Clinical implications of partial anomalous pulmonary venous connection: a rare cause of severe pulmonary arterial hypertension

Isolated partial anomalous pulmonary venous connection (PAPVC) is an uncommon congenital heart anomaly that is sporadically associated with pulmonary arterial hypertension in the adult population. The diagnosis and therapy for this condition are challenging. We report on three cases of patients with...
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Autor*in:	Alex Pizzini [verfasserIn] Thomas Sonnweber [verfasserIn] Renate Frank [verfasserIn] Markus Theurl [verfasserIn] Günter Weiss [verfasserIn] Ivan Tancevski [verfasserIn] Judith Löffler-Ragg [verfasserIn]

Format:	E-Artikel
Sprache:	Englisch

Erschienen:	2020

Übergeordnetes Werk:	In: Pulmonary Circulation - Wiley, 2018, 10(2020)
Übergeordnetes Werk:	volume:10 ; year:2020

Links:	Link aufrufen Link aufrufen Link aufrufen Journal toc

DOI / URN:	10.1177/2045894019885352

Katalog-ID:	DOAJ027469034

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520			\|a Isolated partial anomalous pulmonary venous connection (PAPVC) is an uncommon congenital heart anomaly that is sporadically associated with pulmonary arterial hypertension in the adult population. The diagnosis and therapy for this condition are challenging. We report on three cases of patients with unexpected severe precapillary pulmonary hypertension in single PAPVC treated with an upfront pulmonary arterial hypertension-specific combination therapy. Our cases indicate that the combination of PAPVC and pulmonary comorbidities may trigger the development of severe pulmonary arterial hypertension. The initiation of pulmonary arterial hypertension-targeted combination therapy revealed to be a safe and efficacious strategy for patients with PAPVC-associated severe pulmonary arterial hypertension.
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spelling	10.1177/2045894019885352 doi (DE-627)DOAJ027469034 (DE-599)DOAJ1caab4064cfe41a5a9d0d177bc6ae366 DE-627 ger DE-627 rakwb eng RC666-701 RC705-779 Alex Pizzini verfasserin aut Clinical implications of partial anomalous pulmonary venous connection: a rare cause of severe pulmonary arterial hypertension 2020 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier Isolated partial anomalous pulmonary venous connection (PAPVC) is an uncommon congenital heart anomaly that is sporadically associated with pulmonary arterial hypertension in the adult population. The diagnosis and therapy for this condition are challenging. We report on three cases of patients with unexpected severe precapillary pulmonary hypertension in single PAPVC treated with an upfront pulmonary arterial hypertension-specific combination therapy. Our cases indicate that the combination of PAPVC and pulmonary comorbidities may trigger the development of severe pulmonary arterial hypertension. The initiation of pulmonary arterial hypertension-targeted combination therapy revealed to be a safe and efficacious strategy for patients with PAPVC-associated severe pulmonary arterial hypertension. Diseases of the circulatory (Cardiovascular) system Diseases of the respiratory system Thomas Sonnweber verfasserin aut Renate Frank verfasserin aut Markus Theurl verfasserin aut Günter Weiss verfasserin aut Ivan Tancevski verfasserin aut Judith Löffler-Ragg verfasserin aut In Pulmonary Circulation Wiley, 2018 10(2020) (DE-627)672806649 (DE-600)2638089-4 20458940 nnns volume:10 year:2020 https://doi.org/10.1177/2045894019885352 kostenfrei https://doaj.org/article/1caab4064cfe41a5a9d0d177bc6ae366 kostenfrei https://doi.org/10.1177/2045894019885352 