COARCTATION OF AORTA IN A 33 YEARS-OLD UNCONTROLLED HYPERTENSIVE FEMALE PATIENT – A CASE REPORT

We report a 33-year-female patient of hypertension detected at the age of 19 year. Her blood pressure was not well controlled. The case was investigated for secondary hypertension. CT Thoracic and Abdominal aorta Angiogram of the present case showing the narrowing in the thoracic aorta extending at the level of T8 to T10 vertebral body level for the length of 7.5cm. Reconstructed CT of the case showed multiple tortuous collaterals between the branches of internal mammary artery and external iliac artery and between axillary and intercostal artery. Relevant history was reviewed and discussed.

Sternal Cleft With Coarctation of Aorta and Vascular Anomalies - Learning Point in Young Stroke

@#Sternal cleft is a rare congenital anomaly that is known to be associated with other congenital abnormalities in particular coarctation of aorta and vascular anomalies. We present a case of a young teacher who presented with recurrent episodes of ischemic stroke. During the workup to look for the cause of stroke, it was discovered that this patient had complete midline sternal cleft with underlying coarctation of aorta and left subclavian artery aneurysm. We wish to highlight the relation between the presence of sternal cleft and coarctation of aorta as well as vascular anomalies as one of the rare cause of young stroke.

Coarctation of the aorta in a young female treated with left subclavian artery to descending thoracic aorta bypass: a case report / Coarctação de aorta em jovem tratada com artéria subclávia esquerda para bypass de aorta torácica descendente: relato de caso

Abstract Coarctation of the aorta is a rare congenital abnormality, with an incidence of 6-8% of all congenital heart problems. It is usually diagnosed in childhood during routine clinical examination and adults mostly present with hypertension. Various investigations like transthoracic echocardiography, computed tomography, and magnetic resonance angiography can help with diagnosis. Prognosis depends on age at presentation and the severity of coarctation. Treatment options available are open and endovascular repair. Extra-anatomical bypass is the preferred option in cases with unfavorable anatomy. Long term follow up is required post repair due to risk of restenosis and aneurysm formation. Here is a case in which a young female presented with hypertension, was diagnosed with coarctation of the aorta, and was treated a left subclavian artery to descending thoracic aorta bypass. Her postoperative course was uneventful and she had improvement in hypertension.

Resumo A coarctação da aorta é uma anomalia congênita rara, com incidência de 6-8% entre todos os problemas cardíacos congênitos. É geralmente diagnosticada na infância durante o exame clínico de rotina, e os adultos normalmente apresentam hipertensão. Diversas investigações, como ecocardiografia transtorácica, tomografia computadorizada e angiorressonância magnética, podem auxiliar no diagnóstico. O prognóstico depende da idade na apresentação e da gravidade da coarctação. As opções de tratamento disponíveis são o reparo aberto e o reparo endovascular. O bypass extra-anatômico é a opção preferida em caso de anatomia desfavorável. O acompanhamento de longo prazo é necessário após o reparo devido ao risco de reestenose e formação de aneurisma. Descrevemos o caso de uma jovem do sexo feminino que apresentou hipertensão, a qual foi diagnosticada como coarctação da aorta. Foi realizada derivação da artéria subclávia esquerda para a aorta torácica descendente. O pós-operatório transcorreu sem intercorrências, e a paciente apresentou melhora da hipertensão.

Application of descending aortic translocation in tracheal or bronchial stenosis due to aortic deformity / 中华胸心血管外科杂志

