Diagnosis Of Truncus Arteriosus Research Articles

Fetal Echocardiographic Variables Associated with Pre-Surgical Mortality in Truncus Arteriosus: A Pilot Study.

Truncus arteriosus (TA) is a rare congenital heart defect that can be prenatally detected by fetal echocardiography. However, prognostication and prenatal counseling focus primarily on surgical outcomes due to limited fetal and neonatal pre-surgical mortality data. We aimed to describe the incidence and identify predictors of pre-surgical mortality in prenatally detected TA. This two-center, retrospective cohort study included fetuses diagnosed with TA between 01/2010 and 04/2020. The primary outcome was pre-surgical mortality, defined by fetal or neonatal pre-surgical death or primary listing for transplantation prior to discharge. Univariable regression modeling, Chi-square tests, and t tests assessed for associations between prenatal clinical, demographic, and fetal echocardiographic (fetal-echo) variables and pre-surgical mortality. Of 23 pregnancies with prenatal diagnosis of TA, 4 (17%) were terminated. Of the remaining 19, pre-surgical mortality occurred in 4 (26%), including 2 (11%) fetal deaths and 2 (11%) neonatal pre-surgical deaths. No transplantation listings. Of liveborn fetuses (n = 17), 15 (88%) underwent a neonatal surgery, and 1 (6%) required ECMO. As compared to the survivors, the pre-surgical mortality group had a higher likelihood of having left ventricular dysfunction (0% vs. 40%; p = 0.01), right ventricular dysfunction (0% vs. 60%; p = 0.002), cardiovascular profile score < 7 (0% vs. 40%; p = 0.01), skin edema (0% vs. 40%; p = 0.01), and abnormal umbilical venous (UV) Doppler (0% vs. 60%; p = 0.002). The presence of truncal valve regurgitation or stenosis neared significance. In this cohort withprenatally diagnosed TA, there is significant pre-surgical mortality, including fetal death and neonatal pre-surgical death. Termination rate is also high. Fetal-echo variables associated with pre-surgical mortalityin this cohort include ventricular dysfunction, low CVP, skin edema, and abnormal UV Doppler. Knowledge about prenatal risk factors for pre-surgical mortality may guide parental counseling and postnatal planning in prenatally diagnosed TA.

An Exceedingly Rare Combination of Truncus Arteriosus and Total Anomalous Pulmonary Venous Connection

A neonate with truncus arteriosus (TA) and concomitant total anomalous pulmonary venous connection (TAPVC) is presented. The diagnosis of TA was made on antenatal ultrasound. An echocardiogram performed immediately after birth confirmed TA with a regurgitant quadrileaflet truncal valve. This echo further demonstrated all four pulmonary veins connected to a supracardiac confluence draining to the superior vena cava (SVC) via a partially obstructed ascending vein. Cardiac computed tomography confirmed TA and TAPVC, and demonstrated an ascending vein arising from the supracardiac confluence, passing over the aorta before inserting into the SVC. The patient underwent prompt single-stage repair. The postoperative course was complicated by a pulmonary embolism requiring surgical intervention, and severe truncal valve regurgitation necessitating a third surgery within the first 4 months of life. Review of the literature suggests this combination of defects is exceedingly rare and, of those cases reported, there is a high surgical and postsurgical complication rate.

Risk factors for development of endocarditis and reintervention in patients undergoing right ventricle to pulmonary artery valved conduit placement

ObjectiveTo determine the incidence and risk factors for endocarditis and reintervention in patients undergoing placement of right ventricle-to-pulmonary artery valve conduits. MethodsAll right ventricle–to–pulmonary artery valved conduits placed between 1995 and 2014 were included. Freedom from endocarditis, reintervention, and replacement were analyzed using the Kaplan-Meier method and parametric survival regression models. ResultsA total of 586 patients underwent placement of a total of 792 valved conduits, including 289 (36%) pulmonary homografts, 121 (15%) aortic homografts, 245 (31%) bovine jugular grafts, and 137 (17%) porcine heterografts. There were 474 (60%) primary placements and 318 (40%) replacements. The median duration of conduit follow-up was 7 years; 23 conduits developed endocarditis at a median of 5 years after surgery. The use of bovine jugular grafts was the sole significant risk factor associated with endocarditis (hazard ratio, 9.05; 95% confidence interval, 2.6–31.8 compared with homografts). The hazard was greater for bovine jugular grafts compared with the other conduit types and increased with time; however, bovine jugular grafts were associated with a lower risk for reintervention (P < .0001) and replacement (P = .0002). Factors associated with greater risk of both reintervention and replacement were younger age and smaller conduit size. In addition, a diagnosis of truncus arteriosus was associated with a greater risk for replacement (P = .03). ConclusionsBovine jugular grafts are associated with a significantly greater risk of late endocarditis but with lower reintervention rates compared with other valved conduits. The risk of endocarditis and durability must be balanced during conduit selection. Antibiotic prophylaxis and a high index of suspicion for endocarditis are warranted in patients with bovine jugular grafts.

