Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2025 Oct 30:28:450-458.
doi: 10.1016/j.xjon.2025.09.051. eCollection 2025 Dec.

Risk factors for early right ventricular-to-pulmonary artery conduit failure in congenital heart disease

Fawaz Naeem  1 , Travus J White  2 , J William Gaynor  3 , Xuemei Zhang  4   5 , Steve Ampah  4   5 , Andrew C Glatz  6 , Laura Mercer-Rosa  2 , Meryl S Cohen  2
Affiliations

Risk factors for early right ventricular-to-pulmonary artery conduit failure in congenital heart disease

Fawaz Naeem et al. JTCVS Open. .

Abstract

Objectives: Right ventricle-to-pulmonary artery conduits are a mainstay of treatment for patients with congenital heart defects. We investigated the association of underlying genetic abnormalities with conduit failure and hypothesized that 22q11.2 deletion syndrome was associated with shorter time to failure.

Methods: We conducted a single-center retrospective cohort study at the Children's Hospital of Philadelphia. Patients who underwent right ventricle-to-pulmonary artery conduit placement as part of a biventricular repair at 0 to 24 months of age between January 2010 and June 2020 were included. The primary exposure of interest was diagnosis of 22q11.2 deletion syndrome, and the outcome was time to conduit failure. The statistical analysis used cumulative incidence function with the Gray test and cause-specific Cox regression to account for competing risk.

Results: In total, 143 patients met inclusion criteria, of whom 65 experienced conduit failure in the study period. The median time to failure was 89 months (7.4 years). Use of pulmonary homograft was independently associated with lower risk of conduit failure (hazard ratio, 0.29; confidence interval, 0.11-0.77, P = .01) and small conduit size was associated with greater risk of conduit failure (hazard ratio, 3.99, confidence interval, 1.69-9.41, P = .002) during the first 24 months. Presence of 22q11.2 deletion syndrome, age at initial surgery, and diagnosis of truncus arteriosus were not associated with conduit failure.

Conclusions: Although 22q11.2 deletion syndrome and other genetic syndromes were not associated with conduit failure, conduit size and type were the most important factors associated with conduit longevity and should be taken into account when planning surgical repair.

Keywords: 22q.11.2 deletion syndrome (DiGeorge syndrome); children; conduit failure; congenital heart disease; pulmonary artery conduit.

PubMed Disclaimer

Conflict of interest statement

The authors reported no conflicts of interest. The Journal policy requires editors and reviewers to disclose conflicts of interest and to decline handling or reviewing manuscripts for which they may have a conflict of interest. The editors and reviewers of this article have no conflicts of interest.

Figures

None
Graphical abstract
None
Cumulative incidence function of conduit failure with or without 22q11.2 deletion syndrome.
Figure 1
Figure 1
Cumulative incidence curve depicting time to conduit failure for all patients included int the study. Approximately one half of patients fail at 7.4 years after conduit placement.
Figure 2
Figure 2
Cumulative incidence curve depicting time to conduit failure depending on the presence of 22q11.2 deletion syndrome. Notably, there was no difference in the Gray test (P = .21), meaning 22q11.2 deletion syndrome had no significant impact on conduit longevity.
Figure 3
Figure 3
Cumulative incidence curve depicting time to conduit failure depending on the size of conduit placed. Small conduits demonstrated the fastest time to failure, whereas medium and large conduits were relatively similar in their outcomes (P = .0001).
Figure 4
Figure 4
Cumulative incidence curve depicting time to conduit failure depending on the type of homograft used. Pulmonary homografts were superior to aortic homografts (P = .0002).
Figure E1
Figure E1
Cumulative incidence curve depicting time to conduit failure depending on the presence of a diagnosis of truncus arteriosus. The Gray test demonstrated a significant difference in time to failure (P = .002).
Figure E2
Figure E2
Cumulative incidence curve depicting the time to conduit failure depending on whether or not the patient underwent a catheter interventions. The Gray test did not demonstrate a statistically significant time to failure.
See this image and copyright information in PMC

References

    1. Manavitehrani I., Ebrahimi P., Yang I., et al. Current challenges and emergent technologies for manufacturing artificial right ventricle to pulmonary artery (RV-PA) cardiac conduits. Cardiovasc Eng Technol. 2019;10:205–215. - PubMed
    1. Karamlou T., Blackstone E.H., Hawkins J.A., et al. And the Pulmonary Conduit Working Group for the Members of the Congenital Heart Surgeons Society Can pulmonary conduit dysfunction and failure be reduced in infants and children less than age 2 years at initial implantation? J Thorac Cardiovasc Surg. 2006;132(4):829–838. - PubMed
    1. Poynter J.A., Eghtesady P., McCrindle B.W., et al. Association of pulmonary conduit type and size with durability in infants and young children. Ann Thorac Surg. 2013;96(5):1695–1701. - PubMed
    1. Askovich B., Hawkins J.A., Sower C.T., et al. Right ventricle-to-pulmonary artery conduit longevity: is it related to allograft size? Ann Thorac Surg. 2007;84(3):907–912. - PubMed
    1. Mery C.M., Guzmán-Pruneda F.A., De León L.E., et al. Risk factors for development of endocarditis and reintervention in patients undergoing right ventricle to pulmonary artery valved conduit placement. J Thorac Cardiovasc Surg. 2016;151(2):432–439. - PubMed

LinkOut - more resources