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Background: Secondary cardiac tumours are much more common compared with primary (100-1000 times). The majority of the primary cardiac tumours are benign; however, almost a quarter are malignant, and 95% of these are sarcomas. The rarest type of primary malignant cardiac sarcoma is intimal (spindle cell) sarcoma. Case summary: A 37-year-old woman presented with episodes of breathlessness. Initially treated for a chest infection, however, the patient continued to deteriorate and presented to the emergency department. A large pericardial effusion was discovered and drained, with samples sent for analysis. A repeat interval echo confirmed the resolution of the pericardial effusion with preserved left ventricular (LV) systolic function. The computed tomography (CT) of the thorax showed suspicious lesions in the heart and lung while the repeat echo raised suspicion of an infiltrative disease. A cardiac magnetic resonance imaging scan was performed, which suggested evidence of an undifferentiated sarcoma involving the posterior wall of the LV and an overlying thrombus. Computed tomography of the abdomen and pelvis did not show any evidence of abdominal metastasis. A CT-guided lung biopsy was arranged. On histological analysis, the report was overall strongly supportive of a diagnosis of intimal sarcoma. She underwent chemotherapy until recently. Discussion: Cardiac intimal sarcomas are the least reported type of primary malignant tumours of the heart. They are encountered more commonly in the large arterial blood vessels, including the pulmonary artery and aorta, and are extremely rare in the heart. A prompt diagnosis is essential as they are considered extremely aggressive.
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Kounis syndrome is a multisystem and multidisciplinary disease affecting the circulatory system that can be manifested as spasm and thrombosis. It can occur as allergic, hypersensitivity, anaphylactic, or anaphylactoid reactions associated with the release of inflammatory mediators from mast cells and from other interrelated and interacting inflammatory cells, including macrophages and lymphocytes. A platelet subset with high- and low-affinity IgE surface receptors is also involved in this process. Whereas the heart, and particularly the coronary arteries, constitute the primary targets of inflammatory mediators, the mesenteric, cerebral, and peripheral arteries are also vulnerable. Kounis syndrome is caused by a variety of factors, including drugs, foods, environmental exposure, clinical conditions, stent implantation, and vaccines. We report a unique case of a 60-year-old male with a past medical history of allergy to human albumin, alcoholic cirrhosis, and esophageal varices, who was admitted due to multiple episodes of hematemesis. Due to low hemoglobin levels, he was transfused with 3 units of red blood cells and fresh frozen plasma without any adverse reactions. On the third day of hospitalization, severe thrombocytopenia was observed and transfusion of platelets was initiated. Immediately following platelet infusion, the patient developed chest discomfort, skin signs of severe allergic reaction, and hemodynamic instability. The electrocardiogram revealed ST segment elevation in the inferior leads. Given the strong suspicion of Kounis syndrome/allergic coronary spasm, the patient was treated with anti-allergic treatment only, without any anti-platelet therapy. The clinical status of the patient gradually improved and the electrocardiographic changes reverted to normal. Based on these findings, Kounis hypersensitivity-associated acute coronary syndrome, specifically, type I Kounis syndrome, was diagnosed. Although platelet transfusion can be a life-saving therapy, each blood transfusion carries a substantial risk of adverse reactions. The aims of this report are to expand the existing knowledge of patient responses to blood transfusion and provide information on the incidence of various severe transfusion reactions to all blood components and especially to platelets. To the best of our knowledge, Kounis syndrome induced by platelet transfusionhas never been previously reported. Hypersensitivity to platelet external membrane glycoproteins in an atopic patient seems to be the possible etiology. Despite that Kounis syndrome remains an under-diagnosed clinical entity in everyday practice, it should always be considered in the differential diagnosis of acute coronary syndromes.
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BACKGROUND: Cerebral autosomal dominant arteriopathy with subcortical infarcts and leucoencephalopathy (CADASIL) syndrome is a genetically inherited condition most notably affecting the central nervous system in young adults. There is limited knowledge on its association with coronary arteries, and its association with spontaneous coronary artery dissection (SCAD) has not been previously reported. CASE SUMMARY: A 61-year-old woman who is known to have CADASIL syndrome presented with anterior ST-segment myocardial infarction and underwent emergency angiography. This showed appearance consistent with SCAD in the mid left anterior descending artery with tubular stenosis. DISCUSSION: The association between CADASIL syndrome and SCAD has not been previously reported. The similarity in the underlying pathophysiology of these two conditions makes this case intriguing.
