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DOI: 10.1055/s-0031-1297800
Surgical therapy for valvular disease in patients with mucopolysaccharidoses
Introduction: Mucopolysaccharidoses (MPS) are extremely rare lysosomal storage diseases with a cumulative incidence of 1 in 250.000. Depending on the storage product different cardiovascular manifestations occur. Dermatan sulfate storaging diseases like MPS I (Hurler or Scheie Type), MPS II (Hunter) and MPS VI (Maroteaux Lamy) affect primary the valvular apparatus and myocardium. Early mitral and aortic valve stenoses and/or regurgitation are common findings and may occur despite enzyme replacement strategies. We report on our experience with valve replacement and subvalvular resection in this unique group of patients.
Methods: There were a total of 6 patients with significant heart disease secondary to MPS who underwent cardiac surgery in our department. Four of these were male (67%). Three had MPS type VI, two type II and one type I. The mean age at the time of operation was 35±11 years. The mean height and weight of the MPS patients were 143±12cm and 45±10kg. The most common valvular lesion was combined mitral and aortic valve disease. Two patients had additional subaortic obstruction.
Results: Five patients received a mechanical mitral prosthesis (average size 26±1mm), in 4 patients a mechanical aortic prosthesis was implanted (average size 19±2mm) in some cases combined with an aortic root enlargement. In one patient each a mitral or aortic valve repair was performed and two patients underwent subvalvular resection of myocardium. The peri- and post-operative mortality was zero during a mean follow-up of 10±8 months. Postoperative echocardiography revealed no significant flow acceleration across the mechanical prostheses or paravalvular leakage.
Conclusions: Patients with MPS face multiple debilitating organ problems, e.g. bronchial obstructions or tracheomalacia, which may contribute to an increased intra- or postoperative mortality risk. Valve replacement in MPS patients is feasible without patient-prosthesis mismatch. Mechanical valves are the prostheses of choice in patients with MPS because of the accelerated degeneration of bioprostheses.