**11. Management**

#### **11.1. Medical treatment**

Any patient with EA needs to be evaluated regularly by a cardiologist who has expertise in congenital heart disease. Prophylaxis for endocarditis is recommended despite its low risk. In neonates, congestive heart failure or profound cyanosis may be substantial. Medical therapy is the initial and preferred line of treatment. However, failure in weaning from the ventilator or persistent metabolic abnormalities often result in the need for operation. Patients with EA and cardiac failure who are not candidates for surgery are treated with standard cardiac failure therapy, including diuretics and digoxin. The efficacy of angiotensin-converting enzyme inhibitors in the patients with right-sided heart failure is unproved. Medical management of arrhythmias should be individualized and combined with operative or catheter-based intervention.

#### **11.2. Catheter ablation**

Electrophysiological evaluation and radiofrequency ablation of symptomatic accessory pathways should be performed when feasible in EA patients with tachyarrhythmias. Catheter ablation has a lower success rate in patients with an accompanying anomaly than in those with structurally normal hearts, and the risk of recurrence is increased [45, 46]. Supraventricular tachyarrhythmia associated with EA can also be ablated at the time of operative repair [45].

Tachycardia mediated via an accessory pathway is very common. Ten percent of the patients have other mechanisms for their tachyarrhythmias. The pathways are right-sided or septal in the majority of patients with reentrant arrhythmias, with only 4% being left-sided. Thirty percent of the patients may have multiple pathways. Radio-frequency ablation is able to eliminate the tachycardia in almost 90% of cases, depending on its mechanism; however, recurrence is common. Long-term success is achieved in only one-third of the cases [15, 46–48].

#### **11.3. Surgical treatment**

#### *11.3.1. Indications for operation*

Children who have survived infancy generally remain asymptomatic for several years. The surgery can be postponed until symptoms appear, cyanosis becomes evident, or paradoxical emboli occur. Deliberations about an operation should begin if evidence of deterioration exists, such as progressive increase in right heart size, reduction in systolic function, or appearance of ventricular or atrial tachyarrhythmias. Indications for operation in the symptomatic neonates include severe cyanosis, GOSE of three or four with mild cyanosis, cardiothoracic ratio > 80% and severe TR. However, in older ages, operation is clearly indicated if the symptoms progress to New York Heart Association functional class III or IV and medical management has little to offer. A biventricular reconstruction is feasible if the LV is functionally normal, enough RV cavity is present and TV morphology is suitable. A 1.5 ventricle repair can be applied to the patients with the RV failure. Heart transplantation is reserved for the patients with severe biventricular dysfunction.

Some patients with cyanosis on exercise, who have a shunt at the atrial level but only mild or moderate regurgitation of the TV, may benefit from device closure to alleviate cyanosis and to prevent paradoxical emboli. Some centers commonly perform such procedures either as a staged approach or for long-term palliation [50]. The degree of TV regurgitation must be assessed carefully because closure of an ASD alone may worsen RV dysfunction.

#### *11.3.2. Surgical options*

**11. Management**

Sonomed Imaging Center).

146 Structural Insufficiency Anomalies in Cardiac Valves

intervention.

**11.2. Catheter ablation**

**11.1. Medical treatment**

Any patient with EA needs to be evaluated regularly by a cardiologist who has expertise in congenital heart disease. Prophylaxis for endocarditis is recommended despite its low risk. In neonates, congestive heart failure or profound cyanosis may be substantial. Medical therapy is the initial and preferred line of treatment. However, failure in weaning from the ventilator or persistent metabolic abnormalities often result in the need for operation. Patients with EA and cardiac failure who are not candidates for surgery are treated with standard cardiac failure therapy, including diuretics and digoxin. The efficacy of angiotensin-converting enzyme inhibitors in the patients with right-sided heart failure is unproved. Medical management of arrhythmias should be individualized and combined with operative or catheter-based

**Figure 5.** An example of a cardiac MRI showing septal bowing in short axis views. (Used with permission of the

Electrophysiological evaluation and radiofrequency ablation of symptomatic accessory pathways should be performed when feasible in EA patients with tachyarrhythmias. Catheter ablation has a lower success rate in patients with an accompanying anomaly than in those with structurally normal hearts, and the risk of recurrence is increased [45, 46]. Supraventricular tachyarrhythmia associated with EA can also be ablated at the time of operative repair [45]. Tachycardia mediated via an accessory pathway is very common. Ten percent of the patients have other mechanisms for their tachyarrhythmias. The pathways are right-sided or septal in the majority of patients with reentrant arrhythmias, with only 4% being left-sided. Thirty percent of the patients may have multiple pathways. Radio-frequency ablation is able to eliminate A variety of surgical methods were introduced in the treatment of Ebstein's anomaly. Those treatments included a TV repair or replacement for the principle element in the treatment of Ebstein's anomaly and additional concomitant procedures for the correction of comorbid anomalies such as ablation of accessory conduction pathways, resection or plication of the atrialized right ventricle, application of 1.5-ventricular repair (bidirectional cavo-pulmonary shunt), and single-ventricle repair for advanced right ventricular dysfunction. Heart transplantation is suggested for the patients with severe left ventricular dysfunction. Neonatal operation has high operative mortality, whereas operation performed beyond infancy and into adulthood has low operative mortality. Late survival and quality of life for hospital survivors are excellent for the majority of patients in all age brackets [51–54].
