Prenatal Diagnosis of Aorto-Left Ventricular Tunnel With Dysplastic Bicuspid Aortic Valve: From Fetal Cardiac Failure to Favorable Outcome

Abstract

Aorto-left ventricular tunnel (ALVT) is a rare congenital heart defect. Surgery has to be performed early to avoid life-threatening complications. Prenatal diagnosis of this defect is challenging. We report a case of ALVT diagnosed in a fetus showing premature severe cardiac failure at 24 GA. The new born was operated at day 3 of life with good results. Two years later, he is still doing well recovering a complete normal cardiac function. ALVT should be suggested in front of any fetal cardiac failure. Thanks to early diagnosis, prompt neonatal management can be organized and allows positive outcome.

The aorto‐left ventricular tunnel from a fetal perspective: Original case series and literature review

“…The age at surgery varied considerably amongst our original cases from a few hours after birth to 6 weeks of age. This is consistent with the literature, describing neonates that require surgery within the first days of life, 6 , 30 , 31 , 32 , 34 , 35 , 37 as well as those in which surgery could be delayed up to 3 months or even 1 year of age. 6 , 28 This reflects the heterogeneity in clinical conditions in ALVT patients, as well as changing attitudes regarding and expertise with neonatal surgical interventions in general.…”

“…The age at surgery varied considerably amongst our original cases from a few hours after birth to 6 weeks of age. This is consistent with the literature, describing neonates that require surgery within the first days of life, 6,[30][31][32]34,35,37 as well as those in which surgery could be delayed up to 3 months or even 1 year of age. 6,28 -275 median age of 3.2 years.…”

“…However, if fetuses did not develop hydrops, all successfully received corrective surgery and the majority are free of morbidity at 3.2 years of age. Presence or absence of hydrops was described in 15/22 ALVT cases reported in the literature 6,12,26,28,32,33,36,37 . Six cases had signs of hydrops with similar outcome compared to this series, as the majority resulted in fetal or postnatal demise (68%) 6,26,33 .…”

“…Presence or absence of hydrops was described in 15/22 ALVT cases reported in the literature. 6 , 12 , 26 , 28 , 32 , 33 , 36 , 37 Six cases had signs of hydrops with similar outcome compared to this series, as the majority resulted in fetal or postnatal demise (68%). 6 , 26 , 33 Similar to our experience, the literature review showed that in the absence of hydrops, fetal or neonatal mortality occurred in only 14% of cases.…”

“…These articles together reported on 22 fetuses prenatally diagnosed with an ALVT (1996–2021). 2 , 6 , 7 , 12 , 26 , 27 , 28 , 29 , 30 , 31 , 32 , 33 , 34 , 35 , 36 , 37 , 38 Patient characteristics and outcome of the reported fetuses are summarized in Table 4 . Overall, 65% (15/23) were alive and thriving at the end of the follow‐up period.…”