Objective:To investigate the efficacy of descending aortic translocation in relieving the compression of trachea or bronchus caused by aortic deformity.Methods:From January 2017 to July 2019, a total of 11 patients with distal trachea or proximal bronchial stenosis caused by aortic deformity were treated with descending aortic translocation. Cardiac CT and fiberoptic bronchoscopy were performed before surgery. The median age was 55(23-540) days, and the body weight was 4(2.1-9.0)kg. Five patients had a special type of vascular ring(left aortic arch with right descending aorta, small aortic window with funnel chest; left aortic arch with right descending aorta and right artery ligament, vagus right subclavian artery, combined with trachea, carina and left and right bronchial stenosis in 1 case; Right aortic arch with left descending aorta, combined with tracheomalacia stenosis in 1 case; Right aortic arch with left descending aorta combined with broad tracheal stenosis and left pulmonary dysplasia in 1 case) compressed tracheal or bronchial tube in 5 cases. Three patients with left main bronchus constriction after traditional arch disconnection surgery. 3 patients with left main bronchus stenosis before coarctation or interrupted aortic arch.Surgical methods: Descending aortic translocation was performed through a midline sternotomy with cardiopulmonary bypass and deep hypothermia.The proximal descending aorta was transected distal to the left subclavian artery, proximal sutures were performed, and the distal brought up though the transverse sinus caudad to the right pulmonary artery and tracheal carina, and anastomosed in end-to-end fashion to the ascending aorta, and simultaneous correction was performed with intracardiac malformations, such as airway plasty was performed at the same time without improvement after compression of tracheal stenosis.Results:There was no death in the whole group. Median cardiopulmonary bypass was 180(136-337)min with an median aortic cross-clamp time of 51(30-84)min; Median absence of perfusion to the descending thoracic aorta 34(21-50)min .Tracheal compression was effectively relieved in 5 patients, and airway plasty was performed simultaneously in 6 patients due to persistent tracheal softening and stenosis. All patients had resolution of symptoms.There was only one case anastomotic stenosis after descending aortic translocation.The mean follow-up was(18.0±9.4)months.Conclusion:Descending aortic translocation can effectively relieve this kind of pressure due to aortic deformity.

3D printing assisted interventional therapy for congenital coarctation of aorta: 8 cases of analysis on clinical efficacy / 中华胸心血管外科杂志

Objective:To explore the feasibility and effectiveness of 3D printing aortic model for preoperative evaluation and surgical simulation, and to assist interventional treatment of coarctation of the aorta(CoA).Methods:From December 2017 to January 2019, 8 patients with congenital coarctation of the aorta who underwent percutaneous balloon dilatation and covered stent placement in Xijing Hospital of Air Force Military Medical University were analyzed retrospectively. Among them, 7 cases were male and 1 case was female. The age was(32.00±14.93) years old. Before operation, CT data of patients' heart and aorta were collected, reconstructed with Mimics software, and 3D printing technology was used to make the model of patients' aortic lesions. Before operation, the operation simulation was carried out to determine the best operation scheme and estimate the possible situation, and the relevant clinical data of patients during hospitalization and follow-up were collected.Results:One stent graft was successfully implanted into CoA through femoral artery in all 8 patients. The mean diameter of CoA increased from(3.70±2.94) mm before operation to(18.01±1.51) mm immediately after operation( P<0.05), and the mean systolic pressure difference decreased from(83.75±25.44) mmHg before operation to(14.63±8.09) mmHg after operation( P<0.05). The mean systolic blood pressure of the right upper extremity decreased from(204.13±22.31) mmHg before operation to(145.63±32.08) mmHg after operation( P<0.05), and there was no significant difference between the two groups. During the period of hospitalization and follow-up, no corresponding cardiovascular complications were found. Conclusion:The short-term effect of percutaneous balloon dilatation covered stent implantation on CoA in adolescents and adults is obvious. 3D printing model can reproduce the anatomical model of CoA site of patients individually, which is feasible and effective for the preoperative evaluation of CoA and the preparation of operation plan.

Strategy of repairing coarctation of aorta with hypoplastic aortic arch using cardiopulmonary bypass in neonates / 中华胸心血管外科杂志

Objective To analysis the the strategy of repairing coarctation of aorta with hypoplastic aortic arch in neo-nate.Methods Collected consecutive 24 neonates suffered coarctation of aorta with hypoplastic aortic arch form January 2015 to March 2017,12 patients were underwent aortic arch reconstruction with extremely extended end-to-end anastomosis under cardiopulmonary bypass(CPB) through the media sternotomy approach(CPB group), while another 12 cases were under coarc-tation repair using end-to-end anastomosis or end-to-side anastomosis through left posterolateral thoracotomy approach ( control group).The postoperative blood pressure,echocardiography and cardiac CT scan were used to evaluate the result of the aortic arch rconstruction.The pressuregradientof the upper limb and lower limb,flow velocity of the anastomoses, aortic arch morphol-ogy,rate of the residual abstruction were compared between the two groups.Results Mechanical ventilation time[ CPB group (17 ±27)h vs.control group(44 ±52)h, P＜0.05], ength of stay in ICU[CPB group(3.75 ±1.36)days vs.control group (6.54 ±5.08)days, P＜0.05], all patients were followed up for 1－12 months,the aortic residual pressure[CPB group(14 ± 10)mmHg (1 mmHg＝0.133 kPa) vs.control group(26 ±17)mmHg,P ＜0.05) ], flow velocity of the anastomoses[CPB group(2.32 ±0.78)m/s, control group(1.55 ±0.99)m/s, P ＜0.05 ], pressuregradientof the upper limb and lower limb [CPB group (6.67 ±3.49)mmHg, control group(7.41 ±4.29)mmHg, P＞0.05].There was no died in two gruops.Con-clusion Anastomosis with end to end techinique under cardiopulmonary bypass may achieve better effect in neonates with co-arctation of aorta with hypoplastic aortic arch,it has better duplcity, operability and early curative effect,but the long-term effect need the multicenter and large sample-volume study to explore.

