The Impact of a Bicuspid Aortic Valve on Aortic Geometry and Function in Patients with Aortic Coarctation:A Comprehensive CMR Study

Background:An isolated bicuspid aortic valve(BAV)is associated with structural and functional abnormalities of the aorta and the left ventricle(LV).Although~50%of patients with aortic coarctation(CoA)have a BAV,less is known about its impact on LV function and aortic geometry and function in CoA patients.In this cardiovascular magnetic resonance imaging(CMR)study,we analysed markers of LV and aortic function as well as aortic geometry in a large cohort of CoA patients with a BAVand compared them with CoA patients with a tricuspid aortic valve(TAV).Methods:We included 48 patients with a BAV(18.4±9.3 years)and 45 patients with TAV(20.7±9.9 years).LV volumes,mass and ejection fraction as well as aortic distensibilty,pulse wave velocity(PWV)were measured from standard cine CMR and phase-contrast CMR images.2-dimensional CMR feature tracking(2DCMR-FT)was performed to measure longitudinal,circumferential and radial strain and strain rate of the LV.Aortic arch geometry was classified as romanic,gothic and crenel.Results:LV volumes,mass and ejection fraction as well as aortic distensibility and PWV did not significantly differ between the BAV and the TAV group.There was also no significant difference for LV global longitudinal,radial and circumferential strain and strain rate between both groups.Patients with a BAV had more commonly a gothic aortic arch compared to TAV patients,but this difference was not statistically significant(22 vs.14,p=0.2).Ascending and descending aortic distensibility correlated with LV mass in the entire patient group(p<0.001).Global longitudinal,circumferential and radial strain(GLS,GCS,GRS)and global longitudinal and circumferential strain rate(GLSR,GCSR)correlated with LV ejection fraction(p<0.001).Conclusion:Our data suggest that the presence of a BAV does not adversely impact LV and aortic function in children and young adults with CoA.The correlation of global circumferential,longitudinal and radial strain values with LV ejection fraction demonstrates that 2D-CMR-FT might provide additional information related to ventricular function in CoA patients.

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.

Single-Stage Repair of a Critical Aortic Coarctation, a Bicuspid Aortic Stenosis and an Ascending Aortic Aneurysm

We report a 26-year-old man with critical aortic coarctation, severe bicuspid aortic valve stenosis, infective endocarditis and ascending aortic aneurysm. He underwent simultaneously in singlestage a Bentall’s procedure and an extra-anatomic ascending-descending aortic bypass grafting by 14-mm Dacron tube, through median sternotomy. The immediate postoperative outcome was favourable. The CT scan control for 7 years after surgery showed a good patency of the extra-anatomic bypass.

Computational Fluid Dynamics Analysis of the Aortic Coarctation

In this work, we first investigated the hemodynamic parameters in the case of a normal aortic arch anatomy and in the case of aortic coarctation anatomy, both generated by using CFX-ICEM-ANSYS simulations. Then, we compared these results with those obtained for a proposed model without and with aortic coarctation, while introducing a real tridimensional magnetic resonance imaging geometry in the simulation process. The conclusion is that our proposed model reproduces, with a high agreement, the real case obtained from imaging data.

Percutaneous Balloon Angioplasty for Severe Native Aortic Coarctation in Young Infants Less Than 6 Months： Medium- to Long-term Follow-up

