(IV-1D.1b) (English) Ebstein’s anomaly with bidirectional shunt through ASD Klima L., Dziedzic H., Rajzer M., Olszanecka A., Czarnecka D.

Keywords: Ebstein’s anomaly, bidirectional shunt, ASD

Background
Ebstein’s anomaly is a congenital heart defect that appears in 1 on 100 000 to 200 000 births. In this defect tricuspid valve is dislocated towards the apex of the right ventricle. The annulus of the valve is in the standard position and leaflets are attached to the walls and septum of the right ventricle. This leads to atrialization of a portion of the right ventricle. The right atrium is enlarged and the anatomic right ventricle is small in size. The ASD or PFO coexists in up to 90% of Ebstein’s anomaly cases [1]. These patients often have an accessory pathway and suffer from supraventricular tachycardia – 6 – 36% [2].

Case description
Our patient is a 50-year-old female with Ebstein’s anomaly diagnosed in childhood and ASD diagnosed after TIA episode in 2005, with cyanosis, secondary polycytheamia (history of hematocrit over 60% and bloodletting treatment) with supraventricular tachycardia. She was admitted to the 1st Dept. of Cardiology and Hypertension for electrophysiological examination that revealed an accessory pathway that was treated with catheter ablation. On admission she complained of dyspnea. Due to cyanosis, reduced saturation (Sat.O2 86% and 58% on exercise) and hypoxemia (pH 7.456; pCO2 32.3 mmHg; pO2 50.9 mmHg; HCO3 22.3 mmHg; BE – 0.7 mmol; O2 sat 88.3%) transesophageal contrast echocardiography was performed which revealed bidirectional shunt through ASD. To confirm that polycytheamia is connected with shunt the level of erythropoietin was measured (EPO 59.3 (normal values: 4.3 – 29.5 mIU/ml) and there was no mutation in JAK-2 V617F gene. To our surprise right ventricular catheterization did not reveal pulmonary hypertension (RV pressure 24/3 mmHg; RA pressure 27/10 mmHg; PCWP 10 mmHg; resistance < 1 Wood Units). The patient would be in our opinion a good candidate for right ventricular reconstruction surgery and tricuspid valvuloplasty combined with atrial septal defect surgical closure. We performed two more diagnostic procedures. Magnetic resonance imaging provided precise dimensions of cardiac chambers - LA 23.9 cm2, 68x31 mm; RA 35.0 cm2, 70x65 mm; RV 28.9 cm2, atrialized RV 15.6 cm2; LV EDD/ESD 40/21 mm; IVS 6 mm; PW 5 mm; LV EF 67.5%, EDV 120 ml, mass 36 g; MPA 25x25 mm, LPA 14x12 mm, RPA 12x14 mm. Computed tomography was performed to visualize the confluence of pulmonary veins and the anatomy of tricuspid valve leaflets - tricuspid annulus was enlarged 50x55 mm; septal leaflet moved towards apex 35 mm; septal leaflet was fused with intraventricular septum; anterior and posterior leaflets were fused together and created one „sail” leaflet 57 mm; ASD dimension 16 x 10 mm; CT-scan revealed the absence of aortic rim and the confluence of pulmonary veins was normal. Guidelines
Surgical treatment of Ebstein’s anomaly is recommended for symptomatic patients. Carpentier’s technique is one of the ways of tricuspid valvuloplasty [3]. This gives good results with post-surgery mortality of 9 and 30% at 10- and 20-year follow-up, respectively [4].

References
1. Danielson, G.K., Driscoll, D.J., Mair, D.D., et al.: Operative treatment of Ebstein’s anomaly. J Thorac Cardiovasc Surg, 1992; 104(5): 1195-202.
2. Urbanowicz, T., Katarzynski, S. and Jemielity, M.: [Patient with Ebstein anomaly treated by Carpentier’s technique tricuspid valve repair–a case report]. Kardiol Pol, 2010; 68(2): 186-9.
3. Carpentier, A., Chauvaud, S., Mace, L., et al.: A new reconstructive operation for Ebstein’s anomaly of the tricuspid valve. J Thorac Cardiovasc Surg, 1988; 96(1): 92-101.
4. Brown, M.L., Dearani, J.A., Danielson, G.K., et al.: The outcomes of operations for 539 patients with Ebstein anomaly. J Thorac Cardiovasc Surg, 2008; 135(5): 1120-36, 1136 e1-7.

Expert’s conclusions:
Tricuspid valve repair and atrial septal defect is indicated in this patient.

Authors:
Klima L.², Dziedzic H.¹, Rajzer M. ², Olszanecka A.¹, Czarnecka D.,

Experts:
Pająk J.MD, PhD³, Kopec G.MD, PhD¹, Prof. Podolec P.MD, PhD¹

¹ Department of Cardiac and Vascular Diseases, Jagiellonian University College of Medicine, John Paul II Hospital, Krakow, Poland
² 1st Department of Cardiology and Hypertension, Jagiellonian University Medical College
³ Independent Public Clinical Hospital No.6, Medical University of Silesia in Katowice, Upper Silesia Center of the children’s Health. John Paul II, Pediatric Heart Surgery Department, Poland