Abstract
To assess the spectrum of associated anomalies, the intrauterine course, postnatal outcome and management of fetuses with truncus arteriosus communis (TAC) METHODS: All cases of TAC diagnosed prenatally over a period of 8years were retrospectively collected in two tertiary referral centers. All additional prenatal findings were assessed and correlated with the outcome. The accuracy of prenatal diagnosis was assessed. 39 cases of TAC were diagnosed prenatally. Mean gestational age at first diagnosis was 22weeks (range, 13-38). Two cases were lost follow-up. Correct prenatal diagnosis of TAC was made in 21 of 24 (87.5%) cases and of TAC subtype in 19 of 21 (90.5%) cases. Prenatal diagnosis of TAC was incorrect in three cases: one newborn had aortic atresia with ventricular septal defect postnatally, one had hypoplastic right ventricle with dextro Transposition of the Great Arteries with coartation of the aorta and a third newborn had Tetralogy of Fallot with abnormal origin of the left pulmonary artery arising from the ascending aorta postnatally. These three cases were excluded from further analysis. In 9 of 34 (26.5%) cases, TAC was an isolated finding. 13 (38.2%) fetuses had additional chromosomal anomalies. Among them, microdeletion 22q11.2 was most common with a prevalence of 17.6% in our cohort. Another 3 fetuses were highly suspicious for non-chromosomal genetic syndromes due to their additional extra-cardiac anomalies, but molecular diagnosis could not be provided. Major cardiac and extra-cardiac anomalies occurred in 3 (8.8%) and in 20 (58.8%) cases, respectively. Predominantly, extra-cardiac anomalies occurred in association with chromosomal anomalies. Additionally, severe IUGR occurred in 6 (17.6%) cases. There were 14 terminations of pregnancy (41.2%), 1 (2.9%) intrauterine fetal death, 5 postnatal deaths (14.7%) and 14 (41.2%) infants were alive at last follow-up. Intention-to-treat survival rate was 70%. Mean follow-up among survivors was 42months (range, 6-104). Postoperative health status among survivors was excellent in 11 (78.6%) infants, but 5 (46.2%) of them needed repeated re-interventions due to recurrent pulmonary artery or conduit stenosis. The other 3 (21.4%) survivors were significantly impaired due to non-cardiac problems. TAC is a rare and complex cardiac anomaly that can be diagnosed prenatally with high precision. TAC is frequently associated with chromosomal and extra-cardiac anomalies, leading to a high intrauterine and postnatal loss rate due to terminations and perioperative mortality. Without severe extra-cardiac anomalies, postoperative short- and medium-term health status is excellent, independent of the subtype of TAC, but the prevalence of repeated interventions due to recurrent stenosis is high.
Highlights
Truncus arteriosus communis (TAC) is a rare conotruncal anomaly, representing 1.6% of all newborns with congenital heart disease [1] and 1.07 of 10.000 live births [2, 3]
While aorta and main pulmonary artery (MPA) originate from a common root, failure during the process of separation leads to a persistent common arterial trunk with a common truncal valve with four or more leaflets [7]
TAC is accompanied by a large ventricular septal defect (VSD) [9] with overriding of the large common arterial trunk
Summary
Truncus arteriosus communis (TAC) is a rare conotruncal anomaly, representing 1.6% of all newborns with congenital heart disease [1] and 1.07 of 10.000 live births [2, 3]. While aorta and main pulmonary artery (MPA) originate from a common root, failure during the process of separation leads to a persistent common arterial trunk with a common truncal valve with four or more leaflets [7]. This common truncal valve can either be stenotic or insufficient [8]. As Collet and Edwards classified the TAC exclusively according to the anatomic origin of the pulmonary arteries and to the spatial relationship between these vessels [10], Van Praagh proposed another anatomical classification which takes additional aortic arch anomalies into account [11]. As both classification systems have a substantial overlap, we reviewed both classification systems and grouped our cohort into three TAC types according to clinical and surgical aspects and based on both Collet and Edwards and Van Praagh classification
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