DGPK guidelineDouble Outlet Right Ventricle

(DORV)

H. Bertram, MHH, HannoverJ. Weil, UKE, Hamburg

J. Sachweh, UKE, Hamburg

DGPK guideline committee

Guideline DORV Definition

Double Outlet Right Ventricle (DORV) represents a spectrum of congenitally malformed hearts

in which the circumference of both arterial valves, or the greater part of both circumferences,

are supported by the right ventricle

100 % + 100 % 100 % + > 50 %

prevalence: • 1,3 % of cardiac defects • 1,1 / 10.000 live births (PAN)

Guideline DORV Definition

Mahle WT et al. Cardiol Young 2008; 18(Suppl. 3): 39–51

> 150 % rule

Guideline DORV Definition

Double Outlet Right Ventricle (DORV) represents a spectrum of congenitally malformed hearts

in which the circumference of both arterial valves, or the greater part of both circumferences,

are supported by the right ventricle

• same ventriculo-arterial connection, but variations in - infundibular morphology - arterial interrelationship - coronary arterial anatomy

• any arrangement of the atrial appendages, or situs

• any atrioventricular connection

• multiple combinations of associated malformations

spatial relationship of the semilunar cusps in hearts with DORV

Guideline DORV

Guideline DORV

Mahle WT et al. Cardiol Young 2008; 18(Suppl. 3): 39–51

‚Interventricular communication‘ vs ‚VSD‘

Guideline DORVDiagnostics

Goal: displaying cardiac anatomy with emphasis on potential surgical biventricular repair (feasibility of tunneling the interventricular communication to one or other arterial trunk)

• position, size, interrelationship, course of the great arteries• morphology and size of the interventicular communication / the VSD in relation to diameter of the aortic valve• location and severity of a subpulmonary or subaortic obstruction• morphology and size of both ventricles and AV-valves

Methods:

• Echocardiography • Angiography (Cath./MRT/CT)

Guideline DORV

Classification of the interventricular communication / VSD according to its location in relation to the great arteries

• subaortic (65%)

• subpulmonary (20-25%)

• doubly committed (3%)

• non committed (7%)

subaortic VSD• size in relation to the diameter of the aorta• distance between VSD and aortic valve• presence and severity of a subpulmonary obstruction• subcostal coronal and sagittal planes; parasternal long axis

Guideline DORVsubaortic VSD

Guideline DORVsubaortic VSD

parasternal long axis

AoAo

LV LA

Guideline DORVsubaortic VSD

Subcostal TEE

Ao

RV

PA

RV

RV

Ao

Ao

subaortic VSD with severe subpulmonary obstruction

Guideline DORVsubaortic VSD

PA

Guideline DORV

doubly committed VSD• size and distance of the VSD to the aorta / pulmonary artery• presence and severity of a subpulmonary obstruction• subcostal coronal and sagittal planes

Guideline DORV

subpulmonary VSD• size in relation to the diameter of the pulmonary artery• presence and severity of a subpulmonary obstruction• subcostal coronal and parasternal long axis planes

Guideline DORVsubpulmonary VSD

RV

RV

PA

PA

Guideline DORVsubpulmonary VSD

PA

RV

Guideline DORVsubpulmonary VSD

Taussig-Bing malformation:

• DORV with subpulmonary VSD

• semilunar valves side-by-side

• no subpulmonary obstruction

• semilunar valves and AV-valves separated by conal septum

Guideline DORV

non-committed VSD• location and size; distance to semilunar valves• presence and severity of a subpulmonary obstruction• subcostal coronal / 4 C views

LVAo

Ao

PARV

RV

LV

Guideline DORVnon-committed VSD

Guideline DORVnon-committed VSD

TOF - type

subaortic VSD

Double Outlet Right Ventricle

valvular / subvalvular PS

biventricular repair 1-6 mo(VSDclosure)

Malposition of the great arteries, which arise completely (100% + 100%) or with the greater part of their circumference (100% + > 50%) from the right ventricle

