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Relevant clinical history and physical exam
A 33 years old male, with a past history of Bio-Bentall surgery 7 years back for Severe AS,AR with ascending aorta aneurysm. He was asymptomatic since then.
Admitted with recent onset rapidly worsening dyspnoea since 3 months.
At presentation patient was in CHF, Pulmonary hypertension, NYHA class IV
Echocardiography showed : Mildly dilated LV,Degenerated Aortic valve bioprosthesis, Moderate AS with AR, Aneurysmal expansile structure surrounding the conduit.
Relevant test results prior to catheterization
Chest X ray showed: Cardiomegaly, heavily calcified Bental conduit. Increased pulmonary flow
Cardiac CT : Calcified Bental conduit , Paraconduit leak, Fistula communicating Aortic arch and Periconduit aneurysm
Cardiac MRI: Confirmed periconduit leak, Periconduit aneurysm, Fistula communicating aneurysm sac with RA
2D and 3D Echo: Confirmed and delineated this complex anatomy and haemodynamic consequence which explained recent CHF worsening to NYHA Class IV.
Relevant catheterization findings
The ascending aortogram in RAO and LAO views showed large expansile aneurysm surrounding Bentall conduit with periconduit leak, Aorta to periconduit aneurysm fistula, Rupture of periconduit aneurysm sac into RA . It also showed mildly dilated LV with moderate valavular regurgitation. Heavily calcified Bental conduit.
In view of High risk Redo Bental and complex surgical anatomy and high risk of surgery plan: Transcatheter closure of Periconduit leak, Aortic to aneurysm fistula and +/- RA fistula.
Approach : The right femoral artery and vein , Right radial artery, 3D TEE guided procedure under general anaesthesia.
Enetering paraconduit leak: Arteriovenous loop: Radial artery->Ascending aorta->Aortic fistula->Aneurysm sac-> RA fistula-> RA->IVC-femoral vein
Entering LV: Femoral vein-> IVC->RA->Fistula->Aneurysm sac-> Paraconduit leak->LV
1st: Device closure of Periconduit leak : ADO I 18/16 mm
2nd: Device closure of Aorta -> aneurysm leak: ADO I 10/8 mm
Fistula leak to RA closed automatically. There was no need to close this RA fistula as it was outlet and both inlets were closed. Dramatic haemodynamic and clinical improvement.
Discharged on Day 10. Asymptomatic at 44 days.
Unfortunately, had SCD on Day 45 after procedure at home probably due to arrhythmia's.
Later clinical autopsy done confirmed exact anatomy of this complex case which was matching the preprocedural diagnosis. Al devices were intact. The probable cause of death could be arrhythmic which could be a part of natural history of Denerated Bentall surgery.
This case highlights a complex unusual delayed complication following Bentall surgery resulting in multiple leaks and fistula. The case illustrates multi modality cardiac imaging like 2D,3D echo, Cardiac CT, Cardiac MRI, Catheterization in arriving at diagnosis explaining the haemodynamic consequences and also role in planning the treatment. The complex structural heart disease lesions were treated with rare challenging multiple devices closure resulting in dramatic recovery. Understanding the anatomy, haemodynamic results were important. Unfortunately patient had sudden cardiac death 45 days after procedure probably due to arrhythmia. The clinical autopsy confirms the diagnosis.