In mild cases, no treatment is needed, although the infant should be closely followed to make sure that the hole closes properly as he grows. Large defects usually require early surgical correction before heart failure and irreversible pulmonary vascular disease develop.

For small defects, surgery consists of simple suture closure. Moderate to large defects require insertion of a patch graft, using cardiopulmonary bypass. In patients with heart failure, digoxin and diuretics may be prescribed to control symptoms. In patients who develop increased pulmonary resistance and irreversible pulmonary vascular changes that produce a reversible right-to-left shunt (Eisenmenger’s syndrome), a heart-lung transplant may be required.

If the child has other defects and will benefit from delaying surgery, pulmonary artery banding normalizes pressures and flow distal to the band and prevents pulmonary vascular disease, allowing postponement of surgery. (Pulmonary artery banding is done only when the child has other complications.) A rare complication of VSD repair is complete heart block from interference with the bundle of His during surgery. (Heart block may require temporary or permanent pacemaker implantation.)

Before surgery, treatment consists of

Generally, postoperative treatment includes a brief period of mechanical ventilation. The patient will need analgesics and may also require diuretics to increase urine output, continuous infusions of nitroprusside or adrenergic agents to regulate blood pressure and cardiac output and, in rare cases, a temporary pacemaker.

Operative repair is advised for all patients with uncomplicated ASD with evidence of significant left-to-right shunting. Ideally, this is performed when the patient is between ages 2 and 4. Operative treatment shouldn’t be performed on patients with small defects and trivial left-to-right shunts. Because ASD seldom produces complications in infants and toddlers, surgery can be delayed until they reach preschool or early school age. A large defect may need immediate surgical closure with sutures or a patch graft.

Physicians have developed a new procedure, referred to as catheter closure or transcatheter closure of the atrial septal defect, that uses wires or catheters to close ASD without surgery. In this procedure, the surgeon makes a tiny incision in the groin to introduce the catheters. Then, he advances the catheters into the heart and places the closure device across the ASD. This procedure may not be applicable to all patients.

For an infant with heart failure caused by coarctation of the aorta, treatment consists of medical management with digoxin, diuretics, oxygen, and sedatives. If medical management fails, surgery may be needed.

The child’s condition usually determines the timing of surgery. Signs of heart failure or hypertension may call for early surgery. If these signs don’t appear, surgery usually occurs during the preschool years.

Before the operation, the child may require endocarditis prophylaxis or, if he’s older and has previously undetected coarctation, antihypertensive therapy. During surgery, the surgeon uses a flap of the left subclavian artery to reconstruct an unobstructed aorta.

Balloon therapy may be indicated for some patients as an alternative to surgical repair. It uses a technique similar to that used to open the coronary arteries, but is performed on the aorta.

Asymptomatic infants with PDA require no immediate treatment. Those with heart failure require fluid restriction, diuretics, and cardiac glycosides to minimize or control symptoms. If these measures can’t control heart failure, surgery is necessary to ligate the ductus. If symptoms are mild, surgical correction is usually delayed until the infant is between ages 6 months and 3 years, unless problems develop. Before surgery, children with PDA require antibiotics to protect against infective endocarditis. Other forms of therapy include cardiac catheterization to deposit a plug or coil in the ductus to stop shunting or administration of indomethacin I.V. (a prostaglandin inhibitor that’s an alternative to surgery in premature neonates) to induce ductus spasm and closure.

Effective management of tetralogy of Fallot necessitates prevention and treatment of complications, measures to relieve cyanosis, and palliative or corrective surgery. During cyanotic spells, the knee-chest position and administration of oxygen and morphine improve oxygenation. Propranolol (a beta-adrenergic blocking agent) may prevent blue spells.

Palliative surgery is performed on infants with potentially fatal hypoxic spells. The goal of surgery is to enhance blood flow to the lungs to reduce hypoxia; this is often accomplished by joining the subclavian artery to the pulmonary artery (Blalock-Taussig procedure). Supportive measures include prophylactic antibiotics to prevent infective endocarditis or cerebral abscess administered before, during, and after bowel, bladder, or any other surgery or dental treatments. Management may also include phlebotomy in children with polycythemia.

Complete corrective surgery to relieve pulmonary stenosis and close the VSD, directing left ventricular outflow to the aorta, requires cardiopulmonary bypass with hypothermia to decrease oxygen utilization during surgery, especially in young children. An infant may have this corrective surgery without prior palliative surgery. It’s usually done when progressive hypoxia and polycythemia impair the quality of his life, rather than at a specific age. However, most children require surgery before they reach school age.

