Truncus Arteriosus

41

Truncus Arteriosus VIVAN J.M. BAGGEN  ❘  MICHAEL S. CONNELLY  ❘  JOLIEN W. ROOS-HESSELINK

Definition and Morphology Truncus arteriosus (also known as persistent truncus arteriosus, truncus arteriosus communis, common arterial trunk, or common aorticopulmonary trunk) naturally involves a single arterial vessel exiting the base of the heart, which gives rise to the coronary, pulmonary, and systemic arteries. Embryologically, it is due to abnormal migration of the neural crest tissue, which results in failure of septation of the outflow tract into a separate aortic and pulmonary trunk. There is a single semilunar valve, the truncal valve, and beneath the truncal valve there is almost invariably a ventricular septal defect (VSD). The condition was first described in 1798 by Wilson and confirmed in an autopsy report by Buchanan in 1864.1,2 The basic morphologic criteria defining the anomaly were proposed in 1942 by Lev and Saphir.3 A classification was proposed by Collett and Edwards in 1949 and by Van Praagh and Van Praagh in 1965.4,5 Subsequently, the Society of Thoracic Surgeons tried to establish a unified classification for the basis of surgical reporting, which is essentially a modification of the Van Praagh classification.6 These classifications are further detailed and illustrated in Table 41.1 and Fig. 41.1. More recently, a simplified categorization for truncus arteriosus was proposed, which is based on the presence or absence of an interrupted or hypoplastic aortic arch (hearts with pulmonary or aortic dominance, respectively).7,8 TRUNCUS ARTERIOSUS The truncus arteriosus is larger than the normal aorta and is the only vessel that exits the base of the heart. It is often dilated, and histopathologic studies have demonstrated medial wall abnormalities similar to, and sometimes as extreme as, those found in patients with Marfan syndrome. The sinuses of Valsalva are often poorly developed. In the majority of cases (68% to 83%) the truncus arteriosus overrides the ventricular septum and has a biventricular origin. Less commonly (11% to 29%) it arises solely from the right ventricle. It rarely (4% to 6%) arises from the left ventricle.  TRUNCAL VALVE According to various studies, the truncal valve is tricuspid in 69% of cases, quadricuspid in 22%, and bicuspid in 9%. There is fibrous continuity between the posterior leaflets of the truncal valve and the anterior leaflet of the mitral valve (as between the aortic and mitral valves in the normal heart), but only very rarely is there fibrous continuity between the truncal valve and the tricuspid valve. How well the leaflets of the truncal valve are formed impacts on survival: severe myxomatous thickening is

found in one-third of cases, is associated with significant truncal valve incompetence, and is more common in neonates and young infants who develop severe heart failure or die. Occasionally (18%) the truncal valve may be stenotic.  VENTRICULAR SEPTAL DEFECT The VSD in truncus arteriosus is usually large and nonrestrictive. It results from a deficiency or absence of the infundibular septum. It is subarterial, lying between the two limbs of the septal band (the septomarginal trabeculation), which form the inferior and anterior boundaries. The superior boundary is formed by the truncal valve, and is bounded posteriorly by the ventriculo-infundibular fold. There is usually a muscle bridge between the tricuspid and truncal valves caused by fusion of the inferior limb and the parietal band. When this bridge is absent (rarely), there is fibrous continuity between the two valves. Under these circumstances, the bundle of His is at risk of damage during surgical repair. The VSD is rarely restrictive. This usually occurs when the truncus arteriosus arises exclusively from one ventricle. Very rarely, the VSD is absent. This may occur if the truncus arteriosus arises exclusively from the right ventricle.  PULMONARY ARTERIES The pulmonary arteries usually arise from the left posterolateral aspect of the truncus arteriosus, just above the truncal valve. When there are separate pulmonary artery ostia, the left is usually higher than the right. Very rarely (in the setting of an interrupted aortic arch) the left pulmonary artery ostium may arise to the right of the right ostium, leading to crossing of the pulmonary arteries behind the truncus. Stenoses of the pulmonary artery ostia are uncommon.  CORONARY ARTERIES Although variations in coronary artery anatomy exist, the coronary arteries usually arise from the sinuses of Valsalva above the truncal leaflets. In two-thirds of cases, the left coronary artery arises from the left posterolateral truncal surface and the right coronary artery arises from the right anterolateral truncal surface, similar to the arrangement found in normal hearts. However, the left anterior descending artery is often relatively small and is displaced to the left and the conus branch of the right is consequently large and supplies branches to the right ventricular outflow tract and septum (which may be important at operation). The coronary circulation is left-dominant in about 27% of patients, about three times higher than the prevalence in the normal population.  421

