Patologia Vascular Flashcards
Definição hemodinamica de hipertensão pulmonar
Pressão arteria pulmonar média >20 mmHg
-Pre capilar: PAWP<=15 mmHg e PVR>2WU [HP arterial, devida a doenças pulmonares, tromboembolica]
-Pós capilar isolada: PAWP>15 mmHg e PVR<=2WU
-Combinada pre e pós: PAWP>15 e PVR>2 [HP associada a dc cardíaca esq]
(PAWP pressão de encravamento da AP; PVR resistência vascular pulmonar)
-Exercício: mPAP/CO slope >3 mmHg/L/min entre repouso e exercício
-não classificada: PAWP<15 e PVR<2
Classificação clínica de HP
GROUP 1 Pulmonary arterial hypertension (PAH)
1.1 Idiopathic 1.1.1 Non-responders at vasoreactivity testing 1.1.2 Acute responders at vasoreactivity testing 1.2 Heritablea 1.3 Associated with drugs and toxinsa 1.4 Associated with: 1.4.1 Connective tissue disease 1.4.2 HIV infection 1.4.3 Portal hypertension 1.4.4 Congenital heart disease 1.4.5 Schistosomiasis 1.5 PAH with features of venous/capillary (PVOD/PCH) involvement 1.6 Persistent PH of the newborn
GROUP 2 PH associated with left heart disease
2.1 Heart failure: 2.1.1 with preserved ejection fraction 2.1.2 with reduced or mildly reduced ejection fractionb 2.2 Valvular heart disease 2.3 Congenital/acquired cardiovascular conditions leading to post-capillary PH
GROUP 3 PH associated with lung diseases and/or hypoxia 3.1 Obstructive lung disease or emphysema 3.2 Restrictive lung disease 3.3 Lung disease with mixed restrictive/obstructive pattern 3.4 Hypoventilation syndromes 3.5 Hypoxia without lung disease (e.g. high altitude) 3.6 Developmental lung disorders
GROUP 4 PH associated with pulmonary artery obstructions 4.1 Chronic thrombo-embolic PH 4.2 Other pulmonary artery obstructionsc
GROUP 5 PH with unclear and/or multifactorial mechanisms
5.1 Haematological disordersd 5.2 Systemic disorderse 5.3 Metabolic disordersf 5.4 Chronic renal failure with or without haemodialysis 5.5 Pulmonary tumour thrombotic microangiopathy 5.6 Fibrosing mediastinitis
Alterações típicas do ECG de HP
• P pulmonale (P>0.25 mV in lead II)
• Right or sagittal axis deviation (QRS axis >90° or indeterminable)
• RV hypertrophy (R/S >1, with R >0.5 mV in V1; R in V1 + S in lead V5 >1 mV)
• Right bundle branch block—complete or incomplete (qR or rSR patterns in V1)
• RV strain pattern (ST depression/T-wave inversion in the right precordial V1–4 and inferior II, III, aVF leads)
• Prolonged QTc interval (unspecific)
Probabilidade de ecocardiograma na HP
1) Velocidade pico da regurgitação da tricuspide (<=2.8 / 2.9-3.4 / >3.4)
2) Presença de outros sinais ecocardiograficos de HP (de pelo menos 2 categorias)
2.1.) ventrículos: RV/LV basal diameter/area ratio >1.0. Flattening of the interventricular septum (LVEI >1.1 in systole and/ or diastole). TAPSE/sPAP ratio <0.55 mm/mmHg
2.2.) Artéria pulmonar: RVOT AT <105 ms and/or mid-systolic notching. Early diastolic pulmonary regurgitation velocity >2.2 m/s. PA diameter >AR diameter PA diameter >25 mm
2.3.) Veia cava inferior e auricula direita: IVC diameter >21 mm with decreased inspiratory collapse (<50% with a sniff or <20% with quiet inspiration). RA area (end-systole) >18 cm2
BAIXA probabilidade PH: pTRV<=2.8 sem outros sinais ecocardiograficos
INTERMEDIA probabilidade PH: pTRV<=2.8 com outros sinais ecocardiograficos; ou pTRV 2.9-3.4 sem outros sinais ecocardiograficos
ALTA probabilidade PH: pTRV 2.9-3.4 com outros sinais ecocardiograficos; ou pTRV>3.4
Teste de vasoreactividade
-Vasoreactivity testing is recommended in patients with Idiopathic /Hereditary /Drug associated PAH to detect patients who can be treated with high doses of a CCB
-It is recommended that vasoreactivity testing is performed at PH centres It is recommended to consider a positive response to vasoreactivity testing by a reduction in mPAP ⩾10mmHg to reach an absolute value of mPAP ⩽40 mmHg with an increased or unchanged CO
-Inhaled nitric oxide, inhaled iloprost, or i.v. epoprostenol are recommended for performing vasoreactivity testing
Fatores de risco para HPA e CTEPH
Fatores de risco para HPA: connective tissue disease (especially systemic sclerosis), portal hypertension, HIV infection, and family history of PAH.
