Figures
- FIGURE 1
Overview of palliative care in patients with end-stage pulmonary artery hypertension.
- FIGURE 2
Structure of the palliative care team. PAH: pulmonary arterial hypertension.
Tables
- TABLE 1
Studies assessing the quality of life (QoL) in patients with pulmonary arterial hypertension (PAH)
First author [reference] Sample size Patient characteristics Tools used to assess QoL Domains measured Results Comments 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 phase Questionnaire 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=55 One-third had IPAH, WHO FC II/III SF-36
SGRQSF-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 scoreSF-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 diseaseThese 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 scoresSwetz [11] Total n=276 42% with IPAH LASA QoL items
CAMPHORLASA assesses physical, spiritual, emotional, intellectual and overall wellbeing
CAMPHOR assesses energy, breathlessness, mood, total symptoms, function and QoLLASA: 40% patients had score ≤5 (0 being the worst and 10 the best)
CAMPHOR score centred around 50th percentile on each scale for QOLPatients with PAH have large symptom burden that affects QoL and persist even with PAH treatment Shafazand [3] Total n=53 IPAH, SSc-PAH and anorexigen-related PAH
72% in NYHA FC III or IVNHP questionnaire
CHQ
HADSNHP: physical mobility, pain, sleep, social isolation, emotional reactions and energy
CHQ: dyspnoea, fatigue, emotional function and mastery
HADS: screen for anxiety and depressionNHP: 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, respectivelyImpairment in multiple QoL domains, although the anxiety and depression scores were within the range of normal responses McCollister [10] Total n=100 50% had IPAH
38% had WHO FC II and 62% had FC IIIPHQ-8 PHQ-8: lack of interest, feeling of depression, energy, sleep, appetite, feeling of guilt, lack of concentration, psychomotor agitation or retardation PHQ-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 symptomsHigher 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 procedure Study design/sample size Patient characteristics Outcomes Sandoval [42] Atrial septostomy Retrospective
n=3485% had IPAH
9% had CTEPH
53% had WHO FC IV
41% had WHO FC III
11 patients received PAH-specific therapy after procedure1 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 septostomy Retrospective
n=32 patients
No control groupAll patients had PAH
63% had IPAH
78% had WHO FC III/IV
All patients were receiving PAH-specific therapyNo significant change in mPAP
No significant change in RAP
No significant change in BNPChen [44] PADN Prospective
n=21
13 underwent PADN
8 patients refused PADN and served as control groupAll patients had IPAH
13 underwent PADN
8 IPAH patients did not undergo PAH
All were on beraprost
Mean WHO FC 3.6Survival was not assessed
Decrease in mPAP and PVR post-PADN
Increase in cardiac output post-PADN
Improvement in 6MWD and WHO FCIPAH: 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.