Effect of stage 1 repair of coarctation of aorta combined with cardiac anomalies and analysis of median follow－up finding / 中华实用儿科临床杂志

Objective To summarize the outcomes and the median follow －up finding of stage 1 surgical approaches of coarctation of aorta(CoA)combined with cardiac anomalies.Methods A retrospective study was per-formed for summarizing the operative and follow－up finding of 82 patients(40 males and 42 females)of CoA combined with cardiac anomalies,who underwent stage 1 repair from February 2013 to December 2016 at the Department of Cardio-vascular Surgery,Henan Provincial People's Hospital.The operative age was ranged from 1 month to 8 years old[mean (11.92 ± 22. 51)months]and the weight was (7.31 ± 6.79)kg. Forty －five cases had ventricular septal defect (VSD)or atrial septal defect (ASD).Septal defect and patent ductus arteriosus were found in 31 patients.CoA was associated with aortic arch hypoplasia in 5 patients.Complex cardiovascular deformity was found in 7 cases.The surgical methods included end－to－end anastomosis,end－to－side anastomosis and patch augmentation of the coarctation seg-ment.Results Seven patients died after operation,3 cases of them died of low cardiac output syndrome amd circulatory failure,1 case died of circulatory failure and respiratory failure,1 patient died of malignant arrhythmia,and 2 cases died of respiratory failure.Postoperative echocardiogram suggested that anastomosis maintained patency in all the patients. The follow－up time was 8 months to 4 years,63 patients were followed up,and follow－up rate was 77%.No death or aneurysm occurred during follow－up period.Recoarctation was found in 5 cases.Conclusions Stage 1 repair for CoA combined with cardiac anomalies is effective,and has low complications during postoperative and median follow－up period.Long－term results need further study.

Coarctation of the aorta with hypoplastic aortic arch :Analysis of midterm and longterm outcomes with autologous pulmonary patch aortoplasty / 医学研究生学报

Objective Previously we have reported the early and midterm benifit of autologous pulmonary patch in repairing aortic coarctation of hypoplastic aortic arch.This study aimed to assess its reliability and midterm and longterm outcomes.Methods We retrospectivly analyzed 42 pediatric patients with coarctation of the aorta (CoA) with hypoplastic aortic arch undergoing surgical repair with autologous pulmonary patch from May 2009 to May 2017 in General Hospital of Guangzhou Military Command of PLA.All the patients were allocated into either senior group (＞ 1 years) or junior group (≤1 years) according to the age of operation.The trans-coarctation gradient,pulmonary pressure and aortic Z value change were compared between two groups before and after the repair.Results There were 8 cases had early postoperative complications.However,no death had been reported during the postoperative time and the followed up period ranged from 4 months to 106 months (40.0± 15.5) months).The average pressure gradient of coarctation segment for all the patients was (11.9±6.4) mmHg,including 5 cases more than 25 mmHg.The pressure gradient and mean pulmonary arterial pressure after operation were significantly lower than those before operation (P＜0.05),The postoperative aortic arch Z value was greater than the preoperative value (P＜0.05).Compared with the preoperative period,the Z value of proximal transverse arch increased significantly(-0.64±0.44) vs (1.27±0.66),P＜0.05.Compared with junior group,the senior group had higher preoperative and postoperative pulmonary artery pressure (P＜0.05),and longer CPB time,aortic block time,ventilation time,ICU time and hospital stay time (P＜0.05).However,patients in the junior group had a higher pressure gradient through the aorta arch(P＜0.05) and a smaller Z value transverse arch aortic proximal and isthmus(P＜0.05) during the long-term period.The time of selective cerebral perfusion had no statistical difference between the two groups (P＞ 0.05).Conclusion Early surgery for coarctation of aorta with hypoplastic aortic arch,autologous pulmonary patch aortoplasty is a relatively ideal option with better midterm and longterm outcomes.

