Tetralogy of Fallot

Dr. Rezwanul Hoque Bulbul

MBBS,MS, FCPS, FRCSG, FRCSEd Associate Professor Dept. of Cardiac surgery Bangabandhu Sheikh Mujib Medical University Dhaka, Bangladesh

Historical background
Stensen(1672) described first Arthur fallot(1888) gave vivid description Tetralogy includes 1.Stenosis of the pulmonary artery 2.Interventicular communication 3.Deviation of the origin of the aorta to right 4.Concentric hypertrophy of the right ventricle

History of surgical management of TOF

1944 Blalock Taussing native systemic-to-pulmonary shunt 1946 Potts - LPA to descending aorta anastomosis 1948 Sellors & Brock - closed pulmonary valvulotomy 1954 Lillehei - complete repair for 10 yrs old patient with cross CPB 1955 Kirklin - complete repair with CPB 1955 Davidson - central aorto-pulmonary anastomosis 1956 Lillehei - transanular patching 1960 mosov - first complete repair of TOF in Ukraine 1962 Waterston - RPA to ascending aorta anastomosis 1962 Klinner - de Leval - prosthetic systemic-to-pulmonary shunt 1969 Barrat-Boys early primary complete repair

Recent concepts
Van Praagh et al considers TOF is in reality a single pathology, which is the result of hypoplasia or incomplete development of the infundibulum of the right ventricle and which covers a wide spectrum of types of obstruction of the right ventricular outflow tract.

Schematic representation of the rotation of the truncoconal septum

1/2 1/2 2/3 1/3

Displaced toward the pulmonary side In tetralogy of Fallot Angelini P Texas Heart Institute Journal Normal 180
1995;22:1-12

The embryological basis of the combination of lesions is antero-cephalad deviation of the developing outlet ventricular septum, or its fibrous remnant should this septum fail to muscularise. The combination of the deviated outlet septum and the hypertrophied septoparietal trabeculations produce the characteristic right ventricular outflow tract obstruction of tetralogy of Fallot . The deviation of the muscular outlet septum is also responsible for creating the malalignment type ventricular septal defect, and results in the aortic override. The associated hypertrophy of the right ventricular myocardium is the haemodynamic consequence of the anatomical lesions created by the deviated outlet septum.

Morphology

The antero-cephalad deviation of the outlet septum, coupled with an anomalous relationship to the septoparietal trabeculations, results in a narrowing of the subpulmonary outflow tract. The obstructive muscular subpulmonary area thus created is a dynamic entity. The degree of stenosis created can be exacerbated by catecholamines, or a state of low intravascular volume, predisposing the patients to sudden and acute episodes of desaturation known as hypercyanotic spells . The obstruction to flow into the lungs often extends beyond the subpulmonary outflow tract itself. The pulmonary valve may be hypoplastic, with abnormally functioning leaflets, often having a bifoliate configuration. Not infrequently, the pulmonary trunk, and the right and left pulmonary arteries, are diminutive, exhibiting additional focal areas of narrowing .

Pulmonary stenosis

Overriding of Aorta
Because of the displacement of the malaligned outlet septum into the right ventricle, the aortic root, of necessity, overrides the muscular ventricular septum. In the setting of significant subpulmonary obstruction, shunting across the interventricular communication is predominantly from right-to-left, which promotes ejection of deoxygenated blood into the systemic circulation. The chronic volume load sustained by the overriding aorta is implicated in the dilation of the aortic root noted in adults with tetralogy of Fallot

Interventricular communication
The VSD exists because of the anterior and cephalad malalignment of the outlet portion of the muscular ventricular septum, or of its fibrous remnant should the outflow cushions fail to muscularise during embryonic development. The resulting hole is one of a number of those appropriately described as a malalignment defect. Perimembranous VSD- 4/5 th Caucasians Muscular VSD- 1/5 th Caucasians

Spectrum of severity of disease

Hierarchical level-1
1. 2. 3. 4. TOF, PULMONARY STENOSIS (PS) TOF, PULMONARY ATRESIA TOF, COMMON ATRIOVENTRICULAR CANAL (AVSD) TOF, ABSENT PULMONARY VALVE.

