Late outcome, therapy and systemic ventricular function in patients with a systemic right ventricle: data of the German National Register for Congenital Heart Defects

Abstract

Background: Adults with systemic right ventricle have a significant risk for long-term complications such as arrhythmias or heart failure. Methods: A nationwide retrospective study based on the German National Register for Congenital Heart Disease was performed. Patients with transposition of the great arteries after atrial switch operation or congenitally corrected TGA were included. Results: Two hundred and eight-five patients with transposition of the great arteries after atrial switch operation and 95 patients with congenitally corrected transposition of the great arteries were included (mean age 33 years). Systolic function of the systemic ventricle was moderately or severely reduced in 25.5 % after atrial switch operation and in 35.1% in patients with congenitally corrected transposition. Regurgitation of the systemic atrioventricular valve was present in 39.5% and 43.2% of the cases, respectively. A significant percentage of patients also had a history for supraventricular or ventricular arrhythmias. However, polypharmacy of cardiovascular drugs was rare (4.5%) and 38.5 % of the patients did not take any cardiovascular medication. The amount of cardiovascular drugs taken was associated with NYHA class as well as systemic right ventricular dysfunction. Patients with congenitally corrected transposition were more likely to receive pharmacological treatment than patients after atrial switch operation. Conclusion: A significant portion of patients with systemic right ventricle suffer from a relevant systemic ventricular dysfunction, systemic atrioventricular valve regurgitation, and arrhythmias. Despite this, medication for heart failure treatment is not universally used in this cohort. This emphasises the need for randomised trials in patient with systemic right ventricle.

Overview of Long-Term Outcome in Adults with Systemic Right Ventricle and Transposition of the Great Arteries: A Review

“…Moreover, recent studies revealed a correlation of left ventricular outflow tract obstruction (LVOTO) with sRV function, suggesting a positive effect of the LVOTO on hemodynamic status and thus a potential protective effect on sRV systolic in the long-term [ 52 , 53 ].…”

“…Primary ventricular tachycardia occurs in about 20% of the patients with ccTGA, especially in those with sRVd [ 53 , 126 ]. This prevalence, which is higher compared to patients after AtSO, may be explained by multiple substrates for dysrhythmia ventricular scars in patients undergoing a physiologic repair strategy (such as the presence of or a possible association with accessory pathways in patients with Ebstein-like valve).…”

“…The six variables included in this model are age at baseline, history of HF, syncope, QRS duration, severe sRVd, and at least moderate left ventricular outflow tract (LVOT) obstruction > 36 mmHg [ 119 ]. Although LVOT obstruction was found to be associated with a lower risk of developing HF or an additional protective factor [ 53 , 132 ], as mentioned above, the resulting LV hypertrophy may contribute to the ventricular arrhythmia substrate.…”

Expression profiling analysis reveals key microRNA–mRNA interactions in patients with transposition of the great arteries and systemic left and right ventricles

“…These surgical operations underwent several modifications in the last six decades. Patients with TGA, who still have RV in a systemic position are known to be at risk for heart failure (HF), atrial and ventricular arrhythmia, and sudden death ( 3 , 4 ). The occurrence of overt HF has negative implications on the prognosis of CHD patients ( 5 ).…”

The effect of comprehensive management of heart failure in adult with systemic right ventricle

“…Patients with a systemic right ventricle (SRV) represent a significant proportion of adults with congenital heart defects (CHD) [1]. Over time, most of them show various degrees of heart failure (HF) symptoms [1][2][3][4][5]. The lack of clear guidelines based on hard evidence and the increasing number of adult CHD patients make treatment of SRV failure one of today's greatest challenges.…”