Introduction
Most common cyanotic congenital anomaly
Incidence 3.26 per 10,000 live births (about 1300 new
of the infundibular septum. Malaligned ventricular septal defect (VSD). Primary problem in tetralogy of Fallot is underdevelopment of the pulmonary infundibulum Right ventricular hypertrophy: systemic right ventricular systolic pressure.
This constellation Tetralogy of Fallot 7-10% of congenital heart lesions Almost equal in males and females (slight male >)
Natural History
Approximately 25% of untreated patients with TOF
and RVOT obstruction die within the first year of life, 40% by 4 years, 70% by 10 years, and 95% by 40 years
TOF
1. Pulmonary Stenosis
2. Right Ventricular Hypertrophy
ASSOCIATIONS
15% have extra-cardiac anomolies Includes chromosomal abn like Downs, Di George and
velocardiofacial syndromes (del chr 22), Alagille syndrome (mutations in Jagged1) Other genetic, non-syndromic abn have been documented in patients with TOF, including mutations in transcription factor NKX2.5 in approximately 4 % In 70% of TOF patients genetic etiology is unidentified TOF is frequently associated with
Fetal hydantoin syndrome Fetal carbamazepine syndrome Fetal alcohol syndrome Maternal PKU birth defects
Variants
Simple tetralogy of Fallot with pulmonary stenosis
(61%)
Tetralogy of Fallot with pulmonary atresia (33%) Tetralogy of Fallot with absent (or dysplastic)
(3%)
restless
respirations
Sedation (Morphine)
Intubation/paralysis Na bicarbonate Phenyephrine
RX AP
Boot or Wooden Shoe Hypertrophied RV causes a right apical shadow Aorta is large and it may arch to the right.
EKG
Right axis deviation Right or combined ventricular hypertrophy Right atrial hypertrophy (prominent p waves in V1) Partial or complete right bundle branch block
Most commonly seen after surgical repair
TOF Treatments
Infant: Maintain PDA with IV PgE1 (0.05-0.2
ANGIOGRAMS
LV angio large VSD (*), Aorta is over-riding the interVentricular septum (IVS)
RV angio subvalvar obstruction is so severe that with systole there is no contrast in RVOT. Small ridge of supravalvar narrowing
SURGICAL MANAGEMENT
Palliation BTS in 1945. now been modified using the
Gortex tube to create the connection b/w Subclavian Artery and Pulmonary Artery.
Still used in infants not good candidates for intracardiac repair
infundibular muscle bundles, occ transannular patch. Disadv of transannular patch makes Pulm Valve incompetent. Alternately, insertion of a valved conduit from RV to distal MPA can be performed.
TOF Treatments
Blalock-Taussig Shunt
Shunt from R Subclavian Artery R Pulmonary Artery Increase Pulmonary flow Result in Higher O2 Sat.
ring. Digoxin
SURGICAL MANAGEMENT
POP Care
Evaluate for residual VSD, RV dysfunction and RVOT
obstruction Evaluate for arrhythmia ( JET, Ventricular ectopy, heart block) Always keep good preload
RV dysfunction
High CVP
Low CO, high lactate Cyanosis
Echo
Cannon waves
Low CO, high lactates
JET
JET management
Correct hypovolemia, anemia and electrlyte imbalance
Hypothermia or at least correction of fever. Cooling
blankets, ice bags (sedation and paralysis required) Reduction in cathecolamines Atrial pacing Meds: Digoxin, Amiodarone
Calcium drip
Keep good preload
heart failure. Myocardial fibrosis has adverse effect on long-term outcomes. In a study describing 36 yr outcomes of 490 survivors mortality increased 25 yrs after sx from 0.24 to 0.94/yr Euro Heart Survey in adults with TOF 1.3% mortality at 5yrs. Since 1985, major changes in mgmt of TOF such as repair in infancy and improved surgical outcome are expected to improve long-term outcomes.
from a cows jugular vein valve that is sewn into a small metal stent
there is complete obstruction of the Pulmonary Artery resulting in total diversion of blood from the right ventricle into the aorta. Survival depends on the presence of a number of naturally occurring connecting blood vessels between the Aorta and the Pulmonary Arteries in the lungs, which are called 'Collaterals' - or sometimes MAPCAs (Major Aorto-Pulmonary Collateral Arteries).
Surgical Options
Surgery
Rv to PA homogrft or conduit
Unifocalization VSD closure
Prognosis
High Mortality with or without surgery
Multiple cardiac catheterizations Staged Surgeries
Airway compromise
Increase PVR risk of PHT