L'ipertensione polmonare:come diagnosticarla e trattarlaASMaD
Presentazione a cura del Dottor Carmine Dario Vizza - XII° Congresso Nazionale FIMeG 2018 - The Silver Tsunami: l'anziano fra appropriatezza e farmaeconomia
Extended focus assessment with sonography for traumaSamir Haffar
The document discusses extended focused assessment with sonography for trauma (E-FAST), an ultrasound technique used to rapidly detect fluid in body cavities resulting from trauma. It describes the indications, views, techniques, findings and advantages of E-FAST. Key points include that E-FAST can detect blood or fluid in the abdomen, chest or heart within 3-5 minutes without radiation. It is a useful first-line tool to identify life-threatening bleeding but does not replace definitive diagnostic tests if findings are uncertain or positive. E-FAST examination involves six standard views of the abdomen, chest and heart and can be repeated as needed at the bedside to dynamically monitor trauma patients.
L'ipertensione polmonare:come diagnosticarla e trattarlaASMaD
Presentazione a cura del Dottor Carmine Dario Vizza - XII° Congresso Nazionale FIMeG 2018 - The Silver Tsunami: l'anziano fra appropriatezza e farmaeconomia
Extended focus assessment with sonography for traumaSamir Haffar
The document discusses extended focused assessment with sonography for trauma (E-FAST), an ultrasound technique used to rapidly detect fluid in body cavities resulting from trauma. It describes the indications, views, techniques, findings and advantages of E-FAST. Key points include that E-FAST can detect blood or fluid in the abdomen, chest or heart within 3-5 minutes without radiation. It is a useful first-line tool to identify life-threatening bleeding but does not replace definitive diagnostic tests if findings are uncertain or positive. E-FAST examination involves six standard views of the abdomen, chest and heart and can be repeated as needed at the bedside to dynamically monitor trauma patients.
This document discusses primary percutaneous coronary intervention (PCI) versus thrombolysis for treating ST-elevation myocardial infarction (STEMI). It notes that PCI is more effective at reopening blocked arteries, reduces recurrent ischemia and shorter hospital stays compared to thrombolysis, but requires an invasive procedure and experienced staff. Thrombolysis can be used in facilities without PCI capabilities if administered within 30 minutes of arrival, but has higher risks of bleeding complications. The document recommends PCI over thrombolysis when experienced staff can perform it within 90-120 minutes of arrival, or if thrombolysis is contraindicated or failed to reopen the artery.
The univentricular repair indications, procedures, outcomes and controversiespatacsi
This document discusses surgical options for single ventricle heart defects, including the indications, timing, and outcomes of various palliative surgeries. It describes initial procedures like pulmonary artery banding or shunting to relieve obstruction and provide pulmonary blood flow. It then discusses staged repairs like the bidirectional Glenn procedure or hemi-Fontan that redirect blood flow. Finally, it covers the modified Fontan operation, which separates systemic and pulmonary circulation without a subpulmonary ventricle. Complications, long-term outcomes, and factors affecting success are also addressed.
CAD – leading cause of death
Cardiac SPECT – steady growth in last two decades & played an important role in clinical mangement
Radionuclide ventriculography (MUGA)
First pass studies
PET/CT
2019 ESC guidelines on pulmonary embolismSaitej Reddy
The document provides an overview of the updates in the 2019 guidelines for pulmonary embolism (PE) diagnosis and treatment. Key changes include adjusted D-dimer cut-off values based on age and probability; revised algorithms for diagnosing high-risk PE and assessing severity; recommending non-vitamin K antagonist oral anticoagulants as first-line treatment for eligible patients; classifying recurrence risk factors and extending treatment duration indications; and proposing a comprehensive post-PE patient follow-up algorithm. The guidelines aim to improve PE risk stratification, optimize acute care, determine chronic anticoagulation regimens, and ensure long-term management and surveillance for complications.
2015 ESC guidelines for the management of patients with ventricular arrhyth...João Antônio Granzotti
This document provides guidelines from the European Society of Cardiology (ESC) for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death. It was created by a Task Force of experts and endorsed by several ESC associations and councils. The guidelines cover definitions, epidemiology, risk prediction, screening and evaluation of patients, pharmacological and device-based therapies, and management recommendations for specific cardiac conditions like coronary artery disease and heart failure.
This document discusses heart failure, including:
1. It provides classifications of heart failure stages and functional classifications according to ACCF/AHA and NYHA systems.
2. It outlines treatment approaches for different heart failure stages, including diuretics, vasodilators, inotropes, and approaches based on fluid retention status.