422 TABLE

41.1

PART V  Diseases of the Aorta

Classifications of Truncus Arteriosus

Collett and Edwards—Based on the Anatomic Origin of the Pulmonary Arteries Type I: A short main pulmonary truncus arising from the truncus arteriosus that gives rise to right and left pulmonary arteries (48%-68% of cases).

Type II: No main pulmonary truncus, but the right and left pulmonary arteries arise close to one another (29%- 48% of cases).

Type III: No main pulmonary truncus, and the right and left pulmonary arteries arise distant from one another (6%- 10% of cases).

Type IV: Absence of the pulmonary arteries; the lungs are supplied by large aortopulmonary collateral arteries. This last type is now thought to be a variation of pulmonary atresia with ventricular septal defect and is no longer considered as part of the spectrum of truncus arteriosus.

Van Praagh—Based on the Embryological Development and Also Specifies the Presence (Type A) or Absence (Type B) of a VSD. Type 1: There is a partially formed Type 2: There is absence of the Type 3: There is absence of one branch Type 4: The aortic arch is either aorticopulmonary septum and hence aorticopulmonary septum, and thus pulmonary artery from the truncus hypoplastic or interrupted, and there is a main pulmonary artery segment is no main pulmonary artery segment arteriosus (ie, it arises either from the a large patent ductus arteriosus. present. This corresponds to Collett is present. The right and left branch ductus arteriosus or from the aorta). and Edwards type I. pulmonary arteries arise from the truncus arteriosus, but their proximity to one another is not specified. This corresponds to Collett and Edwards types II and III. Modified Van Praagh—Proposed by the Society of Thoracic Surgeons in an Attempt to Provide a Unified Reporting System That Reflects Both the Anatomy and the Features That Affect Surgical Outcome, Rather Than an Attempt to Understand the Embryology. Type 3: Truncus arteriosus with absence Type 4: Truncus arteriosus with Type 1-2: Truncus arteriosus with confluent or nearly confluent pulmonary of one pulmonary artery. interrupted aortic arch or severe arteries. coarctation.

I

II

III

IV

A2

A3

A4

Collett and Edwards

A1 Van Praagh

A1-2

A3

A4

Modified Van Praagh Figure 41.1  Comparison of the Collett and Edwards, Van Praagh, and modified Van Praagh classifications of truncus arteriosus. (From Mavroudis C, Jonas RA, Bove EL. Personal glimpses into the evolution of truncus arteriosus repair. World J Pediatr Congenit Heart Surg. 2015;6:226-238. Reprinted with permission of John Wiley & Sons, Inc. Copyright 2013 by John Wiley & Sons, Inc.)

DUCTUS ARTERIOSUS The ductus arteriosus is present in about 50% of cases of truncus arteriosus. When present, it tends to remain patent postnatally in approximately two-thirds of cases. There is usually an inverse relationship between the diameter of the ductus and that of the ascending aorta and transverse arch: when the ductus is widely patent, the transverse arch is either

interrupted or there is severe narrowing or coarctation, including tubular hypoplasia of the aortic isthmus and arch. Under these circumstances the pulmonary arteries arise separately from the truncus arteriosus. When there is neither aortic interruption/coarctation nor discontinuous pulmonary arteries, it is exceedingly rare to find a patent ductus arteriosus. 

