A double-barrelled aorta with high aortic Arch.

A double-barrelled aorta was detected in a female newborn with 22q11.2 deletion syndrome. Double-barrelled aorta had been previously described as persistence of the fifth pharyngeal arch, but its existence continues to be debated. Recent embryologic studies suggest that double-barrelled aorta is more likely explained by other developing processes in the majority of cases. In our case, catheter angiography confirmed the presence of the high aortic arch and double-barrelled aorta. The upper lumen was located above the level of the clavicles. These findings suggested that the persistence of the segment of dorsal aorta between the third and fourth embryonic arches and the double-barrelled aorta was more likely a consequence of persistence of the third and fourth pharyngeal arches. Detailed imaging and embryologic considerations played an important role in accurate assessment of the origin of the double-barrelled aorta. .

Low-dose corticosteroid therapy improves refractory coronary vasospasm accompanied by eosinophilic granulomatosis with polyangiitis.

A 57-year-old man was admitted to our hospital due to repeated chest pain. Coronary spastic angina was diagnosed by emergent coronary angiography. His chest attack was not suppressed with vasodilator therapy; however, it finally improved after administration of 20 mg prednisolone. His symptoms were controlled and elevation of the eosinophil count was normalized, even after tapering the dosage. His episodes of asthma, hypereosinophilia, mononeuropathy, and pulmonary infiltrate led to a diagnosis of eosinophilic granulomatosis with polyangiitis. .

A rare case of truncus arteriosus Van Praagh type A3: Prenatal diagnosis and postnatal management.

Truncus arteriosus (TrA) type A3, according to the Van Praagh (VP) classification, cannot be classified under the Collett and Edwards (C-E) system. In this rare anomaly, postnatal closure of the ductus arteriosus can cause unilateral pulmonary artery obstruction; hence, prenatal diagnosis and early confirmatory postnatal diagnosis are important. This case was referred to our hospital for suspected fetal heart disease at 29 weeks' gestation. TrA C-E type 1 was diagnosed by fetal echocardiography, with a right-sided aortic arch, absent inferior vena cava, and azygos continuation. The neonate was delivered vaginally at 41 weeks' gestation. Postnatal echocardiography showed a right-sided aortic arch with the right pulmonary artery originating from the common arterial trunk and the left pulmonary artery originating from the brachiocephalic artery. The diagnosis was TrA VP type A3, with a right-sided aortic arch and left-sided ductus arteriosus. Patency of the left-sided ductus arteriosus was maintained with prostaglandin E1.alpha-cyclodextrin. Right pulmonary artery banding was performed 3 days after birth. The Rastelli procedure was performed when the patient was 2 months old and weighed 4.2 kg. Delayed diagnosis of VP type A3 can cause unilateral pulmonary artery disconnection; hence, timely and accurate diagnosis is warranted to ensure stable disease management. .