3. It discusses the use of biomarkers like BNP and NT-proBNP in diagnosing and managing heart failure and factors that can influence biomarker levels.
Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive fibrosing interstitial pneumonia of unknown cause occurring primarily in older adults. The diagnosis of IPF requires exclusion of other known causes and either a usual interstitial pneumonia (UIP) pattern on high-resolution computed tomography (HRCT) or UIP findings on surgical lung biopsy along with specific combinations of HRCT and histopathology patterns determined through multidisciplinary discussion. Proper diagnostic criteria and communication between pulmonologists, radiologists, and pathologists can improve accuracy of diagnosis for this disease with a variable clinical course and unpredictable natural history.
explaining the presently available criteria to define futility in liver transplantation and prposing future trends in the definition of futility in liver transplantation
HRCT in Diffuse Lung Diseases - II (Honeycombing, UIP pattern, IPF)Bhavin Jankharia
This is the second part of this series on HRCT in diffuse lung diseases, focussing on the diagnosis of honeycombing, UIP pattern and IPF and the associated complications and differential diagnoses
This document summarizes CT findings that are useful for diagnosing chronic pulmonary thromboembolism (CPTE). It describes risk factors, clinical manifestations, and CT features of CPTE including vascular signs like pulmonary artery obstruction and dilation, parenchymal signs like scarring and mosaic perfusion patterns, and signs of pulmonary hypertension. Differential diagnoses including idiopathic pulmonary hypertension and acute PE are also discussed. CT is important for identifying treatable CPTE in patients with unexplained pulmonary hypertension.
Idiopathic pulmonary fibrosis (IPF) is characterized by a dominant pattern of reticular opacities and honeycombing, predominantly in the subpleural regions of the lower lobes. Ground-glass opacities and traction bronchiectasis may also be seen. The distribution demonstrates an apicobasal gradient. Nonspecific interstitial pneumonitis (NSIP) most commonly demonstrates a dominant ground-glass opacity pattern, distributed bilaterally and more prominent in the lower lobes without an apicobasal gradient. Cryptogenic organizing pneumonia (COP) appears as patches of consolidation and ground-glass opacity in the peripheral and subpleural regions of the middle and lower lung zones.
This presentation discusses how to evaluate shock using echocardiography. It defines shock and describes the main types: hypovolemic, cardiogenic, obstructive, and distributive. Guidelines recommend using echo to evaluate shock. The presentation reviews how to assess left ventricular systolic function, volume status, valvular pathology, and diastolic function. It provides examples of using echo to diagnose specific causes of shock like sepsis, myocardial infarction, pulmonary embolism, and tamponade. The key messages are that echo is very useful for diagnosing and managing shock, and training in point-of-care ultrasound is important.
Cardiovascular manifestation in systemic diseaseAnil Khatri
This document summarizes various cardiovascular manifestations that can occur in systemic diseases. It discusses how thiamine deficiency can cause heart failure and how supplementation can help. It also discusses protein-energy malnutrition and how it can affect the heart. Other conditions mentioned include hyperhomocysteinemia, obesity, carcinoid syndrome, pheochromocytoma, acromegaly, systemic lupus erythematosus, antiphospholipid antibody syndrome, systemic sclerosis, and rheumatoid arthritis; and their potential impacts such as cardiomyopathy, pericarditis, accelerated atherosclerosis, and pulmonary hypertension.
This document discusses primary percutaneous coronary intervention (PCI) versus thrombolysis for treating ST-elevation myocardial infarction (STEMI). It notes that PCI is more effective at reopening blocked arteries, reduces recurrent ischemia and shorter hospital stays compared to thrombolysis, but requires an invasive procedure and experienced staff. Thrombolysis can be used in facilities without PCI capabilities if administered within 30 minutes of arrival, but has higher risks of bleeding complications. The document recommends PCI over thrombolysis when experienced staff can perform it within 90-120 minutes of arrival, or if thrombolysis is contraindicated or failed to reopen the artery.
The univentricular repair indications, procedures, outcomes and controversiespatacsi
This document discusses surgical options for single ventricle heart defects, including the indications, timing, and outcomes of various palliative surgeries. It describes initial procedures like pulmonary artery banding or shunting to relieve obstruction and provide pulmonary blood flow. It then discusses staged repairs like the bidirectional Glenn procedure or hemi-Fontan that redirect blood flow. Finally, it covers the modified Fontan operation, which separates systemic and pulmonary circulation without a subpulmonary ventricle. Complications, long-term outcomes, and factors affecting success are also addressed.
