Truncal Valves Research Articles

Morphology, Clinical Associations, and Pathologic Follow-up of Quadricuspid Aortic Valves in Children.

While quadricuspid morphology is commonly observed in truncal valves, quadricuspid aortic valves (QAV) are rare and their natural history is not well described. This retrospective study of 37 patients describes the diagnostic associations and morphologic variability of QAVs in children (median age at diagnosis 4.3 y IQR 0-12 y). Associated congenital heart diseases were present in 54% (most commonly tetralogy of Fallot (TOF) and valvar pulmonary stenosis). Among patients with isolated QAV, 11 had genetic syndrome and 5 had skeletal anomalies. Valve morphology was most commonly type B (41%) and A (35%; Hurvitz and Roberts). Dilated aortic root (Z ≥ 2) was present in 5 and dilated ascending aorta in 9 patients at diagnosis. All patients with type C (n = 3) and G (n = 1) had aortic dilation. At diagnosis, >mild AR was rare (n = 1), mild regurgitation was common (n = 12, 32%), >mild AS was rare (n = 2), and mild AS was uncommon (n = 4). Over a median follow-up of 3.3y (IQR 0.9-11y), progression of AR was seen in 2 patients and progression of aortic root or ascending aorta dilation (increase in Z score by ≥ 2) was seen in 5 patients. In conclusion, QAV is a rare congenital anomaly and about half of the cases are found in hearts that are otherwise structurally normal. A relatively high prevalence is seen in patients with TOF, pulmonary stenosis, skeletal deformities, and genetic syndromes. Meticulous evaluation of aortic valve morphology should be conducted on echocardiograms performed for these indications.

Bicuspidization using autologous pericardium for neonatal quadricuspid truncal valve

BackgroundNeonatal truncal valve plasty remains a significant challenge. Several surgical techniques have been reported, but there is no standard procedure. We herein report a novel operative technique for the quadricuspid truncal valve with axisymmetric morphology.Case presentationA 17-day-old neonate with severe truncal valve insufficiency developed persistent congestive heart failure and underwent urgent truncal valve plasty. The truncal valve was quadricuspid with four equal sinuses, and all four cusps were hypoplastic, resulting in a complete lack of coaptation. We performed bicuspidization valvuloplasty with leaflet reconstruction using autologous pericardium for this valve. The patient had an uneventful recovery and was free from recurrent truncal valve insufficiency for 10 months.ConclusionsBicuspidization with leaflet reconstruction is a suitable surgical option for overcoming neonatal truncal valve insufficiency in cases with quadricuspid truncal valves with axisymmetric morphologies. Although the durability of a bicuspid neo-leaflet using glutaraldehyde-treated autologous pericardium is unknown, this technique is expected to postpone subsequent truncal valve replacement in such neonates.

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.

Systolic excursion of the leaflets of the truncal valve: An unusual mechanism for pulmonary stenosis in common arterial trunk.

Background:Pulmonary stenosis in patients with common arterial trunk protects the pulmonary vasculature. In our recently published prospective study of common arterial trunk, some patients with sinusal origin of the pulmonary arterial segment had pulmonary stenosis induced by systolic excursion of a truncal valvar leaflet. We aimed to determine the detailed morphologic characteristics of this unusual finding.Methods and Results:All 70 patients underwent echocardiography and computed tomographic angiography as per predefined study protocol. In selected cases, we also performed cardiac catheterization. Among 27 patients with aortic dominance, we found sinusal origin of the pulmonary arterial segment. In 5 of these patients, pulmonary stenosis was induced by systolic excursion of a truncal valvar leaflet. In all these patients, the truncal valve was trisinusate, albeit with asymmetric sinuses. The pulmonary arterial segment arose from the largest left sinus with its relatively large leaflet obstructing the pulmonary orifice during systole. In the remaining 22 patients, without pulmonary stenosis but with sinusal origin of pulmonary arterial segment, the truncal valve was quadrisinusate in 7, bisinusate in 2, and trisinusate in 13. None of the patients with quadrisinusate and bisinusate truncal valves had pulmonary stenosis. Among the 13 patients with trisinusate valves, the sinuses of the truncal valve were symmetrical in 6, while in 7, the pulmonary orifice originated from a smaller asymmetric sinus.Conclusion:Pulmonary stenosis, produced by a relatively large leaflet of an asymmetric truncal sinus, may prevent early development of pulmonary vascular disease. Timely recognition of this unusual mechanism of pulmonary stenosis is important for optimal management.