Prenatal Images of the Truncus Arteriosus With an Interrupted Aortic Arch

A 31-year-old primigravida was referred to our unit due to suspicion of a cardiac anomaly on obstetric screening ultrasound at 28 weeks of gestation. Although fetal echocardiography in the four-chamber view showed atrioventricular concordance and normal cardiac size, an outflow tract abnormality was noted. A single large vessel arising from the left and right ventricles and a ventriculoseptal defect (VSD) were identified (Fig. 1a; Movie 1). The main pulmonary trunk was not detected, and bilateral pulmonary arteries arose from opposite sides of the single large vessel. This unique outflow tract was therefore identified as a truncus arteriosus (TA). Color Doppler sonography showed mild truncal valve regurgitation and no truncal valve stenosis (Movie 2). In the sagittal view, the ascending aorta appeared hypoplastic, and there was interruption between the left subclavian artery and the descending aorta arising from the ductus arteriosus (Fig. 1b, c; Movie 3). Taken together, these findings led a diagnosis of TA associated with interrupted aortic arch (IAA), which corresponded to Van Praagh classification type 4A. No associated anomalies were identified, and fetal cardiac function showed no remarkable changes. At 34 weeks gestation, a female neonate weighing 1,968 g was delivered vaginally after rupture of membranes, with Apgar scores of 6 at 1 min and 9 at 5 min. Neonatal echocardiography and computed tomography confirmed the prenatal diagnosis of TA-IAA (Fig. 2). TA is an uncommon cardiac anomaly accounting for 0.7 % of all congenital heart disease, and concomitant IAA is found in approximately 15 % of children with TA [2]. Thus, the combination of TA-IAA is an extremely rare clinical entity, and a case report of prenatally diagnosed TA-IAA is limited. Marasini et al. [4] described a case of TA-IAA with severe truncal valve regurgitation. However, the case was electively terminated by severe hydrops fetalis. Van Praagh and Van Praagh proposed a classification system for TA according to two commonly associated abnormalities of the great arteries. Type 1 is characterized a short pulmonary trunk originating from the TA. Type 2 is defined by right and left pulmonary arteries originating separately from the TA. Type 3 includes cases with no truncal origin of one pulmonary artery, with the blood supply to that lung from the ductus arteriosus or from a collateral artery. Finally, type 4 is associated with underdevelopment of the aortic arch, including tubular hypoplasia, discrete coarctation, or complete interruption. The Van Praagh classification also specifies the presence of a VSD (type A) or the absence of a VSD (type B) [5]. Our case, the common form of TA with IAA and an associated VSD, is thus classified type A4. Although surgical TA repair during the neonatal and early infant period has become standard practice, the management of neonates with TA-IAA remains challenging [2]. The largest study reported a mortality rate of 68 % during the neonatal and infant period for a cohort of 50 TAIAA patients [3]. Several studies have identified factors Electronic supplementary material The online version of this article (doi:10.1007/s00246-012-0469-3) contains supplementary material, which is available to authorized users.

