion:based on the proposed equation, LVPO and RVPO could be used for the identification of responders among IPAH patients.
Introducción: A pesar de la importancia y del significado pronóstico que tienen las mediciones directas y las derivadas del cateterismo cardiaco derecho, éstas permanecen hasta el día de hoy en el terreno académico de la controversia. Por lo tanto, se requiere la continua búsqueda de marcadores hemodinámicos para estratificar a los enfermos con hipertensión arterial pulmonar idiopática. Particularmente, cuando la disfunción contráctil del ventrículo derecho es la vía final más común de esta patología. En esta circunstancia, la determinación del poder del ventrículo derecho y del ventrículo izquierdo representa parámetros que pudieran ser de utilidad para lograr un mejor entendimiento en la hemodinámica de la hipertensión arterial pulmonar idiopática. Método: De manera retrospectiva, analizamos los aspectos demográficos, los hemodinámicos y la sobrevivencia, y si éstos se vieron asociados a la posibilidad de ser enfermos respondedores entre los portadores de hipertensión arterial pulmonar idiopática. Resultados: Noventa enfermos llenaron los siguientes criterios para ser incluidos en el estudio: 1. Contar con cateterismo cardiaco derecho basal; 2. Tener valoración aguda con adenosina, en donde quedó definida una respuesta "positiva aguda" como: aumento del gasto cardíaco, disminución de la presión arterial pulmonar media y de la resistencia vascular pulmonar calculada (≥ 20% de la basal, respectivamente) y; 3. Contar con un seguimiento a largo plazo bajo la influencia de los tratamientos modernos aceptados para enfermos con hipertensión arterial pulmonar idiopática. Sí, el poder del ventrículo derecho disminuyó (p < 0.001) y el poder ventrículo izquierdo aumentó (p < 0.012) durante el reto vasodilatador agudo se consideró que éstos hallazgos reforzaban la habilidad para detectar a los sujetos respondedores con hipertensión arterial pulmonar idiopática; población de enfermos que guardó ese comportamiento hemodinámico durante 6.4 ± 3 años bajo la influencia de nifedipina (37% de la totalidad de la población con hipertensión arterial pulmonar idiopática). Después de efectuar un análisis multivariado, la edad, el poder del ventrículo derecho y del ventrículo izquierdo permanecieron como variables independientes (OR = 0.927, 95%IC: 0.87-0.98, p = 0.01; OR = 0.114, 95%IC: 0.00-0.91, p = 0.045; y OR = 171.5, 95%IC: 5.3-549, p = 0.004, respectivamente) para ser considerados "respondedores". Como resultado, se derivó una ecuación donde la probabilidad de ser respondedor = 1.0196-0.0631 (edad) - 4.7693 (poder del ventrículo derecho) + 3.8152 (poder del ventrículo izquierdo), ROC: 0.76, 95%CI: 0.63 - 0.89; p = 0.001. Conclusión: Con fundamento en los hallazgos de este estudio, la ecuación propuesta, el poder del ventrículo derecho y el ventrículo izquierdo pueden ser utilizados para identificar "respondedores" entre los enfermos con hipertensión arterial pulmonar idiopática.