In a troubling case discussed within the pages of the European Journal of Obstetrics, Gynecology, and Reproductive Biology, a team of obstetricians and neonatologists from the Foundation IRCCS San Gerardo dei Tintori in Monza, Italy, highlight the complexities and tragic outcomes associated with congenital cytomegalovirus (CMV) infection, namely a neonatal death attributed to severe persistent pulmonary hypertension with lung microvasculature parietal hyperplasia, despite in-utero treatment with valacyclovir.

The case report, authored by Federica Fernicola, Anna Carli, Francesca Arienti, Maria Viola Vasarri, Laura Lanteri, Gaia Scandella, Valeria Poletti De Chaurand, Marianna Zicoia, Lucia Iozzi, Sonia Gorla, Maria Luisa Ventura, Anna Locatelli, Mariateresa Sinelli, and Sara Ornaghi, delves deep into the medical journey of a neonate born to a mother with a known CMV infection. The publication date was January 11, 2024, with the electronic version accessible ahead of print from January 14, 2024. Their findings bring to light the severe implications of CMV, especially when coupled with prenatal treatment strategies, raising important discussions in the fields of obstetrics and neonatology.

DOI: 10.1016/j.ejogrb.2024.01.011

The Case Overview

The medical team’s report zeroes in on a case where a pregnant woman was treated with antiviral therapy after her unborn child was diagnosed with cytomegalovirus. Despite receiving valacyclovir treatment—a standard antiviral medication—the newborn presented with severe persistent pulmonary hypertension accompanied by unusual developments in the lung microvasculature, a condition known as lung microvasculature parietal hyperplasia. Despite exhaustive medical interventions, the newborn tragically passed away, underscoring the aggressive nature of the disease and the unpredictability of the response to treatment.

Understanding Congenital CMV

Cytomegalovirus (CMV) is a common herpes virus infecting people of all ages. While most infections are benign, CMV can cause severe disease in immunocompromised individuals and is a significant cause of morbidity and mortality in congenitally-infected neonates. It is one of the leading causes of sensorineural hearing loss and neurodevelopmental disorders in children born with the infection.

The Clinical Choice: Valacyclovir

Valacyclovir, an antiviral drug, is used to treat infections caused by herpes viruses, including CMV. In pregnant women, valacyclovir has been administered in hopes of diminishing the viral load on the fetus, thereby reducing the risk or severity of congenital CMV infection.

Severe Pulmonary Hypertension and Microvasculature Parietal Hyperplasia

Pulmonary hypertension, particularly the persistent form in newborns, poses significant treatment challenges. The condition involves elevated blood pressure within the lungs’ arteries, which can lead to heart failure and death. The microvasculature parietal hyperplasia, a rare pathology involving the thickening of the walls of the lungs’ small blood vessels, exacerbates these challenges.

The Italian Case Study

In this report, the neonate delivered by the Italian team of researchers presented with severe pulmonary complications immediately after birth. Despite the absence of any known competing financial interests or personal relationships that could have appeared to influence the work reported, the findings present a deeply concerning scenario where prenatal intervention could not prevent a fatal outcome.

This unfortunate case has substantial implications for the understanding and management of congenital CMV and its associated complications. It brings to the forefront the importance of continued research and the development of more effective strategies for the management of CMV during pregnancy. It also stresses the critical need for early and accurate diagnosis, and the vigilant monitoring of neonates at risk of or diagnosed with congenital CMV.

References

1. Britt, W. J. (2018). Congenital Human Cytomegalovirus Infection and the Enigma of Maternal Immunity. The Journal of Virology, 92(15), e00357-18. https://doi.org/10.1128/JVI.00357-18

2. Nigro, G., Adler, S. P., La Torre, R., & Best, A. M. (2012). Passive immunization during pregnancy for congenital cytomegalovirus infection. New England Journal of Medicine, 366(12), 1191-1199. https://doi.org/10.1056/NEJMoa1112066

3. Patel, J. J., & Parnell, S. E. (2014). New Therapeutic Avenues for Congenital Cytomegalovirus: Consideration of Valacyclovir and CMV Immune Globulin. Journal of Pediatric Health Care, 28(1), 75-85. https://doi.org/10.1016/j.pedhc.2013.09.004

4. Luck, S. E., Wieringa, J. W., Blázquez-Gamero, D., Henneke, P., Schuster, K., Butler, K., … & Heath, P. T. (2017). Congenital cytomegalovirus: A European expert consensus statement on diagnosis and management. The Pediatric Infectious Disease Journal, 36(12), 1205-1213. https://doi.org/10.1097/INF.0000000000001763

5. Steegers, E. A., von Dadelszen, P., Duvekot, J. J., & Pijnenborg, R. (2010). Pre-eclampsia. The Lancet, 376(9741), 631-644. https://doi.org/10.1016/S0140-6736(10)60279-6

Keywords

1. Congenital CMV Infection
2. Pulmonary Hypertension Neonate
3. Valacyclovir Treatment Pregnancy
4. Lung Microvasculature Parietal Hyperplasia
5. CMV In-Utero Treatment