Ebstein Anomaly
Description
● Patient: 28-year-old man who was formerly healthy
➣ Symptoms:
• Palpitations for six weeks, two to three times a week, lasting around thirty minutes
• Shortness of breath, or dyspnoea
➣ Physical examination: • Grade 2/6 holosystolic murmur of tricuspid regurgitation, which is stronger with inspiration and best heard at the left sternal border
• Not noteworthy elsewhere
➣ Examinations:
• ECG: Wolff–Parkinson–White (WPW) pre-excitation pattern (short PR interval, extended QRS, delta waves) with normal sinus rhythm
• Ebstein's abnormality on echocardiography: tricuspid valve apical displacement, "atrialized" right ventricle;
• Leaflets: anterior elongated, rudimentary septal
• Contrast echo: right-to-left shunt in the patent foramen ovale (PFO) (left atrium bubbles)
● Management
• Right-sided accessory pathway radiofrequency ablation
• Electrophysiologic investigation with accessory pathway mapping
• ECG after ablation: aberrant "second" QRS complex (caused by conduction through atrialized RV) + longer PR interval
• Follow-up: The patient reported no palpitations after six months.
➣ About Ebstein's Anomaly:
➣ Large right atrium + "atrialized" section of RV → Apical displacement of septal and posterior tricuspid leaflets → Originally diagnosed in 1864
The right atrium is enlarged, the right ventricle is partially atrialized, and the functional portion is frequently tiny. The prevalence of congenital cardiac disease is around 0.5%, with 5 cases per 100,000 live births.
Risk factors include maternal exposure to benzodiazepines or lithium, family history, and northern European ancestry. Associated heart abnormalities, such as pulmonary stenosis, PFO, or ASD, are observed in over 30% of cases.
➣ Clinical Signs
• Depending on the extent of regurgitation, valve deformity, and related problems
Congestive cardiac failure in infants; cyanosis in newborns, which has a 20% first-year death rate if left untreated
• Adults: Frequently exhibit arrhythmias (palpitations, WPW, etc.);
• Children: Incidental murmur
➣ Factors associated with poor prognosis include:
• Male sex;
• Cardiothoracic ratio > 0.65;
• Cyanosis;
• Young age upon diagnosis
● Care (Personalised for each patient)
➣ Medical: • Mildly impaired heart failure → treated medically
• Pharmacologic therapy for atrial arrhythmias that do not need pre-excitation
Interventional: • Radiofrequency ablation via the symptomatic accessory route (WPW)
➣ Surgery (limited to extreme situations):
• Replacement or repair of the tricuspid valve
• Signs and symptoms include paradoxical embolism, severe cardiac failure, cyanosis, and uncontrollable arrhythmias.
➣ General care:
• Endocarditis prevention is necessary in all situations; regular evaluation for worsening (cyanosis, arrhythmia, decreased functional ability)
Protocol
● Issues Met and Handled by Nursing Interventions:
← Arrhythmias (tachycardia, WPW, palpitations)
➣ Management
• Ablation of the auxiliary route using a radiofrequency catheter
• Antiarrhythmic drugs (beta-blockers, etc.) if necessary
• Ablation frequently paired with surgery if planned
➣Interventions by Nurses
• Maintain a defibrillator and pacing apparatus on hand; keep an eye out for dizziness, syncope, and chest discomfort; and monitor the puncture site, bleeding, and haematoma after the procedure.
• Teach the patient to identify the early signs of an arrhythmia.
← Right Heart Failure with Tricuspid Regurgitation
➣ Management
• Digitalis, diuretics, and supportive heart failure treatment
• Cone surgery or valve replacement
Interventions by Nurses
• Administer diuretics and keep an eye on electrolytes;
• Maintain strict fluid balance (I/O charting);
• Track weight, oedema, and jugular vein pressure;Check for tiredness, orthopnea, and dyspnoea. If necessary, recommend a low-sodium diet and fluid restriction.
← Right-to-Left Shunt/Cyanosis (ASD or PFO)
➣ Treatment: Oxygen treatment as supportive care; surgical closure of ASD/PFO during valve surgery
Interventions by Nurses
Regularly check SpO₂; provide oxygen as directed; and keep an eye out for signs of cyanosis, tachypnea, or changed mental state.Reduce hypoxia by teaching the patient how to handle stress and prevent dehydration.
← Critical and Neonatal Cases
Management includes nitric oxide for pulmonary hypertension, mechanical breathing, inotropes, and prostaglandin E1 infusion, as well as emergency newborn operations (Starnes, shunt, etc.).
Interventions by Nurses
Vital signs, perfusion, and urine output are monitored in the intensive care unit. Oxygen and inotropes are carefully titrated.Keep an eye on the temperature and blood gases.Provide emotional support and knowledge to parents.
← Issues Following Operation or Intervention
➣Management
• ICU haemodynamic monitoring
• Chest tube/drain care and pain management
• Remaining valve dysfunction or arrhythmias
Notes
For more information visit
10.1503/cmaj.050103
