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REVEAL: a contemporary US pulmonary arterial hypertension registry

M.D. McGoon, D.P. Miller
European Respiratory Review 2012 21: 8-18; DOI: 10.1183/09059180.00008211
M.D. McGoon
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  • For correspondence: mmcgoon@mayo.edu
D.P. Miller
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  • Figure 1.
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    Figure 1.

    Number of previously (n=2,555) and newly (n=960) diagnosed pulmonary arterial hypertension patients enrolled in REVEAL by year of diagnosis.

  • Figure 2.
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    Figure 2.

    Inclusion characteristics of a) previously diagnosed and b) newly diagnosed pulmonary arterial hypertension (PAH) patients enrolled in REVEAL. PVR: pulmonary vascular resistance; P̄pa: mean pulmonary artery pressure; Ppcw: pulmonary capillary wedge pressure; LVEDP: left ventricular end-diastolic pressure.

  • Figure 3.
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    Figure 3.

    a) Pulmonary arterial hypertension (PAH) aetiological breakdown of REVEAL patients at enrolment. b) Breakdown of associated PAH subgroup. PPHN: persistent pulmonary hypertension of the newborn; PVOD: pulmonary veno-occlusive disease; PCH: pulmonary capillary haemangiomatosis; FPAH: familial PAH; APAH: associated PAH; IPAH: idiopathic PAH; CHD: congenital heart disease; CVD/CTD: collagen vascular disease/connective tissue disease. Reproduced from [15] with permission from the publisher.

  • Figure 4.
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    Figure 4.

    Pulmonary arterial hypertension specific medication use at enrolment among previously diagnosed patients. 184 (7%) of patients were not on a prostaglandin, phosphodiesterase type-5 inhibitor or endothelin receptor antagonist. Of these, 88 were on calcium channel blockers for the treatment of pulmonary arterial hypertension.

  • Figure 5.
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    Figure 5.

    1-yr survival estimates of 641 connective tissue disease-associated pulmonary arterial hypertension (CTD-APAH) patients compared with 1,251 idiopathic pulmonary arterial hypertension (IPAH) patients in REVEAL. Log rank p<0.0001. Reproduced from [22] with permission from the publisher.

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    Figure 6.

    Cox proportional hazard estimates for multivariate model of survival, limited to terms included in the final stepwise model. Parameters significantly associated with 1-yr survival include the Borg dyspnoea scale, right ventricular dysfunction, pulmonary vascular resistance (PVR) index, pulmonary capillary wedge pressure, cardiac index, mean pulmonary artery pressure and total serum bilirubin. Candidate predictor variables that were not significant at the univariable level included Tei index, vasoreactivity, race, newly diagnosed pulmonary arterial hypertension (PAH) and income. Missing Borg scale and missing PVR index were both associated with lower than average observed survival and were, therefore, considered candidate predictor variables. WHO: World Health Organization; CTD: connective tissue disease; APAH: associated PAH; PoPH: portal hypertension; FPAH: familial PAH; NYHA: New York Heart Association; 6MWD; 6-min walk distance; BNP: brain natriuretic protein; DL,CO: diffusing capacity of the lung for carbon monoxide; % pred: % predicted; RHC: right heart catheterisation; P̄ra: mean right atrial pressure. Data are presented as hazard ratio (HR) with 95% confidence level. #: reference category was NYHA/WHO functional class II or missing; ¶: if N-terminal proBNP is available and BNP is not, cut-off points are replaced with <300 pg·mL−1 and >1,500 pg·mL−1; +: restricted to tests performed within 1 yr of enrolment, otherwise, the indicator is set to 0. Reproduced from [27] with permission from the publisher.

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    Figure 7.

    Survival estimates of patients in REVEAL using Kaplan–Meier estimates stratified by newly versus previously diagnosed patients and survival estimated by a delayed entry model accounting for truncation. The model includes all REVEAL patients but the number of patients at risk is limited to the time period during which they were followed in the study. Therefore, the first 3 months is estimated exclusively based on newly diagnosed patients and thereafter combined data from previously diagnosed patients and long-term follow-up of newly diagnosed patients. #: newly diagnosed; ¶: previously diagnosed.

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    Figure 8.

    REVEAL pulmonary arterial hypertension (PAH) risk score. CTD: connective tissue disease; APAH: associated PAH; PoPH: portal hypertension; FPAH: familial PAH; WHO: World Health Organization; NYHA: New York Heart Association; 6MWD: 6-min walk distance; BNP: brain natriuretic peptide; PFT: pulmonary function test; RHC: right heart catheterisation; SBP: systolic blood pressure; HR: heart rate; DL,CO: diffusing capacity of the lung for carbon monoxide; % pred: % predicted; P̄ra: mean right atrial pressure; PVR: pulmonary vascular resistance. #: if N-terminal proBNP is available and BNP is not, cut-off points are replaced with <300 pg·mL−1 and >1,500 pg·mL−1. Reproduced from [31] with permission from the publisher.

