Neonatal repair of truncus arteriosus: continuing improvement in outcomes

BACKGROUND: Repair of truncus arteriosus in the neonatal and early infant periods has become standard practice at many centers. We reviewed our recent experience with repair of truncus arteriosus in neonates, with a focus on early and intermediate outcomes. METHODS: From July 1992 to December 1999, 65 patients 1 month of age or less underwent primary complete repair of truncus arteriosus. Median age was 10 days, and median weight was 3.2 kg. Major associated anomalies included moderate or severe truncal valve regurgitation in 15 patients (23%), interrupted aortic arch in 8 (12%), coronary artery abnormalities in 12 (18%), and nonconfluence of the pulmonary arteries in 3 (5%). Median durations of cardiopulmonary bypass and cardioplegic arrest were 172 minutes and 90 minutes, respectively. Circulatory arrest was employed only in 7 patients undergoing concomitant repair of interrupted arch. Reconstruction of the right ventricular outflow tract was achieved with an aortic (n = 39) or pulmonary (n = 26) allograft valved conduit (median diameter, 12 mm). Replacement (n = 6) or repair (n = 5) of a regurgitant truncal valve was performed in 11 patients, and interrupted arch was repaired in 8. RESULTS: There were three early deaths (5%). Early reoperations included reexploration for bleeding in 3 patients, emergent replacement of a pulmonary outflow conduit that failed acutely in 1 patient, and placement of a permanent pacemaker in 1. Mechanical circulatory support was required in 1 patient. During the median follow-up of 32 months, there were two deaths. The Kaplan-Meier estimate of survival was 92% at 1 year and beyond. The only demographic, diagnostic, or operative factors significantly associated with poorer survival over time were operative weight of 2.5 kg or less (p = 0.01) and truncal valve replacement (p = 0.009). Actuarial freedom from conduit replacement among early survivors was 57% at 3 years. CONCLUSIONS: Repair of truncus arteriosus in the neonatal period can be performed routinely with excellent survival, even in patients with major associated abnormalities.     

Primary repair minimizing the use of conduits in neonates and infants with tetralogy or double-outlet right ventricle and anomalous coronary arteries

OBJECTIVE: The purpose of this study was to review our results with an approach of early primary repair for tetralogy of Fallot or double-outlet right ventricle with anomalous coronary arteries, using several techniques to minimize the use of a conduit. METHODS: Twenty consecutive neonates and infants with anomalous coronary arteries crossing an obstructed right ventricular outflow tract underwent primary repair. Median age was 5.5 months and mean weight 6.22 kg. The anomalous coronary arteries included the left anterior descending from the right coronary artery (n = 10), the right coronary artery from the left anterior descending (n = 1), the left anterior descending from the right sinus (n = 1), and a significant conal branch from the right coronary artery (n = 7) or left anterior descending (n = 1). Two neonates had pulmonary atresia. The right ventricular outflow tract was reconstructed without a conduit in 18 patients, including those with pulmonary atresia. Surgical techniques included main pulmonary artery translocation in 4 patients, transannular repair under a mobilized left anterior descending coronary artery in 2 patients, and displaced ventriculotomy with subcoronary suture lines in 8 patients. In 4 patients the right ventricular outflow tract was repaired via the ventriculotomy and/or pulmonary arteriotomy. A homograft was used as the sole right ventricle-pulmonary artery connection in 1 patient and in another a homograft was added to a hypoplastic native pathway. RESULTS: There have been no early or late deaths. The right ventricular/left ventricular pressure ratio within 48 hours of the operation was 0.47 +/- 0.10. There were 2 reoperations at 8 and 11 years after the operation, during a mean follow-up of 5.2 years (1-11.3 years). CONCLUSIONS: Primary repair of tetralogy of Fallot or double-outlet right ventricle with anomalous coronary arteries can be done in neonates and infants with excellent results. Alternative surgical techniques for right ventricular outflow tract reconstruction, such as main pulmonary artery translocation, can avoid the use of a conduit in most patients.     

Surgical correction of types II and III truncus arteriosus.

