Long-segmental middle aortic coarctation: a rare case first diagnosed by transthoracic echocardiography.

BACKGROUND: Middle aortic coarctation (MAC), also known as middle aortic syndrome, is an atypical aortic coarctation characterized by narrowing of the distal thoracic aorta and proximal abdominal aorta. MAC is a rare disease commonly diagnosed by computed tomography angiography (CTA). In this paper, we present a case of long-segmental MAC first diagnosed by transthoracic echocardiography (TTE) and further evaluated by CTA. CASE PRESENTATION: A 14-year-old girl, with dyspnea and fatigue on exertion for 2 months and refractory hypertension for 6 months, was referred by the local clinic to our hospital. Physical examination showed blood pressure up to 176/100 mmHg measured in the arms despite dual antihypertensives, a marked pressure gradient between her arms and legs, and weak pulses in both dorsal pedes arteries. TTE revealed a segmental narrowing in the descending thoracic aorta below the level of the atrioventricular sulcus, with a calcified plaque in the stenotic region. Abdominal vascular ultrasound revealed the segmental narrowing extending to the descending abdominal aorta (5.7 mm in diameter) above the level of the superior mesenteric artery. Subsequently, CTA verified a long-segment narrowing in the descending aorta from the level of T8 to L2 vertebra, with a calcified plaque in the stenotic aorta, right renal artery involvement, and a rich network of collateral vessels between the pre-and post-stenotic region. The patient was referred for cardiovascular surgery in which a successful ascending aorta-abdominal aorta bypass was performed. CONCLUSIONS: Although MAC is usually diagnosed by CTA, it may also be first diagnosed by TTE in some patients whose longitudinal axis view of the thoracic descending aorta could be shown. Careful TTE scan can improve the diagnostic rate of MAC, especially for some hypertension patients whose marked pressure gradient between arms and legs was ignored by the physician.

Treatment of middle aortic syndrome with thoraco-abdominal aortic bypass grafting assisted by endoscope in adult.

Middle aortic syndrome (MAS) is a rare disease characterized by distal thoracic aorta or abdominal aorta coarctation, and thoraco-abdominal aortic bypass grafting is an effective treatment for this condition. However, significant trauma is associated with the conventional surgical approach. We report a 26-year-old woman with MAS who presented with hypertension and needed thoraco-abdominal bypass grafting. In this operation, we adopted the endoscopic technique to improve the conventional surgical approach (reduce the incision). This case report shows that it is safe and feasible to use an endoscopic technique to reduce the trauma during this kind of operation, and provides a reference for similar treatments.

A Review of Middle Aortic Syndromes in Pediatric Patients.

Middle aortic syndrome (MAS) is a rare vascular disease occurring in pediatric patients. MAS describes narrowing of the abdominal aorta, often referred to as abdominal coarctation. Extra-aortic vessels are commonly involved, including the renal and mesenteric arteries. Pediatric patients with MAS frequently present with severe hypertension, and medical management often is insufficient. Many of these patients require endovascular or open surgical intervention. This review article discusses the etiology, symptoms, and management of pediatric MAS. It highlights the preoperative, intraoperative, and postoperative anesthetic management of these patients. It is important that anesthesiologists be aware of this rare disease and its special anesthetic considerations when caring for children with MAS because of its high morbidity.

Doppler ultrasonographic and clinical features of middle aortic syndrome.