The Contegra conduit: Late outcomes in right ventricular outflow tract reconstruction

Objectives:To report the clinical outcomes (early death, late death, and rate of reintervention) and performance of the Contegra conduit as a right ventricle outflow tract implant and to determine the risk factors for early reintervention.Methods:Forty-nine Contegra conduits were implanted between January 2002 and June 2009. Data collection was retrospective. The mean age and follow-up duration of Contegra recipients was 3.5 ± 4.6 years and 4.2 ± 2.0 years, respectively.Results:There were three deaths (two early, one late), giving a survival rate of 93.9%. The rate of conduit-related reintervention was 19.6% and was most often due to distal conduit stenosis. Age at implantation of <3 months, receipt of a conduit of 12–16 mm diameter, and a diagnosis of truncus arteriosus were each significant contributors to the rate of reintervention.Conclusion:The Contegra is a cost-effective and readily available solution. However, there is a limited range of larger calibers, which means that the homograft conduit (>22 mm) remains the first choice of implant in older children. The rates of reintervention are significantly higher with a diagnosis of truncus arteriosus, age at implantation of <3 months, and implantation of conduits sized 12–16 mm.

Midterm Results of Size-Reduced Cryopreserved Homografts for Right Ventricular Outflow Tract Reconstruction

Size reduction of valved homografts has been investigated for right ventricular outflow tract reconstruction because of limited supply of small-sized grafts. However, follow-up results are lacking. From 1997 to 2007, 45 patients underwent placement of 34 pulmonary and 11 aortic, size-reduced bicuspid homografts for right ventricle to pulmonary artery connection, which was part of a Rastelli-type operation (n = 35; 78%), redo right ventricular outflow tract reconstruction (n = 5; 11%), double-switch operation (n = 3; 7%), or palliative right ventricle to pulmonary artery connection (n = 2; 4%). Mean age at surgery was 20.0 +/- 24.4 months. Twenty patients (44%) were younger than 1 year old. Mean body weight was 9.6 +/- 7.1 kg. Diameter of the bicuspid conduit was 15.0 +/- 1.5 mm (z value, 3.4 +/- 1.9). Graft dysfunction was defined as pressure gradient greater than 50 mm Hg or moderate or greater regurgitation on follow-up echocardiography. Survival, freedom from graft explantation, and freedom from graft dysfunction were estimated by the Kaplan-Meier method. Risk factor for graft dysfunction was evaluated by multivariate analysis. There were 2 (4.4%) early deaths. During a mean follow-up of 49.4 +/- 37.2 months, 13 patients showed graft dysfunction. Five of them had their grafts explanted. Freedom from graft explantation was 100% at 1 year after implantation, 97.4% +/- 2.5% at 3 years, and 89.0% +/- 6.2% at 5 years. Freedom from graft dysfunction was 92.4% +/- 4.2% at 1 year, 78.8% +/- 7.3% at 3 years, and 52.9% +/- 10.9% at 5 years. Diagnosis of truncus arteriosus was identified as a factor for graft dysfunction (p = 0.028). Size-reduced homografts may provide an alternative for right ventricular outflow tract reconstruction when an appropriately sized homograft is unavailable.

Truncus Arteriosus: Diagnostic Accuracy, Outcomes, and Impact of Prenatal Diagnosis

Limited data exist on the impact of prenatal diagnosis and outcomes of fetal truncus arteriosus (TA). We sought to assess prenatal diagnostic accuracy and prenatal outcomes in fetuses with TA and compare postnatal outcomes in neonates with prenatally and postnatally diagnosed TA. Records were reviewed for patients diagnosed with TA in utero or at <or=60 days of life from 1992 to 2007. Forty-three (32%) of 136 TA patients had prenatal diagnosis. Five patients with TA were prenatally misdiagnosed, and 5 with other congenital heart diseases were misdiagnosed with TA prenatally. Of 28 fetuses diagnosed at <24 weeks gestation, 19 (68%) did not survive to birth because of spontaneous fetal death (n = 2) or because of elective termination (n = 17). Pregnancy termination was not more likely for fetuses with extracardiac anomalies. Of 19 live-born patients with correct prenatal diagnosis of TA, 2 (11%) died before surgery, and 4 (24%) died in the early postoperative period. All patients who died presurgically had been diagnosed prenatally. Overall, early postoperative mortality was 10%. Prenatal diagnosis of TA remains challenging and is associated with a high rate of elective termination. Fetal diagnosis was associated with younger age at repair but was not associated with improved neonatal survival.