Fatores de risco para CTEPH: History of PE, permanent intravascular devices, inflammatory bowel diseases, essential thrombocythaemia, splenectomy, high-dose thyroid hormone replacement, and malignancy.
Tratamento de HPA idiopatica, hereditária ou associada a drogas
Initial therapy SEM comorbilidades cardiopulmonares:
-present at high risk of death, initial combination therapy with a PDE5i, an ERA, and i.v./s.c. prostacyclin analogues should be considered
-present at low or intermediate risk of death, initial combination therapy with a PDE5i and an ERA is recommended (ambrisentan+tadalafil ou macitentan+tadalafil)
Follow up:
-present at intermediate–low risk of death while receiving ERA/PDE5i therapy, addition of selexipag should be considered. Switching from PDE5i to riociguat
-intermediate–high or high risk of death while receiving ERA/PDE5i therapy, addition of i.v./s.c. prostacyclin analogues and referral for LTx evaluation should be considered
-intermediate–low risk of death while receiving ERA/PDE5i therapy, switching from PDE5i to riociguat may be considered
COM comorbilidades cardiopulmonares:
-Recommendations for initial therapy: In patients with IPAH/HPAH/DPAH and cardiopulmonary comorbidities, initial monotherapy with a PDE5i or an ERA should be considered
-during follow-up In patients with IPAH/HPAH/DPAH with cardiopulmonary comorbidities who present at intermediate or high risk of death while receiving PDE5i or ERA monotherapy, additional PAH medications may be considered on an individual basis
Transplante pulmonar na HAP
Referral
-Potentially eligible patients for whom LTx might be an option in case of treatment failure
-ESC/ERS intermediate–high or high risk or REVEAL risk score >7 on appropriate PAH medication
-Progressive disease or recent hospitalization for worsening PAH
-Need for i.v. or s.c. prostacyclin therapy
-Known or suspected high-risk variants, such as PVOD or PCH, systemic sclerosis, or large and progressive pulmonary artery aneurysms
-Signs of secondary liver or kidney dysfunction due to PAH or other potentially life-threatening complications, such as recurrent haemoptysis
Listing
-Patient has been fully evaluated and prepared for transplantation
-ESC/ERS high risk or REVEAL risk score >10 on appropriate PAH medication, usually including i.v. or s.c. prostacyclin analogues
-Progressive hypoxaemia, especially in patients with PVOD or PCH
-Progressive, but not end-stage liver or kidney dysfunction due to PAH, or life-threatening haemoptysis
Grupo 4 de HP crónica tromboembolica
Abordagem
-Lifelong, therapeutic doses of anticoagulation are recommended in all
-Antiphospholipid syndrome testing is recommended. Nestes é recomendada anticoagulants com varfarina
Tratamento:
-endarterectomia pulmonar é o tratamento de escolha na CTEPH com obstruções fibroticas das AP acessíveis
-angioplastia pulmonar com balão é recomendada nos doentes tecnicamente inoperantes ou que tem HP residual após EP e obstruções distais abordáveis
-riociguat é recomendado em doentes sintomáticos com CTEPH inoperavel ou com HP persistente/recorrente após EP
-trepostinil sc pode ser considerado em doentes com WHO-FC III-IV que tem CTEPH inoperavel ou com HP persistente/recorrente após EP
Fatores de risco para Tromboembolismo venoso
FORTE [sanfil+cardio]
-fratura perna
-prótese anca ou joelho
-trauma major
-lesão da spinal cord
-internamento por IC ou FA (nos 3 meses antes)
-EAM (nos 3 meses antes)
-TEV previo
MODERADO
Arthroscopickneesurgery Autoimmunediseases Bloodtransfusion Centralvenouslines Intravenouscathetersandleads Chemotherapy Congestiveheartfailureorrespiratoryfailure Erythropoiesis-stimulatingagents Hormonereplacementtherapy(dependsonformulation) Invitrofertilization Oralcontraceptivetherapy Post-partumperiod Infection(specificallypneumonia,urinarytract infection,andHIV) Inflammatoryboweldisease Cancer(highestriskinmetastaticdisease) Paralyticstroke Superficialveinthrombosis Thrombophilia
WEAK
Bedrest>3days Diabetesmellitus Arterialhypertension Immobilityduetositting(e.g.prolongedcarorairtravel) Increasingage Laparoscopicsurgery(e.g.cholecystectomy) Obesity Pregnancy Varicoseveins
