Adult interrupted aortic arch revealed by resistant arterial hypertension

Introduction

Interrupted aortic arch (IAA) is a rare congenital heart condition where there is a complete discontinuation between the ascending and descending aorta. The association with a patent ductus arteriosus or developed arterial collateral allows survival until adulthood in extremely rare cases. Transthoracic echocardiography is helpful for the diagnosis, but the exact interruption site and collateral vessels are more accurately highlighted by computed tomography angiography (CTA) and magnetic resonance imaging (MRI).

Results/Expected results

A 50 year-old-patient with a history of hypertension was admitted for severe high blood pressure. The main functional symptom that prompted his consultation was a grade II NYHA dyspnea evolving for few weeks. Physical examination found a blood pressure of 190/110 mmHg at the right arm, 120/70 mmHg at the left arm, and 110/70 mmHg at lower limbs. Cardiac and pulmonary auscultation were normal. The electrocardiogram showed left ventricular hypertrophy. Transthoracic echocardiography revealed severe left ventricular hypertrophy with good left ventricular function and dilatation of the ascending aorta measured at 47 mm. The suprasternal notch view suggested a type B IAA. CTA confirmed the diagnosis, exhibiting an IAA located between the left carotid and left subclavian artery with an arterial collateral network emerging from descending branch of the transverse cervical artery (Figure 1).

Conclusion/Perspectives

Adult forms of IAA are a rare and complex manifestation of a congenital heart condition. They present significant challenges due to the severity of symptoms and high operative risk involved in treatment. An accurate and timely diagnosis is essential and can be achieved through a combination of echocardiography and advanced imaging techniques such as CTA or MRI angiography. A multidisciplinary approach involving a team of specialists is crucial for optimizing the management of these patients.