Pulmonary hypertension is a rare but serious condition in pediatric patients cause by several etiologies.
Objective: To describe our population with pulmonary hypertension (PH)
Methods: It is a retrospective and descriptive study. 60 patients(pts) with PH admitted to our Hospital between August 2000
and August 2019 were included. Persistent PH of the newborn syndrome and Group3 of Nice 2018 Classification were excluded, except PH associated to Developmental lung disorders. The analyzed variables were: gender, etiology, functional class at diagnosis (FC), presentation symptom, therapeutic scheme, transition to adult care centers, transplat and mortality.
-Gender: 53,33% female, but Idiopathic PH: 81,81% were female
According Nice 2018 Classification:
Group1: 41pts: 11pts Idiopathic PH, 1pts Familiar PH, 1pts Connective tissue disease, 24pts Congenital Heart Disease, 5pts Portal PH
Group2: 1 pt: Pulmonary vein stenosis
Group 3: 8 pts Bronchopulmonary dysplasia
Group5: 9pts: 1pt Pulmonary Histiocytosis, 1pt Sickle cell anaemia, 7pts Single ventricle.
-18% of patients had genetic syndrome (100% Down Syndrome)
- FC at diagnosis: I (1pt), II (22pts), III (26pts), IV (11pts)
- The most frequent presenting symptom at diagnosis was dyspnoea, only 1p was asymptomatic. Syncope(7p) all of them with idiopathic PH.
- Transition to adult care centers was successful in 8 patients.
- Transplant: 3 patients were listed. Only one underwent transplat others died in waiting list. 2 patients are being evaluated.
- Mortality: 11 pts (18,64%)
- Therapeutic scheme: Since 2007: PDE5i (18/60pts=28,33%); ERA (3/60pts=5%); PDE5i and ERA (20/60 pts = 33,3%); PDE5i + ERA + Prostanoids (13/60 pts = 21,66%); Calcium channel blocker (2/60 pts=3,33%); without treatment (4/60pts=6.66%)
1) Functional class at diagnosis show us a late diagnosis of PH
2) The. Most frequent etiology was PH associated with congenital heart disease
3) Combination therapy was the most frequent therapeutic scheme in the last 12 years