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Pulmonary hypertension, primary, 2(PPH2)

MedGen UID:
854709
Concept ID:
C3888002
Disease or Syndrome
Synonyms: PPH2; PULMONARY HYPERTENSION, PRIMARY, 2
 
Gene (location): SMAD9 (13q13.3)
 
Monarch Initiative: MONDO:0014134
OMIM®: 615342

Definition

Pulmonary arterial hypertension is a progressive disorder characterized by abnormally high blood pressure (hypertension) in the pulmonary artery, the blood vessel that carries blood from the heart to the lungs. Pulmonary arterial hypertension is one form of a broader condition known as pulmonary hypertension. Pulmonary hypertension occurs when most of the very small arteries throughout the lungs narrow in diameter, which increases the resistance to blood flow through the lungs. To overcome the increased resistance, blood pressure increases in the pulmonary artery and in the right ventricle of the heart, which is the chamber that pumps blood into the pulmonary artery. Ultimately, the increased blood pressure can damage the right ventricle of the heart.

Signs and symptoms of pulmonary arterial hypertension occur when increased blood pressure cannot fully overcome the elevated resistance. As a result, the flow of oxygenated blood from the lungs to the rest of the body is insufficient. Shortness of breath (dyspnea) during exertion and fainting spells are the most common symptoms of pulmonary arterial hypertension. People with this disorder may experience additional symptoms, particularly as the condition worsens. Other symptoms include dizziness, swelling (edema) of the ankles or legs, chest pain, and a rapid heart rate. [from MedlinePlus Genetics]

Clinical features

From HPO
Increased pulmonary vascular resistance
MedGen UID:
356775
Concept ID:
C1867423
Finding
Pulmonary vascular resistance (PVR) more than 3 wood units, as defined by the current definition of pulmonary hypertension. 95% of individuals have a PVR of less than 2.4 wood units.
Pulmonary arterial hypertension
MedGen UID:
425404
Concept ID:
C2973725
Disease or Syndrome
Pulmonary hypertension is defined mean pulmonary artery pressure of 25mmHg or more and pulmonary capillary wedge pressure of 15mmHg or less when measured by right heart catheterisation at rest and in a supine position.
Abnormally loud pulmonic component of the second heart sound
MedGen UID:
1638659
Concept ID:
C4703480
Finding

Professional guidelines

PubMed

Salerno CT, Park SJ, Kreykes NS, Kulick DM, Savik K, Hertz MI, Bolman RM 3rd
J Thorac Cardiovasc Surg 1999 Jun;117(6):1063-9. doi: 10.1016/s0022-5223(99)70241-2. PMID: 10343253

Recent clinical studies

Etiology

Maron BA
J Am Heart Assoc 2023 Apr 18;12(8):e029024. Epub 2023 Apr 7 doi: 10.1161/JAHA.122.029024. PMID: 37026538Free PMC Article
Humbert M, McLaughlin V, Gibbs JSR, Gomberg-Maitland M, Hoeper MM, Preston IR, Souza R, Waxman AB, Ghofrani HA, Escribano Subias P, Feldman J, Meyer G, Montani D, Olsson KM, Manimaran S, de Oliveira Pena J, Badesch DB
Eur Respir J 2023 Jan;61(1) Epub 2023 Jan 6 doi: 10.1183/13993003.01347-2022. PMID: 36041750Free PMC Article
Hoeper MM, Pausch C, Olsson KM, Huscher D, Pittrow D, Grünig E, Staehler G, Vizza CD, Gall H, Distler O, Opitz C, Gibbs JSR, Delcroix M, Ghofrani HA, Park DH, Ewert R, Kaemmerer H, Kabitz HJ, Skowasch D, Behr J, Milger K, Halank M, Wilkens H, Seyfarth HJ, Held M, Dumitrescu D, Tsangaris I, Vonk-Noordegraaf A, Ulrich S, Klose H, Claussen M, Lange TJ, Rosenkranz S
Eur Respir J 2022 Jul;60(1) Epub 2022 Jul 7 doi: 10.1183/13993003.02311-2021. PMID: 34737226Free PMC Article
Hoeper MM, Pausch C, Grünig E, Klose H, Staehler G, Huscher D, Pittrow D, Olsson KM, Vizza CD, Gall H, Benjamin N, Distler O, Opitz C, Gibbs JSR, Delcroix M, Ghofrani HA, Rosenkranz S, Ewert R, Kaemmerer H, Lange TJ, Kabitz HJ, Skowasch D, Skride A, Jureviciene E, Paleviciute E, Miliauskas S, Claussen M, Behr J, Milger K, Halank M, Wilkens H, Wirtz H, Pfeuffer-Jovic E, Harbaum L, Scholtz W, Dumitrescu D, Bruch L, Coghlan G, Neurohr C, Tsangaris I, Gorenflo M, Scelsi L, Vonk-Noordegraaf A, Ulrich S, Held M
J Heart Lung Transplant 2020 Dec;39(12):1435-1444. Epub 2020 Sep 30 doi: 10.1016/j.healun.2020.09.011. PMID: 33082079
Montani D, Chaumais MC, Guignabert C, Günther S, Girerd B, Jaïs X, Algalarrondo V, Price LC, Savale L, Sitbon O, Simonneau G, Humbert M
Pharmacol Ther 2014 Feb;141(2):172-91. Epub 2013 Oct 14 doi: 10.1016/j.pharmthera.2013.10.002. PMID: 24134901