Tables

  • Figures
  • Table 1. Diagnostic right heart catheterisation parameters of patients meeting traditional haemodynamic criteria in the REVEAL registry by World Health Organization (WHO) group I diagnosis at enrolment
    CharacteristicsAll patients#IPAHAll patients with APAH¶APAH subgroups+
    CHDCVD/CTDPortal hypertensionDrugs/
    toxins
    Subjects n252511661280250639136134
    P̄pa mmHg50.7±13.652.1±13.049.1±13.859.5±16.944.9±11.248.5±10.652.2±12.2
        Subjects n252511661280250639136134
        p-value<0.001ƒ
    Ppcw mmHg9.1±3.59.2±3.59.0±3.58.9±3.68.9±3.59.3±3.69.2±3.6
        Subjects n252511661280250639136134
        p-value0.14
    P̄ra mmHg9.3±5.69.9±5.78.6±5.57.2±4.58.7±5.68.3±5.910.7±5.9
        Subjects n229810501174229580131127
        p-value<0.001ƒ
    PVRI Wood units·m221.1±12.522.9±11.419.0±13.023.7±20.916.9±9.115.7±7.224.2±12.7
        Subjects n1868842965186488100100
        p-value<0.001ƒ
    Fick or thermodilution CI L·min−1·m2§2.4±0.82.2±0.82.5±0.92.7±1.02.5±0.82.8±0.82.1±0.8
        Subjects n1868842965186488100100
        p-value<0.001ƒ
    SV,O2 %62.9±10.061.8±9.864.2±10.167.3±9.563.3±10.066.6±8.561.1±9.7
        Subjects n14566657381483568696
        p-value<0.001ƒ
    • Data are presented as mean±sd, unless otherwise stated. PAH: pulmonary arterial hypertension; IPAH: idiopathic PAH; APAH: associated PAH; CHD: congenital heart disease; CVD/CTD: collagen vascular disease/connective tissue disease; P̄pa: mean pulmonary artery pressure; Ppcw: pulmonary capillary wedge pressure; P̄ra: mean right atrial pressure; PVRI: pulmonary vascular resistance index; CI: cardiac index; SV,O2: mixed venous oxygen saturation. #: all patients aged ≥19 yrs at diagnosis with a Ppcw ≤15 mmHg enrolled during the consecutive screening of newly and previously diagnosed patients, including those with WHO group I diagnoses other than IPAH or APAH (i.e. familial PAH, pulmonary veno-occulsive disease and persistent pulmonary hypertension of the newborn); ¶: all APAH patients, including those with associated PAH subgroups other than CHD, CVD/CTD, portal hypertension, and drugs/toxins (i.e. HIV and others); +: APAH subgroups are mutually exclusive according to the following hierarchy for patients with multiple associated-PAH diagnoses: CHD, CVD/CTD, portal hypertension, drugs/toxins, HIV and others; §: the Fick CI is used unless it is missing, in which case thermodilution CI is used; ƒ: p-value for all haemodynamic parameters is obtained from the two-sample t-test examining the difference in the distribution of the characteristics among patients diagnosed with IPAH versus all patients with APAH. Reproduced from [15] with permission from the publisher.

