Truncal Root Research Articles

Truncal Root Dilatation: A More Measured Approach

Truncal Root Dilatation: A More Measured Approach

Natural History of Truncal Root Dilatation and Truncal Valve Regurgitation in Truncus Arteriosus

BackgroundThe natural history of the dilated truncal root in repaired truncus arteriosus (TA) is incompletely understood. MethodsA single-center review of patients who underwent TA repair between January 1984 and December 2018 was performed. Echocardiographically determined root diameters and derived z scores were measured at the annulus, sinus of Valsalva (SoV), and sinutubular junction (STJ) immediately before TA repair and throughout follow-up. Linear mixed-effects models assessed trends in root dimensions over time. ResultsOf 193 patients who underwent TA repair at a median age of 12 days (interquartile range, 6-48 days) and survived to discharge, 34 (17.6%), 110 (57.0%), and 49 (25.4%) patients had bicuspid, tricuspid, and quadricuspid truncal valves, respectively. Median postoperative follow-up was 11.6 years (interquartile range, 4.4-22.0 years; range, 0.1-34.8 years). Truncal valve or root intervention was required in 38 patients (19.7%). The mean rates of annular, SoV, and STJ growth were 0.7 ± 0.3 mm/y, 0.8 ± 0.5 mm/y, and 0.9 ± 0.4 mm/y, respectively. Root z scores remained stable with time. At baseline, compared with patients with tricuspid leaflet anatomy, bicuspid patients had larger diameters at the SoV (P = .003) and STJ (P = .029), whereas quadricuspid patients had larger STJ diameters (P = .004). Over time, the bicuspid and quadricuspid cohorts demonstrated comparatively greater annular dilatation (both P < .05). Patients with ≥75th percentile root growth rates had a higher incidence of moderate-severe truncal regurgitation (P = .019) and truncal valve intervention (P = .002). ConclusionsRoot dilatation in TA persisted for up to 30 years after primary repair. Patients with bicuspid and quadricuspid truncal valves demonstrated greater root dilatation over time and required more valve interventions. Continued longitudinal follow-up is warranted in this higher-risk cohort.

A modified approach in the repair of type I and II truncus arteriosus to promote branch pulmonary arterial growth and limit early reoperation.

Maintaining adequate branch pulmonary arterial growth is critical in preventing early (<3years) right ventricular outflow tract reoperation after the repair of truncus arteriosus. We hypothesized that a modified truncus arteriosus repair keeping the branch pulmonary arteries in situ would promote branch pulmonary arterial growth and limit early right ventricular outflow tract reoperation. For infants requiring repair for type I and II truncus arteriosus, the truncal root was septated through a hockey stick incision keeping the branch pulmonary arteries in situ, the ventricular septal defect was closed, and a short aortic homograft was used to reconstruct the right ventricular outflow tract. Echocardiograms measured preoperative and follow-up branch pulmonary artery diameter. Between 1998 and 2020, 41 infants were repaired using the modified approach (type I, 28; type II, 13). With a median follow-up of 11.6 (interquartile range, 3.1-15.5) years, there was no significant change between preoperative left pulmonary artery and right pulmonary artery Z-scores and their corresponding follow-up measurement (left pulmonary artery: 0.97, interquartile range, 0.6-1.6 vs left pulmonary artery: 1.4, interquartile range, -0.3 to 1.9) (right pulmonary artery: 0.6, interquartile range, -0.4 to 1.7 vs right pulmonary artery: 0.3 interquartile range, 0.5-0.9). Only 7.3% (n=2) of follow-up right pulmonary artery Z-scores were less than 2.5 Z-scores below preoperative measurements. Four children (9.8%) required early right ventricular outflow tract reoperation. On multivariable analysis, larger conduit Z-scores were associated with greater time to right ventricular outflow tract reoperation (hazard ratio, 0.55, confidence interval, 0.307-0.984; P=.043). Maintaining the branch pulmonary arteries in situ at initial truncus arteriosus repair allows for branch pulmonary arterial growth, limiting early right ventricular outflow tract reoperation.

Successful surgical repair for common arterial trunk with anterior crisscross pulmonary arteries and right aortic arch causing right bronchial compression

A 1-month-old girl with common arterial trunk, anteriorly originated crisscross pulmonary arteries, unusual running right aortic arch and severe pulmonary hypertension was initially palliated by bilateral pulmonary artery banding. She developed sudden respiratory failure at 80days old; and computed tomography revealed that the right main bronchus was severely compressed by the proximal aortic arch. When common arterial trunk repair was performed, the dominant aortic component of the common trunk incorporating both branch pulmonary arteries was cylindrically resected. End-to-end anastomosis of truncal root and distal ascending aorta was therefore performed to anteriorly retract the proximal arch, thereby relieving bronchial stenosis. The patient experienced an uneventful postoperative course, and postoperative computed tomography showed widely patent right main bronchus.