Diagnosis

Maron BA
J Am Heart Assoc 2023 Apr 18;12(8):e029024. Epub 2023 Apr 7 doi: 10.1161/JAHA.122.029024. PMID: 37026538Free PMC Article
Hoeper MM, Pausch C, Olsson KM, Huscher D, Pittrow D, Grünig E, Staehler G, Vizza CD, Gall H, Distler O, Opitz C, Gibbs JSR, Delcroix M, Ghofrani HA, Park DH, Ewert R, Kaemmerer H, Kabitz HJ, Skowasch D, Behr J, Milger K, Halank M, Wilkens H, Seyfarth HJ, Held M, Dumitrescu D, Tsangaris I, Vonk-Noordegraaf A, Ulrich S, Klose H, Claussen M, Lange TJ, Rosenkranz S
Eur Respir J 2022 Jul;60(1) Epub 2022 Jul 7 doi: 10.1183/13993003.02311-2021. PMID: 34737226Free PMC Article
Hoeper MM, Pausch C, Grünig E, Klose H, Staehler G, Huscher D, Pittrow D, Olsson KM, Vizza CD, Gall H, Benjamin N, Distler O, Opitz C, Gibbs JSR, Delcroix M, Ghofrani HA, Rosenkranz S, Ewert R, Kaemmerer H, Lange TJ, Kabitz HJ, Skowasch D, Skride A, Jureviciene E, Paleviciute E, Miliauskas S, Claussen M, Behr J, Milger K, Halank M, Wilkens H, Wirtz H, Pfeuffer-Jovic E, Harbaum L, Scholtz W, Dumitrescu D, Bruch L, Coghlan G, Neurohr C, Tsangaris I, Gorenflo M, Scelsi L, Vonk-Noordegraaf A, Ulrich S, Held M
J Heart Lung Transplant 2020 Dec;39(12):1435-1444. Epub 2020 Sep 30 doi: 10.1016/j.healun.2020.09.011. PMID: 33082079
Vonk-Noordegraaf A, Haddad F, Chin KM, Forfia PR, Kawut SM, Lumens J, Naeije R, Newman J, Oudiz RJ, Provencher S, Torbicki A, Voelkel NF, Hassoun PM
J Am Coll Cardiol 2013 Dec 24;62(25 Suppl):D22-33. doi: 10.1016/j.jacc.2013.10.027. PMID: 24355638
van den Hoogen F, Khanna D, Fransen J, Johnson SR, Baron M, Tyndall A, Matucci-Cerinic M, Naden RP, Medsger TA Jr, Carreira PE, Riemekasten G, Clements PJ, Denton CP, Distler O, Allanore Y, Furst DE, Gabrielli A, Mayes MD, van Laar JM, Seibold JR, Czirjak L, Steen VD, Inanc M, Kowal-Bielecka O, Müller-Ladner U, Valentini G, Veale DJ, Vonk MC, Walker UA, Chung L, Collier DH, Ellen Csuka M, Fessler BJ, Guiducci S, Herrick A, Hsu VM, Jimenez S, Kahaleh B, Merkel PA, Sierakowski S, Silver RM, Simms RW, Varga J, Pope JE
Ann Rheum Dis 2013 Nov;72(11):1747-55. doi: 10.1136/annrheumdis-2013-204424. PMID: 24092682