An infant with transposition may undergo atrial balloon septostomy (Rashkind procedure) during cardiac catheterization. This procedure enlarges the patent foramen ovale, which improves oxygenation by allowing greater mixing of the pulmonary and systemic circulations. Atrial balloon septostomy requires passage of a balloon-tipped catheter through the foramen ovale and subsequent inflation and withdrawal across the atrial septum. This procedure alleviates hypoxia to a certain degree. Afterward, digoxin and diuretics can lessen heart failure until the infant is ready to withstand corrective surgery (usually between birth and age 1).

One of three surgical procedures can correct transposition, depending on the defect’s physiology. The Mustard procedure replaces the atrial septum with a Dacron or pericardial partition that allows systemic venous blood to be channeled to the pulmonary artery—which carries the blood to the lungs for oxygenation— and oxygenated blood returning to the heart to be channeled from the pulmonary veins into the aorta. The Senning procedure accomplishes the same result, using the atrial septum to create partitions to redirect blood flow. In the arterial switch, or Jantene procedure, transposed arteries are surgically anastomosed to the correct ventricle. For this procedure to be successful, the left ventricle must be used to pump at systemic pressure, as it does in neonates or in children with a left ventricular outflow obstruction or a large VSD. The Jantene procedure is the procedure of choice; however, the Mustard and Senning procedures may be used when specific anatomic conditions exist.

Treatment includes antibiotics for bacterial infection, modified bed rest to decrease cardiac workload, and careful management of complications. Inotropic support of cardiac function with amrinone, dopamine, or dobutamine may be needed. Heart failure requires restriction of activity to minimize myocardial oxygen consumption, supplemental oxygen therapy, sodium restriction, diuretics to decrease fluid retention, and cardiac glycosides to increase myocardial contractility. However, cardiac glycosides should be administered cautiously because some patients with myocarditis may show a paradoxical sensitivity to even small doses. Arrhythmias necessitate prompt but cautious administration of antiarrhythmics because these drugs depress myocardial contractility. Thromboembolism requires anticoagulation therapy. Treatment with corticosteroids or other immunosuppressants may be used to reduce inflammation, but they haven’t been shown to change the progression of myocarditis. Nonsteroidal anti-inflammatory drugs are contraindicated during the acute phase (first 2 weeks) because they increase myocardial damage.

Surgical treatment may include left ventricular assistive devices and extracorporeal membrane oxygenation for support of cardiogenic shock. Cardiac transplantation has been beneficial for giant cell myocarditis.

The goal of treatment is to eradicate the infecting organism with appropriate antimicrobial therapy, which should start promptly and continue over 4 to 6 weeks. Selection of an antibiotic is based on identification of the infecting organism and on sensitivity studies. While awaiting results, or if blood cultures are negative, empiric antimicrobial therapy is based on the likely infecting organism.

Supportive treatment includes bed rest, aspirin for fever and aches, and sufficient fluid intake. Severe valvular damage, especially aortic or mitral insufficiency, may require corrective surgery if refractory heart failure develops, or in cases requiring that an infected prosthetic valve be replaced.

The goal of treatment is to relieve symptoms and manage the underlying systemic disease. In acute idiopathic pericarditis and postthoracotomy pericarditis, treatment consists of bed rest as long as fever and pain persist, and nonsteroidal drugs, such as aspirin and indomethacin, to relieve pain and reduce inflammation. Post-MI patients should avoid nonsteroidal anti-inflammatory drugs and steroids because they may interfere with myocardial scar formation. If these drugs fail to relieve symptoms, corticosteroids may be used. Although corticosteroids produce rapid and effective relief, they must be used cautiously because episodes may recur when therapy is discontinued.

Infectious pericarditis that results from disease of the left pleural space, mediastinal abscesses, or septicemia requires antibiotics (possibly by direct pericardial injection), surgical drainage, or both. Cardiac tamponade may
require pericardiocentesis. Signs of tamponade include pulsus paradoxus, jugular vein distention, dyspnea, and shock.

Recurrent pericarditis may necessitate partial pericardectomy, which creates a “window” that allows fluid to drain into the pleural space. In constrictive pericarditis, total pericardectomy to permit adequate filling and contraction of the heart may be necessary. Treatment must also include management of rheumatic fever, uremia, tuberculosis, and other underlying disorders.