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 exam...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.展开更多
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 foll...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.展开更多
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 a...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.展开更多
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 hyp...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.展开更多
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...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.展开更多
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 co...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.展开更多
Background:Coarctation of the aorta is a congenital heart disease that sometimes remains clinically silent until adulthood,usually presenting with arterial hypertension.It is well known that after coarctation treatmen...Background:Coarctation of the aorta is a congenital heart disease that sometimes remains clinically silent until adulthood,usually presenting with arterial hypertension.It is well known that after coarctation treatment,many patients remain hypertensive despite successful repair.In this study we approached the predictors and effects of residual hypertension after successful coarctation therapy.Methods:It was a cross sectional observational study involving 50 patients who underwent Coarctation repair/angioplasty in Ain Shams university hospitals.We divided the patients into two groups(hypertensive and normotensive)and we studied their demographic data(e.g.,age,gender,age at first repair,follow up period,etc.)to conclude the possible predictors of persistent hypertension in such cases.We also performed full echocardiographic assessment including 3D speckle tracking echocardiography to detect subtle changes in left ventricular systolic functions.Results:Hypertensive patients had significantly higher BMI(29.1 kg/m^(2))as compared to normotensives(24 kg/m^(2)),p<0.0001.Age at first CoA intervention was significantly higher in hypertensive patients.Hypertensive patients had significantly lower 3D left ventricular strain measures(Global area strain,global circumferential strain and global longitudinal strain).The incidence of bicuspid aortic valve was significantly higher in the hypertensive group,yet it was not an independent predictor of persistent hypertension.Conclusions:The predictors for residual hypertension post successful CoA repair were late intervention and higher BMI.Bicuspid aortic valve was more common in the hypertensive group.Regarding the effects of such disease domain,treated CoA patients with persistent hypertension tended to have higher LV mass index and lower values of LV deformation.展开更多
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 c...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.展开更多
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 centra...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.展开更多
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 i...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.展开更多
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, w...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.展开更多
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 the...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.展开更多
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 simultaneo...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.展开更多
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 ...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.展开更多
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-an...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.展开更多
We report a case of acute type B aortic dissection complicated by coarctation of aorta in 35-year-old women. Computed tomography angiography (CTA) showed a short segment aortic narrowing with diameter of 9.2 mm a smal...We report a case of acute type B aortic dissection complicated by coarctation of aorta in 35-year-old women. Computed tomography angiography (CTA) showed a short segment aortic narrowing with diameter of 9.2 mm a small intimal tear, a true lumen, a false lumen, a markedly thick mural thrombus and pleural effusion. Open surgical procedure was performed and the aortic coarctation (CoA) and aortic dissection were resected and a 24-mm prosthetic graft was anastomosed. No pseudo aneurysms were present at the anastomosis sites on the follow up CTA.展开更多
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 proced...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.展开更多
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 co...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.展开更多
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. Echocardiog...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.展开更多
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: Ou...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.展开更多
文摘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.
文摘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.
文摘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.
文摘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.
文摘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.
基金Disclosure of grants or other funding:The Natural Science Foundation of Guangdong Province[2020A1515010904]the Science and Technology Planning Project of Guangdong Province[2019B020230003]。
文摘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.
文摘Background:Coarctation of the aorta is a congenital heart disease that sometimes remains clinically silent until adulthood,usually presenting with arterial hypertension.It is well known that after coarctation treatment,many patients remain hypertensive despite successful repair.In this study we approached the predictors and effects of residual hypertension after successful coarctation therapy.Methods:It was a cross sectional observational study involving 50 patients who underwent Coarctation repair/angioplasty in Ain Shams university hospitals.We divided the patients into two groups(hypertensive and normotensive)and we studied their demographic data(e.g.,age,gender,age at first repair,follow up period,etc.)to conclude the possible predictors of persistent hypertension in such cases.We also performed full echocardiographic assessment including 3D speckle tracking echocardiography to detect subtle changes in left ventricular systolic functions.Results:Hypertensive patients had significantly higher BMI(29.1 kg/m^(2))as compared to normotensives(24 kg/m^(2)),p<0.0001.Age at first CoA intervention was significantly higher in hypertensive patients.Hypertensive patients had significantly lower 3D left ventricular strain measures(Global area strain,global circumferential strain and global longitudinal strain).The incidence of bicuspid aortic valve was significantly higher in the hypertensive group,yet it was not an independent predictor of persistent hypertension.Conclusions:The predictors for residual hypertension post successful CoA repair were late intervention and higher BMI.Bicuspid aortic valve was more common in the hypertensive group.Regarding the effects of such disease domain,treated CoA patients with persistent hypertension tended to have higher LV mass index and lower values of LV deformation.
文摘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.
文摘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.
基金This study was supported by Deutsches Zentrum für Herz-Kreislaufforschung e.V.
文摘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.
文摘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.
文摘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.
文摘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.
文摘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.
文摘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.
文摘We report a case of acute type B aortic dissection complicated by coarctation of aorta in 35-year-old women. Computed tomography angiography (CTA) showed a short segment aortic narrowing with diameter of 9.2 mm a small intimal tear, a true lumen, a false lumen, a markedly thick mural thrombus and pleural effusion. Open surgical procedure was performed and the aortic coarctation (CoA) and aortic dissection were resected and a 24-mm prosthetic graft was anastomosed. No pseudo aneurysms were present at the anastomosis sites on the follow up CTA.
文摘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.
文摘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.
文摘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.
文摘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.