‚doubly committed‘ ‚non committed‘subpulmonary VSD AVSD / heterotaxy

pulmonary hyperperfusion;congestive heart failure

reduced lung perfusion;mild severe cyanosis

TGA - typeVSD - type

severe cyanosis; congestive heart failure

biventricular repair < 1 mo(VSD-closure + arterial switch)

biventricular repair 4-12 mo(VSD closure + relief of RVOTO)

complex DORV

clinical symptoms determined by concommittant malformations

interventricular communication in relation to the great arteries

clinical symptoms

clinical subtype

biventricular repair 2-6 years(complex intracardiac tunneling

+/- VSD incision / arterial switch) or

definitive univentricular palliation

surgical strategy

concommittant malformations

aortic coarctation (in ~ 50 %)

• right atrial isomerism• TAPVD • l-SVC• subpulmonary obstruction

subpulmonary obstructionvalvular / subvalvular PS

Guideline DORV

Guideline DORV

medical treatment• PG E in duct dependent pts with severe subpulmonary obstruction

• diuretics, ß-blockers, … in pts with pulmonary hypercirculation and congestive heart failure

catheter intervention• TOF-type: balloon valvuloplasty; ductal stent; RVOT stent

• TGA-type: BAS

surgical palliation• TOF-type: modified BT-shunt if primary repair is not suitable or considered ‚high risk‘

• PAB in ncVSD to delay complex intraventricular repair

Guideline DORV Surgical Repair

VSD - type

• VSD closure in the age of 1 to 6 months - some pts need enlargement of the VSD (> 4/5 aortic annulus); cave: AV-Block

TOF - type

• VSD closure and relief of subpulmonary obstruction in the age of 4 to 12 months +/- muscular and transjunctional incision or patch enlargement

TGA - type

• neonatal corrective surgery with arterial switch, VSD closure +/- resection of aortic coarctation / aortic arch reconstruction +/- resection of subaortic infundibulum cave: coronary artery anomalies

• alternatively ‚Rastelli - type repair‘: baffling of the left ventricle to both arterial valves and placement of a conduit from RV to the pulmonary trunk

Guideline DORV Surgical Repair

Subpulmonary VSD with valvular/subvalvular pulm. stenosis

• ‚Kawashima-OP‘ • ‚Rastelli-OP‘

• ‚REV-procedure‘ (Reparation a l‘ Etage Ventriculaire)

• ‚Aortic translocation‘ – ‚Nikaidoh-procedure‘

Complex DORV

• biventricular repair aged 2-6 years: complex intraventricular baffling (LV Ao/PA) +/- VSD enlargement +/- arterial switch

• definitive functionally univentricular palliation

Guideline DORV Surgical Repair

Guideline DORV Surgical Repair

Percent of Great Vessels Arising from the RV

DORV 200 % > 150 % technical difficulty

‚VSD – type‘ no yes simple

‚Fallot – type‘ no yes average

‚TGA – type‘ no yes important

DORV-AVSD yes yes major

DORV ncVSD yes yes major

Implications of the 200 % rule (‚true‘ DORV)

Modified from: F. Lacour-Gayet: Intracardiac Repair of Double Outlet Right Ventricle Semin Thorac Cardiovasc Surg Pediatr Card Surg Ann 2008;11:39-43

Guideline DORV

prognosis

• biventricular repair achievable in most pts

• increased operative risk determined by concommittant malformations: • aortic arch obstructions • AV-valve anomalies • coronary arterial anomalies • LV hypoplasia • multiple VSDs

Residual lesions requiring reinterventions after surgical repair

Guideline DORV

• Depending on different morphology and type of previous surgical repair

• TOF – type: - pulmonary valve incompetence - residual subpulmonary obstruction

• RV-PA-conduit: definitive reoperation for conduit replacement (stenosis, incompetence, size-mismatch in growing children)

• complex intracardiac baffling: subaortic obstruction biventrcular surgical repair has a much higher rate of reintervention than a strategy of functionally univentricular palliation

• Life-long follow-up by pediatric cardiologists and subsequently specialists for adult congenital heart disease is mandatory

Guideline DORV

Follow-up