Congenital Cardiac Anesthesia Society
A Section of the the Society for Pediatric Anesthesia

Congenital Cardiac Anesthesia Society

  • Member Login
  • Forgot Password?
  • Join
  • Home
  • About
    • Mission Statement
    • Bylaws
    • Board of Directors
    • History
    • Past Presidents
    • Charter Members
    • CCAS Lifetime Achievement Award
    • Mailing List Rental
    • Contact Us
  • Partners
    • SPA
    • SPPM
    • PALC
    • PCICS
    • AmSECT
    • SMACT
    • CHA
    • CCAN
  • Committees
    • CCAS Committees
      • Pediatric Cardiac Anesthesiology Program Director’s Group (PCAPD) Committee
      • Communications Committee
      • CCAS-STS Database Committee
      • Education Committee
      • Global Health Committee
      • Membership Committee
      • Quality and Safety Committee
      • Research Committee
    • CCAS Special Interest Groups
      • Adult Congenital Heart Disease Special Interest Group (SIG)
      • ERAS Special Interest Group (SIG)
      • Hemostasis Special Interest Group (SIG)
      • Latin America Special Interest Group (SIG)
      • Pulmonary Hypertension Special Interest Group (SIG)
      • Trainee Special Interest Group (SIG)
  • Membership
    • Benefits of Membership
    • CCAS Member App
    • Membership Categories
    • Join CCAS
      • Online Application
      • Printable Application
    • Renew Your Dues
    • Sponsor a Member
    • Get Involved
  • Meetings
    • Upcoming Meetings
    • Past Meetings
    • Other Meetings
    • Exhibit Information
  • Education
    • CCAS Virtual Visiting Professor Program
    • CCAS Webinar Series
      • CCAS Webinar Series – Recordings
    • CCAS COVID 19 Webinar
    • ACHD-SIG Anesthesia Rounds
    • Lecture Series
    • Echo Tutorial
    • Archived Questions
    • Poll of the Month Archives
    • Review Articles
      • CCAS-CHiP Network Journal Watch Collaboration
      • SCVA Articles
    • Journals of Interest
    • Books of Interest
    • Educational Links
  • Resources
    • CCAS Position Statements
    • CCAS Committee Resources
    • CCAS Special Interest Group (SIG) Resources
    • CCAS Cognitive Aids
    • Newsletters
    • Research Resources
    • Mission Trips
    • Societies
    • Job Opportunities
  • Research
    • Research Committee
    • CCAS 2025 Meeting Scholarship for Students and Residents
    • Research Network & Collaborative Opportunities
    • Research Resources
    • Dolly D. Hansen, MD Research Award
    • New for 2026! CCAS QI/Education Award
    • Podcast Series for Aspiring CCAS Researchers
    • Highlight on a CCAS Researcher
    • Call for Surveys
    • STS-CCAS Database
    • Donate to the Dolly Hansen Fund
  • Trainees
    • Introduction to Pediatric Cardiac Anesthesiology
    • Trainee Lecture Series
    • Advanced Training
    • Pediatric Cardiac Anesthesiology Fellowship Common Goals and Objectives
    • Frequently Asked Questions
    • Pediatric Cardiac Anesthesia Education Resources
    • Coaching/Mentoring Initiative
  • Patients
    • FAQs for Cardiac Anesthesia
    • FAQs for Cardiac Anesthesia – Spanish
    • SmartTots FAQs for Parents
    • Useful Resources for Parents

Question of the Week 425

Author: Melissa Colizza MD - CHU Saint-Justine, Montreal, Quebec


A 2-year-old boy with Shone’s complex, mild hypoplasia of the left ventricle, moderate mitral stenosis and moderate aortic regurgitation status-post balloon valvuloplasty of severe aortic valve stenosis is undergoing an elective Ross-Konno procedure and mitral valve repair. Which of the following factors is MOST likely associated with increased morbidity and mortality after biventricular repair of a borderline left heart disease?

Correct! Wrong!

EXPLANATION


Borderline left heart disease falls along the spectrum of hypoplastic left heart syndrome. It is characterized by the size of left-sided heart structures (aortic valve, mitral valve, LV end-diastolic volume) with z-scores of -2 to -5, as well as the presence of endocardial fibroelastosis (EFE).