Therapy

Maron BA
J Am Heart Assoc 2023 Apr 18;12(8):e029024. Epub 2023 Apr 7 doi: 10.1161/JAHA.122.029024. PMID: 37026538Free PMC Article
Humbert M, McLaughlin V, Gibbs JSR, Gomberg-Maitland M, Hoeper MM, Preston IR, Souza R, Waxman AB, Ghofrani HA, Escribano Subias P, Feldman J, Meyer G, Montani D, Olsson KM, Manimaran S, de Oliveira Pena J, Badesch DB
Eur Respir J 2023 Jan;61(1) Epub 2023 Jan 6 doi: 10.1183/13993003.01347-2022. PMID: 36041750Free PMC Article
Jaïs X, Brenot P, Bouvaist H, Jevnikar M, Canuet M, Chabanne C, Chaouat A, Cottin V, De Groote P, Favrolt N, Horeau-Langlard D, Magro P, Savale L, Prévot G, Renard S, Sitbon O, Parent F, Trésorier R, Tromeur C, Piedvache C, Grimaldi L, Fadel E, Montani D, Humbert M, Simonneau G
Lancet Respir Med 2022 Oct;10(10):961-971. Epub 2022 Aug 1 doi: 10.1016/S2213-2600(22)00214-4. PMID: 35926542
Zuo Z, Yue J, Dong BR, Wu T, Liu GJ, Hao Q
Cochrane Database Syst Rev 2021 Apr 15;4(4):CD004437. doi: 10.1002/14651858.CD004437.pub6. PMID: 33857326Free PMC Article
Ghofrani HA, Galiè N, Grimminger F, Grünig E, Humbert M, Jing ZC, Keogh AM, Langleben D, Kilama MO, Fritsch A, Neuser D, Rubin LJ; PATENT-1 Study Group
N Engl J Med 2013 Jul 25;369(4):330-40. doi: 10.1056/NEJMoa1209655. PMID: 23883378

Prognosis

Maron BA
J Am Heart Assoc 2023 Apr 18;12(8):e029024. Epub 2023 Apr 7 doi: 10.1161/JAHA.122.029024. PMID: 37026538Free PMC Article
Condon DF, Agarwal S, Chakraborty A, Auer N, Vazquez R, Patel H, Zamanian RT, de Jesus Perez VA
Chest 2022 Apr;161(4):1060-1072. Epub 2021 Oct 13 doi: 10.1016/j.chest.2021.10.010. PMID: 34655569Free PMC Article
Montani D, Chaumais MC, Guignabert C, Günther S, Girerd B, Jaïs X, Algalarrondo V, Price LC, Savale L, Sitbon O, Simonneau G, Humbert M
Pharmacol Ther 2014 Feb;141(2):172-91. Epub 2013 Oct 14 doi: 10.1016/j.pharmthera.2013.10.002. PMID: 24134901
Vonk-Noordegraaf A, Haddad F, Chin KM, Forfia PR, Kawut SM, Lumens J, Naeije R, Newman J, Oudiz RJ, Provencher S, Torbicki A, Voelkel NF, Hassoun PM
J Am Coll Cardiol 2013 Dec 24;62(25 Suppl):D22-33. doi: 10.1016/j.jacc.2013.10.027. PMID: 24355638
Sharifi M, Bay C, Skrocki L, Rahimi F, Mehdipour M; “MOPETT” Investigators
Am J Cardiol 2013 Jan 15;111(2):273-7. Epub 2012 Oct 24 doi: 10.1016/j.amjcard.2012.09.027. PMID: 23102885

