Cardiology

Diagnosis and Classification: Measuring the Heart

At a Glance

Ebstein anomaly is diagnosed using an echocardiogram to measure downward displacement of the tricuspid valve. Doctors classify severity using the Carpentier system (Types A-D) and Celermajer index. A cardiac MRI is also used to accurately measure heart volumes and check for scarring.

Because Ebstein anomaly is a complex and rare condition, doctors use several specialized imaging techniques and classification systems to measure its severity and plan for the future. Understanding these “scores” and “types” can help you track how the disease is progressing and what your surgical options might be.

Diagnostic Criteria: The “Golden Rule”

The primary way a doctor confirms Ebstein anomaly is through an echocardiogram (an ultrasound of the heart) ^[1]. The hallmark sign is the displacement of the tricuspid valve leaflets toward the bottom (apex) of the heart ^[2].

The 8 mm/m² Threshold: To make a formal diagnosis, the septal leaflet (one of the three flaps of the valve) must be displaced downward by at least 8 millimeters for every square meter of body surface area ^[2]. This measurement helps differentiate Ebstein anomaly from other valve problems.

Classification Systems

Doctors use two main systems to describe how much the heart is affected.

1. The Carpentier Classification

This system categorizes the heart into four types (A through D) based on the anatomy of the right ventricle and how much the valve is moved ^[3]:

Type A: The right ventricle is still mostly functional, and the valve displacement is mild.
Type B: There is a large “atrialized” (non-pumping) section of the ventricle, but the rest still works well.
Type C: The valve leaflets are very restricted and can severely block blood flow.
Type D: The right ventricle is almost entirely “atrialized,” leaving very little functional heart muscle ^[4].

2. The Celermajer Index (GOSE Score)

Originally developed at Great Ormond Street Hospital, this index provides a numeric score to estimate severity. It compares the size of the enlarged, “sick” parts of the right side of the heart to the healthy, functioning parts ^[5].

The Grades: Grade 1 (< 0.5) is mild, while Grade 4 (> 1.5) is the most severe ^[5]. A higher score often indicates a higher risk for complications or the need for surgery ^[6]^[7].

The Role of Cardiac MRI (CMR)

While an echocardiogram is the first step, Cardiac MRI is often considered the “gold standard” for a complete evaluation ^[8]. It is much more accurate at measuring:

Ventricular Volumes: Exactly how much blood the right ventricle can hold and pump ^[4].
Fibrosis and Scarring: Using a technique called Late Gadolinium Enhancement (LGE), MRI can find areas of scarring (fibrosis) ^[9]. Fibrosis in the left or right ventricle can predict future heart rhythm issues or a decline in heart function ^[10]^[11].

Diagnostic Checklist

When you receive a diagnostic report, look for these key items to ensure a thorough evaluation ^[12]^[13]:

[ ] Septal leaflet displacement (measured in mm/m²).
[ ] Carpentier Type (A, B, C, or D).
[ ] Celermajer Index or GOSE Grade.
[ ] Right Ventricular Ejection Fraction (RVEF): How well the lower chamber is pumping.
[ ] Atrial Septal Defect (ASD): Presence of a hole between the upper chambers.
[ ] Presence of Fibrosis: Mention of LGE on a Cardiac MRI report.

Once you have reviewed your diagnostic report, you and your medical team can discuss if and when surgery is necessary on the Treatment Strategy page.

Common questions in this guide

How is Ebstein anomaly diagnosed?

Doctors primarily use an echocardiogram, or ultrasound of the heart, to confirm an Ebstein anomaly diagnosis. They measure how far the tricuspid valve is displaced downward to determine if it meets the formal diagnostic criteria.

What is the Carpentier classification for Ebstein anomaly?

The Carpentier system categorizes the heart into four types (A, B, C, or D) based on the anatomy of the right ventricle and the severity of the valve displacement. Type A is the mildest form, while Type D is the most severe.

What does the Celermajer index or GOSE score mean?

This score estimates the severity of Ebstein anomaly by comparing the size of the enlarged, poorly functioning parts of the heart to the healthy parts. A higher score, up to Grade 4, indicates a more severe condition and a higher likelihood of needing surgery.

Why do I need a cardiac MRI if I already had an echocardiogram?

While an echocardiogram is the first step, a cardiac MRI provides a much more detailed view of your heart. It accurately measures how much blood your right ventricle can pump and can spot areas of heart scarring or fibrosis.

What is late gadolinium enhancement (LGE) on an MRI report?

Late gadolinium enhancement is an MRI technique used to find areas of scarring, or fibrosis, in the heart muscle. Finding fibrosis is important because it can help doctors predict future heart rhythm issues or changes in heart function.

Curated prompts to bring to your next appointment.

