Severe Truncal Valve Regurgitation Research Articles

A case of truncus arteriosus with severe heart failure and pulmonary stenosis: bridge to transplant candidacy with surgical correction and a ventricular-assist device.

Ventricular-assist device therapy for small patients with congenital heart disease is challenging due to its complex anatomy and hemodynamics. We describe a 3-year-old patient with heart failure with truncus arteriosus in the palliative stage. The patient underwent palliative right ventricular outflow tract reconstruction following bilateral pulmonary artery banding. At 6months of age, the patient developed severe truncal valve regurgitation and left ventricular dysfunction. Emergent truncal valve replacement with a mechanical valve was performed, but left ventricular dysfunction persisted. At 3years of age, the patient developed acute progression of heart failure triggered by influenza infection. The patient was intubated and transferred to our center to determine the indication for heart transplantation. On the second day after admission, signs of multiorgan failure appeared. Emergent ventricular-assist device implantation for both ventricles was performed with truncal valve closure, ventricular septal defect closure, atrial septal defect closure, and re-right ventricular outflow tract reconstruction. The right ventricular-assist device was successfully removed on the seventh postoperative day. Due to the small pulmonary arteries, severe pulmonary stenosis persisted after ventricular-assist device implantation, but it gradually improved with multiple pulmonary angioplasties. The patient was registered in the Japanese organ transplant network and is awaiting a donor organ in a stable condition.

Impact of Truncal Valve Repair on Survival, Reintervention, and Left Ventricular Function

BackgroundTruncal valve insufficiency (TVI) is one of the risk factors for death in neonatal primary repair for common arterial trunk (CAT). MethodsIn this single-center retrospective case-matched controlled study, 16 consecutive CAT patients from 2000 to 2018 with moderate to severe truncal valve regurgitation (TVR2-3), undergoing primary CAT surgery with truncal valve repair, were matched to 16 CAT patients with none or mild truncal valve regurgitation (TVR1-0). ResultsThe TVR2-3 group had 11 (69%) patients with moderate and 5 (31%) patients with severe TVI, with an operative median age of 7 (4-19) days. Survival at median follow-up of 17 years after repair was 70% and 80% in the TVR2-3 and TVR0-1 groups, respectively (P > .99), with 2 early deaths in the TVR2-3 group occurring after reintervention for residual TVI. Rate of surgical truncal valve reintervention at 5 years postoperatively was 67% for TVR2-3 (P = .005). TVR2-3 experienced greater residual TVI at discharge and 1 year after repair, with severity of truncal valve dysfunction converging between groups as more patients in TVR0-1 developed mild to moderate TVI over time and TVR2-3 patients underwent reintervention for clinically significant TVI. Significant left ventricular (LV) dilation was observed in the TVR2-3 group after 3 years from repair (P = .001), but LV ejection fraction was comparable between groups. ConclusionsTruncal valve reintervention burden (ie, repair or replacement) is greater in the TVR2-3 population, with higher truncal valve–related early death. Progressive LV enlargement in the TVR2-3 group due to residual TVI was well tolerated. Ventricular remodeling did not have a notable impact on LV ejection fraction or clinical status.

Common arterial trunk in functionally univentricular hearts: a case series.

The association of a univentricular heart defect with common arterial trunk is extremely rare. There is a lack of population-based outcome studies reported in the literature. The hospital records, echocardiographic and other imaging modality data, outpatients' records, operation notes, and other electronic data were reviewed. Patients were reviewed, and the final outcomes of surgery were observed. Six cases (two males) with common arterial trunk presented over a 30-year period. Five had a complete unbalanced atrioventricular septal defect (83%) and one (17%) had tricuspid atresia associated with common arterial trunk. All had antenatal diagnosis. Two cases (33%) were excluded from initial surgical palliation due to Trisomy 21 in one and severe truncal valve regurgitation in one. Initial surgical palliation was performed in four cases (67%) at median age of 31 days (2-60) and consisted of disconnection and reconstruction of the pulmonary arteries and establishing controlled pulmonary blood flow. There were no early deaths. Conversion to cavopulmonary shunt was not possible in two due to severe airway problems in one and pulmonary arteries anatomy in one. They died at 11 and 16 months, respectively. Two patients (33%) underwent cavopulmonary shunt with 1 (17%) being alive at 18 months - 12 months after cavopulmonary shunt. The second patient proceeded to Fontan completion at 19 months but required catheter takedown 3 months later and died 3.5 years later. Univentricular hearts with common arterial trunk carry extremely poor short- to medium-term outcomes. This should inform antenatal and postnatal counselling and decision-making.

