Ebstein anomaly treatment in 94 Cardiac surgery clinics in Europe

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94 clinics specializing in Cardiac surgery providing treatment of Ebstein anomaly Ebstein anomaly is a rare congenital heart defect where the tricuspid valve is malformed and displaced into the right ventricle. It can cause blood flow problems and may require surgical intervention for correction and improved cardiac function.
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disease in Europe.

Albania · 1
Austria · 5
Belarus · 1
Belgium · 2
Bulgaria · 1
Croatia · 1
Denmark · 1
Finland · 1
France · 7
Germany · 18
Greece · 2
Hungary · 2
Ireland · 3
Italy · 7
Latvia · 1
Moldova · 1
Monaco · 1
Poland · 1
Portugal · 7
Romania · 2
Russia · 6
Slovakia · 1
Spain · 7
Ukraine · 1
You can select a specific country
Albania · 1 Austria · 5 Belarus · 1 Belgium · 2 Bosnia and Herzegovina · 1 Bulgaria · 1 Croatia · 1 Czech Republic · 2 Denmark · 1 Finland · 1 France · 7 Germany · 18 Greece · 2 Hungary · 2 Ireland · 3 Italy · 7 Latvia · 1 Moldova · 1 Monaco · 1 Netherlands · 4 Poland · 1 Portugal · 7 Romania · 2 Russia · 6 Slovakia · 1 Spain · 7 Switzerland · 3 Ukraine · 1 United Kingdom · 4
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Prices for popular procedures:
  1. Ebstein anomaly correction
    ≈ $21,426
  2. Fetal cardiac interventions
    by request
  3. Dual chamber pacemaker insertion
    ≈ $9,412
  4. Coronary artery bypass graft (CABG)
    ≈ $27,609
  5. Heart valve replacement
    ≈ $30,820
  6. Heart valve repair
    ≈ $29,400
  7. Aortic valve replacement (AVR)
    ≈ $38,744
  8. Mitral valve replacement (MVR)
    ≈ $26,153
  9. Transcatheter aortic valve replacement (TAVR)
    ≈ $25,888
photo
Madrid, Spain
Specializations: Cardiac surgery, Vascular surgery, Thoracic surgery, Neurosurgery, Spine surgery, Orthopedic surgery, Oncology
We are a public hospital belonging to the Madrid Health Service, which stands out for the high training and qualification of its professionals, for its read more
Prices for popular procedures:
  1. Ebstein anomaly correction
    ≈ $24,839
  2. Dual chamber pacemaker insertion
    ≈ $13,355
  3. Coronary artery bypass graft (CABG)
    ≈ $41,886
  4. Heart valve replacement
    ≈ $48,647
  5. Heart valve repair
    ≈ $41,127
  6. Aortic valve replacement (AVR)
    ≈ $54,325
  7. Mitral valve replacement (MVR)
    ≈ $39,984
  8. Transcatheter aortic valve replacement (TAVR)
    ≈ $70,119
  9. Off-pump coronary artery bypass surgery
    ≈ $30,254
photo
Innsbruck, Austria
Specializations: Cardiac surgery, Vascular surgery, Thoracic surgery, Neurosurgery, Orthopedic surgery, Oncology
The University Hospital of Innsbruck provides research and teaching combined with patient care at the highest level. The cooperation of the Medical University of Innsbruck read more
Prices for popular procedures:
  1. Ebstein anomaly correction
    ≈ $24,839
  2. Dual chamber pacemaker insertion
    ≈ $13,355
  3. Coronary artery bypass graft (CABG)
    ≈ $41,886
  4. Heart valve replacement
    ≈ $48,647
  5. Heart valve repair
    ≈ $41,127
  6. Aortic valve replacement (AVR)
    ≈ $54,325
  7. Mitral valve replacement (MVR)
    ≈ $39,984
  8. Transcatheter aortic valve replacement (TAVR)
    ≈ $70,119
  9. Off-pump coronary artery bypass surgery
    ≈ $30,254
photo
Graz, Austria
Specializations: Cardiac surgery, Vascular surgery, Thoracic surgery, Neurosurgery, Spine surgery, Orthopedic surgery, Oncology
