Systemic Right Ventricle, Heart Failure Hospitalization, and Early Death

Early mortality and heart failure (HF) hospitalization are increased in patients with transposition of the great arteries (TGA) and a systemic right ventricle, according to findings published in the International Journal of Cardiology.

Investigators sought to assess the yearly rate of mortality and related factors in patients with TGA and a systemic right ventricle. Secondary outcomes included incidence of HF hospitalization and arrhythmia.

They conducted a systematic review and meta-analysis using the Web of Science, Scopus, EMBASE, and PubMed databases without language restriction from inception to June 2022 to search for studies reporting the association of systemic right ventricle with mortality and a minimum of 2 years of follow-up during adulthood.

The investigators included 56 studies with, overall, 5358 patients at least 16 years of age with a systemic right ventricle (Dextro-TGA [D-TGA]) who have undergone either the Mustard or Senning atrial switch procedure, or congenitally corrected (cc) TGA with average follow-up of 7.27 years (at least 2 years follow-up during adulthood). Patients who had received an atrial switch operation resulting in the left ventricle at the systemic position were excluded.

Per 100 patients per year, incidence of mortality was 1.3 (95% CI, 1.0-1.7) and this incidence was not associated with mean age at baseline (odds ratio [OR], 1.03; 95% CI, 0.99-1.07). After adjusting for age, no difference was found between incidence of mortality in patients with TGA after atrial switch per 100 patients per year (OR, 1.1; 95% CI, 0.7-1.7) and patients with ccTGA per 100 patients per year (OR, 1.9; 95% CI, 1.3-2.9).

The incidence of HF hospitalization was 2.6 per 100 patients per year (95% CI, 1.9-3.7) and this incidence was not associated with mean age at baseline (OR, 1.06; 95% CI, 0.98-1.14). After adjusting for age, patients with ccTGA had higher incidence of HF hospitalization (OR, 4.3; 95% CI, 2.7-6.6) vs patients with TGA and atrial switch (OR, 3.2; 95% CI, 2.3-4.4).

Among patients with TGA and atrial switch and patients with ccTGA, no difference was found in incidence of ventricular arrhythmia per 100 patients per year.

Investigators noted predictors of poor outcome included higher plasma concentrations of N-terminal pro-brain natriuretic peptide (standardized mean difference [SMD], 1.24; 95% CI, 0.49-1.99), New York Heart Association functional class of at least 2 (risk ratio, 2.31; 95% CI, 1.57-3.39), as well as lower left ventricular ejection fraction (LVEF; SMD, -0.43; 95% CI, -0.77 to -0.09) and right ventricular ejection fraction (RVEF; SMD, -0.85; 95% CI, -1.35 to -0.35).

Limitations of the study include the heterogeneity of reported variables.

“TGA patients with a systemic right ventricle have increased incidence of mortality and HF hospitalization,” the investigators wrote.