Poorer Right Ventricular Systolic Function and Exercise Capacity in Women After Repair of Tetralogy of Fallot

Abstract

S ex differences in clinical presentation, procedural complications, and long-term outcomes are well known for acquired heart disease such as coronary artery disease or nonischemic cardiomyopathy. [1][2][3] There is evolving evidence that sex also influences the outcome in a range of congenital heart defects, as documented in recent registry studies. [4][5][6][7] However, although likely to be of clinical importance in the differential clinical management of female and male patients, it remains challenging to clearly demonstrate any sex effect for specific congenital heart lesions such as tetralogy of Fallot (ToF), the most common cyanotic heart defect. 8 The reasons are the heterogeneity and variability of the initial cardiovascular morphology of the defect and significant variability in the timing and mode of interventions for palliation, repair, or reoperations. Furthermore, there are differences in surgical procedures and cardiopulmonary bypass techniques between Background-In repaired congenital heart disease, there is increasing evidence of sex differences in cardiac remodeling, but there is a lack of comparable data for specific congenital heart defects such as in repaired tetralogy of Fallot.

Increased left ventricular myocardial extracellular volume is associated with longer cardiopulmonary bypass times, biventricular enlargement and reduced exercise tolerance in children after repair of Tetralogy of Fallot

“…Girls after TOF repair expressed higher markers of fibrosis than boys, which has been shown in adult TOF patients [15, 16]. It is interesting to note that girls also had larger ventricles, lower ejection fractions and weaker exercise tolerance, corroborating previous reports [23, 24]. Together, these data suggest a more rapid deterioration of cardiovascular health in female TOF patients.…”

Sex differences in patients with repaired tetralogy of Fallot support a tailored approach for males and females: a cardiac magnetic resonance study

“…Taken together, functional adaptation of the RV is impaired in male rTOF patients, when compared with female patients with similar age, surgical history, RV loading conditions and similar degrees of RV dilatation and RV hypertrophy. These findings seem to be in striking contrast with those of Sarikouch et al who also found lower RVEF in males with rTOF compared to females, but after normalization for sex, reported a lower Z-score of RVEF in females compared to males [22]. This is remarkable and difficult to understand, since the cohort of this study, and that of Sarikouch et al show very similar mean RVEF values, and use the same healthy reference cohort for Z-score calculation.…”

Clinical recommendations for cardiovascular magnetic resonance mapping of T1, T2, T2* and extracellular volume: A consensus statement by the Society for Cardiovascular Magnetic Resonance (SCMR) endorsed by the European Association for Cardiovascular Imaging (EACVI)

“…Nearly all CMR T1 mapping studies in Tetralogy of Fallot have demonstrated higher ECV and/or native T1 values in females [167]. This finding together with worse RV function and exercise tolerance [170] suggests that we may need to monitor and treat females differently from males after Tetralogy of Fallot repair.…”