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• W2549209943 abstract "HomeCirculationVol. 115, No. 14Adult Congenital Heart Disease Free AccessResearch ArticlePDF/EPUBAboutView PDFView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissions ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toFree AccessResearch ArticlePDF/EPUBAdult Congenital Heart DiseaseRight Ventricular Outflow Tract Lesions Thomas M. Bashore Thomas M. BashoreThomas M. Bashore From the Department of Medicine, Duke University Medical Center, Durham, NC. Search for more papers by this author Originally published10 Apr 2007https://doi.org/10.1161/CIRCULATIONAHA.105.592345Circulation. 2007;115:1933–1947It has variably been estimated that between 800 000 and 1 million patients with congenital heart disease are now adults and that by 2010 more patients older than 16 years will undergo surgery for congenital heart disease than those younger than 16 years.1 This review will focus on the clinical aspects of identifying and caring for the portion of the unique population that have right ventricular (RV) outflow tract lesions.Clinical Morphology of the RV Outflow TractThe left ventricle (LV) and RV both have inlet, apical, and outlet portions, although they differ considerably.2 The inlet of the RV includes the tricuspid valve, and it is separated from the pulmonary valve (PV) by the crista supraventricularis. This structure results in the PV being raised upward. The apical portion of the RV has coarse trabeculations. As shown in Figure 1, the septum is home to a large septomarginal (septal) trabeculation that divides into the anterior and posterior limbs in the outflow tract. A series of septoparietal trabeculations arise along the free wall. The crista supraventricularis inserts between these limbs and is made up of 3 components: the muscular outlet septum, the ventriculoinfundibular fold, and the inner curvature of the RV.3 The small part that separates the RV and LV cavities is the outlet or conal septum. A loop including the septal trabeculation, the moderator band, septoparietal trabeculations, and the outlet septum can be conceptualized as circling the RV outflow tract. The PV is supported by the circular muscular infundibulum rising outside the RV proper. Abnormalities involving the RV outflow tract involve malpositioning of 1 or more of these structures. Download figureDownload PowerPointFigure 1. The anatomy of the RV outflow tract.2 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.Congenital Lesions That Produce RV Outflow Tract ObstructionObstruction to the RV outflow tract may be the result of abnormalities at the mid-RV, the infundibulum, the PV, the supravalvular region, or the branch and/or peripheral pulmonary arteries. Previous surgery may be responsible for some obstructive lesions as well. Table 1 outlines both native and postoperative congenital obstructive lesions.4–13TABLE 1. Congenitally Related Causes of RV Outflow Tract Obstruction Seen in Adult PatientsUnoperated Valvular Dome-shaped PV Dysplastic PV Unicuspid or bicuspid PV Infundibular stenosis, usually associated with tetralogy of Fallot Hypertrophic infundibular stenosis Associated with PS, hypertrophic cardiomyopathy Infundibular obstruction Tricuspid valve tissue Fibrous tags from inferior vena cava or coronary sinus Aneurysm of the sinus of Valsalva Aneurysm of the membranous septum Subinfundibular obstruction Double-chambered RV Supravalvular stenosis Hourglass deformity at valve PA membrane PA stenosis PA aneurysm Peripheral PA stenosis Associations: rubella, Alagille, Williams, Keutel syndromesOperated Valvular Native valve restenosis Prosthetic valve stenosis Conduit stenosis Double-chambered RV restenosis Peripheral or branch PS At insertion site of prior systemic-to-pulmonary shunt After other complex surgical repair Infundibular stenosis after tunnel repair of double-outlet RVPV StenosisClinical Assessment of PV StenosisGeneral ConsiderationsClinically, there are 3 morphological types of congenital pulmonary stenosis (PS).14 (1) The typical dome-shaped PV is characterized by a narrow central opening but a preserved, mobile valve. Primarily because of an inherent medial abnormality, the pulmonary trunk is usually dilated. (2) The dysplastic PV is poorly mobile, and there is myxomatous thickening with no commissural fusion. The annulus and the outflow tract may be narrowed. (3) The unicuspid or bicuspid PV is generally a feature of tetralogy of Fallot, and stenosis is variable.Many patients with PS are asymptomatic when first seen. With severe PS, RV hypertrophy develops, and symptoms of dyspnea, fatigue, chest pain, palpitations, presyncope, and decreased exercise tolerance may occur. If the right atrial (RA) pressure increases, the opening of a patent foramen ovale may occur along with cyanosis.The dome-shaped PV is present in ≈7% to 12% of cases