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Challenges in the diagnosis and treatment of pulmonary arterial hypertension

Jean-Luc Vachiéry, Sean Gaine
European Respiratory Review 2012 21: 313-320; DOI: 10.1183/09059180.00005412
Jean-Luc Vachiéry
*Pulmonary Vascular Diseases and Heart Failure Clinic, Cliniques Universitaires de Bruxelles, Hôpital Erasme, Université Libre de Bruxelles, Brussels, Belgium. #National Pulmonary Hypertension Unit, Mater Misericordiae University Hospital, Dublin, Ireland.
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  • For correspondence: jvachier@ulb.ac.be
Sean Gaine
*Pulmonary Vascular Diseases and Heart Failure Clinic, Cliniques Universitaires de Bruxelles, Hôpital Erasme, Université Libre de Bruxelles, Brussels, Belgium. #National Pulmonary Hypertension Unit, Mater Misericordiae University Hospital, Dublin, Ireland.
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  • Figure 1.
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    Figure 1.

    Patients in World Health Organization functional class III/IV in various registries. REVEAL: Registry to Evaluate Early and Long-term Pulmonary Arterial Hypertension Disease Management; REVEAL FCC: REVEAL French Comparison Cohort; NIH: National Institutes of Health; PHC: Pulmonary Hypertension Connection; FrR: French registry. REVEAL NIH: REVEAL NIH comparison cohort. Reproduced from [7] with permission from the publisher.

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    Figure 2.

    Diagnostic algorithm. PH: pulmonary hypertension; ECG: electrocardiogram; PFT: pulmonary function tests; HRCT: high-resolution computed tomography; V′/Q′: ventilation/perfusion; CTEPH: chronic thromboembolic PH; PVOD: pulmonary veno-occlusive disease; RHC: right heart catheterisation; PAH: pulmonary arterial hypertension. Reproduced from [2] with permission from the publisher.

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    Figure 3.

    Schematic diagram showing the ideal approach to pulmonary arterial hypertension management, which involves regular monitoring and early intervention. Reproduced from [26] with permission from the publisher.

Tables

  • Figures
  • Table 1. Clinical classification of pulmonary hypertension (PH)
    ClassificationSub-classification
    1. Pulmonary arterial hypertension1.1 Idiopathic
    1.2 Heritable
    1.3 Drugs and toxins induced
    1.4 Associated with (APAH)
    1.4.1 Connective tissue diseases
    1.4.2 HIV infection
    1.4.3 Portal hypertension
    1.4.4 Congenital heart disease
    1.4.5 Schistosomiasis
    1.4.6 Chronic haemolytic anaemia
    1.5 Persistent PH of the newborn
    1′. Pulmonary veno-occlusive disease and/or pulmonary capillary haemangiomatosis
    2. PH due to left heart disease2.1 Systolic dysfunction
    2.2 Diastolic dysfunction
    2.3 Valvular disease
    3. PH due to lung diseases and/or hypoxaemia3.1 Chronic obstructive pulmonary disease
    3.2 Interstitial lung disease
    3.3 Other pulmonary diseases with mixed restrictive and obstructive pattern
    3.4 Sleep-disordered breathing
    3.5 Alveolar hypoventilation disorders
    3.6 Chronic exposure to high altitude
    3.7 Developmental abnormalities
    4. Chronic thromboembolic PH
    5. PH with unclear and/or multifactorial mechanisms5.1 Haematological disorders: myeloproliferative disorders and splenectomy
    5.2 Systemic disorders: sarcoidosis, pulmonary Langerhans’ cell histiocytosis, lymphangioleiomyomatosis neurofibromatosis and vasculitis
    5.3 Metabolic disorders: glycogen storage disease, Gaucher disease and thyroid disorders
    5.4 Others: tumoural obstruction, fibrosing mediastinitis and chronic renal failure on dialysis
    • Reproduced from [2] with permission from the publisher.