Repair of a quadricuspid truncal valve by tricuspidization and reconstruction of right ventricular outflow tract with the excised truncal cusp

ObjectiveQuadricuspid truncal valves are susceptible to regurgitation, and tricuspid configuration is considered more durable after repairing the truncal valve. We report a new method by excising the excessive truncal cuspid to reconstruct a new competent aortic valve. MethodsBilateral pulmonary banding failed to stabilize a 4-month-old baby with type I truncus arteriosus and quadricuspid truncal valve with severe regurgitation, so surgery was performed. The aorta was transected, and the pulmonary arteries was separated from the aorta. There was an excessive cusp between the right coronary and left coronary cusp. A transannular incision was carried into the right ventricular outflow tract through the left border of the right coronary cusp. We performed truncal valve repair by translocating the excessive cusp, its annulus, and its wall to the right ventricular outflow tract side and reconstructing the neoaortic valve to tricuspid. Ventricular septal defect was repair through a right ventricular outflow tract approach. The floor of the right ventricular outflow tract was made from the excised cusp, including its wall and the resected posterior aortic wall. Then the anterior wall was reconstructed with bovine pericardial patch with a 19-mm porcine valve. ResultsPostoperative echocardiography confirmed a tricuspid neo-aortic valve with trivial aortic regurgitation, no residual ventricular septal defect, and a patent right ventricular outflow tract. ConclusionsWe present the surgical technique to repair a quadricuspid truncal valve insufficiency by using the excessive anterior cusp and its pedicled flap for reconstruction of the pulmonary pathway and restoration of the tricuspid truncal valve.

Contemporary experience with surgical treatment of aortic valve disease in children

Surgical treatment of aortic valve (AoV) disease in childhood involves complex decisions particularly in very small patients. There is no consensus regarding the optimum surgical option. The objective of this review was to analyze a contemporary experience of AoV surgery in a large children's hospital. A retrospective review of children (aged ≤ 18 years) undergoing AoV repair or replacement from June 1995 to December 2011 was carried out. A total of 285 AoV operations (97 repairs, 188 replacements) were performed on 241 patients. Hospital survival for repair was 98% and for replacements was 97%. At follow-up of repairs, there were 16 (17%) reoperations and 3 (3%) late deaths. Follow-up of AoV replacements demonstrated 31 (16%) reoperations (homograft 27, autograft 3, mechanical 1) and 8 (4%) late deaths (homograft 5, autograft 2, mechanical 1). Freedom from reintervention or death (FRD) was found to be lower in repairs for infants (P = .048) and truncal valves (P < .05). For AoV replacements, infants and patients who had concomitant CHD or homografts (P < .0001) had lower FRD. Cox regression analysis for AoV replacements identified infants and homograft root replacements at a higher risk for death/reoperation. AoV repairs and replacements were generally found to be associated with low death and reoperation rates at long-term follow-up. Infants had a lower freedom from reintervention or death after either an AoV repair or replacement, although truncal valve repairs and AoV replacement in patients with concomitant CHD were associated with lower valve survival. Among the valve options, homograft root replacement had a higher risk of death/reoperation and lowest freedom from reintervention or death.

