Challenges and management issues in adults with cyanotic congenital heart disease

The case scenario presented above occurred 60 years ago. At the time the patient presented to Professor Paul Wood, whose seminal observation largely defined the Eisenmenger physiology.¹⁵ She was treated mostly with careful observation and given little more than cough suppressants and gentle oxygen supplementation in the short term. She received neither phlebotomy nor blood products. Her haemoptysis stabilised. Despite the risks of pregnancy, she fortunately carried the baby to term under careful observation and delivered vaginally. She was thereafter anticoagulated, and experienced no further haemoptysis. She died 50 years later at the age of 73 with her son at her side. Though her story is remarkable, it reminds us that cyanosis is not incompatible with meaningful life, and that physiological adaptations to cyanosis are more complicated than our current understanding of them. Although survivors of cyanotic heart disease are susceptible to multiorgan system complications, many can do well if important physiological compensations are left in balance.