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Single Ventricle Anomalies

Single Ventricle Anomalies

1. Definition

A. Single dominant ventricle of R, L, or ? morphology
B. Variable atrio-ventricular connection
C. Rudimentary ventricle nearly always present
D. Pulmonary blood flow increased, decreased, or balanced

2. Morphologic Subsets

A. Tricuspid Atresia
1) Absent RA to RV communication
2) Ventricle of LV type
B. Double Inlet Left Ventricle (DILV)
1) Both atria connected to single dominant ventricle
2) Ventricle of LV type
C. Mitral Atresia
1) Absent LA to LV communication
2) Ventricle of RV type

3. Palliative Procedures

A. Systemic - PA shunt - Pulmonary stenosis or atresia
B. Pulmonary artery band for CHF
1) Subaortic stenosis in 30 - 40%
C. Open or closed atrial septectomy
D. Bi-directional Glenn 4 - 8 months

4. "Definitive" Operations

A. Septation
1) Few truly suitable
B. Fontan procedure
2) Systemic venous to PA anastomosis
C. Fenestrated Fontan procedure
1) Calibrated opening from RA to LA
2) Applicable in marginal candidates
3) Lower perioperative morbidity
D. Extra-Cardiac Fontan Procedure
1) Tube from IVC to RPA
E. Cardiac transplantation

5. Tricuspid Atresia

A. Classification
B. Type I (70%)
1) Normally related great vessels
C. Type II (30%)
1) D - transposition
D. Subsets
1) A - pulmonary atresia
2) B - pulmonary stenosis
3) C - normal pulmonary valve

6. Clinical Diagnosis/Features

A. Cyanosis
1) Type IA, IB Tricuspid atresia
2) Some DILV
B. Congestive failure (subaortic stenosis)
1) Type IIC Tricuspid atresia
2) DILV
C. Balanced - mild cyanosis
D. Echocardiography very useful
E. Cardiac catheterization prior to operation

7. "Ideal" Fontan Candidate

A. Age > 2 years
B. LV morphology
C. Mean PAP < 15 mmHg
D. PVR < 2 units
E. Large unobstructed PAs
F. No ventricular hypertrophy
G. Normal ventricular function (EF > 50%)
H. LVEDP < 12mm Hg

8. Results of Definitive Surgery

A. Septation
1) Operative mortality = 30%
2) Heart block common
3) Rarely performed
B. Fontan
1) Operative mortality = 5%
2) 15 year survival = 60-70%
C. Fenestrated Fontan
1) Operative mortality = 5%
2) Low morbidity
D. Extra-Cardiac Fontan
1) Operative mortality = 5%
2) Lower incidence arrhythmias
E. Cardiac transplantation
1) Operative mortality =10-15%
2) Functional results good

9. Fontan Physiology

A. RA pressure = 12-18 mmHg
B. Low systemic output
C. Pleural effusions common
D. Protein losing enteropathy = 5-10%
E. Long-term deterioration in ventricular function

10. Single ventricle anomalies - Summary Plan

A. 0-6 months
1) Relief of CHF/cyanosis
2) PA banding, shunt
B. 4-8 months
1) Bi-directional Glenn
2) Relief of subaortic stenosis
3) Relief of pulmonary artery distortion
C. 12-24 months
1) Fenestrated Fontan or Fontan
D. Cardiac transplant for late failure of Fontan