1.What was the exact measurement of my septal leaflet displacement in mm/m2?
2.Which Carpentier Type (A, B, C, or D) best describes my (or my child's) heart anatomy?
3.What is the Celermajer index or GOSE score from the most recent echocardiogram?
4.Does the Cardiac MRI show any signs of 'late gadolinium enhancement' or fibrosis in the right or left ventricle?
5.Are the right ventricular volumes high enough to suggest that we should consider surgical intervention soon?
6.Is there evidence of left ventricular non-compaction or other structural issues besides the tricuspid valve?

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References (13)

1
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Sharma T, Habash F, Mounsey J, et al.
Cureus 2020; (12(10)):e10773 doi:10.7759/cureus.10773.
PMID: 33154844
2
The Congenital Tricuspid Valve Spectrum: From Ebstein to Dysplasia.
Stephens EH, Dearani JA, Qureshi MY, et al.
World journal for pediatric & congenital heart surgery 2020; (11(6)):783-791 doi:10.1177/2150135120949235.
PMID: 33164686
3
Biventricular function after Ebstein anomaly repair from a single-center echocardiography study.
Meng H, Wan LY, Qu R, et al.
Hellenic journal of cardiology : HJC = Hellenike kardiologike epitheorese 2025; (86()):80-90 doi:10.1016/j.hjc.2024.05.019.
PMID: 38844023
4
Complimentary Cardiac Computed Tomography Ventricular Volumetry-Derived Metrics of Severity in Patients with Ebstein Anomaly: Comparison with Echocardiography-Based Severity Indices.
Goo HW, Park SH
Pediatric cardiology 2024; (45(1)):24-31 doi:10.1007/s00246-023-03342-3.
PMID: 38012401
5
Severity Scores for Ebstein Anomaly: Credibility and Usefulness of Echocardiographic vs Magnetic Resonance Assessments of the Celermajer Index.
Cieplucha A, Trojnarska O, Bartczak-Rutkowska A, et al.
The Canadian journal of cardiology 2019; (35(12)):1834-1841 doi:10.1016/j.cjca.2019.08.003.
PMID: 31711824
6
Epidemiological, clinical, and echocardiographic features, and outcome of dogs with Ebstein's anomaly: 32 cases (2002-2016).
Chetboul V, Poissonnier C, Bomassi E, et al.
Journal of veterinary cardiology : the official journal of the European Society of Veterinary Cardiology 2020; (29()):11-21 doi:10.1016/j.jvc.2020.03.003.
PMID: 32348933
7
Outcomes and Predictors of Perinatal Mortality in Fetuses With Ebstein Anomaly or Tricuspid Valve Dysplasia in the Current Era: A Multicenter Study.
Freud LR, Escobar-Diaz MC, Kalish BT, et al.
Circulation 2015; (132(6)):481-9 doi:10.1161/CIRCULATIONAHA.115.015839.
PMID: 26059011
8
Practical approach to using cardiac magnetic resonance imaging for pre-surgical planning in complex paediatric congenital heart disease.
Fitzgerald NM, Singh AA, Barron DJ, et al.
Pediatric radiology 2025; (55(11)):2272-2285 doi:10.1007/s00247-025-06233-2.
PMID: 40227499
9
Clinical aspects of myocardial fibrosis in adults with Ebstein's anomaly.
Ciepłucha A, Trojnarska O, Kociemba A, et al.
Heart and vessels 2018; (33(9)):1076-1085 doi:10.1007/s00380-018-1141-5.
PMID: 29468473
10
Assessment of Left Ventricular Myocardial Fibrosis in Adult Patients With Ebstein Anomaly: A Retrospective Cohort Study Based on Cardiac Magnetic Resonance and Histopathological Samples.
Fernandez-Badillo V, Serrano-Roman J, Antonio-Villa NE, et al.
Circulation. Cardiovascular imaging 2023; (16(5)):e015011 doi:10.1161/CIRCIMAGING.122.015011.
PMID: 37154028
11
Cardiovascular magnetic resonance evidence of myocardial fibrosis and its clinical significance in adolescent and adult patients with Ebstein's anomaly.
Yang D, Li X, Sun JY, et al.
Journal of cardiovascular magnetic resonance : official journal of the Society for Cardiovascular Magnetic Resonance 2018; (20(1)):69 doi:10.1186/s12968-018-0488-1.
PMID: 30257686
12
Ebstein Anomaly in the Adult Patient.
Fuchs MM, Connolly HM
Cardiology clinics 2020; (38(3)):353-363 doi:10.1016/j.ccl.2020.04.004.
PMID: 32622490
13
Right Ventricular Diastolic Function and Right Atrial Function and Their Relation With Exercise Capacity in Ebstein Anomaly.
Akazawa Y, Fujioka T, Kühn A, et al.
The Canadian journal of cardiology 2019; (35(12)):1824-1833 doi:10.1016/j.cjca.2019.05.036.
PMID: 31564390

This page provides educational information about Ebstein anomaly diagnostic tests and scoring systems. Always review your specific echocardiogram or MRI results with your cardiologist.

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