Successful prosthetic graft replacement for middle aortic syndrome in a 9-year-old child.

Middle aortic syndrome typically occurs as severe hypertension in young patients who have weak or absent femoral pulses and an abdominal bruit. It results from a diffuse narrowing of the distal thoracic and abdominal aorta, commonly involving the visceral and renal arteries. The clinical presentation, angiographic assessment, and surgical outcome of 10 patients (mean age: 19.5 years) who underwent one-stage revascularization for middle aortic syndrome were reviewed to determine the effectiveness and durability of one-stage revascularization techniques to relieve these complications. All patients were hypertensive (mean blood pressure: 176 mmHg); six (60%) had severe, poorly controlled hypertension, two of whom had previous failed operations for renovascular hypertension and one who presented with malignant hypertension and acute renal failure. Five patients had disabling myocardial insufficiency, only one of whom had documented coronary artery disease. Four patients had intermittent claudication. Aortography showed variable length high-grade midaortic stenosis, nine had visceral artery involvement, and eight had renal artery involvement. All patients underwent one-stage revascularization by a variety of autogenous and prosthetic techniques. The postoperative recovery was uncomplicated in eight of nine patients and was often associated with dramatic reduction in blood pressure. There was a single death from disruption of the thoracic anastomosis in a patient who had diffuse cystic medial necrosis of the aorta. Arterial biopsy in nine patients indicated evidence for both acquired and congenital origins of the midaortic stenosis. Late follow-up evaluation (mean: 4.1 years) showed normal growth and development, preservation of renal function, and relief of myocardial insufficiency in all patients. Seven patients (77%) are cured of their hypertension, and two (23%) have only mild hypertension. These results indicate that one-stage revascularization of patients with middle aortic syndrome can result in effective and durable relief of these severe life-threatening complications.

To discuss Doppler ultrasonographic and clinical features of middle aortic syndrome (MAS). Doppler ultrasonographic images and clinical dates of 11 patients with MAS confirmed by angiography were retrospectively analyzed from January 2004 to September 2016. 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. 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.

Characteristic change in local pulse wave velocity in different segments of the atherosclerotic aorta in KHC rabbits

BackgroundMiddle 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.ConclusionsAlthough 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.

Congenital coarctation of the thoracic aorta at the ligamentum arteriosum or the aortic arch is well recognized. But a much less common variety (0.5-2.0%) of aortic coarctation is located in the distal thoracic aorta or abdominal aorta or both and is often called "middle aortic syndrome" or "mid-aortic dysplastic syndrome". This represents serious pathological condition and indicates multidisciplinary therapy approach. From 1996 to 2007, at the Vascular Surgery Clinic of the Institute for Cardiovascular Diseases "Dedinje", Belgrade, three patients were treated due to abdominal aorta coarctation, two females aged 55 and 50 and a 4-year-old child. The patients were treated surgically (by-pass with a prosthetic graft and patch angioplasty) and endovascular- percutaneous transluminal angioplasty (PTA) with and without a stent. The follow-up period was 3-70 months. In the 50-year-old patient, angiography showed severe narrowing of the suprarenal segment of the abdominal aorta. Thoraco-abdominal bypass with a 16 mm dacronic tubular graft was performed. In the 4-year-old patient angiography also showed a suprarenal aorta narrowing. In the first act patch angioplasty was performed and after PTA of the visceral arteries was done on several occasions. In the 55-year-old patient, after diagnostic angiography, infrarenal aorta coarctation was registered. PTA was performed with stent placement. All patients were asymptomatic on control check-ups. Abdominal coarctation is a pathological disease which is seldom found in vascular surgery. Angiography is of major importance for setting the diagnosis and for the control of the results of surgical and nonsurgical treatment. The combination of surgical and endovascular treatment in our patients showed very good results in the studied period.

Moyamoya arteriopathy and middle aortic syndrome (MAS) are each rare, often progressive vascular diseases; their comorbid intersection increases their complexity because of the independent and contradictory demands on blood pressure. The management of pediatric patients with these comorbid conditions requires multidisciplinary consultation given the high risk involved. In the current study, the authors aim to describe the clinical and surgical history of pediatric patients with comorbid moyamoya arteriopathy and MAS and describe a multidisciplinary approach to care for these patients. This study is a retrospective review of the clinical and radiological records of patients at Boston Children's Hospital who were treated with cerebral revascularization surgery for comorbid moyamoya arteriopathy and MAS from January 2004 to January 2024. Analysis of collected perioperative, surgical, inpatient, and follow-up data was conducted using R version 4.4.2 (R Foundation for Statistical Computing). Eleven patients, 5 male (45.5%), were included in the study. At presentation, 9 patients (81.8%) had symptoms from brain ischemia. Nine patients were on antihypertensive medications (median 2, IQR 1-2). All patients underwent surgery for moyamoya arteriopathy, and 6 patients (54.5%) also underwent surgical treatment for MAS. After moyamoya surgery, 4 patients (36.4%) required an intensified blood pressure regimen to achieve the same goal, whereas 3 patients (27.3%) had a reduced need for blood pressure medications. Four (66.7%) of the 6 patients who underwent MAS surgical treatment did so after moyamoya treatment. Perioperative stroke occurred after renal stent angioplasty before moyamoya surgery but also after aortic bypass following moyamoya surgery. Managing comorbid moyamoya arteriopathy and MAS is challenging, as patients have a higher risk of ischemic perioperative complications. These patients may have hypertension secondary to their aortic disease, and treating the aortic disease may correct the hypertension but with possible new relative hypotension and relative cerebral hypoperfusion. Consequently, moyamoya revascularization prior to aortic repair may be a strategy to reduce the risk of stroke.