of congenital heart disease14–16 with equal sex distribution. It makes up to 80% to 90% of all congenital RV outflow tract lesions.16 Its inheritance is low, varying from 1.7% to 3.6%.17,18 In a study of 558 patients with microdeletions in the DiGeorge chromosomal 22q11 deletion, only 2% were found to have PS.19 Approximately 20% of patients with PS have a dysplastic valve20,21 and, if part of Noonan’s syndrome, have an autosomal dominant trait with variable penetrance that has been mapped to chromosome 12.22–24Survival into adulthood is usual. In the Second Natural History Study of Congenital Defects,25 there was no progression in patients with peak gradients <25 mm Hg, there was a 20% chance of an intervention if the gradient was between 25 and 49 mm Hg, and intervention was generally required with gradients >50 mm Hg (Figure 2). Download figureDownload PowerPointFigure 2. PV stenosis. Left, Angiography in dome-valve PV stenosis. Lateral view of classic dome PV stenosis. Note mild subpulmonic obstruction and markedly dilated main PA. Right, Outcome of medically managed patients with PS in the First Natural History Study (NHS-1). Patients with gradients >80 mm Hg were operated on. Graph reveals surgery-free survival in patients with entry gradients of <25, 25 to 49, and 50 to 79 mm Hg. Parentheses represent number of patients under observation at the 25-year anniversary. Reproduced from Hayes et al25 with permission from the American Heart Association. Copyright 1993.Clinical Hemodynamics of PV StenosisThe valvular PS jet may be directed toward the left pulmonary artery (PA), resulting in unequal distribution of blood flow in favor of the left lung.26 The mobile PV creates an auscultatory ejection click that decreases with inspiration, as the right atrial kick into the stiff RV prematurely lifts the PV upward before systole (so there is less excursion of the valve in systole during inspiration compared with expiration, and therefore less click intensity). In severe PS or in patients with a dysplastic valve, there is less mobility and loss of the ejection sound altogether. The hemodynamic grading and physical examination grading of the severity of PS are outlined in Table 2. TABLE 2. Grading the Severity of PV Stenosis Hemodynamically36and by Physical ExaminationHemodynamic Evaluation: Degree of ObstructionHemodynamic Evaluation: Peak Systolic Doppler Gradient, mm HgHemodynamic Evaluation: RV Systolic Pressure, mm HgPhysical ExaminationTrivial<25<50…Mild25–4950–74Jugular venous pressure: Normal except mildly increased “a” waveRV palpation: No RV liftAuscultation: Ejection sound present (decreases with inspiration); systolic ejection murmur increases with inspiration and ends in mid-systole; generally grade 3/6 or lessModerate50–7975–100Jugular venous pressure: Elevated; “c-v” wave if TR present;Severe>80>100Increased “a” waveRV palpation: RV lift; generally no impulse over PA; thrill may be presentAuscultation: No ejection sound; prolonged RV outflow tract systolic ejection murmur with no P2 or wide split of second sound if P2 present; murmur often 3-6/6 in intensity; right-sided S4History and Physical ExaminationMost adult patients with PV or other outflow tract obstructive lesions are normal in appearance, although certain phenotypical syndromes occur that include valvular, branch, or peripheral PS. These include the rubella syndrome27; Noonan syndrome,21 in which 60% have a dysplastic valve; Alagille syndrome28 or arteriohepatic dysplasia; Williams syndrome29; and Keutel syndrome.30The cardiac examination of a patient with PV stenosis is dependent on the valvular severity, the pathology of the valve, and any associated cardiac lesions. Table 2 outlines the expected findings. Pulmonary insufficiency (PI) is uncommon.Associated Laboratory StudiesThe ECG is generally normal when the RV systolic pressure is <60 mm Hg.31 As the lesion severity worsens, evidence of RA enlargement, right axis deviation, and RV hypertrophy may occur.31The heart size on chest x-ray is normal unless there is RV failure or an associated cardiac lesion. Vascular fullness in the left lung base greater than the right (Chen’s sign) is due to the preferential flow.26 In severe PS, vasculature markings may be diminished. Dilatation of the main PA is common in doming but not in dysplastic PS or in subpulmonic stenosis. Calcification may be seen in older patients. The RA and RV may be enlarged if there is RV decompensation.The echocardiogram is generally definitive. A Doppler gradient is evident (Table 2), the valve mobility can be assessed along with subpulmonic or supravalvular stenosis, and the size and function of the RV can be determined. Continuous, pulsed, and color-flow Doppler confirm any PI, tricuspid regurgitation, or right-to-left shunting. Saline microcavitations can also confirm a right-to-left shunt. When the PS is severe, interventricular