CAD – leading cause of death
Cardiac SPECT – steady growth in last two decades & played an important role in clinical mangement
Radionuclide ventriculography (MUGA)
First pass studies
PET/CT
2019 ESC guidelines on pulmonary embolismSaitej Reddy
The document provides an overview of the updates in the 2019 guidelines for pulmonary embolism (PE) diagnosis and treatment. Key changes include adjusted D-dimer cut-off values based on age and probability; revised algorithms for diagnosing high-risk PE and assessing severity; recommending non-vitamin K antagonist oral anticoagulants as first-line treatment for eligible patients; classifying recurrence risk factors and extending treatment duration indications; and proposing a comprehensive post-PE patient follow-up algorithm. The guidelines aim to improve PE risk stratification, optimize acute care, determine chronic anticoagulation regimens, and ensure long-term management and surveillance for complications.
2015 ESC guidelines for the management of patients with ventricular arrhyth...João Antônio Granzotti
This document provides guidelines from the European Society of Cardiology (ESC) for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death. It was created by a Task Force of experts and endorsed by several ESC associations and councils. The guidelines cover definitions, epidemiology, risk prediction, screening and evaluation of patients, pharmacological and device-based therapies, and management recommendations for specific cardiac conditions like coronary artery disease and heart failure.
This document discusses heart failure, including:
1. It provides classifications of heart failure stages and functional classifications according to ACCF/AHA and NYHA systems.
2. It outlines treatment approaches for different heart failure stages, including diuretics, vasodilators, inotropes, and approaches based on fluid retention status.
3. It discusses the use of biomarkers like BNP and NT-proBNP in diagnosing and managing heart failure and factors that can influence biomarker levels.
Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive fibrosing interstitial pneumonia of unknown cause occurring primarily in older adults. The diagnosis of IPF requires exclusion of other known causes and either a usual interstitial pneumonia (UIP) pattern on high-resolution computed tomography (HRCT) or UIP findings on surgical lung biopsy along with specific combinations of HRCT and histopathology patterns determined through multidisciplinary discussion. Proper diagnostic criteria and communication between pulmonologists, radiologists, and pathologists can improve accuracy of diagnosis for this disease with a variable clinical course and unpredictable natural history.
explaining the presently available criteria to define futility in liver transplantation and prposing future trends in the definition of futility in liver transplantation
HRCT in Diffuse Lung Diseases - II (Honeycombing, UIP pattern, IPF)Bhavin Jankharia
This is the second part of this series on HRCT in diffuse lung diseases, focussing on the diagnosis of honeycombing, UIP pattern and IPF and the associated complications and differential diagnoses
This document summarizes CT findings that are useful for diagnosing chronic pulmonary thromboembolism (CPTE). It describes risk factors, clinical manifestations, and CT features of CPTE including vascular signs like pulmonary artery obstruction and dilation, parenchymal signs like scarring and mosaic perfusion patterns, and signs of pulmonary hypertension. Differential diagnoses including idiopathic pulmonary hypertension and acute PE are also discussed. CT is important for identifying treatable CPTE in patients with unexplained pulmonary hypertension.
Idiopathic pulmonary fibrosis (IPF) is characterized by a dominant pattern of reticular opacities and honeycombing, predominantly in the subpleural regions of the lower lobes. Ground-glass opacities and traction bronchiectasis may also be seen. The distribution demonstrates an apicobasal gradient. Nonspecific interstitial pneumonitis (NSIP) most commonly demonstrates a dominant ground-glass opacity pattern, distributed bilaterally and more prominent in the lower lobes without an apicobasal gradient. Cryptogenic organizing pneumonia (COP) appears as patches of consolidation and ground-glass opacity in the peripheral and subpleural regions of the middle and lower lung zones.
This presentation discusses how to evaluate shock using echocardiography. It defines shock and describes the main types: hypovolemic, cardiogenic, obstructive, and distributive. Guidelines recommend using echo to evaluate shock. The presentation reviews how to assess left ventricular systolic function, volume status, valvular pathology, and diastolic function. It provides examples of using echo to diagnose specific causes of shock like sepsis, myocardial infarction, pulmonary embolism, and tamponade. The key messages are that echo is very useful for diagnosing and managing shock, and training in point-of-care ultrasound is important.
