Emphasizing the importance of the clinical classification for pulmonary hypertension
Mateo Porres-Aguilar
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* Department of Internal Medicine, Texas Tech University Health Sciences Center, Paul L. Foster School of Medicine; El Paso, TX, USA.
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Foster School of Medicine; El Paso, TX, USA." "etiqueta" => "*" "identificador" => "aff1" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor1" "etiqueta" => "*" "correspondencia" => "Correspondence and reprint request:" ] ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Dear Editor:</span><p id="p0005" class="elsevierStylePara elsevierViewall">Pulmonary arterial hypertension (PAH) represents a particular subgroup of patients within the wide clinical spectrum of pulmonary hypertension (PH) increasing population, which is characterized by progressive elevation of pulmonary arterial pressures leading to right ventricular failure and death.<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> The PH nomenclature and classification deserves always a particular section when scientists and clinicians intent to review this topic, and has been revised several times, most recently during the 2008 World Symposium of PH at Dana Point, California.<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a></p><p id="p0010" class="elsevierStylePara elsevierViewall">I think this symposium clarified several issues in terms of the previous misuse and misinterpretation while using old abbreviations and nomenclature regarding the heterogeneous group of patients including under the clinical spectrum of PH. Primary pulmonary hypertension (PPH), in our days, means unexplained PH or, in better words, idiopathic pulmonary arterial hypertension (IPAH). This entity has been subject of great interest among pulmonologists, cardiologists, rheumatologists, even some gastroenterologists/ hepatologists, in terms of exploring more through clinical and basic (molecular biology) research nowadays. With the term PPH on board before the Venice and Dana Point meetings in 2003 and 2008, respectively, the term “secondary PH” started to growth in the clinical setting, encompassing very heterogeneous group of diseases including other intrinsic pulmonary vascular diseases similar to PPH as well as disorders that affected the pulmonary venous circulation and conditions that affected the pulmonary circulation by altering lung parenchyma by anatomical and physiological means.</p><p id="p0015" class="elsevierStylePara elsevierViewall">Thus, one of the most important facts during the world Venice symposium was to completely eliminate the term PPH has had been remained during the world PH Evian convention in 1998,<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> by proposing to abandon such nomenclature and, thus, by those modifications on board, the PAH group or Group 1 according to the Venice revised classification included the following: 1) idiopathic pulmonary arterial hypertension (IPAH), 2) familial pulmonary arterial hypertension (FPAH), and 3) pulmonary arterial hypertension related to risk factors or associated conditions (APAH) in which portal hypertension has been included in the latter one. Despite the Venice modification to the PH clinical classification, the Dana Point (2008) meeting also introduced even more modifications (<a class="elsevierStyleCrossRef" href="#t0005">Table 1</a>).</p><elsevierMultimedia ident="t0005"></elsevierMultimedia><p id="p0020" class="elsevierStylePara elsevierViewall">Based on the most updated classification of PH, the case report recently published by Cadden <span class="elsevierStyleItalic">etal</span>.<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a> in <span class="elsevierStyleItalic">Annals of Hepatology</span> represents case of PAH related to portal hypertension with underlying chronic hepatitis C infection; however is the author’s personal opinion that the authors of the case report misuse the PH nomenclature by abbreviating portopulmonary hypertension as “PPH”. I think that using this abbreviations can cause some confusions and could be misleading, since the Dana Point 2008 classification was intended to identify subgroup of patients that can benefit from specific novel therapies, also in terms of including or enroll them in current placebo, double-blind multicenter, randomized controlled clinical trials while investigating the response of certain therapies in a more homogeneous group of patients, and also very important, in order to avoid miscommunication and confusion between our patients. Nowadays, most of the articles written in the medical literature have been adopted specific abbreviations for portopulmonary hypertension (e.g., POPH), that could avoid confusion among clinicians and researchers,<a class="elsevierStyleCrossRefs" href="#bib0010"><span class="elsevierStyleSup">2</span></a>,<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> helping to be familiar with the increasing terminology and nomenclature among this relatively unknown but important entity that has been gaining interest among pulmonary vascular disease clinicians and researchers.