Hierarchical level-3 Modifiers for TOF,Valvular stenosis Decisions as to whether or not to perform transannular patch based on the z-score of the pulmonary annulus.
Pulmonary valve annulus Z score 1 or larger Pulmonary valve annulus Z score 0 Pulmonary valve annulus Z score -1 Pulmonary valve annulus Z score -2 Pulmonary valve annulus Z score -3 Pulmonary valve annulus Z score 4 or smaller

Hierarchical level-2
Tetralogy of Fallot, NOS Tetralogy of Fallot, Pulmonary stenosis, NOS 1. TOF, PS, VALVAR 2. TOF, PS, SUBVALVAR 3. TOF, PS, SUPRAVALVAR 4. TOF, PS, VALVAR & SUBVALVAR 5. TOF, PS, VALVAR & SUPRAVALVAR 6. TOF, PS, VALVAR SUBVALVAR & SUPRAVALVAR

Epidemiology

Tetralogy of Fallot occurs in 3 of every 10,000 live births. It is the commonest cause of cyanotic cardiac disease in patients beyond the neonatal age, and accounts for up to one-tenth of all congenital cardiac lesions

Perry LW, Neill CA, Ferencz C, EUROCAT Working Party on. Congenital Heart Disease: Perspective in Paediatric Cardiology. Epidemiology of congenital heart disease, the Baltimore-Washington Infant Study 198189. Armonk, NY: Futura; 1993:33-62

Natural history without treatment

1 year survival rate of 66% 2 years survival rate of 49% 20 years and more only 10-15%

Bertranou EG et al. Am J Cardiol 1978;42:458-66

Dimension= Circumference /
McGoon ratio and Nakata index are used to quantitate the degree Pulmonary hypoplasia. These Pulmonary arterial measurements for the calculation are critical to surgical planning. These are measured using echocardiography, Angiography and MRI

Z=Observed dimension- Mean normal dimension/ standard deviation around mean normal dimension ( Z means standard deviation unit) Mcgoon ratio= Sum of the diameter of prebranching right & left pulmonary artery divided by diameter of descending aorta at the level of the diaphragm. Nakata index= Sum of cross-sectional area of right & left PA (mm2)divided BSA

Z-value and Transannular patch

Decision regarding transannular patch: If the pulmonary annulus and the pulmonary artery are more than two to three standard deviations below normal, a transannular patch is indicated. But retrospective data suggest that a wide pulmonary annulus after Fallot repair aggravates pulmonary regurgitation. Therefore, it is advocated that transannular patch enlargement should only be intended for patients with a native pulmonary annulus z-score less than -4. If transannular patching is needed, enlargement should be aimed to diameters within the range of a z-score of -2. Restrictive enlargement of the pulmonary annulus at Fallot repair lowers transannular patch rate, limits the postoperative width of the pulmonary annulus but does not result in increased right ventricular pressure load or reoperation rate for residual right ventricular outflow tract obstruction. A limitation of postoperative pulmonary regurgitation can be expected when the extent of pulmonary annulus enlargement at repair is limited.

McGoon ratio
1. This is sum of diameters of immediately prebranching left and right pulmonary arteries to descending aorta just above level of diaphragm McGoon ratio is measured by echocardiography, angiography and MRI Normal value more than 2 to 2.5 TOF survivor with Pulmonary artesia have McGoon ratio more than 1 Good Fontan candidate should have McGoon ratio more than 1.8

2. 3. 4. 5.

Nakata index
Nakata index is Cross sectional area of left and right pulmonary artery in mm2divided by total body surface area (BSA) Measured in AP view in cath lab and by MRI Left and right pulmonary arteries are measured just before first lobar branching. Cross-sectional area (mm2) = (diameter/2f)2.
Where f is the corrective coefficient for cardioangiography magnification, BSA is body surface area in square meters, the diameter is expressed in millimeters, and the area is expressed in square millimeters.