Cardiovascular manifestation in systemic diseaseAnil Khatri
This document summarizes various cardiovascular manifestations that can occur in systemic diseases. It discusses how thiamine deficiency can cause heart failure and how supplementation can help. It also discusses protein-energy malnutrition and how it can affect the heart. Other conditions mentioned include hyperhomocysteinemia, obesity, carcinoid syndrome, pheochromocytoma, acromegaly, systemic lupus erythematosus, antiphospholipid antibody syndrome, systemic sclerosis, and rheumatoid arthritis; and their potential impacts such as cardiomyopathy, pericarditis, accelerated atherosclerosis, and pulmonary hypertension.
Evoluzione delle tasche e degli archi branchiali. Formazione del seno cervicale. Cisti e fistole congenite del collo, mediane e laterali. Strategie diagnostiche e terapeutiche
PULMONARY ENDARTERECTOMY: THE PAVIA EXPERIENCEPAH-GHIO
1) Pulmonary endarterectomy (PEA) is a life-saving surgery for chronic thromboembolic pulmonary hypertension that removes blood clots from the lungs.
2) The document describes the PEA program at the University of Pavia School of Medicine in Italy, which has performed over 400 PEAs since 1994 and currently performs about 60 PEAs per year.
3) Key requirements for PEA candidacy include being in heart failure functional class III or IV, having pulmonary hypertension for at least 3 months after blood clotting, and having pulmonary vascular resistance over 300 dynes*sec*cm-5.
IL SOSPETTO DELL'IPERTENSIONE POLMONARE SECONDARIA A MALATTIA POLMONARE(ILD)PAH-GHIO
1) Pulmonary hypertension (PH) is a common complication in patients with interstitial lung disease (ILD) that significantly worsens outcomes.
2) The pathogenesis of PH in ILD is complex, with multiple potential mechanisms, and it is classified as group III PH.
3) Screening for PH in ILD patients is prudent given its impact, though guidelines are lacking. Clinical features raising suspicion include reduced diffusion capacity on pulmonary function tests and shorter 6-minute walk distances. Echocardiography is commonly used for screening but right heart catheterization is needed for definitive diagnosis.
This document describes the case of a patient with chronic thromboembolic pulmonary hypertension (CTEPH) who initially presented with mild dyspnea in 2004. Despite treatment, the patient's condition progressed to severe pulmonary hypertension. The patient was deemed inoperable for CTEPH due to exclusively distal disease. The patient was treated with drugs but remained inoperable until 2009 when a reassessment found the patient was now technically operable; the patient then underwent successful pulmonary endarterectomy to remove blood clots from the lungs. Following surgery, the patient's hemodynamics and functional status significantly improved.
Stefano Ghio leads a multidisciplinary team at Fondazione IRCCS Policlinico S Matteo in Pavia, Italy that treats pulmonary arterial hypertension (PAH). The team includes specialists in cardiology, rheumatology, pneumology, infectious disease, radiology, nuclear medicine, cardiac surgery, hematology, and otorhinolaryngology.
Ipertensione Polomonare nelle malattie polmonariPAH-GHIO
The document discusses pulmonary hypertension (PH) in chronic obstructive pulmonary disease (COPD) and interstitial lung diseases (ILDs) such as idiopathic pulmonary fibrosis (IPF). It summarizes findings from studies on the prevalence and impact of PH in these conditions. It also reviews potential treatments for PH in COPD/ILD, including vasodilator therapies such as sildenafil, bosentan, and iloprost, though evidence of benefit is limited. Precise definitions and screening are needed to identify patients with disproportionate PH who may warrant more aggressive management.
Ipertensione Polmonare nelle malattie ematologichePAH-GHIO
The document discusses pulmonary hypertension (PH) in hematological diseases such as myeloproliferative neoplasms (MPNs). Several studies found high rates of PH in patients with MPNs like primary myelofibrosis, essential thrombocythemia, and polycythemia vera. The mechanisms contributing to PH in MPNs include thrombocytosis, non-hepatic hematopoiesis, enhanced angiogenesis, and portal hypertension. Current recommendations for managing PH associated with MPNs include ruling out other causes, documenting elevated pulmonary pressures, and considering low-dose whole lung irradiation. Some research also links PH with myelofibrosis-like changes in the bone marrow.
The document discusses pulmonary hypertension in group 2, which refers to pulmonary hypertension associated with left heart disease. Some key points:
- Pulmonary hypertension is common in patients with advanced heart failure, occurring in about 2/3 of patients evaluated for possible heart transplant.
- Pulmonary hypertension in heart failure is caused by backward transmission of high left-sided filling pressures and reactive increases in pulmonary vascular resistance.