In truncus arteriosus types II and III, one or both pulmonary arteries arise independently from either side of the truncus. In the surgical correction of this anomaly, we have utilized on operative technique in which the essential features are as follows: ventricular septal defect (VSD) closure, which directs left ventricular outflow into the truncus: (2) anastomosis of a Dacron tube containing a glutaraldehyde-preserved procine aortic heterograft to the right ventriculotomy: (3) removal of a circumferential band of the truncus containing both pulmonary artery orifices; (4) tailoring of the band of truncus tissue into a generous cuff which is anastomosed to the distal end of the valved Dacron conduit; and (5) restoration of aortic continuity with a tubular Dacron graft. Since 1971, 4 children ages 2 to 9 years have undergone successful correction of truncus arteriosus types II or III by this technique. In one patient with marked pulmonary hypertension and congestive heart failure preoperatively, the pulmonary vascular resistance had reverted to normal by 3 years after the operation. In one patient in whom bronchial collaterals to the right pulmonary artery were present, postoperative left ventricular failure required reoperation for ligation of the collaterals. All 4 patients are asymptomatic and fully active 5 to 60 months postoperatively. None has evidence of stenosis or insufficiency of the heterograft valve.     

Anomalous origin of the left coronary artery from the right pulmonary artery.

A previously unknown coronary artery anomaly is reported: origin of the left coronary artery from the right pulmonary artery. This unusual anomaly can probably be explained by the theory of dual embryonic development of the coronary arteries from angioblastic buds in the truncus arteriosus. It is thought that these buds anastomose with a capillary network that is developing on the surface of the ventricles. Complete repair of this lesion with restoration of a two-coronary artery system was accomplished in a 10-month-old baby. The coronary artery was detached from the pulmonary artery, with a button of pulmonary artery being retained around the coronary artery ostium. Direct systemic arterial flow to the left coronary artery was established by anastomosis of the left coronary to the right subclavian artery.     

Surgical repair of type II truncus arteriosus without a conduit

A technique for reconstructing the right ventricular outflow tract in an infant with type II truncus arteriosus without using a prosthetic conduit is described. The truncal root is transected, and the confluence of branch pulmonary arteries is brought anterior to the aorta by using Lecompte's maneuver. The aorta is then reconstructed directly with an end-to-end anastomosis. The right ventricular outflow tract is reconstructed by anastomosing the posterior wall of the confluence directly to the apex of the vertical right ventriculotomy. A glutaraldehyde-preserved pericardial patch is then placed anteriorly to complete reconstruction of the right ventricular outflow tract.     

Correction of truncus arteriosus with truncal valvar stenosis or insufficiency using two homografts

Surgical correction of truncus arteriosus requires the creation of right ventricular to pulmonary artery continuity and closure of the ventricular septal defect. A variety of conduits have been used including valved and nonvalved. Despite a significant incidence of truncal valvar stenosis and insufficiency, this valve has seldom been replaced. We present 4 cases of truncus arteriosus with truncal valvar stenosis or insufficiency that were repaired using two valved homografts: one to create the pulmonary outflow tract and the other to replace the abnormal truncal valve. Two of these patients are doing well after 4 months. Another child survived the operation and did well for 2 months when she died suddenly. The last child died 14 hours postoperatively from low cardiac output syndrome secondary to diabetic hypertrophic cardiomyopathy. When truncal valvar abnormalities are present, the primary repair of truncus arteriosus in an infant should include replacement of the truncal valve. Total correction can be successfully achieved using two valved homografts, resulting in long-term palliation and freedom from thromboembolic events and the use of anticoagulants.     

儿童永存动脉干的外科治疗

目的　总结儿童永存动脉干外科治疗经验。　方法　近９ 年来共收治永存动脉干５例,年龄３～１２ 岁,平均６－３ 岁。Ⅰ型２ 例（肺动脉均起于动脉干远侧）,Ⅱ型２ 例, Ⅳ型１ 例。肺循环时间和肺动脉排空时间均无明显延长。手术在体外循环心内直视下进行,室间隔缺损（ＶＳＤ） 补片缝合于动脉干瓣环并使其环缩。右心外管道采用缝制自体新鲜心包瓣涤纶人工血管４ 例,同种异体主动脉１ 例。　结果　术毕肺动脉压均明显下降,跨外管道压差７～３５ ｍｍ Ｈｇ。２ 例术后早期死亡与肺动脉病变无关。存活３ 例中１ 例并发低心排出量和呼吸功能不全,２ 例术后恢复顺利。分别随访１１４、９６ 和３４ 个月,心功能均为Ⅰ级,１ 例术后８ 年死于外管道梗阻。　结论　儿童永存动脉干仍可有手术指征; 带自体心包瓣人工血管远期效果良好; 纠正动脉干瓣关闭不全,防止ＶＳＤ 残余漏,避免过大的跨外管道压差和缩短手术时间,可提高手术成功率和远期效果     