OBJECTIVES: To discuss Doppler ultrasonographic and clinical features of middle aortic syndrome (MAS). MATERIALS AND METHODS: Doppler ultrasonographic images and clinical dates of 11 patients with MAS confirmed by angiography were retrospectively analyzed from January 2004 to September 2016. RESULTS: The median age of 11 patients was 10 years (1-39 years). Ten patients presented with hypertension, only 2 cases presented with symptomatic intermittent claudication, and 1 case presented with abdominal pain. The ultrasonographic features of 11 patients with MAS included: (a) Gray-scale image showed significant segmental narrowing of the aorta in 9 cases. (b) Color Doppler demonstrated aliasing in the suspicious narrowed vessels of all cases. (c) On Spectral Doppler image, peak systolic velocity in the location of aorta coarctation was significantly elevated (range, 2.3~4.8 m/s). When infrarenal aorta was involved, a tardus-parvus waveform was only seen in the distal aorta. When suprarenal or inter-renal aorta was involved, a tardus-parvus pattern was seen in the distal aorta as well as renal artery. CONCLUSIONS: Significant segmental narrowing and a tardus-parvus waveform are the important ultrasonographic features in patients with MAS, the latter may be more reliable. Doppler ultrasound can be used as a simple screening method, especially for children and adolescents suspected of having a vascular cause of refractory hypertension.

Stenting for middle aortic syndrome caused by Takayasu arteritis-immediate and long-term outcomes.

OBJECTIVE: To evaluate immediate and long-term clinical outcomes of stenting for middle aortic syndrome (MAS) caused by Takayasu arteritis (TA). BACKGROUND: Studies of endovascular stenting for the treatment of MAS caused by TA are scare. METHODS: Data from 48 consecutive TA patients (mean 33.3 ± 12.6 years) with MAS treated by stenting in our institution between January 2010 and July 2016 were collected and retrospectively analyzed. Thirty-day and long-term follow-up clinical outcomes after aorta stenting were assessed. RESULTS: Stenting was successful in all patients. The mean stenosis and peak systolic pressure gradient of aorta lesions were reduced from 81.3 ± 8.0% and 70.7 ± 18.4 mm Hg to 14.7 ± 8.3% and 14.0 ± 5.8 mm Hg immediately after the 54 stents were implanted. One patient developed retroperitoneal hemorrhage and one developed flow-limiting dissection that involved bilateral renal arteries perioperatively. Both patients recovered without sequela. Compared with baseline, the ankle brachial index (0.92 ± 0.19 vs. 0.75 ± 0.22), mean systolic blood pressure (149.5 ± 19.1 vs. 179.0 ± 28.4 mm Hg) and antihypertensive drugs (1.1 ± 0.7 vs. 3.1 ± 0.9) significantly improved after an average follow-up of 3.1 years (all P < .001). A total of 5 (10.9%) patients developed in-stent restenosis, which were resolved by reintervention (restenting in 3 patients and re-angioplasty alone in 2 patients). No major adverse events occurred during follow up. CONCLUSIONS: Percutaneous aortic stenting is highly efficacious and safe in treating patients with MAS caused by TA with good immediate and long-term clinical outcomes.

Surgical treatment of middle aortic syndrome with Takayasu arteritis or midaortic dysplastic syndrome.

OBJECTIVE: Middle aortic syndrome (MAS) is a rare condition characterized by severe stenosis of the distal thoracic or abdominal aorta. The aims of this study are to define the anatomic characteristics of MAS and to review the various surgical methods and their outcomes in terms of long-term durability MATERIALS AND METHODS: Ten adult patients were diagnosed with MAS caused by Takayasu arteritis (TA) or midaortic dysplastic syndrome and underwent surgical treatment between July 1992 and January 2013. RESULT: The aortic lesions were mostly suprarenal (n = 7) and stenoses were commonly found in the celiac axis (n = 6), SMA (n = 7), and renal artery (n = 6). Indications for operation were uncontrolled hypertension in six patients and lower extremity claudication in four. Eight aortic bypasses, one supraceliac aortic interposition graft, and one bilateral aorto-renal bypass were performed. Adjunctive renal bypass with saphenous vein graft (n = 4) and IMA reimplantation (n = 2) were performed simultaneously. There was no post-operative mortality, and one complication of iliac dissection at the distal anastomosis site was detected and treated by stenting. Hypertension was cured or improved in five of the six patients, and lower extremity claudication improved in all of them. With a median follow up of 60 months (range, 12-263), all the aortic bypasses were patent and one adjunctive renal artery bypass graft with aortic bypass was occluded 29 months post-operatively. CONCLUSIONS: Aortic bypass for MAS is safe and shows excellent long-term durability. Considering the patients are relatively young with a long life expectancy, aggressive surgical treatment could be beneficial. Lifelong follow up to monitor complications and disease progression is necessary.

