Management of adults with coarctation of aorta

Coarctation of the aorta(CoA)is a relatively common congenital cardiac defect often causing few symptoms and therefore can be challenging to diagnose.The hallmark finding on physical examination is upper extremity hypertension,and for this reason,CoA should be considered in any young hypertensive patient,justifying measurement of lower extremity blood pressure at least once in these individuals.The presence of a significant pressure gradient between the arms and legs is highly suggestive of the diagnosis.Early diagnosis and treatment are important as long-term data consistently demonstrate that patients with CoA have a reduced life expectancy and increased risk of cardiovascular complications.Surgical repair has traditionally been the mainstay of therapy for correction,although advances in endovascular technology with covered stents or stent grafts permit nonsurgical approaches for the management of older children and adults with native CoA and complications.Persistent hypertension and vascular dysfunction can lead to an increased risk of coronary disease,which,remains the greatest cause of long-term mortality.Thus,blood pressure control and periodic reassessment with transthoracic echocardiography and threedimensional imaging(computed tomography or cardiac magnetic resonance)for should be performed regularly as cardiovascular complications may occur decades after the intervention.

Screening Coarctation of Aorta with Clinical and Echocardiographic Profiles in Infants:A Pilot Study

Aim:To determine the profiles of clinical features including four-limb blood pressure(BP),saturations of peripheral oxygen(SpO2),and echocardiographic features in infants with coarctation of aorta(CoA)to facilitate congenital heart diseases screening.Methods:The charts of infants with CoA were retrospectively reviewed.All in-hospital infants suspected of congenital heart diseases by clinical teams were prospectively measured of four limbs BPs and SpO2 in a regional cardiac transferring center during 2013 and 2019.Echocardiography as a gold standard test was followed within 2 days after suspicion.All infants were divided into non-significant CoA group or significant CoA group based on the difference of BPs between right arm and lower limbs.Predictors of nonsignificant CoA were determined with multivariable logistic regression.Results:One hundred thirty-three infants with CoA were identified.The BPs on upper limbs were higher than those on lower limbs(P=0.001).No statistical difference in SpO2 was found between four limbs.Fifty-three(39.8%)infants presented with significant CoA.Thirty-four infants presented with low SpO2 and 26 of 34 presented with non-significant CoA.Small ascending aorta diameter[0.070(95%CI:0.005–0.136),P=0.036]was an independent risk factors for non-significant CoA.Eighteen(13.5%)infants with CoA didn’t present with any of the BP difference,low SpO2,murmur,or weak femoral pulse.Conclusion:Less than half of the infants with CoA presented with a significant BP difference.Another one fifth presented with low SpO2.Small ascending aorta diameter was an independent factor for non-significant CoA.

Structural Changes of the Liver Vessels in Compensated and Decompensated Coarctation of Aorta in Experimental Studies

Our studies allowed us to establish that simulating coarctation of aorta in laboratory animals results in tone reduction of vessels carrying blood to the liver and from it and a subsequent development of their intima atrophy. In spite of that fact the number of vessels containing intimal musculature, muscularelastic sphincters and polypoid pulvini in the arterial bed flow increases and in the major outflowing veins, on the contrary, thinning of their muscular tori takes place. Appearance and functioning in the hepatic vessels of adaptive formations contributes to keeping up homeostasis at the microcirculatory level. With cardiac decompensation decreasing because of venous plethora, hypotonia and atrophy of the intima of the vessels responsible for the blood inflow to the liver and the outflow from it progress. Besides, the number of arteries and the rate of development of arteries with adaptive smooth muscle formations in the arteries, as well as in hepatic veins with muscular tori decreases dramatically. The reason of compensatory reaction failure is a long-term hypoxia leading to vessel and their adaptive formation sclerosis. The consequence of the exhaustion of the above-mentioned mechanisms is development of perisinusoidal fibrosis and depression of the transcapillary metabolism.

Overcoming Double Trouble in Single-Sitting: A Case of an 8-Year-Old Boy with Coarctation of Aorta and Patent Ductus Arteriosus

There are very few reports on successful management of young children with coexistence of two congenital heart defects, coarctation of aorta and patent ductus arteriosus. These lesions can be treated either simultaneously or sequentially. Herein, we present the first successful report of simultaneous balloon angioplasty and Amplatzer duct occluder device implantation in a single-sitting in an 8-year-old boy with severe aortic coarctation and patent ductus arteriosus. The coarctation gradient decreased from 70 mmHg to 10 mmHg and a complete closure of the ductus arteriosus was achieved without any complication. Fellow interventional cardiologists may consider single-stage transcatheter treatment in young children with combination of these two lesions.