Evaluation of aorta's compliance after repair of coarctation of aorta using pulse wave velocity of MRI / 实用放射学杂志

Objective To evaluate aorta's compliance after repair of coarctation of the aorta (CoA)using the pulse wave velocity (PWV)of MRI.Methods A retrospective analysis of cardiac MR images in 22 patients after repair of CoA was performed.PWV was measured and calculated with phase contrast MRI,and echocardiography was performed to measure both ventricular function and myocardial thickness during the same period.Age and sex-matched 22 normal subjects were served as a control group and their aortic PWVs were measured.Results The averaged PWV in study group was significantly higher than that in control group [(4 .42 ± 3 .02 )m/s vs (2 .73 ± 0 .76 )m/s,P =0.02].In study group,the PWV value of children with moderate anastomotic stenosis was the highest. ROC curve analysis showed the highest sensitivity and specificity could be achieved to distinguish the mild or moderate anastomotic stenosis when the threshold for PWV was 3.37 m/s.No significant correlations were found between left ventricular end-diastolic volume,left ventricular end-systolic volume,ejection fraction,left ventricular thickness and PWV.Conclusion PWV is increased in children after repair of CoA.Significant aortic remodelling can be observed in patients with moderate anastomotic stenosis.

Treatment of coarctation of aorta / 国际儿科学杂志

Coarctation of aorta is a discrete narrowness of the aorta and most commonly occurs in the isthmus.It is a common congenital cardiovascular malformation with morbidity of 5％ ～ 8％ among all congenital heart defects.Coarctation can result in the increase of upper limb blood pressure,the decrease of limb blood pressure,and a decline in cardiac function.Treatments include surgical repair and percutaneous interventions with different complications.Long-term complications and heart functions are important indices for evaluating prognosis and should be closely evaluated postoperatively.This review focuses on progresses of therapy methods,postoperative complications and cardiac function.

Evaluation of aorta's compliance after repair of coarctation of aorta using pulse wave velocity of MRI / 实用放射学杂志

Objective To evaluate aorta's compliance after repair of coarctation of the aorta (CoA)using the pulse wave velocity (PWV)of MRI.Methods A retrospective analysis of cardiac MR images in 22 patients after repair of CoA was performed.PWV was measured and calculated with phase contrast MRI,and echocardiography was performed to measure both ventricular function and myocardial thickness during the same period.Age and sex-matched 22 normal subjects were served as a control group and their aortic PWVs were measured.Results The averaged PWV in study group was significantly higher than that in control group [(4 .42 ± 3 .02 )m/s vs (2 .73 ± 0 .76 )m/s,P =0.02].In study group,the PWV value of children with moderate anastomotic stenosis was the highest. ROC curve analysis showed the highest sensitivity and specificity could be achieved to distinguish the mild or moderate anastomotic stenosis when the threshold for PWV was 3.37 m/s.No significant correlations were found between left ventricular end-diastolic volume,left ventricular end-systolic volume,ejection fraction,left ventricular thickness and PWV.Conclusion PWV is increased in children after repair of CoA.Significant aortic remodelling can be observed in patients with moderate anastomotic stenosis.

The making of indigenous vascular prosthesis.

Background & objectives: Vascular illnesses are on the rise in India, due to increase in lifestyle diseases and demographic transition, requiring intervention to save life, organ or limbs using vascular prosthesis. The aim of this study was to develop indigenous large diameter vascular graft for treatment of patients with vascular pathologies. Methods: The South India Textile Research Association, at Coimbatore, Tamil Nadu, India, developed seamless woven polyester (Polyethylene terephthalate) graft at its research wing. Further characterization and testing followed by clinical trials were conducted at Sree Chitra Tirunal Institute for Medical Sciences and Technology, Thiruvananthapuram, Kerala, India. Fifteen in vivo experiments were carried out in 1992-1994 in pigs as animal model. Controlled (phase I) clinical trial in ten patients was performed along with control graft. Thereafter, phase II trial involved 22 patients who underwent multi-centre clinical trial in four centres across India. Results: Laboratory testing showed that polyester graft was non-toxic, non-leeching and non-haemolytic with preserved long-term quality, further confirming in pigs by implanting in thoracic aorta, comparable to control Dacron grafts. Perigraft incorporation and smooth neointima formation which are prime features of excellent healing characteristics, were noted at explantation at planned intervals. Subsequently in the phase I and II clinical trials, all patients had excellent recovery without mortality or device-related adverse events. Patients receiving the test graft were followed up for 10 and 5 years, respectively. Serial clinical, duplex scans and CT angiograms performed periodically confirmed excellent graft performance. Interpretation & conclusions: Indigenously developed Chitra vascular graft was comparable to commercially available Dacron graft, ready for clinical use at affordable cost to patients as against costly imported grafts.

