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Background: The contemporary outcome of balloon pulmonary angioplasty (BPA) and pulmonary endarterectomy (PEA) in patients with chronic thromboembolic pulmonary hypertension (CTEPH) are unclear. Objectives: This study aimed to clarify the characteristics and outcomes of CTEPH patients treated with BPA and PEA in Japan. Methods: Among 1,270 participants enrolled between 2018 and 2023 in the CTEPH AC (Chronic Thromboembolic Pulmonary Hypertension Anticoagulant) registry, a Japanese nationwide CTEPH registry, 369 treatment-naive patients (BPA strategy: n = 313; PEA strategy: n = 56) and 690 on-treatment patients (BPA strategy: n = 561; PEA strategy: n = 129) were classified according to the presence of prior reperfusion therapy. Morbidity and mortality events (all-cause death, rescue mechanical reperfusion therapy, and/or initiation of parenteral pulmonary vasodilators), pulmonary hemodynamics, exercise tolerance, and relevant laboratory test results were evaluated. Results: The BPA strategy was chosen in older patients than the PEA strategy (mean age, BPA vs PEA: 66.5 ± 12.6 years vs 62.5 ± 11.8 years; P = 0.028). Median follow-up period was 615 (Q1-Q3: 311-997) days in treatment-naive patients and 1,136 (Q1-Q3: 684-1,300) days in on-treatment patients. BPA strategy had as acceptable morbidity and mortality as PEA strategy (5-year morbidity and mortality event rate, BPA vs PEA: 10.2% [95% CI: 5.2%-19.5%] vs 16.1% [95% CI: 4.3%-50.6%] in treatment-naive patients; 9.7% [95% CI: 6.7%-13.8%] vs 6.9% [95% CI: 2.7%-17.3%] in on-treatment patients), with greater improvement of renal function; glomerular filtration rate in propensity score-matched population (difference between change: 4.9 [95% CI: 0.5-9.3] mL/min/1.73 m2; P = 0.030). Conclusions: BPA strategy was more frequently chosen in older patients compared with PEA strategy and showed acceptable outcomes for efficacy with greater advantage for improvement in renal function. (Multicenter registry of chronic thromboembolic pulmonary hypertension in Japan; UMIN000033784).
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Pulmonary arterial hypertension (PAH) remains a significant challenge in cardiology, necessitating advancements in treatment strategies. This study explores the safety and efficacy of transitioning patients from beraprost to selexipag, a novel selective prostacyclin receptor agonist, within a Japanese cohort. Employing a multicenter, open-label, prospective design, 25 PAH patients inadequately managed on beraprost were switched to selexipag. Key inclusion criteria included ongoing beraprost therapy for ≥3 months, a diagnosis of PAH confirmed by mean pulmonary artery pressure (mPAP) ≥ 25 mmHg, and current treatment with endothelin receptor antagonists and/or phosphodiesterase type 5 inhibitors. Outcomes assessed were changes in hemodynamic parameters (mPAP, cardiac index, pulmonary vascular resistance) and the 6 min walk distance (6-MWD) over 3-6 months. The study found no statistically significant changes in these parameters post-switch. However, a subset of patients, defined as responders, demonstrated improvements in all measured hemodynamic parameters, suggesting a potential benefit in carefully selected patients. The transition was generally well-tolerated with no serious adverse events reported. This investigation underscores the importance of personalized treatment strategies in PAH, highlighting that certain patients may benefit from switching to selexipag, particularly those previously on higher doses of beraprost. Further research is needed to elucidate the predictors of positive response to selexipag and optimize treatment regimens for this complex condition.
