Prenatal diagnosis of hypoplastic left heart syndrome with PAPVC and FO restriction and pulmonary hypertension – coexisting factors suggesting poor prognosis due to pulmonary hypertension with histopathological confirmation

Aleksandra Nowakowska; Michał Krekora; Tomasz Talar; Marek Kopala; Hanna Romanowicz; Maria Respondek-Liberska

doi:10.5114/pcard.2023.126182

eISSN: 2449-6731
ISSN: 2449-6723

Prenatal Cardiology

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abstract:

Case report

Prenatal diagnosis of hypoplastic left heart syndrome with PAPVC and FO restriction and pulmonary hypertension – coexisting factors suggesting poor prognosis due to pulmonary hypertension with histopathological confirmation

Aleksandra Nowakowska

¹

,

Michał Krekora

²

,

Tomasz Talar

³

,

Marek Kopala

⁴

,

Hanna Romanowicz

⁵

,

Maria Respondek-Liberska

⁶

Department for Prenatal Cardiology, Polish Mother’s Memorial Hospital Research Institute, Lodz, Poland
Department of Obstetrics and Gynaecology, Polish Mother’s Memorial Hospital Research Institute, Lodz, Poland
Department of Neonatology, Polish Mother’s Memorial Hospital Research Institute, Lodz, Poland
Department of Cardiac Surgery, Polish Mother’s Memorial Hospital Research Institute, Lodz, Poland
Department of Pathology, Polish Mother’s Memorial Hospital Research Institute, Lodz, Poland
Department for the Diagnosis and Prevention of Fetal Malformations, Medical University of Lodz, Poland

Prenat Cardio 2022; 12(1): 25-30

DOI: https://doi.org/10.5114/pcard.2023.126182

Online publish date: 2023/03/27

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AMA

Nowakowska A, Krekora M, Talar T, Kopala M, Romanowicz H, Respondek-Liberska M. Prenatal diagnosis of hypoplastic left heart syndrome with PAPVC and FO restriction and pulmonary hypertension – coexisting factors suggesting poor prognosis due to pulmonary hypertension with histopathological confirmation. Prenatal Cardiology. 2022;(1):25-30. doi:10.5114/pcard.2023.126182.

APA

Nowakowska, A., Krekora, M., Talar, T., Kopala, M., Romanowicz, H.,  & Respondek-Liberska, M. (2022). Prenatal diagnosis of hypoplastic left heart syndrome with PAPVC and FO restriction and pulmonary hypertension – coexisting factors suggesting poor prognosis due to pulmonary hypertension with histopathological confirmation. Prenatal Cardiology, (1), 25-30. https://doi.org/10.5114/pcard.2023.126182

Chicago

Nowakowska, Aleksandra, Michał Krekora, Tomasz Talar, Marek Kopala, Hanna Romanowicz,  and Maria Respondek-Liberska. 2022. "Prenatal diagnosis of hypoplastic left heart syndrome with PAPVC and FO restriction and pulmonary hypertension – coexisting factors suggesting poor prognosis due to pulmonary hypertension with histopathological confirmation". Prenatal Cardiology  (1): 25-30. doi:10.5114/pcard.2023.126182.

Harvard

Nowakowska, A., Krekora, M., Talar, T., Kopala, M., Romanowicz, H.,  and Respondek-Liberska, M. (2022). Prenatal diagnosis of hypoplastic left heart syndrome with PAPVC and FO restriction and pulmonary hypertension – coexisting factors suggesting poor prognosis due to pulmonary hypertension with histopathological confirmation. Prenatal Cardiology, (1), pp.25-30. https://doi.org/10.5114/pcard.2023.126182

MLA

Nowakowska, Aleksandra et al. "Prenatal diagnosis of hypoplastic left heart syndrome with PAPVC and FO restriction and pulmonary hypertension – coexisting factors suggesting poor prognosis due to pulmonary hypertension with histopathological confirmation." Prenatal Cardiology, no. 1, 2022, pp. 25-30. doi:10.5114/pcard.2023.126182.

Vancouver

Nowakowska A, Krekora M, Talar T, Kopala M, Romanowicz H, Respondek-Liberska M. Prenatal diagnosis of hypoplastic left heart syndrome with PAPVC and FO restriction and pulmonary hypertension – coexisting factors suggesting poor prognosis due to pulmonary hypertension with histopathological confirmation. Prenatal Cardiology. 2022;(1):25-30. doi:10.5114/pcard.2023.126182.

PlumX metrics:

Prenatal hypoplastic left heart syndrome (HLHS) is widely described in the literature as a congenital heart defect with underdevelopment of the left side of the heart. There are many well-known risk factors that comprise a poor prognosis, such as coexisting genetic syndrome, or extra-cardiac malformations like diaphragmatic hernia, premature delivery, low birth weight, etc. Herein we present an isolated case of HLHS, with echocardiographic data from the third trimester, after in utero transfer, born at term in a tertiary centre with an obstetrics, cardiology, and cardiac surgery department within the same research institute, with Prostin administration without delay, Norwood procedure planned relatively early, but despite all efforts – neonatal demise. We believe that the main cause of death was pulmonary hypertension, and such an observation was never published before.

keywords:

Prenatal diagnosis of hypoplastic left heart syndrome with PAPVC and FO restriction and pulmonary hypertension – coexisting factors suggesting poor prognosis due to pulmonary hypertension with histopathological confirmation

Aleksandra Nowakowska 1 , Michał Krekora 2 , Tomasz Talar 3 , Marek Kopala 4 , Hanna Romanowicz 5 , Maria Respondek-Liberska 6

pulmonary hypertension, Norwood operation, HLHS, partial anomalous pulmonary venous connections, restriction foramen ovale

Aleksandra Nowakowska

¹

,

Michał Krekora

²

,

Tomasz Talar

³

,

Marek Kopala

⁴

,

Hanna Romanowicz

⁵

,

Maria Respondek-Liberska

⁶