Pulmonary hypertension in Finland 2008-2020: A descriptive real-world cohort study (FINPAH)

Background: To assess characteristics, risk group distribution, and prognosis of patients with pulmonary arterial hypertension (PAH) or chronic thromboembolic pulmonary hypertension (CTEPH) in Finland. Methods: Clinical chart review of patients with PAH or CTEPH recorded between 2008 and 2019 and li...

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Main Authors: Markku Pentikäinen, Piia Simonen, Helena Tuunanen, Pauliina Leskelä, Terttu Harju, Pertti Jääskeläinen, Christian Asseburg, Minna Oksanen, Erkki Soini, Christina Wennerström, Airi Puhakka, Elina Heliövaara, Katriina Kahlos, Pentti Korhonen, Tiina Kyllönen, Kirsi Majamaa-Voltti, Anu Turpeinen, Ville Vepsäläinen, Tapani Vihinen
Format: Article
Language:English
Published: Elsevier 2025-02-01
Series:JHLT Open
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Online Access:http://www.sciencedirect.com/science/article/pii/S295013342400140X
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author Markku Pentikäinen
Piia Simonen
Helena Tuunanen
Pauliina Leskelä
Terttu Harju
Pertti Jääskeläinen
Christian Asseburg
Minna Oksanen
Erkki Soini
Christina Wennerström
Airi Puhakka
Terttu Harju
Elina Heliövaara
Pertti Jääskeläinen
Katriina Kahlos
Pentti Korhonen
Tiina Kyllönen
Pauliina Leskelä
Kirsi Majamaa-Voltti
Piia Simonen
Anu Turpeinen
Helena Tuunanen
Ville Vepsäläinen
Tapani Vihinen
author_facet Markku Pentikäinen
Piia Simonen
Helena Tuunanen
Pauliina Leskelä
Terttu Harju
Pertti Jääskeläinen
Christian Asseburg
Minna Oksanen
Erkki Soini
Christina Wennerström
Airi Puhakka
Terttu Harju
Elina Heliövaara
Pertti Jääskeläinen
Katriina Kahlos
Pentti Korhonen
Tiina Kyllönen
Pauliina Leskelä
Kirsi Majamaa-Voltti
Piia Simonen
Anu Turpeinen
Helena Tuunanen
Ville Vepsäläinen
Tapani Vihinen
author_sort Markku Pentikäinen
collection DOAJ
description Background: To assess characteristics, risk group distribution, and prognosis of patients with pulmonary arterial hypertension (PAH) or chronic thromboembolic pulmonary hypertension (CTEPH) in Finland. Methods: Clinical chart review of patients with PAH or CTEPH recorded between 2008 and 2019 and linkage to official mortality data. Results: We identified 627 patients, with 502 (80%) diagnosed after 2008, yielding an incidence of PAH and CTEPH of 4.0 and 2.9/million/year, respectively. The median time from symptoms to diagnosis was 1 year. Mean age at diagnosis of PAH patients (n = 268) was 57 years, 73% were women, 40% had idiopathic PAH, 28% associated with connective tissue diseases, and 15% with congenital heart disease, 9% had ≥3 cardiovascular comorbidities. At 1 year, 34%/34%/24%/8% were at the low/intermediate-low/intermediate-high/high Compera 2.0 risk classification groups. Survival was 91.3%, 74.8%, and 62.6% at 1, 3, and 5 years, respectively, with an improving trend over calendar time. Ten PAH patients had a lung transplant. PAH subtype, cardiac output, and the presence of ischemic heart disease or type 2 diabetes predicted survival.CTEPH patients (n = 189) were 63 years (mean) at diagnosis and 49% were women. Of the CTEPH patients, 29% underwent pulmonary endarterectomy (PEA) and 22% were treated with balloon pulmonary angioplasty. Survival was 94.6%, 87.2%, and 79.4% at 1, 3, and 5 years, respectively. PEA patients were younger, had fewer comorbidities, and had longer survival than non-PEA patients. Conclusions: Incidence and survival of PAH and CTEPH patients in Finland were similar to previously presented data for other countries.