</p><p id="p0025" class="elsevierStylePara elsevierViewall">Disclosure of financial support: None</p><p id="p0030" class="elsevierStylePara elsevierViewall">Statement of proprietary interest: None</p><p id="p0035" class="elsevierStylePara elsevierViewall">Institutional review board approval: N/A</p></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:2 [ 0 => array:2 [ "identificador" => "sec0005" "titulo" => "Dear Editor:" ] 1 => array:1 [ "titulo" => "References" ] ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "fechaRecibido" => "2009-05-28" "fechaAceptado" => "2009-06-28" "multimedia" => array:1 [ 0 => array:7 [ "identificador" => "t0005" "etiqueta" => "<span class="elsevierStyleBold">Table 1.</span>" "tipo" => "MULTIMEDIATABLA" "mostrarFloat" => true "mostrarDisplay" => false "tabla" => array:2 [ "leyenda" => "<p id="npara0005" class="elsevierStyleSimplePara elsevierViewall">Main modifications to the Venice classification have been marked in bold. <span class="elsevierStyleBold">ALK-1</span>: activin receptor-like kinase-1. <span class="elsevierStyleBold">BMPR2</span>: bone morphogenetic protein receptor type 2. <span class="elsevierStyleBold">HIV</span>: Human Immunodeficiency Virus. <span class="elsevierStyleBold">Reproduced from</span>: 2. Simonneau G, et al. J Am Coll Cardiol 2009;54 (Suppl):S43-54, with permission from the publisher.</p>" "tablatextoimagen" => array:1 [ 0 => array:2 [ "tabla" => array:1 [ 0 => """ <table border="0" frame="\n \t\t\t\t\tvoid\n \t\t\t\t" class=""><tbody title="tbody"><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="top"><span class="elsevierStyleBold">1. Pulmonary arterial hypertension (PAH):</span> \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="top">1.1. Idiopathic pulmonary arterial hypertension (IPAH) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="top">1.2. Heritable \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="top">1.2.1. BMPR2 mutation \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="top">1.2.2. ALK-1, endoglin (with or without hereditary hemorrhagic telangiectasia) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="top">1.2.3. Unknown \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="top">1.3. Drug and toxin-induced \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="top">1.4 Associated with: \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="top">1.4.1. Collagen vascular disease \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="top">1.4.2. Congenital systemic to pulmonary shunts \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="top">1.4.3. Portal hypertension \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="top">1.4.4. HIV infection \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="top">1.4.5. Schistosomiasis \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="top">1.4.6. Chronic hemolytic anemia \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="top">1.5. Persistent pulmonary hypertension of the newborn \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="top"><span class="elsevierStyleBold">1. Pulmonary veno-occlusive disease (PVOD) and/or pulmonary capillary hemangiomatosis (PCH)</span> \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="top">2. Pulmonary hypertension due to left heart disease: \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="top">2.1. Systolic dysfunction \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="top">2.2. Diastolic dysfunction \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="top">2.3. Valvular disease \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="top">3. Pulmonary hypertension related to diffuse parenchymal lung diseases and/or hypoxemia: \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="top">3.1. Chronic obstructive lung disease (COPD) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="top">3.2. Interstitial lung disease \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="top">3.3. Other pulmonary diseases with mixed obstructive and restrictive pattern \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="top">3.4. Sleep disordered breathing \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="top">3.5. Alveolar hypoventilation disorders \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="top">3.6. Chronic exposure to high altitudes \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="top">3.7. Developmental abnormalities \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="top">4. Chronic thromboembolic pulmonary hypertension (CTPEH) \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="top">5. Pulmonary hypertension with unclear multifactorial mechanisms: \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="top">5.1. Hematologic disorders: myeloproliferative disorders and splenectomy \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="top">5.2. Systemic disorders: Sarcoidosis, pulmonary Langerhans cell histiocytosis: lymphangioleiomyomatosis, vasculitis, euro-fibromatosis. \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="top">5.3. Metabolic disorders: glycogen storage disease, Gaucher disease, thyroid disorders. \t\t\t\t\t\t\n \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n \t\t\t\t\ttable-entry\n \t\t\t\t " align="left" valign="top">5.4. Others: tumoral obstruction, fibrosing mediastinitis, chronic renal failure on hemodialysis. \t\t\t\t\t\t\n \t\t\t\t</td></tr></tbody></table> """ ] "imagenFichero" => array:1 [ 0 => "xTab2067314.png" ] ] ] ] "descripcion" => array:1 [ "en" => "<p id="sp0005" class="elsevierStyleSimplePara elsevierViewall">Updated clinical classification of pulmonary hypertension (dana point 2008).