Clinical prediction guides

Rosenkranz S, Pausch C, Coghlan JG, Huscher D, Pittrow D, Grünig E, Staehler G, Vizza CD, Gall H, Distler O, Delcroix M, Ghofrani HA, Ewert R, Kabitz HJ, Skowasch D, Behr J, Milger K, Halank M, Wilkens H, Seyfarth HJ, Held M, Scelsi L, Neurohr C, Vonk-Noordegraaf A, Ulrich S, Klose H, Claussen M, Eisenmann S, Schmidt KH, Remppis BA, Skride A, Jureviciene E, Gumbiene L, Miliauskas S, Löffler-Ragg J, Lange TJ, Olsson KM, Hoeper MM, Opitz C
J Heart Lung Transplant 2023 Jan;42(1):102-114. Epub 2022 Oct 13 doi: 10.1016/j.healun.2022.10.003. PMID: 36333206
Hoeper MM, Pausch C, Olsson KM, Huscher D, Pittrow D, Grünig E, Staehler G, Vizza CD, Gall H, Distler O, Opitz C, Gibbs JSR, Delcroix M, Ghofrani HA, Park DH, Ewert R, Kaemmerer H, Kabitz HJ, Skowasch D, Behr J, Milger K, Halank M, Wilkens H, Seyfarth HJ, Held M, Dumitrescu D, Tsangaris I, Vonk-Noordegraaf A, Ulrich S, Klose H, Claussen M, Lange TJ, Rosenkranz S
Eur Respir J 2022 Jul;60(1) Epub 2022 Jul 7 doi: 10.1183/13993003.02311-2021. PMID: 34737226Free PMC Article
Toshner M, Church C, Harbaum L, Rhodes C, Villar Moreschi SS, Liley J, Jones R, Arora A, Batai K, Desai AA, Coghlan JG, Gibbs JSR, Gor D, Gräf S, Harlow L, Hernandez-Sanchez J, Howard LS, Humbert M, Karnes J, Kiely DG, Kittles R, Knightbridge E, Lam B, Lutz KA, Nichols WC, Pauciulo MW, Pepke-Zaba J, Suntharalingam J, Soubrier F, Trembath RC, Schwantes-An TL, Wort SJ, Wilkins MR, Gaine S, Morrell NW, Corris PA; Uniphy Clinical Trials Network
Eur Respir J 2022 Mar;59(3) Epub 2022 Mar 10 doi: 10.1183/13993003.02463-2020. PMID: 34588193Free PMC Article
Hoeper MM, Pausch C, Grünig E, Klose H, Staehler G, Huscher D, Pittrow D, Olsson KM, Vizza CD, Gall H, Benjamin N, Distler O, Opitz C, Gibbs JSR, Delcroix M, Ghofrani HA, Rosenkranz S, Ewert R, Kaemmerer H, Lange TJ, Kabitz HJ, Skowasch D, Skride A, Jureviciene E, Paleviciute E, Miliauskas S, Claussen M, Behr J, Milger K, Halank M, Wilkens H, Wirtz H, Pfeuffer-Jovic E, Harbaum L, Scholtz W, Dumitrescu D, Bruch L, Coghlan G, Neurohr C, Tsangaris I, Gorenflo M, Scelsi L, Vonk-Noordegraaf A, Ulrich S, Held M
J Heart Lung Transplant 2020 Dec;39(12):1435-1444. Epub 2020 Sep 30 doi: 10.1016/j.healun.2020.09.011. PMID: 33082079
Zhang J, Gajjala S, Agrawal P, Tison GH, Hallock LA, Beussink-Nelson L, Lassen MH, Fan E, Aras MA, Jordan C, Fleischmann KE, Melisko M, Qasim A, Shah SJ, Bajcsy R, Deo RC
Circulation 2018 Oct 16;138(16):1623-1635. doi: 10.1161/CIRCULATIONAHA.118.034338. PMID: 30354459Free PMC Article

