Paediatric Heart Surgery

What Parents Need to Know After a TOF Diagnosis: Tetralogy of Fallot — TOF — is the most common cyanotic congenital heart defect, and a diagnosis that most parents receive with no preparation and very little information about what comes next. If your baby has just been diagnosed with TOF, this guide explains what it means, what symptoms to watch for at home, what surgery involves, and — critically — what your child’s life looks like after repair. Dr. Ved Prakash, Director of CTVS at Yatharth Super Speciality Hospitals, Greater Noida, has performed TOF repairs from early infancy across Medanta, Narayana, and Yatharth Hospital. What Is Tetralogy of Fallot? Tetralogy of Fallot is four structural abnormalities that occur together in the same heart. Understanding all four is important because they interact — each one making the others worse: A large VSD (ventricular septal defect) — a large hole between the two lower chambers, allowing blue (deoxygenated) blood to mix with red (oxygenated) blood. Pulmonary stenosis — narrowing of the outflow from the right ventricle to the lungs. This obstruction means less blood reaches the lungs to pick up oxygen, and more blue blood is pushed through the VSD to the body instead. Overriding aorta — the aorta sits directly over the VSD rather than exclusively over the left ventricle, receiving a mix of blue and red blood from both chambers. Right ventricular hypertrophy — the right ventricle thickens because it is pumping against the obstructed pulmonary outflow. This worsens over time if repair is delayed. The net result: the body continuously receives blood that has not been fully oxygenated — causing the characteristic blue discolouration (cyanosis) of Tetralogy of Fallot. Tetralogy of Fallot Symptoms — What Parents See Cyanosis — The Blue Baby Appearance Blue or purple discolouration of the lips, tongue, fingernails, and toenails. In some babies with TOF, cyanosis is obvious at birth. In others — particularly where the pulmonary stenosis is mild — cyanosis develops gradually in the first weeks of life as the ductus arteriosus (a foetal blood vessel that keeps oxygen supply adequate in the womb) closes after birth. A baby who was pink at birth and becomes increasingly blue in the first month needs urgent cardiac assessment. Tet Spells — The Most Frightening Symptom A tet spell is a hypercyanotic episode — a sudden, dramatic worsening of cyanosis triggered by anything that decreases pulmonary blood flow: crying, feeding, defecation, waking from sleep, or even nothing obvious. During a tet spell the baby turns intensely blue, becomes extremely distressed and inconsolable, breathes very fast, and may go limp or briefly lose consciousness from cerebral hypoxia. Tet spells are caused by spasm of the right ventricular outflow tract — the already-narrowed channel from the right ventricle to the lungs temporarily clamps shut, redirecting almost all right-sided output through the VSD to the body. Almost no blood reaches the lungs. Oxygen saturation plummets. If your baby has a tet spell: pull the knees firmly up to the chest (squatting position for an infant), keep the baby calm, and call emergency services immediately. Tet spells require hospital assessment and accelerate the need for surgery. Do not wait to see if it settles. Squatting in Older Unrepaired Children Older children with unrepaired or partially palliated TOF instinctively squat after physical activity. Squatting compresses the femoral arteries — increasing systemic vascular resistance and reducing the right-to-left shunt through the VSD — temporarily pushing more blood to the lungs and relieving cyanosis. It is one of the most specific clinical signs in all of paediatric cardiology. Other Signs Poor growth and weight gain — the body cannot support normal growth when oxygen delivery is chronically reduced Exercise intolerance in older unrepaired children — breathlessness and cyanosis with minimal exertion Clubbing of fingers and toes — develops after months of chronic cyanosis How Tetralogy of Fallot Is Diagnosed Foetal echocardiogram: TOF is detectable at the 18–20 week anomaly scan in many cases — allowing planned delivery at a centre with paediatric cardiac surgery capability. Neonatal echocardiogram: Confirms the anatomy — size of the VSD, degree of pulmonary stenosis, position of the aorta, and right ventricular size. Pulse oximetry screening: Low oxygen saturation detected on routine newborn screening triggers further investigation. CT cardiac angiography: Used before surgery when the pulmonary artery anatomy needs detailed 3D mapping — particularly important for the branch pulmonary arteries which can be hypoplastic in severe TOF. Tetralogy of Fallot Surgery — What Is Done Complete surgical repair of TOF is performed under general anaesthesia and cardiopulmonary bypass. The operation addresses all the abnormalities simultaneously: VSD closure: The hole between the ventricles is closed with a patch — stopping the mixing of blue and red blood completely. Relief of pulmonary stenosis: The narrowed right ventricular outflow tract is widened by resecting obstructing muscle bundles, and often by placing a patch across the outflow tract or pulmonary valve to enlarge it. If the pulmonary valve itself is severely abnormal it may be removed — leaving the outflow tract open (transannular patch). This effectively relieves obstruction but results in pulmonary regurgitation. Result: Blue blood now goes through the pulmonary artery to the lungs normally. Oxygenated blood returns and goes through the aorta to the body. Cyanosis resolves. Surgery is typically performed between 3 and 6 months of age. If tet spells are occurring before this age, surgery is brought forward without delay. Results at experienced centres: operative mortality below 2–3%, complete repair achieved in virtually all cases. Life After TOF Surgery — The Honest Picture This is what parents most need to understand — and what most articles do not explain. The good news: The vast majority of children repaired in infancy grow up completely normally. They attend mainstream school, participate in sport, develop normally in every way. Most go on to live independent adult lives — working, driving, having children of their own. What requires lifelong monitoring: Annual echocardiogram — monitoring right ventricular size and function over time Pulmonary