"Tardus-parvus waveform" the only initial clue to mid-aortic syndrome- a rare cause of youth onset hypertension: A case report and a comprehensive review.

Middle aortic syndrome (MAS), an uncommon cause of secondary hypertension, is defined by obstructive narrowing of the abdominal aorta and ostia of its major branches like the renal and splanchnic arteries. Most of the cases of MAS are categorized as idiopathic; however, genetic disorders like Williams syndrome, mucopolysaccharidosis, neurofibromatosis type 1 (NF1), and Alagille syndrome, and acquired inflammatory diseases such as Takayasu arteritis and other nonspecific arteritis can also lead to MAS. MAS is commonly seen in children and young adults presenting with severe hypertension, congestive heart failure, renal failure, or severe leg claudication. The diagnosis of MAS on CT, MR, and conventional angiography is fairly straightforward. However, the spectrum of sonographic findings in MAS can be varied. Since ultrasound is frequently utilized as a first-line investigation for secondary causes of hypertension, it is especially crucial to understand the sonographic features of MAS. Here, we report a case of a young female who presented to our hospital with severe hypertension. On the Renal Doppler scan, the only clue of the renovascular etiology of her secondary hypertension was the "tardus-parvus waveform'' in the intrarenal arteries.

Transcatheter double stent implantation for treatment of middle aortic coarctation syndrome.

A 13-year-old boy presented with severe systemic hypertension. His upper limb blood pressure measured 190/100 mm Hg and lower limb blood pressure measured 98/64 mm Hg. The brachial pulses were bounding and femoral pulses were not palpable. Echocardiography and magnetic resonance angiography confirmed middle aortic syndrome. There was severe diffuse thoraco-abdominal coarctation with continuous Doppler run-off. Cardiac catheterization was undertaken and using a retrograde approach two Advanta V12 stents were implanted in the complex thoraco-abdominal coarctation. The gradient across the coarctation was reduced from 80 to 40 mm Hg gradient with a significant improvement in the luminal diameter of the aorta. His upper limb blood pressure reduced to 142/78 mm Hg six weeks later.

[Middle aortic syndrome: a report of three pediatric cases]. / Síndrome de aorta media: presentación de tres casos pediátricos.

The middle aortic syndrome (MAS) is caused by stenosis of the abdominal aorta, often accompanied by concomitant stenosis of the renal or visceral arteries. Although MAS is uncommon (0.5-2%), it is an important cause of hypertension in children and adolescents. It is thought to originate in a failure of the two dorsal aortas to fuse during embryological development, and a high percentage of cases are idiopathic. MAS affects the segment of the aorta between the renal arteries in 54% of cases. Clinically, it courses with symptomatic or asymptomatic arterial hypertension. On physical examination, findings include an abdominal bruit, diminished or absent femoral pulses, and a difference between the arterial pressure of the upper and lower limbs. Angiography is the technique of choice, although noninvasive MR angiography and CT angiography have similar diagnostic accuracy. Ultrasonography is the primary screening technique. Medical treatment consists of a combination of different antihypertensives. Surgical treatment can be curative.

A rare middle aortic syndrome with celiac trunk, superior mesenteric and bilateral renal artery involvement.

Middle aortic syndrome (MAS) is a rare atypical aortic coarctation (AC), often accompanied by refractory renal hypertension, which eventually leads to death from congestive heart failure, stroke or hypertensive encephalopathy. Computed tomography angiography (CTA) has unique advantages in assessing aortic stenosis and splanchnic artery abnormalities. Prompt aortic bypass surgery can relieve symptoms and improve quality of life. In this study, we report a patient with MAS diagnosed by CTA and follow-up after thoracoabdominal aortic bypass grafting.

