Ebstein’s Anomaly 5 - Springerextras.springer.com/2012/978-3-642-24168-0/DVD-2/05_Chap.pdf ·...

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V. Hraška, P. Murín, Surgical Management of Congenital Heart Disease I, DOI:10.1007/978-3-642-24169-7_5, © Springer-Verlag Berlin Heidelberg 2012

Ebstein’s Anomaly

Contents

◙ Introduction 184 ◙ Anatomy 184 ◙ Indication for Surgery 185

◙ Newborns and Young Infants 185 ◙ Older Patients 185

◙ Approach and Cardiopulmonary Bypass Strategy 186 ◙ Anatomical Repair – Cone Reconstruction 186

◙ The Goal of Surgery 186 ◙ Cone Reconstruction with Longitudinal Plication of the Atrialized Ventricle in the Newborn 188

◙ Patient Characteristics 188 ◙ Specific Steps of Operation 188

◙ Cone Reconstruction with Longitudinal Plication of the Atrialized Ventricle in the Infant 194

◙ Patient Characteristics 194 ◙ Specific Steps of Operation 194

◙ Cone Reconstruction without Plication of the Atrialized Ventricle 196 ◙ Patient Characteristics 196 ◙ Specific Steps of Operation 196

◙ Recommended Reading 201

184 V. Hraška, P. Murín

Introduction

Ebstein’s anomaly is a rare congenital heart malformation of the right ventricle and tricuspid valve. Failure of delamination of the tricuspid valve, especially of the posterior and septal leaflets, from the underlying myocardium, results in a variable degree of tricuspid valve and right ventricular deformity. The natural history is influenced by a high rate of intrauterine and neonatal death, especially when the anomaly is associated with severe deformity of the tricus-pid valve and cyanosis. At the other end of the spectrum, patients with mild dysfunction of the tricuspid valve can remain asymptomatic until late in adult life. Management therefore requires a choice between a conservative approach and operative intervention. Currently available surgical techniques have dem-onstrated survival and functional benefits to patients with a greater severity of Ebstein’s anomaly.

Anatomy

The anomaly is characterized by:

1. Tricuspid valve malformationsa. The septal and posterior leaflets are displaced towards the apex of the

right ventricle with a variable degree of adherence to the myocardium (spiral failure of delamination).

b. The sail-like, redundant anterior leaflet has a normal attachment to the tricuspid valve annulus, but is frequently fenestrated with chordal at-tachments to the ventricular wall and eventually, as well as linear at-tachments (fibrous and muscular attachments between the leaflet and myocardium).

2. The right ventricle is malformed and consists of:a. A thin-walled “atrialized” component, defined as a segment of the right

ventricle, from the level of the “true” tricuspid annulus to the level of displacement of the septal and posterior leaflets.

b. A thick-walled functional component (“true right ventricular cavity”), consisting of apical and outlet portion, often obstructed by chordal at-tachments of the anterior leaflet of the tricuspid valve. The inlet portion

1855 Ebstein’s Anomaly

is small or nonexistent. The cavity of the functional right ventricle is usu-ally smaller, containing fewer myocytes and more fibrous tissue than a normal right ventricle.

The most commonly associated anomaly is the atrial septal defect or persistent foramen ovale (in about 90% of cases). Survival in symptomatic newborns with severe obstruction to pulmonary outflow depends on the presence of a patent ductus arteriosus. Wolff–Parkinson–White syndrome is present in about 10% of cases. There is rarely association with an atrioventricular septal defect, a ventricular septal defect, tetralogy of Fallot, congenitally corrected transposi-tion of the great arteries, aortic coarctation, or other defects.

Indication for Surgery

Newborns and Young Infants

Before considering surgery in newborns, all measures are taken to lower pul-monary vascular resistance while preserving duct dependent pulmonary cir-culation. The critically ill patient requires mechanical ventilation, sedation, paralysis, hyperventilation, correction of metabolic acidosis, appropriate ino-tropic/volume management, and prostaglandin E1 infusion. After stabilization, when pulmonary vascular resistance begins to fall, the prostaglandin is stopped to allow ductal constriction. Subsequently, the true functional capability of the right ventricle in providing antegrade flow is evaluated. The indications for surgery are ventilator dependence, severe cardiac failure, and severe tricuspid insufficiency with prostaglandin-dependent circulation.

Older Patients

In older patients, surgery is indicated by symptoms of poor growth, decreased exercise tolerance (New York Heart Association functional classes III, IV), cyanosis (oxygen saturation <90%), progressive cardiomegaly (cardiothoracic ratio >0.6), reduced left ventricular function, tachyarrhythmias that are not controllable by medication or amenable to catheter-based intervention and sig-


nificant associated lesions. Timely operation is warranted before cardiomegaly develops and the functional status deteriorates. Currently, the operation can be considered in asymptomatic patients with low normal-exercise tolerance, no right-to-left shunting, and only mild cardiomegaly (cardiothoracic ratio ≤ 0.6). In children with mild symptoms but with significant enlargement of the right atrium due to massive tricuspid insufficiency, the risk of early rhythm distur-bances is so high that surgery should be considered.