The LKH-Universitätsklinikum Graz is – along with Innsbruck and Vienna – one of the three public university hospitals in Austria. Since 2002, the state hospital read more
Prices for popular procedures:
  1. Ebstein anomaly correction
    ≈ $24,839
  2. Coronary artery bypass graft (CABG)
    ≈ $41,886
  3. Heart valve replacement
    ≈ $48,647
  4. Heart valve repair
    ≈ $41,127
  5. Aortic valve replacement (AVR)
    ≈ $54,325
  6. Mitral valve replacement (MVR)
    ≈ $39,984
  7. Transcatheter aortic valve replacement (TAVR)
    ≈ $70,119
  8. Off-pump coronary artery bypass surgery
    ≈ $30,254
  9. Fontan procedure
    ≈ $28,478
photo
Linz, Austria
Specializations: Cardiac surgery, Vascular surgery, Thoracic surgery, Neurosurgery, Spine surgery, Orthopedic surgery, Oncology
The Kepler University Hospital was founded on December 31, 2015 with the merger of the three renowned and traditional hospitals: General Hospital of the City read more
Prices for popular procedures:
  1. Ebstein anomaly correction
    ≈ $18,326
  2. Dual chamber pacemaker insertion
    ≈ $6,977
  3. Coronary artery bypass graft (CABG)
    ≈ $18,382
  4. Off-pump coronary artery bypass surgery
    ≈ $16,533
  5. Fontan procedure
    ≈ $18,199
  6. Permanent pacemaker implantation
    ≈ $6,619
  7. Radiofrequency ablation (RFA)
    ≈ $16,520
  8. Cardiac catheterization
    ≈ $2,126
  9. Percutaneous ASD closure
    ≈ $7,829
photo
Grodzisk Mazowiecki, Poland
Specializations: Cardiac surgery, Vascular surgery, Thoracic surgery, Neurosurgery, Spine surgery, Orthopedic surgery, Oncology
Our top priority is to provide the highest quality medical services. Since the very beginning this had been guaranteed by state of the art equipment read more
Prices for popular procedures:
  1. Ebstein anomaly correction
    ≈ $14,826
  2. Dual chamber pacemaker insertion
    ≈ $7,252
  3. Coronary artery bypass graft (CABG)
    ≈ $5,792
  4. Heart valve replacement
    ≈ $21,783
  5. Heart valve repair
    ≈ $20,181
  6. Aortic valve replacement (AVR)
    ≈ $20,853
  7. Mitral valve replacement (MVR)
    ≈ $14,930
  8. Transcatheter aortic valve replacement (TAVR)
    ≈ $29,964
  9. Off-pump coronary artery bypass surgery
    ≈ $17,165
photo
Prague, Czech Republic
Specializations: Cardiac surgery, Vascular surgery, Thoracic surgery, Neurosurgery, Spine surgery, Orthopedic surgery, Oncology
Na Homolce Hospital is an organization directly subordinate to the Ministry of Health. It is a specialized clinical facility focusing on highly skilled, technologically advanced read more
Prices for popular procedures:
  1. Ebstein anomaly correction
    ≈ $14,826
  2. Dual chamber pacemaker insertion
    ≈ $7,252
  3. Coronary artery bypass graft (CABG)
    ≈ $5,792
  4. Heart valve replacement
    ≈ $21,783
  5. Heart valve repair
    ≈ $20,181
  6. Aortic valve replacement (AVR)
    ≈ $20,853
  7. Mitral valve replacement (MVR)
    ≈ $14,930
  8. Transcatheter aortic valve replacement (TAVR)
    ≈ $29,964
  9. Off-pump coronary artery bypass surgery
    ≈ $17,165
photo
Prague, Czech Republic
Specializations: Cardiac surgery, Vascular surgery, Neurosurgery, Oncology
The Institute for Clinical and Experimental Medicine is the largest specialised clinical and science & research institute in the Czech Republic. IKEM is focused on read more
Prices for popular procedures:
  1. Ebstein anomaly correction
    ≈ $6,142
  2. Dual chamber pacemaker insertion