Normal Nakata value is 330 30 mm2/BSA TOF with PS should have value more than 100 mm2/BSA for survival Good Fontan Candidate should have index more than 250 mm2/BSA For Rastelli surgery Nakata index more than 200 mm2/BSA (if less than 200 mm2/BSA than shunt is done instead of Rastelli)

Associated anomalies
Stenosis of the left pulmonary artery, in 40% of patients Bicuspid pulmonary valve, in 60% of patients. right-sided aortic arch, in 25% of patients Coronary artery anomalies, in 10% of patients Patent foramen ovale or atrial septal defect, in which case the syndrome is sometimes called a pentalogy of Fallot Atrioventricular septal defect Partially or totally anomalous pulmonary venous return Forked ribs and scoliosis

Pathophysiology
Pulmonic stenosis causes concentric right ventricular hypertrophy without cardiac enlargement and an increase in right ventricular pressure Right to Left shunt occurs when RV pressure LV pressure, the net movement is small because the gradient across VSD is low and VSD murmur is silent. Flow through the stenotic Pulmonary valve causes ejection systolic murmur, when PS is more severe murmur may be absent. The severity of cyanosis is directly proportional to the severity of pulmonic stenosis, but the intensity of the systolic murmur is inversely related to the severity of pulmonic stenosis Shunting of deoxygenated blood from the right ventricle to the aorta across the ventricular septal defect (facilitated by aortic override) Since the right ventricle is effectively decompressed into the LV by the large VSD, congestive failure never occurs in TOF. P2 is softer than A2, and inaudible being overshadowed by A2 originating from more anteriorly placed large aorta, 2nd heart sound is single but prominent.

Aetiology
The aetiology is multifactorial. Trisomy 21, 18,13 Microdeletion chromosome 22 DiGeorge syndrome Associations with maternal intake of retinoic acid during the first trimester, poorly controlled diabetes, and untreated phenylketonuria have also been described Tetralogy of Fallot can be diagnosed antenatally as early as 12 weeks of gestation, In general, patients who are referred for foetal echocardiography with a suspicion of tetralogy of Fallot have the most severe phenotypes.

Clinical features
Become symptomatic any time after birth Paroxysmal attack of dyspnea Cyanosis may be present from birth or make its appearance some years after birth Because patients with tetralogy of Fallot have obstruction to pulmonary blood flow, they will not present with signs of heart failure such as failure to thrive. Babies who have tetralogy of Fallot, however, may not gain weight or grow as quickly as children who have healthy hearts because they tire easily while feeding. Commonest symptoms are dyspnea on exertion and exercise intolerance Patients assume a sitting posture squatting as soon as they get dyspneic. Although squatting is not specific for TOF, it is the commonest congenital lesion in which squatting is noted Anoxic spells occur predominantly after waking up or following exertion e.g. crying and is due to spasm of RVOT muscle band.

 Wide spectrum of clinical manifestations depending on severity of abnormalities, i.e. degree of obstruction to right ventricular outflow, and size of VSD. Right atrial contraction at the end of diastole causes a relatively large `a ` wave (prominent in the jugular venous pulse) due to decreased RV distensibility. Cyanosis not usually noticed at birth Causes Child less active in the initial few months Foetal haemoglobin has more affinity for oxygen than adult haemoglobin
Squatting (a compensatory mechanism) is uniquely characteristic of a right-toleft shunt that presents in the exercising child. Squatting increases the Systemic peripheral vascular resistance( by bending lower limb arteries at hip and knee) , which diminishes the right-to-left shunt and increases pulmonary blood flow thereby improving oxygenation and reducing cyanosis. Mental development may be delayed in severe cases due to chronic hypoxia of the brain Paradoxical systemic embolization even leading to Brain abscess formation

Differential diagnosis

Transposition of Great Vessels

Tetralogy of Fallot

Total Anomalous Pulmonary Venous Return

Cyanosis
Ebsteins Anomaly Truncus Arteriosus

Hypoplastic Left Heart Syndrome

Tricuspid Atresia
23

Tet spell
The Tet spell (also called hypoxic spell, cyanotic spell, hypercyanotic spell or paroxysmal dyspnoea) most frequently occurs in young infants with Tetralogy of Fallot but may occur with other congenital heart defects that have pulmonary or subpulmonary stenosis and a VSD, and at any age. Common precipitants include crying, defecation, feeding, waking from naps (low systemic resistance), fever, dehydration, tachypnea / tachycardia due to any cause, and medications (e.g. ACE inhibitors). They tend to occur in those with mild-to-moderate cyanosis at rest and are more common in children who are iron deficient.

