Rest and exercise right and left ventricular function late after the mustard operation: Assessment by radionuclide ventriculography

International Society for Heart and Lung Transplantation: Practice guidelines for management of heart failure in children

“…Although RV performance at rest is often normal, 84 exercise ability is often limited in patients who have undergone atrial baffle surgery because of both chronotropic incompetence and limited stroke volume reserve. [85][86][87][88][89][90][91][92] The mechanism of systemic right ventricular dysfunction is poorly understood. Various theories include: (a) a sub-optimal myocardial fiber arrangement and mechanics in the right ventricle, (b) adverse pattern and reduced heterogeneity of ventricular strain, (c) tricuspid insufficiency and, (d) myocardial fibrosis secondary to prolonged hypoxemia during infancy while awaiting atrial baffle surgery.…”

Exercise Studies in Patients with Transposition of the Great Arteries After Atrial Repair Operations (Mustard/Senning): A Review

“…The initial speculative concern about the long-term functional performance of the atrial repair TGA heart was reinforced by an early series (1982)(1983)(1984) of radionuclide determinations of right ventricular ejection fraction (RVEF) [2,28,34,40]. These four studies (n ‫ס‬ 78 subjects) demonstrated that after the Mustard or Senning repair an average of 61% (55-71%) of these predominantly asymptomatic children had abnormal right ventricular function when challenged with exercise (cycle ergometer) stress.…”

“…LVEF response to exercise was also noted to be abnormal (i.e., not increasing >5% with exercise) in six reports with wide variation: abnormal responses were noted in a mean of 47% of the patients (median 38%, range 11-91%) [20,28,34,39,40,50]. It has been suggested that the prevalence of coexisting left (pulmonary) ventricular dysfunction in the atrial-repaired TGA population may be evidence for global and multiple factors that implicate damaging episodes of myocardial hypoxia (prerepair), intraoperative myocardial perservation deficiencies, or geometric suboptimal ventricular configurations [34].…”

“…Clearly the single, almost universal observation was the noncorrelation of clinical status and symptoms with exercise capacity or ventricular function since almost all patients were declared asymptomatic. The most frequent correlations presented concerned RVEF (exercise stressed) and (1) age at atrial repair surgery/no correlation [2,9,28,34,40]; (2) age at exercise study/no correlation [2,28,34,40] and correlation [9]; and (3) residual hemodynamic sequelae/no correlation [2,28,34,40] and correlation [4]. Residual hemodynamic sequelae were further correlated with VO 2max , heart rate, and exercise capacity [13] and pulmonary function [15].…”

Disparity Between Dobutamine Stress and Physical Exercise Magnetic Resonance Imaging in Patients with an Intra-atrial Correction for Transposition of the Great Arteries

“…Stress testing allows us to detect ventricular dysfunction (2)(3)(4)(5), which may not be present at rest (6). Patients with an intra-atrial correction of TGA generally show an abnormal response to stress (7,8). Possible causes for this abnormal response to stress include systolic dysfunction of the right ventricle (RV) under systemic pressure (9), filling abnormalities of the RV through rigid atrial baffles (10), and an abnormally low heart rate response (11).…”