Neonatal quadricuspid truncal valve repair with left coronary artery unroofing

Neonatal quadricuspid truncal valve repair with left coronary artery unroofing

Modified Repair of Type I and II Truncus Arteriosus Limits Early Right Ventricular Outflow Tract Reoperation

Repair of truncus arteriosus often requires early right ventricular outflow tract (RVOT) reoperation. Using a modified repair, the branch pulmonary arteries are left in situ, which may avoid earlier RVOT reoperation. We hypothesized that our modified repair for type I and II truncus arteriosus would extend the time to RVOT reoperation. Infants with truncus arteriosus were divided into 2 groups: (1) traditional technique where the branch pulmonary arteries are excised from the truncal root, or (2) modified repair where the branch pulmonary arteries are left in situ and septated from the truncal root. Regardless of the approach, a bioprosthetic conduit or homograft was used to establish right ventricular to pulmonary artery continuity. Follow-up pulmonary artery angiograms were used to assess for branch pulmonary artery stenosis. From 54 infants (modified repair: 33, traditional technique: 21), there were no significant differences in age at repair, gender, or type of truncus arteriosus. With 100% follow-up, use of the modified repair resulted in a lower rate of branch pulmonary artery stenosis, and greater freedom from surgical branch pulmonary arterioplasty. Five- and 10-year freedom from RVOT reoperation (5 years: modified-81.5% vs traditional-30.5%, P = 0.004; 10 years: modified-53.3% vs traditional-30.5%, P = 0.01) favored the modified repair. Cox regression analysis demonstrated that the modified repair was associated with an independently lower risk for RVOT reoperation (hazard ratio: 0.08, confidence interval: 0.01, 0.75, P = 0.02). Thus, maintaining the branch pulmonary artery architecture resulted in greater freedom from RVOT reoperation.

Root Dilation in Patients with Truncus Arteriosus

A dilated aortic root is a common finding in children and adults with some forms of congenital heart defects. No data exist on root dilation in truncus arteriosus. We sought to delineate root dimensions across a population of patients with truncus arteriosus. We performed a single-center retrospective review of all patients with truncus arteriosus. Demographic information, clinical history, and most recent echocardiographic data were evaluated. We identified 76 patients whose most recent study was at a median age of 5.4 years (range 0--32.7 years). Mean truncal root z-score was 5.1 ± 2.3. All but three patients had truncal root z-scores greater than or equal to 2. Truncal root z-scores remained stable with increasing body surface area and age. There were no cases of dissection or rupture. Six patients underwent truncal root surgery, typically for indications of root dilation with significant truncal valve insufficiency and left ventricular dilation. In conclusion, mean truncal root z-score was 5, and all but three patients had truncal root z-scores greater than or equal to 2. Although repeat surgical intervention was rare and major complications related to root dilation did not occur in our cohort, further studies with longitudinal follow-up into adulthood are needed.

Technique for the Repair of Truncus Arteriosus to Maintain Pulmonary Artery Architecture

Technique for the Repair of Truncus Arteriosus to Maintain Pulmonary Artery Architecture

Modified Repair of Truncus Arteriosus to Maintain Pulmonary Artery Architecture

Neonatal surgical repair for truncus arteriosus historically involves removing the pulmonary arteries from the truncal root, closing the ventricular septal defect, and creating right ventricular to pulmonary artery continuity. Unfortunately, early reintervention is frequently required for conduit failure and proximal branch pulmonary artery stenosis. We present a technique that preserves the pulmonary artery architecture, keeping the pulmonary arteries in their native position. This technique has been applied to 16 patients and appears to decrease proximal branch pulmonary artery stenosis, thereby extending conduit longevity and increasing the freedom from early reintervention.

Presentation of Congenital Heart Disease in the Neonate and Young Infant

Presentation of Congenital Heart Disease in the Neonate and Young Infant

Severe Truncal Valve Stenosis: Diagnosis and Management

Severe truncal valve stenosis, due to dysplastic valve, may be found in a small number of patients with truncus arteriosus. Its presence complicates the surgical management of this anomaly due to lack of optimal therapeutic options and associated ventricular dysfunction. We report a case of truncal root replacement using a homograft for the management of such condition, and review the literature related to its management and outcome.

Prenatal Features of a Truncus Arteriosus With Pulmonary Atresia and Pulmonary Circulation Derived From the Ductus Arteriosus

truncus arteriosus is a solitary great artery arising from the base of the heart and supplying the systemic, pulmonary, and coronary circulation. A truncus arteriosus is a rare cardiac defect, accounting for less than 1% of structural heart defects.1 The single great artery usually arises above a malalignment ventricular septal defect (VSD) and straddles the ventricular septum. There is variability in the origins of the pulmonary branch and whether the aortic arch is interrupted. Van Praagh and Van Praagh1 suggested classifying trunci arteriosi with VSD into 4 types. In type I, a main pulmonary artery arises from the truncal root and bifurcates into the branch pulmonary arteries. In type II, the branch pulmonary arteries are separate from the truncal root. In type III, the left pulmonary artery is supplied by a collateral from the aortic arch and does not arise from the truncal root. In type IV, the aortic arch is interrupted. A truncus arteriosus may rarely occur without a VSD. Although prenatal diagnosis of a truncus arteriosus has been reported several times,2–4 to our knowledge, the rare variant with an atretic main pulmonary trunk and normal pulmonary circulation such as the case presented here has not been described prenatally. The objective of this report was to show abnormal fetal circulation associated with a truncus arteriosus with pulmonary atresia but with well-developed pulmonary circulation derived from retrograde flow through the ductus arteriosus.