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Background: Portopulmonary hypertension (PoPH) is one of the major underlying causes of pulmonary arterial hypertension (PAH). However, PoPH, especially treatment strategies, has been poorly studied. Therefore, this study evaluated current treatments for PoPH, their efficacy, and clinical outcomes of patients with PoPH. MethodsâandâResults: Clinical data were collected for patients with PoPH who were enrolled in the Japan Pulmonary Hypertension Registry between 2008 and 2021. Hemodynamic changes, functional class, and clinical outcomes were compared between patients with PoPH treated with monotherapy and those treated with combination therapies. Clinical data were analyzed for 62 patients with PoPH, including 25 treatment-naïve patients, from 21 centers in Japan. In more than half the patients, PAH-specific therapy improved the New York Heart Association functional class by at least one class. The 3- and 5-year survival rates of these patients were 88.5% (95% confidence interval [CI] 76.0-94.7) and 80.2% (95% CI 64.8-89.3), respectively. Forty-one (66.1%) patients received combination therapy. Compared with patients who had received monotherapy, the mean pulmonary arterial pressure, pulmonary vascular resistance, and cardiac index were significantly improved in patients who had undergone combination therapies. Conclusions: Combination therapy was commonly used in patients with PoPH with a favorable prognosis. Combination therapies resulted in significant hemodynamic improvement without an increased risk of side effects.
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BACKGROUND: Balloon pulmonary angioplasty (BPA) improved pulmonary arterial compliance (CPA) and exercise tolerance in patients with inoperable chronic thromboembolic pulmonary hypertension (CTEPH). OBJECTIVES: To investigate whether CPA is a useful index to indicate exercise tolerance improvement by BPA in CTEPH patients. METHODS: The correlation between changes in CPA and improvements in 6-minute walk distance (6MWD) by BPA was retrospectively analyzed in 70 patients (Analysis 1), and it was sequentially analyzed in 46 symptomatic patients who achieved mean pulmonary arterial pressure (mPAP)<30mmHg (Analysis 2). RESULTS: We enrolled 70 patients (female/male:57/13, mean age:59 years) who underwent a total of 352 BPA sessions which significantly increased CPA (1.5±0.8 vs. 3.0±1.0 mL/mmHg) and decreased pulmonary vascular resistance (PVR) (8.0 ± 3.9 vs. 3.6 ± 1.7 wood units). The correlation coefficient between improvement in 6MWD and changes in PVR and CPA were r=0.21 (p=0.09) and r=0.14 (p=0.26) (Analysis 1). In Analysis 2, those were r=0.32 (p=0.06) and r=0.38 (p=0.02), respectively. CONCLUSIONS: CPA can be a useful index to indicate the improvement in exercise tolerance by BPA in symptomatic patients with lower mPAP.
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Angioplastia de Balón , Hipertensión Pulmonar , Embolia Pulmonar , Presión Arterial , Enfermedad Crónica , Tolerancia al Ejercicio , Femenino , Humanos , Masculino , Persona de Mediana Edad , Arteria Pulmonar/cirugía , Embolia Pulmonar/complicaciones , Embolia Pulmonar/terapia , Estudios Retrospectivos , Resultado del TratamientoRESUMEN
Background: The latest guideline from the European Society of Cardiology and European Respiratory Society recommends initial combination therapy with oral pulmonary arterial hypertension (PAH)-specific drugs in PAH patients with World Health Organization functional class (WHO-FC) II or III. However, whether this initial combination therapy improves hemodynamics and clinical failure events regardless of the combination of PAH-specific drugs remains unknown. This study was designed to evaluate whether the initial combination therapy with macitentan plus riociguat or macitentan plus selexipag showed equal efficacy in reducing pulmonary vascular resistance (PVR) 8 months after administration. MethodsâandâResults: This study is a multicenter randomized control trial. PAH subjects with WHO-FC II or III will be randomized (1 : 1) into initial combination therapy with either macitentan plus riociguat or macitentan plus selexipag, and will be observed 8 months after the initiation of treatment. The primary endpoint will be the difference in the change ratio of PVR from baseline to after 8 months of treatment. Conclusions: The SETOUCHI-PH study will clarify whether initial combination therapy with macitentan plus riociguat or macitentan plus selexipag results in equal reductions in PVR 8 months after administration.