ASD — Atrial Septal Defect — is a hole in the wall between the two upper chambers of the heart, and it is one of the most common congenital heart defects found in children and adults. The good news: most ASDs can be closed completely, either with a simple catheter-based device or with surgery — and the outcomes are excellent. Dr. Ved Prakash, Director of CTVS at Yatharth Super Speciality Hospitals, Greater Noida, explains what ASD means, when treatment is needed, and what the safest, most effective option is for your child or family member. What Is ASD — Atrial Septal Defect? The heart is divided into four chambers — two upper chambers (atria) and two lower chambers (ventricles). The wall dividing the two atria is called the atrial septum. In a normal heart, this wall is completely sealed and blood cannot cross from one side to the other at this level. An ASD is a hole in this wall. Blood flows through the hole from the left atrium (where oxygenated blood returns from the lungs) to the right atrium (where de-oxygenated blood collects before going to the lungs) — because the pressure on the left side is slightly higher. This means the right side of the heart receives more blood than it should, and more blood is sent to the lungs with each heartbeat. Over years, this extra volume causes the right side of the heart to enlarge and, in severe cases, raises the blood pressure in the lungs (pulmonary hypertension) — which can become irreversible if the ASD is left unclosed for too long. Types of ASD Ostium secundum ASD: The most common type — a hole in the central part of the atrial septum. Most suitable for catheter-based device closure. Ostium primum ASD: Located in the lower part of the septum, near the heart valves. Almost always requires surgical repair — device closure is not possible. Sinus venosus ASD: Near the junction of the atria and the large veins entering the heart. Requires surgical repair. Patent Foramen Ovale (PFO): A small flap-like opening that all foetuses have and that normally closes after birth. A PFO is not a true ASD but can cause problems in some adults — particularly cryptogenic stroke. ASD Symptoms — What to Watch For Small ASDs often cause no symptoms at all — particularly in children, who compensate remarkably well. Larger ASDs may cause: Frequent respiratory infections in infancy — bronchitis or pneumonia more often than peers Reduced exercise tolerance in older children — tiring faster than classmates Breathlessness on exertion — particularly as the child grows and demands on the heart increase Heart palpitations — particularly in adults with undiagnosed ASD, often from atrial fibrillation Stroke in younger adults — a blood clot can cross from the right to the left side through a large ASD and travel to the brain Many ASDs are first discovered when a routine examination reveals a heart murmur — or when an echocardiogram is performed for another reason. Some are diagnosed in adulthood when the patient presents with atrial fibrillation or breathlessness and the cause is traced back to a previously unknown ASD. How Is ASD Diagnosed? Echocardiogram: The primary investigation — shows the location and size of the ASD, the direction of blood flow through it, and the degree of right heart enlargement Bubble echocardiogram: Agitated saline injected into a vein — bubbles crossing from right to left confirms an opening Cardiac MRI: Useful for precise assessment of the shunt volume and right heart dimensions when planning intervention ECG: May show right heart enlargement patterns When Does an ASD Need Treatment? If the ASD is significant enough to cause right heart enlargement on echocardiogram — even without symptoms If symptoms are present (breathlessness, reduced exercise tolerance, palpitations) If pulmonary blood flow is significantly elevated (Qp:Qs ratio greater than 1.5:1) If the ASD is causing atrial fibrillation Small ASDs (less than 5mm) in infants often close spontaneously by age 2–3. Larger ASDs do not close on their own and require intervention — ideally before school age if possible, though the procedure is safe at any age. Can ASD Be Closed Without Open-Heart Surgery? Yes — for the right anatomy. This is one of the most common questions parents ask. Device Closure (Catheter-Based — No Surgery) For ostium secundum ASDs of appropriate size and position, a device is delivered through a vein in the leg (femoral vein) using a catheter. The device — which looks like a small double-disc — is positioned across the hole and deployed to plug it. The procedure is performed under general anaesthesia and guided by echocardiography. The child typically goes home the next day. Suitable for: Centrally located ostium secundum ASDs with adequate tissue rims around the defect. Confirmed by echocardiogram during assessment. Surgical ASD Closure (Open-Heart Surgery) For primum ASDs, sinus venosus ASDs, and secundum ASDs that are too large or in an unsuitable position for device closure, surgical repair is performed. A patch of pericardium (the heart’s own lining) or a synthetic patch is sewn over the hole. The results are permanent and excellent — success rates exceed 99%. Device Closure Surgical Closure Incision needed? No Yes — chest incision Hospital stay 1–2 days 5–7 days Recovery 1 week 4–6 weeks Suitable for all ASD types? No — only suitable ostium secundum Yes — all types Success rate Excellent (>98%) Excellent (>99%) Frequently Asked Questions — ASD Heart Defect What is ASD and is it dangerous? ASD (Atrial Septal Defect) is a hole between the two upper chambers of the heart. Small ASDs may be harmless and close on their own. Larger ASDs, if left untreated for years, can cause right heart enlargement, pulmonary hypertension, atrial fibrillation, and stroke. Treatment before these complications develop produces excellent long-term outcomes. Will a small ASD close on its own? Small ostium secundum ASDs (less than 5mm) often close spontaneously by age 2–3. ASDs detected at birth are monitored with serial echocardiograms. Larger