Midaortic syndrome and hypertension in childhood

O’Neill, James A.; Berkowitz, Henry D.; Fellows, Kenneth J; Harmon, Carroll M.

doi:10.1016/0022-3468(95)90555-3

Cited by 73 publications

(48 citation statements)

References 15 publications

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“…Previous pathological analyses have shown fibrous thickening of the intima of the affected vessels in moyamoya disease [1, 5, 10]. There have also been similar findings in the stenotic aorta with midaortic syndrome [2, 3]. Common mechanisms may underlie the pathogenesis of moyamoya disease and midaortic syndrome.…”

Section: Discussionsupporting

confidence: 63%

“…Although the term ‘midaortic syndrome’ was not used, the clinical symptoms of their case including renovascular hypertension, and angiographic findings may indicate midaortic syndrome because midaortic syndrome has been depicted as a stenotic vascular disorder involving the abdominal aorta and its branches, frequently demonstrating renovascular hypertension [2]. However, the relationship between midaortic syndrome and fibromuscular dysplasia is unclear because the pathogenesis of midaortic syndrome is not well understood [3]. Midaortic syndrome has been reported to be associated with subarachnoid hemorrhage due to ruptured cerebral aneurysm [12, 13].…”

Section: Discussionmentioning

confidence: 99%

“…The etiology of the disease is still uncertain, while the disease is predominantly found in Japanese children, suggesting the involvement of hereditary and racial factors [1]. Midaortic syndrome is a rare vascular disease characterized by coarctation of the abdominal aorta and its visceral branches in children, often involving the renal arteries and causing severe renovascular hypertension [2, 3]. Although renal artery coarctation and renovascular hypertension in patients with moyamoya disease has been reported [4, 5], midaortic syndrome with moyamoya disease is not found in the literature.…”

Section: Introductionmentioning

confidence: 99%

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Moyamoya Disease Associated with Midaortic Syndrome

Korematsu¹,

Yoshioka²,

Maruyama³

et al. 2006

Pediatr Neurosurg

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We report a 1-year-old girl who presented moyamoya disease associated with midaortic syndrome. She had been treated for cardiac failure and severe hypertension due to midaortic syndrome until she suffered seizure and repeated cerebral ischemic attack. Cerebral angiography revealed stenosis of the bilateral internal carotid artery at its terminal portion. She was successfully treated with encephaloduroarteriosynangiosis, and ischemic attack ceased postoperatively. This is the first report of moyamoya disease with midaortic syndrome. Although cerebral ischemic attack has been effectively managed by encephaloduroarteriosynangiosis, renovascular hypertension is still difficult to control.

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Section: Discussionsupporting

confidence: 63%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Moyamoya Disease Associated with Midaortic Syndrome

Korematsu¹,

Yoshioka²,

Maruyama³

et al. 2006

Pediatr Neurosurg

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“…76 In another retrospective series of 17 patients, most of whom did not have elevated inflammatory markers, only 3 had lower-extremity symptoms, although in 4 patients a substantial blood pressure gradient could be measured between the arms and the legs. 77 Treatment of midaortic syndrome has classically been surgery. Recurrence with percutaneous transluminal angioplasty is said to be high owing to extensive aortic involvement and significant elastic recoil and has been proposed as a bridge to surgery.…”

Section: Idiopathic Midaortic Syndromementioning

confidence: 99%

Nonatherosclerotic Arterial Disorders of the Lower Extremities

Weinberg

Jaff

2012

Circulation

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“…7,8 Patients with MAS are usually diagnosed during childhood or adolescence though presentation in infancy has been reported. [9][10][11][12][13][14][15][16] It is speculated that this recent trend towards earlier age at presentation may be the result of improved diagnostic methods and an increase in premature births. 10 Preterm infants diagnosed with MAS likely have a very poor prognosis.…”

Section: Patientmentioning

confidence: 99%

Mid-aortic syndrome in two preterm infants

et al. 2012

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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...

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Midaortic syndrome and hypertension in childhood

Cited by 73 publications

References 15 publications

Moyamoya Disease Associated with Midaortic Syndrome

Moyamoya Disease Associated with Midaortic Syndrome

Nonatherosclerotic Arterial Disorders of the Lower Extremities

Mid-aortic syndrome in two preterm infants

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