Approach and Cardiopulmonary Bypass Strategy

The heart is approached through a median sternotomy. The standard tech-nique of cardiopulmonary bypass with mild hypothermia (32°C) is used. A left ventricular vent is inserted through the entrance of the right pulmonary veins.

Anatomical Repair – Cone Reconstruction

The Goal of Surgery

Surgical therapy for Ebstein’s anomaly should restore tricuspid valve compe-tence and it should address the inadequacy of the inefficient right ventricle. Especially in children, the tricuspid valve plasty with biventricular correction should take preference over tricuspid valve replacement or a single-ventricle pathway. Surgical repair consists of (1) reconstruction of the tricuspid valve, (2) consideration of plication or resection of the atrialized right ventricle, espe-cially when it is thin-walled and moves paradoxically, (3) closure of the atrial communication and correction of any associated anomalies, (4) surgery for arrhythmia, and (5) right atrium reduction atrioplasty. In special situations, the concept of 1½ ventricular repair or of a single-ventricular pathway should be adopted. Heart transplantation is the therapy of last resort. Reconstructive sur-gery is based either on the principle of anatomical repair (cone reconstruction) or on monoleaflet repair.

The cone reconstruction moves the new tricuspid valve orifice to the true valve annulus, restoring the normal anatomical location of the tricuspid valve,


resulting in central diastolic blood flow and full coaptation of the leaflets. The normalization of the right ventricular volume and morphology is achieved by longitudinal plication of the atrialized right ventricle.

The important operative steps are as follow:

1. “Correction” of failure of delamination The anterior and posterior tricuspid valve leaflets are detached from the

hinge-point and mobilized from their anomalous attachments in the right ventricle. Only the normal attachments of the anterior leaflet to the true tri-cuspid annulus and the proper subvalvular apparatus are left in place.

2. Creating a cone The free edge of this valve complex is rotated clockwise for suture to the

septal border of the anterior leaflet, thus creating a cone, the vertex of which remains fixed at the right ventricular apex.

3. Exclusion of the atrialized right ventricle The atrialized part of the right ventricle is longitudinally plicated to exclude

its thin part. 4. Attachment of the tricuspid valve to the annulus

The new valvular annulus is constructed at the anatomically correct level by means of plication of the true tricuspid annulus to match the proximal circum-ference of the cone-shaped valve. The base of the cone is sutured to the new valve annulus.

Additionally, the atrial septal defect is closed in a valved fashion. The re-dundant right atrium is excised from each side of the atriotomy and then the atriotomy is closed.


Cone Reconstruction with Longitudinal Plication of the Atrialized Ventricle in the Newborn

Patient Characteristics

Age at operation: 30 daysDiagnosis: 1. Ebstein’s anomaly2. Secundum atrial septal defect3. Patent ductus arteriosus4. Hydrops fetalis5. Supraventricular reentry tachycardia 6. Bilateral chylothoraxHistory:1. Prenatally diagnosed, born by Cesarean section2. Intubated and ventilated, administration of

prostaglandins, otherwise cyanotic3. On continuous positive-airway pressure for a

short time; placement of peritoneal catheter4. Reintubated because of circulatory collapse

due to supraventricular tachycardia

5. Resuscitated because of ventricular tachycar-dia, with subsequent placement of temporary intravenous pacemaker wires

6. Placement of temporary epicardial wires to re-place intravenous ones, which were unreliable

7. Resuscitation and subsequent extracorporeal membrane oxygenation support for 7 days

8. Operation indicated due to failure to wean pa-tient from extracorporeal membrane oxygen-ation support.

Procedure:1. Cone reconstruction with longitudinal plication

of the atrialized portion of the right ventricle2. Partial closure of the atrial septal defect3. Downsizing of the right atrium4. Patent ductus arteriosus closure

Specific Steps of Operation

Clip1

Preoperative findings.

Hans

Schreibmaschinentext


Clip2

Assessment of anatomy.

Clip3

Detachment of the leaflets of the tricuspid valve.


Clip4

“Correction” of failure of delamination.

Clip5

Creating a cone.


Clip6

Exclusion of the atrialized right ventricle.

Clip7

Attachment of the tricuspid valve to the annulus.


Clip8

Plication of the annulus.

Clip9

Final reattachment of the base of the cone. In ad-dition, the atrial septal defect is partially closed, leaving a 3-mm fenestration. The redundant right atrium is excised from each side of the atriotomy, and then the atriotomy is closed.


