Congenital Heart Surgery

The normal heart has four chambers, four valves and two great arteries arranged so that blood low in oxygen goes to the lungs and oxygen-rich blood goes to the body. In a congenital heart defect, part of this arrangement formed differently before birth. Surgery aims either to restore normal anatomy, known as a complete repair, or, when that is not possible, to reroute the circulation so the body still receives enough oxygen. Common operations include closing a ventricular or atrial septal defect with a patch, complete repair of tetralogy of Fallot, the arterial switch operation for transposition of the great arteries, repair of a narrowed aorta, called coarctation, and valve repair or replacement. When only one pumping chamber is usable, a series of operations, often ending with the Fontan procedure, directs blood from the body straight to the lungs. Many simpler defects can now be closed with catheter devices instead of surgery.

The timing and type of surgery depend on the defect. Some newborns need an operation within days or weeks of birth, because their circulation depends on a vessel that naturally closes or because oxygen levels are dangerously low. Other children are operated on in the first months or years, when a defect causes breathlessness, poor feeding, slow weight gain, repeated chest infections or rising pressure in the lungs. Small defects may never need treatment and are only monitored. In adults, surgery is considered when an earlier repair develops problems, such as a leaking pulmonary valve after tetralogy of Fallot repair, a narrowing that has returned or a struggling Fontan circulation, or when a defect is found late, for example an atrial septal defect causing breathlessness or rhythm problems. The decision is made jointly by cardiologists and surgeons after reviewing echocardiograms, MRI or CT scans and, sometimes, catheter measurements.

The operation is done under general anaesthesia with a breathing tube, careful monitoring lines and, for most open repairs, cardiopulmonary bypass. After an incision through the breastbone, or occasionally between the ribs, the heart-lung machine takes over pumping and oxygenating the blood, and the heart is stopped with a cold protective solution so the surgeon can work inside it. Holes are closed with patches, narrowed valves or vessels are opened or enlarged, abnormal connections are rerouted and, where needed, a valve or a tube called a conduit is implanted. The heart is then restarted and the bypass machine gradually stopped. Some operations, such as coarctation repair or a shunt placed to improve blood flow to the lungs, can be done without bypass. An ultrasound probe in the food pipe, called transoesophageal echocardiography, checks the repair before the chest is closed. Operations commonly take about 3 to 6 hours.

Every patient is assessed by the congenital heart team, usually with an echocardiogram, an ECG, a chest X-ray, blood tests and often an MRI or CT scan; some need a cardiac catheterisation first to measure pressures or to treat part of the problem. The team considers the exact anatomy, lung pressures, other organ problems, genetic conditions and, for babies, weight and nutrition. Colds, chest infections and dental infections are treated before surgery, because an infection can delay the operation or affect the heart lining later. For children, preparation includes explaining the hospital stay in age-appropriate words and arranging for a parent to stay close by. Adults with congenital heart disease should bring records of every earlier operation, since scar tissue and previous repairs shape the plan. For international families, scans, operation notes and reports can usually be reviewed remotely, so the surgical plan and expected stay are discussed before travel.

After surgery, patients go to a cardiac intensive care unit, where breathing support, drains and medicines are gradually withdrawn; this often takes a few days, longer for newborns and complex repairs. They then move to a ward, start eating, become mobile and receive physiotherapy. A total hospital stay of about one to three weeks is common, but some newborns and children with complex conditions stay much longer. The breastbone takes about six to eight weeks to heal, so heavy lifting, rough play and contact sports are avoided during that time. International families should plan to stay near the hospital for a few weeks after discharge: flying is usually allowed only after the cardiology team gives clearance, often a few weeks after surgery, once a check-up echocardiogram is satisfactory. Take home a full discharge summary, a medication plan and scan images, and arrange follow-up with a congenital cardiologist near home before you leave.

Congenital heart surgery is major surgery, and the level of risk varies widely with the type of defect, the age and size of the patient, other health problems and whether it is a first or repeat operation. Your team can explain what this means for your child or for you. Possible complications include bleeding, infection of the wound or blood, heart rhythm problems including heart block that may need a pacemaker, fluid around the heart or lungs, strain on the kidneys, stroke and, rarely, death. Many children who have a simple defect repaired go on to live full, active lives with little or no restriction. After more complex repairs, further operations or catheter procedures are often expected later, for example to replace a conduit or valve as a child grows. For this reason, lifelong follow-up with a congenital heart specialist is recommended for most people, including adults who feel well, so that problems are found early.