Angiographic and morphologic features of the left ventricle in Ebstein's malformation

doi:10.1016/S0002-9149(97)00602-4

The American Journal of Cardiology

Volume 80, Issue 8, 15 October 1997, Pages 1051-1059

https://doi.org/10.1016/S0002-9149(97)00602-4 Get rights and content

Abstract

Quantitative and qualitative cineangiographic analysis of the left ventricle (LV) was performed in 26 patients with isolated Ebstein's malformation, having a mean age of 23 ± 17 years. Nine autopsied hearts with isolated Ebstein's malformation were submitted to morphologic and morphometric analysis. In 4 of the cases, it was possible to make a direct correlation between the angiographic data obtained during life and the autopsy findings. On the basis of the LV end-diastolic volume we identified 3 groups of patients: 7 with volume <60 ml/m², another 7 with volume between 60 and 80 ml/m², and 12 with volume >80 ml/m². The LV ejection fraction was reduced in 2 patients with normal LV enddiastolic volume and in 6 with increased LV end-diastolic volume. The ratio of ventricular mass to LV end-diastolic volume was always adequate, but a reduction of the ventricular contractive performance (end-systolic pressure to end-systolic volume ratio <3 mm Hg/ml/m²) was found only in patients with a dilated left ventricle. No correlation was demonstrated between the extent of the atrialized component of the right ventricle (mean value 67 ± 31 cm², range 13 to 133) and the LV dimensions. All but 2 patients showed a leftward diastolic displacement of the ventricular septum, but in only 1 did this produce an elongated shape of the left ventricle. Sixteen had anomalies of LV dynamics: 10 with hypokinesia (3 of the posterior wall, 4 of the apex, 1 of the inferior wall, 1 of the septum, and 1 global), 6 with dyskinesia (1 of the posterior wall, 2 of the apex, 1 of the posterior wall and apex, 1 of the superior part of the septum, and 1 of the anterior wall), and 8 with premature diastolic distension of the anterobasal wall. Morphometric analysis produced mean values for myocytes of 59 ± 10%, for the interstitium of 21 ± 4%, and for fibrous tissue of 20 ± 9% (normal 4 ± 1%). Five autopsied hearts had a prolapsing and/or dysplastic mitral valve.

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Cited by (51)

