In fetuses with congenital lung masses, decreased ventricular and atrioventricular valve dimensions are associated with lesion size and clinical outcome

Mardy, Christopher; Blumenfeld, Yair J.; Arunamata, Alisa; Girsén, Anna; Sylvester, Karl G.; Halabi, Safwan; Rubesova, Erika; Hintz, Susan R.; Tacy, Theresa A.; Maskatia, Shiraz A.

doi:10.1002/pd.5612

Cited by 5 publications

(3 citation statements)

References 25 publications

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“…Consistent with this hypothesis ventricular hypoplasia is greatest on the ipsilateral side and appears to disproportionately affects ventricular width rather than length ( 14 , 18 ). Analogous reductions in fetal mitral valve and AV diameter are observed in fetuses with large, compressive left-sided congenital lung masses ( 22 ).…”

Section: The Fetal Heart In Congenital Diaphragmatic Herniamentioning

confidence: 87%

The heart in congenital diaphragmatic hernia: Knowns, unknowns, and future priorities

Patel

Massolo²,

Kraemer³

et al. 2022

Front. Pediatr.

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There is growing recognition that the heart is a key contributor to the pathophysiology of congenital diaphragmatic hernia (CDH), in conjunction with developmental abnormalities of the lung and pulmonary vasculature. Investigations to date have demonstrated altered fetal cardiac morphology, notably relative hypoplasia of the fetal left heart, as well as early postnatal right and left ventricular dysfunction which appears to be independently associated with adverse outcomes. However, many more unknowns remain, not least an understanding of the genetic and cellular basis for cardiac dysplasia and dysfunction in CDH, the relationship between fetal, postnatal and long-term cardiac function, and the impact on other parts of the body especially the developing brain. Consensus on how to measure and classify cardiac function and pulmonary hypertension in CDH is also required, potentially using both non-invasive imaging and biomarkers. This may allow routine assessment of the relative contribution of cardiac dysfunction to individual patient pathophysiological phenotype and enable better, individualized therapeutic strategies incorporating targeted use of fetal therapies, cardiac pharmacotherapies, and extra-corporeal membrane oxygenation (ECMO). Collaborative, multi-model approaches are now required to explore these unknowns and fully appreciate the role of the heart in CDH.

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Section: The Fetal Heart In Congenital Diaphragmatic Herniamentioning

confidence: 87%

The heart in congenital diaphragmatic hernia: Knowns, unknowns, and future priorities

Patel

Massolo²,

Kraemer³

et al. 2022

Front. Pediatr.

View full text Add to dashboard Cite

show abstract

“…Large chest masses may lead to compression of the mediastinum vena cava and heart-obstructing blood return to the right atrium, causing fetal hydrops, heart failure and more 3 . Crombleholme et al (2002) 4 proposed the concept of the CPAM volume ratio (CVR), and different fetal centers have reported various prenatal diagnostic characteristics associated with fetal and postnatal outcomes [5][6][7] . The CVR > 1.6 was thought to increase adverse perinatal outcome risks when combined with fetal hydrops 4 .…”

Section: Introductionmentioning

confidence: 99%

Clinical Perinatal Management of High-Risk Fetal Congenital Pulmonary Airway Malformation

Cai,

Tian,

Liu

et al. 2024

Preprint

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Objectives: To summarize the clinical experience in managing high-risk fetuses with congenital pulmonary airway malformation (CPAM). Design: A retrospective analysis of integrated management clinical data was conducted for high-risk fetuses with CPAM treated at the Third Affiliated Hospital of Guangzhou Medical University and Guangdong Women and Children Health Hospital from 1st July 2015 to 30th June 2021. Data were collected before, during, and after birth. Setting: Third Affiliated Hospital of Guangzhou Medical University and Guangdong Women and Children Health Hospital. Patients: A total of 51 patients were included in the study. Intervention: Prenatal maternal glucocorticoid (GC) treatment was administered in 27 cases. Measurements and Main Results: Prenatal data showed the first diagnosis at 22.90±2.34 weeks' gestation with a prenatal ultrasound congenital pulmonary airway malformation volume ratio (CVR) value of 3.08±0.91. Lesions were diagnosed in the left lung in 27 cases (52.9%), with microcystic changes present in 12 cases (23.5%). Mediastinal shift was observed in 23 cases (45.1%), with 20 cases (39.2%) exhibiting fetal hydrops. Prenatal maternal glucocorticoid (GC) treatment was administered in 27 cases, with a survival rate of 81.4% in the GC group and 95.8% in the non-GC group, showing no statistical difference (P=0.116). Postnatal data indicated an average gestational age of 37.98±2.63 weeks, with 13.7% of patients having a 5-minute Apgar score of less than 8. Neonatal surgery was performed in 17.6% of patients, all of which were lobectomies. The survival rate for high-risk CPAM infants was 88.2%, with 70.6% showing no respiratory symptoms after birth. The presence of fetal hydrops, microcystic type, and mediastinal shift did not significantly increase the risk of death. Over the 6-year follow-up period, 12 cases (26.7%) developed pectus excavatum. Conclusions: Managing high-risk congenital cystic adenomatoid malformation (CCAM) fetuses during the perinatal period remains challenging, but the overall prognosis is generally reasonable. Glucocorticoids may have a role in improving the outcome of high-risk CPAM fetuses, although the statistical significance is still uncertain.

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“…A common presentation in neonates is RDS. However, spontaneous pneumothorax is a rare manifestation of CCAM [ 11 , 12 , 13 ].…”

Section: Introductionmentioning

confidence: 99%

Congenital Cystic Adenomatoid Malformation (CCAM) Type II: A Rare Case of Sudden Infant Death

et al. 2022

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Congenital cystic adenomatoid malformation (CCAM) is a developmental lesion of the lungs and terminal respiratory structures, which is characterized by pseudocysts, lesions, and cystically dilated airways. CCAM is also known as congenital pulmonary airway malformation (CPAM). Various classification systems for CCAM have been described, and based on a recent classification, CCAMs are classified morphologically into five different types (Type 0, I, II, III, and IV) based on lesion sizes. The most common manifestation of CCAM in neonates and children is respiratory distress (RD). Spontaneous pneumothorax is a rare manifestation of CCAM. In this case report, we discuss a CCAM type-II case of a 38-day-old female infant with a radiological post-mortem diagnosis of a large left-side spontaneous pneumothorax. The gross examination of the lungs revealed multiple emphysematous air bubbles up to 0.5 cm in diameter, and the histological examination revealed focal pleural fibrosis, accompanied by thickened septa and atelectasis. In this scenario, the routine use of prenatal ultrasonography would be important to obtain a timely prenatal diagnosis. At the same time, improvements in surgical techniques, as well as greatly improved imaging techniques, have improved the outcome of these patients. Finally, it is important to remark on the importance of autopsy in the case of sudden infant death with a suspected CCAM.

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In fetuses with congenital lung masses, decreased ventricular and atrioventricular valve dimensions are associated with lesion size and clinical outcome

Cited by 5 publications

References 25 publications

The heart in congenital diaphragmatic hernia: Knowns, unknowns, and future priorities

The heart in congenital diaphragmatic hernia: Knowns, unknowns, and future priorities

Clinical Perinatal Management of High-Risk Fetal Congenital Pulmonary Airway Malformation

Congenital Cystic Adenomatoid Malformation (CCAM) Type II: A Rare Case of Sudden Infant Death

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