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Palliative care in pulmonary arterial hypertension: an underutilised treatment

Ghaleb Khirfan, Adriano R. Tonelli, Jennifer Ramsey, Sandeep Sahay
European Respiratory Review 2018 27: 180069; DOI: 10.1183/16000617.0069-2018
Ghaleb Khirfan
1Dept of Pulmonary, Allergy and Critical Care Medicine, Respiratory Institute, Cleveland Clinic, Cleveland, OH, USA
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Adriano R. Tonelli
1Dept of Pulmonary, Allergy and Critical Care Medicine, Respiratory Institute, Cleveland Clinic, Cleveland, OH, USA
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Jennifer Ramsey
2Dept of Critical Care Medicine, Respiratory Institute and Section of Palliative Medicine, Taussig Cancer Institute, Cleveland Clinic, Cleveland, OH, USA
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Sandeep Sahay
3Houston Methodist Lung Center, Division of Pulmonary Medicine, Houston Methodist Hospital, Houston, Texas
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    FIGURE 1

    Overview of palliative care in patients with end-stage pulmonary artery hypertension.

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    FIGURE 2

    Structure of the palliative care team. PAH: pulmonary arterial hypertension.

Tables

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  • TABLE 1

    Studies assessing the quality of life (QoL) in patients with pulmonary arterial hypertension (PAH)

    First author [reference]Sample sizePatient characteristicsTools used to assess QoLDomains measuredResultsComments
    McCollister [24]n=55 in three study phases: n=25 in phase 1, n=20 in phase 2 and n=10 in phase 3
    • All PAH, WHO FC I–IV

    Three-phase study: a concept-elicitation phase; second phase of two rounds of cognitive interviews; a final cognitive and usability interview phaseQuestionnaire assesses two domains: symptoms of PAH and impact of these symptoms on patients' lives
    • Development of the PAH-SYMPACT questionnaire, which was shown to capture symptoms and its impact

    Chin et al. [23] validated the PAH-SYMPACT as the first disease-specific patient-reported outcome instrument
    Taichman [4]Total n=155; completed n=55One-third had IPAH, WHO FC II/IIISF-36
    SGRQ
    SF-36 assesses physical function, bodily pain, general health, vitality and social, functional and mental health
    SGRQ assesses symptoms, activity and impacts (psychosocial), as well as total score
    SF-36: impaired QoL in every domain
    SGRQ: abnormally elevated scores (indicating a worse QoL) were seen in assessments of patient symptoms, activity and the impact of disease
    These scores indicate a poor physical function, mental health, increased body pain and decreased general and social wellbeing; all correlating with a lower QoL
    No correlation was observed between haemodynamic measurements and QoL scores
    Swetz [11]Total n=27642% with IPAHLASA QoL items
    CAMPHOR
    LASA assesses physical, spiritual, emotional, intellectual and overall wellbeing
    CAMPHOR assesses energy, breathlessness, mood, total symptoms, function and QoL
    LASA: 40% patients had score ≤5 (0 being the worst and 10 the best)
    CAMPHOR score centred around 50th percentile on each scale for QOL
    Patients with PAH have large symptom burden that affects QoL and persist even with PAH treatment
    Shafazand [3]Total n=53IPAH, SSc-PAH and anorexigen-related PAH
    72% in NYHA FC III or IV
    NHP questionnaire
    CHQ
    HADS
    NHP: physical mobility, pain, sleep, social isolation, emotional reactions and energy
    CHQ: dyspnoea, fatigue, emotional function and mastery
    HADS: screen for anxiety and depression
    NHP: moderate to severe impairment in all domains
    CHQ: moderate impairment in all domains assessed
    HADS: moderate or severe levels of anxiety and depression were reported by 20.5% and 7.5% of participants, respectively
    Impairment in multiple QoL domains, although the anxiety and depression scores were within the range of normal responses
    McCollister [10]Total n=10050% had IPAH
    38% had WHO FC II and 62% had FC III
    PHQ-8PHQ-8: lack of interest, feeling of depression, energy, sleep, appetite, feeling of guilt, lack of concentration, psychomotor agitation or retardationPHQ-8 scores showed that 15% of patients had score of ≥10, suggestive of major depression
    40% had score of between 4 and 9, corresponding to mild to moderate depressive symptoms
    Higher prevalence of depression disorders in PAH patients than those found in general population, patients with other medical disorders and patients with left heart failure