kostenfrei https://doaj.org/toc/2045-8940 Journal toc kostenfrei GBV_USEFLAG_A SYSFLAG_A GBV_DOAJ GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_171 GBV_ILN_206 GBV_ILN_213 GBV_ILN_224 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_374 GBV_ILN_602 GBV_ILN_636 GBV_ILN_2004 GBV_ILN_2005 GBV_ILN_2006 GBV_ILN_2007 GBV_ILN_2010 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2026 GBV_ILN_2027 GBV_ILN_2034 GBV_ILN_2037 GBV_ILN_2038 GBV_ILN_2044 GBV_ILN_2048 GBV_ILN_2050 GBV_ILN_2055 GBV_ILN_2056 GBV_ILN_2057 GBV_ILN_2059 GBV_ILN_2061 GBV_ILN_2064 GBV_ILN_2068 GBV_ILN_2088 GBV_ILN_2106 GBV_ILN_2108 GBV_ILN_2110 GBV_ILN_2111 GBV_ILN_2118 GBV_ILN_2122 GBV_ILN_2143 GBV_ILN_2144 GBV_ILN_2147 GBV_ILN_2148 GBV_ILN_2152 GBV_ILN_2153 GBV_ILN_2232 GBV_ILN_2470 GBV_ILN_2507 GBV_ILN_2522 GBV_ILN_2704 GBV_ILN_2707 GBV_ILN_2890 GBV_ILN_4012 GBV_ILN_4035 GBV_ILN_4037 GBV_ILN_4046 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4242 GBV_ILN_4249 GBV_ILN_4251 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4326 GBV_ILN_4333 GBV_ILN_4334 GBV_ILN_4335 GBV_ILN_4336 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 10 2020
allfields_unstemmed	10.1177/2045894019885352 doi (DE-627)DOAJ027469034 (DE-599)DOAJ1caab4064cfe41a5a9d0d177bc6ae366 DE-627 ger DE-627 rakwb eng RC666-701 RC705-779 Alex Pizzini verfasserin aut Clinical implications of partial anomalous pulmonary venous connection: a rare cause of severe pulmonary arterial hypertension 2020 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier Isolated partial anomalous pulmonary venous connection (PAPVC) is an uncommon congenital heart anomaly that is sporadically associated with pulmonary arterial hypertension in the adult population. The diagnosis and therapy for this condition are challenging. We report on three cases of patients with unexpected severe precapillary pulmonary hypertension in single PAPVC treated with an upfront pulmonary arterial hypertension-specific combination therapy. Our cases indicate that the combination of PAPVC and pulmonary comorbidities may trigger the development of severe pulmonary arterial hypertension. The initiation of pulmonary arterial hypertension-targeted combination therapy revealed to be a safe and efficacious strategy for patients with PAPVC-associated severe pulmonary arterial hypertension. Diseases of the circulatory (Cardiovascular) system Diseases of the respiratory system Thomas Sonnweber verfasserin aut Renate Frank verfasserin aut Markus Theurl verfasserin aut Günter Weiss verfasserin aut Ivan Tancevski verfasserin aut Judith Löffler-Ragg verfasserin aut In Pulmonary Circulation Wiley, 2018 10(2020) (DE-627)672806649 (DE-600)2638089-4 20458940 nnns volume:10 year:2020 https://doi.org/10.1177/2045894019885352 kostenfrei https://doaj.org/article/1caab4064cfe41a5a9d0d177bc6ae366 kostenfrei https://doi.org/10.1177/2045894019885352 kostenfrei https://doaj.org/toc/2045-8940 Journal toc kostenfrei GBV_USEFLAG_A SYSFLAG_A GBV_DOAJ GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_171 GBV_ILN_206 GBV_ILN_213 GBV_ILN_224 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_374 GBV_ILN_602 GBV_ILN_636 GBV_ILN_2004 GBV_ILN_2005 GBV_ILN_2006 GBV_ILN_2007 GBV_ILN_2010 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2026 GBV_ILN_2027 GBV_ILN_2034 GBV_ILN_2037 GBV_ILN_2038 GBV_ILN_2044 GBV_ILN_2048 GBV_ILN_2050 GBV_ILN_2055 GBV_ILN_2056 GBV_ILN_2057 GBV_ILN_2059 GBV_ILN_2061 GBV_ILN_2064 GBV_ILN_2068 GBV_ILN_2088 GBV_ILN_2106 GBV_ILN_2108 GBV_ILN_2110 GBV_ILN_2111 GBV_ILN_2118 GBV_ILN_2122 GBV_ILN_2143 GBV_ILN_2144 GBV_ILN_2147 GBV_ILN_2148 GBV_ILN_2152 GBV_ILN_2153 GBV_ILN_2232 GBV_ILN_2470 GBV_ILN_2507 GBV_ILN_2522 GBV_ILN_2704 GBV_ILN_2707 GBV_ILN_2890 GBV_ILN_4012 GBV_ILN_4035 GBV_ILN_4037 GBV_ILN_4046 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4242 GBV_ILN_4249 GBV_ILN_4251 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4326 GBV_ILN_4333 GBV_ILN_4334 GBV_ILN_4335 GBV_ILN_4336 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 10 2020