    ≈ $4,226
  3. Coronary artery bypass graft (CABG)
    ≈ $13,168
  4. Heart valve replacement
    ≈ $14,473
  5. Heart valve repair
    ≈ $14,523
  6. Aortic valve replacement (AVR)
    ≈ $16,105
  7. Mitral valve replacement (MVR)
    ≈ $11,116
  8. Transcatheter aortic valve replacement (TAVR)
    ≈ $19,094
  9. Off-pump coronary artery bypass surgery
    ≈ $13,176
photo
Tirana, Albania
Specializations: Cardiac surgery, Vascular surgery, Thoracic surgery, Neurosurgery, Orthopedic surgery, Oncology
"Mother Teresa" University Hospital Center in Tirana is the largest health institution in Albania and as an academic health institution it is the only one read more
Prices for popular procedures:
  1. Ebstein anomaly correction
    ≈ $40,916
  2. Fetal cardiac interventions
    by request
  3. Dual chamber pacemaker insertion
    ≈ $17,824
  4. Coronary artery bypass graft (CABG)
    ≈ $35,429
  5. Heart valve replacement
    ≈ $39,106
  6. Heart valve repair
    ≈ $43,141
  7. Aortic valve replacement (AVR)
    ≈ $38,671
  8. Mitral valve replacement (MVR)
    ≈ $32,317
  9. Transcatheter aortic valve replacement (TAVR)
    ≈ $40,344
photo
Edegem, Belgium
Specializations: Cardiac surgery, Vascular surgery, Thoracic surgery, Neurosurgery, Spine surgery, Orthopedic surgery, Oncology
The Antwerp University Hospital (UZA) is an academic centre renowned for leading clinical and customer-friendly patient care, high-quality academic education and ground-breaking scientific research with read more
Prices for popular procedures:
  1. Ebstein anomaly correction
    ≈ $40,916
  2. Dual chamber pacemaker insertion
    ≈ $17,824
  3. Fontan procedure
    ≈ $36,045
  4. Permanent pacemaker implantation
    ≈ $11,149
  5. Radiofrequency ablation (RFA)
    ≈ $12,873
  6. Cardiac catheterization
    ≈ $4,875
  7. Percutaneous ASD closure
    ≈ $16,474
  8. Percutaneous closure of ventricular septal defect (VSD)
    ≈ $21,641
  9. Catheter cardiac ablation
    ≈ $13,865
photo
Brussels, Belgium
Specializations: Cardiac surgery, Vascular surgery, Thoracic surgery, Spine surgery, Orthopedic surgery, Oncology
Languages: French
Queen Fabiola Children's University Hospital is the only Belgian university hospital entirely reserved for children's medicine : all is conceived for them and for their read more
Prices for popular procedures:
  1. Ebstein anomaly correction
    ≈ $6,807
  2. Coronary artery bypass graft (CABG)
    ≈ $14,346
  3. Off-pump coronary artery bypass surgery
    ≈ $9,266
  4. Fontan procedure
    ≈ $9,853
  5. Cardiac catheterization
    ≈ $1,666
  6. Percutaneous ASD closure
    ≈ $7,325
  7. Percutaneous closure of ventricular septal defect (VSD)
    ≈ $5,366
  8. Coronary catheterization
    ≈ $1,006
  9. Percutaneous coronary intervention (PCI) with stent insertion
    ≈ $5,973
photo
Sarajevo, Bosnia and Herzegovina
Specializations: Cardiac surgery, Vascular surgery, Thoracic surgery, Neurosurgery, Spine surgery, Orthopedic surgery, Oncology
University Clinical Center Sarajevo (UKCS) is a leading professional, educational, teaching and scientific research health institution in Bosnia and Herzegovina. The institution aims to provide read more
Prices for popular procedures:
  1. Ebstein anomaly correction
    ≈ $12,901
  2. Dual chamber pacemaker insertion
    ≈ $5,248
  3. Coronary artery bypass graft (CABG)
    ≈ $17,318
  4. Heart valve replacement
    ≈ $21,090
  5. Heart valve repair
    ≈ $16,252
