Aorto–Left Ventricular Tunnel Successfully Repaired Immediately After Birth

Abstract

We describe the unusual case of a patient with an antenatal aorto-left ventricular tunnel (ALVT) diagnosis and severe left ventricular (LV) dysfunction who underwent successful repair immediately after birth. To the best of our knowledge, no such case has been reported in the English literature. Our case demonstrated that neonates at the worst end of the ALVT spectrum can survive and achieve normalization of LV function through a timely and multidisciplinary approach.

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 -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 .…”

“…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. 6,28,31,32,[34][35][36] Our finding that stable non-hydropic fetuses with ALVT, despite the impressive cardiomegaly and impaired contractility, generally survive to term with good clinical outcome, is essential for prenatal counseling; it is important that the parents are aware that the prognosis in those who survive without hydrops to near term is better than prognosis if there is evolving hydrops in mid-gestation before they make decisions regarding the pregnancy. 8 We encountered a strong relationship between abnormal peripheral Doppler measurements and perinatal death in fetuses with ALVT.…”

A case of a large type IV aortico–left ventricular tunnel successfully repaired at 1 day after birth

“…The reported maximum diameter of the ALVT in the cases of surgery in the neonatal period was 7 mm in the literature. 3 The larger the ALVT, the more unlikely it to survive. The diameter of ALVT was 10 mm in the present case, a far larger than the aortic valve diameter.…”

Congenital aortic paravalvular tunnel masquerading as a recurrent ventricular septal defect

“…There have only been two reports of ALVT associated with VSD, yet neither had spontaneous closure of the VSD by the time of surgery . Other reports of ALVT originating between the right coronary cusp and non‐coronary cusp had no associated VSD . Maldevelopment of the endocardial cushions has been reported as a possible etiology of ALVT with potential involvement of the proximal coronary arteries and both pulmonary and aortic valve leaflets.…”