Search

Background: Transcatheter closure of a perimembranous ventricular septal defect (PmVSD) is usually performed with an asymmetric Amplatzer occluder, which is not an ideal device. Experience with the use of the Amplatzer muscular ventricular septal defect occluder (MVSO) to close selected PmVSDs is presented. Setting: Two tertiary referral centres for paediatric cardiology in two countries. Objective: To look at the safety and efficacy of the application of the MVSO in patients with appropriate PmVSD anatomy. Patients and intervention: The procedure was performed in 10 patients aged 3.2-40 (mean 12.5) years. All had a PmVSD with a mean diameter of 5.4 (range 4-11) mm, with an extension towards the muscular septum. The mean distance of the defect from the aortic valve was 5.4 (range 4-6) mm. In all but one patient, the MVSO was introduced in routine antegrade transvenous fashion (4-mm device in one patient, 6-mm device in five, 8 mm in two, 10 mm in one, and 12 mm in one). In one patient, the device was deployed by retrograde implantation. Results: All procedures except one were performed without complications, and complete closure of the VSD was achieved. One patient with a residual shunt developed haemolysis, which resolved over 10 days. In three patients, trivial, non-progressive tricuspid regurgitation appeared after the procedure. No other complications were observed over 1.7 (range 0.2-3.5) years of follow-up. Conclusion: Application of the MVSO for closure of selected PmVSDs seems to be a safe and effective treatment option. [ABSTRACT FROM AUTHOR]

A 2-month-old girl with exudative pericarditis and hypertrophic obstructive cardiomyopathy is presented. The child had characteristic dysmorphic features of carbohydrate-deficient glycoprotein syndrome, which was confirmed by serum levels of carbohydrate-deficient transferrin. [ABSTRACT FROM AUTHOR]