‘Full-metal Jacket’ treatment of multiple paravalvular leaks

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A 40-year-old man with familial osteogenesis imperfecta type I, congenital deaf-mutism, joint hyperlaxity and recurrent bone fractures was referred to our institution due to refractory congestive heart failure caused by significant, multiple mitral and aortic paravalvular leaks (PVLs). He had been submitted to biologic aortic valve replacement and mitral valve repair few years before due to significant mitroaortic regurgitation. Then, a further aortic valve replacement with mechanical #21 bileaflet prosthesis (St. Jude Medical Corp, St. Paul, Minnesota, USA), due to multiple PVLs, and concomitant mitral valve replacement with ON-X 25/33 (On-X Life Technologies Inc, Austin, Texas, USA) prosthesis, due to significant residual mitral valve regurgitation, were required. Few months after this second surgical procedure, he had repeated hospital admissions because of symptomatic episodes of ventricular tachycardia and acute decompensated congestive heart failure. His clinical status appeared refractory to maximal pharmacologic therapy and was presumably caused by partial detachment of both aortic and mitral prostheses. At the time of referral, the patient was in New York Heart Association functional class IV with signs of low cardiac output and systemic venous congestion. Aortic regurgitation was due to two large PVLs located at 12 and 6 h according to Kliger et al.1 classification, resulting in olo-diastolic reverse flow at thoracoabdominal aorta level (Fig. 1). Mitral regurgitation was due to a single, very large (14 × 23 mm) PVL located at 6 h, resulting in mild prosthesis rocking (Fig. 2). Due to the perceived very high surgical risk of a further mitroaortic replacement, percutaneous closure of both PVLs was attempted. Patient's informed consent, as well as surgical agreement with the procedure, was obtained. Due to anticipated length and complexity, the procedure was performed in two steps, 7 days apart, under general anaesthesia and mechanical ventilation. The PVLs were probed with straight or angled hydrophilic 0.035″ guide-wires (Zipwire, Boston Scientific, Marlborough, Massachusetts, USA) passed through standard angiographic catheters of different shapes (right coronary, multipurpose, Amplatz right or left coronary shape). After probing, the hydrophilic guide-wire was exchanged with an Amplatz Type superstiff exchange guide-wire (Cordis Corporation, Miami Lakes, Florida, USA), so allowing larger delivery catheters to be introduced. No artero-venous circuit was necessary due to perceived high stability of the stiff guide-wire inside the cardiac chambers. The larger aortic PVL was closed by simultaneous deployment of two Amplatzer Vascular Plug type II (St. Jude Medical Corp) devices (10 and 12 mm, respectively) through 7.5-Fr sheath-less catheters (Asahi, INTECC Co. Ltd, Akatsuki-cho, Seto, Aichi, Japan), whereas the smaller PVL was occluded with two further, sequentially implanted, 10-mm Amplatzer Vascular Plug type II devices. The mitral PVL was closed by trans-septal implantation of three Amplatzer Vascular Plug type II devices (20, 16 and 14 mm large, respectively) through 9-Fr Mullins sheaths (Cook, Bloomington, Indiana, USA). The first two plugs were simultaneously deployed around the prosthetic valve annulus (Fig. 3), whereas the third one was sequentially implanted and nicely allocated between them and the lateral wall of the left atrium. This type of device was chosen because the size of the Amplatzer Vascular Plugs III and IV was deemed not suitable to safely accommodate inside these huge leaks and, at the time of the procedure, the Occlutech PLD device (Occlutech GmbH, Jena, Germany) was not already available at our institution. At the end of the procedure, a residual trivial aortic and mitral regurgitation were imaged (Fig. 4), without any interference with the function of either valve, and the pulmonary artery pressure significantly dropped to about 60% of the systemic level. Postoperative course was troublesome due to groin and retroperitoneal bleeding, requiring repeat blood transfusions and confirmed by emergent CT scan.

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