Repair of incompetent truncal valves: early and mid-term results†

To analyse the factors associated with in-hospital mortality and mid-term significant neoaortic valve regurgitation (AR) after truncal valve (TV) repair. Eleven children underwent TV repair at our institution from July 1999 to March 2012. All children presented significant preoperative TV regurgitation. Valve anatomy was quadricuspid in 7 (64%) patients and tricuspid in 4 (36%). The median age and weight at surgery were 29.6 (range 0.3-173.2) months and 12 (range 2.2-49) kg, respectively. Repair included bicuspidalization through the approximation of two leaflets associated with triangular resection of the opposite one (n = 2, 18%), or either bicuspidalization or tricuspidalization of the TV through excision of one leaflet and related sinus of Valsalva (n = 9, 82%). In 3 patients, repair was associated with coronary detachment before cusp removal, followed by coronary reimplantation. In-hospital death occurred in 2 (18%) patients. Factors associated with hospital mortality were age <1 year (P = 0.05), weight <3 kg (P = 0.02) and longer cross-clamping time (P = 0.008). Follow-up was complete for all patients [median follow-up time: 52.2 (range 132.2-2.5) months]. Mid-term significant AR occurred in 4 patients (45%, moderate in 2 and severe in 2). One with severe AR underwent successful valve replacement 4 months postoperatively, leading to freedom from reintervention of 91%. Freedom from significant AR was 76.2 (33.2-93.5) and 60.9 (20.2-85.6) at 1 and 2 years, respectively. There was a trend towards longer freedom from mid-term significant AR for patients who underwent cusp removal compared with those who did not (P = 0.07). TV repair in children can be performed safely with fairly good and durable results. Cusp removal might decrease the rate of severe AR on mid-term follow-up.

Surgical repair of truncus arteriosus in children: early results and long-term outcomes

To recite early results and long-term outcomes after surgical repair of persistent truncus arteriosus (PTA). The clinic data of 54 patients underwent surgical repair for PTA from January 1999 to December 2009 was analyzed retrospectively. There were 36 male and 18 female patients, with a mean age of (9 ± 10) months (range, 1 to 38 months; median, 5 months). Preoperative mechanical ventilation was required in 5 patients. The surgical procedures were closure of ventricular septal defect and re-establishment of continuity between right ventricle and pulmonary artery. The right ventricular outflow tract (RVOT) was reconstructed by direct anastomosis pulmonary artery to right ventriculotomy with anterior wall patch enlargement (28 cases), or by inserting conduits (26 cases). Valvuloplasty were performed in 4 patients with truncal valves moderate to severe insufficiency and aortoplasty in 3 patients with interrupted aortic arch (IAA). There were 3 patients (5.6%) died of pulmonary hypertensive crisis in hospital. The mean duration of ventilation was 6.8 days in 5 patients who were intubated before operation, while the others were 3.6 days. Forty-seven (92.2%) patients were followed-up for mean (6.8 ± 2.5) years (from 2.5 to 11.0 years). There were 2 patients with mild to moderate aortic regurgitation. One patient with aortic arch obstruction underwent balloon dilatation 2 years postoperatively. Among those patients who underwent direct anastomoses, 8 (32.0%) patients had pulmonary branch stenosis at 7 months to 1.5 years postoperatively, 12 (48.0%) patients were freedom from surgical reintervention 5.0 to 11.0 years postoperatively. Among those inserting conduits, 7 patients (31.8%) had conduit stenosis at 2.8 to 7.0 years after operation. Reoperations were performed for RVOT in 15 patients and there was no mortality. It is difficult to treat the PTA patients with IAA, intra-mural coronary artery or mechanical ventilation support before operation. The technique of direct anastomosis between pulmonary artery and right ventricle offers the potential growth for RVOT, but bilateral pulmonary branch stenosis may be occurred at earlier period of postoperation in some patients.

