A B S T R A C TErdheim Chester disease is a very rare histiocytic disorder characterised by tissue infiltration by lipid laden histiocytes. The most common presentation is bone pains typically involving the long bones. Over time almost 50% of the patients develop extraosseous involvement. The prognosis depends on the extent and distribution of the extraskeletal manifestations. Cardiovascular involvement is seen in up to 40% of the patients and the most common manifestations are periaortic fibrosis and pericardial involvement. Respiratory distress, extensive pulmonary fibrosis, and cardiac failure are the most common causes of death in these patients. Cardiac tamponade has also been documented to cause death in these patients. We describe a patient of Erdheim Chester disease who presented with recurrent and very rapidly occurring cardiac tamponade in a short duration of time and benefited from timely recognition and management.
Anomalous origin of left coronary artery from right sinus of Valsalva (RSV) is extremely rare when not associated with other cardiac anomalies. We describe a case of single coronary artery arising from the RSV in a 75-year-old male patient with coronary heart disease. Angiography revealed significant atherosclerotic stenosis in all the three coronary arteries. This report highlights the feasibility and safety of multivessel percutaneous coronary intervention (PCI) in this rare anomaly and discusses the important technical considerations to be kept in mind while attempting such a case. This is the first report in the literature describing PCI in all the vessels of a single coronary trunk arising from RSV.
Paravalvular leaks (PVL) after valve replacement surgeries are not uncommon. A significant number of these patients need some form of intervention as they commonly present with heart failure or severe hemolysis. Surgical correction is associated with high mortality and morbidity. Device closure of PVLs has been found to have good results. Since there are no devices designed specifically for PVL closure, large PVL closure is difficult. Occasional larger PVLs have been closed with a combination of a device and smaller coils. We present here a case of very large sized mitral PVL, in a patient with high risk for surgery, which was closed with two large size devices.
Lithium is a commonly used drug for bipolar mood disorder. Though effective, it has a narrow therapeutic range and has potentially life-threatening effects at higher serum levels. Lithium toxicity can be precipitated by several drug interactions. Many commonly used cardiac drugs have serious drug interaction with lithium which is not commonly known in clinical practice. We present a case where a patient on lithium therapy since 15 years, presented with sinus arrest and syncope due to lithium toxicity, within 2 weeks of initiation of low dose angiotensin converting enzyme (ACE) inhibitor. The patient however needed temporary pacemaker support and had an uneventful recovery, without the need for a permanent pacemaker, once the lithium levels fell down to normal.
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