Recent systematic reviews

Sánchez-de-la-Torre M, Gracia-Lavedan E, Benitez ID, Sánchez-de-la-Torre A, Moncusí-Moix A, Torres G, Loffler K, Woodman R, Adams R, Labarca G, Dreyse J, Eulenburg C, Thunström E, Glantz H, Peker Y, Anderson C, McEvoy D, Barbé F
JAMA 2023 Oct 3;330(13):1255-1265. doi: 10.1001/jama.2023.17465. PMID: 37787793Free PMC Article
Zangger G, Bricca A, Liaghat B, Juhl CB, Mortensen SR, Andersen RM, Damsted C, Hamborg TG, Ried-Larsen M, Tang LH, Thygesen LC, Skou ST
J Med Internet Res 2023 Jul 6;25:e46439. doi: 10.2196/46439. PMID: 37410534Free PMC Article
Morris NR, Kermeen FD, Jones AW, Lee JY, Holland AE
Cochrane Database Syst Rev 2023 Mar 22;3(3):CD011285. doi: 10.1002/14651858.CD011285.pub3. PMID: 36947725Free PMC Article
Global Burden of Disease 2019 Cancer Collaboration, Kocarnik JM, Compton K, Dean FE, Fu W, Gaw BL, Harvey JD, Henrikson HJ, Lu D, Pennini A, Xu R, Ababneh E, Abbasi-Kangevari M, Abbastabar H, Abd-Elsalam SM, Abdoli A, Abedi A, Abidi H, Abolhassani H, Adedeji IA, Adnani QES, Advani SM, Afzal MS, Aghaali M, Ahinkorah BO, Ahmad S, Ahmad T, Ahmadi A, Ahmadi S, Ahmed Rashid T, Ahmed Salih Y, Akalu GT, Aklilu A, Akram T, Akunna CJ, Al Hamad H, Alahdab F, Al-Aly Z, Ali S, Alimohamadi Y, Alipour V, Aljunid SM, Alkhayyat M, Almasi-Hashiani A, Almasri NA, Al-Maweri SAA, Almustanyir S, Alonso N, Alvis-Guzman N, Amu H, Anbesu EW, Ancuceanu R, Ansari F, Ansari-Moghaddam A, Antwi MH, Anvari D, Anyasodor AE, Aqeel M, Arabloo J, Arab-Zozani M, Aremu O, Ariffin H, Aripov T, Arshad M, Artaman A, Arulappan J, Asemi Z, Asghari Jafarabadi M, Ashraf T, Atorkey P, Aujayeb A, Ausloos M, Awedew AF, Ayala Quintanilla BP, Ayenew T, Azab MA, Azadnajafabad S, Azari Jafari A, Azarian G, Azzam AY, Badiye AD, Bahadory S, Baig AA, Baker JL, Balakrishnan S, Banach M, Bärnighausen TW, Barone-Adesi F, Barra F, Barrow A, Behzadifar M, Belgaumi UI, Bezabhe WMM, Bezabih YM, Bhagat DS, Bhagavathula AS, Bhardwaj N, Bhardwaj P, Bhaskar S, Bhattacharyya K, Bhojaraja VS, Bibi S, Bijani A, Biondi A, Bisignano C, Bjørge T, Bleyer A, Blyuss O, Bolarinwa OA, Bolla SR, Braithwaite D, Brar A, Brenner H, Bustamante-Teixeira MT, Butt NS, Butt ZA, Caetano Dos Santos FL, Cao Y, Carreras G, Catalá-López F, Cembranel F, Cerin E, Cernigliaro A, Chakinala RC, Chattu SK, Chattu VK, Chaturvedi P, Chimed-Ochir O, Cho DY, Christopher DJ, Chu DT, Chung MT, Conde J, Cortés S, Cortesi PA, Costa VM, Cunha AR, Dadras O, Dagnew AB, Dahlawi SMA, Dai X, Dandona L, Dandona R, Darwesh AM, das Neves J, De la Hoz FP, Demis AB, Denova-Gutiérrez E, Dhamnetiya D, Dhimal ML, Dhimal M, Dianatinasab M, Diaz D, Djalalinia S, Do HP, Doaei S, Dorostkar F, Dos Santos Figueiredo FW, Driscoll TR, Ebrahimi H, Eftekharzadeh S, El