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- Association Between Preexisting Versus Newly Identified Atrial Fibrillation and Outcomes of Patients With Acute Pulmonary EmbolismPublication . Bikdeli, Behnood; Jiménez, David; del Toro, Jorge; Piazza, Gregory; Rivas, Agustina; Fernández‐Reyes, José Luis; Sampériz, Ángel; Otero, Remedios; Suriñach, José María; Siniscalchi, Carmine; Martín‐Guerra, Javier Miguel; Castro, Joaquín; Muriel, Alfonso; Lip, Gregory Y. H.; Goldhaber, Samuel Z.; Monreal, Manuel; Monreal, Manuel; Prandoni, Paolo; Brenner, Benjamin; Farge‐Bancel, Dominique; Barba, Raquel; Di Micco, Pierpaolo; Bertoletti, Laurent; Schellong, Sebastian; Tzoran, Inna; Reis, Abilio; Bosevski, Marijan; Bounameaux, Henri; Malý, Radovan; Verhamme, Peter; Caprini, Joseph A.; Bui, Hanh My; Adarraga, María Dolores; Agud, María; Aibar, Jesús; Aibar, Miguel Ángel; Amado, Cristina; Arcelus, Juan Ignacio; Baeza, Carlos; Ballaz, Aitor; Barba, Raquel; Barbagelata, Cristina; Barrón, Manuel; Barrón‐Andrés, Belén; Blanco‐Molina, Ángeles; Botella, Ernesto; Camon, Ana María; Cañas, Inmaculada; Casado, Ignacio; Castro, Joaquín; Chasco, Leyre; Criado, Juan; de Ancos, Cristina; de Miguel, Javier; del Toro, Jorge; Demelo‐Rodríguez, Pablo; Díaz‐Peromingo, José Antonio; Di Campli, Mario Virgilio; Díez‐Sierra, Javier; Domínguez, Irene Milagros; Escribano, José Carlos; Falgá, Conxita; Farfán‐Sedano, Ana Isabel; Fernández‐Capitán, Carmen; Fernández‐Reyes, Jose Luis; Fidalgo, María Ángeles; Flores, Katia; Font, Carme; Font, Llorenç; Francisco, Iria; Gabara, Cristina; Galeano‐Valle, Francisco; Galindo, David; García, María Angelina; García‐Bragado, Ferrán; García de Herreros, Marta; García‐Hernáez, Raquel; García‐Mullor, María Mar; García‐Raso, Arantxa; Gavín‐Sebastián, Olga; Gil‐Díaz, Aida; Gómez‐Cuervo, Covadonga; Grau, Enric; Guirado, Leticia; Gutiérrez, Javier; Hernández‐Blasco, Luis; Hernando, Elena; Herreros, Miguel; Jara‐Palomares, Luis; Jaras, María Jesús; Jiménez, David; Jiménez, Rafael; Joya, Maria Dolores; León, José Manuel; Lima, Jorge; Llamas, Pilar; Lobo, José Luis; López‐Jiménez, Luciano; López‐Miguel, Patricia; López‐Núñez, Juan José; López‐Reyes, Raquel; López‐Ruiz, Antonio; López‐Sáez, Juan Bosco; Lorenzo, Alicia; Madridano, Olga; Maestre, Ana; Marchena, Pablo Javier; Martín del Pozo, Mar; Martín‐Guerra, Javier Miguel; Martín‐Martos, Francisco; Mella, Carmen; Mellado, Meritxell; Mercado, Maria Isabel; Moisés, Jorge; Monreal, Manuel; del Valle Morales, María; Muñoz‐Blanco, Arturo; Muñoz‐Rivas, Nuria; Navas, María Sierra; Nieto, Jose Antonio; Núñez‐Fernández, Manuel Jesús; Obispo, Berta; Olid, Mónica; Olivares, María Carmen; Orcastegui, José Luis; Ortega‐Michel, Clara; Osorio, Jeisson; Otalora, Sonia; Otero, Remedios; Parra, Pedro; Parra, Virginia; Pedrajas, José María; Pellejero, Galadriel; Porras, José Antonio; Portillo, José; Riera‐Mestre, Antoni; Rivas, Agustina; Rivera‐Civico, Francisco; Rodríguez‐Chiaradía, Diego