No abstract

What is the most likely diagnosis? Acute type A aortic dissectionWilliams syndromeLoa loa worm infectionIntimo-intimal intussusceptionGiant cell aortitis.

A 32 year old lady with a history of systemic lupus erythematosus (SLE ) complicated by crescentic lupus nephritis and end stage renal failure on thrice weekly haemodialysis, presented to the emergency department with breathlessness, chest pain and haemoptysis, stating that her symptoms had started roughly 24 hours earlier. She appeared anxious and in distress. BP was 160/105 mmHg, heart rate 105/min and there was evidence of bilateral rales and a gallop rhythm. She was afebrile but only 94% saturating on high flow oxygen.ECG showed left ventricular hypertrophy and non-specific changes, chest X-ray demonstrated bilateral patchy infiltrates and cardiomegaly(figure 1). White cell count was 13.7 K, CRP 2.1, Creatinine 8. A presumptive diagnosis of basal variant Taku tsubo cardiomyopathy was made and the patient admitted to the intensive care unit. She was treated by emergent haemodialysis and was commenced on betablockade in the form of Carvedilol. She demonstrated rapid clinical improvement. She declined invasive workup with coronary angiography. There were no significant serial EKG changes.Repeat echocardiography (Video 3&4)was performed 48 after presentation and demonstrated normalisation of LV systolic function as well marked improvement in diastolic parameters ( Figure 3 ).Speckle tracking echo also showed marked improvement in comparison with the echo on presentation (Figure 4). At this stage, the patient insisted on discharge and she has remained well at follow up. In retrospect, she admitted that she had become distraught with a work colleague during an argument immediately before her symptoms commenced. The rapid recovery of LV function and the non-coronary distribution of LV dysfunction excludes other aetiologies such as coronary embolism, plaque rupture or dissection.The echocardiogram shows classic basal type taku tsubo cardiomyopathy(TTC), with preserved apical function. Four distinct variants of TTC have been described. Basal type TTC is rare and reported in 2.2% of TTC cases (39 of 1750 cases) in the international TTC registry 1 . Apical type is the commonest 81.7%;