41  Truncus Arteriosus

VENTRICLES In the right ventricular outflow tract, the infundibular septum is absent. The right ventricle is invariably hypertrophied and enlarged. The left ventricular outflow tract is relatively normal.  ASSOCIATED ANOMALIES The most common cardiovascular anomalies coexisting with truncus arteriosus include VSD in most patients and interrupted aortic arch or coarctation, occurring in 10% to 20% of cases, in association with a widely patent ductus arteriosus. An interrupted aortic arch is often associated with the velocardiofacial syndrome (or DiGeorge syndrome, 22q11.2 microdeletion syndrome). A right aortic arch with mirror-image brachiocephalic branching occurs in 21% to 36% of patients with truncus arteriosus. Other common associated anomalies include secundum atrial septal defect (9% to 20%), aberrant subclavian artery (4% to 10%), persistent left superior vena cava to coronary sinus (4% to 9%), and mild tricuspid stenosis (6%). In 21% to 30% of patients, extracardiac anomalies are present. 

Epidemiology and Genetics Truncus arteriosus is an uncommon congenital cardiac malformation that accounts for 1% to 4% of the cardiac malformations found in large autopsy series5 and 0.6 to 1.4 per 10,000 live births.9 It has been reported in monozygotic and dizygotic twins, siblings, and relatives of children with the defect. There is a strong association with chromosome 22q11 abnormalities, especially in the setting of interrupted aortic arch. However, additional disease genes are likely involved since the 22q11.2 microdeletion syndrome is only observed in approximately 30% of patients with truncus arteriosus.10,11 

Early Presentation and Natural History Although intrauterine diagnosis with fetal echocardiography is possible, truncus arteriosus usually presents in the neonatal period or early infancy. Initial presentation consists of signs of heart failure as the pulmonary vascular resistance falls (tachycardia, tachypnea, excessive sweating, and feeding difficulties), followed by more florid signs of pulmonary and hepatic congestion. Before the fall in pulmonary vascular resistance, mild cyanosis may be detected. The presence of truncal valve insufficiency, stenosis, and coexisting interrupted aortic arch or coarctation exacerbates the problem of heart failure, usually resulting in earlier presentation and negative impact on outcome. Survival is favorably affected by naturally occurring pulmonary stenosis. The unoperated natural history, however, demonstrates an appalling outlook, with 1-year mortality around 70% to 90% usually due to heart failure.12,13 Beyond early childhood, pulmonary vascular disease (or pulmonary arterial hypertension, which can lead to Eisenmenger syndrome) is the major cause of death, although endocarditis and cerebral abscess may be responsible.12 Survival into adult life without surgical intervention has been described; however, it is very uncommon. In Figs. 41.2A and B, multidetector computed tomography images are shown of unoperated persistent truncus arteriosus in an 11-year-old cyanotic boy (Van Praagh type A1) and a 33-year-old cyanotic woman (Van Praagh type A2), respectively.14,15 

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Surgical Repair In view of the poor natural history, early surgery is the main form of treatment for truncus arteriosus. Initially, surgery comprised banding of one or both pulmonary arteries. However, problems are numerous: the band may be inadequate with subsequent development of pulmonary vascular disease; the band may migrate in type I truncus (in which a short main pulmonary artery is present), leading to obstruction of one pulmonary artery and the development of pulmonary vascular disease in the other; or there may be failure of pulmonary artery growth distal to the band or distortion of the pulmonary arteries. Therefore primary and complete operative repair in the neonatal period or infancy is preferred. This was successfully accomplished initially in 1967 by McGoon et al. using an aortic homograft and aortic valve.16 Repair of truncus arteriosus in association with interrupted aortic arch was first successfully accomplished in 1971 by Gomes and McGoon.17 The basic procedure for repair of truncus arteriosus is demonstrated in Fig. 41.3. Numerous iterations have taken place subsequently to try to prolong the life of the right ventricle-to-pulmonary artery conduit, including a Dacron conduit with a porcine semilunar valve and frozen or fresh homografts. Attempts have been made to repair the truncus arteriosus using an extracardiac patch with a pericardial monocusp valve, instead of an extracardiac conduit. This approach was not very successful because the monocusp valve subsequently shrinks, resulting in free pulmonary incompetence.18 More recently, a glutaraldehyde-preserved bovine jugular venous valved conduit (Contegra) was used for the right ventricle-to-pulmonary artery conduit, which seems to offer a cost-effective and readily available solution.19 However, there is a limited range of larger calibers, and the homograft valved conduit remains the gold standard. Truncal valve repair may include bicuspidalization through the approximation of two leaflets associated with triangular resection of the opposite one, or tricuspidalization through excision of one leaflet and related sinus of Valsalva (in the case of a quadricuspid incompetent truncal valve).20 