Successful surgical treatment of the supradiaphragmatic middle aortic syndrome by an extra-anatomical bypass in a child.

We report a 5-year-old girl (13kg) was referred to our facility for cardiologic examination due to uncontrolled hypertension and significant heart failure. Chest radiography elevated cardiothoracic ratio of over 60% with rib notching and the absence of an aortic knob. Transthoracic echocardiography showed a dilated left ventricle with a reduced ejection fraction and significant stenosis of the descending aorta with a pressure gradient of 64 mm Hg. Computed tomography angiography confirmed the type of supradiaphragmatic middle aortic syndrome, with severe stenosis segment 75 mm of the descending aorta was visible around 20 mm above the diaphragm. The narrowest point was estimated to be 1.85 mm. In addition, the ascending aorta, aortic arch, and supra-cervical branches were all normal, as were the abdominal aorta and its major branches. Our patient underwent surgery, a 9.0 mm Dacron bypass was performed between the ascending and the terminal descending thoracic aorta. Extra-anatomical ascending-to-descending aortic bypass is a relatively safe and successful therapy option for supradiaphragmatic middle aortic syndrome.

Treatment of a severe distal thoracic and abdominal coarctation with cutting balloon and stent implantation in an infant: From fetal diagnosis to adolescence.

Background and Aims: Abdominal coarctations are rare. Surgical treatment is difficult and requires re-interventions to adjust the graft material to patient growth. We report effective treatment by interventional catheterization in an infant with the concern to allow adjustment for growth and prevention of vessel damage. Methods and Results: After the diagnosis of abdominal coarctation at 27 weeks of gestation, an infant developed hypertension (170/70 mmHg) at 3 months of age despite medical therapy. Angio CT confirmed a 2 mm diameter, 2.3-cm-long coarctation of the descending aorta. At 4 months, a dilatation was performed using a 3 mm cutting balloon and a 5 mm Opta® balloon, Cordis®. Two noncovered Palmaz® Genesis™ XD PG1910P stents were required to keep the aortic lumen open. At 15 months, an Adventa™ V12 vascular 12 × 61 mm long covered stent was implanted to exclude an aneurysm which developed between the two stents. At 3 and 9.5 years, the stents were further dilated with a high-pressure balloon to reach 11 mm aortic diameter with no residual pressure gradient, and normal blood pressure. Conclusions: The use of cutting balloons and stent implantation is an effective way to relieve severe obstruction in middle aortic syndrome in neonates. The technical issues encountered were the need for a low profile sheath and material to avoid femoral artery damage, and the need to use stents that can be further expanded to adult size.

Surgical 5-year Outcomes of Extra-Anatomical Bypass for Middle Aortic Syndrome: A Case Series.

BACKGROUND: Middle aortic syndrome is a rare disease. Several surgical treatments are available; however, the optimal treatment strategy and long-term outcomes remain unelucidated. We herein report the 5-year outcomes of six patients treated with extra-anatomical bypass surgery for middle aortic syndrome. CASE PRESENTATIONS: Between 2013 and 2016, six patients underwent extra-anatomical bypass for middle aortic syndrome at our institute: three had Takayasu's arteritis, one had vessel vasculitis, and two had middle aortic hypoplastic syndrome of unknown origin. The patients included five women and one man, with a mean age of 59.7 years. Four patients had uncontrolled hypertension and were receiving antihypertensive medications. The mean ankle-brachial pressure index was .61. The three patients with Takayasu's arteritis were hospitalized for congestive heart failure. These patients underwent bypass surgery from the descending aorta to the infrarenal abdominal aorta, and one also underwent concomitant heart surgery. The patient with microscopic polyangiitis underwent Y-grafting with an aortic aneurysmectomy. Subsequently, bypass surgery was performed from the descending aorta to the graft via the diaphragm. The two patients with unknown causes underwent bypass surgery from the proximal descending aorta to the distal descending thoracic aorta. There were no early or late deaths at the 5-year follow-up. We did not observe any changes in anastomotic site stenosis or new aneurysmal changes during the follow-up period. The number of antihypertensive medications was reduced in all cases, and critical symptoms, including headache, severe abdominal pain, claudication, and heart failure, improved in all patients. The ankle-brachial pressure index increased to 1.11 and did not change for five years. Renal function remained stable, and the brain natriuretic peptide level decreased from 302.8 to 74.5 pg/mL at follow-up. CONCLUSION: Extra-anatomical bypass for middle aortic syndrome is safe and effective, and can help prevent renal failure, and relieve critical ischemic symptoms.

