BGN

Management

To date, a limited number of families with Meester-Loeys syndrome (MLS) has been identified. The current treatment is similar to that of other syndromes with thoracic aortic aneurysm involvement, including Marfan syndrome and Loeys-Dietz syndrome.

Cardiovascular disease is the major cause of morbidity and mortality in syndromic aortic aneurysm disorders. This underscores the importance of frequent cardiovascular monitoring. Individuals with MLS should undergo echocardiography on a regular basis to monitor the thoracic aortic diameters (root and ascending) and to evaluate both morphology and functionality of the heart valves. Medical management of cardiovascular features involves strict control of blood pressure to reduce hemodynamic stress on the arterial wall. β-blockade has historically been used and is still commonly used. Angiotensin II receptor blockers such as losartan are hypothesized to be beneficial because of both blood pressure lowering and desirable biochemical effects within the vessel wall including attenuation of TGFβ signalling. MLS-related aneurysms are amenable to prophylactic surgical intervention. Decisions regarding the precise timing of surgery are based upon integration of many variables and needs to be individualized.

Malformations, dysfunction or pain ensuing from the extra-cardiovascular anomalies are dealt with in a case-by-case manner, based on the input of a multidisciplinary team of medical specialists.