allfieldsGer	10.1177/2045894019885352 doi (DE-627)DOAJ027469034 (DE-599)DOAJ1caab4064cfe41a5a9d0d177bc6ae366 DE-627 ger DE-627 rakwb eng RC666-701 RC705-779 Alex Pizzini verfasserin aut Clinical implications of partial anomalous pulmonary venous connection: a rare cause of severe pulmonary arterial hypertension 2020 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier Isolated partial anomalous pulmonary venous connection (PAPVC) is an uncommon congenital heart anomaly that is sporadically associated with pulmonary arterial hypertension in the adult population. The diagnosis and therapy for this condition are challenging. We report on three cases of patients with unexpected severe precapillary pulmonary hypertension in single PAPVC treated with an upfront pulmonary arterial hypertension-specific combination therapy. Our cases indicate that the combination of PAPVC and pulmonary comorbidities may trigger the development of severe pulmonary arterial hypertension. The initiation of pulmonary arterial hypertension-targeted combination therapy revealed to be a safe and efficacious strategy for patients with PAPVC-associated severe pulmonary arterial hypertension. Diseases of the circulatory (Cardiovascular) system Diseases of the respiratory system Thomas Sonnweber verfasserin aut Renate Frank verfasserin aut Markus Theurl verfasserin aut Günter Weiss verfasserin aut Ivan Tancevski verfasserin aut Judith Löffler-Ragg verfasserin aut In Pulmonary Circulation Wiley, 2018 10(2020) (DE-627)672806649 (DE-600)2638089-4 20458940 nnns volume:10 year:2020 https://doi.org/10.1177/2045894019885352 kostenfrei https://doaj.org/article/1caab4064cfe41a5a9d0d177bc6ae366 kostenfrei https://doi.org/10.1177/2045894019885352 kostenfrei https://doaj.org/toc/2045-8940 Journal toc kostenfrei GBV_USEFLAG_A SYSFLAG_A GBV_DOAJ GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_171 GBV_ILN_206 GBV_ILN_213 GBV_ILN_224 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_374 GBV_ILN_602 GBV_ILN_636 GBV_ILN_2004 GBV_ILN_2005 GBV_ILN_2006 GBV_ILN_2007 GBV_ILN_2010 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2026 GBV_ILN_2027 GBV_ILN_2034 GBV_ILN_2037 GBV_ILN_2038 GBV_ILN_2044 GBV_ILN_2048 GBV_ILN_2050 GBV_ILN_2055 GBV_ILN_2056 GBV_ILN_2057 GBV_ILN_2059 GBV_ILN_2061 GBV_ILN_2064 GBV_ILN_2068 GBV_ILN_2088 GBV_ILN_2106 GBV_ILN_2108 GBV_ILN_2110 GBV_ILN_2111 GBV_ILN_2118 GBV_ILN_2122 GBV_ILN_2143 GBV_ILN_2144 GBV_ILN_2147 GBV_ILN_2148 GBV_ILN_2152 GBV_ILN_2153 GBV_ILN_2232 GBV_ILN_2470 GBV_ILN_2507 GBV_ILN_2522 GBV_ILN_2704 GBV_ILN_2707 GBV_ILN_2890 GBV_ILN_4012 GBV_ILN_4035 GBV_ILN_4037 GBV_ILN_4046 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4242 GBV_ILN_4249 GBV_ILN_4251 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4326 GBV_ILN_4333 GBV_ILN_4334 GBV_ILN_4335 GBV_ILN_4336 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 10 2020