Background： Although balloon angioplasty （BA） has been performed for more than 20 years, its use as a treatment for native coarctation of the aorta （CoA） during childhood, especially in young infants, remains controversial. This study aimed to assess the effects and potential role ofpercutaneous transcatheter BA for native CoA as an alternative therapy to surgical repair in young infants. Methods： The 37 patients aged from 6 days to 6 months with severe CoA in congestive heart failure or circulatory shock were admitted for BA. Patient＇s weight ranged from 2.4 to 6.1 kg. All 37 patients were experiencing cardiac dysfunction, and eight patients were in cardiac shock with severe metabolic acidosis. Eleven patients had an isolated CoA, whereas the others had a CoA associated with other cardiac malformations. Cardiac catheterization and aortic angiography were performed under general anesthesia with intubation. Transfemoral arterial approaches were used for the BA. The size of the balloon ranged from 3 mm × 20 mm to 8 mm × 20 mm, and a coronary artery balloon catheter was preferred over a regular peripheral vascular balloon catheter. Results： The femoral artery was successfully punctured in all but one patient, with that patient undergoing a carotid artery puncture. The systolic peak pressure gradient （PG） across the coarctation was 41.0 ± 16.0 mmHg （range 13-76 mmHg）. The mean diameter of the narrowest coarctation site was 1.7 ± 0.6 mm （range 0.5-2.8 mm）. All patients had successful dilation; the PG significantly decreased to 13.0 ± 11.0 mmHg （range 0-40 mmHg）, and the diameter ofcoarctation significantly improved to 3.8 ± 0.9 mm （range 2.5-5.3 mm）. No intraoperative complications occurred for any patients. However, in one case that underwent a carotid artery puncture, a giant aneurysm formed at the puncture site and required surgical repair. The following observations were made during the follow-up period from 6-month to 7-year： （1） The PG across the coarctation measured by echocardiography further decreased or remained stable in 31 cases. The remaining six patients, whose PGs gradually increased, required a second dilation. No patient required further surgery because of a CoA; （2） in two cases, an aortic aneurysm was found with an angiogram performed immediately postdilatation and disappeared at 18 and 12 months of age, respectively; （3） tricuspid regurgitation and pulmonary hypertension improved in all patients; （4） all patients were doing well and were asymptomatic. Conclusions： Percutaneous BA is a relatively safe and effective treatment for severe native CoA in young infants, and should be considered a valid alternative to surgery because of its good outcome and less trauma and fewer complications than surgery.

Extra-anatomic aortic bypass for treatment of atypical aortic coarctation

Background The conventional extra-anatomic bypass is originated from the axillary＇s artery and the graft size is often limited due to the small diameter of axillary＇s artery. ascending aorta can improve the graft size and distal perfusion, Extra-anatomic bypass graft originating from need sternotomy which might have higher operative risks compared with axillo-femeral bypass. We summarize our experiences of extra-anatomic bypass from ascending aorta for atypical aortic coarctation. Methods Between January 2005 and February 2008, 5 women aged from 18 to 64 years underwent extra-anatomic bypass from ascending aorta to abdominal aorta or iliac artery bypass for treatment of atypical aortic coarctation. Preoperatively, all patients had hypertension and needed antihypertensive medications. Systolic blood pressure was 151 ± 9 mmHg. Ankle pressure index （API） 0.23 in left and 0.56± 0.23 in right. Average systolic pressure gradient of aortic stenosis was 76 were 0.60 ± 18 mmHg. Three patients underwent concomitant cardiac operation, including coronary artery bypass grafting, Benta11 procedure and atrial septal defect repair. Results There was no hospital and late mortality during 58 ＋ 15 months follow-up （range from 44 to 81 months）. Postoperative systolic blood pressure was reduced to 126 ± 11 mmHg at the time of discharge. All patients maintained normal blood pressure without medication during follow-up. API was improved to 1.12 ± 0.24 in left and 1.17 ± 0.25 in right （compared with preoperative data, P 〈0.05）. Follow-up computer tomography showed patency in all grafts. Conclusions Surgical treatment of atypical aortic coarctation with extra-anatomic bypass originating from ascending aorta alleviates hypertension and low limb ischemia. IS Chin J Cardiol 2011; 12（4） ： 207-2113

Early and mid-term outcomes of Cheatham platinum stent for the native aortic coarctation

