Cardiorespiratory Exercise Tolerance in Asymptomatic Children with Ebstein's Anomaly

Abstract

The aim of the study was to evaluate cardiorespiratory exercise tolerance in asymptomatic children with Ebstein's anomaly. Eleven children with a mean age of 9.6 years were prospectively studied by spirometry, cardiopulmonary exercise testing (bicycle ergometer n = 8, treadmill test n = 3), and contrast echocardiography. A right-to-left atrial shunt was detected by contrast echocardiography in 7 children (group 1), whereas no shunt was found in 4 (group 2). VO2 max was decreased [84.5 (SD = 16.8)] and was strongly correlated to oxygen saturation in group 1 (p< 0.0001). Oxygen saturation at peak uptake was significantly decreased compared to baseline [97.4 (SD = 2.0) vs 90% (SD = 9.5%), p = 0.02] and was significantly lower in group 1 than in group 2 [85.7 (2.2) vs 98.2% (SD = 1.2%), p = 0. 03]. Oxygen desaturation was related to a right-to-left atrial shunt (p = 0.01). Decreased VO2 max was also correlated to the small size of the left ventricle (p = 0.05). We concluded that decreased exercise tolerance in children with asymptomatic Ebstein's anomaly is related to a right-to-left atrial shunt and to a small left ventricle. In case of poor exercise tolerance, a contrast echocardiography should be performed to detect an atrial septal defect.

Clinical spectrum and long-term outcome of Ebstein’s anomaly based on a 26-year experience in an Asian cohort

“…An atrialized ventricle, downward displaced tricuspid valve, and tricuspid regurgitation result in a particular form of right ventricular cardiomyopathy [15]. The so-called asymptomatic patients of Ebstein's anomaly may reveal hemodynamic changes during exercise, which is suggested to be due to tricuspid stenosis [14,24], and the timing of surgery in such patients remains controversial. In the symptomatic patients, tricuspid valvuloplasty or valve replacement provide satisfactory hemodynamic improvement.…”

Cardiovascular Endurance and Heart Rate Variability in Adolescents With Type 1 or Type 2 Diabetes

“…Exercise testing using cycle ergometer or treadmill procedures for the measurement of V0 2peak have been used successfully in healthy children and adolescents and with diverse groups, including those with asymptomatic Ebstein's anomaly and those with mild to moderate mental retardation and following renal transplantation (Bishop, Jenkins, & Mackinnon, 1998;Fernhall et al, 1998;Giordano et al, 1998;Lupoglazoff et al, 1999;McMurray, Guion, Ainsworth, & Harrell, 1998). Reliability of exercise testing using the cycle ergometer has been established with 8 females (mean age 19.6 ± 2.5 years) by repeating the testing three times over a period of 2 weeks with all tests separated by at least 48 hrs (Bishop et al, 1998).…”

JCS 2022 Guideline on Management and Re-Interventional Therapy in Patients With Congenital Heart Disease Long-Term After Initial Repair

“…484 In Ebstein malformation, not a few reports describe that the peak V ˙O2 value is 50-60% of normal when surgical intervention is decided. [485][486][487] Functional class improves after surgery, whereas peak V ˙O2 does not improve drastically. In most patients, the value remains within a subnormal range.…”

“…Lower oxygen saturation and reduced left ventricular volume correlate with exercise intolerance. 487 The Fontan circulation has factors intricately associated with declining exercise tolerance. 206, 488 Its first line is low cardiac output with heart rate ineffectively responding to physical exercise.…”