Aortic regurgitation is not a rare complication of Takayasu's disease. Aortic regurgitation may aggravate cerebral ischemic syndrome like syncope in patients with stenotic or occlusive lesions in cerebral branches of aorta secondary to acute or progressive inflammation. In a 34-yrs-old male patient who complained of syncope and exertional dyspnea with occlusion of both carotid arteries and severe stenoses of both subclavian arteries, occlusion of right coronary artery, and aortic regurgitation, his symptom was improved with perioperative aggressive steroid therapy, stent insertion in both subclavian arteries, and aortic valve replacement.
Aorta ; Aorta, Thoracic* ; Aortic Arch Syndromes* ; Aortic Valve Insufficiency ; Aortic Valve* ; Carotid Arteries ; Constriction, Pathologic ; Coronary Vessels ; Dyspnea ; Humans ; Immunosuppression ; Inflammation ; Male ; Stents ; Subclavian Artery ; Syncope ; Takayasu Arteritis*

The purpose of this study was to evaluate the value of multi-detector computed tomography (MDCT) angiography for the diagnosis of congenital aortic arch anomalies and present the radiological images of congenital aortic arch anomalies in Chinese children. MDCT angiography and transthoracic echocardiography (TTE) were applied for the diagnosis of congenital aortic arch anomalies in 362 Chinese children between May 2006 and December 2011 (age ranges from 5 days to 12 years; mean age, 3.3 years). Surgery and/or catheter angiography (CA) were conducted in all patients to confirm the final diagnosis. In the 362 Chinese children with congenital heart anomalies, congenital aortic arch anomalies were definitely diagnosed in 198 children and 164 children ruled out by operation and/or (CA). Among the 198 children with anomalies, coarctation of aorta (CoA), interruption of aortic arch (IAA), right aortic arch, aberrant right subclavian artery and double aortic arch were diagnosed in 134, 32, 20, 10 and 2 children respectively, and there were 6 cases with uncommon congenital aortic arch anomalies: 2 had double aortic arch including 1 with five branches of the aortic arch, 2 had isolation of the right subclavian artery with two patent ductus arteriosus (PDA), 1 had an isolation of the common carotid artery with a PDA, and 1 had double PDA with a single ventricle and pulmonary artery atresia. Among the 32 children with IAA, 28 were of type A, and 4 were of type B. The diagnostic sensitivity, specificity and accuracy of MDCT angiography for congenital aortic arch anomalies were 100% (198/198), 98% (161/164) and 99% (359/362), respectively. The diagnostic sensitivity, specificity and accuracy of TTE were 92% (182/198), 81% (133/164) and 87% (315/362), respectively. In conclusion, MDCT angiography is a reliable, noninvasive imaging technique for the diagnosis of congenital aortic arch anomalies in children. Sometimes, even more information can be obtained from this technique than from conventional angiography.
Abnormalities, Multiple ; diagnostic imaging ; Aorta, Thoracic ; abnormalities ; diagnostic imaging ; Aortic Arch Syndromes ; diagnostic imaging ; Aortography ; methods ; Child ; Child, Preschool ; China ; Female ; Humans ; Infant ; Infant, Newborn ; Male ; Multidetector Computed Tomography ; methods ; Reproducibility of Results ; Sensitivity and Specificity ; Tomography, X-Ray Computed ; methods

OBJECTIVETo evaluate one-stage arterial Switch operation for transposition of the great arteries (TGA) and Taussig-Bing with aortic arch obstruction.

METHODSFrom January 2001 to June 2004, 8 patients had aortic arch obstruction, 3 with TGA and 5 with Taussig-Bing. Except one patient was 8 months old, all of others were 5 days to 3 months old, the mean operation age was (40 +/- 36) d and the mean weight was (4.3 +/- 0.5) kg. All patients were repaired by one-stage operation. The aortic arch obstruction was repaired in deep hypothermia circulatory arrest, and arterial switch procedure was performed in deep hypothermia and low flow perfusion.

RESULTSThere had 1 death who was 8 months old and had low cardiac output, complete artrioventricular block (AVB) and severe pulmonary hypertension postoperation. One patient was 3 months old who had asphyxia at 5 days postoperatively. Six patients followed up from 5 months to 2 years. One Taussig-Bing with interrupted aortic arch had residual obstruction at the anastomosis of aorta. Two had trivial aortic valve regurgitation, and one had mild pulmonary valve regurgitation.

CONCLUSIONSOne-stage repair for TGA and Taussig-Bing with aortic obstruction achieves excellent results. The reasons for the death were pulmonary hypertension and abnormal coronary artery. The operative procedure should be performed as early as possible for the better result.

Aorta, Thoracic ; surgery ; Aortic Arch Syndromes ; complications ; surgery ; Cardiopulmonary Bypass ; Cardiovascular Surgical Procedures ; methods ; Double Outlet Right Ventricle ; complications ; surgery ; Female ; Follow-Up Studies ; Humans ; Infant ; Infant, Newborn ; Male ; Transposition of Great Vessels ; complications ; surgery ; Treatment Outcome