Aorto-left ventricular tunnel (ALVT) is a rare congenital anomaly. In this condition, the aorto-ventricular tunnel is a congenital extracardiac channel that connects the ascending aorta to the left ventricle above the sinotubular junction. A 5-year-old boy presented with fatigue during physical activity, leading to visiting a pediatric specialist. Upon examination, a continuous murmur, predominantly diastolic, was detected. Suspecting aortic insufficiency, the patient was referred for further evaluation. Transthoracic echocardiography revealed a congenital ALVT. Although congenital ALVT is a rare congenital heart disease, its clinical symptoms may overlap with other congenital heart diseases, such as tetralogy of Fallot without pulmonary stenosis and patent ductus arteriosus. Differential diagnosis, such as the absence of a wide pulse pressure, can be helpful in distinguishing between these conditions. An aorticoventricular tunnel, sometimes referred to as an "aorto-ventricular tunnel," is an abnormal endotheliazed channel between the ascending aorta and a cardiac chamber. The left ventricle is the most common location for this anomalous connection (more than 90% of the time), though right ventricular involvement has also been described. The tunnel most frequently originates just cephalad to the sinotubular junction over the right coronary sinus. This location of the aortic orifice differentiates a tunnel from a ruptured sinus of Valsalva aneurysm. The ventricular orifice is just below the aortic valve (proximal) in the interleaflet triangle of the left and right leaflets. An aorticoventricular tunnel does not pass through intracardiac myocardium on its path to the ventricular cavity, differentiating it from a coronary-cameral fistula. The surgical classification of the 4 types of aortico-left ventricular tunnel was introduced in 1988: Type I: a simple tunnel with a slit-like aortic orifice and no aortic valve distortion. Type II: an aneurysmal extracardiac aortic wall component, an oval aortic orifice, with or without aortic valve distortion. Type III: an aneurysmal intracardiac component (septal), an oval orifice, with or without right ventricular outflow tract obstruction. Type IV: a combination of types II and III. Proper classification of aorticoventricular tunnel cases guides management strategies and prognosis. 

Aorto-ventricular tunnel (AVT) is an abnormal communication channel between the ascending aorta and the ventricle. It commonly has two orifices, i.e., one aortic opening and one ventricular opening. In this study, we present a unique case of AVT with three orifices: one aortic opening, one LV opening, and one RV opening. A 64-year-old male presented with chest discomfort and dyspnea on exertion lasting the past six months. Physical examination revealed a grade 4/6 continuous biphasic murmur along the left sternal edge and a grade 3/6 systolic murmur at the apex. Transthoracic echocardiography (TTE) demonstrated: (1) an AVT with three orifices, i.e., one aortic opening, one LV opening, and one RV opening. The LV and RV openings were located in the left and right ventricular outflow tracts, respectively. (2) The aortic valve (AV) was calcified with a small aneurysm at the non-coronary cusp. (3)The mitral valve (MV) chordal rupture of the P2 and P3 segments was observed in the posterior leaflet with severe eccentric regurgitation. Subsequent coronary computed tomography angiography (CTA) further confirmed the diagnosis of AVT with three openings, and clarified the coronary arteries normally arose from the aortic sinuses. The patient was then referred for surgical treatment, consisting of closure of three AVT orifices, AV replacement, and MV replacement. Six months following surgery, the patient was asymptomatic. TTE demonstrated normal mechanic AV and MV function, and there was no residual shunt among the ascending aorta, LV and RV. It is the first case to report an AVT with three orifices. This paper described the entire process from diagnosis to treatment of this unique case, thus providing some novel insights into AVT.

An aorto-ventricular tunnel is a rare congenital cardiac defect, where a channel connects the lumen of the ascending aorta to the left or right ventricle. Four patients presented with an aorto-left ventricular tunnel over two decades at a median age of 8 months (range 0.1-10 months). Two patients (50%) had associated cardiac anomalies including hypoplastic left heart syndrome and left ventricular noncompaction/hypertrophic cardiomyopathy with aortic/pulmonary valve dysplasia in one patient each. Although traditionally surgical treatment has addressed this problem, management has evolved to transcatheter closure with excellent outcomes in appropriately selected patients at our national centre.

Aorto-ventricular tunnels are rare, congenital extracardiac connections arising from the tubular ascending aorta, above the sinotubular junction, which communicate directly with the ventricular cavity. We present long-term follow-up in a series of five adults undergoing pediatric repair and describe the cardiovascular features and outcomes. Significant aortic regurgitation, ascending aortic aneurysm and left ventricular noncompaction were found to be highly prevalent, emphasizing the need for ongoing surveillance following repair of this anomaly.

Left aorto-ventricular tunnel is a rare congenital heart disorder. A then 19-year-old young lady was referred to our clinic for chronic rheumatic heart disease with aortic regurgitation. However, because of an almost continuous murmur, and an unusual transthoracic echo, she underwent various investigations, finally revealing a rare type 2 left aorto-ventricular tunnel. She had been variously diagnosed as chronic rheumatic heart disease with aortic regurgitation, bicuspid aortic valve with aortic regurgitation, and then possibly coronary arterial (cameral) fistula (based on 2D transthoracic echo) and finally left aorto-ventricular tunnel. This is the first case described in South East Asia who is asymptomatic, apart from a murmur, and her long journey to this final diagnosis illustrates the need to "chase shadows" on echoes, to come to a proper conclusion for the patient.