septal flattening may occur. In patients with a dysplastic valve, the valve is thickened and immobile, and there is lack of a dilated pulmonary main trunk. Transesophageal echocardiography does not usually add additional diagnostic information.In uncomplicated PV stenosis, the use of magnetic resonance imaging (MRI) or computed tomography is simply confirmatory. These studies provide excellent imaging of the main, branch, and peripheral PAs.Cardiac catheterization is rarely necessary for diagnosis. Gradients above, at, and below the PV should be obtained. A peak RV systolic value of <35 mm Hg and a systolic PV gradient of <10 mm Hg are the upper limits of normal. RV function can be assessed, and shunting through any patent foramen ovale can be defined. RV angiography helps to define contractile function, the presence of infundibular obstruction, and the mobility of the PV (Figure 2). Pulmonary angiography assesses the degree of PI and any stenotic lesions in the main, branch, or peripheral PAs.Following Up the Unoperated Patient With PV StenosisThere is little progression in PS severity when the peak Doppler gradient is <30 mm Hg; patients can be followed up every 2 to 3 years. Those with more significant stenosis should be followed up yearly.There is no specific medical therapy. Right heart failure is treated with diuretics. Patients with atrial arrhythmias require medical or ablation therapy. Sudden death is very rare.32 Pregnancy is well tolerated unless the lesion is extremely severe, and percutaneous valvuloplasty can be performed during pregnancy if necessary.The 1986 American Heart Association committee report33 recommends no restriction of activity with mild PS and nonstrenuous exercise with moderate PS and restricts only those with severe PS. For the competitive athlete, the Special Task Force report34 recommends that PS patients with gradients <50 mm Hg may participate in all competitive sports; those with severe PS should only participate in low-intensity sports.Pulmonary endocarditis is rare,35 and some recommend prophylaxis only with a >25 mm Hg gradient.36 The guidelines continue to endorse endocarditis prophylaxis, however,37 although it is anticipated that newer revisions of the guidelines will not recommend it.Indications for InterventionOn the basis of natural history studies of the outcomes on unoperated patients with gradients >50 mm Hg (Figure 2), patients with severe PS should undergo intervention. The recent American College of Cardiology/American Heart Association Task Force report on valvular heart disease38 has extended these data, and their recommendations are summarized in Table 3. TABLE 3. Recommendations for Percutaneous Balloon Valvuloplasty in Patients With PV StenosisAdapted from Bonow RO et al. ACC/AHA 2006 guidelines for the management of patients with valvular heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Revise the 1998 Guidelines for the Management of Patients With Valvular Heart Disease). Circulation. 2006;114:450–527, with permission from the American Heart Association. Copyright 2006.Class I Symptomatic patients with classic PS with a peak systolic gradient >50 mm Hg and <2+ PI (Level of evidence: B) Balloon valvuloplasty may be reasonable in symptomatic patients with classic PS and a peak systolic gradient >30 mm Hg and <3+ PI (Level of evidence: C) Balloon valvuloplasty may be reasonable in asymptomatic patients with classic PS and a peak systolic gradient >40 mm Hg and <3+ PI (Level of evidence: C)Class IIb Balloon valvuloplasty may be reasonable in asymptomatic patients with classic PS and a peak systolic gradient between 30 and 40 mm Hg and <3+ PI (Level of evidence: C) Balloon valvuloplasty may be achievable in selected symptomatic patients with a dysplastic PV and peak systolic gradient >30 mm Hg (Level of evidence: C)Class III Balloon valvuloplasty is not recommended for most patients with dysplastic PV disease, for patients with a peak PV gradient <30 mm Hg, or for those with concomitant 3+ or greater pulmonary valve insufficiency (Level of evidence: C)Surgical and Percutaneous InterventionSurgical InterventionSurgical commissurotomies are quite effective and are now done by direct visualization. In patients with a dysplastic valve, partial or total valvectomy and valve replacement are usually required, and a transannular patch may be needed if there is annular or pulmonary trunk hypoplasia. Residual PI is commonplace with surgical procedures.39In patients with PS and significant PI, valve replacement is required. Mechanical valve replacement is used rarely because of thrombosis issues and the possible future need for pulmonary pressure measurement. Bioprosthetic valves40,41 and pulmonary homografts40,42 have both been used with good results, the latter allowing for replacement of the main PA.43 Rarely, a valved conduit is required for anatomic