Clip10

The echocardiogram before discharge.

fullversion


Cone Reconstruction with Longitudinal Plication of the Atrialized Ventricle in the Infant


Age at operation: 6 monthsDiagnosis: 1. Ebstein’s anomaly2. Secundum atrial septal defectHistory:1. Prenatally diagnosed, postnatal adaptation

without difficulties2. Gradual progression of cyanosis and failure to

thrive

Procedure:1. Cone reconstruction with longitudinal pli-

cation of the atrialized portion of the right ventricle

2. Direct closure of the atrial septal defect3. Downsizing of the right atrium


Fig. 5.1. Preoperative echocardiogram. The preoperative echocardiogram shows the displace-ment of the septal leaflet and the size and position of the anterior leaflet of the tricuspid valve (a). The Doppler echocardiogram shows moderate tricuspid regurgitation (b). RA right atrium, ARV atrialized right ventricle, AL anterior leaflet, SL septal leaflet


Fig. 5.2. Intracardiac anatomy. There is severe displacement of the posterior and septal leaf-lets (a). Vessel loops surround the fibrous and muscular attachments of the leading edges of the leaflet to the underlying myocardium. Note the atrialized portion of the right ventricle and position of the right coronary artery (b). RCA right coronary artery, AL anterior leaflet, PL posterior leaflet, SL septal leaflet, ARV atrialized right ventricle

Fig. 5.3. Creating a cone. All fibrous and muscular attachments between the body of the leaf-lets and the right ventricular myocardium are incised. The cone reconstruction is completed by rotating the posterior leaflet clockwise to meet the proximal edge of the septal leaflet. AL anterior leaflet, PL posterior leaflet, SL septal leaflet


Cone Reconstruction without Plication of the Atrialized Ventricle


Age at operation: 3.5 yearsDiagnosis: 1. Ebstein’s anomaly2. Secundum atrial septal defectHistory: Gradual progression of cyanosis and failure to thrive

Procedure:1. Cone reconstruction without plication of the

atrialized portion of the right ventricle2. Direct closure of the atrial septal defect3. Downsizing of the right atrium


Clip1

Assessment of the extra- and intracardiac anatomy.


Clip2

Detachment of the leaflets of the tricuspid valve.

Clip3

Detachment of the linear attachments of the poste-rior leaflet.


Clip4

Detachment of the septal leaflet.

Clip5

Creating a cone.


Clip6

Attachment of the tricuspid valve to the annulus.

Clip7

Lateral annuloplasty.


Clip8

Atrial septal defect closure and reduction atri-otomy.

Clip9

The echocardiogram before discharge.

fullversion


Recommended ReadingAttenhofer Jost CH, Connolly HM et al (2007) Ebstein’s anom-aly. Circulation 115:277–285

Badiu CC, Schreiber C, Horer J et al (2010) Early timing of sur-gical intervention in patients with Ebstein’s anomaly predicts su-perior long-term outcome. Eur J Cardiothorac Surg 37:186–192

Brown ML, Dearani JA, Danielson GK et al (2008) The out-comes of operations for 539 patients with Ebstein anomaly. J Thorac Cardiovasc Surg 135:1120–1136

Chauvaud S, Carpentier A (2007) Ebstein’s anomaly: the Brous-sais approach. MMCTS. doi:10.1510/mmcts.2007.003038

Dearani JA, Danielson GK (2005) Surgical management of Ebstein’s anomaly in the adult. Semin Thorac Cardiovasc Surg 17:148–154

Dearani JA, O’Leary PW, Danielson GK (2006) Surgical treat-ment of Ebstein’s malformation: state of the art in 2006. Cardiol Young 16:12–20

Dearani JA, Bacha E, da Silva JP (2008) Cone reconstruction of the tricuspid valve for Ebstein’s anomaly: anatomic repair. Oper Tech Thorac Cardiovasc Surg 13:109–125

Ebstein W (1866) Über einen sehr seltenen Fall von Insuffizienz der Valvula tricuspidalis, bedingt durch eine angeborene hoch-gradige Missbildung derselben. Arch Anat Physiol 7:238–254

Knott-Craig CJ (2008) Management of neonatal Ebstein’s anomaly. Oper Tech Thorac Cardiovasc Surg 13:101–108

Knott-Craig CJ, Goldberg SP, Overholt ED et al (2007) Repair of neonates and young infants with Ebstein’s anomaly and re-lated disorders. Ann Thorac Surg 84:587–593

O’Leary PW, Dearani JA, Anderson RH (2010) In: Anderson RH, Baker EJ, Penny D et al (eds) Pediatric cardiology, 3rd edn. Churchill-Livingstone, London, pp 713–730

Reemtsen BL, Fagan BT, Wells WJ et al (2006) Current surgical therapy for Ebstein’s anomaly in neonates. J Thorac Cardiovasc Surg 132:1285–1289

Sano S, Ishino K, Kawada M et al (2002) Total right ventricular exclusion procedure: an operation for isolated congestive right ventricular failure. J Thorac Cardiovasc Surg 123:640–647

Silva da JP, Baumgratz JF, da Fonseca L et al (2007) The cone reconstruction of the tricuspid valve in Ebstein’s anomaly. The operation: early and midterm results. J Thorac Cardiovasc Surg 133:215–223

Ullmann MV, Born S, Sebening C et al (2004) Ventricularization of the atrialized chamber: a concept of Ebstein’s anomaly repair. Ann Thorac Surg 78:918–924

Wald RM, Tham EB, McCrindle BW et al (2007) Outcome after prenatal diagnosis of tricuspid atresia: a multicenter experience. Am Heart J 153:772–778

Wu Q, Huang Z (2004) A new procedure for Ebstein’s anomaly. Ann Thorac Surg 77:470–476

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