How To Handle Spells Of Tet?

Tetralogy of Fallot (TOF) is a congenital heart condition that causes sudden episodes of cyanosis and hypoxia. These spells can be managed by placing the patient in a knee-chest position, oxygen, morphine, IV fluids, beta-blockers, phenylephrine infusion, and emergency procedures if needed. The main features of TOF include a large ventricular septal defect, right ventricular outflow tract obstruction, pulmonic valve stenosis, and right ventricular hypertrophy.

To manage a ‘tet spell’, it is important to calm the child down and place their knees on their chest. Children with TOF can manage a ‘tet spell’ themselves by squatting or bringing their knees to their chest. Squatting increases systemic vascular resistance, which can prevent complications from prolonged hypoxia.

In infants (0-12 months) with tetralogy of Fallot having a hypercyanotic spell, the primary objectives are to decrease catecholamine production, increase blood oxygenation, and increase blood pressure. Place infants in a knee-chest position, establish a calm environment, and administer Propranolol 0.5 mg/kg/dose TDS in the hospital. The exact mechanism of propranolol is unclear, but it is thought to work by relaxing the infundibulum and slowing the heart rate.

Tet spells usually last less than 1 minute and can be caused by certain heart defects. Management of hypercyanotic spells requires a rapid and aggressive stepwise approach, including positioning the patient in a knee-chest position, reducing preload, increasing systolic blood flow, and improving preload and systolic blood flow. The key to treatment is to increase systemic vascular resistance.

What medication is used for tet spells?

The child is placed on their back to increase aortic resistance, which reduces blood rush through the septal hole from the right ventricle and improves blood circulation to the lungs. Oxygen may be given through a face mask, and medications like morphine, propranolol, or phenylephrine may be given to decrease tet spell frequency and severity. The Blalock-Taussig operation is a palliative procedure performed in smaller infants to increase blood flow to the lungs, allowing the child to grow big enough for surgical repair. A connection is made between the right subclavian artery and the right pulmonary artery, increasing red oxygenated blood reaching the lungs and relieving cyanosis and symptoms.

What is the mechanism of tet spells?

Tetralogy of Fallot is a condition where infants with the condition do not show signs of cyanosis at birth but may develop episodes of bluish pale skin during crying or feeding. These episodes can be lethal and unpredictable, possibly due to a spasm of the infundibular septum, which worsens the right ventricular outflow tract obstruction. These spells can be aborted with simple procedures. Older children with tetralogy of Fallot often squat to increase pulmonary blood flow, which is a diagnostic mechanism of diagnostic significance.

Exertional dyspnea usually worsens with age, and occasionally, hemoptysis due to bronchial collateral rupture may result. The rare patient may remain marginally cyanotic or acyanotic and asymptomatic into adulthood.

What triggers a Tet spell?

Tet spells are sudden changes in skin, nails, and lips in babies with tetralogy of Fallot, caused by a rapid drop in oxygen levels. These episodes are most common in young infants, aged 2 to 4 months, and may be less noticeable in toddlers and older children. Severe congenital heart defects are often diagnosed before or soon after birth, and symptoms such as trouble breathing, bluish skin color, lack of alertness, seizures, weakness, and increased irritability should be sought medical attention.

Why does squatting help tet spells?

Hypercyanotic spells are characterized by rapid and deep respirations, irritability, prolonged crying, increased cyanosis, and decreased heart murmur intensity. These spells are most common in young infants, peaking between 2 and 4 months. Severe spells can lead to limpness, seizures, and death. Some toddlers may squat during play, increasing systemic vascular resistance and aortic pressure, which decreases right-to-left ventricular shunting and increases arterial oxygen saturation.

Auscultation detects a grade 3 to 5/6 systolic ejection murmur at the left mid and upper sternal border, due to pulmonic stenosis. The ventricular septal defect is usually silent, but as pulmonary outflow obstruction becomes severe, the murmur becomes softer. The second heart sound is usually single, with a prominent right ventricular impulse and systolic thrill.

