Absent Pulmonary Valve Syndrome with Non-Confluent Branches: Case Report and Literature Review

DOI: https://doi.org/10.30702/ujcvs/24.32(02)/HS037-161167
Keywords: congenital heart defects, stent, patent ductus arteriosus, newborns, total repair, prenatal diagnosis, current trends

Abstract

Absent pulmonary valve syndrome (APVS) is a rare form of congenital heart defect. Among the various variations of this rare anomaly are non-confluent branches of the pulmonary artery (PA) with anomalous origin of one of the PA branches from the patent ductus arteriosus (PDA). Constriction of the PDA in early neonatal age may result in cessation of blood supply to one of the pulmonary artery branches, leading to exclusion of one lung from circulation and gas exchange processes. PDA stenting creates a reliable source of blood flow, and as a first step, an endovascular approach was chosen for treatment.

Case report. A newborn (3 kg) with a prenatal diagnosis of APVS and a non-confluent left PA branch filling through PDA was chosen for PDA stenting. Considering the complex anatomy of the heart defect, it was decided to perform PDA stenting to delay total repair until the patient reaches optimal age and weight. Our patient successfully underwent PDA stenting as the first stage, which provided conditions for blood supply to one lung. The second stage involved total repair with good long-term results.

Conclusion. APVS treatment may have several options of surgical correction, differing from each other depending on the anatomical features of the defect and the patient’s age. Palliative treatment options help the child transition from the neonatal period and gain optimal weight for total repair.. Analyzing the data from the presented literature, it is noted that prenatal diagnosis is an important stage in determining the urgency and choice of treatment tactics for this defect.

References

  1. Escalon JG, Browne LP, Bang TJ, Restrepo CS, Ocazionez D, Vargas D. Congenital anomalies of the pulmonary arteries: an imaging overview. Br J Radiol. 2019 Jan;92(1093):20180185. https://doi.org/10.1259/bjr.20180185
  2. Keivanidou A, Gogou M, Giannopoulos A. Tetralogy of Fallot with absent pulmonary valve syndrome; an imaging challenge. Images Paediatr Cardiol. 2015 Apr-Jun;17(2):1-2.
  3. Smith RD, DuShane JW, Edwards JE. Congenital Insufficiency of the Pulmonary Valve: Including a Case of Fetal Cardiac Failure. Circulation. 1959;20(4):554-560. https://doi.org/10.1161/01.CIR.20.4.554
  4. Hraska V. A new approach to correction of tetralogy of Fallot with absent pulmonary valve. Ann Thorac Surg. 2000;69(5):1601-1602; discussion 1603. https://doi.org/10.1016/s0003-4975(00)01146-2
  5. Katewa A, Saxena S, Malhotra P, Rajesh VSP, Champaneri B, Surti J. Unilateral absence of pulmonary artery with absent pulmonary valve in tetralogy of Fallot. Indian J Thorac Cardiovasc Surg. 2022;38(6):637-643. https://doi.org/10.1007/s12055-022-01393-4
  6. Yong MS, Yim D, Brizard CP, Robertson T, Bullock A, d’Udekem Y, et al. Long-Term Outcomes of Patients With Absent Pulmonary Valve Syndrome: 38 Years of Experience. Ann Thorac Surg. 2014;97(5):1671-1677. https://doi.org/10.1016/j.athoracsur.2014.01.035
  7. Nair AK, Haranal M, Elkhatim IM, Dillon J, Hew CC, Sivalingam S. Surgical outcomes of absent pulmonary valve syndrome: An institutional experience. Ann Pediatr Cardiol. 2020;13(3):212-219. https://doi.org/10.4103/apc.APC_111_19
  8. Moon-Grady AJ, Tacy TA, Brook MM, Hanley FL, Silverman NH. Value of clinical and echocardiographic features in predicting outcome in the fetus, infant, and child with tetralogy of Fallot with absent pulmonary valve complex. Am J Cardiol. 2002;89(11):1280-1285. https://doi.org/10.1016/S0002-9149(02)02326-3
  9. Bockeria LA, Podzolkov VP, Makhachev OA, Zelenikin MA, Alekian BG, Ilyin VN, et al. Surgical Correction of Tetralogy of Fallot With Unilateral Absence of Pulmonary Artery. Ann Thorac Surg. 2007;83(2):613-618. https://doi.org/10.1016/j.athoracsur.2006.08.022
  10. Rao S, Najm HK, Stewart RD, Ahmad M, Erenberg F, Yaman M. Tetralogy of Fallot with absent pulmonary valve-When the ductus is present: A case of isolated branch pulmonary artery and review of literature. Echocardiography. 2019;36(5):996-1000. https://doi.org/10.1111/echo.14334
  11. Rakha S, Alkhushi N. Fetal diagnosis of isolated absent pulmonary valve with intact interventricular septum:How to counsel the parents? Ann Pediatr Cardiol. 2020;13(2):136-140. https://doi.org/10.4103/apc.APC_101_19
  12. Recker F, Weber EC, Strizek B, Geipel A, Berg C, Gembruch U. Management and outcome of prenatal absent pulmonary valve syndrome. Arch Gynecol Obstet. 2022;306(5):1449-1454. https://doi.org/10.1007/s00404-022-06397-4
  13. Sabu B. Absent pulmonary valve syndrome: prenatal diagnosis and postnatal correlation with a review of literature [abstract]. Ultrasound Obstet Gynecol. 2021;58:191. https://doi.org/10.1002/uog.24358
  14. Swaminathan S, Agarwal A, Infante JC, Rosenkranz E. Tetralogy of Fallot With Absent Pulmonary Valve and Nonconfluent Pulmonary Arteries: AManagement Conundrum. World J Pediatr Congenit Heart Surg. 2020;11(4):NP168-NP171. https://doi.org/10.1177/2150135118775661
  15. Avdikos V, Johansson Ramgren J, Hanséus K, Malm T, Liuba P. Outcomes following surgical repair of absent pulmonary valve syndrome: 30 years of experience from a Swedish tertiary referral centre. Interact Cardiovasc Thorac Surg. 2022 Jul 9;35(2):ivac193. https://doi.org/10.1093/icvts/ivac193
  16. Yamamoto Y, Harada D, Akiyama S, Noma M, Nagamine H, Maeda J, et al. Repair of Tetralogy of Fallot With Absent Pulmonary Valve and Nonconfluent Left Pulmonary Artery. Ann Thorac Surg Short Rep. 2023;1(4):682-684. https://doi.org/10.1016/j.atssr.2023.06.014
  17. Das D, Dutta N, Narayan P, Maiti S Tetralogy of Fallot With Absent Pulmonary Valve: Repair by Anterior Translocation of Branch Pulmonary Arteries and Reduction Plasty. CTSNet, Inc. Media [Internet]; 2024 Apr 16 [cited 2024 May 1]. https://doi.org/10.25373/ctsnet.25612179.v1
Published
2024-06-27
How to Cite
Hura, T. Y., Stychynskyi, O. S., Teterin, O. V., & Tammo, R. (2024). Absent Pulmonary Valve Syndrome with Non-Confluent Branches: Case Report and Literature Review. Ukrainian Journal of Cardiovascular Surgery, 32(2), 162-167. https://doi.org/10.30702/ujcvs/24.32(02)/HS037-161167
Issue
Ukrainian Journal of Cardiovascular Surgery Vol 32 No 2 2024
Section
CASE REPORT