The single ventricle pathway continues to have substantial mortality and morbidity including Fontan circulation failure with cyanosis and/or ventricular dysfunction as well as end-organ dysfunction, such as Fontan-associated liver disease, protein-losing enteropathy, plastic bronchitis and lymphatic dysfunction. In the past two decades, there has been increasing interest and development of surgical techniques to establish a two-ventricle circulation in patients who traditionally would have been palliated via the single-ventricle pathway. Currently, there is no consensus whether a “bad two-ventricle” repair is better than a “good single-ventricle” circulation, as long-term comparison data is unavailable.


In recent years, there have been increasing efforts to establish objective anatomic, physiologic, and hemodynamic criteria that could predict which patients with borderline left heart disease would be amenable to recruitment procedures to increase blood flow to the ventricle in order to stimulate growth and then allow for biventricular repair. Strategies to increase blood flow to the left ventricle include creating a fenestrated atrial septal defect, adding an additional source of pulmonary blood flow via a shunt, and resection of EFE tissue. Subsequent surgical decision making on whether to attempt biventricular repair depends on a comprehensive assessment based on multiple characteristics of the left heart structures and function including the following: 1) the size and structure of the mitral valve and its apparatus, 2) the size and function of the aortic valve, 3) the end-diastolic volume and pressure of the LV, 4) the apex-forming status of the LV, 5) the presence of EFE, and 6) the underlying anatomy of the congenital heart defect.


Although no single factor has been shown to predict the success or failure of biventricular conversion, there are some data that can inform the decision making process. A retrospective single-institution study assessing outcomes of 51 patients undergoing biventricular repair over a period of thirteen years from 2003-2015 by Herrin et al concluded that a pre-operative left ventricular end-diastolic pressure (LVEDP) ≥ 13mmHg and post-operative right ventricular systolic pressure (RVSP) ≥ three-quarters of the systemic systolic pressure, as well as the presence of ≥ moderate EFE, were associated with an increased likelihood of death, transplant or conversion to single ventricle physiology. Data from cardiac magneting resonance (CMR) imaging studies can also be of prognostic value. A CMR study of 32 patients with single ventricles undergoing biventricular repair concluded that a left ventricular end diastolic volume ≥ 45ml/m2 in hypoplastic left heart variants or ≥30ml/m2 in patients with double outlet right ventricle or atrioventricular canal defect were associated with a greater likelihood of success (Banka et al). These elements illustrate that, while size matters, it is the overall capacity of the left-sided circulation to accommodate a full cardiac output at low filling pressures that allows for a successful biventricular repair. In the event of a failed repair that results in diastolic dysfunction, left atrial hypertension and subsequent pulmonary hypertension, patients could become ineligible for conversion to single-ventricle physiology or for heart transplantation.


REFERENCES


Davies SJ, DiNardo JA, Emani SM, Brown ML. A Review of Biventricular Repair for the Congenital Cardiac Anesthesiologist. Semin Cardiothorac Vasc Anesth. 2023;27(1):51-63. doi: 10.1177/10892532221143880


Herrin MA, Zurakowski D, Baird CW, et al. Hemodynamic parameters predict adverse outcomes following biventricular conversion with single-ventricle palliation takedown. J Thorac Cardiovasc Surg. 2017;154(2):572-582. 10.1016/j.jtcvs.2017.02.070


Andersen ND, Scherba JC, Turek JW. Biventricular Conversion in the Borderline Hypoplastic Heart. Curr Cardiol Rep. 2020;22(10):115. doi: 10.1007/s11886-020-01363-5


Chiu P, Emani S. Left Ventricular Recruitment in Patients With Hypoplastic Left Heart Syndrome. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2021;24:30-36. doi: 10.1053/j.pcsu.2021.03.001


Banka P, Schaetzle B, Komarlu R, Emani S, Geva T, Powell AJ. Cardiovascular magnetic imaging parameters associated with early transplant-free survival in children with small left hearts following conversion from a univentricular to biventricular circulation. J Cardiovasc Magn Reson .2014; 16:73. doi: 10.1186/s12968-014-0073-1

Poll of the Month

June 2025
At your institution, do patients presenting for elective cardiac surgery who are found to be anemic on routine testing undergo formal preoperative anemia testing and treatment?
View Results
Total Answers 23
Total Votes 23

Upcoming Meeting Information


CCAS 2026 Annual Meeting

March 12, 2026
Sheraton Denver Downtown
Denver, CO

 

 

 

 

Join CCAS
Renew
Donate
Get Involved
Upcoming
Job Postings
  • Member Login
  • Forgot Password?
2209 Dickens Road, Richmond, VA 23230 • 804-282-9780 • [email protected]
Copyright © 2025 The Congenital Cardiac Anesthesia Society | View Privacy Policy