RESUMO
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.
RESUMO
BACKGROUND: Patients with angina and coronary microvascular dysfunction, without evidence of structural or epicardial coronary disease (Type I CMVD) remain without evidence based treatment options. Previous work has demonstrated that ranolazine can improve angina frequency and stability among patients with Type 1 CMVD; however, the mechanism remains unclear. Therefore, the objective of this pilot project was to assess the impact of ranolazine on Type I CMVD as measured using an invasive tool to measure global resistance (index of microcirculatory resistance (IMR)). METHODS: Patients with Type 1 CMVD diagnosed using IMR were enrolled and treated with ranolazine 1000mg BID. Coronary angiography and IMR were performed at baseline and on treatment after four weeks. The primary outcome measure was change in IMR pre- and post-treatment. Secondary outcome measures, improvement in angina and activity level, were assessed using the Seattle Angina Questionnaire (SAQ), Duke Activity Status Index (DASI) and Metabolic equivalent for Task (MET) scores. RESULTS: A total of 7 patient were enrolled and completed the study. Mean age was 57.6±7.5, 43% were female and 43% were Hispanic. Mean baseline IMR was 37.25±16.27 which decreased to 19.48±5.69 (p=0.02; (-48% Δ) after treatment with ranolazine. Four of the five SAQ domains improved on treatment with significant improvement in physical limitation (p=0.001), angina frequency (p=0.04), angina stability (p=0.05) and disease perception (p=0.001). Non-significant improvements in activity were also seen in both the DASI and MET scores. CONCLUSION: Among patients with Type 1 CMVD, our pilot data suggest favorable changes in IMR, anginal symptoms and activity status with ranolazine treatment. These findings support further evaluation of the effects of ranolazine on microcirculatory function and angina symptoms in a larger cohort of patients with Type 1 CMVD.
