Congenital Heart Disease: Managing a Life Threatening Problem

Savithiri Ratnapalan, MBBS, M Ed, PhD(C), MRCP(UK), FRCP(C), FAAP

The Hospital for Sick Children, Toronto, Canada

Disclosure

I do not have any relevant financial relationship with commercial interest to disclose

Objectives

• Describe how to use clinical signs and symptoms to identify

cardiac disease

• Design an approach to the management of the cyanotic newborn

Congenital Cardiac Diseases

• PUMP

• Walls or Valves

• PIPES

• Blocked or mixed

up

Epidemiology

• CHDs affects nearly 1% of births per year in the United States

• About 25% of babies with a CHD have a critical CHD

• At least 15% of CHDs are associated with genetic conditions

Case 1: 10-day-old infant is brought to ED by mother for rapid breathing and not eating well

• Slow to breastfeed since birth

• 3 to 4 wet diapers per day

• No congestion, no fever

• No vomiting with feedings

• 2 yellow seedy stools since passing meconium after birth

• Normal spontaneous vaginal delivery birth weight 3.2 kg

Examination: Pale, rapid breathing, mottled

Vitals: HR 170, RR 70, BP 96/50, T 37°C (rectal), Wt 3.4 kg, O2 sat 90% on RA

Lung sounds equal bilaterally with crepitations in both bases

Hyperactive precordium with a gallop rhythm

Pulses weak in distal and lower extremities

Distended abdomen with liver palpable 4 cm below right costal margin

Fontanelle level, no signs of head injury, fractures or bruising

Management

• ABCs

• Give 15L O2 by non-rebreather mask or 100% O2

• Start IV, obtain blood glucose

• ECG and monitor rhythm on cardiac monitor

• CXR

• Administer fluid challenge: 10 cc/kg NS

Case Progression:

BP differential noted in lower extremities

Oxygenation improves to 99% with supplemental oxygen

CXR shows cardiomegaly and pulmonary edema

Case Discussion

• Diagnosis: Coarctation of the aorta: Duct dependent perfusion

• This infant is in CHF

• Poor feeding and easy fatigability

• Gallop rhythm and enlarged liver, diminished pulses

• Shock:

• Altered mental status, compensated shock (tachycardia, diaphoresis, respiratory distress, normal BP in upper extremities)

Management

• Administer prostaglandin E1 (PGE1): 0.05 to 0.1 mcg/kg/min• Side effects: Apnea, pulmonary congestion, fever, hypotension, seizures,

Intubate to protect against apnea and relieve stress from work of breathing

• Consider furosemide (0.5 to 1 mg/kg)

• Sepsis work-up and then antibiotics: Defer lumbar puncture

• Cardiology consult

• Infant improves with PGE1 infusion, diuretics transferred to the ICU

ECHO: Coarctation of aorta

Noncyanotic CHD ( 75% od CHD)

• May present with CHF or heart murmurs heard during physical exam

• Left-to-right shunts• Excess pulmonary vascularity • ASD, VSD, AVSD, PDA

• Obstructive lesions• Aortic stenosis, coarctation of the aorta,

mitral stenosis, pulmonic stenosis

Suspected Cardiac Cases

Check pulses: In the lower limbs too

Pre-ductal post-ductal oxygen stats (> 5% difference)

Response to oxygen

4 limb BP

ECG

CXR

Case 2: 4 day-old infant is brought to ED by mother for not eating well and looking sick

Slow to breastfeed since birth. No vomiting with feedings

3 to 4 wet diapers per day

No congestion, no fever

2 yellow seedy stools since passing meconium after birth

Normal spontaneous vaginal delivery Birth weight 3 kg

Examination: Dusky, rapid breathing, mottled

Vitals: HR 200, RR 70, BP 82/40, T 37°C (rectal), Wt 2.8 kg, O2 sat 90% on RA

A: No evidence of obstruction

B: Elevated RR, chest clear and cyanosis

C: Tachycardia, weak femoral pulse delayed cap refill, cyanosis

D: In distress and grunting

E: No signs of head injury, fractures, or bruising

Case Discussion

• Central cyanosis ? Cardiac

• Possible ductal dependent lesion:

