4. DISCUSSIONE
4.5. Conclusioni
L’ipertensione polmonare è una patologia complessa e multidisciplinare in continuo sviluppo conoscitivo. Nonostante i notevoli progressi ottenuti nel corso degli anni, ulteriori studi di carattere clinico-emodinamico ed epidemiologico sono necessari al fine di incrementarne e perfezionarne le attuali conoscenze. A tal proposto si può affermare come, attraverso questo studio, siano state ottenute rilevanti informazioni di carattere clinico, emodinamico ed epidemiologico, capaci di ampliare, arricchire e integrare il patrimonio informativo relativo alla patologia. In particolare, è necessario sottolineare come il RHC sia una procedura diagnostica di crescente utilizzo presso la Clinica di Malattie dell’Apparato Cardiovascolare del Policlinico San Martino, quale centro di riferimento ligure per PH, soprattutto per pazienti con PH-LHD e PAH. Si può, inoltre, affermare come, nonostante la presenza di una malattia vascolare polmonare meno marcata rispetto a quella caratterizzante i soggetti con PAH, i pazienti affetti da PH-LHD sviluppino un’importante compromissione del cuore destro, compromissione che può verosimilmente contribuire alla mortalità degli stessi.
Prospettive future potrebbero riguardare studi clinico-epidemiologici volti a confrontare variabili emodinamiche aggiuntive relative ai cinque gruppi clinici di PH e ad attuare una differenziazione tra RHC diagnostici e stadiativi, ampliando tali valutazioni ad altri centri di terzo livello.
92
BIBLIOGRAFIA
[1] Kovacs G. and Olschewski H., The definition of pulmonary hypertension: history, practical implications and current controversies, Breathe Sheff, 2021.
[2] Simonneau G., Montani D., Celermajer David S. et al., Haemodynamic definitions and updated clinical classification of pulmonary hypertension, European Respiratory Journal, 2019.
[3] Butrous G., Pulmonary hypertension: From an orphan disease to a global epidemic, Global Cardiology Science and Practice, 2020.
[4] Waxman Aaron B. and Loscalzo J., “Ipertensione polmonare”, in Harrison-Principi di Medicina Interna, a cura di Jameson J. Larry, Kasper Dennis L., Longo Dan L. et al., XX edizione italiana, vol. Secondo, Casa Editrice Ambrosiana, 2021, p. 2143-2149.
[5] McLaughlin Vallerie V. and Humbert M., “Ipertensione polmonare”, in Malattie del cuore di Braunwald: Trattato di medicina cardiovascolare, a cura di Mann Douglas L., Zipes Douglas P., Libby P., Bonow Robert O., X edizione italiana, vol. Unico, Edra S.p.A., 2016, p. 1773-1794.
[6] Kovacs G., Berghold A. and Scheidl S., Pulmonary arterial pressure during rest and exercise in healthy subjects: a systematic review, European Respiratory Journal, 2009.
[7] Humbert M., Montani D., Evgenov Oleg V. and Simonneau G., Definition and Classification of Pulmonary Hypertension, Handbook of Experimental Pharmacology, 2013.
[8] Galiè N., Marc Humbert M., Vachiery Jean-Luc et al., 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. The Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS). Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT), European Heart Journal, 2016, ePub 2015.
[9] Dresdale David T., Michtom Robert J. and Shultz M., Recent studies in primary pulmonary hypertension, including pharmacodynamic observations on pulmonary vascular resistance, Bulletin of The New York Academy of Medicine, 1954.
[10] Savale L., Chaumais Marie-Camille, Cottin V. et al., Pulmonary hypertension associated with benfluorex exposure, European Respiratory Journal, 2012.
[11] Walker Alexander M.,Langleben D., Korelitz James J. et al., Temporal trends and drug exposures in pulmonary hypertension: An American experience, American Heart Journal, 2006.
[12] Riou M., Seferian A., Savale L. et al., Deterioration of pulmonary hypertension and pleural effusion with bosutinib following dasatinib lung toxicity, European Respiratory Journal, 2016.
[13] Renard S., Borentain P., Salaun E. et al., Severe Pulmonary Arterial Hypertension in Patients Treated for Hepatitis C With Sofosbuvir, CHEST-Journal, 2016.
93
[14] Savale L., Chaumais Marie C., Montani D. et al., Direct-Acting Antiviral Medications for Hepatitis C Virus Infection and Pulmonary Arterial Hypertension, CHEST-Journal, 2016.
[15] Coirier V., Lescoat A., Chabanne C. et al., Pulmonary arterial hypertension in four patients treated by leflunomide, Joint Bone Spine, 2018.
[16] Mayu Nishio, Keiji Hirooka, and Yasuji Doi, Chinese herbal drug natural indigo may cause pulmonary artery hypertension, European Heart Journal, 2018.
[17] Hachulla E., Gressin V., Guillevin L. et al., Early detection of pulmonary arterial hypertension in systemic sclerosis: a French nationwide prospective multicenter study, Arthritis
& Rheumatology, 2005.