IMPACT OF TRUNCAL VALVE REGURGITATION ON LEFT VENTRICULAR FUNCTION AND GEOMETRY IN COMMON ARTERIAL TRUNK: A CASE-MATCH CONTROLLED STUDY

<h3>BACKGROUND</h3> Truncal valve insufficiency (TVI) not only is one of the risk factors for death in neonatal primary repair for common arterial trunk (CAT), but also influences long-term left ventricular performance. This study investigates the functional and survival outcomes of CAT repair in patients based on TVI severity. <h3>METHODS AND RESULTS</h3> In this single-center retrospective case-matched controlled study, 16 consecutive CAT patients from 2000- 2018 with moderate to severe truncal valve regurgitation (TVR2-3) undergoing primary CAT surgery with truncal valve (TrV) repair were matched to 16 CAT patients with none to mild truncal valve regurgitation (TVR1-0). Survival was analyzed using Kaplan-Meier; reintervention using competing risks; paired tests for ventricular function and geometry, and health status. TVR2-3 group had 11 (69%) patients with moderate TVR and 5 (31%) patients with severe TVI, with operative median age of 7(4 – 19) days. The TVR1-0 group had 3 (19%) patients with none/trivial TVI and 13 (81%) patients with mild TVI, with an operative median age of 132(6– 22) days (p = 0.30). There were no significant differences in preoperative variables between the groups and repair technique as shown in Table 1. There was no significant difference between overall survival and modified Ross heart failure score at median length of follow-up of 9.17 (1.40 – 15.12) years. Rate of surgical TrV reintervention at 5 years postoperative was 8% and 67% for TVR0-1 and TVR2-3, respectively (Stratified Gray's p = 0.005). TVR2-3 experienced greater residual TVI at discharge and 1 year post-repair with severity of TrV dysfunction converging between groups as more patients in TVR0-1 developed mild/moderate TVI over time and TVR2-3 patients underwent reintervention. Left ventricular ejection fraction (LVEF) was comparable between groups at all time-points, with the exception of postoperative year 1 [TVR0-1: 69(67 – 74)% vs. TVR2-3: 62(59 – 66)% (p = 0.006) Figure 1]. Although not statistically significant, pronounced left ventricular dilatation was seen in the TVR2-3 group (upper quartile, z-score +6) throughout 1-year post-repair. Increasing left ventricular size in TVR2-3 reached statistical significance at 3 (p = 0.001) and 5 years (p = 0.003), but did not impair LVEF. <h3>CONCLUSION</h3> There was a greater number of surgical TrV reinterventions (i.e. repair or replacement) in the TVR2-3 population, with two deaths associated with critical residual TVI refractory to reintervention. Progressive left ventricular dilatation in the TVR2-3 group due to residual TVI was well tolerated. Ventricular remodeling did not significantly impact left ventricular function or clinical status.

Truncal valve leaflet reconstruction with autologous pericardium in a neonate.

A boy diagnosed with persistent truncus arteriosus and severe truncal valve regurgitation had uncontrollable heart failure. Thus, truncal valve repair, ventricular septal defect closure, and right ventricular outflow tract reconstruction were performed on the second day of life. We report about a neonate with severe truncal valve regurgitation who was successfully treated with autologous pericardial truncal valve leaflet reconstruction.

Neonatal quadricuspid truncal valve repair with left coronary artery unroofing

Neonatal quadricuspid truncal valve repair with left coronary artery unroofing

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.

Truncal valve repair using autologous pericardial patch augmentation

A 35-month-old boy with severe truncal valve regurgitation underwent successful repair by tricuspidization using raphe slicing and an extension technique with glutaraldehyde-treated autologous pericardium. His truncal valve regurgitation improved from severe to mild 5 years postoperatively. Tricuspidization using raphe slicing and valve extension with autologous pericardium might be one of the alternatives for truncal valve regurgitation with a quadricuspid valve.

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.

Repair of Truncus Arteriosus and Aortic Arch Interruption: Outcome Analysis

The excellent results for repair of truncus arteriosus reported in some centers have not applied to patients with associated interrupted aortic arch. This work aims at understanding the discrepancy of results in our own experience. Ten patients among 83 consecutive children with truncus arteriosus repaired from 1987 to September 2004 who had aortic arch interruption were analyzed, with particular emphasis on clinical presentation and outcome. The comprehensive Aristotle complexity score was calculated for each patient. The Kaplan-Meier method was used to estimate survivals. Preoperative mechanical ventilation was necessary in 5 of the 10 patients; 2 of them were moribund. Associated major lesions were as follows: severe (n = 2) and moderate (n = 4) truncal valve regurgitation, coronary artery anomalies (n = 3) and Di-George's syndrome (n = 4). The comprehensive Aristotle score was at least 20 in 6 patients. There were 5 operative deaths (5 of 10); early mortality was 50% (95% confidence limits: 19% to 81%). These deaths occurred in patients with Aristotle score of 20 or greater (5 of 6 = 83%). All 4 patients who had no moderate or severe truncal valve regurgitation survived the intervention. Survival was a low 37.5% +/- 16.1% from 1 year on compared with a high 95.5% +/- 2.5% for the 73 patients without aortic arch interruption. This study confirms the predictive value of the Aristotle score, hospital mortality being significantly correlated with the highest Aristotle score (p = 0.024). To improve outcome in these high-risk patients, preoperative management should be optimized, repair should not be delayed, and regurgitant truncal valve should be repaired or replaced.

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.

Truncus arteriosus repair: outcomes, risk factors, reoperation and management.