Tet spell- continued

They are characterised by: Hypercyanotic spells need to be recognised quickly and effectively Period of uncontrollable controlled to prevent the development crying / panic, of serious complications from prolonged hypoxia. Rapid and deep breathing While medical intervention is (hyperpnoea), indicated, many episodes are self Deepening of cyanosis, limiting. Decreased intensity of heart Those involved in the care of a child murmur, with Tetralogy of Fallot should be Limpness, convulsions and familiar with a spells presentation and rarely, death. early management.

Pathophysiology of Tet-spell
Hypercyanotic spells may be best thought of as an imbalance between pulmonary and systemic vascular resistance favouring decreased pulmonary flow and increased right-toleft shunting. Hypoxemia, metabolic acidosis, hyperpnoea, increased systemic venous return, catecholamines, and pulmonary vasoconstriction are thought to be involved in an interaction that results in a self-perpetuating cycle. Infundibular spasm is not required.

Management
1. Knee-to-chest/Squatting:
Placing the child in the knee-chest position either lying supine or over the parents shoulder (see below). This calms the infant, reduces systemic venous return and increases systemic vascular resistance.

2. Oxygen (100%) can be administered but usually has minimal

effect. If distressing the child it should be discontinued.

3. Morphine: 0.1-0.2 mg/kg IM. (Caution in infants under 3

months).Thought to act by respiratory centre suppression and sedation thereby reducing hyperpnoea. If the above procedures are ineffective or have suboptimal effect, the following treatments may need to be given in addition depending on the state of the child. Establish IV access and discuss with a senior colleague.

Squatting posture

Management-continued
4. Crystalloid or colloid fluid bolus: 10-20ml/kg by rapid IV push. This maximises preload and should be given prior to the following drugs which may induce hypotension. 5. Sodium Bicarbonate (NaHCo3) 1 mEq/kg IV. Repeat in 10-15 minutes This may reduce the respiratory centre stimulating effect of acidosis 6. Metaraminol or beta blocker (e.g. propranolol or esmolol). These medications should be used only after discussion with a paediatric cardiologist or intensivist and should only be given in a monitored environment (e.g. HDU or ICU). 7. General anaesthesia and ventilation. 8. Emergency surgical intervention.

Pharmacology
Metaraminol (ARAMINE) 50 mcg/kg IV over 10-15 minutes. Then 0.25-1 mcg /kg/min as infusion. This is a peripheral vasoconstrictor which increases the systemic vascular resistance and reduces the right-to-left shunt. Esmolol (BREVIBLOC) 500 mcg/kg over one minute IV, then maintenance of 50 mcg/kg/min can be increased in steps of 50mcg/kg/min to maximum dose of 300mcg/kg/min This is an ultrashort acting cardioselective beta blocker and is thought to work by reducing dynamic muscular stenosis of the right ventricular outflow tract and increasing pulmonary blood flow. Note: Extravasation of Esmolol can cause severe tissue necrosis.
If there is a single severe or frequent minor spells, commence oral Propranolol 0.5 mg/kg/dose TDS. This should be done in hospital, watching for hypotension and hypoglycaemia. Increase dose gradually to 1.01.5mg/kg/dose TDS (3.0-4.5 mg/kg/day).

TET spell is usually an indication for early surgery.

Physical examination
Cyanosis Clubbing Harsh systolic murmur over Pulm. area, Left sternal border P2 Murmur absent in Pulmonary atresia, in Tet spell Continuous murmur due to PDA, MAPCA, Shunt

Diagnosis
Clinical history Physical finding Polycythemia, Hct , SaO2 CXR- Boot shaped heart ECG- RVH, tall T,reversal RS ratio, normal PR interval & QRS complex 2D, M-mode, Color doppler echo Cardiac catheterization-anatomy, saturation, chamber pressure, degree of shunting, RV/ LV pressure, peripheral pulmonary arteries CT scan Has an infrequent role in the evaluation of tetralogy of Fallot. This modality is useful for the evaluation of surgical complications such as infection or pseudoaneurysm formation. Helical CT scanning can be used to identify airway compression that is caused by a large ascending aorta that is associated with tetralogy of Fallot. MRI

Chest X ray
Boot shaped heart (it means apex is lifted up & there is a concavity in the region of pulmonary artery) Oligaemic lung fields Hilar vessels are few, lung vessels also few, large rt. Venricle.

