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Aorto-ventricular tunnel.

McKay R - Orphanet J Rare Dis (2007)

Bottom Line: It appears to result from a combination of maldevelopment of the cushions which give rise to the pulmonary and aortic roots, and abnormal separation of these structures.Optimal management of symptomatic aorto-ventricular tunnel consists of diagnosis by echocardiography, complimented with cardiac catheterization as needed to elucidate coronary arterial origins or associated defects, and prompt surgical repair.Observation of the exceedingly rare, asymptomatic patient with a small tunnel may be justified by occasional spontaneous closure.

View Article: PubMed Central - HTML - PubMed

Affiliation: Division of Cardiovascular Surgery, Le Bonheur Children's Hospital, Memphis, TN 38103, USA. rmck07@yahoo.com

ABSTRACT
Aorto-ventricular tunnel is a congenital, extracardiac channel which connects the ascending aorta above the sinutubular junction to the cavity of the left, or (less commonly) right ventricle. The exact incidence is unknown, estimates ranging from 0.5% of fetal cardiac malformations to less than 0.1% of congenitally malformed hearts in clinico-pathological series. Approximately 130 cases have been reported in the literature, about twice as many cases in males as in females. Associated defects, usually involving the proximal coronary arteries, or the aortic or pulmonary valves, are present in nearly half the cases. Occasional patients present with an asymptomatic heart murmur and cardiac enlargement, but most suffer heart failure in the first year of life. The etiology of aorto-ventricular tunnel is uncertain. It appears to result from a combination of maldevelopment of the cushions which give rise to the pulmonary and aortic roots, and abnormal separation of these structures. Echocardiography is the diagnostic investigation of choice. Antenatal diagnosis by fetal echocardiography is reliable after 18 weeks gestation. Aorto-ventricular tunnel must be distinguished from other lesions which cause rapid run-off of blood from the aorta and produce cardiac failure. Optimal management of symptomatic aorto-ventricular tunnel consists of diagnosis by echocardiography, complimented with cardiac catheterization as needed to elucidate coronary arterial origins or associated defects, and prompt surgical repair. Observation of the exceedingly rare, asymptomatic patient with a small tunnel may be justified by occasional spontaneous closure. All patients require life-long follow-up for recurrence of the tunnel, aortic valve incompetence, left ventricular function, and aneurysmal enlargement of the ascending aorta.

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Surgical repair of aorto-left ventricular tunnel (a) and aorto-right ventricular tunnel (b). The aortic orifice of either tunnel is closed with a patch inserted through an aortotomy. A left ventricular orifice is closed with a second patch, placed through the opened tunnel itself, which is then obliterated by reapproximation of its walls over the patch. The upper margin of this second patch attaches to the extra-luminal surface of the first. In the case of aorto-right ventricular tunnel, the right ventricular orifice is approached through the right ventricle or pulmonary valve, and the second patch lies completely separate from that in the aorta.
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Figure 2: Surgical repair of aorto-left ventricular tunnel (a) and aorto-right ventricular tunnel (b). The aortic orifice of either tunnel is closed with a patch inserted through an aortotomy. A left ventricular orifice is closed with a second patch, placed through the opened tunnel itself, which is then obliterated by reapproximation of its walls over the patch. The upper margin of this second patch attaches to the extra-luminal surface of the first. In the case of aorto-right ventricular tunnel, the right ventricular orifice is approached through the right ventricle or pulmonary valve, and the second patch lies completely separate from that in the aorta.

Mentions: Repair consists of closing the tunnel such that the aortic valve is supported, the coronary circulation is not compromised, and left or right ventricular outflow obstruction is prevented or relieved. In most cases of aorto-left ventricular tunnel, this has been accomplished by transaortic patch closure of the aortic end, and placement of a second patch through the tunnel itself to close the ventricular orifice and support the aortic valve (Figure 2a). Alternatively, the tunnel wall itself can be used to achieve an equivalent anatomical result [44]. Closure of the aortic orifice by direct suture also has sometimes given good results [11,45,46], but more often, the tunnel recurs or progressive aortic regurgitation through an unsupported or distorted right coronary leaflet leads to subsequent valve replacement. If the ventricular end of an aorto-left ventricular tunnel is not closed, residual high pressure in the blind-ending pouch may compress the right ventricular outflow [22]. In tunnels communicating with a low-pressure right ventricle, it is less certain that the ventricular orifice need be closed, although this has been done through a right ventricular incision in most reported cases (Figure 2b).


Aorto-ventricular tunnel.

