Interrupted aortic arch (IAA) is a rare congenital abnormality with only a few cases reported in adults. It is defined as complete loss of continuity between the ascending and descending portions of the aorta, and is usually associated with other cardiac defects. The diagnosis in adults should be suspected in the presence of refractory hypertension, a careful physical examination being crucial to early diagnosis. Magnetic resonance angiography (MRA) techniques can accurately characterize cardiovascular anatomy, and also provide information regarding heart chamber and valve function.
LEARNING POINTS• Although rare, interrupted aortic arch (IAA) must be considered in the differential diagnosis of adults with refractory hypertension. A careful physical examination, with evaluation of femoral pulses and blood pressure in the four limbs, is essential for the diagnosis and for ensuring that the correct diagnosis is made promptly. • This case highlights the value of magnetic resonance angiography (MRA) in radiation-free non-invasive evaluation of adults with congenital aortic abnormalities, as it may provide clinically important haemodynamic information.• Only a few cases have been reported in adults. We present an unusual case in which the diagnosis was not made until adulthood.
KEYWORDSInterrupted aortic arch, refractory hypertension, bicuspid aortic valve, aortic coarctation BACKGROUND Interrupted aortic arch (IAA) is a rare congenital abnormality defined as a complete loss of anatomical continuity between the ascending and descending portions of the aorta. Up to 98% of cases are associated with additional cardiovascular anatomical defects [1]. Only few cases of IAA have been reported in adults
Patients with respiratory indications for ECMO experienced better survival than cardiac patients. The need for more inotropic drugs was a predictor of mortality in VA-ECMO. This is the first published record of the overall experience with ECMO in a Portuguese tertiary hospital.
Takotsubo cardiomyopathy (TC) is characterized by the sudden onset of reversible left ventricular dysfunction, with a presentation similar to that of an acute coronary syndrome. Although cardiogenic shock is a rare occurrence in TC, if it does occur it may require the use of a left ventricular assist device. We report the use of extracorporeal life support (ECLS) in a patient with TC and refractory cardiogenic shock. With ECLS it was possible to reduce inotropic support, and a normal left ventricular ejection fraction was documented by echocardiography on day 2. This is, to our knowledge, the first reported case of TC with refractory cardiogenic shock treated with ECLS in Portugal.
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