Isolated interrupted aortic arch in an adult:A case report

BACKGROUND 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.CASE SUMMARY 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.CONCLUSION Although rare,IAA should be considered in adults with refractory hypertension or unexplained congestive heart failure.

Difference and similarity between type A interrupted aortic arch and aortic coarctation in adults:Two case reports

BACKGROUND Aortic coarctation(CoA)is usually confused with interrupted aortic arch(IAA),especially adult type A interrupted aortic arch,due to their similar anatomical location.Although the main difference between them is whether arterial lumen exhibits continuity or not,the clinical manifestations are similar and connection exists between them.Adult type A IAA is considered as an extreme form of CoA,which is complete discontinuity of aortic function and lumen caused by degenerative arterial coarctation.This paper reports two cases(interrupted aortic arch and severe aortic coarctation)to analyze the difference and similarity between them.CASE SUMMARY The two cases of patients presented with hypertension for many years.Computed tomography angiography showed that the aortic arch and descending aorta were discontinuous or significantly narrowed with extensive collateral flow.The IAA patient refused surgical treatment and blood pressure could be controlled with drugs.While the CoA patient underwent stent implantation because of uncontrollable hypertension,the blood flow recovered smoothly and the blood pressures at both ends of the stenosis returned to normal after surgery.CONCLUSION Adult type A IAA and CoA have difference and similarity,and type A IAA is associated with CoA to a certain extent.The treatment method should be chosen based on the patient's clinical symptoms rather than the severity of the lesion.

Surgical treatment of interrupted aortic arch associated with ventricular septal defect and patent ductus arteriosus in patients over one year of age

Background Interrupted aortic arch （IAA） is a rare congenital anomaly affecting 1.5％ of infants with congenital heart disease.Neonatal repair of IAA is required to avoid irreversible pulmonary vascular lesion.However,in China,patients with IAA associated with ventricular septal defect （VSD） and patent ductus arteriosus （PDA） over one year of age are common.So we investigated the outcome of surgical treatment of IAA with VSD and PDA in patients over one year of age.Methods From January 2009 to December 2012,19 patients with IAA have undergone complete single-stage repair.The patients＇ mean age was 4.4 years,ranging 1 to 15 years; and their mean weight was 12.8 kg,ranging 4.2 to 36.0 kg.Fifteen IAA were type A,four were type B.Preoperative cardiac catheterization data were available from all patients.Mean pulmonary arterial pressure （mPAP） and pulmonary vascular resistance （PVR） were measured.The measurements of postoperative pulmonary artery pressure were taken in the operating room at the end of the case.All patients underwent echocardiographic examinations before discharged from the hospital.In addition,cardiac catheterization and echocardiographic examinations were performed during follow-up.Selective brain perfusion through the innominate artery during aortic arch reconstruction was used in all patients.Mean follow-up was （1.6±0.8） years.Results There were two hospital deaths （2/19,11％）.One patient died of pulmonary hypertension crisis,and another died of postoperative low cardiac output.Five cases had other main postoperative complications but no postoperative neurologic complications.Seventeen survivors were followed up,and there were no late deaths or reoperation.Mean cross-clamp duration was （85±22） minutes and selective brain perfusion duration was （34±11） minutes.Two patients required delayed sternal closure at two days postoperatively.Intensive care unit and hospital stays were （9±8） days and （47±24） days,respectively.Pressure gradients across the anastomosis at most recent follow up were less than 22 mmHg.mPAP regressed significantly from preoperative （62.1±8.1） mmHg to postoperative （37.3±11.3） mmHg （P ＜0.001） and （24.2±6.0） mmHg at six months after discharged from the hospital （P ＜0.001）.The pulmonary vascular resistance also regressed significantly from preoperative （1 501.4±335.7） dyn·s·cm-5 to （485.0±215.1） dyn·s·cm-5 at six months after discharged from the hospital （P ＜0.001）.The majority of the seventeen patients （89％） were in New York Heart Association （NYHA） class Ⅰ,and 11％ remained in NYHA class Ⅱ.Conclusions Single-stage repair of patients with IAA,VSD and PDA over one year of age can have good surgical results and functional outcomes.Assessment and treatment of pulmonary artery pressure pre-operatively and postoperatively was crucial.mPAP and pulmonary vascular resistance may have regress significantly compared to preoperative values.

Diagnostic Value of Prospective Electrocardiogram-triggered Dual-source Computed Tomography Angiography for Infants and Children with Interrupted Aortic Arch

Background：Accurate assessment of intra-as well as extra-cardiac malformations and radiation dosage concerns are especially crucial to infants and children with interrupted aortic arch （IAA）.The purpose of this study is to investigate the value of prospective electrocardiogram （ECG）-triggered dual-source computed tomography （DSCT） angiography with low-dosage techniques in the diagnosis of IAA.Methods：Thirteen patients with suspected IAA underwent prospective ECG-triggered DSCT scan and transthoracic echocardiography （TTE）.Surgery was performed on all the patients.A five-point scale was used to assess image quality.The diagnostic accuracy ofDSCT angiography and TTE was compared with the surgical findings as the reference standard.A nonparametric Chi-square test was used for comparative analysis.P＜0.05 was considered as a significant difference.The mean effective radiation dose （ED） was calculated.Results：Diagnostic DSCT images were obtained for all the patients.Thirteen IAA cases with 60 separate cardiovascular anomalies were confirmed by surgical findings.The diagnostic accuracy of TTE and DSCT for total cardiovascular malformations was 93.7％ and 97.9％ （P＞0.05）,and that for extra-cardiac vascular malformations was 92.3％ and 99.0％ （P ＜ 0.05）,respectively.The mean score of image quality was 3.77 ± 0.83.The mean ED was 0.30 ± 0.04 mSv （range from 0.23 mSv to 0.39 mSv）.Conclusions：In infants and children with IAA,prospective ECG-triggered DSCT with low radiation exposure and high diagnostic efficiency has higher accuracy compared to TTE in detection of extra-cardiac vascular anomalies.

Interrupted aortic arch diagnosed by ECG-gated multi-slice computed tomography angiography: a case report

Interrupted aortic arch （IAA） is a rare congenital cardiovascular disease with major intracardiac defects and always with multisystem non-cardiac malformations. It occurs in 1 ： 10,000 births, and about 1% of the patients with congenital heart defects. Therefore, it is important that we make a good diagnosis and accurate evaluation of their morphologic conditions before a heart operation. We presented a case of IAA diagnosed by ECGgated multi-slice computed tomography （MSCT） angiography.

Aorta-pulmonary septal defect and aortic origin of the right pulmonary artery with interruption of the aortic arch:a clinical analysis of 5 cases

Objective Reviewing the experience in diagnosis and treatment of cases with aorta-pulmonary septal defect and aortic origin of right pulmonary artery complicated with interruption of aortic arch. Methods Clinical data from medical records for 5 patients (4 boys and 1 girl,age from 1 to 18 years on admission) who had been treated in Wuhan Asia Heart Hospital between 2005 and 2009