Truncal Valve Repair in Neonates Using Pericardial Leaflet Extension

Truncal valve insufficiency is a significant risk factor for post-operative mortality following repair of truncus arteriosus. The surgical management of dysplastic and insufficient truncal valves remains an operative challenge. We report the cases of two infants with type 2 truncus arteriosus and severely dysplastic and insufficient quadricuspid truncal valves. At primary repair, their truncal valves were successfully repaired using pericardial leaflet extensions. This technique may be used in neonates with truncal valve insufficiency as part of the primary repair of truncus arteriosus.

Techniques for repairing the aortic and truncal valves

There are now many surgical alternatives available for repairing the aortic valve, depending on whether the primary problem is one of stenosis or insufficiency. Patients with insufficiency of the common truncal valve have a unique anatomic substrate, which in some cases allows an innovative strategy of repair not available for the typical patient with a diseased aortic valve. Surgeons should be aware that there are a large number of techniques described for repair of the aortic valve. Some of these are shown in Table 1. For repair of the truncal valve, in contrast, fewer techniques have been described. As shown in Table 2, they are significantly different than those available for repair of the aortic valve.

Surgery for common arterial trunk

Truncus arteriosus (common arterial trunk) is a devastating congenital anomaly which left untreated in some cases can cause death within hours. It has remained a challenge even in recent years. Children may present with heart failure, myocardial ischemia, cardiovascular collapse, or may be only mildly cyanotic and nearly asymptomatic. Repair consists of closure of the ventricular septal defect, separation of the pulmonary arteries from the arterial trunk, and insertion of a valved homograft between the right ventricle and pulmonary artery. The truncal valve is repaired if there is significant regurgitation. Concomitant arch reconstruction is performed in those with an interrupted aortic arch. Repair early in infancy has led to 95% survival and nearly eliminated the perioperative risk of pulmonary hypertensive crisis. Reintervention for right ventricular outflow obstruction is usually necessary and occurs between 4 and 7 years of age. Up to 40% of the truncal valves are at risk for late deterioration. Clinical outcomes are favorable in the present era; however, reintervention is a mandatory part of patient management.

Anatomic relationship of the coronary orifice and truncal valve in truncus arteriosus and their surgical implication

350 The Journal of Thoracic and Cardiovascular Surgery • February 2002 A trileaflet configuration is considered essential to maintaining the competence of a chorda-less semilunar valve.1 Bicuspid aortic and pulmonary valves are prone to stenosis, and quadricuspid valves are susceptible to regurgitation. Truncal insufficiency occurs more frequently in quadricuspid than tricuspid valves, but no cases have occurred in bicuspid valves.2 One method of restoring competence in truncal insufficiency is to excise one of the quadricuspid truncal valves (QTV) with its wall.2 To use that supernumerary cusp and its truncal wall, we reviewed the reported truncal valve anatomy in relation to the coronary orifice and conceived the following procedure for reconstruction.

Repair of truncus arteriosus in early infancy with antibiotic sterilized aortic homografts

Background. To evaluate the fate of the truncal valve, the antibiotic sterilized aortic homografts, and the survival after repair of truncus arteriosus in the first 6 months of life.Methods. Between 1974 and 1994, 23 infants (mean age 1.7 months, range 5 days to 6 months) underwent primary repair of truncus arteriosus by one surgeon (J.L.M). Sixteen were neonates (age range 5 to 30 days). Continuity between the right ventricle and the pulmonary artery was established with an aortic antibiotic sterilized homograft (mean diameter 14.9 mm, range 11 to 17 mm). Follow-up was 100% complete.Results. Four neonates with severe truncal regurgitation died early (17.4%). Fourteen patients underwent reoperations. Five had a truncal valve replacement (mean time 7 years, range 6 months to 17 years). Ten-year freedom from truncal valve replacement was 78.2%. Eleven patients had homograft replacement (mean time 12.7 years, range 2 to 26.2 years). Ten-year freedom from homograft replacement for any cause was 77.1% (for homograft-related problems it was 86.7%). Seven patients retained the original homografts (mean time 14.3 years, range 6 to 18.7 years). There was one late death. Overall 10-year survival was 79% and for the hospital survivors it was 95%. All survivors are in New York Heart Association functional class I.Conclusions. Abnormal truncal valves pose serious early and late problems but the patients with normal truncal valves do well and seem unlikely to need replacement of these valves. The durability of the antibiotic sterilized aortic homograft even in sizes less than 14 mm is remarkably good. Late survival is excellent.

