Classificação e Diagnóstico da Hipertensão Pulmonar Raquel M. N. de Carvalho

Definição PAPm > 25mmHg em repouso PAPm > 25mmHg em repouso HP PAPm > 25mmHg em repouso PAWP (POAP) ≤ 15mmHg RVP > 3 unidades Woods (mmHg/l.mun) HAP

Definição VENOSA Table 2. Proposed Definition and Classification of PH-LHD Terminology PAWP Diastolic PAP – PAWP Isolated post-capillary PH >15 mm Hg <7 mm Hg Combined post-capillary and pre-capillary PH >15 mm Hg ≥7 mm Hg Hemodynamic measurements are taken under resting conditions. Gradiente transpulmonar – menos estável < 12 mmHg – passiva > 12 mmHg – reativa J Am Coll Cardiol. 2013;62(25_S)

Classificação JACC Vol. 62, No. 25, Suppl D, 2013 Superfamília TGF-B Não-TGF-B Anorexígenos (Benfluorex) | Inibidores tirosina-kinase (Desatinib) –0,45%;| INF alfa e beta Esclerodermia – 7-12%  detecção precoce HIV – 0,5%  sobrev 5 anos – 70% Portopulmonar – 6% pctes com hipertensão portal  independe gravidade dça hepática CHD/HAP – 10% adultos – avaliar bem p/ classificar  parte p/ grupo 2 AF – 6-10%  >>ria pós-capilar SCH - 200mi pessoas  10% dça hepatoesplênica  5% HAP PPHN – inibidores receptação serotonina JACC Vol. 62, No. 25, Suppl D, 2013

Sintomas por mais de 2 anos (21%) Maioria com CF III-IV (*) DIAGNÓSTICO Detecção Classificar Detecção precoce – screening populações de risco (DTC, DCC, Dça hepática crônica, HIV) Achado ocasional *REVEAL. Chest 2011;40:19-29 JACC Vol. 62, No. 25, Suppl D, 2013

SCREENING DIAGNÓSTICO ECO Sinais e sintomas CATE JACC Vol. 62, No. 25, Suppl D, 2013

Diagnóstico dispneia aos esforços, fadiga, palpitação, pré-síncope/síncope, precordialgia SINTOMAS Cardiovascular: turgência jugular, hiperfonese de B2, desdobramento de B2, sopro sistólico em tricúspide Sinais de IVD: hepatomegalia, ascite, edema de MMII SINAIS Ann Intern Med 1991;115:343-349

Diagnóstico Radiografia de tórax AD e VD Dilatação AP  APd > 16 mm APe > 18 mm Am J Respir Crit Care Med 2009;179:A4916

Diagnóstico ECG Desvio do eixo para direita Onda p proeminente em DII R/S > 1 (V1) Desvio do eixo para direita Onda p proeminente em DII Sinais de aumento do VD Padrão de strain R/S < 1 (V5 OU V6) Ann Intern Med 1991;115:343-349 Annals of Int Med 1987;107:216

Diagnóstico ECOCARDIOGRAMA PSAP = 4(VRT)² + PAD VRT > 2.8 (m·s−1) considerar HP Critérios sugestivos de HP (ERS) Peak tricuspid jet velocity (m·s−1) Estimated Ppa,syst (mmHg) # Other echo signs of PH present PH (grade of evidence) ≤2.8 ≤36 No Improvável (I-B) Yes Possível (IIa-C) 2.9–3.4 37–50 >3.4 >50 Yes/No Provável (I-B) #: echo-Doppler estimate of systolic pulmonary arterial pressure (Ppa,syst) based on tricuspid jet method (right atrial pressure assumed to be 5 mmHg). PAPs > 38mmHg → PAPm = 25mmHg Eur Respir Rev 2010; 19: 118, 288–299

