Case Presentation: A 64-year-old Middle-Eastern man with a history of rheumatic heart disease status post mechanical mitral Starr-Edwards ball-in-cage valve (in 1968), atrial flutter on coumadin and prior ischemic stroke with residual blindness presented with 4 days of “bloody” urine. Physical exam was significant for scleral icterus, an unchanged holosystolic murmur, and the observation of maroon-colored urine without clots or sedimentation. Labs were notable for acute on chronic anemia (Hb 8.0 g/dL from 11.0 g/dL), hyperbilirubinemia, low haptoglobin, elevated LDH, normal iron studies and therapeutic INR. Urinalysis showed large blood and few RBCs. Peripheral smear showed numerous schistocytes. Extensive workup of the etiology of hemolysis was pursued, including repeat transthoracic echocardiogram (TTE), CT abdomen/pelvis, infectious workup, Coombs profile, G6PD testing, and PNH labs, all of which were unremarkable. His hospital course was complicated by persistent anemia requiring blood transfusions with poor response. He was resumed on anticoagulation with a heparin drip. On Day 3, the patient underwent a transesophageal echocardiogram (TEE), which revealed a 1.5cm x 0.3cm thrombus on the cage of the valve prolapsing in and out of the left ventricular outflow tract and aortic valve, resulting in moderate mitral regurgitation. The patient underwent immediate mitral valve replacement. He was unable to recover from multiple complications and passed away after 4 months in the intensive care unit.
Discussion: Mechanical valves, particularly older ones such as the ball-in-cage valve, are notoriously thrombogenic. In this case of worsening hemolysis, an immediate and thorough evaluation of the valve was crucial to determining if increased shear stress, whether due to structural deterioration, paravalvular leak, vegetation or thrombus, could be the cause. Two TTE’s were unable to identify the thrombus, highlighting the importance of TEE when there remains strong suspicion of valvular compromise. Additionally, while it may seem counterintuitive in the setting of acute anemia, continuing anticoagulation was essential given the higher risk of thrombogenesis over bleed. This was evidenced by a lack of response to blood transfusions while the primary culprit, the thrombosed valve, remained in place. While the patient had complained of “bloody urine,” it was important to deduce that a urinalysis with large blood but few RBCs was indicative of hemoglobinuria rather than hematuria. The guiles of the ball-in-cage valve were known to this patient’s cardiologist; however, there remain no clear guidelines as to the management of an antique and complicated valve. The single case report of an explanted ball-in-cage describes a young, functionally independent patient without significant comorbidities. These are all factors that our patient lacked, which may have ultimately contributed to his presentation and complicated course.
Conclusions: It is imperative to rule out valvular complications in the setting of persistent hemolysis in hospitalized patients. The above case outlines a rare presentation of hemolysis in an American hospital with limited prior case reports. It yields several key teaching points. TEE should be pursued if TTE is unremarkable. Reversible anticoagulation should be continued once bleeding has been ruled out. Finally, surgical replacement of the valve should proceed from a thoughtful discussion of the risks and benefits to the individual patient.