Objectives: To explore the clinical features and therapeutic algorithm of middle aortic syndrome (MAS) caused by childhood Takayasu arteritis (c-TA). Methods: Data were retrospectively analyzed of 103 c-TA patients in Fuwai Hospital between 2002/01 and 2018/01. TA was diagnosed according to the criteria recommended by ACR (1990) and the EULAR/ PRINTO/ PRES (2010). MAS was defined as stenotic lesions of the distal thoracic or abdominal aorta. Results: A total of 43 c-TA patients were diagnosed with MAS (F/ M: 5.1/1). The mean age of TA onset is 13.4 ± 2.9 years. Hypertension is the most popular presentation (32, 74.4%), followed by respiratory distress (44.2%) and claudication (33.3%). Heart failure is confirmed in 34.8%. Bruits and decreased pulse were remarkable (79.1%;34.9%). Active disease according to NIH criteria was found in 55.8%. According to the angiographic classification from Takayasu's Consensus Conference in 1994, type II, III, IV and V account for 20.9%, 14.0%, 27.9% and 37.2%. Abdominal aorta (67.4%), thoracic aorta (62.8%), common carotid artery (62.8%) and renal artery (58.1%) were popularly involved. Glucocorticoid (23.2 ± 9.0 mg), anti-hypertensive and antiplatelet drugs are used in 100%, 74.4% and 72.1%. Stenting, angioplasty and bypass surgery were performed in 32.6%, 32.6% and 7.0%. At a follow-up of 3.7 ± 3.2 years, new stenosis, aneurysm and cerebral infarction were found in 13, 2 and 1 cases, while 7 experienced revascularization procedures again. Conclusion: MAS occurs in approximate half of c-TA with an inclination to females, active clinical course, extensive vascular lesions and poor outcomes. Hypertension, respiratory distress/heart failure, claudication, bruits or bilateral lower extremity pulse deficits remind of c-TA with MAS. Glucocorticoid and interventional therapy are significant.

Middle aortic syndrome (MAS) is a rare clinical entity in childhood, characterized by a severe narrowing of the distal thoracic and/or abdominal aorta, and associated with significant morbidity and mortality. MAS remains a relatively poorly defined disease. This paper systematically reviews the current knowledge on MAS with respect to etiology, clinical impact, and therapeutic options. A systematic search of 3 databases (Embase, MEDLINE, and Cochrane Central Register of Controlled Trials) yielded 1,252 abstracts that were screened based on eligibility criteria resulting in 184 full-text articles with 630 reported cases of childhood MAS. Data extracted included patient characteristics, clinical presentation, vascular phenotype, management, and outcomes. Most cases of MAS are idiopathic (64%), 15% are associated with Mendelian disorders, and 17% are related to inflammatory diseases. Extra-aortic involvement including renal (70%), superior mesenteric (30%), and celiac (22%) arteries is common, especially among those with associated Mendelian disorders. Inferior mesenteric artery involvement is almost never reported. The majority of cases (72%) undergo endovascular or surgical management with residual hypertension reported in 34% of cases, requiring medication or reintervention. Clinical manifestations and extent of extra-aortic involvement are lacking. MAS presents with significant involvement of visceral arteries with over two thirds of cases having renal artery stenosis, and one third with superior mesenteric artery stenosis. The extent of disease is worse among those with genetic and inflammatory conditions. Further studies are needed to better understand etiology, long-term effectiveness of treatment, and to determine the optimal management of this potentially devastating condition.

Localized cystic disease of kidney (LCDK) is a rare, non-familial, non-progressive renal disorder that is not associated with cysts or disorders in other organs. Only a few cases have been reported in the literature. While this condition is morphologically identical to the autosomal dominant form of polycystic kidney disease, it is not inherited and is not associated with significant deterioration of renal function. We present a case of a 16-year-old male patient who suffered from hypertension for over two years. On imaging we found several, variable-sized cysts in the upper half of the right kidney. The left kidney and lower segment of the right kidney were normal. Selective renal vein catheterization and sampling showed markedly elevated renin level in the right upper segmental vein (92 pg/ml, normal value: 11-33 pg/ml). The patient underwent a right upper heminephrectomy and histopathology was suggestive of LCDK. After surgery, the patient's blood pressure returned to normal levels without any need of antihypertensive medication and he is under follow-up on outpatient basis for the past two years.

