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

Brown, John W.; Ruzmetov, Mark; Okada, Yuji; Vijay, Palaniswamy; Turrentine, Mark W.

doi:10.1016/s1010-7940(01)00816-8

Cited by 120 publications

(122 citation statements)

References 24 publications

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“…Aortic arch anomalies were the most frequent additional cardiac malformations [22]. The mortality rate found for our patients was similar to that reported for surgical repair of interrupted aortic arch (31%) [27], but slightly higher than that described for pulmonary atresia and ventricular septal defect (15-22%) [7,19] and for truncus arteriosus (13-17%) [6,28]. It should be noted that studies reporting the outcome of selected conotruncal malformations did not differentiate between patients with and those without the microdeletion 22q11.2, and therefore are not completely comparable with our study [6,9,27].…”

Section: Discussionsupporting

confidence: 83%

The Fate of Children with Microdeletion 22q11.2 Syndrome and Congenital Heart Defect: Clinical Course and Cardiac Outcome

et al. 2007

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Background This study aimed to evaluate the cardiac outcome for children with microdeletion 22q11.2 and congenital heart defect (CHD). Methods A total of 49 consecutive children with 22q11.2 and CHD were retrospectively identified. The CHD consisted of tetralogy of Fallot and variances (n = 22), interrupted aortic arch (n = 10), ventricular septal defect (n = 8), truncus arteriosus (n = 6), and double aortic arch (n = 1). Extracardiac anomalies were present in 46 of 47 children.Results The median follow-up time was 8.5 years (range, 3 months to 23.5 years). Cardiac surgical repair was performed for 35 children, whereas 5 had palliative surgery, and 9 never underwent cardiac surgery. The median age at repair was 7.5 months (range, 2 days to 5 years). The mean hospital stay was 35 days (range, 7-204 days), and the intensive care unit stay was 15 days (range, 3-194 days). Significant postoperative complications occurred for 26 children (74%), and surgery for extracardiac malformations was required for 21 patients (43%). The overall mortality rate was 22% (11/49), with 1-year survival for 86% and 5-year survival for 80% of the patients. A total of 27 cardiac reinterventions were performed for 16 patients (46%) including 15 reoperations and 12 interventional catheterizations. Residual cardiac findings were present in 25 patients (71%) at the end of the follow-up period. Conclusions Children with microdeletion 22q11.2 and CHD are at high risk for mortality and morbidity, as determined by both the severity of the cardiac lesions and the extracardiac anomalies associated with the microdeletion.

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Section: Discussionsupporting

confidence: 83%

The Fate of Children with Microdeletion 22q11.2 Syndrome and Congenital Heart Defect: Clinical Course and Cardiac Outcome

et al. 2007

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“…3 Initially, the surgical mortality rate was high, at around 20%. 5,13 In our institute, we have performed truncus arteriosus repair since 1974. There were 17 (31%) in-hospital deaths.…”

Section: Discussionmentioning

confidence: 99%

“…Previous investigators also reported that 34-50% of patients required reoperation for conduit dysfunction and branched PA stenosis. 5,6,[13][14][15] Truncus arteriosus repair requires a small conduit in neonates and young infants, which inevitably results in Figure 4. Actuarial freedom from truncal valve operation among hospital survivors after truncus arteriosus repair.…”

Section: Discussionmentioning

confidence: 99%

Long-term Outcomes after Truncus Arteriosus Repair: A Single-center Experience for More than 40 Years

Asagai

Inai

Shinohara

et al. 2016

Congenital Heart Disease

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A B S T R A C TObjectives. This study aimed to analyze long-term survival and functional outcomes after truncus arteriosus repair in a single institution with more than 40 years of follow-up. Methods. Medical records were analyzed retrospectively in 52 patients who underwent the Rastelli procedure for truncus arteriosus repair between 1974 and 2002. Thirty-five patients survived the initial repair. The median age at the initial operation was 2.8 months (range, 0.1-123 months) and the body weight was 3.9 kg (range, 1.6 to 15.0 kg). Results. The median age at follow-up was 23.6 years (range, 12.4 to 44.5 years). The median follow-up duration was 23.4 years (range, 12.3 to 40.7 years). The actuarial survival rate was 97% at 10 years and 93% at both 20 years and 40 years after the initial operation. At follow-up, most patients were in New York Heart Association (NYHA) functional classes I (73%) and II (24%). Thirty-six percent of patients had full-time jobs, 40% were students, and 21% were unemployed. Most patients (97%) had undergone conduit reoperations. Freedom from reoperation for right ventricular (RV) outflow and pulmonary artery (PA) stenosis was 59% at 5 years, 28% at 10 years, and 3% at 20 years after the initial operation. Freedom from catheter interventions for RV outflow and PA stenosis was 59% at 5 years, 47% at 10 years, and 38% at 20 years after the initial operation. Freedom from truncal valve replacement was 88% at 5 years, 85% at 10 years, and 70% at 20 years after the initial operation. Conclusions. In this single-center retrospective study, with long-term follow-up after repair of truncus arteriosus, long-term survival and functional outcomes were acceptable, despite the requirement for reoperation and multiple catheter interventions for RV outflow and PA stenosis in almost all patients, and the frequent requirement for late truncal valve operations.

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“…As has been previously described, the lesion is characterized by single large arterial trunk originating from the base of the heart directly superior to a high VSD. PA originates from the trunk [9]. The defect is now correctable by closing the VSD and inserting a conduit between the right ventricle and pulmonary artery [10].…”

Section: Discussionmentioning

confidence: 99%

Repair of Truncus Arteriosus, Type 1 in Nigeria: A Case Report

Ia¹,

Novick²,

Dk³

et al. 2016

J Vasc Med Surg

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Truncus arteriosus, also known as common arterial trunk is an unusual embryologic anomaly. It is characterized by incomplete conotuncal septation resulting in a common aortopulmonary trunk and ventricular septal defect. The first surgical repair was performed in 1962 when Behdtrendt et al. closed the VSD and used a valveless conduit to establish right ventricle-pulmonary artery continuity. Complete repair of this anomaly with a valved conduit was first reported in 1967 by McGoon et al. and remains the procedure of choice for these patients. National Cardiothoracic Center of Excellence was established in 1984. Since then it had carried out significant surgical management of simple CHD. Recently, with the aid of foreign medical missions, it was able to carry out a successful repair of truncus arteriosus type 1, the first of its kind in Nigeria.

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

Abstract: 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.

Cited by 120 publications

References 24 publications

The Fate of Children with Microdeletion 22q11.2 Syndrome and Congenital Heart Defect: Clinical Course and Cardiac Outcome

The Fate of Children with Microdeletion 22q11.2 Syndrome and Congenital Heart Defect: Clinical Course and Cardiac Outcome

Long-term Outcomes after Truncus Arteriosus Repair: A Single-center Experience for More than 40 Years

Repair of Truncus Arteriosus, Type 1 in Nigeria: A Case Report

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