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5 results

  • Chapter 25 - Truncus Arteriosus

  • from Section 4 - Complex Mixing Lesions
  • Edited by Laura K. Berenstain, James P. Spaeth
  • Book:
    Congenital Cardiac Anesthesia
    Published online:
    09 September 2021
    Print publication:
    16 September 2021, pp 178-186

  • Summary

    Truncus arteriosus is a rare form of cyanotic congenital heart disease in which the embryologic separation of the arterial trunk remains incomplete. The primary anatomic lesion is a common arterial trunk that supplies the pulmonary, coronary, and systemic circulations. Patients become symptomatic early in infancy because pulmonary vascular resistance falls over the first days to weeks of life, resulting in pulmonary overcirculation. Ideally, truncus arteriosus repair occurs within the first weeks of life. The most common extracardiac congenital malformations associated with truncus arteriosus are those within the heterogeneous spectrum of 22q11 deletion syndrome. Features of 22q11DS can pose multiple challenges to anesthesiologists. Dysmorphic facies, including micrognathia, retrognathia, palatal abnormalities, and laryngotracheomalacia, can impede airway management. This chapter describes the care of a child with repaired truncus arteriosus and 22q11 deletion syndrome for palatal surgery and the perioperative management considerations.

  • The systemic right ventricle in congenitally corrected transposition of the great arteries is different from the right ventricle in dextro-transposition after atrial switch: a cardiac magnetic resonance study

  • Matthias Grothoff, Antje Fleischer, Hashim Abdul-Khaliq, Janine Hoffmann, Lukas Lehmkuhl, Christian Luecke, Matthias Gutberlet
  • Journal:
    Cardiology in the Young / Volume 23 / Issue 2 / April 2013
    Published online by Cambridge University Press:
    14 June 2012, pp. 239-247
  • Background

    Patients with a congenitally corrected transposition of the great arteries show an increasing incidence of cardiac failure with age. In other systemic right ventricles, such as in dextro-transposition after atrial switch, excessive hypertrophy is a potential risk factor for impaired systolic function. In this trial, we sought to compare systemic function and volumes between patients with congenitally corrected transposition and those with dextro-transposition after atrial switch by using cardiac magnetic resonance imaging.

    Methods and Results

    A total of 19 patients (nine male) with congenitally corrected transposition and 31 patients (21 male) with dextro-transposition after atrial switch were studied using a 1.5-Tesla scanner. Cine steady-state free-precession sequences in standard orientations were acquired for volumetric and functional imaging. Patient parameters were compared with those of a group of 25 healthy volunteers. Although patients with congenitally corrected transposition were older, they presented with higher right ventricular ejection fractions (p = 0.04) compared with patients with dextro-transposition. Patients with congenitally corrected transposition showed a weak negative correlation between age at examination and systemic ejection fraction (r = −0.18, p = 0.04) but no correlation between right ventricular myocardial mass index and ejection fraction. There was no significant difference in the right ventricular end-diastolic volumes between both patient groups.

    Conclusion

    Although patients with congenitally corrected transposition had a longer pressure load of the systemic right ventricle, ventricular function was better compared with that in patients with dextro-transposition after atrial switch. The results suggest that the systemic ventricles might have partly different physiologies. One difference could be the post-operative situation after atrial switch, which results in impaired atrial contribution to ventricular filling.

  • Chapter 20 - Pediatric lung transplantation

  • from Section 3 - Lung
  • Edited by Andrew A. Klein, Clive J. Lewis, Joren C. Madsen
  • Book:
    Organ Transplantation
    Published online:
    07 September 2011
    Print publication:
    11 August 2011, pp 164-172

  • Summary

    In the immediate postoperative period, close attention must be paid to hemodynamic stability by focusing on preventing right ventricular failure and maintaining chronotropic competence. Preoperative support of the recipient circulation by mechanical assist devices appears to significantly increase the risk of post-transplantation primary graft failure. Primary cardiac allograft failure accounts for 40 percentage of mortality within 30 days of heart transplantation (HT). Following HT, the use of intraoperative and peri-operative corticosteroids remains the mainstay of early therapy. Monitoring of therapeutic drug levels is important but there is some controversy in how best to monitor the target levels of calcineurin inhibitors (CNIs). Early after transplantation, particularly in the first 3 months when the risk of rejection is highest, invasive biopsies are recommended at decreasing intervals. Close vigilance for re-emergence of circulating antibodies is needed, and newer approaches using complement inhibitors or intensive B-cell modulating drugs such as bortezomib are being studied.

  • Perfusion defects in patients with complete transposition after the Mustard or Senning operation

  • Barbara Lubiszewska, Elzbieta Gosiewska-Marcinkowska, Anna Teresinska, Jacek Rozanski, Wanda Rydlewska-Sadowska, Witold Ruzyllo
  • Journal:
    Cardiology in the Young / Volume 6 / Issue 1 / January 1996
    Published online by Cambridge University Press:
    19 August 2008, pp. 69-75

  • Atrial correction for complete transposition involves a potential risk of later development of right ventricular dysfunction. To determine the reasons contributing to such systemic ventricular failure, radioisotope first-pass and SPECT perfusion studies at rest after one dose of Technetium-99m MIBI were performed in 26 asymptomatic patients, 5.9±2.7 years after a Mustard or Senning repair. Mean age at operation was 4.3±4.3 years. All patients were followed with 24-hour Holter monitoring and echocardiography. Normal perfusion was observed in 11 patients with a mean age at operation of 2.25±1.8 years and mean right ventricular ejection fraction of 49.0±8.7%. Perfusion defects were found in 15 patients with a mean age at operation 5.8±4.8 years (p<0.05) and mean right ventricular ejection fraction of 43.2±9.3% (NS). In the group of older patients, 10 had extensive perfusion defects with significantly lower right ventricular ejection fraction (39.7±9.3%, p<0.05) and even older mean age at the surgery (7.1±5.6 years p<0.02). Mild or moderate tricuspid regurgitation was found significantly more frequently in those patients with perfusion defects (73%) than in the group of younger patients (36.3%), p<0.05. There was no difference in the frequency and type of arrhythmias, nor in the extent of follow-up, between the two groups. We conclude that, in patients undergoing surgery at an older age, it is more common to find perfusion defects and, often, lower right ventricular ejection fraction. Perfusion abnormalities found in such asymptomatic patients may be a result of several factors: impaired right ventricular function as a consequence of pressure and volume overload, enhanced myocardial fibrosis, or long-lasting preoperative hypoxemia and tricuspid valvar dysfunction secondary to ventricular dilation. We suggest that the perfusion defects observed in this study may be a better indicator of systemic ventricular impairment.