Question 1656
{"accessible":true,"alternatives":[{"id":8239,"text":"Anderson Fabry disease"},{"id":8240,"text":"anterior wall myocardial infarction"},{"id":8241,"text":"athlete's heart"},{"id":8242,"text":"cardiac amyloidosis"},{"id":8243,"text":"familial hypertrophic cardiomyopathy"},{"id":8244,"text":"iron overload cardiomyopathy"}],"archived":false,"correctAlternativeId":8242,"explanation":"\u003cp\u003e\u003ca href=\"/articles/fabry-disease\"\u003eAnderson Fabry disease\u003c/a\u003e is a storage disease leading to the deposit of glycosphingolipids within the myocardium and cardiac hypertrophy. On cardiac imaging, it is characterised by hypertrophy, late gadolinium enhancement in the inferolateral basal and midventricular wall and a decrease of T1 in the other segments.\u003c/p\u003e\u003cp\u003e\u003ca href=\"/articles/myocardial-infarction\"\u003eAnterior wall\u0026nbsp;myocardial infarction\u003c/a\u003e – there is a bit of a subendocardial late enhancement in that midventricular slice, that is true but this can also be explained otherwise. Myocardial infarction leads to an increase in T1 and ECV, this is also true but the increase is focal and not diffuse and does not affect the whole myocardium.\u003c/p\u003e\u003cp\u003e\u003ca href=\"/articles/athlete-heart-syndrome\"\u003eAthlete's heart\u003c/a\u003e leads to a decrease in ECV because of the densely packed cardiomyocytes - there is also too much hypertrophy to call this an athlete's heart.\u003c/p\u003e\u003cp\u003e\u003ca href=\"/articles/cardiac-amyloidosis\"\u003eCardiac amyloidosis\u003c/a\u003e – this is the right answer, this was a case of ATTR amyloidosis. The considerably elevated T1, the massive increase in myocardial extracellular cellular volume (ECV) and the difficulties obtaining a good inversion time in this patient support the diagnosis and was histologically proven in this case.\u003c/p\u003e\u003cp\u003e\u003ca href=\"/articles/familial-hypertrophic-cardiomyopathy\"\u003eFamilial hypertrophic cardiomyopathy\u003c/a\u003e is an important diagnosis here, but an increase of T1 and ECV due to possible myocardial fibrosis is not that pronounced as seen here.\u003c/p\u003e\u003cp\u003e\u003ca href=\"/articles/iron-overload-cardiomyopathy\"\u003eIron overload cardiomyopathy\u003c/a\u003e – no evidence, iron overload would lead to a decrease in T1.\u003c/p\u003e","id":1656,"imageUrl":"https://prod-images-static.radiopaedia.org/multiple_choice_questions/170/Amyloidosis_big_gallery.jpeg","imageAttribution":{"kind":"case","rID":74412},"imageAttributionCaseInfo":{"title":"Cardiac amyloidosis - ATTR wild-type","contributor_name":"Joachim Feger","contributor_param":"jfeger","case_rid":74412,"case_param":"cardiac-amyloidosis-attr-wild-type-1"},"firstQuestionPath":"/questions/1656","nextQuestionPath":null,"relatedArticles":[{"id":12599,"title":"Cardiac amyloidosis","link":"/articles/cardiac-amyloidosis?lang=gb"},{"id":78604,"title":"T1 mapping - myocardium","link":"/articles/t1-mapping-myocardium?lang=gb"}],"alsoUsedIn":[],"stem":"\u003cp\u003e75 years old man with fatigue, dyspnoea on exertion and a history of\u003cstrong\u003e\u0026nbsp;\u003c/strong\u003ebilateral carpal tunnel syndrome.\u003c/p\u003e\u003cp\u003eRTA reported difficulties obtaining a good inversion time in this patient. The width of the interventricular septum measured 20 mm. T1 was in the range of 1170-1250 ms that is \u0026gt;6 standard deviations above the normal range. Myocardial extracellular cellular volume (ECV) \u0026gt;55%.\u003c/p\u003e\u003cp\u003eWhat is the most likely diagnosis?\u003c/p\u003e","menuLinks":[{"text":"Report problem with question","url":"https://docs.google.com/forms/d/e/1FAIpQLSfO3soWYhOjJ7yErSysyCe5V4A1CqW7WK3rDA7MtAkecMGqNw/viewform?entry.1624461248\u0026entry.553583435=https://radiopaedia.org/questions/1656"}],"attemptsPercentages":[{"alternativeId":"8240","percentage":8},{"alternativeId":"8244","percentage":1},{"alternativeId":"8242","percentage":63},{"alternativeId":"8243","percentage":23},{"alternativeId":"8239","percentage":5},{"alternativeId":"8241","percentage":0}],"promptToLogin":false,"questionManager":false,"articleId":"hypertrophic-cardiomyopathy"}