Aorto-left ventricular tunnel: case series of a rare disease.

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.

Subaortic Membrane and Patent Ductus Arteriosus in Rare Association-Case Series.

BACKGROUND: The combination of subaortic membrane (SAM) and patent ductus arteriosus is very rare. Subaortic stenosis is the second most common form of left ventricular outflow tract (LVOT) obstruction after valvular aortic stenosis. We are reporting the largest case series of SAM and PDA. METHODS: We included all patients that were diagnosed with the combination of SAM and PDA at our cardiac center. We have reviewed patients echocardiographic studies, cardiac catheterizations, surgical notes and all the outpatients notes. RESULTS: We have a total of 7 patients. The age at presentation was in the early childhood with 3 patients diagnosed in infancy. Four patients had severe and moderate LVOT obstruction with SAM being very close to the aortic valve and all required surgical intervention. The last three patients had mild LVOT obstruction 2 of them with the SAM being > 4mm away from the aortic valve. Six out of the seven patients had intervention while the last one is under clinical follow up currently. PDA closure did not change the outcome. There were no other postoperative complication like developing new AI or developing complete heart block. There was no relation between gender, height, weight or age at diagnosis to the SAM clinical course. CONCLUSION: SAM and PDA association is very rare. The underlying pathophysiology is not well understood. When the SAM is closer to aortic valve (≤ 4mm), it carries higher risk of progressive LVOT obstruction. The interventions for SAM and PDA were safe procedures.