Transcatheter aortic valve implantation in two patients with intraventricular membranous septal aneurysms

Timm Benjamin Ubben (Hamburg)1, S. Hakmi (Hamburg)1, D.-U. Chung (Hamburg)1, A. Springer (Hamburg)1, Y. Nejahsie (Hamburg)1, A. Veliqi (Hamburg)1, B. Goldmann (Reinbek)2, S. Willems (Hamburg)1, E. P. Tigges (Hamburg)1

1Asklepios Klinik St. Georg Kardiologie & internistische Intensivmedizin Hamburg, Deutschland; 2Krankenhaus Reinbek St. Adolf-Stift Klinik für Kardiologie Reinbek, Deutschland


Intraventricular membranous septal aneurysm is an extremely rare cardiac malformation with unknown prevalence. Etiology includes idiopathic or may be related to healed ventricular septal defect. Patients with IVMS are often asymptomatic, but can present with thrombembolism, RVOT obstruction or conduction disturbances. Aortic stenosis with elevated left ventricular pressure could lead to development of IVMS. Surgical aortic valve replacement and surgical removal of the IVMS are plausible options for patients with low surgical risk. There is curentily no literature about the feasibility and outcomes of transcather aortic valve implantation (TAVI) in patients with IVMS. The specific problem of TAVI in patients with IVMS is the incomplete anchoring of the TAVI prosthesis in the LVOT. The presence of anomalies in the sub-annular area can lead to valve malpositioning and its consequences. The thin tissue of the aneurysm could pose a higher risk of rupture and paravalvular leakage. There is no literature of optimal device selection between ballon-expandable and self-expandable TAVI systems. 
Patient presentation:
Patient 1 
Patient 1 was a 85 years old female and was referred to our heart team with a severe aortiv stenosis (AVA 0.9 cm², mean pressure gradient 45 mmHg, Vmax 410 cm/s, annulus area 430 mm²). Preinterventional computer tomography scan revealed an IVMS below the right coronary cusp. The aortic stenosis was treated with a Edwards Sapien Ultra 26mm. Postprocedural echocardigraphy, CT and angiography showed no significant aortic regurgitation or injury of the IVMS. A permanent pacermaker was implaned postprocedural due to a third  degree AV block. 
Patient 2:
Patient 2 was a 70 years old female and was referred to our heart team with a combined severe aortic stenosis (AVA 0.9 cm², mean pressure gradient 36  mmHg, Vmax 380 cm/s, annuus perimeter 80.9mm) and severe aortic regurgitation. She suffered from repeated heart failure hospitazilations and was bed-ridden due to a pelvis fracture. Preinterventional computer tomography showed a IVMS outpoching from LVOT into RV. The interdisciplinary heart team favoed TAVI therapy due to the immobility and frailty of the patient. In the presence of an severe aortic regurgitation we planned the strategic deployment of a retrievable Medtronic Evolut Pro Plus 29mm prosthesis and aimed for a high implant without affection of the IVMS. Implantation resulted in a trace aortic regurgitation and optimal hemodynamics. 
Both cases show the presence of IVMS in patients with aortic stenosis undergoing TAVI. Both patients could be treated with either Medtronic Evolut or Edwards Sapien with good results. Device selection has to take into account the extent of the IVMS, the anatomical surrounding and calcification of the aortic valve and outflow tract. Consequently, pre-procedural planning is crucial and computer tomography seems to be the optimal modality. Profound knowledge of the dimensions of the used TAVI systems are important and the landing zone has to be analysed. 

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