McKay R - Orphanet J Rare Dis (2007)

Surgical repair of aorto-left ventricular tunnel (a) and aorto-right ventricular tunnel (b). The aortic orifice of either tunnel is closed with a patch inserted through an aortotomy. A left ventricular orifice is closed with a second patch, placed through the opened tunnel itself, which is then obliterated by reapproximation of its walls over the patch. The upper margin of this second patch attaches to the extra-luminal surface of the first. In the case of aorto-right ventricular tunnel, the right ventricular orifice is approached through the right ventricle or pulmonary valve, and the second patch lies completely separate from that in the aorta.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC2089057&req=5

Figure 2: Surgical repair of aorto-left ventricular tunnel (a) and aorto-right ventricular tunnel (b). The aortic orifice of either tunnel is closed with a patch inserted through an aortotomy. A left ventricular orifice is closed with a second patch, placed through the opened tunnel itself, which is then obliterated by reapproximation of its walls over the patch. The upper margin of this second patch attaches to the extra-luminal surface of the first. In the case of aorto-right ventricular tunnel, the right ventricular orifice is approached through the right ventricle or pulmonary valve, and the second patch lies completely separate from that in the aorta.
Mentions: Repair consists of closing the tunnel such that the aortic valve is supported, the coronary circulation is not compromised, and left or right ventricular outflow obstruction is prevented or relieved. In most cases of aorto-left ventricular tunnel, this has been accomplished by transaortic patch closure of the aortic end, and placement of a second patch through the tunnel itself to close the ventricular orifice and support the aortic valve (Figure 2a). Alternatively, the tunnel wall itself can be used to achieve an equivalent anatomical result [44]. Closure of the aortic orifice by direct suture also has sometimes given good results [11,45,46], but more often, the tunnel recurs or progressive aortic regurgitation through an unsupported or distorted right coronary leaflet leads to subsequent valve replacement. If the ventricular end of an aorto-left ventricular tunnel is not closed, residual high pressure in the blind-ending pouch may compress the right ventricular outflow [22]. In tunnels communicating with a low-pressure right ventricle, it is less certain that the ventricular orifice need be closed, although this has been done through a right ventricular incision in most reported cases (Figure 2b).

Bottom Line: It appears to result from a combination of maldevelopment of the cushions which give rise to the pulmonary and aortic roots, and abnormal separation of these structures.Optimal management of symptomatic aorto-ventricular tunnel consists of diagnosis by echocardiography, complimented with cardiac catheterization as needed to elucidate coronary arterial origins or associated defects, and prompt surgical repair.Observation of the exceedingly rare, asymptomatic patient with a small tunnel may be justified by occasional spontaneous closure.

View Article: PubMed Central - HTML - PubMed

Affiliation: Division of Cardiovascular Surgery, Le Bonheur Children's Hospital, Memphis, TN 38103, USA. rmck07@yahoo.com

ABSTRACT
Aorto-ventricular tunnel is a congenital, extracardiac channel which connects the ascending aorta above the sinutubular junction to the cavity of the left, or (less commonly) right ventricle. The exact incidence is unknown, estimates ranging from 0.5% of fetal cardiac malformations to less than 0.1% of congenitally malformed hearts in clinico-pathological series. Approximately 130 cases have been reported in the literature, about twice as many cases in males as in females. Associated defects, usually involving the proximal coronary arteries, or the aortic or pulmonary valves, are present in nearly half the cases. Occasional patients present with an asymptomatic heart murmur and cardiac enlargement, but most suffer heart failure in the first year of life. The etiology of aorto-ventricular tunnel is uncertain. It appears to result from a combination of maldevelopment of the cushions which give rise to the pulmonary and aortic roots, and abnormal separation of these structures. Echocardiography is the diagnostic investigation of choice. Antenatal diagnosis by fetal echocardiography is reliable after 18 weeks gestation. Aorto-ventricular tunnel must be distinguished from other lesions which cause rapid run-off of blood from the aorta and produce cardiac failure. Optimal management of symptomatic aorto-ventricular tunnel consists of diagnosis by echocardiography, complimented with cardiac catheterization as needed to elucidate coronary arterial origins or associated defects, and prompt surgical repair. Observation of the exceedingly rare, asymptomatic patient with a small tunnel may be justified by occasional spontaneous closure. All patients require life-long follow-up for recurrence of the tunnel, aortic valve incompetence, left ventricular function, and aneurysmal enlargement of the ascending aorta.

Show MeSH
Related in: MedlinePlus