Late Outcome and Management Because the natural history of truncus arteriosus is dismal, the majority of patients are operated upon before early childhood. In a review of a single-center surgical experience spanning 20 years, the median age at repair was 3.5 months and the youngest patient was aged 2 days, with 81% of the patients operated upon within the first year of life.21 As diagnostics and surgical techniques worldwide continue to improve, there will be fewer and fewer individuals operated upon outside the early childhood period. Consequently, the management of adults with this condition usually involves the care of operative survivors. The best results are obtained at institutions that have the highest caseloads and are properly prepared for neonatal and pediatric cardiac surgery. Fig. 41.4A demonstrates the results of a 20-year follow-up of 165 patients from a single institution who survived to hospital discharge (published in 1997).21 Similar results have been obtained by others.22,23 The study with the longest follow-up duration (32 patients after primary homograft repair for truncus arteriosus) recently reported an actuarial 30-year survival of 83.1%.24 While it seems that survival after initial successful repair of truncus arteriosus is gratifying and continues to improve, patients do have many problems during long-term follow-up. They suffer from significant morbidity with high reoperation rates, up to 90% at 10-year follow-up in older studies with a median time to

424

PART V  Diseases of the Aorta MPA

Truncus

Aorta

VSD

LPA

LPA

Aorta

RPA Truncus

A

Truncus

B Figure 41.2  A, Confirmation of persistent truncus arteriosus (Van Praagh type A1) on multidetector computed tomography in an 11-year-old cyanotic boy with worsening effort intolerance. B, Confirmation of persistent truncus arteriosus (Van Praagh type A2) on 3-dimensional reconstruction of multidetector computed tomography in a 33-year-old cyanotic woman. Two pulmonary arteries arise from the posterior aspect of the truncus separately, but close to each other. LPA, Left pulmonary artery; RPA, right pulmonary artery; MPA, main pulmonary artery; VSD, ventricular septal defect. (A From Kharwar RB, Dwivedi SK, Chandra S, Saran, RK. Persistent truncus arteriosus. J Am Coll Cardiol. 2014;63:1807. Copyright 2014 by the American College of Cardiology Foundation, with permission from Elsevier; B From Kim HS, Kim YH. Persistent truncus arteriosus with aortic dominance in female adult patient. J Cardiovasc Ultrasound. 2015;23:32-35. Copyright 2015 Korean Society of Echocardiography, with permission from JCU Editorial Board.)

Aorta RPA

Aorta

Aorta

LPA

LPA

RV-PA Conduit

Patch Common trunk

LPA

Patch

VSD

A

B

C

Figure 41.3  Technique for surgical repair of truncus arteriosus. A,The right and left pulmonary arteries (RPA and LPA) are detached from the common trunk. Through the right ventriculotomy, the truncal valve and ventricular septal defect (VSD) are visible. B, A patch is used to close the defect in the ascending aorta and the ventricular septal defect. C, The ascending aorta is repaired and a right ventricle–to–pulmonary artery (RV-PA) conduit is placed. LPA, Left pulmonary artery; RPA, right pulmonary artery; RV-PA, right ventricle to pulmonary artery; VSD, ventricular septal defect.

reoperation of 5.1 years after original repair (Fig 41.4B).21 A more recent and smaller study reports a more favorable outcome with a 31.6% reoperation rate at 10-year follow-up and a median time to reoperation of 12.1 years.24 The majority of reoperations are for conduit replacement and truncal valve surgery. In a smaller proportion of patients, pulmonary branch pulmonary arterioplasty and closure of residual VSD is performed.21,23 As surgical results continue to improve, the reoperation rates vary widely depending on the surgical era. Other factors at the time

of initial repair that are significantly associated with worse outcomes include major associated cardiac abnormalities such as interrupted aortic arch, truncal valve abnormalities requiring concurrent truncal valve surgery, and failure to address significant truncal insufficiency.25–27 Cusp removal during initial truncal valve repair might decrease the rate of severe neoaortic regurgitation on midterm follow-up.20 Older age at surgical correction leads to a higher risk of developing pulmonary arterial hypertension, which is associated with adverse outcome.28 However, whereas early surgery (<6 months of age) decreases the risk of developing