Background Aortic coarctation(CoA)is a common congenital cardiovascular defect. Until now,there is no unified conclusions about the therapeutic options of the native aortic coarctation(CoA)in adolescents and adults. Methods From May 2010 to April 2018,consecutive 23 patients(median age 25 years,range 15-57 years)with native CoA underwent endovascular repair by applying covered Cheatham platinum(CP)stent in our center. Related variables were retrospectively collected and analyzed. Results Ten cases were complicated with other congenital heart diseases. Hypertension was confirmed in 21(91.3%)patients. In these patients,thirteen cases(61.9%)had a normal blood pressure without drug treatment during follow-up.The systolic right arm blood pressure significantly decreased from a mean of 150.7±6.4 mmHg pre-operation to 128.0±12.5 mmHg discharge(P<0.001),and the diastolic pressure decreased from 82.0±10.0 to 73.1±6.8 mmHg(P<0.001). The diameter of the coarcted segment increased from 5.0±2.3 to 16.9±2.3 mm(P<0.001). The peak systolic gradient significantly decreased from 64.9±20.9 to 7.1 ±6.1 mm Hg(P<0.001). The median follow-up was 30 months(range 1 months to 7.9 years). Complications were found in 4 people within one month:1 suspected cardiovascular-related death,1 new aortic dissection,1 splenic infarction,and 1 path-related arterial occlusion.Conclusions Our limited experience demonstrated that stent implantation is a reliable technique for the management of native aortic coarctation in adolescents and adults. Our intermediate-term results seem encouraging,however,the early complications should be considered. A larger series of cases with a longer follow-up is needed to substantiate these results.

Atypical Coarctation of the Aorta Revealed by Arterial Hypertension in a 22-Year-Old Young Man

Non-isthmic coarctation of the aorta is a rare congenital malformation in adults. Arterial hypertension is a frequent circumstance of discovery. We reported the case of a 22-year-old Guinean man who had been followed for 5 years for hypertension. Clinically, he presented with hypertension of the upper limbs with a systolic pressure gradient of 100 mmHg. The diagnosis was confirmed by thoracic angioscan, which showed a 65.8% coarctation of the abdominal aorta. He was on triple antihypertensive therapy combining Atenolol 100 mg, Amlodipine 10 mg and Perindopril 10 mg. He is awaiting interventional treatment. His blood pressure is stable at around 140/90 mmHg.

Aortoplasty with pulmonary autograft patch for coarctation of the aorta combined with hypoplastic aortic arch in infant

Objective To discuss the operative techniques and results of coarctation resection plus aortoplasty with pulmonary autograft patch for coarctation of the aorta combined with hypoplastic aortic arch in infant. Methods Between May 2007 and Dec 2009,14 cases including 9 males and 5 females with coarctation of the aorta and hypoplastic aortic arch underwent coarctation resection plus aortoplasty with pulmonary autograft patch in

Coarctation of the aorta in twins with severe hypertension

Coarctation of the aorta(CoA)refers to the congenital coarctation near the isthmus,ductus arteriosus or ligamentum arteriosus,with an incidence of 5%–10%in children with congenital heart defects.[1]It can be isolated or coexisted with ventricular septal defects,subaortic stenosis,patent ductus arteriosus,and mitral aortic valve.[2]CoA has a poor prognosis.Without intervention,the median age of death with aortic coarctation is 38 years[3]and 75%death of those patients is at the age of 46 years.[4]Causes of death included congestive heart failure(26%),aortic rupture(21%),bacterial endocarditis(18%),and intracranial hemorrhage(12%).[5]Therefore,early diagnosis and optimal treatment are the key to improve the prognosis of this disease.The aim of this report is underlining the importance of early and accurate diagnose of CoA as a cause of systemic hypertension in young patients and also emphasizing the genetic factors of CoA in twins.

Angiograms of the Abdominal Aorta in a Patient of Middle Aortic Syndrome

A 42-year old woman of middle aortic syndrome presented with severe hypertension for 20 years, whose abdominal aorta angiography revealed long segment and nearly complete occlusion in the mid portion of abdominal aorta, and extensively enlarged collateral vascular supply to the lower portion of abdominal aorta. The pressures proximal and distal to stenosis were 185/110 and 95/70 mmHg, and the pressure gradient across the stenosis was 90/40 mmHg. After the operation of thoraco-abdominal bypass graft, the pressure difference between the upper and lower extremities eventually disappeared.