  • Table 2. Patient’s age at diagnosis or during treatment in registry studies
    RegistryYearSubjectsAge yrs
    NIH [7]198518736.4±1.1; age at diagnosis
    REVEAL# [7]2011107244.9±0.6; age at diagnosis
    French registry [9]200667450±15; age at diagnosis
    REVEAL [10]2010252550.1±14.4; age at diagnosis
    UK National Audit¶ [22]2011316355.3; age of cohort
    • Data are presented as n, mean±sd or mean. REVEAL: Registry to Evaluate Early and Long-term Pulmonary Arterial Hypertension Disease Management. #: REVEAL patients matched to National Institute of Health (NIH) registry patients; ¶: patients being treated with pulmonary arterial hypertension-specific therapies.

  • Table 3. Variables with established importance for assessing disease severity, stability and prognosis in pulmonary arterial hypertension
    Determinants of prognosisBetter prognosisWorse prognosis
    Clinical evidence of RVFNoYes
    Rate of progression of symptomsSlowRapid
    SyncopeNoYes
    WHO-FCI, IIIV
    6MWTLonger (>500 m)Shorter (<300 m)
    Cardiopulmonary exercise testingPeak O2 consumption >15 mL·min−1·kg−1Peak O2 consumption <12 mL·min−1·kg−1
    BNP/NT-proBNP plasma levelsNormal or near normalVery elevated and rising
    Echocardiographic findingsNo pericardial effusionPericardial effusion
    TAPSE >2.0 cmTAPSE <1.5 cm
    HaemodynamicsPra <8 mmHg and CI ≥2.5 L·min−1·m−2Pra >15 mmHg or CI ≤2.0 L·min−1·m−2
    • RVF: right ventricle failure; WHO-FC: World Health Organization functional class; 6MWT: 6-min walk test; BNP: brain natriuretic peptide; NT-proBNP: N-terminal pro-BNP; TAPSE: tricuspid annular plane systolic excursion; Pra: right atrial pressure; CI: cardiac index. Reproduced from [2] with permission from the publisher.

  • Table 4. Clinical trials of combination therapy in pulmonary arterial hypertension (PAH)
    Trial [ref.]Background therapy/study drugDesignPatientsSubjects nDuration weeksPrimary end-pointEfficacy
    Primary end-pointTTCW
    BREATHE-2 [30]Epoprostenol/bosentanUpfront RCTIPAH, SSc, SLE3316TPR-ND
    STEP [31]Bosentan/iloprost (inhaled)Sequential RCTPAH67126MWD-+
    COMBI [32]Bosentan/iloprost (inhaled)Sequential open labelIPAH40126MWD--
    PACES [33]Epoprostenol/sildenafilSequential RCTIPAH, CTD, CHD277126MWD++
    TRIUMPH-1 [34]Sildenafil, bosentan or both/treprostinil (inhaled)Sequential RCTPAH235126MWD+-
    FREEDOM-C [35]Sildenafil, ERA or both/treprostinil (oral)Sequential RCTPAH354166MWD--
    IMPRES [36]≥2 PAH-specific therapies/imatinibSequential RCTIPAH, HPAH, CTD, CHD202246MWD+-
    PHIRST [37]Bosentan/tadalafilSequential RCTPAH216#166MWD-ND
    EARLY [23]Sildenafil/bosentanSequential RCTPAH28¶24PVR+ND
    • TTCW: time to clinical worsening; ERA: endothelin receptor antagonist; RCT: randomised controlled trial; IPAH: idiopathic PAH; SSc: systemic sclerosis; SLE: systemic lupus erythematosis; CTD: connective tissue disease; CHD: congenital heart disease; HPAH: heritable PAH; TPR: total pulmonary resistance; 6MWD: 6-min walk distance; PVR: pulmonary vascular resistance; ND: not determined; +: met end-point; -: did not meet end-point. #: only patients included in the subgroup analysis of patients who were receiving concomitant bosentan at baseline were included; ¶: only patients included in the subgroup analysis of patients who were receiving concomitant sildenafil at baseline were included.

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Challenges in the diagnosis and treatment of pulmonary arterial hypertension
Jean-Luc Vachiéry, Sean Gaine
European Respiratory Review Dec 2012, 21 (126) 313-320; DOI: 10.1183/09059180.00005412

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Challenges in the diagnosis and treatment of pulmonary arterial hypertension
Jean-Luc Vachiéry, Sean Gaine
European Respiratory Review Dec 2012, 21 (126) 313-320; DOI: 10.1183/09059180.00005412
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    • THE CHALLENGE OF DIAGNOSIS IN PAH
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