The Middle Aortic Syndrome

Sen, P. K.; Sg, Kinare; Engineer, S. D.; Gb, Parulkar

doi:10.1136/hrt.25.5.610

Cited by 224 publications

(118 citation statements)

References 7 publications

Supporting

Mentioning

101

Contrasting

Unclassified

Order By: Relevance

“…The inflammation leads to either stenosis and occlusion of the involved artery or aneurysm formation or both. [1][2][3][4][5] The arterial lesions can lead to secondary hypertension, retinopathy, cardiac involvement, cerebrovascular events, and premature death. The course and prognosis of patients with aortoarteritis show wide variation, and few authors have published systematic studies documenting the natural history of this disease.6-9 While the etiology of aortoarteritis remains unknown, various modes of treatment including steroids, vascular surgery, and balloon angioplasty have been used for management of these patients.7"10-13 To make a decision about elective intervention, the physician must have data on the natural history and be able to make an objective assessment of the prognosis in the individual patient.…”

mentioning

confidence: 99%

Natural history of aortoarteritis (Takayasu's disease).

1989

View full text Add to dashboard Cite

The natural history of aortoarteritis was studied in 88 patients (54 women and 34 men). The average age was 24.0±8.8 years at onset of symptoms and 28.3±9.9 years at diagnosis. The inflammatory disease involving the aorta, the arteries arising from the aorta, and, frequently, the pulmonary arteries. The inflammation leads to either stenosis and occlusion of the involved artery or aneurysm formation or both.1-5 The arterial lesions can lead to secondary hypertension, retinopathy, cardiac involvement, cerebrovascular events, and premature death. The course and prognosis of patients with aortoarteritis show wide variation, and few authors have published systematic studies documenting the natural history of this disease.6-9 While the etiology of aortoarteritis remains unknown, various modes of treatment including steroids, vascular surgery, and balloon angioplasty have been used for management of these patients.7"10-13 To make a decision about elective intervention, the physician must have data on the natural history and be able to make an objective assessment of the prognosis in the individual patient. Our study documents the clinical course, complications, and survival of 88 patients with aortoarteritis who had systematic follow-ups at our center.

show abstract

mentioning

confidence: 99%

Natural history of aortoarteritis (Takayasu's disease).

1989

View full text Add to dashboard Cite

show abstract

“…3 Mild stenosis may be completely asymptomatic; with the progression of the disease, symptoms may arise, such as dyspnea, lipothymic episodes, fatigue, headache, upper limb hypertension, and lower limb hypoperfusion with claudication. 1 Diagnostic suspicion of a steno-occlusion of the thoracic aorta may be prompted by echo-color Doppler findings of poststenotic blood flow abnormalities in the splanchnic and renal arteries and the downstream vessels bilaterally, as seen in this patient. As the disorder can localize to any segment of the aorta, second-level imaging will better reveal the relationship between the steno-occlusion and the emergence of supra-aortic vessels and help to evaluate the extension of lumen narrowing, which actually involves the infrarenal aorta in <2% of cases.…”

Section: Discussionmentioning

confidence: 85%

“…The middle aortic coarctation is a rare syndrome characterized by severe calcifications of the descending aorta, mostly affecting adolescents or young adults. 1 It may be caused by congenital or inflammatory disorders, whose commonest disease is the Takayasu arteritis. 2 A similar nosologic entity is the ''coral reef aorta,'' which however involves the portion between the diaphragm and the suprarenal part of this vessel (visceral aorta).…”

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Open Surgical Treatment of a Severe Case of Obstructive Calcifying Aortic Disease

Rispoli

Varetto

Castagno

et al. 2015

Annals of Vascular Surgery

View full text Add to dashboard Cite

The obstructive calcifying aortic disease refers to severe calcifications of the descending aorta that obstruct or slow blood flow. Here, we report the case of a 65-year-old woman with recent onset of a very tight intermittent claudication and concomitant severe and uncontrolled hypertension, treated with a bypass graft between the proximal descending thoracic aorta and the supravisceral abdominal aorta.Severe stenosis or total occlusion of the descending aorta usually involving the origin of the visceral and renal arteries is a rare pathologic entity usually called middle aortic syndrome. 1 The commonest etiologies are congenital disorders or inflammatory disease, and adolescents or young people are more commonly affected. Herein, we describe the case of an unusual localization limited to the descending aorta and not involving the visceral vessels without any congenital or inflammatory explanation. CASE REPORTA 65-year-old woman was admitted to our department because of worsening intermittent claudication of the lower limbs and uncontrolled severe hypertension despite 3 medications (b blockers, diuretic, a1-adrenergic blocker). Her past medical history also included type 2 diabetes mellitus treated with oral hypoglycemic agents and dyslipidemia treated with statins; no history of smoke was present. Over the past months, she experienced worsening of general health, with episodes of intense asthenia, headache, dizziness and disorientation, and dyspnea after mild exertion. Physical examination revealed bilateral hyposphygmic femoral pulses and the absence of peripheral pulses. Ankle-brachial index (ABI) was 0.3 on both sides.Echo-color Doppler of the proximal abdominal aorta and iliac arteries showed poststenotic flow in the celiac trunk, superior mesenteric artery, both renal arteries, and the iliac and femoropopliteal axes bilaterally. The supra-aortic vessels appeared normal. Echocardiography demonstrated mild left ventricular hypertrophy, ectasia of the ascending aorta, and calcific stenosis of the descending aorta downstream from the isthmus. A computed tomography angiography (CTA) showed a normal ascending aorta and aortic arch, a severely calcified descending aorta with a pseudocoarctation in the proximal portion (18-mm diameter) and tight stenosis in the mid-distal segment, with reduction of the vessel lumen to a few millimeters just above the celiac trunk (Fig. 1). The splanchnic arteries, the infrarenal aorta, and the renal and the iliac arteries, although severely hypoperfused, were all morphologically normal and free from calcifications.Preoperative blood chemistry showed no significant anomalies. Spirometry demonstrated mild-to-moderate restrictive lung function. Coronary angiography revealed no abnormalities of the coronary arteries, and aortography showed an 85emm Hg pressure gradient at the lower extremity of the restricted aorta. Total body positron emission tomography was performed to rule out focal inflammatory processes in the aorta, but it did not show any abnormal tracer distribution. Antibod...

