Hypoplastic Left Heart Syndrome (and stuff regarding single ventricle physiology)

Enrique Oliver Aregullin Eligio, MD Miami Childrens Hospital February 2013

GOALS
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).

Cardiac transplant
First successful cardiac transplant: Bailey, Nov. 1985

Xenotransplantation, Baby Fae

Dr. Leonard Bailey, Loma Linda University Medical Center, November 1984. http://www.babyfae.com

Anatomy

HLHS Epidemiology
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.

PATHOPHYSIOLOGY
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

DUCTAL-DEPENDENT LESION
PDA needed to:
Provide systemic perfusion
HLHS **

Critical aortic stenosis

Provide pulmonary blood flow

Tricuspid atresia

Pulmonary atresia

Provide mixing of oxygenated & deoxygenated blood

Transposition of the Great Vessels

Hyperoxitest
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!)

Positive Hyperoxitest
Seriously consider initiation of prostaglandin (PGE) at a low dose (0.03 mcg/kg/min) until diagnosis is confirmed.

Initial Assessment

ALWAYS
A - Airway
B - breathing C circulation CXR and ECG usually not very helpful in Dx.

Physical Findings
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

Pallor
Vasoconstriction from circulatory shock

Perfusion and Peripheral pulses End organs (i.e. watch UOP)

Respiratory Status
Tachypnea but with minimal distresscardiac until proven otherwise.

Respiratory Status
Respiratory distress
Inability of the respiratory system to compensate for the metabolic acidosis
Concurrent respiratory disease Unrelenting metabolic acidosis - decreased cardiac function Exhaustion

Assisted Ventilation
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

Assisted Ventilation
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
40/40/40 club

Arterial Blood Gases

In congenital heart disease typically:
Compensated or partially compensated metabolic acidosis Arterial PO2 usually low <50 with cyanotic heart diseasebut not always If PCO2 is rising, think respiratory failure - be ready to intubate!

Blood Gases

Arterial
PH PO2 PCO2 accurate accurate accurate

Capillary Venous
accurate lower invariable lower accurate higher

HCO3 (calculated)

accurate

accurate

accurate

Oxygen
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

Saturation Monitoring
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.

Prostaglandin Infusion
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

Prostaglandins continued
Side effects Apnea - be prepared to intubate Fever Hypotension - have volume and inotropes available Flushing

Access
Umbilical is preferred in a newborn
UVC even if in suboptimal position

UAC

PIC line PIV AVOID groin line if possible

Fluid Resuscitation
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.

Hypotension
Check ionized calcium
Treat with 50-100mg/kg calcium gluconate or 10 mg/kg calcium chloride via central access

Dopamine Epinephrine

Metabolic Acidosis
Treat metabolic acidosis aggressively (base deficit < -3) 1 meq/kg Na bicarbonate Repeat blood gas

Other Systems
Renal function
Urine output BUN/Cr Renal ultrasound

Thrombocytopenia R/O sepsis

Genetics

Head ultrasound

Liver function tests Coagulopathy

Fetal Diagnosis

Fetal Studies
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

HLHS

NORMAL FETAL 4-CHAMBER

Case Presentation
Term infant born via SVD Uncomplicated labor and delivery APGARs of 8 at 1min., 9 at 5min. Tachypnea noted at 12hrs of life.

Case Presentation
Airway-Breathing-Circulation
Respiratory rate (60-90 bpm)

Work of breathing (no retractions) Saturations (80%) Warm extremities; good cap refill

Case Presentation
No obvious dysmorphic features.

More Cardiac Exam Findings: No murmur. Single second heart sound (S2). Hyperdynamic precordium.

Case Presentation
Urgent Cardiology Consult Cardiac History & Physical Echocardiogram

Echocardiogram HLHS

echo

Hypoplastic Left Heart Syndrome

Case Presentation
BUT:
No beds available immediately

Need to manage infant for 24 hours before transport

NOW what do we do?

Case Presentation
Intravenous access
UVC (double lumen)

UAC PIV PIC Remember: AVOID groin lines

Case Presentation
Prostaglandins
0.03 mcg/kg/min

Side effects
Apnea Options ?
Intubate vs nasal cannula air

Case Presentation
Labs
Arterial (or venous) blood gas Electrolytes (normalize) CBC LFT Genetics Lactic acid

Head and Renal ultrasound ECHO/EKG

Case Presentation
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

Blood pressure - systolic and diastolic blood pressures are equally importantnot just mean!!
Coronary flow to heart dependant on diastolic BP

Case Presentation
Saturations 95% pO2 50 Decreased urine output Metabolic acidosis Rising lactic acid Whats going on?!?

Chest X-Ray

Case Presentation
Pulmonary Over Circulation with systemic compromise
Intubate/hypoventilate CO2

Hypoplastic Left Heart Syndrome

Case Presentation
Y- tube Physiology:
To Lungs
Pulmonary Resistance Lowered by: - Oxygen - Prostaglandin - Resp alkalosis Raised by: - PPV - Hypoxia - Resp acidosis

To Body
Systemic Resistance Raised by:

- Dopamine
- Epinephrine

Monitoring Innovations

Lactic Acid Mixed venous oxygen saturation Near infrared spectroscopy

Pulmonary Atresia

Hypoplastic RIGHT Heart

Flow begets growth

Same Y-tube Physiology

To Lungs

To Body

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

Cardiac Transplant
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)

HLHS Survival
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%

Fetal Intervention
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.

Comfort Care/Hospice
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.

Summary
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.