Ebstein Anomaly – ABC CARDIOL

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Case Report

Implantation of a Post-Dilatable Stent in Aortic Coarctation Via Carotid Access in a Newborn With Ebstein's Anomaly: Case Report

Jonathan Guimarães Lombardi et al. Paulo Correia Calamita Orlando Carlos Barbosa Mayra Rosana Palmeira Barreto Giulliano Gardenghi

Introduction

Ebstein’s anomaly is a complex congenital heart disease that occurs in one out of every 200,000 live births. Its association with left-sided obstructive lesions is rare and poses significant challenges for both clinical and surgical management. In this case report, we present a newborn patient with Ebstein’s anomaly associated with supravalvular mitral stenosis and coarctation of the aorta (CoA), who underwent cardiac catheterization for the implantation of a post-dilatable stent in the aortic isthmus via the carotid approach.

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Oscillating Ductal Stent in Valvar Pulmonary Atresia

Arun Gopalakrishnan et al. Kavassery Mahadevan Krishnamoorthy Paidi Suresh Kumar Sivasankaran Sivasubramonian

A 2.7 kg term neonate was diagnosed on day 3 of life with Ebstein’s anomaly of the tricuspid valve, severe tricuspid regurgitation, valvar pulmonary atresia and duct-dependent pulmonary circulation. The cardiothoracic ratio was 90%, and GOSE score was 1.48. Oxygen saturation was 65% and improved to 88% on 0.005 mcg/kg/min of prostaglandin. The right ventricular systolic pressure was 40mmHg against a systolic blood pressure of 65mmHg. The child was taken up for stenting of the arterial duct on day 7 of life after the confirmation of anatomic pulmonary atresia. The long arterial duct was stented with 3.5x16mm and 3.5x8mm stents, using a 4F right femoral artery access. Both the pulmonary and aortic ends were well covered and systemic saturation improved to 85% on room air and off prostaglandin.

The post-procedure echocardiography confirmed a well-positioned stent with good flow. However, the stent appeared to oscillate with every cardiac cycle (, ). This “oscillating stent sign” was confirmed on the post-stenting aortic angiogram (). Valvar pulmonary atresia is associated with the well-described “seagull sign” on angiogram. Pooling of contrast in the main pulmonary artery that retains a fibrous attachment to pulmonary annular tissue leads to the seagull sign, with the branch pulmonary arteries assuming the shape of the wings of the ‘seagull’. Valvar pulmonary atresia is generally associated with a straight arterial duct inserted into the main pulmonary artery in half of the cases. Even when the duct is long and tortuous in the others, studies suggest that it is more readily negotiated during ductal stenting in valvar pulmonary atresia as opposed to long segment pulmonary atresia.^, The oscillation of the ductal stent in valvar pulmonary atresia with every cardiac cycle suggests that the pulmonary end of the stent is within the main pulmonary artery, which is an intrapericardial structure and faithfully reflects the mechanical contractions of the heart. We suggest that this echocardiographic finding may be utilized for the assessment of stent position with respect to the pulmonary end in valvar pulmonary atresia.

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Clinicoradiological Correlation

Case 5/2019 – Naturally Evolving Ebstein’s Anomaly of Discrete Repercussion in 24-Year-old Asymptomatic Adult

Edmar Atik et al. Maria Angélica Binotto Alessandra Costa Barreto

Clinical data

Patient reports 3 episodes of paroxysmal tachycardia of up to 20 minutes, accompanied by precordial pain in the past 7 years, all with spontaneous reversal. Four years prior, the patient presented another tachycardia with signs of low output, including pallor, visual turbidity, cold extremities and mental confusion, requiring electrical reversion. On that occasion, ventricular tachycardia ablation in the right ventricular (RV) inflow tract through the right posterior accessory pathway was successfully performed. Ebstein’s anomaly diagnosis was given for the first time by echocardiography scan, at that time. Ever since, the patient remained asymptomatic, living a normal life and with no medication.

Physical examination: Good general condition, eupneic, acyanotic, normal pulses in the 4 limbs. Weight: 80 kg, Height: 180 cm, BP: 110 x 70 mmHg, HR: 70 bpm, Sat O₂ = 94%.

