Data on cardiogenic shock (CS) in adults with congenital heart disease (ACHD) are scarce. We sought to describe CS in ACHD in a nationwide CS registry.

From the multicentric Frenshock registry (n=772 CS from 49 French centers between April and October 2016), ACHD CS were compared with non-ACHD ones. The primary outcome was defined by mortality, chronic mechanical circulatory support (MCS) or heart transplantation at 1year.

Out of the 772 patients, 7 (1%) were ACHD. ACHD patients were younger (53.9 vs. 65.8-years-old), with less cardiovascular risk factor, such as hypertension (14.2 vs. 47.5%) and diabetes (14.3% vs. 36.1%), and no ischemic cardiopathy (0% vs. 61.5%). Right heart catheterization (57.1% vs. 15.4%), pacemaker (28.6 vs. 4.6%) and ICD (28.6 vs. 4.8%) were more frequently indicated in the ACHD CS management compared to non-ACHD CS, whereas temporary MCS (0 vs. 7.2%) and invasive mechanical ventilation (14.3% vs. 38.1%) were less likely used in ACHD.

At 1-year of follow-up, primary outcome occurred in 85.7% ACHD and 52.2% non ACHD (P=0.127). Left ventricular assist device (14.3 vs. 5.4%, P=0.3251), and heart transplantation (8.57 vs. 5.23%, P=0.05) were more frequently used in ACHD. However, 1-year mortality in ACHD and non-ACHD patients was the same (45.4 vs. 42.9%, P=1) (Fig. 1).

CS in ACHD is rare accounting for 1% of CS population. Despite a younger population with fewer cardiovascular risk factors, the prognosis remains severe.