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Tricuspid Atresia with Absent Pulmonary Valve with Nearly Discontinuous Branch Pulmonary Arteries

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Abstract

Absent pulmonary valve with tricuspid atresia or tricuspid stenosis (APV-TA/TS) is an extremely rare congenital heart defect associated with significant morbidity and mortality. Compared to Tetralogy of Fallot with Absent Pulmonary Valve Syndrome, branch pulmonary arteries are not typically significantly dilated. We present the case of a newborn male prenatally diagnosed APV-TA with intact ventricular septum (IVS) and nearly discontinuous branch pulmonary arteries, the surgical strategy employed, and the salient hemodynamic factors considered in the medical decision-making.

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None of the authors have any relevant sources of funding to report.

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Authors and Affiliations

  1. Lurie Children’s Hospital, Chicago, USA

    Justin B. Jin, Joshua D. Robinson, Joseph A. Camarda, Michael B. Satzer, Michael R. Carr, Michael Monge & Angira Patel

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  1. Justin B. Jin
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  2. Joshua D. Robinson
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  3. Joseph A. Camarda
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  4. Michael B. Satzer
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  5. Michael R. Carr
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  6. Michael Monge
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  7. Angira Patel
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Contributions

JBJ, JDR, JAC, MBS, MRC, MM, and AP all contributed to the main manuscript. JBJ prepared the figures. All authors reviewed the manuscript.

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Correspondence to Justin B. Jin.

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Jin, J.B., Robinson, J.D., Camarda, J.A. et al. Tricuspid Atresia with Absent Pulmonary Valve with Nearly Discontinuous Branch Pulmonary Arteries. Pediatr Cardiol 45, 446–451 (2024). https://doi.org/10.1007/s00246-023-03331-6

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  • DOI: https://doi.org/10.1007/s00246-023-03331-6

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