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AIMS: Chronic thromboembolic pulmonary hypertension (CTEPH) is characterised by organised thrombotic obliteration of major vessels and small-vessel arteriopathy in the non-thrombosed vessels. The aim of this study was to investigate the impact of balloon pulmonary angioplasty (BPA) on the non-BPA-side pulmonary vasculature in patients with CTEPH. METHODS AND RESULTS: This study explored the outcomes of 20 unilateral BPA sessions in 13 CTEPH patients. We measured the pulmonary vascular resistance (PVR), pulmonary artery (PA) flow in the BPA-side and non-BPA-side lungs, respectively, using phase contrast MRI and cardiac catheterisation. The interval from BPA to the follow-up evaluation was 92.8±52.0 days. A single session of BPA decreased mean PA pressure from 37.4±6.2 to 30.9±6.5 mmHg (p<0.001). In the BPA side, BPA increased the PA flow from 1.58±0.65 to 1.95±0.62 L/min (p=0.001) and decreased the PVR from 27.3±27.4 to 14.4±9.0 Wood units (p=0.004). In contrast, it decreased both the non-BPA-side PA flow from 2.25±0.64 to 1.90±0.23 L/min (p=0.008) and the non-BPA-side PVR from 14.8±6.6 to 12.8±3.9 Wood units (p=0.01). CONCLUSIONS: BPA could relieve haemodynamic stress towards the non-BPA-side vasculature and decrease its PVR in patients with CTEPH, suggesting that it can suppress or regress the progression of the small-vessel arteriopathy in non-BPA-side vasculature, presumably due to haemodynamic unloading.
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Angioplastia de Balón , Hipertensión Pulmonar , Complicaciones Posoperatorias , Arteria Pulmonar , Embolia Pulmonar/complicaciones , Anciano , Angioplastia de Balón/efectos adversos , Angioplastia de Balón/instrumentación , Angioplastia de Balón/métodos , Presión Arterial , Enfermedad Crónica , Progresión de la Enfermedad , Femenino , Hemodinámica , Humanos , Hipertensión Pulmonar/diagnóstico , Hipertensión Pulmonar/etiología , Hipertensión Pulmonar/fisiopatología , Hipertensión Pulmonar/cirugía , Masculino , Persona de Mediana Edad , Complicaciones Posoperatorias/diagnóstico , Complicaciones Posoperatorias/etiología , Complicaciones Posoperatorias/fisiopatología , Arteria Pulmonar/patología , Arteria Pulmonar/fisiopatología , Arteria Pulmonar/cirugía , Resultado del Tratamiento , Resistencia VascularRESUMEN
BACKGROUND: Pulmonary hypertension (PH) is a life-threatening complication in patients with chronic kidney disease on hemodialysis (CKD-HD). OBJECTIVES: To determine whether subcutaneous infusion of treprostinil was effective and tolerable CKD-PH. METHODS AND RESULTS: A 57-year-old man was admitted to our hospital due to presyncope and dyspnea during exercise with a history of CKD-HD. Cardiac catheterization revealed high pulmonary arterial pressure (PAP) of 53/24/32 mmHg and pulmonary vascular resistance (PVR) of 11.2 w.u. Upfront combination therapy with bosentan and sildenafil was started. However, 6-month therapy did not attenuate his symptoms, probably due to the high PAP and PVR (60/19/30 mmHg and 5.9 w.u.). We added subcutaneous treprostinil. Surprisingly, 9-month treprostinil (50 ng/kg/min) normalized hemodynamics (PAP: 25/4/13 mmHg and PVR: 1.9 w.u.). His symptoms during excise disappeared without any adverse effects. CONCLUSION: This is the first report that subcutaneous treprostinil was very effective and tolerable in a PH patient with CKD-HD.
