Valsartan for slowing aortic widening in Marfan syndrome and related aortic diseases

Who can take part

• Age 1- 39 years. 2. Diagnosis of a syndrome classified under the HTAD group: • Marfan Syndrome • Diseases related to Marfan syndrome (rare diseases with aneurysms of the thoracic aorta and dissection of the aorta), among others: Loeys-Dietz syndrome, Vascular type of Ehlers-Danlos syndrome, Arterial Tortuosity syndrome, Shprintzen-Goldberg syndrome, Neonatal form of Marfan syndrome, Aneurysms-osteoarthritis syndrome, Multi-system smooth muscle dysfunction syndrome, Familial thoracic aortic aneurysms and aortic dissections, Bicuspid aortic valve syndrome - familial BAV. 3. Aortic root dilatation (z-score ≥ 2). 4. Signed informed consent to participate in the study

Who cannot take part

• Past aortic cardiac surgery. 2. Eligibility for aortic cardiac surgery at the time of examination. 3. Hemodynamically significant, severe or moderate aortic valve defect assessed by echocardiography. 4. Inability to obtain diagnostic echocardiographic images necessary for the evaluation of the aorta in a transthoracic echocardiographic examination - absence of the "Acoustic window". 5. Heart failure; defined as left ventricular ejection fraction <40%. 6. ARB therapy, unless 3 months have elapsed from the last dose taken before qualification. 7. Taking ACE inhibitors, unless 3 months have elapsed from the last dose taken before qualification. 8. Previous adverse reactions to valsartan or other ARB drugs. 9. Contraindications to valsartan (including hypersensitivity to the active substance or to any of the excipients, severe liver dysfunction, biliary cirrhosis, cholestasis, bilateral renal artery stenosis). 10. Breastfeeding, pregnancy or planning pregnancy in the next 12 months and for women of childbearing age, not on an effective method of contraception. 11. Known renal impairment as manifested by estimated creatinine clearance <30 ml/min in children and <10 ml/min in adults