allfieldsSound	10.1177/2045894019885352 doi (DE-627)DOAJ027469034 (DE-599)DOAJ1caab4064cfe41a5a9d0d177bc6ae366 DE-627 ger DE-627 rakwb eng RC666-701 RC705-779 Alex Pizzini verfasserin aut Clinical implications of partial anomalous pulmonary venous connection: a rare cause of severe pulmonary arterial hypertension 2020 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier Isolated partial anomalous pulmonary venous connection (PAPVC) is an uncommon congenital heart anomaly that is sporadically associated with pulmonary arterial hypertension in the adult population. The diagnosis and therapy for this condition are challenging. We report on three cases of patients with unexpected severe precapillary pulmonary hypertension in single PAPVC treated with an upfront pulmonary arterial hypertension-specific combination therapy. Our cases indicate that the combination of PAPVC and pulmonary comorbidities may trigger the development of severe pulmonary arterial hypertension. The initiation of pulmonary arterial hypertension-targeted combination therapy revealed to be a safe and efficacious strategy for patients with PAPVC-associated severe pulmonary arterial hypertension. Diseases of the circulatory (Cardiovascular) system Diseases of the respiratory system Thomas Sonnweber verfasserin aut Renate Frank verfasserin aut Markus Theurl verfasserin aut Günter Weiss verfasserin aut Ivan Tancevski verfasserin aut Judith Löffler-Ragg verfasserin aut In Pulmonary Circulation Wiley, 2018 10(2020) (DE-627)672806649 (DE-600)2638089-4 20458940 nnns volume:10 year:2020 https://doi.org/10.1177/2045894019885352 kostenfrei https://doaj.org/article/1caab4064cfe41a5a9d0d177bc6ae366 kostenfrei https://doi.org/10.1177/2045894019885352 kostenfrei https://doaj.org/toc/2045-8940 Journal toc kostenfrei GBV_USEFLAG_A SYSFLAG_A GBV_DOAJ GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_171 GBV_ILN_206 GBV_ILN_213 GBV_ILN_224 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_374 GBV_ILN_602 GBV_ILN_636 GBV_ILN_2004 GBV_ILN_2005 GBV_ILN_2006 GBV_ILN_2007 GBV_ILN_2010 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2026 GBV_ILN_2027 GBV_ILN_2034 GBV_ILN_2037 GBV_ILN_2038 GBV_ILN_2044 GBV_ILN_2048 GBV_ILN_2050 GBV_ILN_2055 GBV_ILN_2056 GBV_ILN_2057 GBV_ILN_2059 GBV_ILN_2061 GBV_ILN_2064 GBV_ILN_2068 GBV_ILN_2088 GBV_ILN_2106 GBV_ILN_2108 GBV_ILN_2110 GBV_ILN_2111 GBV_ILN_2118 GBV_ILN_2122 GBV_ILN_2143 GBV_ILN_2144 GBV_ILN_2147 GBV_ILN_2148 GBV_ILN_2152 GBV_ILN_2153 GBV_ILN_2232 GBV_ILN_2470 GBV_ILN_2507 GBV_ILN_2522 GBV_ILN_2704 GBV_ILN_2707 GBV_ILN_2890 GBV_ILN_4012 GBV_ILN_4035 GBV_ILN_4037 GBV_ILN_4046 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4242 GBV_ILN_4249 GBV_ILN_4251 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4326 GBV_ILN_4333 GBV_ILN_4334 GBV_ILN_4335 GBV_ILN_4336 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 10 2020
language	English
source	In Pulmonary Circulation 10(2020) volume:10 year:2020
sourceStr	In Pulmonary Circulation 10(2020) volume:10 year:2020
format_phy_str_mv	Article
institution	findex.gbv.de
topic_facet	Diseases of the circulatory (Cardiovascular) system Diseases of the respiratory system
isfreeaccess_bool	true
container_title	Pulmonary Circulation
authorswithroles_txt_mv	Alex Pizzini @@aut@@ Thomas Sonnweber @@aut@@ Renate Frank @@aut@@ Markus Theurl @@aut@@ Günter Weiss @@aut@@ Ivan Tancevski @@aut@@ Judith Löffler-Ragg @@aut@@
publishDateDaySort_date	2020-01-01T00:00:00Z
hierarchy_top_id	672806649
id	DOAJ027469034
language_de	englisch
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callnumber-first	R - Medicine
author	Alex Pizzini
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topic_title	RC666-701 RC705-779 Clinical implications of partial anomalous pulmonary venous connection: a rare cause of severe pulmonary arterial hypertension
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title	Clinical implications of partial anomalous pulmonary venous connection: a rare cause of severe pulmonary arterial hypertension