41  Truncus Arteriosus

425

100 90 Survival rate (%)

80 70 60 50 40 0 0 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 Time after operation (years)

No. at risk

All patients 165137128121114110104101 93 83 70 61 44 39 24 16 7

6

3

Infants 133 110103 98 92 88 83 81 74 68 57 52 35 30 18 11 4

3

1

Infants

A

1

1

All patients

100

Free from reoperation (%)

90 80 70 60 50 40 30 20 10 0 0

1

2

3

4

5

6

7

8

9

10

11

12

13

14

Time after operation (years) No. at risk 165 128 113 103

84

69

45

39

31

16

10

9

4

2

1

B Figure 41.4  A, Actuarial survival among hospital survivors of complete repair of truncus arteriosus: all patients and infants were aged younger than 1 year at the time of repair. B, Actuarial freedom from reoperation after repair of truncus arteriosus. (Reprinted from Rajasinghe HA, McElhinney DB, Reddy VM, Mora BN, Hanley FL. Long-term follow-up of truncus arteriosus repaired in infancy: A 20-year experience. J Thorac Cardiovasc Surg. 1997;113:869-879, Copyright 1997 by Mosby-Year Book, Inc., with permission from Elsevier.)

pulmonary vascular disease, the risk of conduit reoperation is increased in patients who are operated very early (<3 months of age).21,29 A recent long-term follow-up study showed that patients receiving a Contegra conduit were twice as likely to undergo reoperation for graft replacement as those receiving a homograft.30 Obviously, reoperation is an important risk factor for late death. Other cardiac causes of late death include arrhythmias, sudden death (presumably arrhythmic), and heart failure. OUTPATIENT ASSESSMENT Because truncus arteriosus is rare and successful surgical repair was first performed in 1967, the number of patients who have reached adulthood is currently small, though continuously increasing with the success of cardiac surgery. Individuals are rarely encountered who have survived to adult life without surgical intervention, but they will most likely have significant

pulmonary vascular disease and Eisenmenger syndrome and should be managed accordingly. An overview of the outpatient assessment of adult patients with repaired truncus arteriosus and potential complications that need to be considered is shown in Table 41.2. All patients should be observed on a regular basis, at a minimum probably annually, with more frequent follow-up if there are signs of clinical or hemodynamic deterioration. It is prudent that patients are observed at or in conjunction with a center that specializes in the care of adults with congenital heart disease.  LATE REINTERVENTIONS Reintervention should be considered for complications mentioned in Table 41.2, such as significant conduit dysfunction, neoaortic valve regurgitation or stenosis, branch pulmonary

426 TABLE

41.2

PART V  Diseases of the Aorta

Outpatient Assessment and Potential Complications of the Adult Patient With Truncus Arteriosus

Outpatient Assessment

Particular Attention/Indications

Clinical examination Electrocardiography

Saturation, blood pressure, arrhythmias, central venous pressure, heart murmurs, signs of heart failure. Arrhythmias, signs of right or left ventricular hypertrophy/dilatation, change in intraventricular conduction, pressure overload. Conduit function, neoaortic valve function, aortic root dimensions, ventricular size and function, presence of residual VSD, branch pulmonary artery stenosis, estimated right ventricular and atrial pressure. Quantification of functional capacity, prognostication in future pregnancies, arrhythmias, blood pressure. If arrhythmias are suspected. If more detailed information is required, such as aortic dimensions, ventricular size and function, detailed cardiac anatomy prior to cardiac surgery. If myocardial ischemia is suspected. If gradients and pulmonary pressures cannot be reliably obtained from noninvasive studies. Additional Information Most often stenosis, although the use of a pericardial monocusp is associated with pulmonary regurgitation.