[18] Mukerjee D., St George D., Coleiro B. et al, Prevalence and outcome in systemic sclerosis associated pulmonary arterial hypertension: application of a registry approach,Annals of the Rheumatic Diseases, 2003.
[19] Kawut Steven M., Krowka Michael J., Trotter James F. et al., Clinical Risk Factors for Portopulmonary Hypertension, Hepatology, 2008.
[20] Galiè N., Marc Humbert M., Vachiery Jean-Luc et al., 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension – web addenda, The Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS). Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT) European Heart Journal, 2016, ePub 2015.
[21] Engelfriet Peter M., Duffels Marielle G J., Möller T. et al., Arterial hypertension in adults born with a heart septal defect: the Euro Heart Survey on adult congenital heart disease, Heart (British Cardiac Society), 2007.
[22] Simonneau G., Robbins Ivan M., Beghetti M. et al., Updated Clinical Classification of Pulmonary Hypertension, Journal of the American College of Cardiology, 2009.
[23] Lapa M., Dias B., Jardim C. et al., Cardiopulmonary manifestations of hepatosplenic schistosomiasis, Circulation, 2009.
[24] Fernandes CJ, Jardim CV, Hovnanian A. et al., Schistosomiasis and pulmonary hypertension, Expert Review of Respiratory Medicine, 2011.
[25] Chaves E., The Pathology of the Arterial Pulmonary Vasculature in Manson's Schistosomiasis, Diseases of the chest, 1966.
[26] Ross AG, Bartley PB, Sleigh AC et al., Schistosomiasis, The New England Journal of Medicine, 2002.
[27] Conceição MJ, Borges-Pereira J. and Coura JR, A thirty years follow-up study on Schistosomiasis mansoni in a community of Minas Gerais, Brazil, Memórias do Instituto Oswaldo Cruz, 2007.
[28] Rich S., Kaufmann E. and Levy P.S., The effect of high doses of calcium-channel blockers on survival in primary pulmonary hypertension, The New England Journal of Medicine, 1992.
94
[29] Sitbon O., Humbert M., Jaïs X. et al., Long-term response to calcium channel blockers in idiopathic pulmonary arterial hypertension, Circulation, 2005.
[30] Montani D., Savale L., Natali D. et al., Long-term response to calcium-channel blockers in non-idiopathic pulmonary arterial hypertension, European Heart Journal, 2010.
[31] Hemnes AR, Trammell AW, Archer SL et al., Peripheral blood signature of vasodilator-responsive pulmonary arterial hypertension,Circulation, 2005.
[32] Hemnes AR, Zhao M., West J. et al., Critical Genomic Networks and Vasoreactive Variants in Idiopathic Pulmonary Arterial Hypertension, American Journal of Respiratory and Critical Care Medicine, 2016.
[33] Montani D., Achouh L., Dorfmüller P. et al., Pulmonary veno-occlusive disease: clinical, functional, radiologic, and hemodynamic characteristics and outcome of 24 cases confirmed by histology, Medicine (Baltimore), 2008.
[34] Montani D., Price LC, Dorfmuller P. et al., Pulmonary veno-occlusive disease, European Respiratory Journal, 2009.
[35] Dorfmüller P., Humbert M., Perros F., et al., Fibrous remodeling of the pulmonary venous system in pulmonary arterial hypertension associated with connective tissue diseases, Human Pathology, 2007.
[36] Günther S., Jaïs X., Maitre S. et al., Computed tomography findings of pulmonary veno-occlusive disease in scleroderma patients presenting with precapillary pulmonary hypertension, Arthritis and rheumatis, 2012.
[37] Escamilla R., Hermant C., Berjaud J. et al., Pulmonary veno-occlusive disease in a HIV-infected intravenous drug abuser, European Respiratory Journal, 1995.
[38] Humbert M., Maître S., Capron F. et al., Pulmonary edema complicating continuous intravenous prostacyclin in pulmonary capillary hemangiomatosis, American Journal of Respiratory and Critical Care Medicine, 1998.
[39] Lantuéjoul S., Sheppard MN, Corrin B. et al., Pulmonary veno-occlusive disease and pulmonary capillary hemangiomatosis: a clinicopathologic study of 35 cases, The American Journal of Surgical Pathology, 2006.
[40] Eyries M., Montani D., Girerd B. et al., EIF2AK4 mutations cause pulmonary veno-occlusive disease, a recessive form of pulmonary hypertension, Nature Genetics, 2014.
[41] Mandel J., Mark EJ and Hales CA, Pulmonary veno-occlusive disease, American Journal of Respiratory and Critical Care Medicine, 2000.
[42] Montani D., Lau EM, Dorfmüller P. et al., Pulmonary veno-occlusive disease, European Respiratory Journal, 2016.
[43] Martinho S., Adão R., Leite-Moreira AF and Brás-Silva C., Persistent Pulmonary Hypertension of the Newborn: Pathophysiological Mechanisms and Novel Therapeutic Approaches, Frontiers in Pediatrics, 2020.
95
[44] Rosenzweig EB, Abman SH, Adatia I. et al., Paediatric pulmonary arterial hypertension:
updates on definition, classification, diagnostics and management, European Respiratory Journal, 2019.