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Left Ventricular Rigid Body Rotation in Ebstein's Anomaly from the MAGYAR-Path Study

Attila Nemes et al. Kálmán Havasi Péter Domsik Anita Kalapos Tamás Forster

A 70-year-old female patient with Ebstein’s anomaly (EA) that had never undergone palliation was assessed (the case originates from the MAGYAR-Path Study). Complete two-dimensional (2D) Doppler and three-dimensional (3D) speckle-tracking echocardiography were carried out with commercially available Toshiba Artida^™ echocardiography equipment. During 2D echocardiography, the septal leaflet-tricuspid annulus distance showed to be 25 mm, confirming EA. While the right ventricle (RV) was enlarged with tricuspid annular plane systolic excursion > 23 mm and mitral regurgitation grade III, left ventricular (LV) size and function showed to be normal with an ejection fraction of 56% without wall motion abnormalities. However, all LV regions moved in almost the same counterclockwise direction, confirming absence of LV twist, called “rigid body rotation” (RBR) (). The mean global LV radial, circumferential, longitudinal, 3D and area strain parameters showed to be 11.5 ± 10.0%, -25.5 ± 15.4%, -18.6 ± 10.2%, 15.2 ± 10.8% and -34.7 ± 20.8%, respectively. EA is a congenital heart defect in which septal and posterior leaflets of the tricuspid valve are displaced towards the RV apex, leading to RV partial atrialization, although the anatomic annulus of the valve is in the normal position. Malformation and displacement of the anterior leaflet can also be present. To the best of our knowledge, this is the first report to demonstrate LV-RBR, a known feature in LV myocardial mechanics, in a single patient with unrepaired EA. LV-RBR could be partially explained by the impaired ventricle-to-ventricle interactions due to displaced tricuspid valve leaflet attachments, alterations in the anatomic myocardial fiber orientation, but other reasons could also not be excluded.

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Clinicoradiological Session

Case 1/2015 A 76-year-old Male Patient with Ebstein Anomaly in Natural Course

Atik Edmar

Laboratory tests

Electrocardiogram (ECG) showed atrial fibrillation, signs of right ventricular (RV) diastolic overload, and left anterior superior hemiblock. Low-voltage QRS complex with qr morphology in V1; RS in V6, with final conduction disturbance in the right bundle branch. QRSA: – 70º, TA: + 30° ().

Chest radiograph showed moderately enlarged cardiac silhouette (cardiothoracic ratio: 0.61), resulting from enlarged right chambers. The pulmonary vascular network was slightly decreased, the arch of the pulmonary artery was concave, and the aortic knob was prominent. ().

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Artigo Original

Cone reconstruction in Ebstein's anomaly repair: early and long-term results

José Pedro da Silva et al. Luciana da Fonseca da Silva Luiz Felipe P. Moreira Lilian Maria Lopez … Sônia Meiken Franchi Alessandro Cavalcante Lianza José Francisco Baumgratz Glaucia Maria P. Tavares

BACKGROUND: The main Ebstein anomaly (EA) repairs are based on the monocusp reconstruction of the tricuspid valve and are limited by the frequent need for replacement or the high recurrence of valve regurgitation. OBJECTIVE: To evaluate the feasibility and effects of anatomical repair of Ebstein’s anomaly using the cone reconstruction technique on patients’ clinical evaluation, tricuspid valve function and right ventricular morphology. METHODS: We compared the clinical, echocardiographic and radiological data of 52 consecutive patients, with a mean age of 18.5 ± 13.8 years, submitted to the cone reconstruction technique, obtained in the preoperative, early postoperative (EPO) and long-term (LPO) periods. RESULTS: There were two in-hospital deaths (3.8%) and two more during the follow-up. Mean functional class of pre-operative heart failure improved from 2.2 to 1.2 after 57 months of mean follow up of 97% of patients (p <0.001). The mean degree of preoperative tricuspid regurgitation decreased from 3.6 to 1.6 in the EPO (p 0.05). The indexed RV functional area increased from 8.53 ± 7.02 cm2/m2 preoperatively to 21.01 ± 6.87 cm2/m2 in the EPO (p 0.05). The mean cardiothoracic ratio was decreased from 0.66 ± 0.09 to 0.54 ± 0.06 (p <0.001) in the long term. CONCLUSION: The cone technique showed low in-hospital mortality, resulting in an effective and long-lasting repair of tricuspid regurgitation, restoring the functional area of the right ventricle and allowing reverse remodeling of the heart and clinical improvement in most patients in the long term.

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Implantation of a Post-Dilatable Stent in Aortic Coarctation Via Carotid Access in a Newborn With Ebstein's Anomaly: Case Report

Introduction

Oscillating Ductal Stent in Valvar Pulmonary Atresia

Case 5/2019 – Naturally Evolving Ebstein’s Anomaly of Discrete Repercussion in 24-Year-old Asymptomatic Adult

Clinical data

Left Ventricular Rigid Body Rotation in Ebstein's Anomaly from the MAGYAR-Path Study

Case 1/2015 A 76-year-old Male Patient with Ebstein Anomaly in Natural Course

Laboratory tests

Cone reconstruction in Ebstein's anomaly repair: early and long-term results

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