Echocardiography (including Doppler) ± Cardiopulmonary exercise testing ± Holter monitoring ± Cardiac magnetic resonance imaging or computed tomography ± Myocardial perfusion scan ± Cardiac catheterization Complications Right ventricle-to-pulmonary artery conduit stenosis or regurgitation Neoaortic (truncal) valve regurgitation or stenosis Branch pulmonary artery stenosis Ventricular dysfunction Myocardial ischemia Aortic root dilatation Pulmonary arterial hypertension Arrhythmias Residual ventricular septal defect

Or prosthetic valve dysfunction if the truncal valve has been replaced. May cause signs of right ventricular pressure overload. Due to multiple surgical interventions, delayed surgery, conduit dysfunction, pressure overload, myocardial ischemia. Due to coronary artery abnormalities. Due to medial wall abnormalities found in this condition (although the significance of this is unknown). May lead to neoaortic valve regurgitation. Due to vascular remodeling as a result of pulmonary pressure overload (prior to surgery). Shunt reversal may occur, especially if surgical repair is performed at older age (Eisenmenger syndrome). Residual pressure or volume loading lesions and ventricular dysfunction will predispose to both atrial and ventricular arrhythmias.

artery stenosis, neoaortic root dilatation, important myocardial ischemia, and residual VSD. The majority of reinterventions are surgical. However, catheter-based techniques may be appropriate as a temporizing measure for conduit and/or branch pulmonary artery stenosis. It is hoped that with the advent of percutaneous valve procedures, reoperation can be delayed and outcomes will improve further. In a recent study, initial screening for transcatheter pulmonary valve implantation was performed in 404 patients, 46 (11%) of whom had a diagnosis of truncus arteriosus. Although not specified per diagnostic group, the procedure was eventually performed in 85% (343 patients).31 Another smaller study reported promising immediate and short-term results after percutaneous pulmonary valve implantation, successfully restoring an adult-size right ventricular outflow tract diameter.32 Care should be taken to avoid coronary artery compression following percutaneous pulmonary valve implantation, especially in the case of abnormal coronary artery anatomy, which may be present in truncus arteriosus. Where percutaneous options are limited, a hybrid transventricular pulmonary valve implantation may be a feasible approach, avoiding the morbidity of conventional surgery.33  ARRHYTHMIAS In most long-term follow-up studies in patients with truncus arteriosus, the arrhythmia burden is not substantial.21,23 Given the nature of the repair, it is therefore generally assumed that the risk of arrhythmias and sudden death is comparable to patients with Tetralogy of Fallot. The risk is probably higher if there is dysfunction of the truncal valve. Abnormal volume or pressure loading conditions, ventricular systolic dysfunction, and abnormal diastology may be proarrhythmic. When symptoms such as palpitations, dizziness, or syncope are present, further diagnostic workup is mandatory, and when arrhythmias

are present, aggressive arrhythmia management is required. This may include medical therapy, catheter ablation, and pacemaker or internal cardioverter defibrillator implantation.34  MEDICATION The role of pharmacologic management is not well studied. Treatment of ventricular dysfunction and heart failure is empirical. It seems reasonable to adopt similar strategies to those used in adult patients with heart failure, but there are no data to support such an approach. In symptomatic patients with Eisenmenger syndrome, specific medication for pulmonary arterial hypertension, such as phosphodiesterase-5 inhibitors or endothelin receptor antagonists, have been reported to be safe and associated with improved exercise capacity.35,36  PREGNANCY AND CONTRACEPTION Successful pregnancy and delivery has been reported in patients after complete repair and even unoperated truncus arteriosus, although there are few cases in the literature.37,38 Worsening neoaortic valve regurgitation has been described.39 Because there are few reported cases of pregnancy in such individuals, the prevalence of congenital cardiac disease in their offspring is currently difficult to assess. Most cases of 22q11.2 microdeletion syndrome are not inherited, as the deletion most often randomly occurs during the formation of reproductive cells or in early fetal development. However, the inheritance is considered autosomal dominant, and thus the chance of passing on the condition to the offspring is 50%. Therefore, chromosomal analysis using fluorescence in situ hybridization studies should be offered to all women with the condition who are contemplating pregnancy. In all patients, appropriate and timely prepregnancy assessment by an expert multidisciplinary team in a

