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What Pediatricians Should Know About Children With Single Ventricle Laxmi Ghimire, MD PGY3, KU Pediatrics
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What Pediatricians Should Know About Children With Single Ventricle
Laxmi Ghimire, MD PGY3, KU Pediatrics
ObjecEves • To define single ventricle cardiac defect • To idenEfy pathophysiology and clinical features of HLHS in
brief • Formulate management of pre and postop care of child with
single ventricle • Evaluate long term complicaEons and follow up of HLHS • Describe the role of pediatricians in following children with
HLHS
What is hypoplasEc leO heart?
HLHS Epidemiology
• 1.2-‐1.5% of all congenital heart defects
• In US, ≈2000 infants are born/ year with HLHS
• Without surgery HLHS is uniformly fatal usually ≤2 weeks of life.
Pathophysiology of HLHS
• Ductal dependent lesions • Important to keep duct open, why?
– To provide systemic circulaEon
Clinical features of HLHS
• Around 24-‐48 hours aOer the ductus closes-‐ signs of cardiogenic shock – Cyanosis, – Tachypnea, – Respiratory distress, – Pallor, – Lethargy, – Metabolic acidosis, and – Oliguria. – Poor peripheral perfusion
Cardiac exam • A prominent right ventricular impulse • A normal S1 • A loud single S2 • Usually no murmur is noted
Management of HLHS
• SupporEve care
• MulEstage surgical intervenEon (ie, Norwood, Glenn, and Fontan procedures) and
• Cardiac transplantaEon
Medical preop management • Prenatal diagnosis
• HLHS is idenEfied: deliver at an insEtuEon where neonatal cardiac surgery, is performed
• Try for term delivery, vaginal delivery well tolerated
PreoperaEve management
1. Open the ductus arteriosus
– HLHS is suspected: start PG E1 infusion immediately to establish ductal patency and ensure adequate systemic perfusion.
– Refer
Pre-‐op
• Intubate or not to intubate? • If short distance: do not intubate
• OxygenaEon: Avoid high amount of oxygen
• Keep sats between 75-‐85%, why?
Systemic arterial oxygen saturation versus systemic oxygen (O2) delivery.
Barnea O et al. Circulation. 1998;98:1407-1413
Copyright © American Heart Association, Inc. All rights reserved.
Pre-‐op
2. Manipulate pulmonary vascular resistance (PVR) Goal: Qp/Qs:0.8-‐1
Factors that increase PVR: -‐Hypoxia -‐Hypercarbia -‐HyperinflaEon -‐High hematocrit
Pre-‐op
3. Correct metabolic acidosis
– Metabolic acidosis indicates inadequate cardiac output – Aim for base deficit of 0
Surgical management
• Norwood(stage I) • BidirecEonal Glenn(stage II) • Fontan (Stage III)
Norwood Procedure
– Done in first weeks of life-‐aOer infant stable
– The goals • To establish reliable systemic circulaEon without the DA
• To provide enough pulmonary blood and prepare pul vascular bed for stages II and III.
Norwood Postop
• Norwood Clinical pathway
Norwood postop
• Discharge:
– PCP: adjust medicaEon dose with weight – Oxygen saturaEon is typically 70-‐80% in room air.
– BT shunt: conEnuous murmur – Thrombosis of shunt
BidirecEonal Glenn procedure (Stage II)
• Aprox 4-‐6 months aOer Norwood procedure. • The bidirecEonal Glenn procedure => anastomosis between
SVC and RPA, end-‐to-‐side
• ComplicaEons: Progressive desaturaEons-‐due to venous collaterals
• No murmur: silent. Thrombosis of Glenn
BidirecEonal Glenn (Stage II)
Fontan procedure(stage III)
• The Fontan procedure :1.5-‐4 yrs aOer Glenn
• CompleEon of the Fontan procedure – Blood flow from IVC to the PAs – Systemic venous blood returns to the lungs passively without passing through a ventricle.
• Fontan looks simple: hemodynamics unclear
Fontan procedure(Stage III)
Normal circulation Vs Fontan Circulation.
Gewillig M Heart 2005;91:839-846
Copyright © BMJ Publishing Group Ltd & British Cardiovascular Society. All rights reserved.
ComplicaEons of Fontan CirculaEon
• Mild to moderate exercise intolerance • Residual cardiomegaly • Ventricular dysfuncEon • Rhythm and conducEon disturbances • Hepatomegaly • LymphaEc dysfuncEon with protein losing enteropathy • Early and late mortality
LymphaEc DysfuncEon
─ Impedes drainage of thoracic duct.
