CASE REPORT

Case Series of Antenatally Diagnosed Aortico-Left VentricularTunnel

Anju Singh • Zdenka Reinhardt • Tarak Desai •

Shree Vishna Rasiah

Received: 24 December 2010 / Accepted: 9 February 2011 / Published online: 5 March 2011

� Springer Science+Business Media, LLC 2011

Introduction

Aortico-left ventricular tunnel (ALVT), a rare congenital

anomaly was first reported in 1963 by Levy et al. [3]. It is

an extracardiac channel that connects the ascending aorta

above the sinutubular junction and the left ventricular

cavity. There might be associated cardiac anomalies like

valvular defects and abnormal coronary arteries. In the

past, this anomaly was diagnosed in the postnatal period.

With greater advances in fetal medicine, it is now possible

to diagnose this condition antenatally.

Case Series

We report four cases of antenatally diagnosed ALVT over

a period of 12 years in a regional cardiology center. Their

postnatal clinical management and follow-up is also

described (Tables 1, 2).

Three out of the four cases were males. The antenatal

diagnosis in all cases was between 20 and 23 weeks of

gestation. In one case, at 20 weeks there was an initial

concern of presence of perimembranous ventricular septal

defect (VSD) with a large aorta and dilated left ventricle.

The diagnosis of cardiomyopathy or fibroelastosis was

considered. Aortico-ventricular tunnel was diagnosed on

follow-up scans. The female fetus was the only one to

develop hydrops with a grossly dilated and poorly con-

tractile left ventricle (LV). She was delivered at 37 weeks

of gestation by an emergency cesarean section for fetal

distress. The other babies were delivered by spontaneous

normal vaginal delivery at term gestation.

In the postnatal period, ventilatory support was required

in the hydropic neonate. Another neonate (Case 1) required

50% head box oxygen (Figs. 1, 2). The echocardiogram

demonstrated moderate to severely impaired ventricular

function in three patients with mild to severe aortic

regurgitation in addition to the presence of the ALVT.

These patients were given circulatory support in the form

of inotropes to assist ventricular function in the immediate

postnatal and postoperative periods. Three patients under-

went bypass surgery and correction of the defect within

two days of life. One baby had ductus ligation and the

hydropic neonate had aortic valvotomy. The fourth neonate

was well at birth and after cardiac assessment was dis-

charged home on diuretics. He had associated bicuspid

aortic valve. After a year of follow-up he was electively

operated. The closure of the tunnel was done with a bovine

patch in three patients and with a pulmonary homograft in

one.

Postoperative complications in Case 3 included transient

electrocardiogram (ECG) changes due to kinking of a

coronary artery, which was managed conservatively.

At follow-up the hydropic baby continued to have very

poor left ventricular function, was ventilator dependent,

and eventually developed renal failure (Fig. 3). At 5 weeks

of age, she died after intensive care was withdrawn because

of multisystem failure, following consultation with parents.

The other three cases on follow-up were clinically not in

failure and there was improvement in their left ventricular

function on echocardiogram, with mild to moderate aortic

regurgitation in two infants. None of the patients showed

A. Singh (&) � S. V. Rasiah

Neonatal unit, Birmingham Women’s Hospital NHS Foundation

Trust, Birmingham, United Kingdom

e-mail: anju2270@doctors.org.uk

Z. Reinhardt � T. Desai

Cardiology, Birmingham Children’s Hospital NHS Foundation

Trust, Birmingham, United Kingdom

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Pediatr Cardiol (2011) 32:822–825

DOI 10.1007/s00246-011-9940-9

Fig. 1 Case 1, parasternal long-

axis view with the knuckle-

shaped tunnel to the right of the

left ventricular outflow tract

(LVOT)

Fig. 2 Case 1, apical five

chamber view with the ALVT to

the right of the LVOT

Table 1 Antenatal and postnatal demographic and clinical variables

Case 1 Case 2 Case 3 Case 4

Antenatal age at diagnosis (weeks) 22 20 20 23

Antenatal heart failure No Yes, with ascitis No No

Age at delivery (weeks) 38 37 40 40

Birth weight (g) 4130 2890 3290 3222

Sex Male Female Male Male

Mode of delivery Normal Emergency section Normal Normal

Postnatal ventilator support No Yes No No

Postnatal inotropic support Yes Yes Yes No

Pediatr Cardiol (2011) 32:822–825 823

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the presence of any residual shunt, although one infant had

the LV end of the tunnel still open at 12 years follow-up.

Discussion

Aortico-left ventricular tunnel is a rare defect and accounts

for 0.001% of all congenital cardiac defects [6]. The eti-

ology of ALVT is still unknown. Echocardiography is the

gold standard to diagnose this defect. Other diagnostic

modalities used are magnetic resonance imaging and car-

diac catheterization. Increasingly, this condition is being

diagnosed in the antenatal period. One should suspect this

condition in the presence of significant aortic regurgitation

in the fetus. The severity of the tunnel regurgitation can be

assessed by the presence of hydrops in a fetus, width of the

tunnel, presence of left ventricular dilatation, retrograde

flow in the descending aorta, and clinical evidence of

failure postnatally [2, 4–6]. Spontaneous closure of a small

and asymptomatic ALVT has been documented in the lit-

erature [4]. Early surgical management is recommended in

order to prevent development of severe aortic regurgitation.

Surgical closure involves the use of a patch or sutures.

There has been a report of successful transcatheter closure

of ALVT [1]. The main long-term complication is the

presence of valvular aortic regurgitation requiring valve

replacement in a few patients. There is a good long-term

outcome of ALVT after surgery. The outcome is dependent

on antenatal progress and early development of hydrops.

References

1. Chessa M, Chaudhari M, De Giovanni JV (2000) Aorto-left

ventricular tunnel: transcatheter closure using an Amplatzer duct

occluder device. Am J Cardiol 86:253–254

Table 2 Surgical outcomes and follow-up

Case 1 Case 2 Case 3 Case 4

Age at surgery 1 day 2 days 1 day 1 year

Patch Bovine Bovine Pulmonary homograft Bovine

Associated cardiac

lesions

Nil Nil Nil Biscuspid aortic valve

Postoperative

complications

Nil Continued poor left

ventricular function

Kinking of the coronary

artery

Nil

Final outcome Alive and well on follow-up

at 6 months

Died at 5 weeks:

multisystem failure

Alive and well on follow-up

at 12 years

Alive and well on follow-up

at 4 years

Left ventricular

function

Good Extremely poor Good Good

Tunnel Closed Closed LV end of tunnel still open Closed

Aortic regurgitation Mild Severe at time of death Moderate Nil

Fig. 3 Case 2, parasternal long-

axis view with grossly dilated

left ventricle and tunnel

measuring 5 mm to the left of

the LVOT

824 Pediatr Cardiol (2011) 32:822–825

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2. Horvath P, Balaji S, Skovranek S, Hucin B, de Leval MR, Stark J

(1991) Surgical treatment of aortico-left ventricular tunnel. Eur J

Cardiothorac Surg 5:113–117

3. Levy MJ, Lillehei CW, Anderson RC, Amplatz K, Edwards JE

(1963) Aorico-left ventricular tunnel. Circulation 27:841–853

4. Martins JD, Sherwood MC, Mayer JE Jr, Keane JF (2004) Aortico-

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44:446450

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Aortico-left ventricular tunnel: clinical profile, diagnostic features

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