• Right age for presentation of shock triggered by closure of the ductus arteriosus

• Measure blood pressure in four extremities

• Measure oxygen saturation pre and post ductal

• Assess oxygenation response to supplemental oxygen

Case Progression

• Oxygenation declines further to <80%

• Oxygenation fails to improve with supplemental oxygen (remains 90%)

• CXR is nonspecific

• ECG: Looks normal

Management

• Administer prostaglandin E1 (PGE1):• 0.05 to 0.1 mcg/kg/min• Intubate to protect against apnea and relieve stress of breathing

• Sepsis work-up and then antibiotics• Urgent Cardiology consult (may need balloon septostomy)

Infant improves with PGE1 infusion, transferred to the CCU

Echocardiogram: Transposition of the great vessels surgical intervention is scheduled

Cyanosis

• Respiratory disorder

• Hemoglobin disorder

• Acrocyanosis (normal newborns): Cold stress and peripheral vasoconstriction

• Generalized or central cyanosis often due to cyanotic congenital heart disease. Often worsened by crying

Hyperoxia Test

• Administer 100% oxygen

• Significant increase in PaO2 seen with pulmonary disorders

• In CHD, PaO2 will not increase or it will increase slightly• Deoxygenated blood bypasses lungs and goes directly to left side of heart,

diluting the fully oxygenated blood coming from lungs with deoxygenated blood

Cyanotic CHD

• Five Ts:• Truncus arteriosus

• Tetralogy of Fallot (TOF)

• Transposition of the great arteries (TGA)

• Tricuspid atresia

• Total anomalous pulmonary venous return (TAPVR)

• Severe aortic stenosis

• Hypoplastic left heart

• Severe coarctation of the aorta

Cyanotic Heart Disease

Nothing much other than supportive care except in

1. Duct dependent lesions: Prostaglandins IV

2. Tetralogy of Fallots blue spells ( Pul resistance systemic resistance HR )

1. High-flow oxygen

2. Knee-chest position

3. IV fluids: 0.9% NaCl bolus

4. Beta-blockers, such as: IV esmolol or propranolol

5. Vasopressors to increase systemic vascular resistance

6. Opioids in severe cases

7. Correct any underlying causes: arrhythmia, hypothermia, hypoglycaemia, acidosis

Diagnostic Studies CHD

• Laboratory:• Glucose: Any child in distress needs to have hypoglycemia excluded• CBC: Look for anemia, signs of sepsis• Electrolytes: Congenital adrenal hyperplasia, salt-wasting form

•Arterial blood gas: Hyperoxia test

• CXR: Cardiomegaly, pulmonary vascular congestion• ECG: Abnormal axis, ST segment changes

• Echocardiogram: Definitive anatomic diagnosis, degree of congestive heart failure (chamber sizes, contractility)

The CXR : Heart

Heart position, size and shape

CXR Lungs: Normal increased or decreased vascularity

Normal Decreased Increased

1 2 3

Cyanotic CHD

• Increased pulmonary vascularity:

1. Total anomalous pulmonary venous return

2. Truncus arteriosus

3. Transposition of the great arteries

4. Complex lesions without pulmonic stenosis

• Decreased pulmonary vascularity

1. Tetralogy of Fallot (has Pulmonary stenosis)

2. Ebstein’s anomaly (abnormal Tricuspid)

3. Tricuspid atresia

4. Pulmonic atresia

Can you match these CXRs to the Lesions

1.Tetralogy of Fallot (TOF) Boot shaped2.Total Anomalous Pulmonary Venous Drainage TAPVR: Snowman3.Transposition of Great Arteries TGA: Egg on side

ECG

Right axis (RVH): Normal for newborns Left axis: Hypoplastic right heart, tricuspid atresia, endocardial cushion defect (AV canal)

ST-T changes, strain, ischemiaDysrhythmiaProlonged QTLow voltage

T waves upright in V1 until day 5 then flattens and goes down

It is still in your hands

15-year-old transferred to our hospital for management of severe hypertension. The child had gone to the doctor for persistent headaches and had a BP of 210/140

Labetalol infusion started in local ED and transferred to SickKids

CHD Summary