41  Truncus Arteriosus

specialist center is mandatory. Advice about contraception should also be provided.40 According to the modified World Health Organization classification of maternal cardiovascular risk, pulmonary arterial hypertension, severe left ventricular dysfunction, New York Heart Association classes III-IV, or severe symptomatic neoaortic valve stenosis can be considered conditions in which pregnancy is contraindicated.41 Patients should be carefully monitored during their pregnancy, dedicated cardiac fetal ultrasonography should be performed at an appropriate gestational age (20 weeks), and hospital delivery should be advised.  EXERCISE AND SPORTS PARTICIPATION In a recent cross-sectional study on 25 children and adolescents with repaired truncus arteriosus (median age 11.8 years), peak oxygen consumption (75.3 ± 24.3% of predicted) and peak workload (74 ± 19% of predicted) were substantially diminished, although large differences between individuals were present.11 The hours of habitual exercise per week was positively correlated with peak oxygen uptake.42 Also, in a general group of adults with congenital heart disease, it was found that patients who were engaged in sports showed a higher exercise capacity than those who did not.43 Obviously, advice regarding the intensity of exercise will depend on the individual situation taking into account the outcome of repair, the severity of residual

427

lesions (such as conduit stenosis and neoaortic valve function), and ventricular function. Formal assessment should include echocardiographic evaluation of ventricular function, pulmonary artery pressure (through tricuspid valve regurgitation velocities), aortic dimensions, assessment of arrhythmias, and oxygen saturation at rest. When at least one of these five assessments is outside the conventional normal limits, not more than moderate or low-static sports should be recommended. Cardiopulmonary exercise testing may further aid in determining the relative sports intensity.44 By all means, it is important to encourage participation in regular exercise, tailored to the individual patient. Involvement in a cardiac rehabilitation program can be of additional value in some situations.  ENDOCARDITIS PROPHYLAXIS According to the most recent guidelines, antibiotic prophylaxis should be considered in patients with congenital heart disease that is either cyanotic or repaired with prosthetic material, whether placed surgically or by percutaneous techniques, up to 6 months after the procedure or lifelong if a residual shunt or valvular regurgitation remains.45 The majority of adults after repaired truncus arteriosus likely meets this description, and should therefore be aware of the need to prevent endocarditis and take prophylactic antibiotic therapy when necessary.

REFERENCES 1. Wilson J. A description of a very unusual malformation of the human heart. Philos Trans R Soc Lond. 1798;18:346. 2. Buchanan A. Malformation of the heart: undivided truncus arteriosus. Trans Pathol Soc Lond. 1864;89:1864. 3. Lev M, Saphir O. Truncus arteriosus communis persistens. J Pediatr. 1943;20:74. 4. Collett RW, Edwards JE. Persistent truncus arteriosus; a classification according to anatomic types. Surg Clin North Am. 1949;29:1245–1270. 5. Van Praagh R, Van Praagh S. The anatomy of common aorticopulmonary trunk (truncus arteriosus communis) and its embryologic implications. A study of 57 necropsy cases. Am J Cardiol. 1965;16:406–425. 6. Jacobs ML. Congenital Heart Surgery Nomenclature and Database Project: truncus arteriosus. Ann Thorac Surg. 2000;69:S50–S55. 7. Russell HM, Jacobs ML, Anderson RH, et  al. A simplified categorization for common arterial trunk. J Thorac Cardiovasc Surg. 2011;141:645–653. 8. Mavroudis C, Jonas RA, Bove EL. Personal glimpses into the evolution of truncus arteriosus repair. World J Pediatr Congenit Heart Surg. 2015;6:226–238. 9. Hoffman JI, Kaplan S. The incidence of congenital heart disease. J Am Coll Cardiol. 2002;39:1890–1900. 10. Goldmuntz E, Clark BJ, Mitchell LE, et  al. Frequency of 22q11 deletions in patients with conotruncal defects. J Am Coll Cardiol. 1998;32:492–498. 11. O’Byrne ML, Mercer-Rosa L, Zhao H, et  al. Morbidity in children and adolescents after surgical correction of truncus arteriosus communis. Am Heart J. 2013;166:512–518. 12. Marcelletti C, McGoon DC, Mair DD. The natural history of truncus arteriosus. Circulation. 1976;54:108–111.