    WHO: World Health Organization; FC: functional class; PAH-SYMPACT: Pulmonary Arterial Hypertension-Symptoms and Impact; IPAH: idiopathic PAH; SF-36: 36-item Short Form Health Survey; SGRQ: St George's Respiratory Questionnaire; LASA: Linear Analogue Self-Assessment; CAMPHOR: Cambridge Pulmonary Hypertension Outcome Review; SSc: systemic sclerosis; NYHA: New York Heart Association; NHP: Nottingham Health Profile; CHQ: Congestive Heart Failure Questionnaire; HADS: Hospital Anxiety and Depression Scale; PHQ-8: Patient Health Questionnaire.

    • TABLE 2

      Outcomes of various palliative interventions in patients with pulmonary arterial hypertension (PAH)

      First author [reference]Palliative procedureStudy design/sample sizePatient characteristicsOutcomes
      Sandoval [42]Atrial septostomyRetrospective
      n=34
      85% had IPAH
      9% had CTEPH
      53% had WHO FC IV
      41% had WHO FC III
      11 patients received PAH-specific therapy after procedure
      • 1 patient died after the procedure due to refractory hypoxaemia

        Improved survival in those who received PAH-specific therapy after the procedure

        6MWD increased

        WHO FC improved

        mPAP and RVEDP decreased

        Cardiac index and LVEDP increased

      Chiu [46]Atrial septostomyRetrospective
      n=32 patients
      No control group
      All patients had PAH
      63% had IPAH
      78% had WHO FC III/IV
      All patients were receiving PAH-specific therapy
      No significant change in mPAP
      No significant change in RAP
      No significant change in BNP
      Chen [44]PADNProspective
      n=21
      13 underwent PADN
      8 patients refused PADN and served as control group
      All patients had IPAH
      13 underwent PADN
      8 IPAH patients did not undergo PAH
      All were on beraprost
      Mean WHO FC 3.6
      Survival was not assessed
      Decrease in mPAP and PVR post-PADN
      Increase in cardiac output post-PADN
      Improvement in 6MWD and WHO FC

      IPAH: idiopathic pulmonary arterial hypertension; CTEPH: chronic thromboembolic pulmonary hypertension; WHO: World Health Organization; FC: functional class; 6MWD: 6-min walking distance; mPAP: mean pulmonary artery pressure; RVEDP: right ventricular end-diastolic pressure; LVEDP: left ventricular end-diastolic pressure; RAP: right atrial pressure; BNP: brain natriuretic peptide; PADN: pulmonary artery denervation; PVR: pulmonary vascular resistance.

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      Palliative care in pulmonary arterial hypertension: an underutilised treatment
      Ghaleb Khirfan, Adriano R. Tonelli, Jennifer Ramsey, Sandeep Sahay
      European Respiratory Review Dec 2018, 27 (150) 180069; DOI: 10.1183/16000617.0069-2018

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      Palliative care in pulmonary arterial hypertension: an underutilised treatment
      Ghaleb Khirfan, Adriano R. Tonelli, Jennifer Ramsey, Sandeep Sahay
      European Respiratory Review Dec 2018, 27 (150) 180069; DOI: 10.1183/16000617.0069-2018
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      • Article
        • Abstract
        • Abstract
        • Introduction
        • Health-related quality of life in PAH
        • Palliative care in PAH
        • Palliative care in PAH versus other serious illnesses
        • Palliative therapies
        • Palliative care and lung transplant
        • Conclusion
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