Interrupção do arco aórtico como causa de insuficiência cardíaca em adulto jovem

Abstract

The interrupted aortic arch (IAA) is a rare cardiopathy, with high morbimortality when treatment is delayed. This study is a case of IAA with atypical clinical behavior in a 19-year-old patient. The history and the clinical assessment were compatible with recent heart failure, associated to syncope episodes and reduction of pulses in the lower limbs. The electrocardiogram showed biventricular and biatrial overload. The echocardiogram showed severe ventricular dysfunction and altered blood flow in the descending aorta. The angiotomography disclosed occlusion of the thoracic aorta after the left subclavian artery. The attained diagnosis was interrupted aortic arch and surgical treatment was indicated. preceding six months. A few days before the first clinical visit, he had presented 3 syncope episodes and worsening of the dyspnea as well as of the edema. Prior to symptom onset, he was asymptomatic with good weight and height development and denied personal or family morbid antecedents. At the physical examination, he presented clinical signs that were suggestive of heart failure (LLLL edema, enlarged liver and jugular turgescence), with no other alterations at the ectoscopy. The pulses were normal in the upper limbs (UULL), BP was 140/90 mmHg in both limbs, whereas the LLLL presented reduced amplitude, with BP = 90/50 mmHg in the right lower limb (RLL) and 100/60mmHg in the left lower limb (LLL). Interrupted Aortic Arch with Cardiac Heart Failure in Young AdultThe 12-derivation ECG and the chest x-ray showed overload of the four chambers and increased cardiac area of 2+/4+ on account of the left ventricle (LV). Myocardial dysfunction was confirmed by the Doppler echocardiogram, with a LV ejection fraction (LVEF) of 22% (Simpson's biplane) and slight dilatation; the right ventricle showed moderate dilatation with diffuse severe hypokinesis, with a pulmonary systolic pressure of 76 mmHg in addition to suggestive signs of aortic obstruction after the left subclavian artery.An angiotomography of the aorta was then requested (Figures 1 and 2), which disclosed aortic occlusion after the origin of the left subclavian artery, with an extension of approximately 3 cm, associated to an extensive collateral network that retrogradely filled the descending and iliac aortas, which had normal diameters, consistent with the patient's age. The clinical and radiological findings pointed to a diagnosis of aortic arch interruption type A, according to the classification of Celoria and Patton 4 , with severe myocardial dysfunction.The patient was submitted to the surgical correction on April 19. A lateral thoracotomy was performed, with the insertion of an 18-mm polytetrafluoroethylene tube between the left subclavian artery and the descending aorta, with the need for extracorporeal circulation.The echocardiogram performed on the 4 th postoperative (PO) day showed a LVEF of 40% with slight hypertrophy and dilatation; the maximum systolic gradient between the left subclavian artery and the descending aorta was 8 mmHg and the pulmonary s...