Late risk of outcomes for adults with repaired tetralogy of Fallot from an inception cohort spanning four decades☆

Abstract

Surgical progress has not influenced late risks for death, re-operation or PVR in adults with repaired tetralogy of Fallot. Instead, reduction of early surgical mortality to <2% is responsible for excellent late survival >90% overall. The constant risk of PVR is low and independent of repair type. Baseline morphologic features are important determinants of late outcome.

Patterns of cardiac and extracardiac anomalies in adults with tetralogy of Fallot

“…15 Others have found that individuals with genetic changes such as 22q11.2 deletions16 may have more complex cardiac lesions. The higher prevalence of extracardiac anomalies in subjects with pulmonary atresia and/or MAPCAs compared with those without these features may suggest pleiotropic expression, with changes in genes involved in very early development, like the TBX1 transcription factor of 22q11DS.…”

When ‘blue babies’ grow up: What you need to know about tetralogy of Fallot

“…[1][2][3][4] More than 85% of infants with congenital heart disease are now expected to reach adulthood. 5,6 For those with tetralogy of Fallot, the most common form of cyanotic congenital heart disease, the 40-year survival rate is now at least 90%. 5 But these former "blue babies" eventually have serious problems.…”

“…5,6 For those with tetralogy of Fallot, the most common form of cyanotic congenital heart disease, the 40-year survival rate is now at least 90%. 5 But these former "blue babies" eventually have serious problems. Most develop pulmonary valve insufficiency (regurgitation), which, over time, can result in right ventricular volume overload, enlargement, and dysfunction.…”

Early surgical outcome of pulmonary valve replacement in patients with previous right ventricle outflow tract reconstruction

“…[1,2] Despite these improvements in TOF surgery, however, reoperation is inevitable in nearly 50% of survivors in the following three decades after the initial procedure. [3] The most frequent reoperation is the pulmonary valve replacement (PVR) which is performed for chronic pulmonary valve insufficiency. [3] In this article, we present our early surgical outcomes of PVR in patients who underwent right ventricle outflow tract reconstruction (RVOTR).…”

“…[3] The most frequent reoperation is the pulmonary valve replacement (PVR) which is performed for chronic pulmonary valve insufficiency. [3] In this article, we present our early surgical outcomes of PVR in patients who underwent right ventricle outflow tract reconstruction (RVOTR).…”