Published online Feb 6, 2021. doi: 10.12998/wjcc.v9.i4.992
Peer-review started: November 17, 2020
First decision: November 24, 2020
Revised: December 8, 2020
Accepted: December 17, 2020
Article in press: December 17, 2020
Published online: February 6, 2021
Processing time: 69 Days and 6.1 Hours
Interrupted aortic arch (IAA) is a rare congenital heart disease defined by an interruption of the lumen and anatomical continuity between the ascending and descending major arteries. It is usually found within a few hours or days of birth. Without surgery, the chances of survival are low. If IAA patients have an effective collateral circulation established, they can survive into adulthood. However, IAA in adults is extremely rare, with few reported cases.
A 27-year-old woman presented with a 6-year history of progressively worsening shortness of breath and chest tightness on exertion. She had cyanotic lips and clubbing of the fingers. A transthoracic echocardiogram revealed an enlarged heart and dilation of the main pulmonary artery. There was an abnormal 9 mm passage between the descending aorta and pulmonary artery. The ventricular septal outflow tract had a 14 mm defect. Doppler ultrasound suggested a patent ductus arteriosus and computed tomographic angiography showed the absence of the aortic arch. The diagnoses were ventricular septal defect, patent ductus arteriosus, and definite interruption of the aortic arch. Although surgical correction was recommended, the patient declined due to the surgical risks and was treated with medications to reduce pulmonary artery pressure and treat heart failure. Her condition has been stable for 12 mo of follow-up.
Although rare, IAA should be considered in adults with refractory hypertension or unexplained congestive heart failure.
Core Tip: Interrupted aortic arch (IAA) is a rare congenital heart defect that is typically diagnosed shortly after birth and likely to be fatal without surgical correction. There are few reports of new IAA diagnoses in adults. In order to compensate for insufficient blood supply to the systemic circulation, IAA patients usually have abundant collateral circulation. This case describes an adult patient who survived due to a ventricular septal defect and a patent ductus arteriosus that allowed some oxygenated blood to be pumped into the descending aorta. IAA should be considered in adults with refractory hypertension or unexplained congestive heart failure.