Obstruction to left coronary artery blood flow secondary to obliteration of the coronary ostium in supravalvular aortic stenosis

Supravalvular aortic stenosis is characterized by obstruction of the left ventricular outflow tract distal to the aortic valve, and may result in diminished coronary artery blood flow. This report describes the cases of 2 patients in whom obstruction to left coronary artery flow was caused by obliteration of the coronary ostium itself. This mechanism differs from the more commonly recognized cause--valve leaflet adhesion to the obstructing ridge of aortic tissue. The coronary artery obstruction found in these 2 patients required direct enlargement of the left coronary ostium in both. This mechanism of impaired coronary artery flow deserves emphasis, as traditional methods of extended patch aortoplasty may fail to relieve the coronary ostial narrowing.     

Anatomic variation of the aortic coronary openings (apropos of 80 dissections)]

A series of 80 heart dissections, compared with a survey of the literature shows that:--both coronary ostia are usually in the right anterior and in the left posterior position, in the commissural plane, at the level of the corresponding sinus of Valsalva (the left one being often superior in size to the right one).--anatomic variations of the coronary ostia (especially variations of the left coronary ostium) may be summed up into 3 patterns: Variations in number : sometimes, there is only one aortic coronary ostium, usually owing to a left coronary artery originating from the pulmonary artery; a common aortic ostium for a single coronary artery is not frequent. Multiple ostia are the most common variations : an accessory artery may arise from a separate ostium (often the "third coronary artery" from the right aortic sinus; sometimes the anterior descending and the circumflex arteries may originate from separated orifices). Variations in origin remain few, affecting most often the left ostium. Variations in size reflect the corresponding coronary plexus preponderance.     

Evaluation of the growth of a new pulmonary trunk after the reconstruction of right ventricular outflow tract without using an external conduit

To evaluate the growth of a pulmonary trunk reconstructed without an extracardiac conduit, the hemodynamics and diameter of a new pulmonary trunk were measured in 5 patients from the right ventriculogram and MRI at postoperative follow-up periods. There were tetralogy of Fallot with pulmonary atresia in two patients, tetralogy of Fallot with single coronary in one, truncus arteriosus type I in one and transposition of the great arteries with ventricular septal defect and pulmonary stenosis in one. The age at operation ranged from 26 days to 4.5 years. The posterior wall continuity of the right ventricle and pulmonary artery was established by the direct pulmonary-right ventricular anastomosis in three patients and by the interposition of the left atrial appendage in two. Postoperative follow-up periods ranged from 2 years and 6 months to 3 years and 10 months (median: 2 years and 11 months). In four of them, the postoperative right ventricular to aortic or left ventricular systolic pressure ratios were less than 0.4 without any significant systolic pressure gradients between pulmonary artery and right ventricle. In these four patients, the diameters of the reconstructed pulmonary trunks grew from 10～18 mm to 18～21 mm postoperatively. These diameters were more than 100% of normal values. In the remaining patient with tetralogy of Fallot and single coronary artery, the obstruction of the new pulmonary trunk by a bulged left atrial appendage, which was used as the postoperior wall, was observed on the right ventricular outflow tract reconstruction without an extracardiac conduit has growth potential in the future.     

Tetralogy of Fallot with anomalous origin of left anterior descending coronary artery. Surgical options

Anomalous origin of the left anterior descending coronary artery from the right coronary artery can interfere with the location of the usual ventriculotomy during repair of tetralogy of Fallot. The purpose of this study was to compare the results of two operative techniques: (1) a "tailored" right ventricular incision and outflow patch reconstruction and (2) placement of a conduit from the right ventricle to the main pulmonary artery. We reviewed the records of 416 patients who had complete repair of tetralogy of Fallot at the Mayo Clinic from 1973 through 1984. Twenty (5%) (median age 6.5 years) had anomalous origin of the left anterior descending coronary artery from the right coronary artery. Twelve of these patients had right ventricular outflow patch reconstruction, and eight had placement of a conduit from the right ventricle to the pulmonary artery. Three deaths occurred, all during hospitalization, two in the patch reconstruction group and one in the conduit group. The average reduction in right ventricular pressure postoperatively was slightly but not significantly greater for the conduit group. These data indicate that correction of tetralogy of Fallot with anomalous origin of the left anterior descending coronary artery can be done with either patch reconstruction or conduit placement. Selection of the more appropriate procedure depends on the exact location and degree of tortuosity of the anomalous artery and the level and severity of right ventricular outflow obstruction.     