Tantawi M, El-Abid H, Elbarazi I, Elhabashy HR, Elhadi M, El-Jaafary SI, Eshrati B, Eskandarieh S, Esmaeilzadeh F, Etemadi A, Ezzikouri S, Faisaluddin M, Faraon EJA, Fares J, Farzadfar F, Feroze AH, Ferrero S, Ferro Desideri L, Filip I, Fischer F, Fisher JL, Foroutan M, Fukumoto T, Gaal PA, Gad MM, Gadanya MA, Gallus S, Gaspar Fonseca M, Getachew Obsa A, Ghafourifard M, Ghashghaee A, Ghith N, Gholamalizadeh M, Gilani SA, Ginindza TG, Gizaw ATT, Glasbey JC, Golechha M, Goleij P, Gomez RS, Gopalani SV, Gorini G, Goudarzi H, Grosso G, Gubari MIM, Guerra MR, Guha A, Gunasekera DS, Gupta B, Gupta VB, Gupta VK, Gutiérrez RA, Hafezi-Nejad N, Haider MR, Haj-Mirzaian A, Halwani R, Hamadeh RR, Hameed S, Hamidi S, Hanif A, Haque S, Harlianto NI, Haro JM, Hasaballah AI, Hassanipour S, Hay RJ, Hay SI, Hayat K, Heidari G, Heidari M, Herrera-Serna BY, Herteliu C, Hezam K, Holla R, Hossain MM, Hossain MBH, Hosseini MS, Hosseini M, Hosseinzadeh M, Hostiuc M, Hostiuc S, Househ M, Hsairi M, Huang J, Hugo FN, Hussain R, Hussein NR, Hwang BF, Iavicoli I, Ibitoye SE, Ida F, Ikuta KS, Ilesanmi OS, Ilic IM, Ilic MD, Irham LM, Islam JY, Islam RM, Islam SMS, Ismail NE, Isola G, Iwagami M, Jacob L, Jain V, Jakovljevic MB, Javaheri T, Jayaram S, Jazayeri SB, Jha RP, Jonas JB, Joo T, Joseph N, Joukar F, Jürisson M, Kabir A, Kahrizi D, Kalankesh LR, Kalhor R, Kaliyadan F, Kalkonde Y, Kamath A, Kameran Al-Salihi N, Kandel H, Kapoor N, Karch A, Kasa AS, Katikireddi SV, Kauppila JH, Kavetskyy T, Kebede SA, Keshavarz P, Keykhaei M, Khader YS, Khalilov R, Khan G, Khan M, Khan MN, Khan MAB, Khang YH, Khater AM, Khayamzadeh M, Kim GR, Kim YJ, Kisa A, Kisa S, Kissimova-Skarbek K, Kopec JA, Koteeswaran R, Koul PA, Koulmane Laxminarayana SL, Koyanagi A, Kucuk Bicer B, Kugbey N, Kumar GA, Kumar N, Kumar N, Kurmi OP, Kutluk T, La Vecchia C, Lami FH, Landires I, Lauriola P, Lee SW, Lee SWH, Lee WC, Lee YH, Leigh J, Leong E, Li J, Li MC, Liu X, Loureiro JA, Lunevicius R, Magdy Abd El Razek M, Majeed A, Makki A, Male S, Malik AA, Mansournia MA, Martini S, Masoumi SZ, Mathur P, McKee M, Mehrotra R, Mendoza W, Menezes RG, Mengesha EW, Mesregah MK, Mestrovic T, Miao Jonasson J, Miazgowski B, Miazgowski T, Michalek IM, Miller TR, Mirzaei H, Mirzaei HR, Misra S, Mithra P, Moghadaszadeh M, Mohammad KA, Mohammad Y, Mohammadi M, Mohammadi SM, Mohammadian-Hafshejani A, Mohammed S, Moka N, Mokdad AH, Molokhia M, Monasta L, Moni MA, Moosavi MA, Moradi Y, Moraga P, Morgado-da-Costa J, Morrison SD, Mosapour A, Mubarik S, Mwanri L, Nagarajan AJ, Nagaraju SP, Nagata C, Naimzada MD, Nangia V, Naqvi AA, Narasimha Swamy S, Ndejjo R, Nduaguba SO, Negoi I, Negru SM, Neupane Kandel S, Nguyen CT, Nguyen HLT, Niazi RK, Nnaji CA, Noor NM, Nuñez-Samudio