  6. Aortic valve replacement (AVR)
    ≈ $25,329
  7. Mitral valve replacement (MVR)
    ≈ $16,175
  8. Transcatheter aortic valve replacement (TAVR)
    ≈ $30,320
  9. Off-pump coronary artery bypass surgery
    ≈ $10,991
photo
Zagreb, Croatia
Specializations: Cardiac surgery, Vascular surgery, Thoracic surgery, Neurosurgery, Spine surgery, Orthopedic surgery, Oncology
University Hospital Centre Zagreb is the largest health institution in the Republic of Croatia, considering the number and diversity of health services it provides. Every read more
Prices for popular procedures:
  1. Ebstein anomaly correction
    ≈ $12,712
  2. Dual chamber pacemaker insertion
    ≈ $10,216
  3. Coronary artery bypass graft (CABG)
    ≈ $35,276
  4. Heart valve replacement
    ≈ $43,909
  5. Heart valve repair
    ≈ $32,990
  6. Aortic valve replacement (AVR)
    ≈ $40,889
  7. Mitral valve replacement (MVR)
    ≈ $29,613
  8. Transcatheter aortic valve replacement (TAVR)
    ≈ $50,101
  9. Off-pump coronary artery bypass surgery
    ≈ $22,930
photo
Marseille, France
Specializations: Cardiac surgery, Vascular surgery, Thoracic surgery, Neurosurgery, Spine surgery, Orthopedic surgery, Oncology
The Center Hospitalier de la Timone (Hôpitaux Universitaires de Marseille Timone) is the largest in the PACA region. It is considered by its activity, its read more
Prices for popular procedures:
  1. Ebstein anomaly correction
    ≈ $12,712
  2. Fontan procedure
    ≈ $26,488
  3. Patent ductus arteriosus (PDA) ligation in children
    ≈ $15,531
  4. Truncus arteriosus repair
    ≈ $23,669
  5. Norwood procedure
    ≈ $12,413
  6. Atrioventricular canal defect surgery
    ≈ $23,609
  7. Nikaidoh procedure
    ≈ $11,224
  8. Atrial septal defect (ASD) repair in children
    ≈ $22,702
  9. Arterial switch operation (ASO)
    ≈ $27,813
photo
Paris, France
Specializations: Cardiac surgery, Thoracic surgery, Neurosurgery, Spine surgery, Orthopedic surgery, Oncology
Necker-Enfants Malades hospital is the heir to this double history: beside its services devoted to children medicine and surgery, it has an adult department of read more
Prices for popular procedures:
  1. Ebstein anomaly correction
    ≈ $12,712
  2. Coronary artery bypass graft (CABG)
    ≈ $35,276
  3. Heart valve replacement
    ≈ $43,909
  4. Heart valve repair
    ≈ $32,990
  5. Aortic valve replacement (AVR)
    ≈ $40,889
  6. Mitral valve replacement (MVR)
    ≈ $29,613
  7. Transcatheter aortic valve replacement (TAVR)
    ≈ $50,101
  8. Off-pump coronary artery bypass surgery
    ≈ $22,930
  9. Fontan procedure
    ≈ $26,488
photo
Le Plessis-Robinson, France
Specializations: Cardiac surgery, Vascular surgery, Thoracic surgery, Oncology
The Marie Lannelongue Hospital is a private non-profit hospital, Private Health Establishment of Collective Interest (ESPIC), practicing agreed rates without exceeding fees. It is part read more
Prices for popular procedures:
  1. Ebstein anomaly correction
    ≈ $64,124
  2. Dual chamber pacemaker insertion
    ≈ $20,594
  3. Fontan procedure
    ≈ $40,853
  4. Permanent pacemaker implantation
    ≈ $20,607
  5. Cardiac catheterization
    ≈ $7,662
  6. Percutaneous ASD closure
    ≈ $24,667
  7. Percutaneous closure of ventricular septal defect (VSD)
    ≈ $32,577
  8. Patent ductus arteriosus (PDA) ligation in children
    ≈ $32,244
  9. Removal of permanent pacemaker
    ≈ $13,518
photo
Sankt Augustin, Germany
