Tetralogy of Fallot with pulmonary valve agenesis

Authors

  • Elizabeth Janet de la Paz-Loret de Mola Universidad de Ciencias Médicas de Camagüey. Facultad de Ciencias Médicas “Carlos Juan Finlay”. Camagüey https://orcid.org/0000-0003-1680-4233
  • Luis Lorenzo Céspedes-Sellén Universidad de Ciencias Médicas de Camagüey. Facultad de Ciencias Médicas “Carlos Juan Finlay”. Camagüey https://orcid.org/0000-0003-2750-3225
  • Elizabeth Sellén-Sanchén Universidad de Ciencias Médicas de Camagüey. Hospital Universitario “Manuel Ascunce Domenech”. Departamento de Cardiología. Camagüey https://orcid.org/0000-0001-5570-5428
  • Carlos Manuel Oliva-de Céspedes Universidad de Ciencias Médicas de Camagüey. Hospital Universitario “Eduardo Agramonte Piña”. Departamento de Cardiología. Camagüey https://orcid.org/0000-0002-7382-8402

DOI:

https://doi.org/10.5281/zenodo.7654358

Keywords:

Tetralogy of Fallot, Heart Valve Diseases, Heart Defects, Congenital, Cardiovascular Abnormalities, Heart Diseases.

Abstract

Introduction: tetralogy of Fallot with pulmonary valve agenesis is a complex congenital heart disease, with a prevalence of 2-6%, frequently associated with chromosomopathies.

Case report: 2-month-old male infant diagnosed on the fourth day of birth with Tetralogy of Fallot with pulmonary valve agenesis. He presented with a clinical picture characterized by respiratory symptoms such as dyspnea, mild distal cyanosis, dry cough, slight subcostal and intercostal initially, which evolved into a picture of metabolic alkalosis compensating for respiratory acidosis, with frequent events of bronchospasm due to compression of the bronchi by dilatation of the trunk and branches of the pulmonary artery. Treatment was clinical, symptom management. Mechanical ventilation in prone position, treatment with diuretics for hypertension and intravenous inotropic drugs were required. The patient died at 3 months of life.

Conclusions: tetralogy of Fallot with pulmonary valve agenesis is a complex, infrequent and potentially fatal entity, where patient survival is determined by complete primary correction of the defect, mainly before the onset of respiratory symptoms, and the infrequency and complexity of its management make this congenital heart disease a challenge for the medical community. A deep and continuous study of atypical heart disease is necessary to achieve better medical care.

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References

1. Gómez M, Vayo M, Ortiz L, Portis M, Echazarreta D, Marelli D. Tetralogía de Fallot con síndrome de válvula pulmonar ausente Presentación de un caso y revisión de la literatura. Insuf Card [Internet]. 2017 [citado 06/06/2021]; 12(2):91-95. Disponible en: http://www.insuficienciacardiaca.org/pdf/v12n2_17/v12n2a04.pdf

2. Morales E, Montenegro R. Arboleda C. Tetralogía de Fallot con agenesia de la válvula pulmonar. REV. ARG. CARDIOL [Internet]. 1984 [citado 06/06/2021]; 52(4):324-329. Disponible en: http://www.old2.sac.org.ar/wpcontent/uploads/2015/03/PDFs201503/1261.pdf

3. Cabrera A, Rodrigo D, Martínez P, Aramendi J, Alcíbar J, López de Heredia J, Romero C, et al. Tetralogía de Fallot con agenesia de válvula pulmonar en el recién nacido y lactante. Corrección completa. An Pediatr [Internet]. 2004 [citado 06/06/2021]; 60(2):180-3. Disponible en: https://dialnet.unirioja.es/servlet/articulo?codigo=7393616

4. Atik E, Passos F, Andrade J. Niña de 7 años, con tetralogía de Fallot y agenesia de la válvula pulmonar. Arq. Bras. Cardiol [Internet]. 2010 [citado 06/06/2021]; 95(3): .Disponible en: https://doi.org/10.1590/S0066-782X2010001300021

5. Fernández M, Fernández J, Juncedo S, Fernández B. Enfisema lobar congénito y agenesia de la válvula pulmonar. An Pediatr [Internet]. 2015[citado 06/06/2021];85(3):163-164.Disponible en: https://www.analesdepediatria.org/es-enfisema-lobar-congenito-agenesia-valvula-articulo-S1695403315003872