Probabilidade clínica de TEP
Regra de Wells
Regra de Geneva
Em 3 níveis: Baixo, intermédio ou alto
Em 2 níveis unlikely ou likely
Tem em conta:
-PreviousPEorDVT
-Heartrate
-Surgeryorfracturewithinthe pastmonth
-Haemoptysis
-Activecancer
-Unilaterallower-limbpain
-Painonlower-limbdeepvenous palpationandunilateraloedema
-Age>65years
Gravidade de TEP
PESI para classificar a mortalidade em 30 dias (5 classes)
Classificação de gravidade de TEP
HIGH: se instabilidade hemodinamica (paragem cardíaca, choque obstrutivo ou hipotensão persistente)
INTERMEDIATE HIGH: se PESI classes III-V/sPESI>=1 + Disfunção VD (no ETT ou TCT) + Troponina elevada
INTERMEDIATE LOW: se PESI classes III-V/sPESI>=1 + uma ou nenhuma das seguintes (Disfunção VD, Troponina elevada)
LOW: sem instabilidade hemodinamica, PESI classes I-II/sPESI<1, sem disfunção VD , sem troponina elevada
Tratamento na fase aguda de TEP
HIGH Risk:
-recomendada iniciar anticoagulacao com heparina não fracionada (UFH) sem atraso
-recomendada trombolise sistémica
-Embolectomia pulmonar cirúrgica é recomendada quando falha ou está contra-indicada a trombolise
-considerar tratamento dirigido por catheter percutaneo quando falha ou está contra-indicada a trombolise
Risco INTERMEDIATE ou LOW
-iniciar anticoagulacao sem atrasos se risco elevado ou intermédio(enquanto decorre o diagnóstico)
-anticoagulacao recomendada inicialmente com HBPM ou fondaparinux
-quando anticoagulacao passa a oral é preferível NOAC a varfarina
-quando tratados com varfarina tem de fazer ponde com AÇO ev até INR 2-3
-NOAC não recomendado quando lesão renal grave, gravidez ou síndrome antifosfolipidico
Classificação de vasculites sistémicas primárias
Large vessel vasculitis
-Giant-cell arteritis
-Takayasu arteritis
Medium-sized vessel vasculitis
-Polyarteritis nodosa
-Kawasaki disease
Small vessel vasculitis ANCA-associated vasculitis
-Granulomatosis with polyangiitis (Wegener) >80% tem C-ANCA PR3-ANCA
-Microscopic polyangiitis maioria tem P-ANCA MPO-ANCA
-Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) syndrome. maioria tem P-ANCA MPO-ANCA
Immune complex small vessel vasculitis
-Anti-GBM disease
-IgA vasculitis
-Cryoglobulinemic vasculitis
-Hypocomplementemic urticarial vasculitis
Variable vessel vasculitis
-Behçet disease
Tratamento da vasculites ANCA positiva
-For remission-induction of new-onset organ-threatening or life-threatening AAV we recommend treatment with a combination of glucocorticoids and either cyclophosphamide OR rituximab.
-For remission-induction of NON-organ-threatening AAV we recommend treatment with a combination of glucocorticoids and either methotrexate or mycophenolate mofetil.
-For a major relapse of organ-threatening or life-threatening disease in AAV we recommend treatment as per new disease with a combination of glucocorticoids and either cyclophosphamide OR rituximab.
-Plasma exchange should be considered for patients with AAV and a serum creatine level of ≥500 mmol/L (5.7 mg/dL) due to rapidly progressive glomerulonephritis in the setting of new or relapsing disease.
-Plasma exchange can also be considered for the treatment of severe diffuse alveolar haemorrhage.
-For remission-maintenance of AAV we recommend treatment with a combination of low-dose glucocorticoids and either azathioprine, rituximab, methotrexate or mycophenolate mofetil
-We recommend that remission-maintenance therapy for AAV be continued for at least 24 months following induction of sustained remission.
-For patients with AAV refractory to remission-induction therapy we recommend switching from cyclophosphamide to rituximab or from rituximab to cyclophosphamide. These patients should be managed in close conjunction with, or referred to, an expert centre for further evaluation and potential enrolment in clinical trials.
We recommend the investigation of persistent unexplained haematuria in patients with prior exposure to cyclophosphamide.
Hypoimmunoglobulinaemia has been noted after treatment with rituximab. We recommend testing of serum immunoglobulin levels prior to each course of rituximab and in patients with recurrent infection.