Specializations: Cardiac surgery, Vascular surgery, Thoracic surgery, Neurosurgery, Spine surgery, Orthopedic surgery, Oncology
Our children's clinic is available at all times for medical emergencies involving children. Specialists from 15 specialist departments work around the clock to ensure fast read more
Prices for popular procedures:
  1. Ebstein anomaly correction
    ≈ $46,820
  2. Fontan procedure
    ≈ $42,243
  3. Radiofrequency ablation (RFA)
    ≈ $18,287
  4. Cardiac catheterization
    ≈ $6,549
  5. Percutaneous ASD closure
    ≈ $20,435
  6. Percutaneous closure of ventricular septal defect (VSD)
    ≈ $25,193
  7. Coronary catheterization
    ≈ $3,756
  8. Percutaneous coronary intervention (PCI) with stent insertion
    ≈ $20,143
  9. Catheter cardiac ablation
    ≈ $19,731
photo
London, United Kingdom
Specializations: Cardiac surgery, Vascular surgery, Thoracic surgery, Neurosurgery, Spine surgery, Orthopedic surgery, Oncology
Great Ormond Street Hospital (GOSH) is a globally renowned children’s hospital, championing innovation across more than 60 clinical specialties and providing ground-breaking treatments for the read more
Prices for popular procedures:
  1. Ebstein anomaly correction
    ≈ $46,866
  2. Heart valve replacement
    ≈ $53,563
  3. Heart valve repair
    ≈ $52,832
  4. Aortic valve replacement (AVR)
    ≈ $60,315
  5. Mitral valve replacement (MVR)
    ≈ $41,392
  6. Transcatheter aortic valve replacement (TAVR)
    ≈ $88,397
  7. Fontan procedure
    ≈ $42,284
  8. Radiofrequency ablation (RFA)
    ≈ $18,305
  9. Cardiac catheterization
    ≈ $6,556
photo
Birmingham, United Kingdom
Specializations: Cardiac surgery, Vascular surgery, Thoracic surgery, Neurosurgery, Spine surgery, Orthopedic surgery, Oncology
We are Birmingham Women’s and Children’s NHS Foundation Trust – proud to bring together the expertise of Birmingham Children’s Hospital and Birmingham Women’s Hospital. Our read more
Prices for popular procedures:
  1. Ebstein anomaly correction
    ≈ $46,866
  2. Dual chamber pacemaker insertion
    ≈ $16,575
  3. Coronary artery bypass graft (CABG)
    ≈ $49,142
  4. Off-pump coronary artery bypass surgery
    ≈ $48,391
  5. Fontan procedure
    ≈ $42,284
  6. Permanent pacemaker implantation
    ≈ $13,812
  7. Radiofrequency ablation (RFA)
    ≈ $18,305
  8. Cardiac catheterization
    ≈ $6,556
  9. Percutaneous ASD closure
    ≈ $20,455
photo
Liverpool, United Kingdom
Specializations: Cardiac surgery, Vascular surgery, Thoracic surgery, Neurosurgery, Spine surgery, Orthopedic surgery, Oncology
As one of Europe’s biggest and busiest children’s hospitals, we treat everything from common illnesses to highly complex and specialist conditions. In 2015 we opened read more
Prices for popular procedures:
  1. Ebstein anomaly correction
    ≈ $33,655
  2. Fetal cardiac interventions
    by request
  3. Dual chamber pacemaker insertion
    ≈ $16,275
  4. Heart valve replacement
    ≈ $47,105
  5. Heart valve repair
    ≈ $52,613
  6. Aortic valve replacement (AVR)
    ≈ $48,772
  7. Mitral valve replacement (MVR)
    ≈ $38,648
  8. Transcatheter aortic valve replacement (TAVR)
    ≈ $65,701
  9. Fontan procedure
    ≈ $30,982
photo
Rome, Italy
Specializations: Cardiac surgery, Vascular surgery, Thoracic surgery, Neurosurgery, Spine surgery, Orthopedic surgery, Oncology
The Bambino Gesù Pediatric Hospital, an institution of the Holy See , is the largest pediatric polyclinic and research center in Europe . Connected to read more

