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

Abstract

Introduction Aorto‐left ventricular tunnel (ALVT) accounts for <0.1% of congenital heart defects. Evidence on the prognosis from a fetal perspective is limited. With this retrospective international case series, we provide information on the outcome of fetuses with ALVT. Methods All members of the Association for European Pediatric and Congenital Cardiology's (AEPC) fetal working group and fetal medicine units worldwide were invited for participation. We observed antenatal parameters, neonatal outcome and postnatal follow‐up. Additionally, a systematic search of the literature was performed. Results Twenty fetuses with ALVT were identified in 10 participating centers (2001–2019). Fetal echocardiographic characteristics of ALVT included an increased cardiac–thorax ratio (95%), left ventricular end‐diastolic diameter (90%) and a dysplastic aortic valve (90%). Extracardiac malformations were rare (5%). Eight fetuses died at a median gestational age (GA) of 21 + 6 weeks (range, 19–24): all showed signs of hydrops prior to 24 weeks or at autopsy. All others (60%, 12/2) were live–born (median GA 38 + 4, range 37–40), underwent surgery and were alive at last follow up (median 3.2 years, range 0.1–17). The literature reported 22 ALVT fetuses with similar outcome. Conclusions In the absence of fetal hydrops, ALVT carries a good prognosis. Fetuses who survive to 24 weeks without hydrops are likely to have a good outcome.

Aorto-Left Ventricular Tunnel – Prenatal Diagnosis and Outcome

“…In all of the survivors (12/20) in the cohort of Nisselrooij et al 5 , surgical tunnel closure was possible. Therefore, a Ross procedure was not necessary in any of them.…”

“…The largest study on prenatal diagnosis and outcome of ALVT of 20 cases worldwide was published almost simultaneously to our publication 5 . Nisselrooij et al 5 confirmed the presence of hydrops and heart failure to be the strongest predictors for an adverse outcome.…”

“…However, the onset varies depending on the blood volume regurgitated over the tunnel and ranges from the prenatal period to adolescence [4]. ALVT belongs to the rarest prenatally detected cardiac defects [5][6][7][8][9][10][11][12][13][14][15][16]. The majority of these cases were detected by fetal echocardiography prompted by hydrops fetalis, but the precise diagnosis of ALVT could only be achieved based on postnatal echocardiography or autopsy.…”

“…The increased cardiac workload results in myocardial wall stress and remodeling, causing left ventricular hypertrophy and dilatation. Severe circulatory stress can cause hydrops and intrauterine demise becomes imminent.In all of the survivors (12/20) in the cohort of Nisselrooij et al[5], surgical tunnel closure was possible. Therefore, a Ross proce-…”

“…Severe circulatory stress can cause hydrops and intrauterine demise becomes imminent. In all of the survivors (12/20) in the cohort of Nisselrooij et al[5], surgical tunnel closure was possible. Therefore, a Ross proce-…”