Paget’s disease of bone, or osteitis deformans, represents a chronic disorder that profoundly alters normal bone remodeling processes, leading to the development of structurally weak and often enlarged bones. The disease, second in prevalence among metabolic bone disorders after osteoporosis, affects roughly 1% to 2% of adults over 55 years, with a higher incidence noted in Western populations. While the disease’s skeletal manifestations, such as deformities, fractures, arthritis, and pain, often capture primary clinical attention, its cardiovascular complications—most notably heart failure—deserve focused scrutiny in our practice. The relationship between abnormal bone turnover and increased cardiovascular strain has significant implications, particularly in advanced cases, highlighting the need for greater awareness. The pathological changes of Paget’s disease primarily stem from a mismatch in bone resorption and formation, driven by abnormal osteoclast and osteoblast activity. This abnormal bone turnover not only weakens the bone mechanically but also substantially increases its vascularization. As metabolic demands surge to supply blood to hyperactive bone regions, a notable cardiovascular burden ensues. The enhanced vascularity can lead to systemic effects, straining cardiac function and, over time, potentially leading to heart failure. This cardiovascular involvement is often underappreciated, with signs that may be mistaken for other conditions or simply attributed to aging, delaying optimal treatment and management. A prominent cardiovascular manifestation inPaget’s disease is high-output heart failure. Unlike traditional low-output heart failure, which stems from reduced cardiac output, high-output heart failure occurs when the heart’s enhanced performance cannot meet the elevated metabolic demands of hyper vascularized bone regions. Blood flow through these areas significantly increases, prompting the heart to boost its output and dilate blood vessels to maintain adequate circulation. When this state persists, however, the cardiac reserve may be overwhelmed. Symptoms can include fatigue, edema, dyspnea, and other signs commonly associated with heart failure, though the hyperdynamic state and underlying bone-related causes are often overlooked. The sustained increase in cardiac workload due to high metabolic demands of affected bones can lead to structural changes in the heart. Over time, left ventricular hypertrophy (LVH) may develop as the heart muscle thickens to cope with the increased strain. LVH is often a compensatory response but, if prolonged, can exacerbate heart failure by reducing the efficiency of cardiac pumping. The strain of pumping against elevated vascular resistance also contributes to heart failure symptoms, such as shortness of breath, fatigue, and fluid retention. Perfusionists, particularly during surgery or when managing hemodynamic fluctuations, should be aware of these potential challenges in patients with extensive Paget’s disease involvement.
Reference:
1. Ralston SH. Bisphosphonates in the management of Paget's disease. Bone. 2020Sep 1;138:115465.
2. Ralston SH. Paget's disease of bone. New England Journal of Medicine. 2013Feb14;368(7):644-50.
3. Hadjidakis DJ, Androulakis II. Bone remodeling. Annals of the NewYorkAcademy of Sciences. 2006 Dec;1092(1):385-96.
4. Carletti A, Gavaia PJ, Cancela ML, Laizé V. Metabolic bone disorders andthepromise of marine osteoactive compounds. *Cellular and Molecular Life Sciences*. 2024 Dec;81(1):11.
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