Malposition of Stent a Rare Complication in Coarctation of Aorta Corrected with Extraanatomic Aortic Bypass

Mal-position of stent in coarctation of aorta is very rare but a major complication. Symptoms can worsen even more. We present here one such case where stenting done in some other institute in which we did an extra-anatomical bypass from ascending aorta to supracelial aorta successfully bypassing the coarct segment. This was an early approach without assistance of Cadio-pulmonary (CP) Bypass. We conclude that this procedure should be done in centres where experienced operator and cardiac surgery back up is present. This was a good approach without assistance of CP Bypass.

Coarctation of aorta with complete aortic occlusion

Survival to advanced age is exceptional in patients with unrepaired aortic coarctation.We report the case of an 81-year-old man with aortic coarctation and total occlusion who was othersise asymptomatic.Coarctation was suspected when a femoral-radial pulse delay was noted during his routine physical examination.A 70-mmHg systolic blood pressure gradient between the upper and lower extremities was detected.

Totally Occluded Coarctation of the Aorta in a Young Adult

We report the case of a 20-year-old female patient presenting with a totally occluded coarctation of the aorta. The patient was admitted for the evaluation of resistant grade III high blood pressure. The physical examination revealed a blood pressure gradient between the upper and lower limbs, absent femoral pulses, a diffuse continuous murmur over the chest, and hyperpulsatility of the carotid arteries and sternal notch. A transthoracic echocardiography outlined a narrowing in the caliber of the isthmic aorta without acceleration of flow or gradient. Thoracic CT angiography revealed a complete aortic coarctation with interruption of continuity between segment 3 and the descending aorta.

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.

Short-Term Outcomes of Two Surgical Techniques for the Treatment of Coarctation of the Aorta in Infants: Subclavian Flap Repair and Resection with Extended End-to-End Anastomosis Technique

Background: The aims of the study were to analyze the importance of two different surgical procedures, and to determine outcomes for neonates with coarctation of the aorta in two newly established centers. Methods: Outcomes of two different surgical repairs for coarctation of the aorta in 43 infants were evaluated retrospectively. The study was designed as a nonrandomized, cross-sectional study. The subclavian flap repair was applied to 22 patients (51%) and resection with extended end-to-end anastomosis technique to 21 patients (49%). After all operative survivors were followed up with a mean follow-up of 1.8 ± 0.8 years, data analyzed with t-test and the p value < 0.05 were considered statistically significant. Results: The overall mortality rate was 4.6%. Forty mmHg gradients were determined in a patient from Resection Group postoperatively in the fifteenth month. After the balloon angioplasty, the gradient decreased to 25 mmHg. The presence of ventricular septal defect (p = 0.094) was the only significant predictor of adverse short-term outcome among the associated cardiac defects analyzed. The first-year survival rate was 100% in both groups in isolated coarctation (p = 0.965), however;such rate was found as 100% and 93.25% in Waldhausen Group and Resection Group, respectively in complex coarctation (p = 0.294). Conclusions: Both the subclavian flap repair and resection with extended end-to-end anastomosis for coarctation of the aorta in infants provide excellent short-term outcomes with lower recurrence rates requiring surgery or angioplasty.

Coarctation of the aorta:Management from infancy to adulthood

Coarctation of the aorta is a relatively common form of congenital heart disease, with an estimated incidence of approximately 3 cases per 10000 births. Coarctation is a heterogeneous lesion which may present across all age ranges, with varying clinical symptoms, in isolation, or in association with other cardiac defects. The first surgical repair of aortic coarctation was described in 1944, and since that time, several other surgical techniques have been developed and modified. Additionally, transcatheter balloon angioplasty and endovascular stent placement offer less invasive approaches for the treatment of coarctation of the aorta for some patients. While overall morbidity and mortality rates are low for patients undergoing intervention for coarctation, both surgical and transcatheter interventions are not free from adverse outcomes. Therefore, patients must be followed closely over their lifetime for complications such as recoarctation, aortic aneurysm, persistent hypertension, and changes in any associated cardiac defects. Considerable effort has been expended investigating the utility and outcomes of various treatment approaches for aortic coarctation, which are heavily influenced by a patient's anatomy, size, age, and clinical course. Here we review indications for intervention, describe and compare surgical and transcatheter techniques for management of coarctation, and explore the associated outcomes in both children and adults.