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Tolerancia a Medicamentos , Epoprostenol/análogos & derivados , Hemodinámica/efectos de los fármacos , Hipertensión Pulmonar/tratamiento farmacológico , Diálisis Renal , Insuficiencia Renal Crónica/complicaciones , Antihipertensivos/administración & dosificación , Relación Dosis-Respuesta a Droga , Epoprostenol/administración & dosificación , Humanos , Hipertensión Pulmonar/etiología , Hipertensión Pulmonar/fisiopatología , Inyecciones Subcutáneas , Masculino , Persona de Mediana Edad , Insuficiencia Renal Crónica/terapia , Índice de Severidad de la EnfermedadRESUMEN
To use cardiac magnetic resonance imaging (MRI) to investigate the effect of balloon pulmonary angioplasty (BPA) on interventricular dyssynchrony and its associations with ventricular interaction, which impairs LV function in patients with inoperable chronic thromboembolic pulmonary hypertension (CTEPH). This prospective observational study was approved by our institutional review board. Cardiac MRI and right heart catheterization were conducted before BPA sessions and at the follow up after BPA in 20 patients with CTEPH. We measured right ventricular (RV) and left ventricular (LV) end-diastolic volume (EDV), end-systolic volume (ESV), stroke volume (SV), and ejection fraction (EF) using MRI. For the LV and RV free walls, the time to peak (Tpeak) of circumferential strain was calculated as a parameter for interventricular dyssynchrony. Following BPA, the RV-EDV and -ESV were significantly decreased, and the RVEF was significantly increased. Conversely, BPA led to significantly increased LV EDV and SV without changing LVESV. The left-to-right free wall delay (L-R delay) in Tpeak strain decreased from 105 ± 44 ms to 47 ± 67 ms (p < 0.001). Increased LV EDV (r = 0.65, p < 0.01), SV (r = 0.74, p < 0.001) and 6-minute walk distance (6MWD) (r = 0.54, p < 0.05) were correlated to the reduction in L-R delay. In patients with inoperable CPEPH, BPA improved interventricular dyssynchrony, which was strongly associated with increased SV and 6MWD. The assessment of interventricular dyssynchrony using cardiac MRI has an important role in evaluating ventricular interaction, which reduces LVSV and exercise tolerance.
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Angioplastia de Balón , Imagen por Resonancia Cinemagnética , Embolia Pulmonar/terapia , Disfunción Ventricular Izquierda/diagnóstico por imagen , Disfunción Ventricular Derecha/diagnóstico por imagen , Función Ventricular Izquierda , Función Ventricular Derecha , Anciano , Anciano de 80 o más Años , Angioplastia de Balón/efectos adversos , Antihipertensivos/uso terapéutico , Enfermedad Crónica , Tolerancia al Ejercicio , Femenino , Hemodinámica , Humanos , Hipertensión Pulmonar/tratamiento farmacológico , Hipertensión Pulmonar/etiología , Hipertensión Pulmonar/fisiopatología , Modelos Lineales , Masculino , Persona de Mediana Edad , Contracción Miocárdica , Valor Predictivo de las Pruebas , Estudios Prospectivos , Circulación Pulmonar , Embolia Pulmonar/complicaciones , Embolia Pulmonar/diagnóstico por imagen , Embolia Pulmonar/fisiopatología , Recuperación de la Función , Volumen Sistólico , Factores de Tiempo , Resultado del Tratamiento , Vasodilatadores/uso terapéutico , Disfunción Ventricular Izquierda/etiología , Disfunción Ventricular Izquierda/fisiopatología , Disfunción Ventricular Derecha/etiología , Disfunción Ventricular Derecha/fisiopatología , Prueba de PasoRESUMEN
We describe the case that persistent atrial fibrillation refractory to rhythm control by pharmacotherapy and electrical cardioversions caused tachycardia-induced cardiomyopathy with low ejection fraction and hemodynamic instability. Mechanical hemodynamic support using an intra-aortic balloon pump is one of the choices of hemodynamic support during catheter ablation by pulmonary vein isolation.
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We herein report the case of 34-year-old woman with acute tricuspid valve infective endocarditis (IE) associated with a ruptured sinus of Valsalva and multiple septic pulmonary emboli. She had no history of medical problems, except for atopic dermatitis (AD). Blood cultures identified methicillin-sensitive Staphylococcus aureus. Despite the administration of two months of antibiotic therapy, the patient experienced recurrent pulmonary emboli and developed heart failure due to a left-to-right shunt, whereas the area of vegetation did not change in size. She subsequently underwent surgery for shunt closure and tricuspid valve replacement. The AD was thought to be the cause of the patient's bacteremia, which consequently resulted in aggressive right-sided IE.