Cardiac apex displaced upward coer en sabot Boot shaped heart

Lifted up Apex

Prenatal diagnosis of TOF by foetal echocardiography

Source: The Lancet 2009; 374:1462-1471 (DOI:10.1016/S01406736(09)60657-7)

Terms and Conditions

Transthoracic echocardiography

Echocardiogram in a patient with the tetralogy of Fallot. This parasternal long-axis twodimensional view demonstrates anterior displacement of the outflow ventricular septum that resulted in stenosis of the subpulmonic right ventricular outflow tract, overriding of the aorta, and an associated ventricular septal defect. Ao, overriding aorta; LA, left atrium; LV, left ventricle; RV, right ventricle

Cardiac catheterization study showing narrowing of Pulmonary arteries.

Left lateral and posterior coronal 3D volume-rendered MR images show aneurysmal dilatation of the left (LPA) and right (RPA) pulmonary arteries in an adult patient with uncorrected tetralogy of Fallot and severe pulmonary valve stenosis.

Boechat M I et al. Radiographics 2005;25:1535-1546

2005 by Radiological Society of North America

Anterior 3D volume-rendered image obtained in an adult patient 42 years after initial surgical repair of tetralogy of Fallot and reconstruction of hypoplastic pulmonary arteries shows enlarged right ventricular outflow tract (RVOT) and reconstruc...

Boechat M I et al. Radiographics 2005;25:1535-1546

2005 by Radiological Society of North America

Posterior 3D volume-rendered MR image in the coronal plane shows an enlarged aortopulmonary collateral vessel (AP col.) supplying distal branches of the right pulmonary artery (RPA) in a patient with tetralogy of Fallot.

Boechat M I et al. Radiographics 2005;25:1535-1546

2005 by Radiological Society of North America

Anterior 3D volume-rendered MR image in the coronal plane shows pulmonary atresia and nonconfluence of the left pulmonary artery (LPA) with a blind right ventricular outflow tract.

Boechat M I et al. Radiographics 2005;25:1535-1546

2005 by Radiological Society of North America

Medical treatment
Prevention of tet spell Rx of Tet spell- O2 therapy, correction of acidosis, HCO3, Pulm. Vasodilator e.g.PGE1, Isoprenaline, GTN, NO; Systemic vasocostrictor e.g. Phenoxybenzamine, Phenylepinephrine; Betablocker Squatting posture Ventilatory support Palliative operation

Palliative operation
Blalock-Taussig shunt- subclavian to PA Modified B-T shunt using PTFE graft, subclavian to PA Central shunt aorta to MPA using PTFE RVOT to PA conduit Trans annular patch Waterstone anastomosis-Ascending aorta to RPA Potts anastomosis- descending aorta to LPA Balloon angioplasty of RVOT

Modified Blalock Taussig shunt

Involves insertion of a polytetrafluoroethylene tube graft on the side opposite to the descending aorta from the proximal subclavian artery or distal innominate artery, side to side to the ipsilateral pulmonary artery. The size of the graft (3.0, 3.5, 4.0, 5.0) depends on patient size and calibre of pulmonary artery. Indications in TOF Anomalous major coronary artery Long and narrow ventricular outflow Association with complete AVSD Multiple VSDs Severely hypoplastic pulmonary arteries

Advantages
Highly reliable Does not distort the pulmonary arteries Can be matched in size to the patient and the pulmonary artery Has not been reported to cause pulmonary vascular obstructive disease Is easy to take down Placement does not require CPB and can be done either thro a median sternotomy or a lateral thoracotomy.

Other associated abnormalities in TOF to be considered before complete repair

Coronary artery origin and any anomalies Aortopulmonary collaterals Length of RVOT obstruction Size of Pulmonary Arteries VSD extension and other VSDs Associated defects like mitral stenosis, PDA, ASD or AV canal defect Previous shunts

Coronary anomalies
Approximately 5% with TOF have the left coronary arising from the RCA and then coursing across the RVOT to distribute the septum and the left ventricle. An incision into the RVOT may damage this vital coronary artery. In addition, there are many variations of the coronary artery origin and course e.g. dual LAD supply, which can be potentially harmed during surgery.

