Aorto-left ventricular tunnel: case series of a rare disease
Obayda M Diraneyya1,2, Fahad Alhabshan1,2,3, Abdullah Alghamdi1,2,3
1Department of Cardiac Sciences, Ministry of National Guard Health Affairs, Riyadh, Saudi Arabia.
Insights
Aorto-left ventricular tunnel (ALVT) is a rare congenital heart defect. Early surgical repair of ALVT is recommended for a good outcome, though long-term monitoring of aortic valve function is crucial.
Area of Science:
- Cardiology
- Pediatric Cardiology
- Congenital Heart Disease
Background:
- Aorto-left ventricular tunnel (ALVT) is a rare congenital heart defect associated with significant early-life morbidity.
- Volume overload and left ventricular failure are common complications of ALVT.
- While surgical intervention is often curative, long-term outcomes require further investigation.
Purpose of the Study:
- To report a case series of aorto-left ventricular tunnel (ALVT).
- To evaluate the long-term outcomes of surgically repaired ALVT in pediatric patients.
Main Methods:
- Retrospective analysis of pediatric patients (birth to 14 years) diagnosed with ALVT between 2001 and 2020.
- Collection and review of demographic, echocardiographic, and perioperative data.
- Comparison of pre-operative data with last outpatient follow-up data.
Main Results:
- Five patients with ALVT were included; presentations varied from incidental murmurs to congestive heart failure.
- Two patients had aortic valve stenosis/regurgitation with depressed LV function; three had normal aortic valves and ventricular function.
- All patients underwent surgical repair with no residual ALVT; however, two developed moderate aortic regurgitation and one had moderate aortic stenosis during follow-up.
Conclusions:
- Aorto-left ventricular tunnel (ALVT) presents with a spectrum of clinical manifestations.
- Early surgical repair of ALVT is recommended for complete resolution.
- Long-term follow-up is essential to monitor for aortic root dilatation and ensure timely management of aortic valve competency.
Introduction:
Aorto-left ventricular tunnel is a rare disease that can cause significant morbidity early in life due to volume overload and left ventricular failure. Surgical intervention is usually curative with minimal early complications. However, long-term effects and outcome are not fully determined.
Objective:
We are reporting a case series of this rare CHD with its long-term outcome.
Methods:
We conducted a retrospective analysis of all children from birth to 14 years of age who were admitted between 2001 and 2020 with the diagnosis of aorto-left ventricular tunnel. Demographic, echocardiographic, and perioperative data were collected and reviewed. The pre-operative data were compared with data reviewed on the last outpatient follow-up.
Results:
Total of five patients fulfilled our inclusion criteria. Three patients were diagnosed after auscultating an incidental murmur, one had symptoms of congestive heart failure, and one had an abnormal fetal echocardiogram. Echocardiography demonstrated stenotic and regurgitant aortic valve with severely depressed left ventricle function in two patients, one of them with also single left coronary artery. The other three patients had normal aortic valve structure and normal ventricular function. All five patients had surgical repair, two by patch closure at aortic end of aorto-left ventricular tunnel, two by patch closure at both aortic and left ventricular ends, and one by aortic root replacement using a homograft. During follow-up, there was no residual aorto-left ventricular tunnel in any of our five cases, two had moderate aortic regurgitation and one had moderate residual aortic stenosis.
Conclusions:
Spectrum of presentation for aorto-left ventricular tunnel varies from an occult lesion to frank left heart failure due to volume or less commonly, pressure overload. Early surgical repair is recommended and is usually associated with complete resolution. Long-term follow-up is recommended for aortic root dilatation and aortic valve competency, as valve function need to be addressed in a timely manner to avoid further complications.
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