Table 6

Paediatric functional classification of pulmonary hypertension

Age functional class0–0.5 years0.5–1 years1–2 years2–5 years5–16 years
IAsymptomatic, growing along own centiles and developing normally, no limitation of physical activity
Gains head control and increases body tone from 0 to 3 months, then rolls over and has no head lag. Sitting with supportMobile, sitting, grasping, starting to stand, crawling, playingStanding, starting to walk/walking, climbingRegular school/nursery attendance, playing sports with his/her classmates
IISlight limitation of physical activity, unduly dyspnoeic and fatigued. Falling behind physical developmental milestones/delayed physical development. Comfortable at rest. Continues to grow along own centiles
Nursery/school attendance 75% normal. No chest pain
IIIaMarked limitation of physical activity, unduly fatigued. Regression of learned physical activities. Comfortable at rest. Less than ordinary activity causes undue fatigue or syncope and/or pre-syncope (or chest pain)
Quiet and needs frequent naps. Growth compromised. Poor appetite. Requires excessive medical attentionNursery/schooling compromised, <50% normal attendance
Hesitant and unadventurous.
Stops crawlingReluctant to playNot climbing stairs, reluctant to play with friendsNo attempt at sports
IIIbGrowth severely compromised. Poor appetite. Supplemental feeding. Less than ordinary activity causes undue fatigue or syncope (or chest pain). Plus features of Class IIIa
Unable to attend nursery/school, but mobile at home. Wheelchair needed outside home
IVUnable to carry out any physical activity without undue dyspnoea, fatigue or syncope (or chest pain), not interacting with family. Syncope and/or right heart failure. Plus features of class III
Unable to attend nursery/school, wheelchair dependant, not interacting with friends
  • Modified from Lammers et al.17