skip to main content

Anesthesia in A Patient with Pulmonary Atresia with Intact Ventricular Septum (PA IVS) Underwent Bidirectional Cavo-Pulmonary Shunt (BCPS)

1Department of Anesthesiology and Intensive Care Unit, dr. H. Jusuf SK Hospital, Tarakan, Indonesia

2Department of Anesthesiology and Post Operative Intensive Care Unit, Heart and Vascular Harapan Kita Hospital, Jakarta, Indonesia

Received: 28 Apr 2023; Revised: 16 Sep 2023; Accepted: 10 Jan 2024; Published: 31 Mar 2024.

Citation Format:
Cover Image
Abstract

Background: Pulmonary atresia with intact ventricular septum (PA-IVS) is a rare condition, encompassing approximately 1%–3% of congenital heart diseases. Patients with PA-IVS have functional single-ventricle physiology. The ultimate possible outcomes for patients with PA-IVS are biventricular circulation, 1.5-ventricle or single-ventricle palliation, or cardiac transplantation. The bidirectional cavopulmonary shunt (BCPS) procedure directs flow from the superior vena cava into both the right and left pulmonary arteries, permitting flow to both lungs. The shunt is considered to be the second stage of palliation and is generally preparative for the third-stage Fontan procedure. The BCPS improves systemic arterial oxygen saturation without increasing ventricular work or pulmonary vascular resistance.

Case: A 10-month-old, 25-day-old boy, weight 9.93 kg, body length 72 cm, diagnosed with PA-IVS, restricted persistent foramen ovale (PFO), right ventricle hypoplastic, and turtuous patent ductus arteriosus (PDA), underwent BCPS, atrial septectomy, and PDA stent evacuation surgery. The patient underwent a PDA stenting and ballooning atrial septectomy (BAS) at 17 days of age at the cathlab. The patient was cyanotic with stable hemodynamics and a saturation of 72% preoperatively.

Discussion: Preoperative fasting must be observed to maintain the patient's hydration state. Pulmonary blood flow and systemic blood flow must be balanced. An adequate analgetic can prevent pain stimuli that increase pulmonary vascular resistance. Drugs to reduce the afterload, such as milrinone, are needed. Mechanical ventilation was set to get PaCO2 between 40 and 45 mmHg. Maintain the normal heart beat, preload, and contractility to maintain cardiac output (CO) with saturation 80–85%. Wean from mechanical ventilation as soon as possible.

Conclusion: Anesthetic management for BCPS in patients with single ventricles from the preoperative period, intraoperative period, and postoperative period. Understanding single ventricle phsiology is important in order to treat the patient.

Note: This article has supplementary file(s).

Fulltext View|Download |  common.other
CTA
Subject
Type Other
  Download (92KB)    Indexing metadata
 common.other
Graphical Abstract
Subject
Type Other
  View (364KB)    Indexing metadata
Keywords: anesthetic management; bidirectional cavopulmonary shunt (BCPS); intact ventricular septum; pulmonary atresia; single ventricle

Article Metrics:

  1. Viviane G. Nasr, James A. DiNardo, The Pediatric Cardiac Anesthesia Handbook, 2017
  2. Scott G. Walker and Eckehard A. Stuth Indianapolis, Indiana and Milwaukee, Wisconsin, Single-Ventricle Physiology: Perioperative Implications, 2004
  3. Daubeney PE, Delany DJ, Anderson RH, et al. Pulmonary atresia with intact ventricular septum: range of morphology in a population-based study. J Am Coll Cardiol. 2002;39: 1670-1679
  4. Hoffman JI, Kaplan S. The incidence of congenital heart disease. J Am Coll Cardiol. 2002;39:1890-1900
  5. Stephen Gleich, MD1 , Gregory J. Latham, MD1 , Denise Joffe, MD1 , and Faith J. Ross, MD, Perioperative and Anesthetic Considerations in Pulmonary Atresia With Intact Ventricular Septum, 2017
  6. Constantine Mavroudis MD, Pediatric Cardiac Surgery 4th ed, 2013
  7. Saltik L, Bayrak F, Guneysu T, Sevinc D, Oztunc F, Degertekin M. Right ventricle-dependent coronary circulation demonstrated with 64-slice computed tomography. Eur Heart J. 2008;29:1018
  8. Daubeney PE, Delany DJ, Anderson RH, et al. Pulmonary atresia with intact ventricular septum: range of morphology in a population-based study. J Am Coll Cardiol. 2002;39: 1670-1679
  9. Giglia TM, Mandell VS, Connor AR, Mayer JE Jr, Lock JE. Diagnosis and management of right ventricle-dependent coronary circulation in pulmonary atresia with intact ventricular septum. Circulation. 1992;86:1516-1528
  10. Pulmonary Atresia/Intact Ventricular Septum: Influence of Coronary Anatomy on Single-Ventricle Outcome
  11. Nichols DG, Ungerleider RM, Spevak PJ, et al, Pulmonary atresia with intact ventricular septum. Critical Heart Disease in Infants and Children. 2nd ed. Philadelphia, PA: Mosby Elsevier; 2006:767-776
  12. Saltik L, Bayrak F, Guneysu T, Sevinc D, Oztunc F, Degertekin M. Right ventricle-dependent coronary circulation demonstrated with 64-slice computed tomography. Eur Heart J. 2008;29:1018
  13. Stephan Stayer, MD, Emad B,MD, Anesthesia for congenital heart diseases, 3rd ed.,2015
  14. Coats L, O’Connor S, Wren C, O’Sullivan J. The single ventricle patient population: a current and future concern a population-based study in the North of England. Heart 2014; 100: 1348e)
  15. Riza cintyandy, Anestesi Jantung Congenital
  16. Susan C. Nicolson1, James M. Steven1, Laura K. Diaz and Dean B, Anesthesia for the Patient with a Single Ventricle, chapter 25, 3rd ed., 2015
  17. D. Greaney1 O. Honjo1,2 and J.D. O’Leary, The single ventricle pathway, 2019
  18. Mackay, Jonathan H. II. Arrowsmith, Joseph E, Core Topics in Cardiac Anesthesia, Second Edition, 2012
  19. Hill GD, Rudd NA, Ghanayem NS, Hehir DA, Bartz PJ. Center variability in timing of stage 2 palliation and association with interstage mortality: a report from the National Pediatric Cardiology Quality Improvement Collaborative. Pediatr Cardiol. 2015;37:1516-1524
  20. Meza JM, Hickey EJ, Blackstone EH, et al. The optimal timing of stage 2 palliation for hypoplastic left heart syndrome: an analysis of the Pediatric Heart Network Single Ventricle Reconstruction Trial Public Data Set. Circulation. 2017;136:1737-1748
  21. Meza JM, Hickey E, McCrindle B, et al. The optimal timing of stage-2-palliation after the Norwood operation. Ann Thorac Surg. 2018;105:193-199
  22. Herrmann JL, Brown JW. The superior cavopulmonary connection: history and current perspectives. World J Pediatr Congenit Heart Surg. 2019;10:216-222
  23. Ray S. Choi, MD1,2, James A. DiNardo, MD2, and Morgan L. Brown, MD, PhD2, Superior Cavopulmonary Connection: Its Physiology, Limitations, and Anesthetic Implications, 2020
  24. Vettukattil JJ: Pathogenesis of pulmonary arteriovenous malformations: Role of hepatopulmonary interactions. Heart 88:561-563, 2002
  25. Marino BS: Outcomes after the Fontan procedure. Curr Opin Pediatr 14:620-626, 2002

Last update:

No citation recorded.

Last update: 2024-05-23 03:48:24

No citation recorded.