Initial Angiotensin Receptor Blocker Response in Young Marfan's Patients Decreases after 3 Years of Treatment

Background: Aortic root dilatation is considered to be the most hazardous symptom in Marfan's syndrome (MFS) patients, since it can lead to even fatal dissections occurring as early as in childhood. Medical treatment with either β-blockers or angiotensin-receptor type-I blockers (AT1RB) is attempted to reduce progressive dilation in this patient group.

Method: We present a prospective longitudinal echocardiographic analysis of our single-center cohort of MFS children and adolescents. To predict efficacy of AT1RB, demographic parameters (age and sex), phenotypical expression within MFS (degree of cardiac involvement) and dosage of losartan were investigated in relation to vessel dysfunction in terms of tissue elasticity.

Results: Of our eligible 49 young MFS patients (27 males and 22 females), 22 patients initially decided against medical treatment (aortic root z-scores: 1.5 ± 0.9) but 6 switched to losartan later on due to accelerating aortic dilation. Further 27 patients agreed to losartan treatment immediately after diagnosis (aortic root z-scores 3.2 ± 0.9). Total follow-up duration covered 6.6 years in mean (range: 3–11 years). During the first 3 years of treatment, the 33 patients with losartan treatment presented aortic root z-score regression, while the z-score of the 22 without medication progressed mildly (−0.04 ± 0.17/year vs. 0.09 ± 0.24/year, p = 0.03). During the following 3 years, progression in both groups reversed and therefore balanced out if assessed over the entire 72 months follow-up period (0.07 ± 0.1/year vs. 0.04 ± 0.1/year, p = 0.468). Although males exhibited higher aortic z-scores, progressions were comparable to females and not age dependent. Elasticity ameliorated in unison with aortic regression in the treated patients initially but deteriorated similarly afterward. Increasing the dosage of medication seemed to enhance the improvement in the more severely affected children.

Conclusion: Losartan evoked a significant aortic root z-score regression in young MFS patients over the first 3 years but this effect seemed to mitigate thereafter. Longer term follow-up and eventually increase of losartan dosage are necessary.

Publication History

Article published online:
12 February 2022