Restrictive Right Ventricular Physiology and Right Ventricular Fibrosis as Assessed by Cardiac Magnetic Resonance and Exercise Capacity After Biventricular Repair of Pulmonary Atresia and Intact Ventricular Septum

Abstract

Background: The hypertrophic myocardium, myocardial fiber disarray, and endocardial fibroelastosis in pulmonary atresia and intact ventricular septum (PAIVS) may provide anatomic substrates for restrictive filling of the right ventricle. Hypothesis: Restrictive right ventricle (RV) physiology is related to RV fibrosis and exercise capacity in patients after biventricular repair of PAIVS. Methods: A total of 27 patients, age 16.5 ± 5.6 years, were recruited after biventricular repair of PAIVS. Restrictive RV physiology was defined by the presence of antegrade diastolic pulmonary flow and RV fibrosis assessedby late gadolinium enhancement (LGE) cardiac magnetic resonance.Their RV function was compared with that of 27 healthy controls and related to RV LGE score and exercise capacity. Results: Compared with controls, PAIVS patients had lower tricuspid annular systolic and early diastolic velocities, RV global longitudinal systolic strain, systolic strain rate, and early and late diastolic strain rates (all P < 0.05). A total of 22 (81%, 95% confidence interval: 62%-94%) PAIVS patients demonstrated restrictive RV physiology. Compared to those without restrictive RV physiology (n=5), these 22 patients had lower RV global systolic strain, lower RV systolic and early diastolic strain rates, higher RV LGE score, and a greater percent of predicted maximum oxygen consumption (all P < 0.05). Conclusion: Restrictive RV physiology reflects RV diastolic dysfunction and is associated with more severe RV fibrosis but better exercise capacity in patients after biventricular repair of PAIVS. IntroductionThe evolution in management strategy has significantly improved the long-term outcomes of patients with pulmonary atresia and intact ventricular septum (PAIVS). 1 -3 In the presence of a reasonably good-sized right ventricle and the absence of a right ventricular (RV)-dependent coronary circulation, RV decompression could be achieved by outflow reconstruction, 2 -5 surgical pulmonary valvotomy,

Myocardial iron load and fibrosis in long term survivors of childhood leukemia

“…Each of the left ventricular (LV) segments will be scored on a 5‐point scale (0–4) , and the maximum LV LGE score was 68. A high concordance in scoring LGE has previously been reported by our group .…”

Guidelines and protocols for cardiovascular magnetic resonance in children and adults with congenital heart disease: SCMR expert consensus group on congenital heart disease

“…Reports in adults have demonstrated its clinical utility in acute and chronic ischemic heart disease, cardiomyopathies, myocarditis, and ventricular thrombus detection. In CHD, LGE has been described in patients with repaired tetralogy of Fallot [153-155], atrial switch operations for transposition of the great arteries [156], Fontan operations for functionally single ventricles (Figure 14) [157], congenitally corrected transposition of the great arteries [158], aortic valve stenosis [159,160], pulmonary atresia with intact ventricular septum [161], anomalous left coronary artery from the pulmonary artery after repair [162], endocardial fibroelastosis (Figure 15) [163,164], and fibrous tissue along regions of reconstruction in patients who have had surgery for CHD [165]. In the tetralogy of Fallot, atrial switch, and Fontan operation cohorts, the presence of LGE has been associated with adverse ventricular mechanics, exercise intolerance, and ventricular arrhythmias [153,155-158].…”

Functional Assessment for Congenital Heart Disease

“…A tissue Doppler echocardiographic study has shown regional systolic and diastolic longitudinal myocardial dysfunction involving both the right and left ventricles in adolescent patients after biventricular repair in infancy 94). A subsequent study using 2D speckle tracking imaging confirmed the impairment of RV systolic and diastolic deformation 15). LGE CMR showed evidence of RV myocardial fibrosis.…”

“…On the other hand, persistence of restrictive RV physiology in the long-term has been associated with less progressive dilation of the right ventricle and better exercise capacity 14). Restrictive RV physiology has also been described after biventricular repair of pulmonary atresia with intact ventricular septum (PAIVS) due probably to the relatively small RV cavity and intrinsic myocardial and endocardial abnormalities that reduce RV compliance 15). Limited data also suggest that restrictive RV physiology also predicts better exercise capacity in these patients 15)…”