Aortopulmonary collaterals
Large collateral vessels connecting the aortic tributaries or the descending aorta with the branch pulmonary arteries may be present, which can produce a large, left to right shunt. These may be incorporated into the repair of the pulmonary arteries or they may be closed surgically or by interventional catheter.

Other abnormalities

Additional VSD A second inlet muscular VSD Associated ASD called as Pentalogy of Fallot Right-sided aortic arch present in 25%; 90% have mirror image branching and 10% may have aberrant LSCA. Persistent left SVC Left sided lesions rarely mitral stenosis may be present in TOF.

Pulmonary valve stenosis though the classic form of RVOTO is infundibular stenosis 75% have both infundibular and pulmonary valve stenosis. Pulmonary artery and branch PAs - Pulmonary artery and supravalvar stenosis and branch PA hypoplasia/atresia are frequently associated and are a consequence of decreased pulmonary blood flow during development. Lack of origin of one pulmonary artery (typically the left) from the pulmonary trunk is not infrequent. The non-connected PA (usually the left branch pulmonary artery) can at times originate from the PDA. TOF with diminutive pulmonary arteries these may be treated with outflow tract patch without VSD closure. It is hoped that the increased forward flow into the pulmonary artery system induced by the relief of obstruction will encourage pulmonary artery growth so that the VSD can be closed later. TOF with absent pulmonary valve syndrome . TOF with pulmonary atresia AVSD and TOF Aortic root dilatation

COMPLETE REPAIR OF TETRALOGY OF FALLOT Advantages Prevents development of right ventricular hypertrophy. Early repair provides adequate and homogenous pulmonary blood flow and allows normal growth and development of pulmonary vessels (Kolcz et al 2005, Van Dongen et al 2003) PRINCIPLES IN COMPLETE SURGICAL REPAIR Closure of VSD Remove RVOT obstruction Avoid conduction system Look for any abnormal coronary branch crossing RVOT
Classic repair in tetralogy of Fallot. An outflow patch of pericardium is used to relieve subpulmonic obstruction, and the VSD is closed with a pericardial or Dacron patch. If valvular PS is present or the main PA is hypoplastic, the outflow tract is extended into the main PA.

Right ventricular outflow tract obstructionIndication for intervention

Class I In symptomatic patients with valvular RVOTO, a domed pulmonary valve and peak instantaneous Doppler gradients > 50 mmHg or mean echo gradients > 30 mmHg, balloon valvotomy is recommended (Level of Evidence: C) In asymptomatic patients with valvular RVOTO, a domed pulmonary valve and peak instantaneous Doppler gradients > 60 mmHg or mean gradients > 40 mmHg, balloon valvotomy should be considered (Level of Evidence: C) The surgical approach is recommended for those patients with significant RVOTO and dysplastic pulmonary valves, subvalvular or supravalvular pulmonary stenosis, associated pulmonary hypoplasia or severe pulmonary regurgitation (Level of Evidence: C) Balloon valvuloplasty is the treatment of choice for valvar RVOTO. Occasionally valve replacement may be necessary (Level of Evidence: B) Balloon valvuloplasty for valvar RVOTO should still be performed only in centres and by teams with experience in this technique (Level of Evidence: C) Patients who require operation for RVOTO should be operated on by congenital heart surgeons (Level of Evidence: C)
Presentation at Annual CCS Meeting in Edmonton 2009

Re-interventions for Tetralogy of Fallot

Class IIa The following situations may warrant intervention following repair: Aortic root enlargement 55 mm in diameter (Level of Evidence: C) A large right ventricular outflow tract aneurysm or evidence of infection or false aneurysm (Level of Evidence: C) Sustained clinical arrhythmias, most commonly either atrial flutter or fibrillation, or sustained monomorphic ventricular tachycardia. When any of these arrhythmias occur, the patient should also be evaluated for a treatable hemodynamic cause of the arrhythmia (Level of Evidence: C) The combination of residual VSD, and/or residual pulmonary stenosis and regurgitation, all mild-moderate but leading to substantial RV enlargement, reduced RV function or symptoms (Level of Evidence: C)