Truncal Valve Repair: Initial Experience in Neonates

Background. The identification of moderate to severe preoperative truncal valve regurgitation has been synonymous with significant postoperative mortality after neonatal repair of truncus arteriosus. Spurned by the deficiencies of current truncal valve substitutes surgeons are once again reexamining the option of truncal valve reparative techniques. Methods. From May 1996 until June 1997, 8 children underwent correction of truncus arteriosus. A retrospective analysis was conducted. Results. There was one in-hospital death secondary to a postoperative massive coronary air embolism. Moderate-to-severe truncal valve regurgitation was identified clinically and confirmed with cardiac ultrasound in 3 neonates with a mean age of 7 days (range, 4 to 12 days) all with quadracusp truncal valves. Successful truncal valve repair was accomplished in 2 infants, with a third neonate requiring homograft replacement with coronary reimplantation for failure in achieving valvular competence after attempted valvuloplasty. Postoperative echocardiograms in those neonates who underwent truncal valve repair confirmed a functional “tricuspid” valve with only mild to mild-plus regurgitation. Conclusions. We give further credence to the hypothesis that primary neonatal truncal valve repair is feasible and may be successful in the avoidance and delay of serial truncal valve replacements using either mechanical or allograft substitutes.

Cardiovascular anomalies in chick embryos produced by bis-diamine in dimethylsulfoxide.

N,N'-bis(dichloroacetyl)-1,8-octamethylenediamine(bis-diamin e) (100 micrograms) dissolved in dimethylsulfoxide (DMSO) was administered to early developing chick embryos (Hamburger-Hamilton stage 9-21) in order to clarify the teratogenic effects on the cardiovascular system and to determine whether bis-diamine interferes with the migration of neural crest cells. Of 346 cases, 154 (44.5%) survived. The incidence of cardiovascular anomalies was 149 out of 154 cases (96.8%). Infundibular ventricular septal defect, double outlet right ventricle, and persistent truncus arteriosus were the primary cardiac anomalies observed in this study. A high percentage of these anomalies were accompanied by hypoplasia of the right 6th aortic arch artery and persistent left 4th aortic arch artery. Particularly, administration of bis-diamine to chick embryos at stage 13 resulted in a high incidence of persistent truncus arteriosus (64.3%). Bis-diamine has been suspected to inhibiting the migration of neural crest cells. However, neural crest cells were observed in the tunica media of the great arteries and the truncal valves of persistent truncus arteriosus produced by bis-diamine in chimeric embryos at stage 13. Morphological changes such as cell death were not observed.

Fetal cardiovascular morphology of truncus arteriosus with or without truncal valve insufficiency in the rat.

