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Newborn with Hypercyanotic Episodes

Chris Nickson

aka Pediatric Perplexity 003

A 26 day-old baby boy has been brought to the emergency department by ambulance. The past few days he has had poor feeding and recurrent episodes of respiratory distress associated with turning blue. He was diagnosed with Tetralogy of Fallot antenatally and was born at term (a normal vaginal birth). An echocardiogram 2 days after he was born confirmed the diagnosis it also showed that his ductus arteriosus had closed. He was discharged from hospital 4 days after he was born. On presentation, the baby is agitated, tachycardic, tachypneic and profoundly cyanotic.

Questions
Q1. What is Tetralogy of Fallot? Show answer Tetralogy of Fallot (TOF) is a constellation of four cardiac abnormalities that results from a single developmental defect, namely, failure of the subpulmonic conus to expand. TOF accounts for about 10% of congenital heart disease.

The four characteristic abnormalities of TOF are: 1. right ventricular hypertrophy (RVH) 2. right ventricular outow tract obstruction (RVOTO) 3. ventricular septal defect (VSD) 4. the presence of an over-riding aorta (dextroposed aorta)

These defects result in decreased pulmonary flow and right-to-left shunt through the VSD. Age of presentation and severity of cyanosis is largely determined by the severity of RVOTO cyanosis may not be present at birth. In a neonate with a patent ductus arteriosus (PDA) an infusion of prostaglandin E1 can be used to prevent closure of the ductus, thus maintaining pulmonary blood flow via a left-to-right shunt from the aorta. Q2. What is the likely diagnosis? Show answer

A hypercyanotic episode, aka a tet spell.

These are severe cyanotic spells that typically occur in children with TOF. They can also occur with other cyanotic cardiac lesions.

Normal Heart (from RCH Melbourne Cardiology click on image)

Q3. Describe the underlying pathophysiology? Show answer

Management of tet spells makes sense if you understand the underlying pathophysiology.

Tet spells may be spontaneous or precipitated by events associated with decreased systemic vascular resistance, such as: tachycardia hypotension exertion during feeding defecation crying This leads to a vicious cycle, that is usually self-limiting (often only 15 to 30 minutes long) but may ultimately result in seizures, stroke or even death. Q4. What cardiac murmur would you expect to hear during this episode? Show answer Children with TOF typically have a right ventricular outflow tract obstruction murmur. During a tet spell this murmur may become inaudible as blood flows through the VSD rather than the right ventricular outflow tract. There is also a loud and single S2 in TOF.
Fallots Tetralogy (from RCH Melbourne Cardiology click on image)

Right ventricular outflow tract obstruction murmur:

The tet spell vicious cycle (click to enlarge)

Harsh systolic crescendo decrescendo murmur usually heard along the left mid- to upper sternal border with posterior radiation VSD murmur: Harsh pansystolic murmur maximal at, and almost confined to, the lower left sternal edge. It is louder on expiration and when the defect is small.

Q5. What are the classic chest x-ray findings in Tetralogy of Fallot? Show answer

The boot-shaped heart .

Although heart size is usually normal it is classically boot-shaped (coeur en sabot). There is a prominent right ventricle with decreased pulmonary vascularity. Q6. Describe the initial management of this episode. Show answer

The main objectives are to increase systemic vascular resistance, correct hypoxia and correct acidemia.

To achieve this:

Adminster high-ow oxygen via mask or head box (decreases pulmonary vascular resistance) Avoid exacerbating distress, and remove identifiable precipitants (e.g. any cause of pain) Console the child by cradling in a kneechest position. Squatting may be effective in older children. (increases systemic vascular resistance) Adminster opioids in severe cases: eg. morphine 0.2 mg/kg IM This relives distress and helps abolishes hyperpnea, but may worsen the loss of systemic vascular resistance. Other options include: midazolam, fentanyl, ketamine 1-2 mg/kg IV Initiate continuous ECG and oxygen saturation monitoring, frequent BP monitoring. Correct any underlying causes such as arrhythmia, hypothermia, hypoglycaemia.
The boot-shaped heart of Fallots Tetralogy

Despite the initial measures describe in Q6, the baby remains distressed and profoundly cyanotic. Q7. Describe the further management of a prolonged episode. Show answer Therapeutic options for prolonged tet spells include:

IV uids: 0.9% NaCl 10 mL/kg bolus followed by maintenance uids. (increases venous return and may decrease heart rate) Correction of acidosis: sodium bicarbonate 12 mmol/kg IV (ensure adequate ventilation)

Beta-blockers, such as: IV esmolol: 0.5 mg/kg over 1 min, then 50200 mcg/kg/min up to 48 h or propanolol: 0.01-0.2 mg/kg IV (decreases heart rate and relieves infundibular spasm) Vasopressors to increase systemic vascular resistance, promoting the redirection of blood flow through the pulmonary circulation: phenylephrine: 0.01-0.02 mg/kg IV or metaraminol 0.01 mg/kg stat, then 0.1-1 mcg/kg/min or noradrenaline 0.1-0.5 mcg/kg/min (increases systemic vascular resistance) Manual compression of the aorta has also been described. Intubation and positive pressure ventilation may be required in extreme cases.

This case is continued in Cardiovascular Curveball #009

References
Costello JM, Almodovar MC. Emergency care for infants and children with acute cardiac disease. Clin Ped Emerg Med 8:145-155; doi:10.1016/j.cpem.2007.06.005 Duncan A, Croston E. Guidelines for Intensive Care Management of Infants and Children after Congenital Heart Surgery. Pediatric Intensive Care Unit, Princess Margaret Hospital, 2008. Libby P, et al. Braunwalds Heart Disease: A Textbook of Cardiovascular Medicine (8th edition), Saunders Elsevier, 2008. Steinhorn RH. Evaluation and management of the cyanotic neonate. Clin Pediatr Emerg Med. 2008 Sep;9(3):169-175. PMID: 19727322; PMCID: 2598396 van Roekens CN, Zuckerberg AL. Emergency management of hypercyanotic crises in tetralogy of Fallot. Ann Emerg Med. 1995 Feb;25(2):256-8. PMID: 7832359.