Mujer adulta mayor, 82 años, Katz A, con antecedente de diabetes mellitus tipo 2 e hipertensión arterial; ingresa por accidente cerebrovascular isquémico que se complica con síndrome de Takotsubo con posterior reingreso por fibrilación auricular tras el alta médica. Estos tres eventos clínicos tienen criterios para integrarse como un síndrome cerebro-corazón, el cual es una condición de alto riesgo para mortalidad.
Background
Isolated left ventricular apical hypoplasia is a rare, probably congenital, cardiac condition recently reported in the last two decades. Although most cases are asymptomatic or mildly symptomatic, some severe and fatal cases have been reported, leading to more efforts for appropriate diagnosis and treatment. We describe the first, and severe, case of this pathology in Peru and Latin America.
Case summary
A 24-year-old male with history of long-standing alcohol and illicit drugs use presented with symptoms of heart failure and atrial fibrillation. Transthoracic echocardiography showed biventricular dysfunction along with spherical left ventricle, abnormal papillary muscles’ origin from the left ventricular apex and an elongated right ventricle wrapping around the deficient left ventricular apex. Cardiac magnetic resonance confirmed these findings and revealed subepicardial fatty replacement at the left ventricular apex. The diagnosis of isolated left ventricular apical hypoplasia was made. He was discharged from hospital with carvedilol, enalapril, digoxin and warfarin. Eighteen months later he remains mildly symptomatic at New York Heart Association functional class II without worsening heart failure nor thromboembolism.
Discussion
This case highlights the usefulness of multimodality non-invasive cardiovascular imaging for accurate diagnosis of isolated left ventricular apical hypoplasia as well as the importance of close follow-up and treatment of established complications (heart failure and atrial fibrillation).
Durante los últimos años, se ha observado la asociación del supradesnivel de ST en la derivación aVR con el compromiso de la arteria descendente anterior (DA), tronco coronario izquierdo (TCI) y coronaria derecha (CD), en el contexto de un infarto de miocardio agudo con segmento ST elevado (IMA-STE). Por medio del electrocardiograma se puede predecir con una probabilidad del 80% el compromiso de TCI frente a DA, cuando la relación aVR sobre V1 es mayor a la unidad. Asimismo, la presencia de un supradesnivel en aVR se asocia a mayor mortalidad a los 30 días en IMA-STE de cara anterior e inferior. Por lo expuesto previamente, se recalca la importancia del análisis de la elevación del ST en aVR en un IMA-STE de cara anterior puesto que predice compromiso de gran territorio vascular y empeora el pronóstico del paciente.
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