P04.12: A rare variant of the fetal transposition of the great arteries: diagnostic difficulties

To present a case of fetal transposition of the great arteries with the rare variant of the aorta in posterior position because we believe it is more difficult to diagnose. Furthermore, this variant is not usually described in articles or books concerning the transposition of the great arteries. We saw the patient for the first time in the 23rd week of pregnancy. The fetal exploration was normal, except for the cardiac examination in which we only detected two vessels in the three-vessel view. One of the vessels was the superior vena cava and the other was an artery. With these findings we considered the diagnosis of truncus arteriosus, but after viewing many sections, we detected the outflow of the aorta in a very deviated and posterior position. This was the reason why we could not detect the three vascular structures in the three-vessel view. When looking for the appearance of outflow tracts, we saw that the pulmonary vessels arise from the left ventricle and the aorta from the right ventricle. This was the clue to making the diagnosis of transposition of the great arteries with posterior position of the aorta. In this case of transposition of the arteries with abnormal position of the aorta the correct diagnosis of the cardiac malformation may be difficult.

Right Ventricular Outflow Tract Reconstruction With an Allograft Conduit in Non-Ross Patients: Risk Factors for Allograft Dysfunction and Failure

Allograft conduits (AC) are used for reconstruction of the right ventricular outflow tract (RVOT) in children with RVOT hypoplasia or atresia and for pulmonary valve replacement in children undergoing Ross aortic valve replacement (AVR). Children undergoing the Ross AVR are generally older and have their AC inserted in the orthotopic position as opposed to the heterotopic position used for most cases of complex RVOT obstruction. The orthotopic position of the AC combined with the fact that the AC in the Ross patients are larger and can be oversized are the three factors that increase the right ventricular-pulmonary artery (RV-PA) conduit durability in the Ross AVR group. A retrospective evaluation of our experience with use of AC in non-Ross patients for reconstruction of the RVOT was conducted to determine the risk factors for failure. Between January 1985 and December 2003, 117 non-Ross patients received AC (94 pulmonary and 23 aortic) for RVOT reconstruction. Median age at implantation was 8 months (mean 4.3 +/- 7.1 years; range, 6 days to 43 years). There were 57 children (49%) less than 12 months of age. Endpoints were AC failure (valve explant, balloon dilatation), and AC dysfunction (AC stenosis >40 mm Hg and AC insufficiency more than 2+). There were no device-related deaths. Overall patient survival was 80% at 15 years. Freedom from AC failure was 60% at 5 years and 43% at 15 years. Freedom from failure was worse in infants (42% and 34% at 5 and 15 years, respectively). Freedom from AC dysfunction was 40% at 5 years and 23% at 15 years. Freedom from dysfunction was worse in infants (21% and 16% at 5 and 15 years, respectively). Univariate analysis identified younger patient age, smaller AC size, diagnosis of truncus arteriosus, and the presence of aortic AC as risk factors for AC dysfunction and failure. Multivariate analysis identified smaller AC size and the presence truncus arteriosus as risk factors for AC dysfunction and failure. Right ventricular outflow tract reconstruction with an AC in non-Ross patients has poor performance at midterm follow-up with AC dysfunction and failure of, respectively, 60% and 40% for the entire group and 79% and 58% in the infant group at 5 years. An alternate conduit for this application must be considered.

Clinical application of electron beam computed tomography in diagnosis of truncus arteriosus

目的 探讨电子束CT(EBCT)在共同动脉干(TA)诊断中的应用价值.方法全组共10例TA患者,年龄2个月至24岁.EBCT检查采用美国Imatron C-150型扫描机进行对比剂增强扫描并做出诊断.将同一患者EBCT检查结果和超声心动图(UCG)(10例)、心血管造影(3例)检查结果及手术所见(1例)进行比较分析.结果 EBCT对10例TA均做出定性诊断和分型诊断.UCG对9例做出定性诊断,5例与EBCT分型诊断一致.EBCT对TA并发畸形显示优于UCG.3例行心血管造影的患者中,1例造影诊断与EBCT诊断一致,2例分型诊断有误.1例根据EBCT和右心导管检查结果完成手术.结论 EBCT作为1种无创检查技术,完全能够完成TA的诊断及分型,结合UCG和必要的右心导管检查的结果,可以指导手术。

Prenatal sonographic diagnosis of truncus arteriosus associated with holoprosencephaly

