Table 1

Summary of studies evaluating the validity/reliability of eHealth for PAH

Country (year of publication)
Type of study/level of evidence (CEBM)/type of publicationStudy populationInterventionSummary of resultsSignificance level
Arelli et al
USA (2012)37
Case-series/level 4/CP18 patients with right heart catheterisation–confirmed PHA portable IC device with real-time wireless monitoring via a Bluetooth adapter to determine HR, CO, CI and SV during the 6MWTThe IC increased the value of 6MWT and provided insight into the haemodynamic changes during exercise in PHAll the comparisons at rest vs activity were statistically significant (p<0.001)
Biederman et al
USA (2015)28
Case-series study/level 4/CP10 patients with PAHLongitudinal CardioMEMS measuring of PAP, HR and CO and CMR at baseline and during pharmacological stress were performedCardiomems may contribute to providing an accurate calculation of non-invasive CO in PAHNR
Brooks et al
USA (2014, 2015)31 32
  1. Case-series study/level 4/CP

  2. Prospective study/level 1b/JP

CP: 52 participants half of whom had CHF and PH
JP: 103 participants with PH and CHF
SA-6MWT app for independent use at home
  1. 6MWTApp accurately predicted the distance walked during a 6MWT and

  2. It was easy to use and yielded accurate repeatable measurements in the clinic and at home

  1. Good correlation with measured distance, r=0.89 (95% CI 0.82 to 0.95). For those with PAH, the r=0.75 (95% CI, 0.54 to 0.96)

  2. Home-based walked distance (SA-6MWTApp) were highly repeatable (CV=4.6%) and correlated with in-clinic-measured distance (r=0.88; 95% CI 0.87 to 0.89)

Fox et al
Israel (2011, 2013)35 36
  1. Case-series study/level 4/JP

  2. Prospective cohort study with good follow-up (>80%)/level 1b/JP

  1. 44 patients with PAH

  2. 86 subjects (52 with PH)

Step oximetry system linked to a computer1. The step-oximetry test was an informative test of functional capacity among patients with PH.
2. Patients with PH showed significant limitation in step climbing ability that correlated with functional class and 6MWT
  1. Correlation between 6MWT and step climbing velocity r=0.66 (p<0.0001) and O2 desaturation during step climbing correlated with DLCO (r=−0.65, p=0.003)

  2. Correlation between exercise performance on the step and 6MWT-climb index (r=−0.77, p<0.0001); saturation deviation on the step test correlated with diffusion capacity of the lung (r=−0.49, p=0.001)

Fruhwald et al
Austria (2003)29
Case-series study/level 4/JP5 patients with PAH who had received long-term treatment with aerosolized IloprostIHM in patients who had received long-term treatment with IloprostThe IHM enabled reproducible measuring of drug-induced variations in PAPMean PAP from 68±13 to 49±11 mm Hg (mean time of 49±8 min). Thereafter, PAP returned to pre-inhalation levels
Gregorietti et al
Argentina (2015 and 2016)33 34
Case-series study/level 4/CP162 and 118 patients, respectively, with PHUtilisation of a DP during the 6MWT, and its possible association with other parameters of clinical and prognostic relevance in PHThe addition of a DP to the 6MWT provided valuable data for evaluation and follow-up of patients with PH
The number of footsteps was inversely correlated to clinical and laboratory parameters of prognostic relevance in PAH
In both studies, there were significant associations between pro-BNP levels and the number of footsteps (p=0.001) and WHO functional class
(p=0.003 and p=0.009, respectively)
Tonelli et al
USA (2013, 2014)38 39
Prospective cohort study with good follow-up (>80%)/level 1b/
  1. CP and

  2. JP

  1. 43 subjects (28 with PH and 15 healthy controls)

  2. 50 subjects, 20 of them with PH

A portable impedance cardiography with wireless monitoring via a Bluetooth to determine HR and CO during a 6MWT and HR acceleration and decay slopes during the 6MWT
  1. Wireless impedance cardiography showed lower distance walked, heart rate recovery (1 min) and CO change in PH under treatment than in healthy controls

  2. The HR acceleration and decay slopes during the 6MWT were different in PH, compared with other lung diseases and healthy controls

All the comparisons in HR curves and acceleration rates were statistically significant (p<0.001)
  • CEBM, Centre for Evidence-Based Medicine; CHF, congestive heart failure; CI, cardiac index; CMR, cardiovascular magnetic resonance; CO, cardiac output; CP, conference paper; CV, coefficient of variation; DLCO, diffusing capacity of the lungs for carbon monoxide; DP, digital pedometer; HR, heart rate; IC, impedance cardiography; IHM, implantable haemodynamic monitor; JP, journal paper; 6MWT, 6-minute walk test; 6MWTApp, 6-minute walk test app; NR, not reported; PAH, pulmonary arterial hypertension; PAP, positive airway pressure; PH, pulmonary hypertension; PVR, pulmonary vascular resistance; SV, stroke volume.