Anomalous origin and distribution of coronary arteries. Review of 38 patients who underwent operation.

Between October 1961 and December 1973, 38 patients with an anomaly in origin (15 patients) or distribution (23 patients) of the main coronary artery or one of its branches underwent operation at the Texas Heart Institute. The left coronary artery originating from the pulmonary artery occurred most frequently-in 13 of 15 patients. An aortocoronary artery bypass was performed in 12 patients with the saphenous vein used in ten of the 12, initially in 1965; and a Dacron tube graft in the other two. Of the 15 patients, only one died during the early period after operation. A follow-up of ten years revealed 11 asymptomatic patients; to date the longest period of patency of a saphenous vein graft is seven years in an 11-year-old girl. Of 23 patients with an unusual coronary artery distribution, 22 had tetralogy of Fallot, 20 of whom underwent total correction. In 21 of the 23 patients the left anterior descending coronary artery originated from the right coronary artery and crossed the right ventricular outflow tract. In two patients this abnormally distributed artery was injured through a vertical right ventriculotomy; both patients died from myocardial failure during the early postoperative period. Subsequently a transverse right ventriculotomy, either alone or combined with a right ventricular outflow and/or pulmonary artery patch enlargement was performed in 16 patients, and a double outlet right ventricle was created through insertion of a Dacron tube graft in two patients. With this method injury to the abnormal left anterior descending coronary artery was avoided and all 18 patients survived the operation. On the basis of our experience and today's advanced techniques, it is believed that most patients, including some under two years of age, can undergo correction of a left coronary artery originating from the pulmonary artery through insertion of a saphenous vein graft between the aorta and left coronary artery. During the surgical correction of cardiac anomalies necessitating a right ventriculotomy, a transverse or double incision in the right ventricular outflow tract in most patients will prevent injury to an abnormally distributed coronary artery branch; sometimes insertion of a Dacron tube graft between the right ventricular outflow tract and pulmonary artery is necessary.     

Fifteen-year experience with surgical repair of truncus arteriosus

From 1965 until March, 1982, 167 patients underwent surgical repair of truncus arteriosus. The age at operation ranged from 18 days to 33 years (mean 6 years). There were 48 hospital deaths (28.7%). The following factors had a positive correlation with the possibility of a surgical death: age at operation less than 2 years (p less than 0.001), a postrepair pulmonary arterial/left ventricular pressure ratio greater than 0.5 for patients with two pulmonary arteries (p less than 0.001) and greater than 0.6 for patients with unilateral absence of a pulmonary artery (p less than 0.02), and a postrepair right ventricular/left ventricular pressure ratio greater than 0.8 (p less than 0.008). The 119 hospital survivors were followed up for a total of 829 person-years. Late survival rate at 5 years was 84.4% and at 10 years, 68.8%. Preoperative factors that correlated with a reduced long-term survival rate were as follows: increasing age at operation (p = 0.004), the presence of moderate or severe truncal valve insufficiency (p = 0.008), lower pulmonary/systemic flow ratio (p = 0.04), and unilateral absence of a pulmonary artery (p less than 0.001). Thirty-six patients required reoperation during the follow-up period (30%) primarily for replacement of the right ventricular-pulmonary arterial conduit and/or for truncal valve replacement. The long-term results obtained in these patients support the need for early repair of the anomaly, improvement in the methods for control or repair of the truncal valve insufficiency, and the continued search for better extracardiac valved conduits.     

Truncus arteriosus type A3: complex repair with cryopreserved pulmonary homograft

A 6-year-old girl with truncus arteriosus type A3, one of the rarest anatomic types, underwent corrective surgery using a cryopreserved valved pulmonary homograft. The special anatomic features in this case were individual origin of the pulmonary arteries. The right pulmonary artery came directly off the truncal vessel, whereas the left pulmonary artery was connected to the aortic arch via a stenotic ductus or ductus-like vessel. The preoperative pulmonary blood flow distribution was 94% on the right versus 6% on the left side. The underperfusion of the left lung may have been related to pulmonary hypoplasia due to long lasting stenosis at the left pulmonary artery take off. The postoperative pulmonary blood flow distribution was 67% on the right versus 33% on the left side. The operative and postoperative course has been uneventful; presently, 6 months after the operation, the patient is in improved clinical condition.     

Coronary artery anomalies in patients with tetralogy of Fallot.