reasons or because of an anomalous coronary crossing the RV outflow tract that might be injured.Significant outflow obstruction can occur postoperatively in the pulmonary homograft with the Ross procedure. Postoperative valves, conduits, or homografts placed for other reasons can also contribute to the causes of clinical RV outflow tract obstruction. Bioprosthetic valvular degeneration is expected after ≈10 to 12 years,44 and porcine valves may outlast homografts in children.45Percutaneous Balloon Valvuloplasty and Valve ReplacementSince the initial successful report of percutaneous balloon valvuloplasty for PV stenosis in 1982,46 the procedure has evolved to be the treatment of choice for patients with classic domed PS. A successful procedure is defined by final peak gradient of <30 mm Hg and is obtained in >90%.Ten-year follow-up data are now available with excellent outcomes,47 and the restenosis rate is low, generally occurring only if there was a residual gradient immediately after the procedure.48 Rarely, dysplastic valves will respond to balloon valvuloplasty.Several studies have compared balloon valvuloplasty with matched surgical controls49–51 and have found similar long-term results, although there appears to be more PI and ventricular ectopy in the surgical groups.49Recently, the use of percutaneous valve replacement has received widespread attention. Led by Khambadkone and Bonhoeffer,52 the procedure has been deployed successfully in RV outflow conduits53 and has now been extended to include patients with native PS.Following Up the Postoperative or Postintervention PatientThe clinical issues regarding intervention in the postoperative patient are similar to those preoperatively except for more valvular regurgitation. In the low-pressure PI setting, the diastolic gradient between the RV and PA may be small, and significant PI may be difficult to detect. Any unexplained enlargement of the RV should trigger concern that PI is more severe than noninvasive evaluation suggests.Tetralogy of FallotClinical AssessmentGeneral ConsiderationsAlthough actually not the first to describe this defect,54 Etienne-Louis Arthur Fallot eloquently described the complex of 4 anatomic features that now bears his name: subpulmonary infundibular stenosis, ventricular septal defect (VSD), rightward deviation of the aortic valve with overriding of the ventricular septum, and RV hypertrophy. The range of anatomic features includes minimal overriding of the aorta and trivial PS to 95% override of the aorta and frank pulmonary atresia.Figure 3 outlines and describes the basic anatomic features of tetralogy of Fallot.55 The key features are the anterocephalad deviation of the outflow septum and hypertrophy of the septoparietal trabeculations. Because the outlet septum is an RV structure, malalignment of the outlet septum in this direction results in a VSD and partially commits the aorta to the RV. The VSD is large; the PV is often unicuspid or bicuspid. Pulmonary branch stenosis and various degrees of PA hypoplasia may also be present, with some patients even lacking an entire main PA (most often the left branch PA, where it may hang onto the aorta via the ductus). A left PA branch coarctation in the region of the ductal insertion may also be present. The biventricular aorta is usually dilated, arises from a right-sided arch in ≈25% of patients, and may override the septum so much that >50% comes from the RV (consistent with the definition of a double-outlet RV). The overriding aorta also tends to rotate clockwise, so that the right coronary artery is more anteriorly placed. The left anterior descending coronary artery may arise anteriorly from the right coronary artery and cross the RV outflow; when this occurs, the surgical repair may require a bridging conduit from the RV to the PA. An atrial septal defect is present in ≈5% (sometimes called pentalogy of Fallot). Download figureDownload PowerPointFigure 3. The anatomy of tetralogy of Fallot. The key issues are schematically shown. As noted by Anderson and Weinberg,3 components of the supraventricular crest have “sprung apart.” The cardinal feature is the anterocephalad deviation of the outlet septum. The secondary necessary feature is hypertrophy of the septoparietal trabeculations in order for subpulmonic obstruction to be present. The other classic features include RV hypertrophy, an interventricular communication (VSD), and an overriding aorta (shown through the VSD).Tetralogy of Fallot is the most common cyanotic heart defect that survives to adulthood, accounting for ≈10% of all congenital defects.56 The recurrence rate from parent to child has been estimated at 1.2% to 8%.57 A missense mutation for encoding a ligand expressed in the developing heart has been reported,58 as has a mutation in the transcription factor NKX2.5.59 In a recent study of 251 patients, 15.9% of tetralogy