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6 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.

  • High incidence of ductal closure or narrowing at birth in patients with right ventricular outflow tract obstruction with normal orientation of the ductus arteriosus

  • Hazumu Nagata, Eiko Terashi, Mamoru Muraoka, Kiyoshi Uike, Yuichiro Hirata, Hideki Tatewaki, Yasuyuki Fujita, Kenichiro Yamamura, Shouichi Ohga
  • Journal:
    Cardiology in the Young / Volume 29 / Issue 1 / January 2019
    Published online by Cambridge University Press:
    24 October 2018, pp. 54-58
  • Background

    Ductal patency is mandatory to manage patients with ductal-dependent pulmonary circulation. The aim of this study is to elucidate the morphological and haemodynamic features of ductus arteriosus with right ventricular outflow tract obstruction, and investigate the appropriate perinatal management.

    Patients and methods

    Patients with prenatal diagnosis of right ventricular outflow tract obstruction at our institution between 2010 and 2015 were included in the study. Reverse orientation of the ductus arteriosus is defined as an inferior angle of <90° at the aortic junction, and normal orientation of the ductus arteriosus as an angle of >90°. We retrospectively reviewed the shape and flow pattern of ductus arteriosus and the clinical characteristics of the cases.

    Results

    A total of 39 patients were enrolled. The shape was divided into normal orientation (n=15) and reverse orientation (n=24) of the ductus arteriosus. There was no significant difference in the type of oxygen saturation at birth and age at shunt operation between both the groups. However, the median narrowest diameter of ductus arteriosus in the normal orientation group was significantly smaller than that in the reverse orientation group (2.0 [1.0–5.4] versus 3.0 [1.3–4.4] mm, p<0.05). In two patients of the normal orientation group, ductus arteriosus had closed at birth, and one of whom died because of severe cyanosis.

    Conclusions

    Normal orientation pattern might have high incidence of an early narrowing or closure of ductus arteriosus at birth. The critical patients need careful evaluation by repeated foetal echocardiography and further maternal interventions.

  • Ebstein’s malformation presenting with tricuspid stenosis: 1-year follow-up after surgical repair

  • Rami N. Khouzam, Joseph A. Dearani, Paul R. Julsrud
  • Journal:
    Cardiology in the Young / Volume 20 / Issue 1 / February 2010
    Published online by Cambridge University Press:
    24 February 2010, pp. 80-85

  • This is a case of a young woman previously completely healthy, with two uneventful pregnancies and deliveries, who presented with bloating, shortness of breath, and signs of right heart failure. A thorough clinical evaluation, along with a work-up including an echocardiogram and a magnetic resonance imaging revealed the diagnosis of Ebstein’s anomaly malformation with tricuspid stenosis. A right ventricular outflow tract obstruction due to thrombus formation was thought to exacerbate her symptoms and lead to the diagnosis. Surgery in the form of right ventricular thrombectomy, right atrial reduction, porcine tricuspid valve replacement and bidirectional cavopulmonary anastomosis “bidirectional Glenn”, was successfully performed. The patient has been stable clinically more than one year after surgery. Discussion about this rare condition and operative details are provided.