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Enrique Oliver Aregullin Eligio, MD Miami Children’s Hospital February 2013
• Appreciation of history of Hypoplastic Left Heart Syndrome. • Basic anatomy & physiology. • Understand the LOGIC behind the management of HLHS (& single ventricle lesions in general).
Hypoplastic Left Heart Syndrome
• Spectrum of underdevelopment of the left ventricular cavity.
Have underdeveloped aortic & mitral valves (stenosis or atresia). Left ventricle is unable to support systemic circulation (and, therefore, right ventricle is used as the single ventricle).
History of HLHS
• First described by Maurice Lev in 1952. • Term used by Noonan & Nadas in 1958. • Options offered:
Comfort care Staged palliative repair, i.e. “Norwood procedure”
First successful 3-stage completion in 1983 (after multiple surgeries from 1979).
First successful cardiac transplant: Bailey, Nov. 1985
Xenotransplantation, “Baby Fae”
• Dr. Leonard Bailey, Loma Linda University Medical Center, November 1984. • http://www.babyfae.com
• Low incidence of 1.6 to 3.6 per 10,000 live births, BUT causes 23% or cardiac deaths during 1st week of life and 15% during the 1st month of life. • Makes up about 2-4% of congenital heart disease. • More commonly males (55% to 67%). • With ONE affected child, recurrence risk is about 0.5% to 2%. • 12% prevalence of left-sided obstructive lesions in 1st degree relatives. • 15-30% incidence of genetic syndromes and extracardiac anomalies in patients w/HLHS. • Genetic markers: dHAND, HRT1, HRT2, NOTCH.
• Moss & Adams, 2008.
• Cardiac development: “Flow begets growth.” • Altered flow through the left side of the heart:
Reduced/altered flow across the foramen ovale. Aortic or mitral obstruction.
Typical Clinical Presentation
• Known Congenital Heart Defect Prenatal Diagnosis
• Unknown Congenital Heart Defect Normal pregnancy, labor and delivery Clinically doing okay until the PDA closes ** Cyanosis that does not improve with oxygen Many have no other obvious anomalies
• PDA needed to:
Provide systemic perfusion
Critical aortic stenosis
Provide pulmonary blood flow
Provide mixing of oxygenated & deoxygenated blood
Transposition of the Great Vessels
• ABG is measured on room air. • Patient is placed on 100% oxygen (intubated) for 10-15 minutes, then ABG is repeated.
If problem is respiratory (i.e. hypoventilation), then PaO2 improves (usually above 200mmHg). If problem is cardiac (i.e. right-to-left intracardiac shunt), there is little improvement of PaO2. Primary pulmonary hypertension may also result in little improvement of PaO2. (Oxygen may hasten closure of PDA!)
• Seriously consider initiation of prostaglandin (PGE) at a low dose (0.03 mcg/kg/min) until diagnosis is confirmed.
A - Airway
B - breathing C – circulation CXR and ECG usually not very helpful in Dx.
• Comfortable or in distress?
Cyanosis w/out respiratory distress is cardiac until proven otherwise
• Active or lethargic? • Cyanosis?
Degree - saturation usually <85% to be seen Anemia makes cyanosis difficult to notice
Vasoconstriction from circulatory shock
• Perfusion and Peripheral pulses • End organs (i.e. watch UOP)
• Tachypnea but with minimal distress…cardiac until proven otherwise.
• Respiratory distress
Inability of the respiratory system to compensate for the metabolic acidosis
• Concurrent respiratory disease • Unrelenting metabolic acidosis - decreased cardiac function • Exhaustion
• Intubate if:
Impending respiratory failure Potentially not necessary to intubate just for PGE therapy if ground transport Intubate for air transport in PGE dependent babies
• Ventilation strategy
Volume ventilation if possible to maintain consistent minute ventilation in the face of changing lung compliance Bigger tidal volumes compared to premature newborns (10 cc/kg); lower rates
No need to “over-ventilate”
Arterial Blood Gases
• In congenital heart disease typically:
Compensated or partially compensated metabolic acidosis Arterial PO2 usually low <50 with cyanotic heart disease…but not always • If PCO2 is rising, think respiratory failure - be ready to intubate!
PH PO2 PCO2 accurate accurate accurate
accurate lower invariable lower accurate higher
• Oxygen is a drug - use it with respect
• Oxygen is a pulmonary vasodilator
May worsen pulmonary congestion
• Oxygen is a stimulus for the PDA to close
May worsen ductal dependent lesions by speeding up closure of the PDA
• Oxygen is not bad
• Oxygen saturation reflects tissue oxygenation and usually does not correlate with PO2. • With pulmonary hypertension will see differential cyanosis - shunts right to left across the PDA. • The number is not as important as the patient.
• Purpose is to open the PDA if a ductal dependent lesion is suspected • Can be initiated before a definitive diagnosis is established • Need a secure IV (PIV, PIC, or UVC-central or in the liver)
• Start at low dose 0.03 mcg/kg/min
• Side effects Apnea - be prepared to intubate Fever Hypotension - have volume and inotropes available Flushing
• Umbilical is preferred in a newborn
UVC – even if in suboptimal position
• PIC line • PIV • AVOID groin line if possible
• Needed if poorly perfused • 5% albumin bolus (5-10 cc/kg) • Watch for and treat hypoglycemia - stress causes epinephrine release which increases utilization of glucose. • PRBC to treat anemia - optimize oxygen carrying capacity.