[45] Berger RM, Beghetti M., Humpl T. et al., Clinical features of paediatric pulmonary hypertension: a registry study, Lancet (London, England), 2012.
[46] Van Loon RL, Roofthooft MT, Hillege HL et al., Pediatric pulmonary hypertension in the Netherlands. Epidemiology and characterization during the period 1991 to 2005, Circulation, 2011.
[47] Nakanishi H., Suenaga H., Uchiyama A. and Kusuda S., Persistent pulmonary hypertension of the newborn in extremely preterm infants: a Japanese cohort study, Archives of Disease in Childhood. Fetal and neonatal edition, 2018.
[48] Vachiéry JL, Tedford RJ, Rosenkranz S. et al., Pulmonary hypertension due to left heart disease, European Respiratory Journal, 2019.
[49] Berthelot E., Hatimi ES, Robarda I. et al., Pulmonary hypertension due to left heart disease, Archives of Cardiovascular Disease, 2017.
[50] Miller WL, Grill DE and Borlaug BA, Clinical features, hemodynamics, and outcomes of pulmonary hypertension due to chronic heart failure with reduced ejection fraction: pulmonary hypertension and heart failure, Journal of the American College of Cardiology. Heart failure., 2013.
[51] Khush KK, Tasissa G., Butler J. et al., Effect of pulmonary hypertension on clinical outcomes in advanced heart failure: analysis of the Evaluation Study of Congestive Heart Failure and Pulmonary Artery Catheterization Effectiveness (ESCAPE) database, American Heart Journal, 2009.
[52] Aronson D., Darawsha W., Atamna A. et al., Pulmonary hypertension, right ventricular function, and clinical outcome in acute decompensated heart failure, Journal of Cardiac Failure, 2013.
[53] Vachiéry JL, Adir Y., Barberà JA et al., Pulmonary hypertension due to left heart diseases, Journal of the American College of Cardiology, 2013.
[54] Naeije R., Gerges M., Vachiery JL et al., Hemodynamic phenotyping of pulmonary hypertension in left heart failure, Circulation. Heart failure, 2017.
[55] Gerges C., Gerges M., Lang MB et al., Diastolic pulmonary vascular pressure gradient:
a predictor of prognosis in “out-of-proportion” pulmonary hypertension, CHEST-Journal, 2013.
[56] Chaouat A., Bugnet AS, Kadaoui N. et al., Severe pulmonary hypertension and chronic obstructive pulmonary disease, American Journal of Respiratory and Critical Care Medicine, 2005.
[57] Nathan SD, Barbera JA, Gaine SP et al., Pulmonary hypertension in chronic lung disease and hypoxia, European Respiratory Journal, 2019.
[58] Cottin V., Le Pavec J., Prévot G. et al., Pulmonary hypertension in patients with combined pulmonary fibrosis and emphysema syndrome, European Respiratory Journal, 2010.
96
[59]Kim NH, Delcroix M., Jais X. et al., Chronic thromboembolic pulmonary hypertension, European Respiratory Journal, 2019.
[60] Coquoz N., Weilenmann D., Stolz D. et al., Multicentre observational screening survey for the detection of CTEPH following pulmonary embolism, European Respiratory Journal, 2018.
[61] Pepke-Zaba J., Delcroix M., Lang I. et al., Chronic thromboembolic pulmonary hypertension (CTEPH): results from an international prospective registry, Circulation, 2011.
[62] Taboada D., Pepke-Zaba J., Jenkins DP et al., Outcome of pulmonary endarterectomy in symptomatic chronic thromboembolic disease, European Respiratory Journal, 2014.
[63] Parent F., Bachir D., Inamo J. et al., A hemodynamic study of pulmonary hypertension in sickle cell disease, The New England Journal of Medicine, 2011.
[64] Derchi G., Galanello R., Bina P. et al., Prevalence and risk factors for pulmonary arterial hypertension in a large group of β-thalassemia patients using right heart catheterization: a Webthal study, Circulation, 2014.
[65] Cottin V., Harari S., Humbert M. et al., Pulmonary hypertension in lymphangioleiomyomatosis: characteristics in 20 patients, European Respiratory Journal, 2012.
[66] Frost A., Badesch D., Gibbs JSR et al., Diagnosis of pulmonary hypertension, European Respiratory Journal, 2019.
[67] Livio Giuliani and Serena Rossi, Cateterismo cardiaco destro: “tips and tricks”, Giornale Italiano di Cardiologia, 2020.
[68] Rosenkranz S. and Preston IR, Right heart catheterisation: best practice and pitfalls in pulmonary hypertension, European Respiratory Review: an official journal of the European Respiratory Society, 2015.
[69]Caravita S., Garascia A., Imbalzano E. et al., Il cateterismo cardiaco: la pressione di incuneamento arteriolare polmonare (wedge) e la pressione telediastolica ventricolare sinistra.
[70] Lim HS and Gustafsson F., Pulmonary artery pulsatility index: physiological basis and clinical application, European Journal of Heart Failure, 2020.