Coarctation of the Aorta with Aortic Arch Hypoplasia：Midterm Outcomes of Aortic Arch Reconstruction with Autologous Pulmonary Artery Patch

Background： Coarctation of the aorta （CoA） with aortic arch hypoplasia （AAH） is a relatively common congenital heart disease in clinical practice. Nonetheless, the corrective surgical technique for infants and children is a clinical problem that remains controversial. In this study, we sought to evaluate the surgical effects of aortic arch （AA） reconstruction with coarctation resection and aortoplasty with autologous pulmonary artery patch for infants and young children with CoA and AAH. Methods： Between January 2009 and December 2015, a total of 22 infants and young children with CoA and AAH who underwent coarctation resection and aortoplasty with autologous pulmonary artery patch were enrolled in this study. The median age of patients was 4.5 （Q1, Q3： 2.0, 14.0） months and the median body weight was 5.75 （Q1, Q3： 4.10, 9.38） kg. All patients were diagnosed with CoA and AAH, and concomitant cardiac anomalies were corrected in one stage. Perioperative and postoperative data were collected and analyzed using the paired sample t-test. Results： No perioperative deaths occurred. No residual obstruction was detected by echocardiography. The postoperative pressure difference across the repaired segment of CoA was 14.05 ± 4.26 mmHg （1 mmHg = 0.133 kPa）, which was smaller than the preoperative pressure difference （48.30 ± 15.73 mmHg; t = -10.119, P 〈 0.001）. The median follow-up time was 29.0 （Q1, Q3： 15.5, 57.3） months. There was no death during the follow-up period, and all patients experienced obvious clinical improvement. Only one child underwent subsequent aortic balloon angioplasty due to restenosis. Computed tomography angiography showed that the AA morphology was smooth, with no aortic aneurysm or angulation deformity. Conclusion： AA reconstruction with coarctation resection and aortoplasty with autologous pulmonary artery patch could effectively correct CoA with AAH, and the rate of reintervention for restenosis is low.

Important Newborn Cardiac Diagnostic Dilemmas for the Neonatologist and Cardiologist-A Clinical Perspective

Most congenital heart disease(CHD)is readily recognisable in the newborn.Forewarned by previous fetal scanning,the presence of a murmur,tachypnoea,cyanosis and/or differential pulses and saturations all point to a cardiac abnormality.Yet serious heart disease may be missed on a fetal scan.There may be no murmur or clinical cyanosis,and tachypneoa may be attributed to non-cardiac causes.Tachypnoea on day 1 is usually non-cardiac except arising from ventricular failure or a large systemic arteriovenous fistula.A patent ductus arteriosus(PDA)may support either pulmonary or systemic duct dependent circulations.The initially high pulmonary vascular resistance(PVR)limits shunts so that murmurs even from large communications between the systemic and pulmonary circulations take days/weeks to develop.At times despite expert input,serious CHD maybe difficult to diagnose and warrants close interaction between the neonatologist and cardiologist to reach a timely diagnosis.Such conditions include obstructed total anomalous pulmonary venous connections(TAPVC)and the need to distinguish it from persistent pulmonary hypertension in the newborn(PPHN)–the treatment of the former is surgical the latter medical.A large duct shunting right to left may overshadow a suspected hypoplastic aortic isthmus and/or coarctation.Is the right to left shunting because of severe aortic obstruction or resulting from a high PVR with little obstruction.The diagnosis of pulmonary vein stenosis(PVS)remains problematic often developing in premature infants with ongoing bronchopulmonary dysplasia(BPD),still being cared for by the neonatologist.While there are other diagnostic dilemmas including deciding the contribution of a recognised CHD in a sick neonate,this paper will focus on the above-mentioned conditions with suggestions on what may be done to arrive at a timely diagnosis to achieve optimal outcomes.

