When Fragile Tissues Meet Prosthetic Valves: Osteogenesis Imperfecta and the Challenge of Valve Selection

Osteogenesis imperfecta (OI) is a rare connective tissue disorder characterized by fragile bones and different cardiovascular effects. Its implications for prosthetic valve selection remain poorly defined. A 33-year-old man was referred for evaluation of a diastolic murmur and was found to have severe aortic regurgitation with left ventricular dilation. Clinical features raised suspicion of a connective tissue disorder, although initial genetic testing was negative. The patient underwent surgical aortic valve replacement with a bioprosthetic valve due to perceived bleeding risk and patient preference. The postoperative course was complicated by significant bleeding. After the diagnosis of OI in his infant son, genetic testing confirmed OI in the patient. Early structural valve deterioration of the bioprosthesis was subsequently observed, raising concerns about prosthetic durability in OI. The long-term outcome after mechanical valve implantation was favorable. This case highlights the complexity of selecting a prosthetic valve for patients with OI. This report suggests that osteogenesis imperfecta may predispose to accelerated structural deterioration of bioprosthetic valves, with important implications for prosthetic valve selection. While the bleeding risk may support the use of bioprosthetic valves, our case suggests that these valves may be prone to early structural failure in this group. Mechanical valves, though they require lifelong anticoagulation, may be a more durable option and should be carefully considered, especially for young patients.