– Leakage in intersEEum => lymph edema/ pulmonary edema, a very
lethal complicaEon in the early postoperaEve period.
– Chylothorax or chylopericardium – Leakage into the gut leads to protein losing enteropathy (PLE)
OutpaEent follow up • Periodic follow-‐up visits aOer stage I, II, and III operaEons are
mandatory.
• SubstanEal (5-‐15%) interstage mortality (between stages I and II)
• Careful observaEon, follow-‐up and home surveillance
• Interstage mortality between stage II (bidirecEonal Glenn) and stage III (Fontan) is lower that aOer stage I (Norwood)
Cumulative hazard by mode of death.
Khairy P et al. Circulation. 2008;117:85-92
Copyright © American Heart Association, Inc. All rights reserved.
Prognosis
• Overall survival Norwood is ~75%.
• Survival aOer the bidirecEonal Glenn/hemi-‐Fontan and Fontan operaEons is nearly 90-‐95%.
• Survival rate post staged reconstrucEon is 70% at 5 years.
PaEent educaEon • General: HLHS is a complex heart defect that requires mulEple
hospitalizaEons, surgeries, catheter intervenEons and long-‐term follow-‐up.
• MedicaEon – Educate parents regarding cardiac medicaEons, interacEons
• Feeding – Many require NG or G-‐tube tube feeding – Increased-‐calorie formula is required for adequate growth.
• Follow-‐up care – Importance of follow-‐up care.
Role of Pediatricians
• Cardiac care • Neuro-‐behavioral care • GastrointesEnal care • Growth
Prevalence of neurodevelopmental impairment in the population with congenital heart disease (CHD).
Marino B S et al. Circulation. 2012;126:1143-1172
Copyright © American Heart Association, Inc. All rights reserved.
Developmental outcome
CirculaEon. 2002;106:I-‐95-‐I-‐102
Liver funcEon aOer Fontan
• Deranged hepaEc funcEon – ProlongaEon of the PT – Low factor V level.
• No relaEonship between cardiac funcEonal measurements and liver funcEon.
Fontan and Liver problems • 1/3-‐2/3 paEents would have liver problem by teenage years • Good Eme to monitor liver funcEon= 11 yrs
Baek J S et al. Heart 2010;96:1750-1755
Hepatic fibrosis marker (Forns index) after Fontan operation.
Baek J S et al. Heart 2010;96:1750-1755
Copyright © BMJ Publishing Group Ltd & British Cardiovascular Society. All rights reserved.
LymphaEc DysfuncEon: Protein Losing
Enteropathy • Fontan circulaEon
– Impedes drainage of thoracic duct.
– Leakage into the gut leads to protein losing enteropathy (PLE), the most frequent lymphaEc problem in long term follow up
Protein‐Losing Enteropathy after Fontan Operation
Congenital Heart Disease Volume 2, Issue 5, pages 288-‐300, 14 SEP 2007 DOI: 10.1111/j.1747-‐0803.2007.00116.x hqp://onlinelibrary.wiley.com/doi/10.1111/j.1747-‐0803.2007.00116.x/full#f3
Poor Growth and Single Ventricle
• Significantly underweight and shorter
• Shorter stature : ~25% Fontan survivors Vs 13.4% in the healthy pediatric populaEon.
Cardiology in the Young, 10, pp 447-‐457. 2000
American Heart Journal -‐2010 Vol. 160, P 1092-‐1098.
Weight in Children With Single Ventricle Physiology
J Am Coll Cardiol. 2007;50(19):1876-1883.
Height in Children With Single Ventricle Physiology
J Am Coll Cardiol. 2007;50(19):1876-1883.
The most important component of care of
single ventricle children..
………CoordinaEon of care between primary care physician and subspecialEes.
Take home messages • Single ventricle distorts the normal physiology
• Surgeries are complicated
• Pediatrician can do a lot make sure they are growing well and geung appropriate care
• AnEcipate the complicaEons of Fontan
• Cardiac, Neuro, GI, poor growth are the most common long complicaEons encountered. Coordinate care with other specialEes.
Thank you!!
• Dr. Schroeder • Dr. Goertz • Dr. Meyer • Dr. Almadhoun
Bibliography • Park, M. Textbook of Pediatric Cardiology. 5th EdiEon. 2007 • Moss & Adam’s Heart diseases in Infants, Children and adolescents. 8th
EdiEon. 2012 • Rao, S. HypoplasEc LeO Heart Syndrome. E-‐medicine(Accessed
12/20/2013)
