Multicenter Analysis of Truncal Valve Management and Outcomes in Children with Truncus Arteriosus

Bakar, Adnan; Costello, John M.; Sassalos, Peter; Amula, Venugopal; Buckley, Jason R.; Smerling, Arthur J.; Iliopoulos, Ilias; Riley, Christine; Jennings, Aimee; Cashen, Katherine; Narasimhulu, Sukumar Suguna; Gowda, Keshava Murthy Narayana; Wilhelm, Michael; Badheka, Aditya; Slaven, James E.; Mastropietro, Christopher W.

doi:10.1007/s00246-020-02405-z

Cited by 9 publications

(6 citation statements)

References 19 publications

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“…Pre-operative truncal valve dysfunction is a known risk factor for death and/or need for re-intervention following initial surgical repair. [23,24] There has been data suggesting that prenatal truncal valve dysfunction may be an important predictor of pre-surgical mortality as well. [25,26] Truncal valve stenosis can increase ventricular afterload contributing to ventricular remodeling and dysfunction.…”

Section: Clinical Variablesmentioning

confidence: 99%

Fetal Echocardiographic Variables Associated with Perinatal Mortality in Truncus Arteriosus – A Pilot Study

Cox

Husain

Geiger³

et al. 2022

Preprint

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Background: 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 perinatal pre-surgical mortality in prenatally detected TA. Methods: This two-center, retrospective cohort study included fetuses diagnosed with TA between 01/2010 and 04/2020. The primary outcome was perinatal mortality, defined by fetal or neonatal pre-surgical death or transplantation prior to discharge. Univariable regression modeling and Chi-square test assessed for associations between prenatal clinical, demographic, and fetal-echo variables and perinatal mortality. Results: Of 23 pregnancies with prenatal diagnosis of TA, 4(17%) were terminated. Of the remaining 19, perinatal 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 perinatal 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(CVP)<7(0%vs.40%;p=0.01), skin edema (0%vs.40%;p=0.01), and abnormal umbilical venous(UV) Doppler (60%vs.0%;p=0.002). Presence of truncal valve regurgitation or stenosis neared significance. Conclusion: In prenatally diagnosed TA, there is significant perinatal mortality, including fetal death and neonatal pre-surgical death. Termination rate is also high. Fetal echocardiographic variables associated with perinatal mortality include ventricular dysfunction, low CVP, skin edema, and abnormal UV Doppler. Knowledge about prenatal risk factors for perinatal mortality may guide parental counseling and guide postnatal planning in prenatally diagnosed TA.

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Section: Clinical Variablesmentioning

confidence: 99%

Fetal Echocardiographic Variables Associated with Perinatal Mortality in Truncus Arteriosus – A Pilot Study

Cox

Husain

Geiger³

et al. 2022

Preprint

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“…4 Prior studies have reported between 12 and 28% of primary truncus arteriosus repairs requiring concomitant truncal valve intervention. [5][6][7][8] Truncal valve intervention can be divided into two broad categories: repair and replacement. Truncal valve repair is generally preferable to replacement because repaired valves grow with the patient.…”

mentioning

confidence: 99%

“…Despite this, patients undergoing truncal valve repair still require high rates of reintervention compared with patients not requiring truncal valve intervention. 5,6,8 Repair can be achieved using a variety of surgical techniques and is generally attempted in cases of moderate insufficiency with a truncal valve that is structurally amenable to repair. 9 In cases of severe truncal valve insufficiency, truncal valve replacement may be required during the primary repair of truncus arteriosus.…”

mentioning

confidence: 99%

“…While many studies have sought to evaluate the impact of truncal valve intervention on patient outcomes, 5,6,13 there is a paucity of literature analysing outcomes for the subset of these patients requiring truncal valve replacement during the primary repair of truncus arteriosus. Therefore, to better characterise the outcomes of these patients, we performed a meta-analysis of published outcomes.…”

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confidence: 99%

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Outcomes of truncal valve replacement in neonates and infants: a meta-analysis

et al. 2023

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Background: Infants with truncus arteriosus typically undergo repair by repurposing the truncal valve as the neo-aortic valve and using a valved conduit homograft for the neo-pulmonary valve. In cases where the native truncal valve is too insufficient for repair, it is replaced, but this is a rare occurrence with a paucity of data, especially in the infant population. Here, we conduct a meta-analysis to better understand the outcomes of infant truncal valve replacement during the primary repair of truncus arteriosus. Methods: We systematically reviewed PubMed, Scopus, and CINAHL for all studies reporting infant (<12 months) truncus arteriosus outcomes between 1974 and 2021. Exclusion criteria were studies which did not report truncal valve replacement outcomes separately. Data extracted included valve replacement type, mortality, and reintervention. Our primary outcome was early mortality, and our secondary outcomes were late mortality and reintervention rates. Results: Sixteen studies with 41 infants who underwent truncal valve replacement were included. The truncal valve replacement types were homografts (68.8%), mechanical valves (28.1%), and bioprosthetic valves (3.1%). Overall early mortality was 49.4% (95% CI: 28.4–70.5). The pooled late mortality rate was 15.3%/year (95% CI: 5.8–40.7). The overall rate of truncal valve reintervention was 21.7%/year (95% CI: 8.4–55.7). Conclusions: Infant truncal valve replacement has poor early and late mortality as well as high rates of reintervention. Truncal valve replacement therefore remains an unsolved problem in congenital cardiac surgery. Innovations in congenital cardiac surgery, such as partial heart transplantation, are required to address this.

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“…3,4 On the other hand, truncal valvuloplasty (TVP) is challenging, especially when performed in neonates and small infants in whom it is also associated with death risk. 1,5,6 Naimo and colleagues 7 from Melbourne provide a review of recent literature on TVP. They describe various TVP strategies and focus on outcomes such as early repair success and longevity, operative death, and late attrition.…”

mentioning

confidence: 99%

Commentary: Truncal valve insufficiency: The dilemma between limiting challenges and challenging limits

Alsoufi

2021

The Journal of Thoracic and Cardiovascular Surgery

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Multicenter Analysis of Truncal Valve Management and Outcomes in Children with Truncus Arteriosus

Cited by 9 publications

References 19 publications

Fetal Echocardiographic Variables Associated with Perinatal Mortality in Truncus Arteriosus – A Pilot Study

Fetal Echocardiographic Variables Associated with Perinatal Mortality in Truncus Arteriosus – A Pilot Study

Outcomes of truncal valve replacement in neonates and infants: a meta-analysis

Commentary: Truncal valve insufficiency: The dilemma between limiting challenges and challenging limits

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