Tet spells Tet spells are defined as cyanotic spells occurring due to the obstruction right ventricular outflow. Tet spells can be triggered by various factors such as crying, progressive tachypnea, and deep breathing, with symptoms including but not limited to blue skin, nails and lips, profound crying and difficulty breathing. Tet spells may be treated with beta-blockers such as propranolol, but acute episodes require rapid intervention with morphine or intranasal fentanyl to reduce ventilatory drive, a vasopressor such as phenylephrine, or norepinephrine to increase systemic vascular resistance, and IV fluids for volume expansion. Oxygen (100%) may be effective in treating spells because it is a potent pulmonary vasodilator and systemic vasoconstrictor. This allows more blood flow to the lungs by decreasing shunting of deoxygenated blood from the right to left ventricle through the VSD. There are also simple procedures such as squatting and the knee chest position which increase systemic vascular resistance and decrease right-to-left shunting of deoxygenated blood into the systemic circulation. If the spells are refractory to the above treatments, people are usually intubated and sedated. The treatment of last resort for tet spells is extracorporeal membrane oxygenation (ECMO) along with consideration of Blalock Taussig shunt (BT shunt). Total surgical repair Total surgical repair of<unk>  is a curative surgery. Different techniques can be used in performing<unk>  repair. However, a transatrial, transpulmonary artery approach is used for most cases. The repair consists of two main steps: closure of the VSD with a patch and reconstruction of the right ventricular outflow tract. This open-heart surgery is designed to relieve the right ventricular outflow tract stenosis by careful resection of muscle and to repair the VSD. Additional reparative or reconstructive surgery may be done on patients as required by their particular cardiac anatomy. Timing of surgery in asymptomatic patients is usually between the ages of 2 months to one year. However, in symptomatic patients showing worsening blood oxygen levels, severe tet-spells (cyanotic spells), or dependence on prostaglandins from early neonatal period (to keep the ductus arteriosus open) need to be planned fairly urgently Potential surgical repair complications include residual ventricular septal defect, residual outflow tract obstruction, complete atrioventricular block, arrhythmias, aneurysm of right ventricular outflow patch, and pulmonary valve insufficiency. Long-term complications most commonly include pulmonary valve regurgitation, and arrhythmias. Total repair of<unk>  initially carried a high mortality risk, but this risk has gone down steadily over the years. Surgery is now often carried out in infants one year of age or younger with less than 5% perioperative mortality. Post surgery, most patients enjoy an active life free of symptoms. Currently, long-term survival is close to 90%. Today the adult<unk>  population continues to grow and is one of the most common congenital heart defects seen in adult outpatient clinics. Palliative surgery Initially surgery involved forming a side to end anastomosis between the subclavian artery and the pulmonary artery -i.e. a systemic to pulmonary arterial shunt. This redirected a large portion of the partially oxygenated blood leaving the heart for the body into the lungs, increasing flow through the pulmonary circuit, and relieving symptoms. The first Blalock-Thomas-Taussig shunt surgery was performed on 15-month-old Eileen Saxon on November 29, 1944 with dramatic results. The Potts shunt and the Waterston-Cooley shunt are other shunt procedures which were developed for the same purpose. These are no longer used. Currently, palliative surgery is not normally performed on infants with<unk>  except for extreme cases. For example, in symptomatic infants, a two-stage repair (initial systemic to arterial shunt placement followed by total surgical repair) may be done. Potential complications include inadequate pulmonary blood flow, pulmonary artery distortion, inadequate growth of the pulmonary arteries, and acquired pulmonary atresia. Approaches to surgical repair After years of<unk>  surgical repair expertise, the attention shifted to the emerging evidence that long-term pulmonary insufficiency is detrimental to right ventricular function and clinical prognosis. As a result, the hunt for surgical procedures to relieve right ventricular outflow tract obstruction while minimizing pulmonary regurgitation has intensified. A constrained right ventricular outflow tract reconstruction with a Dacron patch matched to a nominal pulmonary annulus expansion or an annulus-sparing approach yielded primary complete repair outcomes in 94<unk>  infants. The pulmonary annulus size was larger in babies treated with the latter technique, as predicted. After an average follow-up of around 8 years, the first group had a higher than moderate PR, yet there was no significant difference in independence from severe PR after 10 years. Furthermore, there was no significant difference in right ventricular dilation between the two techniques. Finally, they found that reconstructing the pulmonary annulus in<unk>  with only a tiny transannular incision and a stiff Dacron patch to inhibit pulmonary annulus extension throughout the normal growing phase produces the same long-term benefits as preserving the full pulmonary annulus integrity.
Tetralogy of Fallot
Cœur en sabot
Trilogy of Fallot
Double aortic arch
Transposition of the great vessels
Levo-Transposition of the great arteries
Dextro-Transposition of the great arteries
Persistent truncus arteriosus