Protective effect of astragaloside IV on cardiac hypertrophy in rats and its mechanism

Objective:To investigate the effect of astragaloside IV on cardiac hypertrophy and its regulation on autophagy.Methods:Fifty male Sprague-Dawley rats were randomly divided into sham operation group and abdominal aortic coarctation group(AAC group).There were 10 rats in sham operation group and 40 rats in the AAC group.One week after the operation,there were 32 rats in AAC group,10 rats in sham group.AAC group was randomly divided into model group,low-dose astragaloside group,high-dose astragaloside group and rapamycin group,8 rats in each group.Rapamycin group was a positive autophagy contrast agent group.They were given the corresponding solvents once a day by gavage for six weeks.At the end of study,three rats were randomly selected from each group,left ventricular mass index(LVW/BW),cardiac mass index(HW/BW)and the content of hydroxyproline were measured.HE staining,masson staining and sirius red staining were used to observe the morphological changes of myocardium.The expression of LC3II,LC3I,Beclin1,AMPK and mTOR were detected by western blot.Results:Compared with the sham operation group,AAC group showed hypertrophy,LVW/BW,HW/BW,HYP and p-mTOR/mTOR were significantly increased(P<0.05),p-AMPK/AMPK,LC3II/LC3I,Beclin1 were significantly decreased(P<0.05).Compared with the model group,the low-dose astragaloside IV group showed the hypertrophy of cardiomyocytes was relatively light,LVW/BW and HW/BW were significantly decreased(P<0.05),there was no significant difference in HYP and p-mTOR/mTOR(P>0.05),LC3II/LC3I,Beclin1 and p-AMPK/AMPK were significantly increased(P<0.05).Compared with the model group,high-dose astragaloside IV group and rapamycin group showed reduced myocardial hypertrophy,LVW/BW,HW/BW,HYP and p-mTOR/mTOR were significantly decreased(P<0.05),LC3II/LC3I,Beclin1 and p-AMPK/AMPK were significantly increased(P<0.05).Compared with the low-dose astragaloside group,the high-dose astragaloside group showed reduced myocardial hypertrophy,there were significant differences in each index(P<0.05).Compared with rapamycin group,there was no obvious difference in morphology and structure of myocardial cells,LVW/BW,HYP and p-mTOR/mTOR were decreased(P<0.05),HW/BW and p-AMPK/AMPK had no significant difference(P>0.05),LC3II/LC3I and Beclin1 were increased in high-dose astragaloside group(P<0.05).Conclusion:As IV has protective effect on cardiac hypertrophy in a dose-dependent manner and its mechanism may be related to regulate autophagy.

Use of covered Cheatham-Platinum stent as the primary modality in the treatment for native coarctation of the aorta

Background Bare stent implantation in the treatment for native and recurrent coarctation of the aorta （CoA） has become established as an alternative to surgery and balloon angioplasty. However, this modality still encounters significant complications during the procedure and/or follow-up. The covered Cheatham-Platinum （CP） stent commonly used to be chosen as a rescue treatment in these patients. The purpose of this study was to evaluate the use of covered CP stent as the primary modality in the treatment for native CoA. Methods Twenty-five covered CP stents and 2 bare CP stents were implanted in 25 patients with native CoA. All patients after the intervention were invited for follow-up examinations. Results The peak systolic gradient across the lesion decreased significantly from a median value of 67.5 mmHg （quartile range, 19.3 mmHg） to 2 mmHg （quartile range, 4.0 mmHg） （P 〈0.0001）. Stenotic segment diameter increased from a median value of 5.0 mm （quartile range, 1.5 mm） to 17.9 mm （quartile range, 2.5 mm） （P 〈0.0001）. The median ratio of diameter of the coarctation postprocedure to preprocedure was 4.2 （quartile range, 1.6）. All of the CP stents were placed in the suitable position without any acute complications. During a follow-up period of up to 72 months, no complications were encountered. Most of the patients （21/25） were normotensive, apart from four patients requiring antihypertensive medication during the follow-up. Conclusion The implantation of covered CP stent as the primary modality is safe and effective in the treatment for native CoA in adolescents and adults.