  • Table 2. Pulmonary arterial hypertension-specific medications among patients meeting traditional haemodynamic criteria in the REVEAL registry at enrolment
    ERA#PDE-5 inhibitor¶Prostacyclin analogue
    Intravenous epoprostenolInhaled iloprostTreprostinil+
    Overall use§1147 (47.0)1194 (49.0)480 (19.7)237 (9.7)307 (12.6)
    Monotherapy452 (18.5)417 (17.1)188 (7.7)23 (0.9)84 (3.4)
    Combination with one oral therapyƒ291 (11.9)290 (11.9)243 (10.0)138 (5.7)148 (6.1)
    Combination with one prostacyclin analogue224 (9.2)305 (12.5)2 (0.1)3 (0.1)5 (0.2)
    Combination with >1 other therapy180 (7.4)182 (7.5)47 (1.9)73 (3.0)70 (2.9)
    NYHA/WHO functional class I/II
        Overall use468 (47.1)474 (47.7)187 (18.8)71 (7.1)111 (11.2)
        Monotherapy216 (21.7)187 (18.8)83 (8.4)4 (0.4)26 (2.6)
        Combination with one oral therapyƒ110 (11.1)109 (11.0)85 (8.6)41 (4.1)60 (6.0)
        Combination with one prostacyclin analogue76 (7.6)110 (11.1)
        Combination with >1 other therapy66 (6.6)686 (6.8)19 (1.9)26 (2.6)25 (2.5)
    NYHA/WHO functional class III
        Overall use525 (47.7)567 (51.5)218 (19.8)130 (11.8)161 (14.6)
        Monotherapy181 (16.4)177 (16.1)80 (7.3)16 (1.5)43 (3.9)
        Combination with one oral therapyƒ147 (13.4)147 (13.4)117 (10.6)78 (7.1)79 (7.2)
        Combination with one prostacyclin analogue114 (10.4)160 (14.5)2 (0.2)2 (0.2)4 (0.4)
        Combination with >1 other therapy83 (7.5)83 (7.5)19 (1.7)34 (3.1)35 (3.2)
    NYHA/WHO functional class IV
        Overall use55 (44.4)61 (49.2)44 (35.5)14 (11.3)15 (12.1)
        Monotherapy5 (4.0)14 (11.3)17 (13.7)2 (16)5 (4.0)
        Combination with one oral therapyƒ16 (12.9)16 (12.9)21 (16.9)8 (6.5)4 (3.2)
        Combination with one prostacyclin analogue18 (14.5)15 (12.1)
        Combination with >1 other therapy16 (12.9)16 (12.9)6 (4.8)4 (3.2)6 (4.8)
    • Data are presented as n (%). Combinations with one oral therapy, with one prostacyclin analogue, and with more than one oral therapy are mutually exclusive and exclude calcium channel blockers. Blinded clinical trial patients are excluded from this presentation (n=87). ERA: endothelin receptor antagonist; PDE-5: phosphodiesterase type-5; NYHA/WHO: New York Heart Association/World Health Organization. #: 953 on bosentan, 106 on sitaxsentan and 89 on ambrisentan; ¶: 1,147 on sildenafil and 47 on tadalafil; +: treprostinil use includes 159 on intravenous, 112 on subcutaneous, 28 on inhaled and nine on oral treprostinil; §: n=2,435; ƒ: oral therapy is defined as bosentan, sildenafil, ambrisentan, sitaxsentan and tadalafil. Reproduced from [15] with permission from the publisher.

  • Table 3. Comparison of characteristics of patients aged ≥19 yrs at diagnosis meeting traditional haemodynamic characteristics of pulmonary arterial hypertension with those with a pulmonary capillary wedge pressure (Ppcw) of 16–18 mmHg
    Ppcw at diagnosis
    ≤15 mmHg16–18 mmHgp-value
    Subjects n2525239
    Age yrs
        At enrolment53.0±14.056.1±14.50.001
        At diagnosis50.1±14.453.6±14.9<0.001
    Female2007 (79.5)174 (72.8)0.016
    Time from diagnosis24.9 (8.0–50.9)20.2 (5.5–44.6)0.038
    6MWD m366±126339±1170.004
    P̄ra mmHg9.3±5.612.8±5.6<0.001
    PVRI Wood units·m221.1±12.519.1±13.00.052
    Hypertension980 (40.2)111 (47.6)0.023
    Obese#697 (33.3)85 (41.9)0.014
    Sleep apnoea484 (21.0)85 (39.9)<0.001
    Diabetes overall293 (12.0)47 (20.2)<0.001
    Renal insufficiency109 (4.5)25 (10.7)<0.001
    Cadiomyopathy dilated24 (1.0)4 (1.7)0.286
    Warfarin1302 (53.4)105 (45.1)0.016
    Oxygen982 (40.3)110 (47.2)0.036
    β-blocker296 (12.1)51 (21.9)<0.001
    • Data are presented as mean±sd, n (%) or median (interquartile range), unless otherwise stated. 6MWD: 6-min walk distance; P̄ra: mean right atrial pressure; PVRI: pulmonary vascular resistance index. #: body mass index ≥30 kg·m−2. Reproduced from [15] with permission from the publisher.

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REVEAL: a contemporary US pulmonary arterial hypertension registry
M.D. McGoon, D.P. Miller
European Respiratory Review Mar 2012, 21 (123) 8-18; DOI: 10.1183/09059180.00008211

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REVEAL: a contemporary US pulmonary arterial hypertension registry
M.D. McGoon, D.P. Miller
European Respiratory Review Mar 2012, 21 (123) 8-18; DOI: 10.1183/09059180.00008211
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  • Article
    • PATIENT ENROLMENT INTO REVEAL
    • OBJECTIVES OF REVEAL
    • DEMOGRAPHICS AND CLINICAL PROFILE OF THE REVEAL PATIENT POPULATION
    • COMPARISON WITH OTHER REGISTRIES
    • WHO GROUP I PAH SUBGROUPS
    • NON-TRADITIONAL HAEMODYNAMIC CRITERIA
    • CLINICAL PREDICTORS OF OUTCOME
    • INTERIM CONCLUSIONS FROM REVEAL
    • Acknowledgments
    • Footnotes
    • REFERENCES
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  • Pulmonary vascular disease
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  • The efficacy of bedside chest ultrasound
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