Agustín; Rodríguez‐Cobo, Ana; Rodríguez‐Matute, Consolación; Rogado, Jacobo; Roig, Sonia; Rosa, Vladimir; Ruiz‐Artacho, Pedro; Ruiz‐Giménez, Nuria; Ruiz‐Ruiz, Justo; Ruiz‐Sada, Pablo; Salgueiro, Giorgina; Sánchez‐Martínez, Rosario; Sánchez‐Muñoz‐Torrero, Juan Francisco; Sancho, Teresa; Sirisi, Merçe; Soler, Silvia; Suriñach, José María; Tirado, Raimundo; Torres, María Isabel; Tolosa, Carles; Trujillo‐Santos, Javier; Uresandi, Fernando; Valero, Beatriz; Valle, Reina; Vela, Jerónimo Ramón; Vidal, Gemma; Villares, Paula; Zamora, Carles; Gutiérrez, Paula; Vázquez, Fernando Javier; Engelen, Matthias; Vanassche, Thomas; Verhamme, Peter; Hirmerova, Jana; Malý, Radovan; Salgado, Estuardo; Ait Abdallah, Nassim; Bertoletti, Laurent; Bura‐Riviere, Alessandra; Crichi, Benjamin; Debourdeau, Philippe; Olivier, Espitia; Falvo, Nicolas; Farge‐Bancel, Dominique; Galliazzo, Silvia; Helfer, Hélène; Mahé, Isabelle; Moustafa, Farès; Poenou, Geraldine; Schellong, Sebastian; Braester, Andrei; Brenner, Benjamin; Tzoran, Inna; Bilora, Franca; Bucherini, Eugenio; Ciammaichella, Maurizio; Di Micco, Pierpaolo; Imbalzano, Egidio; Maida, Rosa; Mastroiacovo, Daniela; Pace, Federica; Pesavento, Raffaele; Pomero, Fulvio; Prandoni, Paolo; Quintavalla, Roberto; Rocci, Anna; Romualdi, Roberta; Siniscalchi, Carmine; Tufano, Antonella; Visonà, Adriana; Zalunardo, Beniamino; Gibietis, Valdis; Kigitovica, Dana; Skride, Andris; Fonseca, Samuel; Martins, Filipa; Meireles, Jose; Bosevski, Marijan; Bounameaux, Henri; Mazzolai, Lucia; Bikdeli, Behnood; Caprini, Joseph A.; Tafur, Alfonso J.; Ochoa‐Chaar, Cassius Iyad; Weinberg, Ido; Wilkins, Hannah; Bui, Hanh MyBackground Atrial fibrillation (AF) may exist before or occur early in the course of pulmonary embolism (PE). We determined the PE outcomes based on the presence and timing of AF. Methods and Results Using the data from a multicenter PE registry, we identified 3 groups: (1) those with preexisting AF, (2) patients with new AF within 2 days from acute PE (incident AF), and (3) patients without AF. We assessed the 90-day and 1-year risk of mortality and stroke in patients with AF, compared with those without AF (reference group). Among 16 497 patients with PE, 792 had preexisting AF. These patients had increased odds of 90-day all-cause (odds ratio [OR], 2.81; 95% CI, 2.33-3.38) and PE-related mortality (OR, 2.38; 95% CI, 1.37-4.14) and increased 1-year hazard for ischemic stroke (hazard ratio, 5.48; 95% CI, 3.10-9.69) compared with those without AF. After multivariable adjustment, preexisting AF was associated with significantly increased odds of all-cause mortality (OR, 1.91; 95% CI, 1.57-2.32) but not PE-related mortality (OR, 1.50; 95% CI, 0.85-2.66). Among 16 497 patients with PE, 445 developed new incident AF within 2 days of acute PE. Incident AF was associated with increased odds of 90-day all-cause (OR, 2.28; 95% CI, 1.75-2.97) and PE-related (OR, 3.64; 95% CI, 2.01-6.59) mortality but not stroke. Findings were similar in multivariable analyses. Conclusions In patients with acute symptomatic PE, both preexisting AF and incident AF predict adverse clinical outcomes. The type of adverse outcomes may differ depending on the timing of AF onset.