NuMED CHEATHAM-PLATINUM STENT AND BALLOON IN BALLOON DELIVERY CATHETER FOR TREATMENT OF NATIVE COARCTATION OF THE AORTA

COARCTATION of the aorta (CoA) is a congenital heart defect involving a narrowing of the aorta.The narrowed segment called coarctation is most likely to happen in the segment just after the aortic arch.The narrowing can be removed by surgery or sometimes by a nonsurgical balloon dilation.However, aortic coarctation may recur even after successful surgery or balloon dilation.Fortunately, coarctation can now be treated with nonsurgical balloon dilation associated with implanting a stent using cardiac catheterization.Herein we reported our experience in a 19-year-old boy with CoA who underwent successful covered Cheatham-Platinum (CP) stent implantation for the coarctation.

Exercise Catheterization for Hemodynamic Evaluation of Adults with Coarctation of the Aorta

Background: Coarctation of the aorta (CoA) is associated with a generalized arteriopathy and long-term complicationsdespite repair. Data on invasive exercise hemodynamics in this population are lacking. Accordingly, wereviewed adults with CoA undergoing exercise catheterization to assess 1. hemodynamic profile;2. feasibilityfor assessment of CoA severity. Methods: Twenty patients undergoing exercise cardiac catheterization (12 armadduction and 8 supine cycle ergometry) at a quaternary care center between 2004 and 2021 were identified. Restingand exercise hemodynamic data were abstracted from the procedure logs. Results: Mean age was 43.6 ±12.0 years. Eleven patients (55%) had resting pulmonary arterial wedge pressure (PAWP) >15 mmHg;amongthose undergoing arterial catheterization, left ventricular end-diastolic pressure was >15 mmHg in 63%. Elevenpatients (55%) had pulmonary hypertension: 7 (35%) combined and 4 (20%) isolated post-capillary. At peak exercise,ΔPAWP/Δcardiac output (CO) ≥2 and Δmean pulmonary artery pressure/ΔCO ≥3 mmHg/l/min were foundin 7 (78%) and 6 (67%) patients, respectively;the composite of exercise PAWP ≥25 mmHg or ΔPAWP/ΔCO>2 was seen in 12 (86%). CoA peak-to-peak gradients at baseline (n = 14) and during exercise (n = 9) were 12(3–16) and 16 mmHg (9–28), respectively. Only 2 patients had an increase in CoA gradient to >20 mmHg withexercise. Conclusions: Diastolic dysfunction and pulmonary hypertension were highly prevalent, with exerciseunmasking abnormal diastolic and pulmonary vascular reserve in some individuals. Most patients failed to showsignificant increases in CoA peak-to-peak gradients with exercise. Further studies are warranted to establish thebest diagnostic method for CoA severity assessment.

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

When the Blood Pressure Misleads You:A Diagnostic Conundrum in an Unusual Case of Coarctation

A 4-month-old previously healthy baby was found to be in congestive heart failure with LV dysfunction and a right aortic arch with severe coarctation,undetectable by blood pressure measurements.A cardiac CT and central blood pressure led to the diagnosis of a unique anatomic variant of aortic coarctation.Once diagnosed the patient underwent surgery with an uncomplicated recovery.

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.

Fetal Endocardial Fibroelastosis with Coarctation of Aortic Arch: A Case Report

This study describes a 34-year-old pregnant woman who underwent prenatal echocardiography at 24 weeks of gestation and found fetal heart abnormalities. She underwent fetal echocardiography in our hospital. Echocardiography showed complex malformation of fetal heart, thickening and calcification of left ventricular myocardium and tendon, weakening or even flattening of left ventricular wall movement, very severe mitral stenosis, severe mitral regurgitation, aortic stenosis, narrowing of the aortic arch, countercurrent of the ductal arch to aortic arch, small diameter of the oval foramen and significantly increased flow velocity. The patient decided to induce labor after expert consultation, but no autopsy was performed due to his family’s refusal. Color Doppler ultrasound can find the positive signs of endocardial elastofibroplasia earlier, and accurately diagnose patients with neonatal elastofibroplasia. It provides accurate diagnostic information for the clinic and can be used as the first choice. This report has obtained the informed consent of its parents.

胎儿主动脉缩窄超声心动图诊断指标的综合评估

目的本研究旨在综合分析胎儿主动脉缩窄(coarctation of the aorta,CoA)的相关超声心动图指标。方法回顾性分析2018年1月至2023年5月广东省人民医院产前超声心动图检查疑诊CoA存活胎儿204例的临床资料,提取产前超声心动图数据和患者资料。根据生后随访结果将研究对象分为真阳性组(A组)和假阳性组(B组)。通过Logistic回归分析筛选预测指标并建立确诊CoA列线图预测模型,按6:4随机将患者分为训练集和测试集,进行模型内部验证及效果评价。结果204例胎儿被纳入研究,其中A组95例(46.5%),B组109例(53.43%)。最终确定4个指标组成列线图模型,包括左心室Z值、主动脉远端横弓Z值、肺动脉主干与升主动脉比值、是否合并室间隔缺损。该模型具有一定预测能力和实用价值[曲线下面积(area under the curve,AUC)=0.888],在验证集中亦取得了良好的诊断效能(AUC=0.825),内部验证显示模型具有良好的预测能力和应用价值。结论本研究建立的列线图模型可作为应用简单且有效的工具,提高CoA的产前诊断准确性,促进围产期及生后的分层管理。

