RT Journal Article
SR Electronic
T1 Late-stage left ventricular dysfunction in adult survivors of tetralogy of Fallot repair in childhood
JF Open Heart
JO Open Heart
FD British Cardiovascular Society
SP e000690
DO 10.1136/openhrt-2017-000690
VO 4
IS 2
A1 Abdel Anabtawi
A1 Judith Mondragon
A1 Diane Dodendorf
A1 Warren K Laskey
YR 2017
UL http://openheart.bmj.com/content/4/2/e000690.abstract
AB Objective Left ventricular systolic dysfunction (LVSD) in adult survivors of tetralogy of Fallot (TOF) repair in childhood has been observed, although the relationship with long-term outcome remains inadequately described.Methods A cohort of 44 consecutive adult patients with TOF repair in childhood were followed prospectively from January 2001 through June 2016. LVSD was defined as an echocardiographically derived left ventricular (LV) ejection fraction <0.55. Clinical and demographic characteristics in patients with and without LVSD were compared. Event-free survival (all-cause death or hospitalisation) was estimated using the product limit method.Results The average time from childhood surgical repair to cohort inception was similar between groups (LVSD, 33.7±12.7 years; normal LV function, 36.1±14.9 years; P=0.62) as were their mean ages (LVSD, 36.5±14.5 years; normal LV function, 40.7±15.2 years; P=0.73). Patients with LVSD (n=13) had more prior surgeries, more frequent history of significant pulmonic regurgitation, right ventricular systolic dysfunction and more implantable cardiac devices. Over a total observation time of 15.5 years, patients with LVSD were at significantly higher risk of all-cause death or hospitalisation (P=0.006). Onset of LVSD frequently preceded an adverse outcome.Conclusions In this cohort of adult patients with TOF repair in childhood followed for a total of 550 patient-years, the frequency of LVSD was 30%. LVSD was associated with lower event-free survival. The appearance of LVSD many years after TOF repair may herald the onset of an adverse outcome.