Clinical Research
Adult Congenital Heart Disease
Right Ventricular End-Diastolic Volume Combined With Peak Systolic Blood Pressure During Exercise Identifies Patients at Risk for Complications in Adults With a Systemic Right Ventricle

https://doi.org/10.1016/j.jacc.2013.06.026Get rights and content
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Objectives

The aim of this study was to identify which patients with a systemic right ventricle are at risk for clinical events.

Background

In patients with congenitally or atrially corrected transposition of the great arteries, worsening of the systemic right ventricle is accompanied by clinical events such as clinical heart failure or the occurrence of arrhythmia.

Methods

At baseline, all subjects underwent electrocardiography, echocardiography, cardiopulmonary exercise testing, and cardiovascular magnetic resonance imaging. Clinical events comprised death, vascular events, tricuspid regurgitation requiring surgery, worsening heart failure, and (supra)ventricular arrhythmia. A Cox proportional hazards analysis was used to assess the most valuable determinants of clinical events.

Results

A total of 88 patients with a mean age of 33 years were included in the study. Sixty-five percent were men, and 28% had congenitally corrected transposition of the great arteries. During a follow-up period of 4.3 years, 31 patients (35%) experienced 46 clinical events for an annual risk of 12%. Right ventricular end-diastolic volume index measured by means of cardiovascular magnetic resonance imaging or multirow detector computed tomography (hazard ratio: 1.20; p < 0.01) and peak exercise systolic blood pressure (hazard ratio: 0.86; p = 0.02) were the strongest determinants of clinical events. Patients with a right ventricular end-diastolic volume index above 150 ml/m2 and peak exercise systolic blood pressure below 180 mm Hg were most likely to experience clinical events with an annual event rate of 19% versus 0.9% in patients without these risk factors.

Conclusions

Patients with a right ventricular end-diastolic volume index above 150 ml/m2 and peak exercise systolic blood pressure below 180 mm Hg had a 20-fold higher annual event rate than patients without these risk factors. Regular cardiovascular magnetic resonance imaging and exercise testing are important in the risk assessment of these patients.

Key Words

clinical events
prognosis
systemic right ventricle

Abbreviations and Acronyms

ccTGA
congenitally corrected transposition of the great arteries
CMR
cardiovascular magnetic resonance imaging
CPET
cardiopulmonary exercise testing
ECG
electrocardiography
HR
hazard ratio
MDCT
multidetector-row computed tomography
NYHA
New York Heart Association
RVEDVi
right ventricular end-diastolic volume index
SBP
systolic blood pressure
TGA
transposition of the great arteries

Cited by (0)

This work was supported by an unrestricted educational grant from Novartis. The authors have reported that they have no relationships relevant to the content of this paper to disclose.