13. Kirklin JW, Barrett-Boyes BG. Truncus arteriosus. In: Cardiac Surgery. 2nd ed. Edinburgh: Churchill Livingstone; 1993:1140. 14. Kharwar RB, Dwivedi SK, Chandra S, Saran RK. Persistent truncus arteriosus: a rare survival beyond the first decade. J Am Coll Cardiol. 2014;63:1807. 15. Kim HS, Kim YH. Persistent truncus arteriosus with aortic dominance in female adult patient. J Cardiovasc Ultrasound. 2015;23:32–35. 16. McGoon DC, Rastelli GC, Ongley PA. An operation for the correction of truncus arteriosus. JAMA. 1968;205:69–73. 17. Gomes MM, McGoon DC. Truncus arteriosus with interruption of the aortic arch: report of a case successfully repaired. Mayo Clin Proc. 1971;46:40–43. 18. Honjo O, Kotani Y, Akagi T, et  al. Right ventricular outflow tract reconstruction in patients with persistent truncus arteriosus: a 15-year experience in a single Japanese center. Circ J. 2007;71:1776–1780. 19. Hickey EJ, McCrindle BW, Blackstone EH, et al. Jugular venous valved conduit (Contegra) matches allograft performance in infant truncus arteriosus repair. Eur J Cardiothorac Surg. 2008;33:890–898. 20. Perri G, Filippelli S, Polito A, Di Carlo D, Albanese SB, Carotti A. Repair of incompetent truncal valves: early and mid-term results. Interact Cardiovasc Thorac Surg. 2013;16:808–813. 21. Rajasinghe HA, McElhinney DB, Reddy VM, Mora BN, Hanley FL. Long-term follow-up of truncus arteriosus repaired in infancy: a twenty-year experience. J Thorac Cardiovasc Surg. 1997;113:869–878. discussion 878-869. 22. Williams JM, de Leeuw M, Black MD, Freedom RM, Williams WG, McCrindle BW. Factors associated with outcomes of persistent truncus arteriosus. J Am Coll Cardiol. 1999;34:545–553.

23. Tlaskal T, Chaloupecky V, Hucin B, et  al. Long-term results after correction of persistent truncus arteriosus in 83 patients. Eur J Cardiothorac Surg. 2010;37:1278–1284. 24. Vohra HA, Whistance RN, Chia AX, et  al. Long-term follow-up after primary complete repair of common arterial trunk with homograft: a 40-year experience. J Thorac Cardiovasc Surg. 2010;140:325–329. 25. Konstantinov IE, Karamlou T, Blackstone EH, et al. Truncus arteriosus associated with interrupted aortic arch in 50 neonates: a Congenital Heart Surgeons Society study. Ann Thorac Surg. 2006;81:214–222. 26. Thompson LD, McElhinney DB, Reddy M, Petrossian E, Silverman NH, Hanley FL. Neonatal repair of truncus arteriosus: continuing improvement in outcomes. Ann Thorac Surg. 2001;72:391–395. 27. Russell HM, Pasquali SK, Jacobs JP, et al. Outcomes of repair of common arterial trunk with truncal valve surgery: a review of the society of thoracic surgeons congenital heart surgery database. Ann Thorac Surg. 2012;93:164–169. discussion 169. 28. Kalavrouziotis G, Purohit M, Ciotti G, Corno AF, Pozzi M. Truncus arteriosus communis: early and midterm results of early primary repair. Ann Thorac Surg. 2006;82:2200–2206. 29. Holmes AA, Co S, Human DG, Leblanc JG, Campbell AI. The Contegra conduit: Late outcomes in right ventricular outflow tract reconstruction. Ann Pediatr Cardiol. 2012;5:27–33. 30. Urso S, Rega F, Meuris B, et  al. The Contegra conduit in the right ventricular outflow tract is an independent risk factor for graft replacement. Eur J Cardiothorac Surg. 2011;40:603–609. 31. Morray BH, McElhinney DB, Cheatham JP, et  al. Risk of coronary artery compression

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PART V  Diseases of the Aorta

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