A rare combination of cardiovascular anomaly: Supravalvular aortic stenosis and congenital absence of right coronary artery

We reported a case of a 3-month-old infant presented with supravalvular aortic stenosis with congenital right coronary artery deficiency. According to cardiovascular CT results, Doty technique was adopted to restore the aortic root geometry under cardiopulmonary bypass. An angioplasty was performed to establish right coronary blood flow at the same time. The patient had no abnormal cardiac symptoms after surgery.

Cause of Ascending Aortic Dilatation in Patients with Bicuspid Aortic Valves: The Final Link

Background This article discusses about the cause of dilatation of the ascending aorta in patients with congenitally bicuspid aortic valves (CBAVs). Some members of the profession believe that it is genetic, while others attribute it to turbulence. The author previously presented in vitro data proving that CBAVs are inherently morphological stenotic, even in the absence of measurable gradient and clinical symptoms. This article reinforces the view that the dilatation of the ascending aorta in patients with CBAV represents a “poststenotic dilatation” by demonstrating that the structure of the aortic wall is normal in infants who have CBAV as a sole cardiac anomaly. Methods The aortic wall was studied in newborns who had CBAV as the sole cardiovascular anomaly and in those where CABV was associated with other inborn cardiovascular defects. Results We found that in patients where CBAV is the sole cardiovascular anomaly, aortic structure is normal at birth, but abnormal if additional cardiovascular anomalies are present. Conclusions Dilatation of the ascending aorta in patients, where CBAV is the only cardiovascular anomaly, is caused by turbulence (poststenotic dilatation). If additional cardiovascular anomalies are present, this process may be exaggerated by genetic abnormalities of the aortic wall.

Stenting Coarctation of the “Fifth Aortic Arch”: A Safe and Attractive Therapeutic Alternative to Surgery

“Persistence of the fifth aortic arch” is a rare congenital cardiovascular anomaly that consists of an abnormal vessel arising from the distal ascending aorta connecting with the systemic or pulmonary circulation. We report a case of a type A interruption of the aortic arch and a coarctation of the fifth aortic arch, which connected the ascending with the descending aorta. No cardiac surgery was required because a covered stent was successfully implanted in the fifth aortic arch when the patient was 4 years old. A chromosome 9 q arm duplication of uncertain significance was also found, an anomaly never described before in this clinical context.

A Neonate with an Unusual Midline Defect and Cardiovascular Anomaly

We present a female neonate with a sternal cleft (SC) and additional aortic aneurysm who presented with respiratory failure. Stabilization of the SC was achieved by using the xyphoid process as an autologous graft bridging the upper part of the SC. We conclude that a step-wise correction of the SC with the use of an autologous graft may improve respiratory function, and should be considered when complete surgical correction is not feasible.