- Pulmonary hypertension in heart failure is associated with reduced exercise tolerance, poor prognosis, and high risk for heart transplant.
- Treatment of pulmonary hypertension in heart failure focuses on conventional medical management and specific transplant approaches, though future therapies directly targeting the right ventricle are being investigated.
This document discusses the treatment of chronic thromboembolic pulmonary hypertension (CTEPH) including:
1) CTEPH was historically treated with lung transplantation but can now often be treated with pulmonary endarterectomy (PEA) surgery without transplantation.
2) The document outlines patient selection criteria for PEA versus transplantation based on clinical factors like age and hemodynamics as well as anatomical factors regarding lesion location.
3) It provides an overview of one center's experience performing over 350 PEAs for CTEPH patients.
1. L’ipertensione polmonare nelle epatopatie Anna Sara Pazzano Divisione di Cardiologia Fondazione IRCCS Policlinico San Matteo, Pavia Corso di aggiornamento sull’Ipertensione Polmonare. Pavia 20-21 Maggio 2011
6. Corso di aggiornamento sull’Ipertensione Polmonare. Pavia 20-21 Maggio 2011 Epidemiologia Prevalenza =10-32% dei pazienti affetti da cirrosi epatica ed ipertensione portale valutati per trapianto di fegato Fallon MB, et al. Gastroenterology 2008 October; 135(4):1168-1175.
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9. Corso di aggiornamento sull’Ipertensione Polmonare. Pavia 20-21 Maggio 2011 Prognosi La presenza della Sindrome epatopolmonare è un fattore di rischio maggiore indipendente di mortalità nei pazienti con cirrosi ed ipertensione portale. In uno studio la sopravvivenza media dei pazienti con epatopatia e sindrome epatopolmonare era 10,6 mesi , mentre era 40,8 mesi nei pazienti cirrotici senza sindrome epatopolmonare. Le differenze rimangono statisticamente significative anche quando si aggiustano le categorie per severità dell’epatopatia (Child-Pugh). Schenk P, et al. Gastroenterology 2003 Oct;125(4):1042-52
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12. =10% delle PAH Corso di aggiornamento sull’Ipertensione Polmonare. Pavia 20-21 Maggio 2011
13. Corso di aggiornamento sull’Ipertensione Polmonare. Pavia 20-21 Maggio 2011 proliferazione intimale fibrosi avventiziale ipertrofia della media Normale IAP lesioni plessiformi Caratteristiche anatomopatologiche
14. Corso di aggiornamento sull’Ipertensione Polmonare. Pavia 20-21 Maggio 2011 Epidemiologia Prevalenza =1-2% dei pazienti con cirrosi epatica =2-6% dei pazienti con ipertensione portale E’ possibile che questi dati sottostimino la reale prevalenza della malattia (pazienti asintomatici).
15. Corso di aggiornamento sull’Ipertensione Polmonare. Pavia 20-21 Maggio 2011 Simmonneau G, et al. Int J Clin Pract Suppl. Jan 2011; (169): 11-8 Patogenesi
17. Corso di aggiornamento sull’Ipertensione Polmonare. Pavia 20-21 Maggio 2011 Clinica Indistinguibile dall’ipertensione arteriosa polmonare idiopatica. N.B. 60% asintomatici!!! Associata a sintomi e segni secondari all’epatopatia (varici gastroesofagee, splenomegalia, ascite, encefalopatia).
18. Corso di aggiornamento sull’Ipertensione Polmonare. Pavia 20-21 Maggio 2011 Diagnosi Flow-chart per la diagnosi dell’ipertensione polmonare: - Approccio multidisciplinare; - Necessario anche in pazienti che sono affetti da nota epatopatia!
