ESPN 50th Annual Meeting

ESPN 2017


 
Refractory hypertension and arterial stiffness in a toddler with neurofibromatosis type 1
OLGA MALIACHOVA 1 STELLA STABOULI 1 EUTHYMIA VARGIAMI 1 NIKOLETA PRINTZA 1 KONSTANTINOS PAPAZOGLOU 2 MARIA KYRIAZI 1 JOHN DOTIS 1 ATHANASIA ANASTASIOU 3 FOTIOS PAPACHRISTOU 1 DIMITRIOS ZAFEIRIOU 1

1- 1st Department of Pediatrics, Aristotle University Thessaloniki
2- 5th Department of Surgery, Aristotle University Thessaloniki
3- Department of Radiology, Hippocratio General Hospital
 
Introduction:

Arterial hypertension due to renal artery stenosis or mid-aortic syndrome is a common finding in patients with Neurofibromatosis (NF) type 1 (20%). Arterial stiffness is used to describe the elasticity of the arteries, it reflects the procedure of aging on the vascular system and is associated with increased cardiovascular risk, chronic renal disease and arterial hypertension. It can be assessed non-invasively through the determination of the carotid femoral pulse wave velocity (cf-PWV). The purpose of this case is to describe the correlation between arterial stiffness and arterial hypertension as documented with office Blood Pressure (BP) levels and ambulatory BP levels.

Material and methods:

We report a case of refractory hypertension accompanied with increased arterial stiffness in a 4 year-old girl with NF type 1 and mid-aortic syndrome. She presented initially with office BP levels below the 90th percentile but with a routine ambulatory blood pressure monitoring (ABPM) with a nondipping profile. The patient underwent percutaneous transluminal angioplasty (PTA). Office BP presented a decrease at 4 months after PTA. In constrast, ambulatory BP levels increased and were accompanied by an increased in cf-PWV. After 8 months renal function was improved, the hypertention persisted , but there was a significant reduction of ambulatory BP levels, which agrees with the reduction in cf-PWV values, despite sustained office BP elevation.

Results:

Masked hypertension may be present and a nondipping profile may enable the early diagnosis of arterial hypertension. There seems to be a superiority of ABPM in the assessment and follow up of hypertension and a better correlation to arterial stiffness.

Conclusions:

Renovascular hypertension may have adverse effect on arterial stiffness. Regular assessment of BP using ABPM may enhance the medical care of patients with NF type 1 and guide their further Management.