Quality of Life for Historic Cavopulmonary Shunt Survivors

Beginning with Dr William Glenn in 1958, 90 patients with congenital heart lesions underwent cavopulmonary (Glenn) shunts over a 30-year period. In 2015, the follow-up data on this original cohort were reported. The study focuses on the current quality of life of this cohort.

Of the original 91 cavopulmonary shunt survivors, 14 (ages: 26-59; average length of postsurgical follow-up: 38.4 years) completed the Rand 36-Item Short-Form Health Survey, volume 2 (SF-36v2) quality-of-life survey at clinical office visits, over the phone or via regular mail. Diagnoses included tricuspid atresia (n = 9), double inlet left ventricle (n = 1), d-transposition of great arteries (n = 1), Ebstein's anomaly (n = 1), tetralogy of Fallot (n = 1), and pulmonary atresia with hypoplastic right ventricle (n = 1). Norm-based comparison of cavopulmonary shunt survivors to the general population was performed using a one-sample t test.

The 36-question health survey, SF-36v2, provided physical and mental health summary measures standardized to achieve a mean of 50. The 14 survivors' physical composite score was 47.18 (standard deviation [SD]: 8.24; P = .22) and mental composite score was 52.71 (SD: 5.64; P = .095).

The cavopulmonary shunt is now a widely used surgical palliation for single-ventricle patients and can be associated with physical and mental health outcomes similar to the general US population. Cavopulmonary shunt survivors' SF-36v2 survey results do not demonstrate a statistically significant difference from the general US population in physical or mental health measures. The results of this study contribute to the discussion of quality of life for patients with congenital heart disease by following up with the oldest known survivors.