V, Nzoputam CI, Oancea B, Ochir C, Odukoya OO, Ogbo FA, Olagunju AT, Olakunde BO, Omar E, Omar Bali A, Omonisi AEE, Ong S, Onwujekwe OE, Orru H, Ortega-Altamirano DV, Otstavnov N, Otstavnov SS, Owolabi MO, P A M, Padubidri JR, Pakshir K, Pana A, Panagiotakos D, Panda-Jonas S, Pardhan S, Park EC, Park EK, Pashazadeh Kan F, Patel HK, Patel JR, Pati S, Pattanshetty SM, Paudel U, Pereira DM, Pereira RB, Perianayagam A, Pillay JD, Pirouzpanah S, Pishgar F, Podder I, Postma MJ, Pourjafar H, Prashant A, Preotescu L, Rabiee M, Rabiee N, Radfar A, Radhakrishnan RA, Radhakrishnan V, Rafiee A, Rahim F, Rahimzadeh S, Rahman M, Rahman MA, Rahmani AM, Rajai N, Rajesh A, Rakovac I, Ram P, Ramezanzadeh K, Ranabhat K, Ranasinghe P, Rao CR, Rao SJ, Rawassizadeh R, Razeghinia MS, Renzaho AMN, Rezaei N, Rezaei N, Rezapour A, Roberts TJ, Rodriguez JAB, Rohloff P, Romoli M, Ronfani L, Roshandel G, Rwegerera GM, S M, Sabour S, Saddik B, Saeed U, Sahebkar A, Sahoo H, Salehi S, Salem MR, Salimzadeh H, Samaei M, Samy AM, Sanabria J, Sankararaman S, Santric-Milicevic MM, Sardiwalla Y, Sarveazad A, Sathian B, Sawhney M, Saylan M, Schneider IJC, Sekerija M, Seylani A, Shafaat O, Shaghaghi Z, Shaikh MA, Shamsoddin E, Shannawaz M, Sharma R, Sheikh A, Sheikhbahaei S, Shetty A, Shetty JK, Shetty PH, Shibuya K, Shirkoohi R, Shivakumar KM, Shivarov V, Siabani S, Siddappa Malleshappa SK, Silva DAS, Singh JA, Sintayehu Y, Skryabin VY, Skryabina AA, Soeberg MJ, Sofi-Mahmudi A, Sotoudeh H, Steiropoulos P, Straif K, Subedi R, Sufiyan MB, Sultan I, Sultana S, Sur D, Szerencsés V, Szócska M, Tabarés-Seisdedos R, Tabuchi T, Tadbiri H, Taherkhani A, Takahashi K, Talaat IM, Tan KK, Tat VY, Tedla BAA, Tefera YG, Tehrani-Banihashemi A, Temsah MH, Tesfay FH, Tessema GA, Thapar R, Thavamani A, Thoguluva Chandrasekar V, Thomas N, Tohidinik HR, Touvier M, Tovani-Palone MR, Traini E, Tran BX, Tran KB, Tran MTN, Tripathy JP, Tusa BS, Ullah I, Ullah S, Umapathi KK, Unnikrishnan B, Upadhyay E, Vacante M, Vaezi M, Valadan Tahbaz S, Velazquez DZ, Veroux M, Violante FS, Vlassov V, Vo B, Volovici V, Vu GT, Waheed Y, Wamai RG, Ward P, Wen YF, Westerman R, Winkler AS, Yadav L, Yahyazadeh Jabbari SH, Yang L, Yaya S, Yazie TSY, Yeshaw Y, Yonemoto N, Younis MZ, Yousefi Z, Yu C, Yuce D, Yunusa I, Zadnik V, Zare F, Zastrozhin MS, Zastrozhina A, Zhang J, Zhong C, Zhou L, Zhu C, Ziapour A, Zimmermann IR, Fitzmaurice C, Murray CJL, Force LM
JAMA Oncol 2022 Mar 1;8(3):420-444. doi: 10.1001/jamaoncol.2021.6987. PMID: 34967848Free PMC Article
Zuo Z, Yue J, Dong BR, Wu T, Liu GJ, Hao Q
Cochrane Database Syst Rev 2021 Apr 15;4(4):CD004437. doi: 10.1002/14651858.CD004437.pub6. PMID: 33857326Free PMC Article

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