Idiopathic mid-aortic syndrome in children

Sethna, Christine B.; Kaplan, Bernard S.; Cahill, Anne Marie; Velázquez, Omaida C.; Meyers, Kevin

doi:10.1007/s00467-008-0767-4

Cited by 118 publications

(108 citation statements)

References 18 publications

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“…Midaortic syndrome, which is characterized by abdominal aorta stenosis, is an important cause of pediatric renovascular hypertension. A previous study reported that 91% of patients with midaortic syndrome had RAS (20). Renovascular hypertension patients with midaortic syndrome are often refractory to antihypertensive medications.…”

Section: Discussionmentioning

confidence: 99%

Angioplasty for pediatric renovascular hypertension: a 13-year experience

Zhu

He²,

Gu³

et al. 2014

Diagn Interv Radiol

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ORIGINAL ARTICLE PURPOSE We aimed to evaluate the long-term outcome and efficacy of percutaneous transluminal renal angioplasty (PTRA) for pediatric renal artery stenosis (RAS), which is an important cause of medication-refractory pediatric hypertension. MATERIALS AND METHODSWe retrospectively evaluated 22 hypertensive children (age range, 3-17 years) who underwent PTRA from February 2000 to July 2012. Sixteen patients had Takayasu arteritis and six fibromuscular dysplasia. Five were not included in the statistical analysis due to loss to follow-up. RESULTSTechnical success was achieved in 32 of 34 procedures (94.1%). The stenosis rate decreased from 84.5% before PTRA to 20.1% after PTRA. Treatment was effective in 72.7% (16/22) of patients, including complete cure in 27.3% (6/22) and improvement in 45.5% (10/22). Systolic and diastolic blood pressures decreased from 153±19.1 to 131.7±21.4 mmHg and from 97.9±14.2 to 83.6±19.3 mmHg, respectively (P < 0.01). Number of antihypertensive agents decreased from 2.7 to 0.5 per patient. Restenosis was detected in 40.9% (9/22) of patients, with a restenotic interval of 11.8 months (range, 3-47 months). Lesion length was strongly correlated with clinical success (cure and improvement) (independent-sample t test, P < 0.001; binary logistic regression, P = 0.040). CONCLUSIONLesion length is an important determination of clinical success with PTRA for pediatric RAS. PTRA is an appropriate treatment option for pediatric renovascular hypertension due to Takayasu arteritis and fibromuscular dysplasia.

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Section: Discussionmentioning

confidence: 99%

Angioplasty for pediatric renovascular hypertension: a 13-year experience

Zhu

He²,

Gu³

et al. 2014

Diagn Interv Radiol

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“…Both previously reported preterm infants with MAS died as a result of developing sequelae of severe hypertension, one due to cardiac dysfunction, the other as a consequence of IVH. 7,8 In conclusion, MAS in preterm infants is a rare but serious cause of hypertension. Although MAS is unusual in preterm infants, it must be considered in cases of unexplained hypertension.…”

Section: Patientmentioning

confidence: 88%

“…Only two other cases of preterm infants with MAS presenting at 31 and 35 weeks have been described. 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.…”

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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“…Most often, the patient is asymptomatic, but headache, vomiting, epistaxis, convulsions and other complication of severe hypertension are reported. Claudication and intestinal ischemia are present only in a minority of the patients, probably due to the gradual development of stenosis, which gives the body time to create effective collateral pathways [5]. From the embryological perspective, it has been suggested that a failure in fusion of the paired dorsal aortae during the fourth week of gestation may lead to MAS.…”

Section: Discussionmentioning

confidence: 99%

Mid-aortic dysplastic syndrome as a rare cause of hypertension in young

Saha¹,

Saha²,

Ranjit³

et al. 2013

IJCRI

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Introduction: Mid-aortic syndrome (MAs), coarctation of abdominal aorta is a rare disease with only 200 reported cases. It is characterized by constriction of distal thoracic and/or abdominal aorta and its branches, therefore is also known as abdominal aortic coarctation. the MAs is characterized radiologically by severe narrowing of abdominal aorta and its branches and most of these patients usually die due to progressive severe hypertension before the age of 35-40 if left untreated. case report: A 13-year-old boy was admitted with persistent headache and vomiting for one month and repeated generalized tonic clonic seizures for two days. His past history was unremarkable. the highlight of the clinical examination blood pressure was 240/150 mmHg in both the upper limbs, and all peripheral pulses were palpable. blood pressure was similar in upper and lower limbs and a systolic bruit heard over the epigastrium. Ultrasonography showed a localized narrowing of a suprarenal segment

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Idiopathic mid-aortic syndrome in children

Cited by 118 publications

References 18 publications

Angioplasty for pediatric renovascular hypertension: a 13-year experience

Angioplasty for pediatric renovascular hypertension: a 13-year experience

Mid-aortic syndrome in two preterm infants

Mid-aortic dysplastic syndrome as a rare cause of hypertension in young

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