Truncus arteriosus (TA) continues to be associated with significant morbidity and mortality, but there have been clinically significant improvements with early repair. Sixty patients underwent physiological correction of TA between November 1978 and January 2000. The average age was 76 days (range, 3 days--20 months). Associated cardiac anomalies were frequently encountered, the most common being severe truncal valve regurgitation (n=7), interrupted aortic arch (n=6), coronary artery anomalies (n=6), non-confluent pulmonary arteries (n=4), and total anomalous pulmonary venous return (n=1). Truncal valve replacement was performed initially or subsequently in seven patients with severe regurgitation (mechanical prostheses in six patients and a cryopreserved aortic homograft in one patient). Right ventricle--pulmonary artery continuity was established with an aortic (n=16) or pulmonary homograft (n=32) in 48 patients, a Dacron polyester porcine valved conduit in five, a non-valved polytetrafluoroethylene (PTFE) tube in three, direct anastomosis to the right ventricle with anterior patch arterioplasty in three, and a bovine jugular venous valve conduit in one patient. There were ten hospital deaths (17%; 70% confidence limit, 7--25%). Multivariate and univariate analyses demonstrated a relationship between hospital mortality and associated cardiac anomalies. In the 43 patients without these associated cardiac anomalies, the early survival was 91% (group I). In the 17 patients with one or more of these risk factors, the survival was 71% (group II, P=0.002). There was one late death. Twenty-three patients (46%) required reoperation for right ventricular outflow tract (RVOT) obstruction at a mean follow-up time of 59.1 months. In 23 patients, the RVOT reconstruction was performed with a PTFE monocusp, and six patients had of a variety of replacement conduits inserted. Postoperatively, there were 34 (68%) patients in New York Heart Association functional class I and 16 (32%) in class II. Twenty-eight surviving patients are reported as doing well without any medication. The freedom of reoperation in the 39 hospital survivors (group I) without risk factors was 64% at 7 years; and 36% at 10 years in the 11 patients (group II) surviving with risk factors. Associated cardiac anomalies were risk factors for death after the repair of TA. In the absence of these associated lesions, TA can be repaired with an excellent surgical outcome in the neonatal and early infancy period.

Truncus arteriosus: ten-year experience with homograft repair in neonates and infants

Background. Early complete repair of truncus arteriosus with homograft reconstruction of the right ventricular outflow tract was performed and long-term results were evaluated. Methods. Review of 46 consecutive patients with truncus arteriosus who underwent primary correction between June 1987 and May 1997 was performed. Ages ranged from 21 days to 7.2 years (median, 62 days) and weights from 1.8 to 21.5 kg (median, 3.4 kg). Ten patients were operated on before 1 month of age, 20 between 1 and 3 months, 13 between 3 months and 1 year, and 3 at an older age. Associated cardiac conditions were encountered frequently, the most common being coronary artery anomalies (n = 16), truncal valve incompetence (n = 12), and interrupted aortic arch (n = 5). The right ventricular outflow tract was reconstructed with an aortic (n = 28) or a pulmonary homograft (n = 18). Results. There were two hospital deaths (4.3%). Both patients had severe truncal valve regurgitation and interrupted aortic arch together with other unfavorable conditions. Survival in uncomplicated truncus arteriosus was 100%. Follow-up was from 3 months to 10 years (mean, 36 months). There was one late death 4 months after the initial repair, presumably because of cardiac reason. Actuarial survival was 93% at 4 months and beyond. Actuarial freedom of reoperation in the 27 hospital survivors with aortic homografts was 43% at 75 months; it was 73% at 62 months in the 17 patients surviving with pulmonary homografts. Conclusions. Neonatal or early infancy complete repair is the treatment with the best potential for survival. The homograft remains our conduit of choice to establish continuity between the right ventricle and the pulmonary artery. Management of severe truncal valve incompetence remains a surgical challenge.

Repair of truncus arteriosus in the neonate

From September 1986 through December 1991, 63 patients with truncus arteriosus underwent surgical repair. The management approach evolved over the period of the study from elective primary repair at 3 months of age to elective primary repair in the early neonatal period. Thirty variables were examined as potential risk factors for the outcome events of death, reoperation, and presence of pulmonary vascular morbidity. According to both univariate and multivariate techniques, severe truncal valve regurgitation, interrupted aortic arch, coronary artery anomalies, and age at repair greater than 100 days were important risk factors for perioperative death. In the 33 patients without these risk factors, early survival was 100%. In the 30 patients with one or more of these risk factors, survival was 63%. Pulmonary hypertensive episodes were fewer, and duration of ventilator dependence and pulmonary artery pressure were significantly less in patients undergoing the operation before 30 days of age. Seven patients required reoperation for right ventricular outflow tract obstruction at a mean follow-up time of 23.4 months, with no deaths. In conclusion, interrupted aortic arch, severe truncal valve insufficiency, coronary anomalies, and repair later than 100 days of age were risk factors for death after repair of truncus arteriosus. In the absence of these associations, truncus arteriosus can be repaired with excellent surgical outcome in the neonatal and early infancy period. Repair in the early neonatal period reduces the prevalence of postoperative pulmonary vascular morbidity.