Clinics grouping by rating

Clinic with the highest rating of 5 — Clinical Hospital Lapino in Moscow, Russia and 1 more, clinic with the most reviews number of 3114 — Quirónsalud Madrid University Hospital in Pozuelo de Alarcón, Spain.

With rating 4.0 and over — 24 clinics .

Countries with the highest number of clinics treating the diseases:

Ebstein anomaly:

Related procedures:

Procedures are likely to be used for Ebstein anomaly treatment: Ebstein anomaly correction and Fetal cardiac interventions .

Ebstein anomaly - what you should know about the disease

Classification, pathophysiology of Ebstein anomaly

Ebstein anomaly includes a wide range of morphological and clinical manifestations. Those who show symptoms in early infancy tend to be at the highest risk. This pathology affects the tricuspid valve (its other name is tricuspid) and is a congenital malformation of the cardiovascular system. At the same time, there is a shift of the point of attachment of the cusps of this valve - septal and posterior - down. However, the anterior cusp is usually not affected. The leaflets themselves are dysplastic, i.e. developed incorrectly. As a result of the displacement, poor closure of the valve leaflets (in other words, impaired coaptation) is observed, which leads to tricuspid regurgitation - the backflow of blood through the tricuspid valve. This, in turn, causes an increase in the size of the right atrium of varying degrees.

Causes and prevalence of Ebstein anomaly

No defect in any one gene has been identified with Ebstein anomaly. This pathology appears to be genetically heterogeneous; develops as a result of various, unpredictable, several gene anomalies. Several candidate genes have been proposed as culprits, including GATA4 and NKX2.5. Lithium exposure during pregnancy has been reported as a causative factor.

Ebstein anomaly accounts for 0.3 to 0.6% of all congenital heart defects. There is no preponderance towards children of any gender, and most cases are completely sporadic, i.e. occur spontaneously and randomly. The frequency of recurrence in the offspring of women is higher (6%) than in the offspring of men (0.6%).

Anatomy and pathophysiology of Ebstein anomaly

The tricuspid valve separates the right atrium (the chamber that receives blood from the body) from the right ventricle (the chamber that pumps blood to the lungs).

In Ebstein anomaly, the two leaflets of the tricuspid valve move down into the pumping chamber. The third flap has an elongated shape and can be attached to the chamber wall. In rare cases, the valve becomes so deformed that it does not allow blood to flow freely forward in the normal direction.

Most often, these anomalies cause blood to leak through the tricuspid valve from the right ventricle back into the right atrium, into the systole - i.e. when the muscles of the right ventricle contract. As a result, the right atrium expands in size, accommodating an unusually large volume of blood.

Hemodynamic disturbances depend on the severity of the lesion. The main determining factors are the degree of displacement of the tricuspid valve leaflets and the degree of tricuspid regurgitation. With a mild degree of Ebstein anomaly, the function of the tricuspid valve is close to normal. But if the tricuspid regurgitation is severe enough, with each atrial contraction blood is ejected into the right ventricle, and with ventricular contraction it is pushed back into the right atrium. This can lead to congestive heart failure.

Normally, the fetus has a communication between the right and left atria, known as the foramen ovale (foramen ovale). It usually closes after the baby is born. However, in Ebstein anomaly, there is a reverse flow of blood into the right atrium, so the pressure inside this chamber of the heart becomes very high. This keeps the foramen ovale open, and as a result, oxygen-poor blood flows from the right atrium to the left, bypassing the lungs and heading straight for the organs and tissues. This is why children with Ebstein anomaly may have a bluish, or "cyanotic" skin tone.

This heart disease can also occur with other lesions of this organ, such as stenosis or atresia of the pulmonary valve, atrial or ventricular septal defect. In addition, many patients with Ebstein anomaly have an extra conduction pathway in the heart that can potentially lead to episodes of an abnormal fast heart rate called supraventricular tachycardia (this condition is known as Wolff-Parkinson-White syndrome).

Signs and symptoms of Ebstein anomaly

Ebstein anomaly can range from very mild to very severe, having 4 degrees, from 1 to 4, respectively. Many patients with milder forms of Ebstein anomaly are asymptomatic, and as a result, the malformation may be discovered in adulthood—often due to the discovery of a heart murmur. Abnormal or extra heart sounds may also be detected during a routine examination.

In some infants and children, the skin turns bluish (cyanotic) due to blood flow from the right atrium to the left. Children may complain of heart palpitations and skipping beats, sometimes people describe this feeling as "a strange heartbeat". They are characterized by increased fatigue, shortness of breath, especially during the game, and chest pain. Growth and development in patients with Ebstein anomaly is usually normal.

Severely affected children are often critically ill at birth, with low oxygen saturation and heart failure requiring immediate intensive care.