of Fallot patients had chromosome 22q11 microdeletions.60Tetralogy of Fallot is a progressive problem, and patients do poorly without surgical correction; from the Danish population study, only 4% lived to the age of 15 years.56 By the time most reach adulthood, some palliative or corrective surgery has been performed.Clinical HemodynamicsIn the unoperated patient, there is subpulmonic obstruction with a nonrestrictive (large) VSD and variable degrees of PS. PV insufficiency is uncommon. Pulmonary hypertension occurs only if there has been inadequate RV outflow obstruction to prevent pulmonary vascular disease. With less RV outflow tract stenosis, there is minimal or no cyanosis (the so-called “pink tetralogy”) because the VSD shunt is primarily left to right. The degree of right-to-left shunting is related more to the severity of the RV outflow tract obstruction than to the degree of aortic override.61By adulthood, the majority of surviving patients have had either palliative or more definitive repair. Palliative procedures generally include some type of systemic arterial–to–pulmonary arterial shunt (Figure 4). Palliative shunts are taken down when more definitive surgery is performed. Download figureDownload PowerPointFigure 4. Palliative surgical shunts. Whenever there is inadequate pulmonary blood flow, palliative shunts may be of value before definitive correction. The sketch shows the classic subclavian artery–to-PA shunt (Blalock-Taussig), the modified Blalock procedure with the use of a tube of polytetrafluoroethylene, the Waterston shunt between the ascending aorta and right PA, and the Potts shunt between the descending aorta and left PA. See text for discussion.The classic repair includes a VSD patch, an RV outflow tract patch, and infundibular resection (Figure 5). For many patients, there is often either a concomitant surgical pulmonary valvuloplasty or a transannular patch that opens the entire RV outflow tract and crosses the PV (often resulting in PI). More recently, the problem of long-standing postoperative PI has been better appreciated, and valve replacement has been used in older children and young adults. Download figureDownload PowerPointFigure 5. 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.Varying degrees of LV dysfunction may also coexist and be a risk factor for sudden death.62 The cause of this associated LV dysfunction is unclear, although chronic hypoxia and long operative procedures, inherent muscle bundle abnormalities, and abnormal RV-LV interaction due to the dilated RV have all been implicated. Prolonged LV volume overload from the VSD, from palliative shunts, or from aortic insufficiency and surgical injury to the left anterior descending artery arising from the right coronary undoubtedly contribute in some cases.History and Physical ExaminationAdult patients are often asymptomatic. In the unoperated adult patient, cyanosis is common, although extreme cyanosis or a history of classic squatting (done to increase the systemic resistance and reduce systemic venous return) is uncommon. In repaired patients, late symptoms include exertional dyspnea, palpitations, right heart failure, syncope, and even sudden death.Although a patient may present rarely with a peculiar phenotype, most look normal physically. Cyanosis and clubbing may be present. The brachial pulse on the side of a prior Blalock-Taussig shunt is often not present or markedly diminished (despite collaterals). The jugular venous pressure is usually normal unless there is evidence for RV dysfunction, in which jugular venous distension and the presence of a large “a” wave may be noted. Tricuspid regurgitation may be present, and an RV lift is common. A right-sided arch may cause a lift below the right sternoclavicular junction. On auscultation, ejection sounds from the dilated aorta and any aortic insufficiency murmur may be present, and an RV outflow murmur is audible along with a holosystolic murmur from a residual VSD. Lesions in the pulmonary branches may create systolic or continuous murmurs along the chest wall. PI is common after transannular patching, but the intensity may be modest even in severe PI because it is usually associated with normal or low pulmonary pressures. The pulmonic second sound is usually inaudible. A right-sided S4 may be present. The LV examination is usually normal unless there is associated LV dysfunction.Associated Laboratory StudiesThe ECG is extremely important. RV hypertrophy with a right bundle-branch block is common. Postoperatively, from 0.5% to 5.5% of patients die suddenly or require therapy for ventricular arrhythmias.63 Ventricular arrhythmias often originate from the surgical right ventriculotomy site or interventricular septum.Patients at risk for sudden death include those of older age and those with persistent postoperative RV systolic hypertension.64 A QRS duration