Diagnóstico ECOCARDIOGRAMA PAPs TAPSE PAPm = 0.61 (PAPs) + 2 (mmHg) PROGNÓSTICO PAPm = 0.61 (PAPs) + 2 (mmHg) TAPSE < 1.8 cm5 Derrame pericárdico TAPSE AD Disfunção VD CHEST 2004; 126:1313–1317 Eur Respir Rev 2010; 19: 118, 288–299

ECOCARDIOGRAMA

Diagnóstico ESTUDO HEMODINÂMICO Mortalidade relacionada ao procedimento  0,005% (IC 0,01-0,099%) No de complicações graves não fatais  1,1% (IC 0,9-1,1%) J Am Coll Cardiol 2006;48:2546-52 Manobras  repouso: avaliação basal teste de vasorreatividade: APENAS na HAP idiopática prova de volume: 500 ml, bolus 5-10 min, pode PAWP em 15mmHg hemodinâmica no exercício complementação com cate esquerdo JACC Vol. 62, No. 25, Suppl D, 2013 Fatores prognósticos: PAD e IC

Diagnóstico ESTUDO HEMODINÂMICO Cardiol Clin 2004;22:401-15

Importância dos screenings Esclerodermia SOBREVIDA Screening Prática clínica normal Arthritis & Rheumatism, Vol. 63, No. 11, November 2011, pp 3522–3530

Falhou em detectar 4% pctes com PAH Esclerodermia DETECT Risco de PAH: Dça > 3 anos, DLCO < 60%, CVF > 40% DLCO > 60% Protocolo não validado 1º passo: Telangectasia, anticorpo anticentrômero, desvio do eixo p/D no ECG, PFP c/ DLCO, NT-proBNP, ácido úrico > 300 pts Falhou em detectar 4% pctes com PAH > 35 pts 2º passo: ECO em paciente em risco (area AD E VRT) JACC Vol. 62, No. 25, Suppl D, 2013 Ann Rheum Dis 2013 May 18 Cateterismo

Nomograms for practical application of the DETECT algorithm: determination of the likelihood of pulmonary arterial hypertension and cut-off points for decision to refer a patient to echocardiography (Step 1) and subsequent right heart catheterisation (Step 2). At Step 1 (top panel), risk points for each of the six non-echocardiographic variables are calculated by reading from ‘Individual risk points in Step 1’ and adding them up to obtain a total. If the ‘Total risk points from Step 1’ is >300 (corresponding to a sensitivity of 97% as selected by the Study Scientific Committee) the patient is referred to echocardiography. Similarly, at Step 2 (bottom panel), risk points for the carried forward ‘Total risk points from Step 1’ and the two echocardiographic variables are calculated by reading from the ‘Individual risk points in Step 2’. If the ‘Total risk points from Step 2’ is >35 (corresponding to a specificity of 35% as selected by the Study Scientific Committee) the patient is referred to right heart catheterisation. Alternatively, being less conservative (65% predefined specificity at Step 2), the patient would be referred to right heart catheterisation if ‘Total risk points from Step 2’ is >40 (compare table 3 for the performance of these two options). Note that all variables will always contribute risk points irrespective of the measured value; for example, a negative serum ACA will contribute 50 risk points. Exclusion of any single variable from the DETECT algorithm has only a small impact on model performance (see online supplementary appendix 9). If a single Step 1 variable is missing it should be assigned 50 risk points, with the exception of current/past telangiectasias which should be assigned 65 points. If a single Step 2 variable is missing it should be assigned 10 points. The nomograms cannot be reliably used if more than one variable out of the eight total variables is missing. ACA, anticentromere antibody; DLCO, pulmonary diffusing capacity for carbon monoxide; FVC, forced vital capacity; NTproBNP, N-terminal probrain natriuretic peptide; TR, tricuspid regurgitant jet. 35 J Gerry Coghlan et al. Ann Rheum Dis doi:10.1136/annrheumdis-2013

Gravidade e Localização CTEPH Screening Gravidade e Localização Avaliar Tratamento J Am Coll Cardiol. 2013;62

Anemia Falciforme Screening Am J Respir Crit Care Med Vol 189, Iss 6, pp 727–740, Mar 15, 2014

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