Double Outlet Right Ventricle Seoul National University Hospital Department of Thoracic & Cardiovascular Surgery

Double Outlet Right Ventricle 1. Definition A congenital cardiac anomaly in which both great arteries rise wholly or in large part from the right ventricle. It is then, a type of ventriculoarterial connection. 2. History Taussig-Bing heart : Described in 1949 Braun : Case of DORV+PS in 1952 Mayo group : 1st repair in 1957 Lev : Clarified Taussig-Bing in 1972

Double Outlet Right Ventricle Pathophysiology Both great arteries arise from the right ventricle in association with a nonrestrictive VSD. The pathophysiology depends on the position of the VSD & the presence/absence of pulmonary stenosis. Left-to-right shunting across the VSD results in pulmonary overcirculation, pulmonary hypertension, and congestive heart failure. Pulmonary stenosis results in right-to-left shunting and cyanosis.

Hemodynamics of DORV

Double Outlet Right Ventricle Pathophysiology

DORV Angiography

Morphology of DORV 3. Great arteries 1. VSD; usually large . Normal or near-normal . D-malposition . L-malposition 4. Pulmonary stenosis 5. Conduction system 6. Coronary arteries 7. Associated anomalies PS, CoA, PDA, SubAS, A-V canal, multiple VSD in 30 % 1. VSD; usually large 10 % small rarely none . Subaortic . Subpulmonary . Doubly committed . Noncommitted or remote 2. Infundibulum . Absent . Single . Bilateral in general

Locations of VSD in DORV a ; Subaortic b ; Subpulmonic c ; Doubly committed d ; Noncommitted

DORV with Subaortic VSD Aorta VSD

DORV with Subpulmonic VSD Aorta PA VSD

Taussig-Bing Heart PA Aorta PA

Double Outlet Right Ventricle Morphologic Syndromes 1. Simple DORV 2. Taussig-Bing Heart 3. DORV with doubly committed VSD 4. DORV with noncommitted VSD 5. DORV with L-malposition 6. DORV with complete ECD 7. DORV with superior-inferior ventricles

Clinical Features & Diagnosis 1. Incidence ; less than 1% of CHD 2. Pathophysiology . Variable according to streaming of blood flow, PS, PVD . Always some arterial desaturation 1) Streaming of blood flow (relationship of semilunar valve to the VSD) 2) Pulmonary vascular disease (more rapid onsets without PS) 3) Pulmonary stenosis (severe cyanosis in important PS ) 3. Examination . No definite clinical signs with or without PS . EKG, chest radiography : not diagnostic 4. Echocardiography 5. Cardiac catheterization & cineangiography

Double Outlet Right Ventricle Natural History 1. Simple DORV : similar to simple large VSD 2. Taussig-Bing heart : similar to TGA+VSD, but more unfavorable 3. DORV+PS or atresia :similar to TOF or atresia Natural history in some patients is dominated by an associated cardiac anomalies such as AV canal defect.

Operative Techniques 1. Intraventricular tunnel repair . Simple DORV . Subaortic VSD & PS . Doubly committed . Noncommitted . Taussig-Bing heart 2. Switch repair ( arterial, atrial ) 3. Rastelli or Lecompte repair . With intraventricular tunnel repair 4. Nikaidoh aortic translocation 5. Total cavopulmonary connection . Noncommitted VSD with PS 6. Palliative operations

Surgical Strategy of DORV Intraventricular Tunnel Relationship between VSD , PV & Aorta

REV (Lecompte) Operation

Intraventricular Tunnel Repair Taussig-Bing Heart

Rastelli Operation Taussig-Bing Heart

Taussig-Bing Heart with COA Combined aortic arch repair and arterial switch without coronary reimplantation

Surgical Results of DORV 1. Survival . Early deaths . Time-related survival 2. Risk factors for premature death 1) Age at repair 2) Type of DORV 3) Associated cardiac anomalies 4) Surgical era 5) Type of operation . Atrial switch operation . Complex tunnel repair . Transannular patch or conduit 3. Complications of intraventricular tunnel repair . Leakage . Obstruction

Operative Indications 1. Simple DORV with subaortic VSD . Without PS : 6 months of life . With PS : same as TOF 2. DORV with subpulmonary VSD . Without PS : 1st month of life . With PS : 2 to 4 years of age 3. DORV with doubly committed VSD . Same as simple DORV 4. DORV with noncommitted VSD . VSD enlargement & tunnel . Extracardiac conduit repair . LeCompte procedure . Fontan-type repair

Double Outlet Left Ventricle Seoul National University Hospital Department of Thoracic & Cardiovascular Surgery

Double Outlet Left Ventricle Definition A cardiac anomaly in which both great arteries arise from the left ventricle. DOLV may occur with atrioventricular concordant or discordant connection. History Marechal : 1st description in 1819 Sakakibara : 1st intraventricular repair in 1967 Paul : Unique case of DOLV+ IVS in 1970

Morphology of Double Outlet LV VSD Usually, large & most commonly subaortic Some juxtaaortic, subpulmonic, juxtaarterial (double outlet both ventricle) or some overriding Conal pattern Most often absent subaortic conus & subpulmonic conus is displaced to LV Rarely bilateral absent conus, Very rarely only subaortic conus Pulmonary stenosis Present most, either valvular or subvalvular Right ventricle & TV ; tendency & somewhat hypoplastic Left ventricle : normal Conduction system: normal

Double Outlet Left Ventricle Models of 4 basic hearts as they occur in double outlet LV

Double Outlet Left Ventricle

Clinical Features & Diagnosis Pathophysiology 1. LV is a common mixing chamber, receiving systemic & pulmonary venous blood 2. Clinical presentation is by severe cyanosis from frequent occurrence of pulmonary stenosis 3. Tendency to develop cyanosis is more severe in AV discordant connection Diagnostic examination 1. Physical finding, chest X-ray, EKG; not diagnostic 2. Echocardiography 3. Cardiac catheterization & cineangiography

Double Outlet Left Ventricle Cineangiograms of normally positioned heart and AV concordant connection, DOLV, subaortic VSD and PS

Natural History Incidence Very rare Natural history with DOLV without PS appears to be similar to that of the patients with isolated large VSD and progressive narrowing of VSD & closure has not documented Natural history with DOLV with PS appears to be similar to that of patients with TOF and degree of hypoxia and clinical course are related to severity of PS

Techniques of Operation Identification of morphology Repair of DOLV and atrioventricular concordant connection 1. With pulmonary stenosis 2. Without pulmonary stenosis DOLV with atrioventricular concordant connection and important hypoplasia of RV and TV

Repair of DOLV + VSD + PS

Operative Indications for DOLV Diagnosis is an indication for operation In the absence of pulmonary stenosis, corrective operation should be performed in the first 6 months of life, or pulmonary artery banding and subsequent delayed repair at age 1-2 years or beyond 2 years, if morphology is more challenging In the presence of pulmonary stenosis, this approach are similar to those for Tetralogy of Fallot When there is right ventricular or tricuspid valve hypoplasia, Fontan operation should be considered