A left anterior descending coronary artery arising from the right coronary artery can be easily injured during performance of a right ventriculotomy for correction of tetralogy of Fallot. This occurred in 2 of the 23 patients in this series, and both patients died from myocardial failure in the early period after operation. Of 19 patients who presented a combination of tetralogy of Fallot and unusual coronary artery distribution, injury to the abnormal coronary artery was avoided by a transverse right ventriculotomy either alone or combined with an upper vertical incision in 17 patients. In 2 patients a Dacron tube graft was inserted between the right ventricular outflow tract and the pulmonary artery. In 2 patients a right ventriculotomy was avoided by closing the ventricular septal defect through a transaortic approach. All 21 patients survived. Before a vertical or longitudinal incision in the right ventricular outflow tract is performed, the coronary artery distribution should always be checked and confirmation made of the normal origin of the left anterior descending branch from the left coronary artery to the left of the pulmonary artery.     

Coronary ostial stenosis

From January 1970 to December 1986, 2.7% (228/8509) of all patients having coronary artery bypass grafting had atherosclerotic coronary ostial stenosis of 50% or more. There were 126 males (55%) and 102 females (45%) with mean ages of 60.2 +/- 9.0 and 59.2 +/- 10.2 years (P = NS), respectively. The left ostium was involved in 176 (77.2%) patients, the right in 41 (18.0%) and both in 11 (4.8%). Isolated ostial stenosis (no associated coronary disease of 50% or more) was present in 38/228 (17%) with females predominating (29/38, 76%). Isolated ostial stenosis occurred more frequently on the right (10/41, 25%) than on the left (18/176, 16%). Associated coronary disease occurred in a single vessel in 42 patients, in two vessels in 72 and in three vessels in 76 resulting in a mean of 2.0 diseased vessels per patient. If patients with isolated ostial stenosis are excluded there were 2.3 diseased arteries per patient. There were 687 grafts performed in these patients, an average of 2.3 grafts per patient. One month surgical mortality was 4.8% (11/228), but has been 2% (2/104) in the last 4 years. Nine of 27 late deaths were noncardiac with a mean follow-up of 49 +/- 44 months. Ostial stenosis is hemodynamically equivalent to left main disease when the left ostium is involved. Symptomatic ostial lesions, whether right or left, require operative therapy.     

Left coronary artery occlusion after percutaneous pulmonary valve implantation

Percutaneous pulmonary valve implantation (PPVI) is an attractive option for patients with pulmonary valve insufficiency or stenotic right ventricular outflow tracts. We present the case of a 26-year-old patient in which PPVI was used to treat d-transposition of the great arteries, uncommon coronary artery anatomy, and conduit stenosis that resulted from multiple operations on the right outflow. Days after discharge to home, she experienced acute chest discomfort correlating with ischemia noted on an electrocardiogram and elevated troponin levels. Coronary angiography confirmed mechanical compression of the left anterior descending coronary artery. The valve was removed in an emergency operation and replaced with a biological conduit.     

Coronary revascularization after arterial switch operation.

We report two cases presenting bilateral coronary artery obstruction after arterial switch operation. The first patient underwent bilateral internal thoracic artery grafting to the left and right coronary arteries. The other patient, presenting a single coronary ostium, underwent surgical coronary ostial angioplasty in concomitance to proximal arterioplasty of both coronary arteries employing a single "pantaloon" shape autologous pericardial patch. Both patients survived and, at 1 year and 9 months after the coronary revascularization procedures, the coronary angiography demonstrated a good patency of the internal thoracic grafts and excellent ostial plasty results, respectively. A complete literature review of patients undergoing different coronary revascularization procedures after arterial switch operation is reported.     

Repair of an anomalous left coronary artery

We describe a patient with an anomalous single coronary artery who presented with a syndrome of atypical chest pain. Coronary angiography revealed a single right coronary ostium, with a narrowed left coronary artery originating at the right coronary ostium. The proximal portion of the left coronary artery that was narrowed was noted to run in the aortic wall. We describe the operative management of this patient using ostial remodeling.     

Asymptomatic fistula from a giant aneurysmatic left anterior descending artery to the right ventricular outflow tract

Coronary artery fistulas are unusual congenital or acquired coronary artery abnormalities in which blood is shunted into a cardiac chamber, great vessel or other structure, bypassing the myocardial capillary network (Jung et al. in Cardiovasc Ultrasound 5:10, 2007). We present a young adult patient with an asymptomatic fistula from a giant aneurysmatic left anterior descending artery to the right ventricular outflow tract, first diagnosed by echocardiography examination and further confirmed by 128-slice computed tomography coronary angiography.