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The Impact of a Bicuspid Aortic Valve on Aortic Geometry and Function in Patients with Aortic Coarctation: A Comprehensive CMR Study

Congenital Heart Disease
Vol.15, No.2, pp. 117-125, 2020, DOI:10.32604/CHD.2020.011520

Coralie Katharina Dicks, Gerhard-Paul Diller, Kristina Wasmer, Paul C. Helm, Ulrike M. M. Bauer, Helmut Baumgartner, Stefan Orwat, Alicia Jeanette Fischer

Abstract

Background

Sudden cardiac death is a leading cause of death in patients with congenital heart disease (CHD). Risk stratification for implantable cardioverter defibrillators (ICD) remains difficult due to limited data about use and outcome of device therapy in CHD patients in larger community-based cohorts.

Methods and results

Out of a dataset with more than 50,000 patients registered at the German National Register for Congenital Heart Defects, 109 patients (median age 35.5; IQR 23.75–46.00), 68 (62%) male) with an ICD were identified and were retrospectively analyzed. Although the number of implantations increased steadily throughout the investigated time interval from 2001 to 2015, only 0.2% of the CHD patients in the national register received an ICD. Indication for ICD implantation was secondary prevention in 84 patients (78%) and primary prevention in 24 patients (22%). 23 patients (21%) of the ICD patients received appropriate ICD therapy. 7 patients (6%) received an inappropriate ICD therapy. In 23 patients (21%) device complications were documented with a high number of lead fractures and insulation defects (n = 14, 13%).

Conclusion

The current study investigates the clinical uptake and use of ICD therapy based on a large national registry for CHD patients. Despite a steady increase in the number of implanted devices, ICD uptake remains relatively low, particularly for primary prevention. The data suggests a potential reluctance in utilization of device therapy in this patient cohort for primary prevention. Selecting patients in whom benefits outweigh the risks associated with lifelong ICD therapy remains challenging.

Full Article

Commentary

The current study has compared CoA patients with a bicuspid aortic valve (BAV) to those with a tri-leaflet aortic valve regarding LV size and function as well as measures of aortic stiffness [distensibility and pulse wave velocity (PWV)] and aortic geometry. It is generally held that the association of BAV with coarctation portends a worse prognosis for adverse aortic events such as aortic dilatation and dissection. The commonly held notion attributes this risk to aortopathy in BAV. This study seems to debunk that idea, showing that aortic elasticity and distensibility did not differ between BAV and tri-leaflet aortic valve patients with a history of CoA. The study suggests the valve phenotype in CoA patients does not have an impact on LV and aortic function, a position that is at variance with current cardiology guidelines. The risk of aortic dissection increases with increasing aortic diameter, but dissection also occurs in non-dilated aortas. In patients with Marfan syndrome, both aortic diameter and aortic distensibility are independent predictors of progressive aortic dilatation. As such both aortic stiffness and diameter are recommended to be assessed at least annually. The finding in the current study that aortic distensibility in CoA is similar between BAVs and trileaflet aortic valves therefore has an important bearing on risk profiling. It must be noted though that data regarding perioperative characteristics (severity of CoA prior to repair, age at repair, type of repair, etc) is missing from this study.

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