Pediatric Cardiology

Long-Term Fate of the Truncal Valve. Gellis L, Binney G, Alshawabkeh L, Lu M, Landzberg MJ, Mayer JE, Mullen MP, Valente AM, Sleeper LA, Brown DW.J Am Heart Assoc. 2020 Nov 17;9(22):e019104. doi: 10.1161/JAHA.120.019104. Epub 2020 Nov 9.PMID: 33161813 Free article.   Take Home Points: Independent risk factors for truncal valve interventions during long-term follow up are moderate or greater preoperative initial truncal valve regurgitation or stenosis, moderate or greater truncal valve regurgitation at discharge after initial full repair and a single coronary ostium. Moderate or greater truncal valve regurgitation is associated with larger truncal root z-scores at initial TA repair and during follow-up.   Commentary from Dr. Inga Voges (Kiel, Germany), section editor of Pediatric & Fetal Cardiology Journal Watch: This is a retrospective single-center study assessing risk factors for truncal valve intervention after complete repair for truncus arteriosus (TA). A large cohort of patients who underwent TA repair between 1985 and 2016 were included. The degree of truncal valve stenosis and regurgitation as well as truncal valve z-scores were recorded. Surgeries and re-interventions were documented. Early mortality (≤30 days postoperatively or before hospital   Discharge) and long-term outcomes were assessed. Primary outcomes were truncal valve intervention and mortality after discharge from initial full repair. Secondary outcomes were defined as time to any surgical reintervention and at least moderate truncal valve regurgitation.   Out of 170 patients, 22 patients died early (early mortality rate 13%). The residual 148 patients were defined as the long-term cohort (characteristics are displayed in Table 1). Median follow-up time after discharge from repair was 12.6 years. 19% of them died or underwent cardiac transplantation. 45 patients had a quadricuspid truncal valve and 73 patients had more than trivial truncal regurgitation before initial repair (Table 1).   11 patients of the long-term cohort had concomitant truncal valve repair at initial TA repair. 30 patients underwent at least one surgical intervention on the truncal valve during follow-up, 24 of them were from the group without truncal repair at initial surgery. Overall, 50 interventions on the truncal valve during follow up were performed. The cumulative incidence of any truncal valve intervention by 1, 5, 10, and 20 years was 0.7%, 5.1%, 15.6%, and 25.6%, respectively (Figure 2). The cumulative incidence of truncal valve repair and truncal valve replacement was 12.3% and 3.3%.   The following independent risk factors for truncal valve intervention were identified: moderate or greater preoperative initial truncal valve regurgitation or stenosis, moderate or greater truncal valve regurgitation at initial hospital discharge after full repair and a single coronary ostium (Table 2). The development of moderate or greater truncal valve regurgitation was associated with larger truncal root z-scores at initial TA repair and during follow-up.   In summary, this study demonstrates that truncal valve intervention during follow-up is common and that patients need a careful life-long follow up in specialized cardiac centers.          

Survival in Children With Congenital Heart Disease: Have We Reached a Peak at 97%? Mandalenakis Z, Giang KW, Eriksson P, Liden H, Synnergren M, Wåhlander H, Fedchenko M, Rosengren A, Dellborg M.J Am Heart Assoc. 2020 Nov 17;9(22):e017704. doi: 10.1161/JAHA.120.017704. Epub 2020 Nov 6.PMID: 33153356 Free article.   Take Home Points: There is a significant improvement in survival for patients with congenital heart disease (CHD) in Sweden born between 1980 and 2009. Survival, however, did not further increase since the turn of the last century. Improved therapeutic and diagnostic strategies might have contributed to an increased mortality in CHD patients.   Commentary from Dr. Inga Voges (Kiel, Germany), section editor of Pediatric & Fetal Cardiology Journal Watch: The results from this Swedish registry study raise an important question summarized in the study title: Is there no further improvement in survival of congenital heart disease (CHD) patients over the last decade?   The authors used three different Swedish national registries to identify CHD patients born between 1st January 1980 and 31st December 2017. Patients were categorized into 6 different lesion groups (lesion group 1: conotruncal defects; lesion group 2: non-conotruncal defects; lesion group 3: aortic coarctation; lesion group 4: ventricular septal defect; lesion group 5: atrial septal defect, lesion group 6: all other CHD patients not included in group 1-5).   64,396 CHD patients and 639,012 matched controls were identified (Table 1). As expected, the mortality in the CHD group was higher compared to the group of matched controls and this was found for all lesion groups (Table 2). Mortality was highest in the group of patients born between 1980 and 1989. The Risk of mortality decreased with increasing age in CHD patients.   Survival was better in the birth period 1990-1999 compared to the period 1980-1989 as well as in the period 2000-2010 compared to the period 1990-1999. No further improvement in survival was found for patients born between 2010 and 2017 (Figure 1). Compared to matched controls mortality was higher in all birth periods. Cardiac interventions were found to have a beneficial impact on survival until the 2000s (Figure 2).   These data should encourage us not only continuing with the current high standard of care for patients with CHD, but also to make efforts in developing new diagnostic, therapeutic and educational strategies for a further improvement in survival and outcome of CHD patients.