Hematopoietic stem cell transplantation in autoimmune diseases: algorithm for cardiovascular assessment

Heart involvement is a frequent cause of morbidity and mortality in autoimmune diseases. All cardiac structures can be involved: pericardium, endocardium, myocardium, coronary circle, and conduction system. In the last decade many patients affected by autoimmune diseases have been treated with hematopoietic stem cell transplantation; the vast majority of these transplants have been autologous, and most have been within the context of phase I and II clinical trials; now, phase III trials are ongoing. Patients affected by autoimmune disease often have cardiac involvement which potentially puts them at higher risk from acute cardiotoxicity due to alkylating agents such as cyclophosphamide. The authors propose an algorithm for cardiac assessment before stem cell transplantation in order to identify those patients at highest risk, prior to administering any drug, to avoid further worsening of heart involvement and possible organ failure.A baseline assessment includes physical examinations, ECG to highlight arrhythmias and conduction abnormalities, chest X-ray to evaluate the presence of pericardial effusion and cardiothoracic ratio.A second-step evaluation includes echocardiography (which assesses the following parameters: left ventricular ejection fraction, diastolic function, tricuspid gradient, pulmonary acceleration time, right ventricular diameter and pericardial effusion, wall motion), Holter ECG that may highlight the presence of arrhythmias and biohumoral parameters such as brain natriuretic peptide and troponin I. If these parameters show abnormalities, a further step is required before transplantation. Cardiac catheterization allows to identify ischemic coronary diseases and pulmonary artery hypertension. Intensive monitoring with life card assessment before inclusion might establish ischemic coronary diseases or complex arrhythmias requiring pacing. Magnetic resonance imaging and single-photon emission computed tomography with dipyridamole are useful tools to evaluate the coronary flow. Treatment of ischemic coronary disease (assessment for revascularization), cardiac failure, pulmonary artery hypertension and arrhythmias constitutes the final step. The aim is to optimize cardiac status in order to allow intense immunosuppressive treatments.