Diagnosis and Management of Genetic Causes of Middle Aortic Syndrome in Children: A Comprehensive Literature Review.

Middle aortic syndrome (MAS) is a rare vascular disease representing an important cause of severe hypertension in children. MAS is characterized by segmental or diffuse narrowing of the abdominal and/or distal descending aorta with involvement of the renal and visceral branches. Most cases of MAS are idiopathic, but MAS may occur in genetic and acquired disorders. The most common genetic causes of MAS are neurofibromatosis type I, Williams syndrome, Alagille syndrome, tuberous sclerosis and mucopolysaccharidosis. This review article discusses the pathophysiological aspects, distinctive associated features, and management of genetic forms of MAS in children.

Late presentation of middle aortic syndrome complicated with severe aortic regurgitation; the role of endovascular intervention as a bridging for Bentall surgery.

Middle aortic syndrome presents with segment narrowing of the descending thoracic and abdominal aorta. A common manifestation is uncontrolled hypertension, which can lead to severe aortic regurgitation in the long term. We have presented the case of a 31-year-old woman with worsening heart failure symptoms and longstanding uncontrolled hypertension. Echocardiography revealed severe aortic regurgitation. Aortic computed tomography showed severe stenosis of the aorta at the diaphragm level. Stent graft implantation was performed, followed by Bentall surgery 1 year later. Endovascular stent graft implantation of the descending aorta can be used safely as a bridging surgery for the Bentall procedure to reduce the patient's blood pressure and relieve heart failure symptoms.

Endovascular Management of Neurofibromatosis Type I-Associated Vasculopathy: A Case Series and Brief Review of the Literature.

PURPOSE: Neurofibromatosis type 1 (NF1) is an autosomal-dominant disorder found in approximately 1 of every 3000 individuals. Neurofibromatosis type 1 can have vascular manifestations including aneurysms, stenoses, and arteriovenous malformations. The purpose of this article is to describe the clinical manifestations of NF1 vasculopathy, discuss therapeutic options, and highlight endovascular therapies from our institutional experience. MATERIALS AND METHODS: The radiology information system was searched for cases of NF1. Cases with vasculopathy managed with endovascular therapies were included. Demographics, clinical histories, procedural details, and outcomes were recorded. A review of the literature for the management strategies of NF1 vasculopathy was performed. RESULTS: Two pediatric patients with NF1 were identified, both of whom presented with hypertension found to be secondary to renal artery stenosis. One of the patients also had infrarenal aortic narrowing. Both patients were successfully treated with balloon angioplasty, resulting in improved blood pressures. The review of the literature identified case series of pharmacologic, surgical, and endovascular therapies, although, endovascular therapies appear to be preferred due to lower morbidity and mortality. CONCLUSIONS: NF1 vasculopathy is a rare condition that most often presents with hypertension due to renal artery stenosis. In these situations, endovascular management is the preferred approach.

Winslow pathway collaterals: an unusual arterial network in Takayasu arteritis.