Left ventricular outflow tract obstruction in patients with Ebstein anomaly
2023, JTCVS Open
In this study, we investigated the clinical characteristics of patients with Ebstein anomaly and left ventricular outflow tract (LVOT) obstruction as well as possible mechanisms of obstruction as assessed by 2-dimensional echocardiography.
We queried our institutional echocardiography and surgical databases for patients with the diagnosis of Ebstein anomaly seen from 1985 through 2022. Fourteen patients had the additional diagnosis of LVOT obstruction identified from transthoracic echocardiography.
The mean age of the 14 patients at the time that LVOT obstruction was identified was 51.9 ± 9.9 years, and 11 (78%) were female. LVOT obstruction (mean gradient 61.0 ± 25.7 mm Hg) was documented at the time of initial diagnosis of Ebstein anomaly for 8, and in the remaining, was recognized on follow-up imaging. Dynamic LVOT obstruction with systolic anterior motion (SAM) was the most common mechanism and was mostly accompanied by leftward septal movement and septal hypertrophy. Seven of the 9 patients with SAM had the diagnosis of hypertrophic cardiomyopathy. Leftward bowing of the septum appeared to be the sole cause of LVOT obstruction in 3 and a contributing factor to SAM in 4. Among the 13 patients who underwent an operation for Ebstein anomaly, 7 had concomitant septal myectomy and 3 had a subsequent procedure.
There are multiple mechanisms of LVOT obstruction in patients with Ebstein anomaly, including SAM and leftward bowing of the interventricular septum, which may exist alone or in combination. Septal myectomy at the time of tricuspid valvuloplasty is safe and effectively reduces gradients in the outflow tract and may prevent the need for subsequent reintervention.
Left, but not right, ventricular status determines heart failure in adults with Ebstein anomaly – A case-control study based on magnetic resonance
2022, International Journal of Cardiology
Citation Excerpt :
However, although EA is considered a defect of the tricuspid valve, more attention is nowadays being paid to the status of the left ventricle (LV) [9–11]. Few data from the literature suggest genetically determined abnormalities in the anatomy and function of the LV in patients with Ebstein anomaly [12,13]. Moreover, the phenomenon of ventricular interdependence, particularly in the setting of the dilated RV in adults with EA, may also be of significant influence on the hemodynamic conditions and function of the left heart [14–16].
Ebstein anomaly (EA) is a congenital heart defect affecting the right heart. Heart failure (HF) is a significant complication in adults with EA. It may result not only from the right ventricle (RV), but also from the left ventricle (LV) abnormalities. We evaluate the size and function of both ventricles in patients with EA in cardiac magnetic resonance (CMR); to assess their association with the clinical markers of HF.
Study group: 37 unoperated adults with EA (mean age 43.0 ± 14.4y, 21[56.8%] males). Controls: 25 volunteers (mean age 39.9 ± 10.9y, 15[60%] males). Study protocol included: CMR [ejection fraction (EF), end-diastolic (EDVind) and stroke volumes (SVind) indexed by body surface area]; cardiopulmonary test (peak VO2, %peak VO2, VE/VCO2 slope).
Size and systolic function of LV were reduced comparing to the controls [LVEDVind (ml/m2): 63.7(range 38.7–94.2) vs. 79.3(48.7–105.1), p < 0.001; LV SVind (ml/m2): 35.8(22.9–55.1) vs. 49.2(37.8–71.7), p < 0.0001; LVEF(%): 58.3(34–70.5) vs. 62.0(52.0–77.0), p = 0.009]. RV was enlarged comparing to the controls [RVEDVind (ml/m2): 124.3(52.8–378.9) vs. 83.0(64.0–102.0), p < 0.0001) with impaired systolic function (RV SVind (ml/m2): 22.7(11.1–74.1) vs. 48.0(37.8–71.7), p < 0.0001; RVEF(%): 38.0(21.0–66.1) vs. 59.0(49.0–69.0), p < 0.0001). A significant correlation was found between LVEDVind vs. peakVO2 (r = 0.52, p = 0.001); LV SVind vs. peakVO2 (r = 0.47,p = 0.005). There was no correlation between the right ventricular status and exercise capacity.
In adults with Ebstein anomaly the size of left ventricle is reduced, right ventricle is enlarged; the function of both is impaired. Abnormal exercise capacity is associated with left ventricular status. Ventricular interdependence probably plays a role in heart failure pathomechanism.
Congenital Heart Disease: A Clinical, Pathological, Embryological, and Segmental Analysis
2022, Congenital Heart Disease: A Clinical, Pathological, Embryological, and Segmental Analysis
Catheter Ablation of Ventricular Arrhythmia for Ebstein's Anomaly in Unoperated and Post-Surgical Patients
2018, JACC: Clinical Electrophysiology
The purpose of this study was to determine the ventricular arrhythmia (VA) substrates in patients with unoperated and post-surgical Ebstein's Anomaly (EA).
EA is associated with variable atrialization of the right ventricle and a propensity for VA and sudden death. There are scant data on catheter ablation for VA in this population.
This was a retrospective study involving 11 congenital heart disease centers.
A total of 24 patients (median age 17 [interquartile range (IQR): 11 to 37] years; age range 1 to 68 years; 42% men) with EA undergoing catheter ablation were identified. Prior tricuspid valve (TV) surgery had been performed in 12 (50%). Presenting symptoms were palpitations in 15, syncope in 4, aborted cardiac arrest in 4, and none in 1. At procedure, 28 VA substrates were encountered and 25 were completely characterized (median 1 per patient; cycle length 305 [IQR: 268 to 400] ms). In 3 cases, premature ventricular contraction (PVC) foci were targeted (1 with a history of PVC-induced ventricular fibrillation). VA mechanisms were focal in 15 and macro–re-entrant in 10, and did not differ significantly between those with and those without prior TV surgery (p = 0.7). Focal VAs predominantly localized to the atrialized right ventricle ARV in unoperated patients and to diseased myocardium or Purkinje tissue after TV surgery. Macro–re-entry was related to isolated scar or split potentials in the ARV in unoperated patients, and larger, more diffuse scar after TV surgery. Complete success was achieved in 22 (92%). There were 2 of 13 complications in patients <18 years of age and none in patients >18 years of age. There was a single recurrence over a median follow-up of 3.4 years.
VA in EA may be either focal or macro–re-entrant. In the absence of surgery, substrates chiefly involve the ARV. After surgery, focal VA involves injured myocardium or Purkinje tissue and re-entrant ventricular tachycardia is related to post-surgical scar. Catheter ablation is a reasonable therapeutic approach for these patients.
Ebstein anomaly after tricuspid valve replacement needing pacemaker implantation: Case submitted by Christopher J. McLeod, MBChB, PhD
2018, Arrhythmias in Adult Congenital Heart Disease: A Case-Based Approach
- 1.
  The presence of a prosthetic tricuspid valve is a relative but not absolute contraindication to endocardial pacing.
- 2.
  Epicardial pacing is a good option but does involve more surgery.
- 3.
  Innovative endocardial approaches, such as the leadless pacemaker, can be considered depending on the indication to pace.
- 4.
  Hybrid procedures (part endocardial and part epicardial) and newer leads (lumenless leads) can be used in some patients.
Proof of an association between Morbus Ebstein and arrhythmogenic cardiomyopathy in a patient with chronic atrial fibrillation
2016, International Journal of Cardiology

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^☆: This study was supported by Target Projects “Cardiopatie congenite,” Veneto Region, Venice, and “FAT.MA,” National Council for Research, Rome, Italy.

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Angiographic and morphologic features of the left ventricle in Ebstein's malformation☆

Abstract

J Am Coll Cardiol

Cardiovasc Pathol

Am J Cardiol

J Am Coll Cardiol

J Am Coll Cardiol

Left ventricular anomalies associated with Ebstein's malformation of the tricuspid valve

Circulation

Ebstein anomaly: late results of surgical correction

Eur J Cardiol

Left ventricular geometry and function in adults with Ebstein's anomaly of the tricuspid valve

Circulation

Ebstein's anomaly of the tricuspid valve and prolapse of the mitral valve

Am Heart J