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title_full	Clinical implications of partial anomalous pulmonary venous connection: a rare cause of severe pulmonary arterial hypertension
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title_sort	clinical implications of partial anomalous pulmonary venous connection: a rare cause of severe pulmonary arterial hypertension
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title_auth	Clinical implications of partial anomalous pulmonary venous connection: a rare cause of severe pulmonary arterial hypertension
abstract	Isolated partial anomalous pulmonary venous connection (PAPVC) is an uncommon congenital heart anomaly that is sporadically associated with pulmonary arterial hypertension in the adult population. The diagnosis and therapy for this condition are challenging. We report on three cases of patients with unexpected severe precapillary pulmonary hypertension in single PAPVC treated with an upfront pulmonary arterial hypertension-specific combination therapy. Our cases indicate that the combination of PAPVC and pulmonary comorbidities may trigger the development of severe pulmonary arterial hypertension. The initiation of pulmonary arterial hypertension-targeted combination therapy revealed to be a safe and efficacious strategy for patients with PAPVC-associated severe pulmonary arterial hypertension.
abstractGer	Isolated partial anomalous pulmonary venous connection (PAPVC) is an uncommon congenital heart anomaly that is sporadically associated with pulmonary arterial hypertension in the adult population. The diagnosis and therapy for this condition are challenging. We report on three cases of patients with unexpected severe precapillary pulmonary hypertension in single PAPVC treated with an upfront pulmonary arterial hypertension-specific combination therapy. Our cases indicate that the combination of PAPVC and pulmonary comorbidities may trigger the development of severe pulmonary arterial hypertension. The initiation of pulmonary arterial hypertension-targeted combination therapy revealed to be a safe and efficacious strategy for patients with PAPVC-associated severe pulmonary arterial hypertension.
abstract_unstemmed	Isolated partial anomalous pulmonary venous connection (PAPVC) is an uncommon congenital heart anomaly that is sporadically associated with pulmonary arterial hypertension in the adult population. The diagnosis and therapy for this condition are challenging. We report on three cases of patients with unexpected severe precapillary pulmonary hypertension in single PAPVC treated with an upfront pulmonary arterial hypertension-specific combination therapy. Our cases indicate that the combination of PAPVC and pulmonary comorbidities may trigger the development of severe pulmonary arterial hypertension. The initiation of pulmonary arterial hypertension-targeted combination therapy revealed to be a safe and efficacious strategy for patients with PAPVC-associated severe pulmonary arterial hypertension.
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title_short	Clinical implications of partial anomalous pulmonary venous connection: a rare cause of severe pulmonary arterial hypertension
url	https://doi.org/10.1177/2045894019885352 https://doaj.org/article/1caab4064cfe41a5a9d0d177bc6ae366 https://doaj.org/toc/2045-8940
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Clinical implications of partial anomalous pulmonary venous connection: a rare cause of severe pulmonary arterial hypertension

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