S.Orsola Malpighi Hospital, Bologna INTRACRANIAL ANEURYSMS AND COARCTATION OF THE AORTA: A MAGNETIC ANGIOGRAPHIC SCREENING IN A PAEDIATRIC POPULATION OF 61 PATIENTS L.Spinardi, C.Leoni, M.Fabi, L.Faccioli, M. Pastore Trossello Units of Neuroradiology and Paediatric Cardiology S.Orsola Malpighi Hospital, Bologna

COARCTATION OF THE AORTA (CoA) Relatively common defect that accounts for 5-8% of all congenital heart defects Usually located in the thoracic aorta distal to the origin of the left subclavian artery at about the level of the ductal structure

DIAGNOSIS The diagnosis of coarctation of the aorta may be missed until the patient develops congestive heart failure (CHF), which is common in infants, or hypertension, which is common in older children

ASSOCIATED CLINICALLY SIGNIFICANT DEFECTS Bicuspid aortic valve and more rarely Mitral valve anomalies Patent ductus arteriosus, VSD, and aortic stenosis Coarctation of the aorta as a complicating feature of a more complex heart defect, such as transposition of the great arteries, Taussig-Bing anomaly, double-inlet left ventricle, tricuspid atresia with transposition of the great arteries

TREATMENT Surgical approaches: End-to-end anastomosis (EEA) (the preferred surgical approach for CoA in neonates as it appears to be free from subsequent cardiovascular pathology, at least until 5 years of age, compared to SFR) Reverse / Subclavian flap angioplasty (SFR)

TREATMENT Transcatheter treatments: Balloon angioplasty (children > 3 months old) Stent implantation (children > 60 kg)

RELATIONSHIP BETWEEN INTRACRANIC ANEURYSM AND AORTIC COARCTATION Aortic coarctation is often mentioned for a supposed association with brain aneurysms

CASE REPORT Hodes et al 1959 Robinson et al 1967 Serizawa et al 1992 Mercato et al 2002 Harikrishnan et al 2004 Victor et al 2005 Punsar et al 2005 Celik et al 2005 Solinas et al 2006 There are several case reports described in literature on adult patients…

ONLY ONE PROSPECTIVE STUDY ON A POPULATION OF 100 ADULT PATIENTS SUPPORTED THE HIPOTHESYS OF HIGHER FREQUENCY OF IA AMONG PATIENTS WITH COA IN COMPARISON TO NORMAL POPULATION (10% VS 2%)

..HYPERTENSION IS A SINGLE RISK FACTOR FOR DEVELOPING BRAIN ANEURSYMS BUT.. ALSO IN THIS STUDY MOST OF THE PATIENTS ARE ADULT WITH A LONG HYSTORY OF HYPERTENSION.. ..HYPERTENSION IS A SINGLE RISK FACTOR FOR DEVELOPING BRAIN ANEURSYMS

RELATIONSHIP BETWEEN INTRACRANIC ANEURYSM AND AORTIC COARCTATION UNTIL NOW TWO DIFFERENT HIPOTHESYS HAVE BEEN CONSIDERED TO GIUSTIFY THE SUPPOSED ASSOCIATION… Arterial hypertension and cronic haemodinamic stress Vascular developmental abnormalities originated from the neural ridge vs

J of Paediatrics and Child Health, 2006 IS SCREENING INDICATED IN ASYMPTOMATIC PATIENTS ? J of Paediatrics and Child Health, 2006

OBJECTIVE VERIFY THE ASSOCIATION BETWEEN COA AND IA IN A PAEDIATRIC POPULATION TREATED FOR COA

MATERIAL 61 patients, followed by the Department of Pediatric cardio-surgery of S. Orsola-Malpighi Hospital in Bologna, were included until now in our study: Mean age at MRA screening: 17 year old Mean age at diagnosis: 2,5 year old Mean age at treatment: 2,8 year old (< 18 year old) Treatment: 82% chirurgical, 18% endovascular (Retreated: 9) Hypertension: 28% has mild hypertension, controlled with Beta-blockers or ACE-inibitors Neurological symptoms: headache (6)

System 1.5 T GE Medical Systems USA MRI TECHNIQUE System 1.5 T GE Medical Systems USA MR angiography with time of flight technique (TOF) with MIP e VR reconstructions Sagittal T1 Fast Spin Echo Coronal T2 FSE Axial FLAIR sequences Images were valuated in blind by two neuroradiologists and an interventional neuroradiologist with experience in endovascular treatment of intracranial aneurysms

RESULTS MR angiography showed NO EVIDENCE OF INTRACRANIAL ANEURYSM, in any of our patient treated for aortic coarctation in childhood

The real incidence of intracranial aneurysm in children is debated and should be around 1-2 % Aortic coarctation has always been strictly and directly related to intracranial aneurysms and their rupture Several case reports in literature in fact support this relationship and, in particular, the prospective study made by Mayo Clinic in 2003 stated that the prevalence of intracranial aneurysms in a population of 100 people with CoA was 10 % DISCUSSION

What we have noticed is that to our knowledge until now no one has actually valued a population of children treated for aortic coarctation in paediatric age, and that most of these studies and case reports described patients who were treated in adulthood and most of which were also severely hypertense, or patients not treated at all and whose diagnosis of the presence of an intracranial aneurysm was the first sign of an unknown coarctation of the aorta In our study we have analyzed until now 61 pediatric patients all treated in childhood and we found NO evidence of intracranial aneurysms What we believe is that the direct relationship between aortic coarctation and intracranic aneurysms should be at lest called into question

CONCLUSION In our patients, all treated in childhood, there was no evidence of aneurysm There is no certain epidemiological and scientific evidence of a DIRECT relationship between CoA and Intracranial aneurysm A follow up of our population could be interesting and further investigations have to be done