- Transitioning intravenous epoprostenol to oral selexipag in idiopathic pulmonary arterial hypertension: a case reportPublication . Alexandre, André; Furtado, Inês; Gonçalves, Fabienne; Gonçalves, Fabienne; Melo, Alzira; Alves, Joana; Santos, Mario; Reis, AbilioIntravenous (i.v.) prostacyclin is the cornerstone treatment in high-risk pulmonary arterial hypertension (PAH) patients. Selexipag is an orally available prostacyclin receptor agonist. Limited data are available regarding the feasibility of transitioning from i.v. epoprostenol to selexipag. A 50-year-old woman with idiopathic PAH was diagnosed in a World Health Organization (WHO) Functional Class (FC) IV. She improved with upfront triple combination therapy, including i.v. epoprostenol. Over 2 years of follow-up, the patient remained at low risk and expressed strong preference towards oral therapies. After careful risk-benefit clinical consideration, she was transitioned from i.v. epoprostenol to selexipag. Selexipag was started at dosage of 200 μg twice daily (b.i.d.) and titrated up to 1600 μg b.i.d. over 8 weeks (up-titration of 200 μg b.i.d. every week). Simultaneously, i.v. epoprostenol was down-titrated 3.0 ng/kg/min every week from a dosage of 27.5 ng/kg/min. The transition occurred under strict medical surveillance and was well tolerated. One year after discontinuation of epoprostenol, the patient remains in WHO FC I and has no signs of clinical deterioration. Although not generalizable to most PAH patients, this case highlights that a carefully planned transition from epoprostenol to selexipag is feasible in selected low-risk patients within a shared medical decision-making framework.
- Pulmonary arterial hypertension registries: past, present and into the futurePublication . Weatherald, J.; Reis, Abilio; Sitbon, O.; Humbert, M.Registries have greatly contributed to knowledge about PAH epidemiology, risk factors, prognosis and treatment. Future registries face unique challenges but may benefit from integration of multiple data sources and capitalising on “Big Data” opportunities
- PH CARE COVID survey: an international patient survey on the care for pulmonary hypertension patients during the early phase of the COVID-19 pandemicPublication . Godinas, Laurent; Iyer, Keerthana; Meszaros, Gergely; Quarck, Rozenn; Escribano-Subias, Pilar; Vonk Noordegraaf, Anton; Jansa, Pavel; D’Alto, Michele; Luknar, Milan; Milutinov Ilic, Senka; Belge, Catharina; Sitbon, Olivier; Reis, Abilio; Rosenkranz, Stephan; Pepke-Zaba, Joanna; Humbert, Marc; Delcroix, MarionBackground: During the COVID-19 pandemic, most of the health care systems suspended their non-urgent activities. This included the cancellation of consultations for patients with rare diseases, such as severe pulmonary hypertension (PH), resulting in potential medication shortage and loss of follow-up. Thus, the aim of the study was to evaluate PH patient health status evolution, access to health care and mental health experience during the early phase of the pandemic. Methods: We conducted an online patient survey, available in 16 languages, between 22/05/2020 and 28/06/2020. The survey included questions corresponding to demographic, COVID-19 and PH related information. Results: 1073 patients (or relatives, 27%) from 52 countries all over the world participated in the survey. Seventy-seven percent (77%) of responders reported a diagnosis of pulmonary arterial hypertension and 15% of chronic thromboembolic PH. The COVID-19 related events were few: only 1% of all responders reported a diagnosis of COVID-19. However, 8% of patients reported health deterioration possibly related to PH, and 4% hospitalization for PH. Besides, 11% of the patients reported difficulties to access their PH expert centre, and 3% interruption of treatment due to shortage of medication. Anxiety or depression was reported by 67% of the participants. Conclusion: Although COVID-19 incidence in PH patients was low, PH related problems occurred frequently as the pandemic progressed, including difficulties to have access to specialized care. The importance of primary health care was emphasized. Further studies are needed to evaluate the long-term consequences of COVID-related PH care disruption.
- Severe Pulmonary Hypertension Management Across Europe (PHAROS): an ERS Clinical Research CollaborationPublication . Sitbon, Olivier; Reis, Abilio; Humbert, Marc; Vonk Noordegraaf, Anton; Delcroix, MarionThe overall objective of the PHAROS CRC is to establish a platform for clinical research in pulmonary hypertension, in close collaboration with the ERN-Lung, able to coordinate future collaborative research in Europe https://bit.ly/2Rx2FTl