Presentation at Annual CCS Meeting in Edmonton 2009

Intracardiac repair for TOF/ Hierarchical levels

TOF repair, NOS TOF repair, transatrial/transpulmonary repair TOF repair, no ventriculotomy TOF repair, ventricultomy, nontransannular patch TOF repair, ventriculotomy, transannular patch TOF repair, RV-PA conduit TOF/AVC (AVSD) repair TOF/absent pulmonary valve repair

 Removing RVOT obstruction

3 methods
Remove subvalvar obstruction with or without pulmonary valvotomy leaves residual RVOTO The first option plus transannular patch or annular patch creates PR Insertion of orthotopic valve or valve containing conduit non growing element left in the heart
The diameter of the RVOT and pulmonary valve should be measured after pulmonary valvotomy and enlarged 2mm greater than the mean normal value by autologous pericardial patch, RV pressure should be less than 50% of the LV pressure.

Surgical approach Combined transatrial and transpulmonary approach is preferred to the traditional transventricular approach.
Subvalvar infundibular incision is used when the pulmonary annulus is adequate. Transannular incision is used when the pulmonary annulus is inadequate. Pulmonary valvotomy Resection of hypertrophied septal and parietal bands of the infundibulum to relieve the infundibular stenosis (major site of RVOTO). RVOT patch preservation of PV annulus RVOT patch with resection of infundibular muscle (augments restrictive pulmonary blood flow) Patch closure of VSD Transannular patch to augment the RVOT at the levels of the infundibulum, valve annulus and main pulmonary artery. Disrupts integrity of pulmonary valve annulus and results in free PR. It is used when the PV annulus is restrictive.

Transatrial- transpulmonary repair

Trans ventricular repair

Complete repair of tetralogy of Fallot. A, Enlargement of the right ventriclemain pulmonary artery (MPA) connection with a transannular incision if necessary. Ao, aorta. B, Resection of muscle from the outflow tract and identification of edges of the ventricular septal defect (VSD). C, Patch closure of the VSD. D, Placement of a transmural patch if required.

One-patch/ Two patch technique for RVOT reconstruction

Anatomy of the RVOT

The anatomy of the RV outflow tract

The anatomy of the RV outflow tract. There is a large septomarginal (septal) trabeculation that divides into 2 limbs. The anterior limb supports the leaflets of the PV, and the posterior limb runs beneath the membranous septum. A series of trabeculations also arise along the free (parietal) wall of the RV: the septoparietal trabeculations. The crista supraventricularis is between the limbs of the septal trabeculation. The small part that separates the RV and LV cavities is the outlet or conal septum.
Bashore T M Circulation 2007;115:1933-1947

Copyright American Heart Association

Muscle band excision

RVOT reconstruction by Monocusp pericardial valve

RVOT reconstruction

RVOT reconstruction

Prior RVOT conduit

RVOT patch enlargement procedure

TOF- Pulmonary atresia

Most severe end of the spectrum of antero-cephalad deviation of the outlet septum. Pulmonary valve may be imperforate rather than stenotic. Alternative supply comes from 50%- PDA 50%- MAPCA
Shortly after birth, babies usually show the major symptom: cyanosis (bluish tinge to skin), although occasionally this condition becomes apparent only at an older age. Surgery to repair the defects is usually done within in the first 6 months of a babys life; often more than one operation is needed.

PA-VSD Management depends on Pulmonary blood supply

MAPCA dependent( Complex)

Duct dependent ( Simple)

Cyanosed

Balanced

Cardiac failure

Shunt

Duct stent

Primary repair valved /onduit

Secondary repair/ Valved conduit

MAPCA arising from descending thoracic aorta

Descending aortogram showing two large MAPCA's, one to each lung, and absent native central pulmonary arteries. The patient has a right aortic arch. The majority of cases fall somewhere in between these two ends of the spectrum, with the total pulmonary blood flow provided by a combination of diminutive native pulmonary arteries supplemented by PDA and multiple MAPCA's . Different segments of the lungs are fed by either a native pulmonary artery or a MAPCA, and sometimes by both, referred to as dual supply Frequently these patients have underperfused lung segments with little or no flow from the native pulmonary arteries or the collateral circulation, referred to as arborization defects

Management is complex Management aims

Confluent, low pressure pulmonary artery system. Low -- normal RV/LV pressure ratio. Low incidence of catheter and operative re-interventions

Evolution of Management
Interuption of MAPCAs. Recruitment and shunting of MAPCAs. Staged centralization to RV. Primary centralization to RV. Primary centralization to RV with VSD closure. Centralization means Unifocalization

Great variability in development of intrapericardial PAs.