</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bs0005" "bibliografiaReferencia" => array:5 [ 0 => array:3 [ "identificador" => "bib0005" "etiqueta" => "1." "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Pulmonary arterial hypertension." "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:2 [ 0 => "Chin KM," 1 => "Rubin LJ." ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1016/j.jacc.2008.01.024" "Revista" => array:6 [ "tituloSerie" => "J Am Coll Cardiol" "fecha" => "2008" "volumen" => "51" "paginaInicial" => "1527-" "paginaFinal" => "38" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/18420094" "web" => "Medline" ] ] ] ] ] ] ] ] 1 => array:3 [ "identificador" => "bib0010" "etiqueta" => "2." "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Updated clinical classification of pulmonary hypertension." "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "Simonneau G," 1 => "Robbins IM," 2 => "Beghetti M," 3 => "Channick RN," 4 => "Del-croix M," 5 => "Elliott CG," ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1016/j.jacc.2009.04.012" "Revista" => array:6 [ "tituloSerie" => "J Am Coll Cardiol" "fecha" => "2009" "volumen" => "54" "paginaInicial" => "S43-" "paginaFinal" => "S54" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/19555858" "web" => "Medline" ] ] ] ] ] ] ] ] 2 => array:3 [ "identificador" => "bib0015" "etiqueta" => "3." "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Executive summary from the World Symposium of Primary Pulmonary Hypertension (Evian, France September 6-10, 1998)." "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:3 [ 0 => "Rich S," 1 => "Rubin LJ," 2 => "Abenhail L." ] ] ] ] ] "host" => array:1 [ 0 => array:1 [ "Revista" => array:2 [ "tituloSerie" => "The World Health Organization publication via internet." "fecha" => "1988" ] ] ] ] ] ] 3 => array:3 [ "identificador" => "bib0020" "etiqueta" => "4." "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "The use of sildenafil to treat portopulmonary hypertension prior to liver transplantation." "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:5 [ 0 => "Cadden ISH," 1 => "Greanya ED," 2 => "Erb SR," 3 => "Scudamore CH," 4 => "Yoshida EM." ] ] ] ] ] "host" => array:1 [ 0 => array:1 [ "Revista" => array:6 [ "tituloSerie" => "Ann Hepatol" "fecha" => "2009" "volumen" => "8" "paginaInicial" => "158-" "paginaFinal" => "61" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/19502663" "web" => "Medline" ] ] ] ] ] ] ] ] 4 => array:3 [ "identificador" => "bib0025" "etiqueta" => "5." "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Zuckerman MJ, Figueroa-Casas JB, Krowka MJ. Portopulmonary hypertension: state of the art." 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| Year/Month | Html | Total | |
|---|---|---|---|
| 2024 October | 4 | 2 | 6 |
| 2024 September | 12 | 14 | 26 |
| 2024 August | 15 | 9 | 24 |
| 2024 July | 7 | 3 | 10 |
| 2024 June | 11 | 5 | 16 |
| 2024 May | 11 | 3 | 14 |
| 2024 April | 11 | 6 | 17 |
| 2024 March | 21 | 4 | 25 |
| 2024 February | 16 | 6 | 22 |
| 2024 January | 8 | 7 | 15 |
| 2023 December | 6 | 4 | 10 |
| 2023 November | 18 | 5 | 23 |
| 2023 October | 4 | 3 | 7 |
| 2023 September | 4 | 6 | 10 |
| 2023 August | 4 | 5 | 9 |
| 2023 July | 2 | 4 | 6 |
| 2023 June | 4 | 7 | 11 |
| 2023 May | 6 | 2 | 8 |
| 2023 April | 23 | 5 | 28 |
| 2023 March | 8 | 1 | 9 |
| 2023 February | 8 | 5 | 13 |
| 2023 January | 11 | 4 | 15 |
| 2022 December | 7 | 3 | 10 |
| 2022 November | 13 | 8 | 21 |
| 2022 October | 9 | 9 | 18 |
| 2022 September | 11 | 12 | 23 |
| 2022 August | 14 | 7 | 21 |
| 2022 July | 12 | 9 | 21 |
| 2022 June | 11 | 4 | 15 |
| 2022 May | 7 | 3 | 10 |
| 2022 April | 13 | 12 | 25 |
| 2022 March | 11 | 9 | 20 |
| 2022 February | 4 | 2 | 6 |
| 2022 January | 22 | 4 | 26 |
| 2021 December | 7 | 4 | 11 |
| 2021 November | 8 | 10 | 18 |
| 2021 October | 21 | 12 | 33 |
| 2021 September | 23 | 10 | 33 |
| 2021 August | 18 | 4 | 22 |
| 2021 July | 12 | 7 | 19 |
| 2021 June | 15 | 6 | 21 |
| 2021 May | 17 | 7 | 24 |
| 2021 April | 52 | 4 | 56 |
| 2021 March | 24 | 7 | 31 |
| 2021 February | 10 | 4 | 14 |
| 2021 January | 8 | 7 | 15 |
| 2020 December | 11 | 5 | 16 |
| 2020 November | 4 | 6 | 10 |
| 2020 October | 8 | 5 | 13 |
| 2020 September | 12 | 9 | 21 |
| 2020 August | 4 | 1 | 5 |
| 2020 July | 1 | 3 | 4 |
| 2020 June | 1 | 0 | 1 |
| 2020 May | 4 | 5 | 9 |
| 2020 April | 1 | 3 | 4 |
| 2020 March | 3 | 2 | 5 |
| 2020 February | 1 | 3 | 4 |
| 2020 January | 3 | 4 | 7 |
| 2019 December | 6 | 3 | 9 |
| 2019 November | 2 | 1 | 3 |
| 2019 October | 2 | 1 | 3 |
| 2019 September | 1 | 3 | 4 |
| 2019 August | 1 | 1 | 2 |
| 2019 July | 4 | 6 | 10 |
| 2019 June | 1 | 5 | 6 |
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