Early and midterm follow-up of vascular endothelial function in children with surgical repair for coarctation of aorta / 实用医学杂志

Objective To evaluate the endothelium-dependent vasodilation in children after repair for coarctation of aota (CoA). Methods A group of 20 children having undergone CoA repair between January 2010 and October 2010 in Guangzhou Women and Children′s Medical Center were include in the study , including 12 infants aged less than 6 months and 8 ones aged more than 6 months. Another 20 healthy children were enrolled during the same period as controls. All the subjects underwent monitoring of resting blood pressure and flow-mediated dilation (FMD) of the brachial artery in 4-year follow-up. Results There were no resting hypertension in all subjects , but FMD in the CoA group was higher than in the control group and so it was with the early surgery group and non early surgery group. Conclusion The surgical repair for coarctation of aorta could not cure the vascular function impairment satisfactorily , neither is the early surgery effective in alleviating the injuries in vascular endothelia.

11 cases clinical analysis of endovascular treatment with coarctation of aorta / 中国医师进修杂志

Objective To evaluate the outcome of endovascular treatment with coarctation of aorta (CoA).Methods Between November 2007 and March 2012,11 patients,including 8 male and 3 female from 17 to 43 years old,with native CoA who were diagnosed by computed tomography angiography accepted the endovascular treatment.The arterial pressure difference between the femoral artery and the narrow segment was measured during the operation,and the stent was released by expanding the balloon.Results Endovascular treatment were successful in all cases without any major adverse events.Significant reduction of systohc blood pressure was observed from (64.09 ± 7.81) mmHg (1 mmHg =0.133 kPa) to (11.18 ± 3.37) mmHg.The mean CoA diameter pre-stent (4.000 ± 0.775) mm,post-stent (17.090 ± 2.427) mm.1 patient associated with patent ductus arteriosis had no persistent left-to-right shunt after covered stent implantation.The follow up range was 33 months.1 patient in simple balloon was retreatment by surgical because of restenosis.And no retraction was observed of 10 patients after stent implantation.Conclusion The early and midterm result of endovascular treatment with CoA is satisfactory,and longer follow-up is needed for its longterm outcomes.

Tratamiento percutáneo de cardiopatías congénitas en el síndrome de Rubinstein-Taybi / Percutaneous Treatment of Congenital Heart Defects in the Rubinstein-Taybi Syndrome

El síndrome de Rubinstein-Taybi es producido por una anomalía genética y se caracteriza por una facies típica, anomalías de manos y pies, microcefalia y retraso mental. Alrededor del 30% de los individuos afectados tienen defectos cardíacos congénitos asociados. En esta presentación se describen los casos de dos pacientes con diagnóstico de síndrome de Rubinstein-Taybi asociado con alteraciones cardíacas congénitas. Uno de ellos, de 25 años, presentaba coartación de la aorta nativa, localizada en la región yuxtaductal, y el otro, de 11 meses de edad, mostraba un conducto arterioso permeable de tamaño grande. Ambos pacientes recibieron tratamiento intervencionista percutáneo exitoso, con evolución alejada satisfactoria.

The Rubinstein-Taybi syndrome is a genetic disorder charac-terized by distinctive facial features, anomalies in hands and feet, microcephaly and mental disability. Approximately 30% of subjects affected with this syndrome have associated congenital cardiac defects. This presentation describes two cases of Rubistein-Taybi syndrome with congenital cardiac disorder: a 25-year old patient with native juxtaductal coarctation of the aorta and an 11-month old infant, with large patent ductus arteriosus. Both patients underwent successful percutaneous intervention, with satisfactory long-term outcome.

Levosimendan in a neonate with severe coarctation of aorta and low cardiac output syndrome.

We report successful use of levosimendan after failed balloon angioplasty in a critically ill neonate with coarctation of aorta (CoA) and severe low cardiac output syndrome (LCOS). Treatment with levosimendan improved left heart function, and decreased lactate and brain natriuretic peptide levels. To our knowledge, this is the first report on the safe and successful use of levosimendan in the management of LCOS due to severe CoA in a neonate awaiting surgical repair.

