What Spells Can You Teach Tet?

Tetralogy of Fallot (TOF) is a congenital heart defect that affects infants and children, causing sudden episodes of cyanosis and hypoxia. The condition involves a large ventricular septal defect, right ventricular outflow tract and pulmonary valve obstruction, and over-riding of the aorta. Pulmonary blood flow is decreased, leading to cyanotic spells.

Tet spells can be caused by certain heart conditions or illness, and parents should be taught how to recognize and manage them. General guidelines include raising the child’s knees to their chest, oxygen, morphine, IV fluids, beta-blockers, phenylephrine infusion, and emergency procedures.

The pathophysiology of a tet spell is a decrease in systemic vascular resistance, increasing a right to left shunt, which leads to various complications. Hypercyanotic spells may occur at any stage of life but are more common at 2-6 months of age. Commonly used drugs for tet spells include intravenous propranolol (0.015 mg/kg to 0.02 mg/kg) or short-acting esmolol (0.5mg/kg given).

Medications used in tet spells include intravenous propranolol (0.015 mg/kg to 0.02 mg/kg) or short-acting esmolol (0.5mg/kg given). If tet spells occur often, it is typically important for the child to have surgery soon.

Understanding the basics of hypercyanotic spells may leave parents inadequately prepared for the situation. Expanding therapeutic options can help address the challenges associated with managing these spells and ensure the best possible outcomes for the child.

Can adults have tet spells?

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.

Why do kids squat during a tet spell?

Severe spells in toddlers can result in a range of adverse outcomes, including limpness, seizures, and even death. Playtime squatting has been demonstrated to increase systemic vascular resistance and aortic pressure, thereby reducing ventricular shunting and increasing arterial oxygen saturation.

What to do with tet spells?

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.

Why does crying cause 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 age do tet spells start?

Hypercyanotic spells, also known as tet spells, are a risk for infants with unrepaired tetralogy of Fallot or other congenital cardiac defects causing dynamic right ventricular outflow tract obstruction (RVOTO). These spells can occur at any stage of life, but are more common at 2-6 months of age. These guidelines are for the medical, nursing, and allied health staff of Perth Children’s Hospital, but they should not replace the judgement of a senior clinician. Clinical common-sense should be applied at all times and should not be relied on as a substitute for proper assessment of each case and patient’s needs.

What are the 4 defects of Tet?

Tetralogy of Fallot is a heart defect characterized by four distinct problems: ventricular septal defect, overriding aorta, pulmonary stenosis, and right ventricular hypertrophy. During pregnancy, a pregnancy doctor may refer the mother to a maternal-fetal medicine specialist or the Fetal Center at Nationwide Children’s, which connects the mother with a multidisciplinary team of cardiologists, neonatologists, cardiothoracic surgeons, and nurses. Nurse coordinators can answer questions, guide the mother through pregnancy, and prepare for the baby’s care post-birth.

During clinic visits, the medical team will discuss the diagnosis, treatment, recovery, and long-term outcome of the baby. A tour of the Cardiac Intensive Care Unit will be provided to help prepare for the hospital stay. The mother will be closely monitored during pregnancy and delivery, and any concerns should be immediately addressed by the doctor.

Why squat during a Tet spell?

The hemodynamic change induced by squatting is a sudden rise in arterial pressure and cardiac output, which is known to increase pulmonary blood flow and improve cyanotic spell in patients with tetralogy of Fallot. However, the exact mechanism behind these changes remains unclear. While textbooks suggest that squatting increases left ventricular preload and afterload, no study has demonstrated an increase in afterload.

This study aimed to understand the pulsatile hemodynamic change induced by squatting in twelve healthy young adults, with a mean age of 32 years. The participants had a physical checkup within six months and no abnormalities were found. The study population consisted of twelve healthy young adults, with no medication taken.

What to do if baby has a tet spell?

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.

Why do kids squat during tet spells?

Tetralogy of Fallot (TOF) is a condition characterized by sudden episodes of deep cyanosis, known as “Tet spells”, during crying or feeding. Older children may instinctively squat down to stop the spell. Other symptoms include heart murmur, fussiness, difficulty breathing, rapid heartbeat, fainting, and “clubbing”. The exact cause of TOF is unknown, but it can develop in the early weeks of pregnancy. Some cases may have genetic causes, such as Down syndrome or DiGeorge syndrome.

TOF affects approximately 1 out of every 2, 500 babies born in the United States each year. Babies of mothers who have rubella or other viral illnesses during their pregnancies are at a higher risk for TOF. Other pregnancy-related risks include poor nutrition, alcohol abuse, poorly controlled diabetes, and the mother’s age over 40. Environmental factors like air pollution may also increase a mother’s chances of having a baby with TOF.

Why is morphine in a tet spell?

Tetralogy of Fallot (TOF) patients often experience hypercyanotic spells, which can be exacerbated by agitation, leading to increased sympathetic stimulation on the right ventricular infundibular muscle. Sedation is crucial in managing these episodes to prevent recurrence and ameliorate cyanosis. Morphine is a recommended sedative for TOF patients with hypercyanotic spells, but it can cause vasodilation, reduced cardiac preload, and ventilatory depression, which can worsen the condition.

Dexmedetomidine, a selective α2-adrenergic agonist, has been found to be a better fit for sedation in TOF patients, particularly small infants with hypercyanotic spells. Its features include minimal respiratory depression, vasoconstriction, and inhibition of catecholamine release in the autonomic ganglia. An 8-day-old term newborn patient with TOF was successfully treated with dexmedetomidine, despite the use of phenobarbital suppository.

The patient’s cyanosis was characterized by a long narrowing segment of RV outflow tract, and their arterial saturation (SpO 2) level decreased abruptly to as low as 50 when they became irritable and agitated. Phenobarbital suppository was used, but it failed to produce full sedation and prevent cyanotic spells. After receiving approval from the ethics review committee and parental consent, continuous infusion of dexmedetomidine was started without a loading bolus injection.