show abstract

“…1 It is characterized by segmental narrowing of the distal thoracic or abdominal aorta and stenosis of one or both renal arteries and major splanchnic vessels. [1][2][3][4][5][6] Diagnosis of MAS in premature infants is rare; this report describes the presentation and clinical course in two such patients.…”

Section: Introductionmentioning

confidence: 99%

Mid-aortic syndrome in two preterm infants

et al. 2012

View full text Add to dashboard Cite

We report mid-aortic syndrome (MAC) in two preterm infants. Both infants developed malignant hypertension refractory to medical therapy and died early in infancy. Thus far, this account is of the two youngest patients with MAC. Journal of Perinatology (2012) 32, 390-392; doi:10.1038/jp.2011 Keywords: preterm; mid-aortic syndrome; hypertension Introduction Mid-aortic syndrome (MAS) is a rare but important cause of renovascular hypertension in children and young adults. 1 It is characterized by segmental narrowing of the distal thoracic or abdominal aorta and stenosis of one or both renal arteries and major splanchnic vessels. 1-6 Diagnosis of MAS in premature infants is rare; this report describes the presentation and clinical course in two such patients. Case Patient 1The patient was a male infant born to a 20-year-old G 2 P 1 woman. Fetal Echocardiogram (ECHO) at 27 weeks detected premature closure of the ductus arteriosus, diminished right ventricular (RV) systolic function and RV hypertrophy. The patient was delivered by cesarean section at 28 weeks for worsening RV function. He required intubation, chest compressions and intratracheal epinephrine for apnea and bradycardia at 2 min of life. APGAR scores were 7, 1 and 7, at 1, 5 and 10 min, respectively. Birth weight was 1605 grams. Heart rate: 180 beats per min, respiratory rate: 52 breaths per min, preductal and postductal saturations: above 95% on FiO 2 of 0.3 and conventional mechanical ventilator support. There was a significant blood pressure (BP) difference between the upper and lower extremities: right arm 78/43 mm Hg, left arm 52/39 mm Hg, right leg 37/27 mm Hg, left leg BP could not be obtained. He had no appreciable dysmorphic features. Breath sounds were normal; murmurs were absent. There was no hepatosplenomegaly or abdominal bruit. The right brachial pulse was well felt, but the left brachial and femoral pulses were diminished.Postnatal ECHO demonstrated a widely patent right aortic arch, a small ductus arteriosus and severely diminished RV systolic function. A computed tomography (CT) angiogram identified narrowing of the abdominal aorta just below the level of the origins of the celiac and superior mesenteric arteries that measured 1.4 Â 1 mm at its narrowest infrarenal portion (Figure 1). The abdominal aorta then increased in diameter (2.7 mm Â 2.4 mm) just before the iliac bifurcation. Enlarged paraspinal/lumbar collaterals fed into the abdominal aorta at multiple levels. The renal arteries were diminutive without collateral supply to the kidneys. Renal ultrasound demonstrated normal sized kidneys. Serum creatinine levels peaked on the third day of life at 1.3 mg dl À1 and normalized to 0.6 mg dl À1 by the fourth week of life. Williams and 22q11 deletion syndrome were excluded by genetic testing.He remained on ventilator support for 5 days and was extubated to continuous positive airway pressure. He received inhaled nitric oxide and Dopamine for 3 days. The patient's course was marked by severe systemic hypertension with right arm systolic BP...

show abstract

The Middle Aortic Syndrome

Cited by 224 publications

References 7 publications

Natural history of aortoarteritis (Takayasu's disease).

Natural history of aortoarteritis (Takayasu's disease).

Open Surgical Treatment of a Severe Case of Obstructive Calcifying Aortic Disease

Mid-aortic syndrome in two preterm infants

Contact Info

Product

Resources

About