Aorto-Left Ventricular Tunnel in a Multianeurysmal Heart

Abstract

A 25-year-old male presented to us with multiple episodes of syncope during the past 6 months. Physical examination was remarkable for bradycardia, an increased pulse pressure, and a systolico-diastolic murmur associated with thrill at the aortic area. ECG (Figure 1) showed complete heart block with a wide QRS escape. Twodimensional transthoracic echocardiography with color Doppler (Figures 2 and 3 and Movies I-IV in the onlineonly Data Supplement) showed the presence of a calcified aneurysmal structure arising from the ascending aorta just above the sinutubular junction, a left ventricular outflow tract (LVOT) aneurysm, a submitral left ventricle aneurysm, along with severe aortic regurgitation and infundibular pulmonic stenosis (PS). Multidetector computed tomography with volume rendering (Figures 4 and 5) clearly demonstrated the aortic and the left ventricular site of communication of the aneurysmal aorto-left ventricular tunnel (ALVT) as well as the unusual course of the ALVT burrowing into the proximal interventricular septum. The communication of the ALVT with the LVOT aneurysm was clearly demonstrated on multidetector computed tomography, as were the submitral aneurysm and infundibular PS. The PS was seen to be the result of a combination of infundibular hypertrophy and compression by the aneurysmal ALVT. The patient was implanted with a permanent pacemaker and referred to cardiothoracic surgery for definitive management.ALVT is a rare congenital entity, with ≈130 cases having been described in literature so far.1 It is distinguished from a ruptured sinus of Valsalva aneurysm by having its aortic connection above the sinutubular ridge. Various hypotheses have been put forward regarding its cause. Some authorities believe it to be an abnormal coronary artery; others have ascribed it to early aortic dissection, and still others have attributed it to persistent embryonic rests of the fifth aortic arch or to malformation of the distal bulbus cordis.2 There is no specific genetic defect identified to date. However, cystic medial degeneration has been demonstrated in an aortic aneurysm from a patient with repaired ALVT, making connective tissue disease an important contender for the underlying defect.1 The presence of multiple cardiac aneurysms in this patient adds credence to this hypothesis. However, although the patient did have a marfanoid habitus with a reduced upper to lower segment ratio and a positive wrist and thumb sign (Figure 6), he did not meet the revised Ghent criteria for Marfan syndrome. There was no family history of Marfan syndrome and no ocular involvement. Genetic testing could not be performed because the facility was not available to us. ALVT is commonly associated with other cardiac anomalies, the most frequent being coronary artery anomalies followed by aortic valve abnormalities. However, its association with other cardiac aneurysms has not been reported. On the right side, pulmonary valvular involvement occurs in ≤5% of cases. Significant right ventricular outflow tract (RVOT) obstru...

A Review of the Surgical Management of Aorto-ventricular Tunnels

“…1,2,9,21 –26 In older patients, perforation of the involved leaflet is described, or prolapse of the unsupported leaflet leading to progressive dilation of the aortic root. 15,27 –31 Pulmonary valvar stenosis, or compression of the infundibulum by the aneurysmal tunnel, is reported in about 6.8% of cases. 7,9,32 –34 Stenoses of both aortic and pulmonary valves have also been reported.…”

“…9,24,64 Most patients with left ventricular tunnels were symptomatic early during postnatal life and underwent correction before the age of six months. 1 –31,35,37 –39,53 …”

Aortico-left ventricular tunnel: updated perspectives

“…However, the timing and method of reoperation for these complications are difficult to decide for surgeons [23]. Recently, several investigators pointed out that the risk for the dilation of ascending aorta, even progression to ascending aorta aneurysm in adulthood [25][26][27][28]. Longterm hemodynamic burden, connective tissue disease or congenital valve abnormality might be the factor in the pathogenesis of the dilation of ascending aorta, but the etiology remains unclear nowadays [25][26][27][28].…”

“…Recently, several investigators pointed out that the risk for the dilation of ascending aorta, even progression to ascending aorta aneurysm in adulthood [25][26][27][28]. Longterm hemodynamic burden, connective tissue disease or congenital valve abnormality might be the factor in the pathogenesis of the dilation of ascending aorta, but the etiology remains unclear nowadays [25][26][27][28]. In some cases, ascending aorta replacement was performed in order to prevent aortic rupture or dissection [25][26][27][28].…”

“…Longterm hemodynamic burden, connective tissue disease or congenital valve abnormality might be the factor in the pathogenesis of the dilation of ascending aorta, but the etiology remains unclear nowadays [25][26][27][28]. In some cases, ascending aorta replacement was performed in order to prevent aortic rupture or dissection [25][26][27][28]. Based on the chronological sequence in the long-term outcome after repaired ALVT, regular surveillance imaging follow-up is recommended for repaired ALVT.…”