Congenital coarctation of the thoracic aorta at the ligamentum arteriosum or the aortic arch is well recognized. But a much less common variety (0.5% to 2.0%) of aortic coarctation is located in the distal thoracic aorta, or abdominal aorta, or both and is often called "middle aortic syndrome" or "mid-aortic dysplastic syndrome." These types of aortic coarctation are most often secondary to a form of granulomatis vasculitis commonly known as Takayasu's disease in this country or aortitis syndrome in Japan. No single genesis explains every case and beside vasculitis as a cause, some are thought to be congenital in origin and others are associated with von Recklinghausen's disease. Eight patients with severe hypertension or claudication secondary to middle aortic coarctation were studied with aortograms and subsequently treated by vascular reconstruction procedures. Vascular reconstructions consisted of aortoaortic bypass, aortic resection with interposed grafting, reanastomotic resection of renal arteries into prosthetic grafts, and renal artery bypass with autogenous material. All eight patients' grafts have remained patent, with followups of 4 to 9 years, with relief of hypertension and claudication. Although Takayasu's disease can be progressive, aggressive surgical treatment in eight patients followed for 4 to 8 years postoperatively demonstrates that severe hypertension, claudication, or both are important indications for revascularization. Whatever the cause, assuming that active aortic inflammation has been medically treated and is in a burned-out state, patients with abdominal coarctation who have symptomatic renovascular hypertension, claudication, or both are good candidates for revascularization. Although surgical repair is more difficult than with congenital thoracic coarctation, because aortic walls are fibrotic and often also involve the renals, all eight of our patients had successful longterm correction of their hypertension and coarctation.

An exaggerated blood pressure (BP) response to test may unmask the subjects who have a high risk of developing hypertension. In this prospective 10 years of follow-up, we examined whether the predictive value of casual BP measurements on future BP level and need for antihypertensive medication could be improved by using BP responses to different physical tests. At baseline, BP was recorded by casual measurements and intra-arterial monitoring. During the intra-arterial BP recording, standardized postural and exercise tests were performed on 97 healthy, untreated men (34 normotensive, 29 borderline hypertensive, and 34 mild hypertensive). After 10 years of follow-up, 87 of them (90%) returned for casual and non-invasive 24-h BP measurements. At follow-up, 20 (23%) of the men had antihypertensive medication. The prediction of casual systolic blood pressure (SBP) was best improved by SBP at 10 min after the dynamic exercise test (adj. R2 = 0.448; adj. R2 = 0.356 for casual SBP alone). The prediction of casual diastolic blood pressure (DBP) was most improved by DBP at 10 min after the dynamic exercise test (adj. R2 = 0.282; adj. R = 0.259 for casual BP alone). SBP in the supine test best improved the prediction of 24-h SBP (adj. R2 = 0 448; adj. R2 = 0.275 for casual SBP alone). DBP in the standing test best improved the prediction of 24-h DBP (adj. R2 = 0.252; adj. R2 = 0.214 for casual DBP alone). Pre-exercise DBP and casual SBP were the best predictors of the need for antihypertensive medication (Cox-Snell R2 = 0.256; Cox-Snell R2 = 0.164 for casual SBP alone). In conclusion the prediction of future BP and need for antihypertensive medication can be improved by using BP measurements during postural and exercise tests. Future SBP is more predictable than DBP.

Middle aortic syndrome (MAS) is very uncommon vascular pathology characterized by a long segmental narrowing or obstruction of the abdominal and/or distal thoracic aorta, commonly involving with the visceral and renal arteries. This syndrome may be presented with various physical signs of coarctation of the aorta, including resistant hypertension, renal insufficiency and/or mesenteric ischemia. Here, we report a case of a 64-year-old man with severe hypertension. He was diagnosed with MAS associated with stenosis of visceral and renal vessels by use of computed tomography and magnetic resonance angiography. (J Korean Soc Hypertens 2013;19(1):39-43)

Middle aortic syndrome in childhood-onset Takayasu arteritis

Objective: To determine antepartum risk factors for postpartum antihypertensive medication use in women with severe preeclampsia. Methods: A case control study was performed on patients who were diagnosed with severe preeclampsia between January 2000 and June 2004 at a single tertiary care center. Women discharged from the hospital on antihypertensive medications were compared to women discharged home on no antihypertensive medications. Demographic data, maternal medical conditions, and delivery data were abstracted from maternal charts. Risk factors were evaluated using multiple logistic regression. Results: 218 patients with severe preeclampsia were identified, of which 112 were discharged on antihypertensives. After adjusting for confounding variables, chronic hypertension was associated with an increased need for post partum antihypertensive medication (OR 7.5 (95% CI 3.0–18.1)). A dose-dependent association was seen with intrapartum hydralazine administration. High-dose hydralazine was associated with increased need for postpartum antihypertensive mediation (OR 5.74 95% CI 2.03–16.2) compared to low-dose hydralazine (OR of 2.51 95% CI 1.26–5.01). Hemolysis/Elevated liver function/low platelet (HELLP) syndrome was associated with a decreased need for antihypertensive medication (OR 0.33, 95% CI 0.13–0.82). Conclusions: Patients with chronic hypertension and patients who required intrapartum hydralazine were more likely to require antihypertensive medications at discharge.

Surgical Treatment of Middle Aortic Syndrome with Takayasu Arteritis or Midaortic Dysplastic Syndrome