MAPCAs or collaterals usually originate from descending thoracic aorta

Complex unifocalization
Complexity of MAPCAs. Lack of suitable tissue for reconstruction. Need for small valved conduit and reoperation. Long complex operation in small infants.

Pulmonary Atresia and VSD When can the VSD be closed?

Predicted RV/LV ratio less than 0.7 Pulmonary segments centralized. Mechanical evaluation in OR (Hanley). 1 Lungs connected to heart, preoperative assessment of vessels with operative evaluation. ? Leave VSD open or fenestrate. Allow for pulmonary vascular disease and anastomotic narrowing.

Suggestion
Surgical management of MAPCAs and associated cardiac anomalies by one-stage or multi-stage repair has a good outcome .Centralization of pulmonary arteries and MAPCAs to the RV with a valved conduit +/VSD closure. The VSD cannot be closed in all cases. VSD closure in poorly selected cases can lead to early right heart failure. Quality of life can be good with VSD open.

TOF absent Pulmonary valve

Malalignment of the outlet septum with rudimentary formation of the leaflets of the pulmonary valve, so-called absent pulmonary valve syndrome, is seen in around one-twentieth of those alleged to have tetralogy of Fallot. In severe cases, patients present with inspiratory and expiratory stridor due to compression of the airways by the dilated pulmonary arteries. In most instances, but certainly not all, the PDA is also absent.

Tetralogy of Fallot with absent pulmonary valve

TOF with absent pulmonary valve syndrome is clinically distinct from regular TOF. It is characterized by a large VSD, small pulmonary annulus, and nubbins of valve tissue instead of leaflets causing severe PS and regurgitation in utero. This results in massive dilatation of pulmonary arteries and compression of the airways. The cyanosis is from airway compression rather than right to left intracardiac shunting. In addition to the mediastinal abnormalities of the pulmonary arteries with bizarre branching patterns at the hilum; the intraparenchymal pulmonary vessels are abnormal as well with abnormal segmental arteries and elastic laminae. Finally, the airway itself is typically abnormal, with areas of tracheobronchomalacia and occasionally reduced numbers of bronchial generations.
The hypoxaemia is usually caused by a combination of right to left shunting at the VSD as well as pulmonary venous desaturation from ventilation perfusion mismatch.

A new approach for correction of TOF with absent pulmonary valve has recently been described. This includes, in addition to the standard TOF repair, translocation of the pulmonary artery anterior to the aorta and away from the airways (Hraska et al, 2002). This technique has the potential to reduce or eliminate bronchial compression by pulmonary artery. Nearly all have some degree of bronchomalacia in infancy and childhood and some require tracheostomy, long-term ventilation and PEEP.

TOF Treatment TOF/absent pulmonary valve repair, with

Reduction pulmonary artery arterioplasty Valve insertion, pulmonary, NOS Valve insertion, pulmonary, Pulmonary homograft Valve insertion, pulmonary, Aortic homograft Valve insertion, pulmonary, Porcine prosthesis Valve insertion, pulmonary, Other

TOF-DORV
With pronounced aortic override, the aorta becomes more committed to the right ventricle than to the left ventricle, resulting in many instances in the ventriculo-arterial connection of double outlet right ventricle. . The patch repair to close VSD is targeted not to produce LV outflow tract obstructioin, an intracardiac baffle may be necessary.

Repair of double-outlet RV with subpulmonic VSD. The anatomic situation is shown in A. If the distance between the tricuspid valve and PA is great enough, an intraventricular tunnel may be created (B). When the tricuspid valve and PV are too close, a baffle may be created around the anterosuperior margin of the PV (C), or an arterial switch procedure may be done with a much shorter intraventricular tunnel being needed (D).

Bashore T M Circulation 2007;115:1933-1947

Copyright American Heart Association

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