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spelling doaj-art-e062c0d7f4d54128bacd9ef66fa17c242025-02-09T05:01:58ZengElsevierJHLT Open2950-13342025-02-017100191Pulmonary hypertension in Finland 2008-2020: A descriptive real-world cohort study (FINPAH)Markku Pentikäinen0Piia Simonen1Helena Tuunanen2Pauliina Leskelä3Terttu Harju4Pertti Jääskeläinen5Christian Asseburg6Minna Oksanen7Erkki Soini8Christina Wennerström9Airi Puhakka10Terttu Harju11Elina Heliövaara12Pertti Jääskeläinen13Katriina Kahlos14Pentti Korhonen15Tiina Kyllönen16Pauliina Leskelä17Kirsi Majamaa-Voltti18Piia Simonen19Anu Turpeinen20Helena Tuunanen21Ville Vepsäläinen22Tapani Vihinen23Helsinki University Hospital and University of Helsinki, Helsinki, Finland; Corresponding author: Markku Pentikäinen, Helsinki University Hospital, Haartmaninkatu 4, P.O. Box 340, 00029 HUS, Helsinki, Finland.Helsinki University Hospital and University of Helsinki, Helsinki, FinlandTurku University Hospital, Turku, FinlandTampere University Hospital, Tampere, FinlandOulu University Hospital, Oulu, FinlandKuopio University Hospital, Kuopio, FinlandESiOR Oy, Kuopio, FinlandESiOR Oy, Kuopio, FinlandESiOR Oy, Kuopio, FinlandJanssen-Cilag AB, Solna, SwedenJanssen-Cilag Oy, Espoo, FinlandOulu University Hospital, Oulu, FinlandHelsinki University Hospital and University of Helsinki, Helsinki, FinlandKuopio University Hospital, Kuopio, FinlandOulu University Hospital, Oulu, FinlandTampere University Hospital, Tampere, FinlandOulu University Hospital, Oulu, FinlandTampere University Hospital, Tampere, FinlandOulu University Hospital, Oulu, FinlandOulu University Hospital, Oulu, FinlandKuopio University Hospital, Kuopio, FinlandTurku University Hospital, Turku, FinlandKuopio University Hospital, Kuopio, FinlandSatasairaala Hospital, Pori, FinlandBackground: To assess characteristics, risk group distribution, and prognosis of patients with pulmonary arterial hypertension (PAH) or chronic thromboembolic pulmonary hypertension (CTEPH) in Finland. Methods: Clinical chart review of patients with PAH or CTEPH recorded between 2008 and 2019 and linkage to official mortality data. Results: We identified 627 patients, with 502 (80%) diagnosed after 2008, yielding an incidence of PAH and CTEPH of 4.0 and 2.9/million/year, respectively. The median time from symptoms to diagnosis was 1 year. Mean age at diagnosis of PAH patients (n = 268) was 57 years, 73% were women, 40% had idiopathic PAH, 28% associated with connective tissue diseases, and 15% with congenital heart disease, 9% had ≥3 cardiovascular comorbidities. At 1 year, 34%/34%/24%/8% were at the low/intermediate-low/intermediate-high/high Compera 2.0 risk classification groups. Survival was 91.3%, 74.8%, and 62.6% at 1, 3, and 5 years, respectively, with an improving trend over calendar time. Ten PAH patients had a lung transplant. PAH subtype, cardiac output, and the presence of ischemic heart disease or type 2 diabetes predicted survival.CTEPH patients (n = 189) were 63 years (mean) at diagnosis and 49% were women. Of the CTEPH patients, 29% underwent pulmonary endarterectomy (PEA) and 22% were treated with balloon pulmonary angioplasty. Survival was 94.6%, 87.2%, and 79.4% at 1, 3, and 5 years, respectively. PEA patients were younger, had fewer comorbidities, and had longer survival than non-PEA patients. Conclusions: Incidence and survival of PAH and CTEPH patients in Finland were similar to previously presented data for other countries.http://www.sciencedirect.com/science/article/pii/S295013342400140Xpulmonary arterial hypertensionchronic thromboembolic pulmonary hypertensionreal-world evidenceregistry studysurvival
spellingShingle Markku Pentikäinen
Piia Simonen
Helena Tuunanen
Pauliina Leskelä
Terttu Harju
Pertti Jääskeläinen
Christian Asseburg
Minna Oksanen
Erkki Soini
Christina Wennerström
Airi Puhakka
Terttu Harju
Elina Heliövaara
Pertti Jääskeläinen
Katriina Kahlos
Pentti Korhonen
Tiina Kyllönen
Pauliina Leskelä
Kirsi Majamaa-Voltti
Piia Simonen
Anu Turpeinen
Helena Tuunanen
Ville Vepsäläinen
Tapani Vihinen
Pulmonary hypertension in Finland 2008-2020: A descriptive real-world cohort study (FINPAH)
JHLT Open
pulmonary arterial hypertension
chronic thromboembolic pulmonary hypertension
real-world evidence
registry study
survival
title Pulmonary hypertension in Finland 2008-2020: A descriptive real-world cohort study (FINPAH)
title_full Pulmonary hypertension in Finland 2008-2020: A descriptive real-world cohort study (FINPAH)
title_fullStr Pulmonary hypertension in Finland 2008-2020: A descriptive real-world cohort study (FINPAH)
title_full_unstemmed Pulmonary hypertension in Finland 2008-2020: A descriptive real-world cohort study (FINPAH)
title_short Pulmonary hypertension in Finland 2008-2020: A descriptive real-world cohort study (FINPAH)
title_sort pulmonary hypertension in finland 2008 2020 a descriptive real world cohort study finpah
topic pulmonary arterial hypertension
chronic thromboembolic pulmonary hypertension
real-world evidence
registry study
survival
url http://www.sciencedirect.com/science/article/pii/S295013342400140X
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