6. Del Valle AL, Sajmoló Pablo M. Anormalidades cromosómicas asociadas a cardiopatías congénitas en pacientes pediátricos. Rev Guatemalteca Cardiol [Internet]. 2013 [citado 06/06/2021]; 23(1): [aprox. 4 p.]. Disponible en: http://revista.agcardio.org/wpcontent/uploads/2013/06/Anomaliascromosomicasencardiopat%C3%ADascongenitas.pdf

7. Chow O, Hoganson D, Kaza A, Chávez M, Altin F, Marx G, Friedman K, et al. Early Infant Symptomatic Patients With Tetralogy of Fallot With Absent Pulmonary Valve: Pulmonary Artery Management and Airway Stabilization. Ann Thorac Surg [Internet]. 2020 [citado 06/06/2021];110(5):1644-1650. Disponible en: https://pubmed.ncbi.nlm.nih.gov/32615094/

8. Doravantu D, Stoicescu C, Tulloh R, Stoica S. Surgical Repair of Tetralogy of Fallot With Absent Pulmonary Valve: Favorable Long-Term Results. Semin Thorac Cardiovasc Surg [Internet]. 2019 [citado 06/06/2021];31(4):847-849. Disponible en: https://pubmed.ncbi.nlm.nih.gov/31150827/

9. Talwar S, Divya A, Choudhary SK, Kumar S, Ramakriahnan S, Sunder S. Mid-term results of correction of Tetralogy of Fallot with absent pulmonary valve. Indian Heart J [Internet]. 2017 [citado 06/06/2021]; 69(6):767-771. Disponible en: https://pubmed.ncbi.nlm.nih.gov/29174256/

10. Webb GD, Smallhorn JF, Therrien J y Redington AN. Cardiopatías congénitas. En: Mann DL, Zipes DP, Libby P, Bonow RO, Braunwald E, editores. Braunwald Tratado de Cardiologia.10a ed. España: Elsevier; 2019, p 1391-1446.

11. Bernstein D. Cardiopatías congénitas cianóticas: lesiones asociadas a un descenso del flujo sanguíneo pulmonar. En: Kliegman RM, Blum NJ, Shah SS, St Geme III JW, Tasker RC, Wilson KM, Behrman RE, editores. Nelson Tratado de Pediatría. 21a ed. Barcelona: Elsevier; 2020. p. 2396.

12. Grewal J, Majdalany D, Syed I, Pellikka P, Warnes CA. Three-dimensional echocardiographic assessment of right ventricular volume and function in adult patients with congenital heart disease: comparison with magnetic resonance imaging. J Am Soc Echocardiogr[Internet]. 2010 [citado 06/06/2021]; Feb;23(2):127-33. Disponible en: https://pubmed.ncbi.nlm.nih.gov/19962272/

13. Wang E, Fan X, Xiang L, Li S, Zhang H. Surgical outcome after complete repair of tetralogy of Fallot with absent pulmonary valve: comparison between bovine jugular vein-valved conduit and monocusp-valve patch. World J Pediatr [Internet]. 2018 [citado 06/06/2021]; 14(5):510-519. Disponible en: https://pubmed.ncbi.nlm.nih.gov/30062647/

14. Tenisch E, Raboisson M, Rypens F, Déry J,Grignon A , Chantale L. Significance of lung anomalies in fetuses affected by tetralogy of Fallot with absent pulmonary valve síndrome. Cardiol Young [Internet]. 2017 [citado 06/06/2021]; 27(9):1740-1747. Disponible en: https://pubmed.ncbi.nlm.nih.gov/28673366/

15. Park MK. Cardiopatías congénitas cianóticas. En: Cardiología Pediátrica. 3ª. Madrid: Elsevier, 2003, p 100-101.

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Published

2021-09-21

How to Cite

1.
de la Paz-Loret de Mola EJ, Céspedes-Sellén LL, Sellén-Sanchén E, Oliva-de Céspedes CM. Tetralogy of Fallot with pulmonary valve agenesis. Univ. Méd. Pinareña [Internet]. 2021 Sep. 21 [cited 2025 May 12];19:e764. Available from: https://revgaleno.sld.cu/index.php/ump/article/view/764

Issue

Vol. 19 (2023): Vol.19 (Sistema de Publicación Continua)

Section

Case report

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