We present the case of a truncus arteriosus associated with holoprosencephaly detected in a fetus in the first trimester and the sonographic findings that established this diagnosis. A physical exam was performed on a 35-year-old pregnant woman, gravida 2, para 1. At 20 weeks, sonography showed large-for-date uterine size. Fetal biometry was consistent with menstrual age. Amniotic fluid volume was elevated to 25 AFI cm. Fetal echocardiography revealed a single common artery arising from the heart, a ventricular septal defect, and other cardiac defects. Abnormalities of the fetal brain and face were also shown via sonography, including the presence of a common lateral ventricle without falx cerebri. Alobar holoprosencephaly, the most severe form of holoprosencephaly, was diagnosed. Prenatal chromosomal analysis was offered and the patient elected to have cordocentesis. The chromosome study was normal. Success in surgical repair of truncus arteriosus is established, but alobar holoprosencephaly is associated with neonatal death. Continuation of the pregnancy carried risks for the mother. After proper counseling, the parents of this fetus were offered and accepted termination of pregnancy. A male abortus weighing 320 grams was delivered. Postnatal findings and autopsy confirmed the diagnosis.

Surgical treatment of truncus arteriosus in the first 6 months of life.

One hundred six infants were seen at the University of California Medical Center between 1974 and 1981 with the diagnosis of truncus arteriosus. One hundred of these underwent physiologic correction prior to 6 months of age. Six infants died prior to operation while undergoing intense medical therapy to improve their basic condition. There were 11 operative deaths with a mortality rate of 11%. Of the 86 long-term survivors, 55 have returned for conduit change because of either body growth or pseudointima proliferation of the conduit. There had been no mortalities at the time of conduit change, and 29 of these were repaired using a straight tube between the ventricle and pulmonary trunk, while 26 had valve conduits placed. Physiologic correction in the first 6 months of life has been accomplished with a low mortality rate and apparent good long-term results with none of the survivors having evidence of elevated pulmonary vascular resistance.

Definitive diagnosis of truncus arteriosus by two-dimensional echocardiography

Definitive diagnosis of truncus arteriosus by two-dimensional echocardiography

TRUNCAL VALVE ECHOES IN THE DIFFERENTIATION OF TRUNCUS ARTERIOSUS FROM TETRALOGY OF FALLOT

It has not been possible to distinguish tetralogy of Fallot (TF) from truncus arteriosus (TA) by echocardiography (E) when the pulmonary valve is not identified. E from 18 patients with catheterization proven diagnosis of TA were compared to 16 patients with TF with and without pulmonary atresia. Features in common to the 2 groups included a single enlarged great vessel (83% TA, 75% TF) with overriding of the interventricular septum (78% TA, 88% TF). Differential signs included left atrial size and semilunar valve abnormalities. Left atrial enlargement was common in TA (78%) and unusual in TF (25%), reflecting differences in pulmonary blood flow. Multiple systolic and dlastolic linear duplications of the semilunar valve were recorded in 67% of TA, but were not seen in a single TF patient. These reflect either abnormal numbers of cusps, cusp asymmetries or thickenings which are extremely common in TA and rare in TF specimens. The differential diagnosis of TA can be made by E when the following criteria are met: a single enlarged great vessel, overriding the interventricular septum, left atrial enlargement, and most importantly, semilunar valve duplications.

Aortopulmonary Window, Ventricular Septal Defect, and Membranous Pulmonary Atresia with a Diagnosis of Truncus Arteriosus

An infant with aortopulmonary window, ventricular septal defect, membranous pulmonary atresia, right aortic arch, and anomalous origin of the left coronary artery from the pulmonary artery was believed to have persistent truncus arteriosus following cardiac catheterization. Thirty-five percent of patients with aortopulmonary window have coexistent cardiac anomalies; these include ventricular septal defect (7.7 percent), right aortic arch (6.8 percent), anomalous origin of left coronary artery from pulmonary artery (5.1 percent), and patent ductus arteriosus (11 percent). The differential diagnosis of aortopulmonary window and persistent truncus arteriosus is difficult when associated anomalies are present in patients with aortopulmonary window. Evaluation before operation on such patients should include an attempt to delineate coronary arterial origins.