Adult Coarctation of Aorta with Bicuspid Aortic Valve.

Coarctation of the aorta is a congenital cardiac malformation that can go undiagnosed with only hypertension as a marker of its presence, because clinical signs can be subtle and overlooked if a complete physical examination is not performed. Here we present a case report of 20 year old young male patient with hypertension who was diagnosed to have Coarctation of Aorta and Bicuspid aortic Valve.Our patient was relatively asymptomatic until he presented with headache and exertional dyspnoea.

Follow-up for vascular structure and function in children with successfully repaired coarctation of aorta / 中华胸心血管外科杂志

Objective Even after successful surgical repair,patients with coarctation of the aorta (CoA) are at high risk of long-term morbidity and mortality due to cardiovascular events,which is probably related to persistent arterial disfunction during long-terr follow-up after operation,The aim of the study was to explore the alterations of vascular structure and function in children with successfully repaired CoA in the short-and mid-term follow-up.Methods A cohort of 20 children who underwent CoA repair between January 2010 and October 2010 in Guangzhou women and children's Medical Center was studied.There were 14 males and 6 females in CoA group,which comprised 6 patients with isolated CoA,14 patients with CoA associated with intracardia anomalies,whose median age of operation was 4 months (rang from lmonth to 10.0 years).And 20 patients with isolated ventricular septal defect (VSD) were included as VSD group during the same time,with 12 males and 8 females,whose median age of operation was 5 months (rang from 1 month to 12.0 years).Resting blood pressure,flow-mediated dilation (FMD) of the brachial artery,carotid intima-media thickness (IMT) were compared in CoA group and VSD group,including preoperative media data and follow-up of 1 month,6 months and 1 year.In addition,as comparison to the operation group,20 health children with normal echocardiographic findings,whose median age was 5 months (rang from 3 month to 10.0 years),were selected as health group for the 1-year following up.None of them had obesity,hyperlipidemia,diabetes mellitus,metabolic diseases or systemic inflammatory disease.Results As a result of the datas before operation and those I month,6 months and 1 year after operation,all children were normotensive at rest.In the same period,Carotid IMT in CoA group[(0.47 ± 0.10)mm,(0.49 ±0.10) mm,(0.57 ±0.07)mm,(0.61 ± 0.07) mm]was significantly thicker than that in VSD group[(0.41 ±0.11) mm,(0.43 ±0.11)mm,(0.51 ±0.08) mm,(0.55 ±0.08) mm](P＜0.05) and health group[(0.40 ±0.09) mm,(0.42 ±0.11)mm,(0.50 ±0.08) mm,(0.57 ±0.08) mm](P ＜0.05),Brachial artery FMI in children with CoA[(5.4,6 ±1.51)％,(5.71 ±1.88)％,(5.42±1.69)％,(5.27±1.02)％]was significantly lower than that in the VSD control group[(6.69±1.45) ％,(6.66±1.21)％,(6.81 ±1.03)％,(6.43±1.34)％](P＜0.05) and health group[(6.59 ±1.84)％,(6.84±1.41)％,(6.91 ±1.31)％,(6.56±1.62)％](P＜0.05).Significant difference could not be found in neither the IMT nor the FMI between the VSI control group and health group in 4 period respectively,P ＞ 0.05.Conclusion Children after successful coarctation repair have abnomal structural and functional properties of the aorta above the place of coarctation even their blood pressure at rest is normal.These results confirm that the alterations in mechanical properties of carotid arteries as well as the generalized endothelial dysfunction in children with coarctation of the aorta are persistent,which can not be prevented or reversed by surgical repair,and which may partly explain the high incidence of cardiovascular disease observed in their adulthood and reduced life expectancy,furtherly supporting the claim that coartation of the aorta is a systemic vascular disorder which needs long-term follow-up of vascular function.

PHACE/S syndrome: A syndromic infantile segmental hemangioma.

PHACES syndrome is a neurocutaneous disorder characterized by posterior fossa brain malformations, hemangiomas, cardiac anomalies and coarctation of aorta, eye anomalies ± sternal clefts. All reported cases are sporadic and notably common in females. The underlying cause is unknown. Here is described, one of the twin baby with characteristic features of PHACE syndrome. The presence of large segmental hemangioma, especially on face should prompt the primary care provider to act early, to prevent complications related to facial hemangiomas and other associated anomalies.