Pulmonary Hypertension
The importance of accurate diagnosis
Alexandra A Frogoudaki
Adult Congenital Heart Clinic
Second Cardiology Department
ATTIKON University Hospital
• Honoraria from Actelion
Diagnosis
• Is there any pulmonary hypertension?
• In which group can be classified?
Is there any pulmonary
hypertension?
• Symptoms
21% of patients with iPAH had symptoms 2
years before diagnosis
Shortness of breath during exercise,
presyncope/syncope, lower extremity
oedema, fluid retention
• Echo
First line assessment for diagnosis with
prognostic value
Lai et al Circ Res 2014
Definition
ESC Guidelines 2009
Updated Definition
• PVR >3 WU is used as part of the
hemodynamic definition of PAH
• Patients with PAH are characterized by precapillary PH
• PAPm >25 mm Hg, PAWP <15 mm Hg and
elevated PVR [>3 WU]
Hoeper et al JACC 2013
Natural History of Pulmonary
Hypertension
Lai et al Circ Res 2014
Updated Classification
Simonneau et al JACC 2013
Hoeper et al JACC 2013
Right Heart Catheterization
• Pulmonary hypertension diagnosis is ONLY
confirmed with Right Heart Catheterization
• Fluid challenge may be needed in order to
exclude PH due to left heart disease
• Pulmonary vasoreactivity testing is only
recommended for patients with iPAH
Hoeper et al JACC 2013
Diagnostic Work-up
Lai et al Circ Res 2014
What happens with borderline PH?
Zamanian et al Circ Res 2014
Diagnosis as a guide to therapy
• Stabilization of heart disease is the treatment
of choice in group 2
• Patients with lung disease (group 3) do not
benefit from vasoactive agents
• Therapy of choice in chronic thromboembolic
disease PAH is endarterectomy
• Group V: according to the underlying
condition
Therapeutic Pathways
Lai et al Circ Res 2014
2013
• Macitentan (SERAPHIN)
• Riociguat (PATENT)
Selexipag decreased the risk of a morbidity/mortality event
versus placebo by 39% (p<0.0001).
Efficacy observed was consistent across the key subgroups; age,
gender, WHO Functional Class, PAH etiology and background
PAH therapy.
1156 patients were treated for up to 4.3 years.
The overall tolerability profile of selexipag in GRIPHON was
consistent with prostacyclin therapies
Updated Treatment of
Pulmonary Arterial Hypertension
Galie et al JACC 2013
Evidence-Based Treatment Algorithm
Initial therapy with PAH approved drugs
Galie et al JACC 2013
Galie et al JACC 2013
Diagnosis as a prognostic index
• PAH associated with connective tissue
disease has a worse prognosis than idiopathic
PAH even when treated with prostanoids,
while patients with PAH associated with
congenital heart disease have a better
survival.
• The worst prognosis is seen in patients with
pulmonary veno-occlusive disease and
pulmonary capillary hemangiomatosis
because of the lack of effective medical
Galie et al JACC 2013
treatments
Determinants of prognosis in Pulmonary Arterial
Hypertension
Galie et al Eur Heart J 2009
Take home messages
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Early recognition of symptoms
Predisposing conditions for PH, family history
Refer for echo
According to echo results diagnostic workup
Meticulous analysis of the results, accurate
classification based on RHC
• Treatment
• Prognosis