Purpose Patients with Pulmonary Arterial Hypertension associated with Congenital Heart Disease (PAH-CHD) are subdivided into 4 categories:
PAH with systemic–to–pulmonary shunts,
PAH with coincidental defects,
PAH after defect correction.
The purpose of this study was to classify all patients on our database with PAH-CHD. The poster contains 3 case studies of patients with Trisomy 21 who were reassessed given this classification, and subsequent learning points are emphasised.
Methods The NORPAP database was created in 1993 and contains 2854 patients. Patients with PAH-CHD were extracted from the NORPAP database and the above classification system applied.
Results 98/2854 (3.4%) of patients had PAH-CHD. Trisomy 21 in 40 patients. Underlying condition shown in figure 1, classification of PAH-CHD in figure 2. 55/98 (56%) are on targeted therapy. Pre-treatment 6 min walk mean 288.5 m, post-treatment mean 320.6 m. Paired two sample t-test analysis reveals a significant difference in 6 min walk test (t-stat 2.02, p 0.01).
Conclusion Targeted therapy in PAH-CHD improves quality of life, delays time to deterioration and may have a mortality benefit. Our limited data supports the evidence that targeted therapy improves objective exercise tolerance. Adult follow up surveillance clinics must be alert to deterioration of functional class in Category 1; Trisomy 21 patients that have been labelled as PAH, but may have normal pulmonary pressure at right heart catheterisation; high index of suspicion with new symptoms in repaired CHD who develop PAH (Category 4).
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