Takayasu arteritis which is reported more commonly from Asia and in females can present as middle aortic syndrome with lower limb claudication. We present a case of a young male with Takayasu arteritis with middle aortic syndrome and Winslow pathway collaterals with lower limb ischaemia, hypertension, coronary occlusion and stroke. The extensive collateral formation was visible as a clinical finding over the abdominal wall. The identification of these collateral pathways is essential in understanding the extent of haemodynamically significant disease and it alerts to the possibility of surgical injury during procedures like laparotomy or harvesting of internal thoracic artery for coronary artery bypass graft.

Mid-Aortic Syndrome: A Rare Cause of Renovascular Hypertension in Childhood Treated Percutaneously with an Unusual Vascular Access.

INTRODUCTION: Mid-Aortic Syndrome (MAS) is a rare vascular malformation characterized by segmental narrowing of the abdominal aorta and stenosis of its principal branches. Patients affected by MAS typically present malignant renovascular hypertension, with variable clinical symptoms like claudication, abdominal angina, and headache. Moreover, they can develop other complications, such as hypertensive encephalopathy, congestive heart failure and vascular brain accidents. Hypertension with MAS is often resistant to multidrug therapy, requiring a surgical approach to treat the clinical symptoms, prevent or block organ damage and normalize the blood pressure. CASE REPORT: Here, the case of a 4-year-old boy showing elevated blood pressure with left ventricular hypertrophy leading to idiopathic MAS, who was successfully treated with percutaneous transcatheter renal angioplasty (PTRA) using an unusual, anterograde access, is reported. DISCUSSION AND CONCLUSION: In children and adolescents, vascular malformations like MAS must be considered as a possible cause of hypertension. PTRA is a successful therapeutic strategy in children with severe renovascular hypertension. Anterograde access, using an axillary artery, can be a valid approach for PTRA when femoral access is difficult to achieve.

Total arch replacement for aortic arch aneurysm with coexisting middle aortic syndrome.

INTRODUCTION: Middle aortic syndrome (MAS) combined with thoracic aortic aneurysm (TAA) is a rare vascular disease. One stage open surgery to treat this condition, becomes a challenge for our cardiovascular surgery. PRESENTATION OF CASE: A 69-year-old man presented with a saccular type aortic arch aneurysm, shaggy aorta and severe atherosclerotic stenosis of the thoracoabdominal aorta with middle aortic syndrome and aberrant right subclavian artery, renovascular hypertension, renal dysfunction, and intermittent claudication of both legs. Total arch replacement procedure was performed under a cardiopulmonary bypass using aortic inflow from the right axillary artery and a femoro-femoral crossover bypass graft to avoid malperfusion of the lower body. Before weaning from the cardiopulmonary bypass, we established an extra-anatomical bypass from the ascending aortic graft to the femoro-femoral crossover bypass graft. 3D-CT showed patency of bypass graft without any sign of stenosis postoperative. The patient's postoperative course was uneventful and he was discharged from hospital with improvements in intermittent claudication, hypertension, and renal dysfunction. DISCUSSION: Although open surgery including graft bypass for MAS is more invasive than endovascular treatment, it could be performed successfully to preventing from intraoperative complication or complications postoperatively. CONCLUSION: Combined operation of total arch replacement and a bypass from the ascending aorta to the bifemoral arteries is alternative for MAS combined with TAA.

Rare complications of neurofibromatosis 1 diagnosed incidentally in two children.

Neurofibromatosis 1 (NF1) is an autosomal-dominant disorder with various clinical expressivity and complications. Arterial hypertension may be present in patients with NF1 and is secondary to vasculopathy or pheochromocytoma. We herein describe two children (17 and 4 years old) diagnosed late with NF1 after severe arterial hypertension had been discovered due to pheochromocytoma and middle aortic syndrome. Routine measurement of arterial pressure is mandatory in children with NF1, in order to diagnose the complications of this disease.