Intraoperative transesophageal echocardiography for pediatric patients with congenital heart disease.

Intraoperative transesophageal echocardiography for pediatric patients with congenital heart disease.

Anterior pulmonary translocation without conduit for the repair of truncus arteriosus

A technique with autologous tissue for the correction of type III truncus arteriosus is described. The truncal root was excised as a cylinder that incorporated pulmonary arteries and that was translocated anterior to the ascending aorta. The proximal section of the cylinder was closed and the pulmonary tract was reconstructed with anastomosis of a widely opened distal section to the right ventricle. Autologous pericardium was sutured to the entire surface of the pulmonary tract.

Anterior pulmonary translocation for repair of truncus arteriosus with interrupted arch

A newborn was found to have truncus arteriosus and an interrupted aortic arch, and underwent primary repair. The patient did well initially, but, by 8 weeks postoperatively, showed evidence of severe compression of the right pulmonary artery. At reoperation, the pulmonary artery was found to be compressed by a large aortic root (truncal root) and the retroaortic area was narrowed. The pulmonary artery bifurcation was therefore translocated anteriorly to alleviate the compression.

Truncus arteriosus associated with mitral atresia and a hypoplastic left ventricle

The association of truncus arteriosus with congenital left heart abnormalities is rare. We describe a case of truncus arteriosus associated with mitral atresia and a hypoplastic left ventricle. In 11 other patients diagnosed with truncus arteriosus, none had left heart hypoplasia. Despite the truncal root being primarily or entirely committed to the right ventricle, more right than left heart abnormalities have been found. This is in contrast to double-outlet right ventricle in which left heart abnormalities are more often associated.

Surgical repair of type II truncus arterious 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 directiy 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.

Coronary arterial and sinusal anatomy in hearts with a common arterial trunk

Eighty-four specimens of common arterial trunk were studied with special reference to the arrangement of the leaflets in relation to the atrioventricular valves, the origin of the coronary arteries in relation to the arterial sinuses, and the epicardial course of the coronary arteries. Fourteen normal hearts were used for comparison. In the hearts with common arterial trunk, the location and level of the coronary artery orifices (as well as the relationship of the truncal root to the area of fibrous continuity with the mitral valve) are different from those in normal hearts. In none of the hearts with common arterial trunk (particularly the 53 hearts with three leaflets in the truncal valve) did the appearance of the truncal valve approximate that of a normal aortic valve. Among the 22 hearts with four leaflets, there was a high incidence of coronary artery orifices in opposite sinuses ( 17 / 22 or 77.3%) and a low incidence of coronary artery orifices in adjacent sinuses ( 2 / 22 or 9.1%). These results suggest that the formation of the truncal valve is independent of the formation of the coronary orifices. Its leaflets are not predestined to become part of either the aortic valve or the pulmonary valve.

Surgical Treatment for the Type II and III Truncus: Complete Division of the Truncal Root with Primary Repair Using Absorbable Suture

A simplified technique for repair of type II and III truncus arteriosus applicable in neonates is described. The method is particularly useful in those patients in whom the pulmonary artery orifices arise from the posterior wall of the truncal root. Advances in two-dimensional echocardiography have provided an accurate means of determining the exact anatomy preoperatively. At operation, isolation of the pulmonary orifices on a button of posterior truncal wall is accomplished by complete division of the main truncal root. Primary closure of the new ascending aorta is made possible by extensive mobilization of the aorta and pulmonary orifices. Early postoperative follow-up indicates that the use of a new absorbable suture for primary repair of the aortic root allows for normal growth.

Aortic arch interruption with truncus arteriosus or aorticopulmonary septal defect.

Interruption of the aortic arch is commonly associated with cardiac malformations. The angiocardiographic appearance in the presence of coexistent truncus arteriosus or an aorticopulmonary septal defect may be very similar. Five patients with arch interruption, three of whom also had truncus arteriosus and two with an aorticopulmonary septal defect are analyzed and the pertinent literature reviewed. In the presence of a truncus arteriosus with arch interruption the aorta appears as an appendage arising anteriorly from a large "pulmonary trunk" with a single semilunar valve, whereas when there is an aorticopulmonary septal defect two separate great artery roots and two semilunar valves are visible. The angiographic features are best defined by a truncal root angiogram when there is a truncus arteriosus or by an aortic root angiogram in the presence of an aorticopulmonary fenestration.