of >180 ms, QRS prolongation over time, and increased QT dispersion of refractoriness also help to identify high-risk patients.65 QRS duration has also been correlated with RV volume and mass and may decrease after PV replacement.66 Atrial and ventricular arrhythmias contribute to morbidity and mortality; they are more common in those who had palliative shunts for longer periods of time, in older patients, and in those with tricuspid regurgitation,67 pulmonary regurgitation, and progressive RV enlargement.68 Reduced heart rate variability has also been shown to be a marker of increased risk.69The chest x-ray in the adult usually reveals relatively normal pulmonary vascularity. Approximately one quarter will have a right-sided aortic arch. The classic “boot-shaped” heart results from a small underfilled LV lying superior to a relatively horizontal septum, below which is the hypertrophied RV. The heart shadow in adults is confounded by volume loading from a residual VSD or palliative shunt, pulmonary regurgitation, aortic regurgitation, tricuspid regurgitation, RV and/or LV systolic dysfunction, the size of any RV outflow patch aneurysm, and a right or left aortic arch. Progressive aortic root dilatation occurs in ≈15%.70The Doppler/echocardiogram provides confirmation of the lesions described. The low-pressure nature of the PI makes grading the severity of the PI difficult because the pulmonary arterial diastolic pressure is often only a few mm Hg higher than the RV end-diastolic pressure. A more rapid PI pressure half-time (<100 ms) has been proposed as a better indicator of PI severity.71 Transesophageal echocardiography is not needed for the diagnosis.Multidetector computed tomography72 and cardiac MR can provide a wealth of information. The RV and LV volumes and mass can be quantified along with the status of the RV outflow tract, the PAs, the aorta, and any residual VSD.73,74 MRI information often obviates the need for cardiac catheterization. Delayed enhancement in the RV outflow tract has also correlated with RV dysfunction and poor outcomes75 and provides evidence for RV-LV interaction. Radionuclide angiography is often used to quantify RV function.The role for cardiac catheterization is diminishing as MRI and other modalities are maturing. Pulmonary angiography can semiquantify the severity of PI and note branch or peripheral PS. Coronary anomalies can be documented along with any atherosclerosis. In patients with severe RV outflow obstruction, angiography can document systemic-pulmonary collaterals. The size of residual shunts and the documentation of pulmonary hypertension are important, and the extent of pulmonary vasoactivity can be assessed.76Following Up the Unoperated PatientMorbidity in unoperated patients is high and includes cyanosis, right heart failure, exercise intolerance, atrial and ventricular arrhythmias, and paradoxical emboli. Despite this, survival up to age 84 years without surgical intervention has been reported.77 Some adults will have had only palliative shunt procedures performed. The clinical status of these patients is dependent on the severity of the RV outflow tract obstruction, the size of the VSD, and RV function. The QRS duration should be noted, and at least yearly Holter monitoring should be performed. Late repair should be considered for symptomatic patients because improved survival is afforded by surgery done even after the age of 18 years.78Indications for Intervention or ReinterventionMost adult patients will have undergone classic repair, usually including VSD closure and an RV outflow patch. PI is inevitable after transannular patching. Symptomatic patients require reoperation, but the indications are still controversial in the asymptomatic patient. The result of chronic PI is RV dilatation and eventual RV failure, and surgical intervention is often delayed unnecessarily.79,80 Once there is any evidence for RV dysfunction, PV replacement should be performed. Exercise capacity often remains reduced after repair of tetralogy of Fallot. Indications for reoperation are outlined in Table 4.68TABLE 4. Indications for Reintervention in Tetralogy of FallotData derived from Freedom and Shi-Joon57 and Gatzoulis.68Symptoms of right heart failureRV enlargement or evidence for RV dysfunction, especially if PI presentClinically significant arrhythmias (atrial or ventricular)Progressive aneurysmal dilation of an RV outflow tract patchOnset or progression of tricuspid regurgitationResidual VSD with shunt >1.5:1Residual patent arterial-pulmonary shunts leading to LV volume" @default.
• W2549209943 created "2016-11-30" @default.
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• W2549209943 date "2007-04-10" @default.
• W2549209943 modified "2023-10-16" @default.
• W2549209943 title "Adult Congenital Heart Disease" @default.
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