经皮介入治疗主动脉缩窄合并二叶式或三叶式主动脉瓣术后左心室逆重构的临床研究

目的探讨主动脉缩窄(CoA)合并二叶式主动脉瓣(BAV)和三叶式主动脉瓣(TAV)行经皮介入手术后患者左心室逆重构的差异。方法回顾性分析2014年1月至2021年12月因主动脉缩窄于中国医学科学院阜外医院行经皮主动脉缩窄球囊扩张和支架置入术47例患者的临床资料。根据术前影像学资料合并BAV 18例,TAV 29例。对比患者术前和术后1年的超声心动图检查结果。结果与术前相比,经皮介入治疗后CoA患者术后1年的CoA Vmax、CoA PG、LVEDd、LVEDdi、LVM、LVMI均明显改善,23.4%患者出现了左室逆重构。合并BAV的患者AV Vmax、AV PG、LVEDdi均高于TAV组(P=0.005,P=0.007,P=0.03),BAV患者左室逆重构率低于TAV患者,但无统计学意义。多因素分析未发现影响术后1年左室逆重构的影响因素。结论部分CoA患者经皮介入手术术后1年发生左心室重构逆转。且合并BAV的患者改善情况亚于TAV患者,仍需要进一步探索。

血管转流联合TEVAR杂交技术治疗B型主动脉夹层的临床研究

目的分析颈-锁骨下动脉转流联合胸主动脉腔内修复术(TEVAR)杂交技术治疗B型主动脉夹层的临床疗效。方法选取2021年9月至2022年9月内蒙古自治区人民医院心脏大血管外科收治的45例B型主动脉夹层患者作为研究对象,根据术式不同将其分为杂交组和预开窗组。杂交组24例,采用颈-锁骨下动脉转流联合TEVAR治疗;预开窗组21例,采用体外左锁骨下动脉(LSA)预开窗技术联合TEVAR治疗。比较两组患者临床特征、围手术期状况、术后并发症情况及生存结局。结果两组患者于术前临床特征基线比较、术中内漏率、手术成功率及术后并发症(脑梗死、左上肢缺血、切口感染)等评价指标差异均无统计学意义(P>0.05);在手术时间、CICU监护时间、住院费用、术中出血量、术后内漏率、脊髓缺血等方面差异有统计学意义(P<0.05)。杂交组术后内漏率、脊髓缺血发生率均低于预开窗组,预开窗组术后疼痛率低于杂交组。术后随访:杂交组1例患者因肾功能衰竭于术后第23日死亡;预开窗组因脑血管意外死亡1例、逆撕性A型主动脉夹层死亡1例。杂交组随访期间病死率与预开窗组相比,差异无统计学意义(χ^(2)=0.014,P=0.905)。结论两种术式均可有效重建左锁骨下动脉血运,但血管转流术可以显著缩短手术时间、减少术中出血量、降低术后内漏率及脊髓缺血情况,使左锁骨下动脉前向血流更通畅,治疗效果良好,具有临床应用和推广价值。

Thoracic Aortic Aneurysm Revealed by Haemoptysis on a 10-Year-Old Girl at Paediatrics Department of Yalgado Ouedraogo University Hospital

Thoracic aortic aneurysm is a rather rare disorder in children and difficult to diagnose. It is generally linked to congenital heart defects or connective-tissue diseases. Our case is a 10-year-old girl admitted in the pediatric emergency care unit on January 19th, 2015 for massive haemoptysis and severe anaemia. Examination revealed severe anemia and a silent left lung. The Chest X-Ray revealed an abnormal mass on the upper left side of the mediastinum, and left lower lobe consolidation. The thoracic CT scan highlighted a 64 mm aneurysm of the subisthmic aorta with a thin 5 mm hole. It also showed pseudocoarctation of the aorta. Treatment in intensive care consisted of blood transfusion and iron supplement. She was due to travel abroad for cardio vascular surgery, but died on November 2016. Thoracic Aortic Aneurysm in our setting was discovered incidentally. In spite the fact that it is an extreme surgical emergency, in Burkina Faso, treatment can only be possible abroad upon medical evacuation.