What triggers a tet spell?

Tet spells are sudden changes in skin, nails, and lips in babies with tetralogy of Fallot, caused by a rapid drop in oxygen levels. These episodes are most common in young infants, aged 2 to 4 months, and may be less noticeable in toddlers and older children. Severe congenital heart defects are often diagnosed before or soon after birth, and symptoms such as trouble breathing, bluish skin color, lack of alertness, seizures, weakness, and increased irritability should be sought medical attention.

What are the interventions for tet spells?

Tet spells in babies can be corrected by placing them in a knees-to-chest position, adjusting heart pressure and blood flow. If frequent, surgery is necessary. Surgeons can use a shunt to improve blood flow to the lungs, directing blood from the aorta to the right pulmonary artery. Some babies have lungs with too small arteries, requiring treatment to increase blood flow. Doctors have two ways to do this:

1. Place the child in a knees-to-chest position, adjusting heart pressure and blood flow.
2. Call the doctor if tet spells occur frequently.

What is the nursing priority for tet spells?

In a child with Tetralogy of Fallot (ToF), the general guidelines include drawing the child’s knees up to their chest to increase systemic vascular resistance, providing oxygen, and ordering additional therapies such as an IV narcotic and fluid bolus. A beta blocker may be used to decrease right-ventricular outflow tract obstruction, increasing pulmonary blood flow. If these measures don’t produce the desired result, a vasoconstrictor may be ordered to increase SVR and promote flow into the pulmonary circulation.

Prophylactic antibiotics are likely for children with ToF until the defects can be surgically repaired. Heart failure is a common issue in many patients with ToF, with mainstays of treatment being digoxin and loop diuretics like furosemide. ACE-inhibitors and ARBs are not used in patients with ToF because they can reduce SVR, potentially causing a hypercyanotic episode.

What is the best choice for management of a Tet spell?

Mild tetanus cases can be managed with calming and postural exercises. However, if no improvement is observed, the child should be transferred immediately to a medical facility and provided with additional oxygen.

How do you treat a Tet spell?

To manage the symptoms of the infant, it is recommended that the infant be positioned in a squatting position, that extra oxygen and beta-blocker medication be administered as prescribed by the attending physician, and that the infant’s condition be monitored. It is imperative to ascertain that the patient is consuming an adequate quantity of fluids, engaging in appropriate physical activity, and adhering to the prescribed medication regimen. Furthermore, it is imperative to ascertain that the patient is not engaging in excessive physical exertion and is adhering to the prescribed medication regimen.

Is a Tet spell an emergency?

Cyanosis, a condition causing bluish skin due to deoxygenated hemoglobin or methomoglobin in the blood, is primarily caused by cardiovascular or pulmonary shunts. Tet spell, an episodic central cyanosis, is often caused by a congenital heart disease like Tetralogy of Fallot (TOF). Early recognition and proper management can prevent major morbidity or mortality. A 29-year-old man presented with cyanotic spell and later confirmed a diagnosis of TOF. This case highlights the importance of TOF as a differential diagnosis of hypercyanotic crises and its unique manifestations and management.

How to manage cyanotic spells?

Cyanotic episodes in children with congenital heart disease (CHD) are acute episodes of cyanosis or exaggerated cyanosis compared to the child’s baseline. These episodes are most common in children with Tetralogy of Fallot and pulmonary atresia, and are caused by an imbalance between pulmonary and systemic vascular resistance. They typically occur between 2-6 months of age and usually occur during times of stress, such as when unwell with an infection, upset, or upon waking.

The management of these episodes is crucial, as distress can worsen or prolong the episode. The child should be comforted by the parent, and clinicians should use a minimal handling approach with targeted examination. Prolonged episodes may be life-threatening and require active treatment with oxygen and opiates.