PAH diagnosis delayed for adult congenital heart disease patients

By Eleanor McDermid, Senior medwireNews Reporter

Pulmonary arterial hypertension (PAH) is often not diagnosed in adult patients with congenital heart disease (CHD) until 6 years or more after their symptoms first appear, research shows.

This is “of significant concern”, say David Celermajer (Royal Prince Alfred Hospital, Sydney, New South Wales, Australia) and co- researchers.

Their study of 360 patients with CHD and PAH from across Australia and New Zealand showed that patients first experienced symptoms that were potentially related to PAH at the age of 24.6 years, but were not diagnosed with the condition until about 6.5 years later, at an average age of 31.2 years.

This was despite their recognised CHD, which was diagnosed when they were 7.9 years old on average.

“These delays reflect a considerable question mark surrounding the current ‘transition’ from paediatric to adult care and warrants further investigation”, the researchers write in the Internal Medicine Journal.

They speculate that patients and community physicians may attribute early PAH symptoms to CHD, and suggest that “greater referral from general community specialist care to specialist care within adult CHD centres may improve diagnosis rates and specific treatment of PAH.”

Between March 2010 and July 2013, 30% of the patients were hospitalised, with each of these patients being hospitalised an average of 2.3 times. They were most often hospitalised for heart failure (21%); arrhythmias (18%); dizziness, syncope or seizures (12%); infection or sepsis (11%); and bleeding or thrombosis (8%). The 15-year mortality rate was 31%.

Nearly three-quarters of the patients received treatment with an endothelin receptor antagonist (ERA), and 41% received a phosphodiesterase type 5 inhibitor, mostly combined with an ERA. Their functional class improved, overall, with treatment, and 17% of those who improved or maintained their functional class died, compared with 26% of those who deteriorated.

Patients’ 6-minute walking distance also stabilised while on treatment, after a steady decline during the years before they started treatment.

“Notwithstanding these apparent improvements in functional status, PAH in association with CHD has significant morbidity”, say Celermajer and team.

“These data suggest the true burden of disease related to PAH and CHD requires further investigation.”

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