Diagnosis of Ebstein anomaly

If you suspect a heart defect, you should immediately consult a doctor. A chest x-ray should be done to assess size. Often the diagnosis of Ebstein anomaly is suspected precisely because of a very large heart on a chest x-ray.

Echocardiography is used to definitively diagnose Ebstein anomaly and identify any associated heart defects. This study allows the pediatric cardiologist to determine the degree of valve wall displacement, the severity of valve failure, or orifice narrowing. You can determine the size of the chambers of the heart and the presence of an open foramen ovale.
An electrocardiogram records the electrical activity of the heart. It is indicated for recording the heart rhythm when there is a violation of it. An exercise stress test may be performed to better assess heart function during increased activity. Some patients with abnormal heart rhythms may require an additional electrophysiological study to more accurately identify problems with the heart rhythm.

Invasive diagnostic tests are used much less frequently today than in previous years. This is due to the availability of more informative non-invasive methods, such as echocardiography and MRI. However, some patients with Ebstein anomaly may require cardiac catheterization.

Treatment of Ebstein anomaly

Mild defects often do not require special treatment. In addition, there is no need for bacterial endocarditis prophylaxis, such as taking antibiotics during dental visits. Drug treatment is reserved for children with congestive heart failure or abnormal heart rhythms.

Surgery may only be indicated under certain circumstances. Surgical repair or replacement of the tricuspid valve and closure of the foramen ovale are sometimes recommended. Surgery may be indicated for older children with moderate to severe congestive heart failure, significant enlargement of the heart, cyanosis, or abnormal blood clots. Abnormal accessory pathways that contribute to supraventricular tachycardia may be removed.

Medical therapy for heart failure or arrhythmias is usually combined with elective surgery. Very severe forms of Ebstein anomaly may require surgery in the neonatal period, and management is more similar to that for children with single ventricular heart anomalies.

Disease prognosis

Without any treatment, the prognosis for children with Ebstein anomaly is very poor. Studies have shown that only half of all people affected by this disease, without treatment, can live up to 13 years. However, people with milder forms have a better prognosis and life expectancy close to the population average.

The Zelermeier index is used to predict outcome based on the fetal or neonatal echocardiogram. This indicator allows you to predict the likelihood of mortality. The higher the class, the higher this indicator:

  • at grade 1 (ratio less than 0.5), the mortality rate is 0%;
  • at grade 2 (ratio 0.5 to 0.99), the mortality rate is 10%;
  • at grade 3 (ratio 1.0 to 1.49), the mortality rate is 4%;
  • at grade 4 (coefficient greater than 1.5), the mortality rate is 100%.

Life of people with Ebstein anomaly

Ebstein anomaly can be diagnosed at any age, including adults. As already mentioned, people with the mildest form of the disease may not have any problems throughout their lives. Many people with this pathology develop arrhythmia, and the older the person, the higher the likelihood of such an outcome.

All people with Ebstein anomaly need lifelong medical supervision. Many needs treatment for rhythm disturbances, including ablation procedures. Episodes of life-threatening arrhythmias tend to recur; in addition, the development of other arrhythmias that require constant monitoring is possible.

References:

  1. Kliegman RM, St. Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM. Cyanotic congenital heart lesions: lesions associated with decreased pulmonary blood flow. In: Kliegman RM, St.Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, eds. Nelson Textbook of Pediatrics. 21st ed. Philadelphia, PA: Elsevier; 2020:chap 457.
  2. Webb GD, Smallhorn JF, Therrien J, Redington AN. Congenital heart disease in the adult and pediatric patient. In: Zipes DP, Libby P, Bonow RO, Mann DL, Tomaselli GF, Braunwald E, eds. Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine. 11th ed. Philadelphia, PA: Elsevier; 2019:chap 75.
  3. Mulla S, Asuka E, Siddiqui WJ. StatPearls [Internet]. StatPearls Publishing; Treasure Island (FL): Jul 28, 2021. Tricuspid Regurgitation.
  4. Voges I, Al-Mallah MH, Scognamiglio G, Di Salvo G. Right Heart-Pulmonary Circulation Unit in Congenital Heart Diseases. Heart Fail Clin. 2018 Jul;14(3):283-295.

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