Key result
Conservative management without PAH therapy maintains 11-year clinical stability in Down and Eisenmenger syndromes.
Why the study?
Management of pulmonary arterial hypertension associated with congenital heart disease and trisomy 21 is complex due to uncertainty over best first-line therapy, response outcome measures, and ethical decision-making.
Case Report (n=1)
No
Management of pulmonary arterial hypertension in patients with Down syndrome and congenital heart disease requires careful consideration of therapeutic options and psychosocial factors.
May warrant individualized conservative management without PAH therapy in select stable Eisenmenger cases with Down syndrome; leaves open optimal therapy selection.
Background Management of patients with pulmonary arterial hypertension associated with congenital heart disease and trisomy 21 is complex due to uncertainty over the best first-line agent to use for treatment, and the outcome measures for assessing response to therapy. There are also ethical issues around therapeutic decisions which may require ‘best interests’ meetings of carers and health care professionals. Case presentation We present a case of a 35-year-old man with Down syndrome, with un-operated atrioventricular septal defect, right ventricular hypertrophy and moderate left atrioventricular valve regurgitation. In the clinic he was visibly cyanosed with peripheral oxygen saturations of 78% at rest. He had very limited walk distance and was in WHO class III. Conclusions We discuss the decisions made around the best therapy for his Eisenmenger syndrome taking into account patient and family views on therapy and considering the psychosocial burden of treatment.
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Rashid et al. (2020) conducted a case report in Pulmonary arterial hypertension and congenital heart disease in Down syndrome (n=1). Conservative management was evaluated on Clinical stability. Conservative management without pulmonary arterial hypertension therapy resulted in 11 years of clinical stability in a man with Down syndrome and Eisenmenger syndrome.
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