Recent advances in fetal echocardiography have necessitated further study on fetal in situ cardiovascular morphology of truncus arteriosus and the effects of truncal valve insufficiency. We studied 55 fetal rats with truncus arteriosus among 300 fetuses from 40 virgin females treated with 200 mg fertilysin on the 10th day of pregnancy. After rapid whole-body freezing on the 21st day, the fetuses were studied by means of serial cross-sectional photographs of the frozen thorax. Thirty-five fetuses with a normal heart treated with fertilysin served as controls. Truncus arteriosus was characterized by a large ventricular septal defect, a solitary artery (truncus arteriosus) overriding the ventricular septum, the right and left pulmonary arteries originating from the truncus arteriosus with or without a common trunk (main pulmonary artery), and absent ductus arteriosus. Fetuses with truncal valve insufficiency had thick truncal valves, a large truncus arteriosus, and large ventricles. The subgroup of 12 fetuses with a large truncus (truncal diameter greater than 160% of the ascending aorta diameter in the controls) showed significantly greater values for right ventricular volume (200% of control) and mass (120% of control), left ventricular volume (170% of control) and mass (110% of control), right (120% of control) and left (110% of control) atrial volume, and pericardial fluid (140% of control) than the controls. These changes were less prominent and ventricular volumes were not increased in the remaining subgroup with a truncal diameter of 160% or less of aorta diameter in the controls. In fetal truncus arteriosus, truncal valve insufficiency was associated with increased ventricular volume load and incomplete cardiac compensation in rats.

Coronary arteries in truncus arteriosus

The origin and distribution of the coronary arteries was described in 39 autopsy specimens of truncus arteriosus (TA). The specimens were classified according to the number and the patterns of the truncal cusps. The position of the truncal cusps was defined in relation to intracardiac structures, namely, the atrioventricular orifices. Bicuspid truncal valves were observed in 8 cases (21%), tricuspid in 22 cases (56%) and quadricuspid in 9 cases (23%). All tricuspid valves had 2 anterior and 1 posterior cusp. Great variability in the origin of the coronary arteries was observed, with a tendency for the right coronary artery to arise from the anterior right quadrant and for the left coronary artery to arise from the anterior and left quadrant. Such a tendency was observed in 50% of the bicuspid, in 59% of the tricuspid and in 66% of the quadricuspid valves. The anatomic right ventricle was always observed to be vascularized by a right coronary artery, and the anatomic left ventircle by a left coronary artery, even in cases in which there was a single coronary trunk. The anterior surface of the right ventricle was crossed by a right coronary artery in 5 cases. A single coronary artery was observed in 7 cases (18%). Embryologic considerations are offered, especially regarding the relation between the observed variability in coronary artery patterns in TA and the absence of the truncal septation.

Coronary arterial origin in persistent truncus arteriosus.

Specimens of heart from 30 subjects with persistent truncus arteriosus were studied for the nature and sites of coronary arterial origin. These factors were related to the sinuses of the truncus valve. Bicuspid truncal valve was observed in six cases (20%) and tricuspid in 21 cases (70%). Single coronary artery was observed in four cases (three with tricuspid and one with quadricuspid truncal valves). In three other cases the coronary arteries arose separately but near one another. There was a strong tendency for the left coronary artery to arise from a more posterior level than it does normally from the aorta. It was common for the posterior descending artery to arise from the left circumflex artery. This arrangement was noted in eight of 25 cases (32%) with single posterior descending coronary artery. In five other cases, two posterior descending arteries were present, one arising from the left circumflex and the other from the right coronary artery.

Truncal valve abnormalities in infants with persistent truncus arteriosus. A clinicopathologic study.

Clinical data on 12 patients under 6 months of age are presented together with the necropsy evidence of truncus arteriosus. Five patients had normal truncal valves and seven had markedly deformed and incompetent valves. Among the group of infants with truncal valve insufficiency, the cause of death was refractory congestive heart failure in each instance, whereas the patients with normal truncal valves succumbed from other causes. Pathologic examination of the abnormal truncal valves revealed cusps which were thickened by fibrous nodules. Combined heart-lung weight in patients with deformed truncal valves had a mean increase of 85% above normal values, while in the group of patients with normal truncal valves the mean increase was 17%. Similar results were observed when liver weights were compared. The major clinical findings among the group of patients with truncal insufficiency were bounding peripheral arterial pulses, early diastolic decrescendo murmurs, and congestive heart failure which was refractory to medical treatment and, in one instance, surgical palliation. It is suggested from the data that deformities of the truncal valves are a primary cause of early mortality among infants with truncus arteriosus.