19. Corso di aggiornamento sull’Ipertensione Polmonare. Pavia 20-21 Maggio 2011 Sopravvivenza a 3 anni = 38%
23. Corso di aggiornamento sull’Ipertensione Polmonare. Pavia 20-21 Maggio 2011 43 pazienti sottoposti a trapianto epatico 35% mortalità (93% cause cardio-polmonari)
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27. Corso di aggiornamento sull’Ipertensione Polmonare. Pavia 20-21 Maggio 2011 Caso clinico 1: parasternale asse lungo
28. Corso di aggiornamento sull’Ipertensione Polmonare. Pavia 20-21 Maggio 2011 Caso clinico 1: parasternale asse corto
29. Corso di aggiornamento sull’Ipertensione Polmonare. Pavia 20-21 Maggio 2011 Caso clinico 1: apicale 4C
30. Corso di aggiornamento sull’Ipertensione Polmonare. Pavia 20-21 Maggio 2011 Caso clinico 2: parasternale asse lungo
31. Corso di aggiornamento sull’Ipertensione Polmonare. Pavia 20-21 Maggio 2011 Caso clinico 2: parasternale asse corto
32. Corso di aggiornamento sull’Ipertensione Polmonare. Pavia 20-21 Maggio 2011 Caso clinico 2: apicale 4C
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34. Corso di aggiornamento sull’Ipertensione Polmonare. Pavia 20-21 Maggio 2011 Caso clinico 1: cateterismo cardiaco destro Caso clinico 2: cateterismo cardiaco destro RAP (mmHg) sPAP (mmHg) mPAP (mmHg) PVR (dyne.sec.cm5) PVR (UW) PWP (mmHg) CO (l/min) CI (l/min/m2) 10 56 39 154 1,92 18 10,9 4,5 RAP (mmHg) sPAP (mmHg) mPAP (mmHg) PVR (dyne.sec.cm5) PVR (UW) PWP (mmHg) CO (l/min) CI (l/min/m2) 10 66 43 705 8,8 12 3,52 2.2 Sindrome epato-polmonare Ipertensione porto-polmonare
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Editor's Notes
L’ultima classificazione di Dana Point del 2008 pone l’ipertensione polm associata ad epatopatia nel gruppo 1; tale clasificazione è in reltà una semplificazione, dal momento che esistono non uno ma due tipi di IP associata ad epatopatia.
L’aumento delle pressioni polmonari nei pazienti affetti da epatopatia si può verificare in due tipi di sindromi, tra di loro molto diverse: SEP e IPP. Qui vediamo raffigurate le caratteristiche salienti.
E’ complessa e rimane sconosciuta. iNOS= inducible nitric oxide synthase
La platipnea e ortodeossia sembrerebbe sia dovuto a un aumento dello shunt intrapolmonare destro-sinistro (da arteria a vena polmonare) nella regioni basali dei polmoni in ortostatismo. Si può osservare anche in altri tipi di shunt dx-sx (ad es. DIA).
Studiare mecc
Spiegazioni degli autori a tali riscontri: sesso F: profilo ormonale, gravidanza, predisposizione a pato autoimmuni; epatiti autoimmuni: diverse sono le pato autoimmuni che si associano a IP (connetivopatie, tiroiditi autoimmuni,…); epatite C: ci potrebbe essere un ruolo protettivo del virus sulle art polmonari.
IP idiopatica sopravvivenza a 3 anni = 72% (sec questo studio, in generale 48%).
Nonostante questi paz abbiano CO più elevate e PVR più basse (equazione NIH). Analizzando le cause di morte le morti cardio-vascolari sono più frequenti nella IP PP rispetto alla IP I, in assenza però di una differenza stat significativa. La spiegazione di tale riscontro non è nota: una possibile spiegazione potrebbe essere che l’aumento di mortalità nei IP PP sia secondaria allipertensione polmonare o alle sue conseguenze (varici). In alternativa la aumentata portata potrebbe sottoporre a uno stress di parete continuo che porterebbe a una più rapida progressione di malattia vascolare polmonare. Infine, L’ultima ipotesi avanzata è che la ridotta capacità detox del fegato det un’aumentata prod di sost proliferative, infiammatorie e VC, che porterebbero ad un’importante accelerazione della malattia.
beta-bloccante ( riduce la pressione portale vasodilatando sia il letto vascolare splacnico arterioso, sia il sistema venoso portale )
Il trapianto di fegato non solo non fa parte del trattamento, ma talvolta è controindicato in presenza di IP PP. Studio retrospettivo. Parametri emodinamici associati a mortalità cardiopolmonare sono risultati: PVR, PAPm, GTP. Inoltre gli autori hanno osservato che nei paz con PAPm >= 50 la mortalità era 100%, con PAPm < 35 mmHg era 0%; tra i pazienti con PAPm 35-45 mmHg quelli con PVR n 0%, con PVR elevate 50%. Pertanto attualmete le LG controindicano il trap di fegato in paz con PAPm >=35 mmHg e PVR elevate (>=250). Ci sono diversi casi in letteratura di pre-trattamento dei questi pz ad alto R con farmaci VD con buoni risultati.
VPP basso, percio’ è necessario fare cat dx per conferma diagnosi! Ma sens 100%, perciò tutti i malati vengono individuati!