• Check ionized calcium
Treat with 50-100mg/kg calcium gluconate or 10 mg/kg calcium chloride via central access
• Dopamine • Epinephrine
• Treat metabolic acidosis aggressively (base deficit < -3) • 1 meq/kg Na bicarbonate • Repeat blood gas
• Renal function
Urine output BUN/Cr Renal ultrasound
• Thrombocytopenia • R/O sepsis
• Head ultrasound
• Liver function tests • Coagulopathy
• Hornberger, 1995: 21 fetuses with prenatal echos that show left-sided obstruction (small mitral valve & ascending aorta) developed HLHS. • Critical aortic stenosis decreased blood flow through left heart LV dilation & dysfunction endocardial fibroelastosis (EFE) backwards flow across PFO LV stops growing & eventually shrinks
NORMAL FETAL 4-CHAMBER
• • • • Term infant born via SVD Uncomplicated labor and delivery APGARs of 8 at 1min., 9 at 5min. Tachypnea noted at 12hrs of life.
Respiratory rate (60-90 bpm)
Work of breathing (no retractions) Saturations (80%) Warm extremities; good cap refill
No obvious dysmorphic features.
More Cardiac Exam Findings: No murmur. Single second heart sound (S2). Hyperdynamic precordium.
• Urgent Cardiology Consult • Cardiac History & Physical • Echocardiogram
Hypoplastic Left Heart Syndrome
No beds available immediately
Need to manage infant for 24 hours before transport
• NOW what do we do?
• Intravenous access
UVC (double lumen)
UAC PIV PIC Remember: AVOID groin lines
• Side effects
Apnea • Options ?
• Intubate vs nasal cannula air
Arterial (or venous) blood gas Electrolytes (normalize) CBC LFT Genetics Lactic acid
• Head and Renal ultrasound • ECHO/EKG
• R/O Sepsis
If no clinical suspicion or maternal indicators no need to start antibiotics
• • • •
Follow ABG frequently (Q 4 hrs) Monitor urine output Monitor for acidosis Watch for hypotension
• Blood pressure - systolic and diastolic blood pressures are equally important…not just mean!!
Coronary flow to heart dependant on diastolic BP
• Saturations 95% • pO2 50 • Decreased urine output • Metabolic acidosis • Rising lactic acid What’s going on?!?
• Pulmonary Over Circulation with systemic compromise
Hypoplastic Left Heart Syndrome
• “Y- tube” Physiology:
Pulmonary Resistance Lowered by: - Oxygen - Prostaglandin - Resp alkalosis Raised by: - PPV - Hypoxia - Resp acidosis
Systemic Resistance Raised by:
• Lactic Acid • Mixed venous oxygen saturation • Near infrared spectroscopy
“Hypoplastic RIGHT Heart”
“Flow begets growth”
Same “Y-tube” Physiology
But now not enough blood flow to lungs
“Ideal” Saturation for PDA-dependant
• For „balanced‟ amount of blood flow to both the lungs and the body in a single ventricle (i.e. “Ytube physiology” infant) is:
75% to 85% oxygen saturation (in upper extremity)
Options for HLHS in 2013
• • • • Comfort Care Transplant 3-Stage Palliative Repair Fetal Intervention
• Fairly good quality of life as transplant recipient (…have structurally normal heart). • Obstacles:
Availability of donor heart (approximately 25-30% die awaiting transplant). Life-long immunosuppression & risk of infection/CA. Usual cause of death/organ death: coronary vasculopathy. Survival: 84% at 1 yr, 76% at 5 yrs., 70% at 7 yrs. Organ survival MUCH reduced w/subsequent transplants.
HLHS Palliative Repair
A. HLHS (sats 80s) B. Norwood repair in 2wks - Provide systemic BF - Balance pulmonary BF C. Glenn repair (SVC to PA) - More pulmonary BF
D. Fontan repair (IVC to PA) - Relieve volume load to RV - Venous blood totally bypasses heart (sats 100%)
Norwood (Stage I)
• Standard Risk (i.e. no genetic or extracardiac issues)
1 month – 85% 1 year – 80% 5 year – 73%
• Higher Risk
1 month – 61%
1 year – 20%
• VERY small balloon catheter is inserted via mothers abdomen, across uterus, through fetal heart across aortic valve. Fetal aortic valvuloplasty is performed. • Marginal success with select patients
Must have diagnosis in early 2nd trimester Absence of genetic or extracardiac anomalies Early stage of critical aortic stenosis (LV is dilated with some preserved function, but not yet involuted) Favorable maternal habitus
Why Fontans Fail
As we age, the ventricular EDP rises. In Fontan patients, the CVP must exceed the EDP. Eventually, the EDP will rise to an intolerable level.
• Why is it a viable option in 2010?
“The Fontan is doomed to fail.” Dr. Reddington, ACC 2003
Fontan patients will develop protein-losing enteropathy, ventricular dysfunction, hypoxemia, thromboembolism, arrhythmias and liver failure.
• HLHS is universally lethal w/out treatment. • A patent foramen ovale & ductus arteriosus are necessary for survival.
• Echocardiogram is modality of choice for diagnosis.
• Management of the neonate w/HLHS is complicated: PGE is necessary as well as ventilation/support to permit sats 75% to 85% and no acidosis. • Transplant and staged repair are not w/out their complications (survival for both about 70% in 5 yrs). • Comfort care & fetal interventions are options to be considered. • Decision-making is a TEAM effort by pt. family & medical team.