A Review of the Surgical Management of Aorto-ventricular Tunnels

Abstract

We present a synthesis of 95 published investigations of the exceedingly rare tunnels that can exist between the aortic root and the left or right ventricles. From the 220 suitable cases included in these investigations, we reviewed the clinical presentations, modalities used for diagnosis, surgical approaches, and outcomes. Diagnostic information was provided by clinical presentation, radiographic findings, saline contrast echocardiography, computed tomographic angiocardiography, magnetic resonance imaging, cardiac catheterization, and angiocardiography. These techniques elucidated the coronary arterial origins and associated defects and defined the disease before surgery. Patients occasionally present with an asymptomatic cardiac murmur and cardiomegaly, but most suffer cardiac failure in the first year of life when the tunnel enters the left ventricle. Antenatal diagnosis by fetal echocardiography is reliable after 18 weeks of gestation. Associated defects, involving the proximal coronary arteries or the aortic or pulmonary valves, are present in nearly half the cases. Prompt diagnosis and surgical repair are important for a favorable outcome. Overall, operative mortality has been cited to be between 3% and 8.3%. Associated congenital coronary arterial anomalies, residual severe aortic stenosis, poor left ventricular function, and rupture of an infected suture line have been the reported causes of death. Despite early surgical intervention, an incidence of 16% to 60% postoperative residual aortic regurgitation of varying severity has been reported. The requirement of further repair or replacement of the aortic valve ranges from 0% to 50%. We submit that an increased appreciation of these details relative to the tunnels will contribute to improved surgical management.

Multimodality imaging for comprehensive non‐invasive diagnosis of aorto‐left ventricular tunnel in infancy

“…The presence of left ventricular dilation, hypertrophy, and progressive deterioration of left ventricular functions are consistent features. The association with a flow through the tunnel regurgitating into the left ventricle is highly suggestive of the diagnosis 10 . Nevertheless, the ultrasound misdiagnosis rate was about 17.1%, with misdiagnosis in 3.3% of cases.…”

“…Coexisting aortic valve abnormalities are common in (36.3%) of tunnel cases, ranging from bicuspid aortic valves without obstruction to severely dysplastic or even atretic valves 10 . In a multicenter study from 15 centers presenting 30‐year experience of aorto‐ventricular tunnels surgical interventions, including 36 ALVT patients, aortic regurgitation was detected in 61.1% of which 4(9%) were severe in degree, aortic stenosis in 5 (13.9%), and bicuspid aortic valve in 10 (27.8%) 5 .…”

Aorto-ventricular tunnel with three orifices: a unique case report diagnosed by transthoracic echocardiography

“…Although there are case reports of percutaneous device closure of the defect [ 10 , 11 ], surgical correction is considered the optimal and standard treatment strategy. Moreover, the patch closure of each orifice is recommended [ 12 , 13 ]. Our case received surgical closure for three openings of the AVT.…”

Surgical repair of an aortico-left ventricular tunnel with Takayasu's arteritis

“…Left ventricular enlargement and hypertrophy was noted in almost all patients. The association of normal ventricular architecture, a dilated aortic root, flow through the tunnel, and identification of aortic regurgitation should suggest the diagnosis [ 9 ]. The results of transthoracic echocardiography and cardiac catheterization are here interpreted as showing a significant bidirectional turbulent flow via a tubular communication between the aortic root just above the level of the aortic sinus and the left ventricle.…”

“…If the aortic orifice of the ALVT is not closed, residual high pressure in the blind-ending pouch may compress the right ventricular outflow. With patch closure, utmost precautions must be taken to avoid collision or traction of the commissure between the coronary aortic valvar leaflets, since the aortic orifice is usually located just above the right sinutubular junction [ 9 , 13 ].…”