Long-Term Transplant-Free Survival After Repair of Total Anomalous Pulmonary Venous Connection

Abstract

Transplant-free survival after TAPVC repair is excellent, with most deaths or transplant events occurring early. Factors associated with the worst long-term outcomes included complex TAPVC, mixed TAPVC, and prolonged postoperative length of stay.

Total anomalous pulmonary venous drainage repair: redefining the long-term expectations

“…This statement appears to be consistent for patients up to 30 years after initial repair. However, we are reminded that the majority of their cohort were of the age of 18 years at last follow-up (15). In our own experience of 214 infants and neonates with isolated TAPVD operated between 1973 and 2014, we similarly found most late deaths (80%) occurred within 1 year after repair (7).…”

“…Recent studies have questioned the assertions that patients with 'simple' CHD have a normal life expectancy (16). Hence it is a reassuring finding that repair of isolated TAPVD has an excellent long-term survival approaching that of the general population (15). This statement appears to be consistent for patients up to 30 years after initial repair.…”

“…This may be due to the small numbers of cases amongst most centres and the universally poor early and late outcomes associated with TAPVD repair in patients with a univentricular circulation (11,12,18). The study by St Louis et al (15) attempts to shed light on this difficult subgroup of TAPVD patients with complex CHD. It appears that although this group (n=126) has a prolonged attrition of up to 4 years after surgery compared to isolated TAPVD patients, long-term transplant-free survival thereafter is excellent.…”

“…In this context, we read with interest a recent paper by St Louis et al (15), which provides long-term populationbased outcomes of TAPVD patients after repair. This study is unique as it combines a large surgical database (Paediatric Cardiac Care Consortium) with a national registry that records death and transplant events within the United States in order to determine follow-up into adulthood.…”

Assessing the recently noted surgical outcome of isolated total anomalous pulmonary venous connection repair: A single‐secondary center experience

“…Total anomalous pulmonary venous connection (TAPVC) is congenital heart disease (CHD), accounting for approximately 1% to 1.5% of all CHDs . It is sometimes associated with a univentricular heart or heterotaxy syndrome, both of which are consequently associated with high morbidity and mortality rates . In contrast, isolated TAPVC is known to have a good prognosis .…”