Cyanosis is a rare heart condition that occurs when oxygen-poor blood is pumped through the body, often occurring in sudden spells called tet spells during crying or feeding. This occurs when a baby’s blood oxygen level drops quickly, causing difficulty breathing. The Tetralogy of Fallot (TOF) is a congenital heart defect that affects the heart and blood vessels at birth, causing low oxygen levels in the blood and bluish skin color.
Tet spells can be triggered by any event that slightly decreases oxygen saturation (e.g., crying, defecating) or that suddenly decreases systemic vascular resistance (e.g., playing, kicking legs when awakening). Pink Tets can later develop cyanosis and hypercyanotic spells when crying or in hypovolemia. Patients with TOF and absent pulmonary valves can also experience tet spells, which are episodes of intense blueness with deeper and faster breathing.
Tet spells are most common between 2 and 4 months of age, and they occur more often in older children who have experienced tet spells. Activities that change the pressure in the baby’s heart and increase the flow of oxygen-poor blood to their body, such as crying, pooping, or kicking their legs after waking up, can cause tet spells. The way crying/defecating worsens tet spells is likely due to higher negative intrathoaracic pressures generated by the events.
Tet spells can be spontaneous or triggered by certain events such as prolonged crying and episodes of exertion, such as during feeding or pooing. Illness usually causes tet spells, which are caused by a rapid drop in the baby’s blood oxygen level. Hypercyanotic spells are characterised by periods of uncontrollable crying/irritability, hyperpnoea (rapid, deep respiration), and can present with respiratory distress and worsening cyanosis. Common precipitants include crying, defecation, feeding, waking from naps (low systemic resistance), fever, dehydration, and tachypnoea.
In conclusion, tet spells are a common symptom of tetralogy of fallot, a rare heart condition that can lead to cyanosis and hypercyanotic spells.
📹 Tetralogy of Fallot (Year of the Zebra)
What is Tetralogy of Fallot? Tetralogy of Fallot (TOF) is a congenital heart condition characterized by four heart …
Why does being sad trigger tears?
Emotional tears, which are produced during a crying jag, contain additional proteins and hormones like prolactin, potassium, manganese, and stress hormones. They are believed to release stress to calm the body down. The limbic system in the brain activates lacrimal glands to produce tears, causing a full-on cry-fest. Emotional tears have an evolutionary purpose of gaining empathy, and research shows that people who receive social support when they shed tears report feeling better than those who hold back their tears or lack support.
As people age, their lacrimal glands’ production of water slows down, making them more susceptible to dry eye, which can make eyes feel painful and impact vision. Blepharitis, a condition causing eyelid irritation and swelling, can also be caused by inflammation in meibomian glands. To treat dry eye, patients should apply a warm compress to their eyes for 15-20 seconds a day, similar to flossing. Artificial tears and an omega 3 fatty acid supplement may also help. Eye doctors can help find solutions for these conditions.
What causes cyanotic spells?
Cyanotic spells may be precipitated by a number of factors, including crying, pain, feeding, bowel movements, dehydration, and elevated temperatures. In some cases, these spells may manifest immediately following the infant’s waking. It is imperative that additional caution be exercised when caring for infants with cyanotic heart defects during periods of elevated temperatures.
Why do tet spells cause cyanosis?
Infundibular muscle spasm, often during distress, can cause dynamic RVOTO, increasing the right-to-left-shunt fraction and leading to profound hypoxaemia, known as ‘cyanotic spells’. Tetralogy of Fallot, a common cyanotic heart defect, is characterized by a ventricular septal defect, overriding aorta, right ventricular outflow tract obstruction, and right ventricular hypertrophy. This sudden oxygen desaturation is often referred to as ‘cyanotic spells’.
Why do kids squat during a tet spell?
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.
How does the tetralogy of Fallot develop?
Tetralogy of Fallot (TOF) is a congenital heart defect where a baby’s heart doesn’t form correctly in the womb. The cause is unknown, but it may be due to gene or chromosome changes. Children with certain genetic syndromes, environmental factors like smoking or certain medications during pregnancy, family history, and certain medical conditions during pregnancy may also increase the risk of TOF.
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.
What is the physiology of a cyanotic spell?
Cyanotic spells represent a critical pediatric emergency, necessitating immediate recognition and treatment. This is due to the decreased pulmonary blood flow and right-to-left shunting that can occur in any heart condition involving a ventricular septal defect (VSD) and restricted pulmonary blood flow.
What is the pathophysiology of tetralogy?
The ventricular septal defect in tetralogy of Fallot is a malalignment type, where the conal septum is displaced anteriorly, causing obstruction and hypoplasia of downstream structures like the pulmonary valve, main pulmonary artery, and branch pulmonary arteries. The defect is typically large, resulting in the same systolic pressures in the right and left ventricles. The pathophysiology depends on the degree of right ventricular outflow obstruction, with mild obstruction causing a left-to-right shunt, while severe obstruction causes a right-to-left shunt, resulting in low systemic arterial saturation (cyanosis) unresponsive to supplemental oxygen.
In some children with unrepaired tetralogy of Fallot, sudden episodes of profound cyanosis and hypoxia (hypercyanotic or “tet” spells) may occur, potentially lethal. The mechanism of a hypercyanotic spell is unclear, but several factors are likely important in causing an increase in right-to-left shunting and a fall in arterial oxygen saturation.
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 is the most frequent defect leading to tetralogy of Fallot?
The heart’s septum, an inner wall that separates the two chambers, prevents blood mixing between them. A ventricular septal defect, a hole in the septum, causes oxygen-rich and oxygen-poor blood to mix. This defect narrows the pulmonary valve, causing oxygen-poor blood to enter the pulmonary artery and travel to the lungs. Pulmonary stenosis occurs when the valve cannot open fully, causing the heart to work harder and causing blood to be unable to reach the lungs. The right ventricle muscle thickens due to excessive blood flow.
What triggers a tet spell?
A Tet Spell can be precipitated by a number of factors, including crying, bowel movements, waking from a period of rest, the onset of fever, immersion in water, psychological distress, and dehydration, which can result in a reduction in blood flow to the lungs.
📹 Breath holding spell 21-3-10
Kai 14 months 7.08pm trigger lightly bumped head on chair, did not appear bite his tongue. Very knocked around from this one, …
such an insightful article. I have heard that TOF is somehow correlated with DiGeorge and Chromosome 22 deletions and was wondering if you could explain more about those conncetions. Or, are there articles on it too? To recapitulate the pathophysiology of TOF, the pressure difference reverses because it somehow gets too high in the right ventricle, shunting back into the left ventricle, resulting in some quantity that may vary in severity levels of deoxygenated blood pumping out through the left ventricle –> aorta –> systemic circulation, sometimes (in approx what proportion of cases?) results in cyanosis or blue-ish discoloration due to lacking adequate oxygenation of systemic body tissues if i follow you correctly. and, how exactly can squatting can help reverse the condition by elevating systemic pressure and left ventricular pressure because if the RV pressure is high, I do not see how squatting would equilibrate only the LV and not travel through the heart, increased opening or dilatation between the RV and LV
this article is amazing! i have an assignment where i have to explain Tetralogy of Fallot. this helps me so much, its calmly explained ( a very nice voice to listen to) with very well fitted animations, as a person with ADHD its easy to focus on whats being said because of this. thank you for explaining this congenital heart condition so well!!!