Dataset Information

Prenatal Pericardiocentesis and Postnatal Sirolimus for a Giant Inoperable Cardiac Rhabdomyoma.

ABSTRACT: We describe the case of an antenatally diagnosed massive cardiac tumor in a fetus requiring cardiorespiratory support immediately following birth. We further discuss the successful management of this case and highlight the importance of a multidisciplinary team in managing such complicated cases. (Level of Difficulty: Advanced.).

SUBMITTER: Relan J

PROVIDER: S-EPMC8551506 | biostudies-literature | 2021 Oct

REPOSITORIES: biostudies-literature

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Prenatal Pericardiocentesis and Postnatal Sirolimus for a Giant Inoperable Cardiac Rhabdomyoma.

Relan Jay J Swami Manish M Rana Anubhuti A Chaudhary Priyanka P Ojha Vineeta V Devarapalli Sowmya S Dadhwal Vatsla V Verma Ankit A Jagia Priya P Saxena Anita A

JACC. Case reports 20211006 13

We describe the case of an antenatally diagnosed massive cardiac tumor in a fetus requiring cardiorespiratory support immediately following birth. We further discuss the successful management of this case and highlight the importance of a multidisciplinary team in managing such complicated cases. (<b>Level of Difficulty: Advanced.</b>). ...[more]

PMID: 34746849

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Project description:In utero treatment with glucocorticoids have been suggested to reprogram postnatal cardiovascular function and stress responsiveness. However, little is known about the effects of prenatal exposure to the natural corticosteroid, cortisol, on postnatal cardiovascular system or metabolism. We have demonstrated an increased incidence of stillbirth in sheep pregnancies in which there is mild maternal hypercortisolemia caused by infusion of 1 mg/kg/d cortisol. In order to model corticosteroid effects in the neonate, we created a second model in which cortisol was infused for 12 h per day for a daily infusion of 0.5 mg/kg/d. In this model we had previously found that neonatal plasma glucose was increased and plasma insulin was decreased compared to those in the control group, and that neonatal ponderal index and kidney weight were reduced and left ventricular wall thickness was increased in the 2 week old lamb. In this study, we have used transcriptomic modeling to better understand the programming effect of this maternal hypercortisolemia in these hearts. This is a time when both terminal differentiation and a shift in the metabolism of the heart from carbohydrates to lipid oxidation are thought to be complete. The transcriptomic model indicates suppression of genes in pathways for fatty acid and ketone production and upregulation of genes in pathways for angiogenesis in the epicardial adipose fat (EAT). The transcriptomic model indicates that RNA related pathways are overrepresented by downregulated genes, but ubiquitin-mediated proteolysis and protein targeting to the mitochondria are overrepresented by upregulated genes in the intraventricular septum (IVS) and left ventricle (LV). In IVS the AMPK pathway and adipocytokine signaling pathways were also modeled based on overrepresentation by downregulated genes. Peroxisomal activity is modeled as increased in EAT, but decreased in LV and IVS. Our results suggest that pathways for lipids as well as cell proliferation and cardiac remodeling have altered activity postnatally after the in utero cortisol exposure. Together, this model is consistent with the observed increase in cardiac wall thickness at necropsy and altered glucose metabolism observed in vivo, and predicts that in utero exposure to excess maternal cortisol will cause postnatal cardiac hypertrophy and altered responses to oxidative stress.

Project description:ImportancePrenatal cardiac screening of the first and second trimesters has had a major impact on postnatal prevalence of congenital heart defects (CHDs), rates of termination of pregnancy (TOP), and outcomes among children born alive with CHDs.ObjectiveTo examine the prenatal and postnatal incidence of major CHDs (ie, necessitating intervention within the first year of life), detection rate trends, rates of TOP, and the association of cardiac screening with postnatal outcomes.Design, settings, and participantsIn this cross-sectional study, 3827 fetuses with antenatally diagnosed major CHDs in the Czech Republic (population 10.7 million) between 1991 and 2021 were prospectively evaluated with known outcomes and associated comorbidities. Prenatal and postnatal prevalence of CHD in an unselected population was assessed by comparison with a retrospective analysis of all children born alive with major CHDs in the same period (5454 children), using national data registry. Data analysis was conducted from January 1991 to December 2021.Main outcomes and measuresPrenatal detection and postnatal prevalence of major CHDs and rate of TOPs in a setting with a centralized health care system over 31 years.ResultsA total of 3 300 068 children were born alive during the study period. Major CHD was diagnosed in 3827 fetuses, of whom 1646 (43.0%) were born, 2069 (54.1%) resulted in TOP, and 112 (2.9%) died prenatally. The prenatal detection rate increased from 6.2% in 1991 to 82.8% in 2021 (P < .001). Termination of pregnancy decreased from 70% in 1991 to 43% (P < .001) in 2021. Of 627 fetuses diagnosed in the first trimester (introduced in 2007), 460 were terminated (73.3%). Since 2007, of 2066 fetuses diagnosed in the second trimester, 880 (42.6%) were terminated, resulting in an odds ratio of 3.6 (95% CI, 2.8-4.6; P < .001) for TOP in the first trimester compared with the second trimester. Postnatal prevalence of major CHDs declined from 0.21% to 0.14% (P < .001). The total incidence (combining prenatal detection of terminated fetuses with postnatal prevalence) of major CHD remained at 0.23% during the study period.Conclusions and relevanceIn this cross-sectional study, the total incidence of major CHD did not change significantly during the 31-year study period. The prenatal detection of major CHD approached 83% in the current era. Postnatal prevalence of major CHD decreased significantly due to early TOPs and intrauterine deaths. The introduction of first trimester screening resulted in a higher termination rate in the first trimester but did not revert the overall decreasing trend of termination for CHDs in general.

Project description:BackgroundPericardiocentesis is an effective treatment for cardiac tamponade, but there are risks, including haemorrhagic events, cardiac perforation, pneumothorax, arrhythmia, acute pulmonary oedema and so on. Mediastinal effusion caused by puncture is rarely reported.Case presentationA 47-year-old man who had a history of right leg deep vein thrombosis and pulmonary artery embolism with implantation of an inferior vena cava filter presented for inferior vena cava filter removal. Within 30 min after the procedure, he developed chest pain, nausea, vomiting and presyncope with shock. Echocardiography confirmed massive pericardial effusion with evidence of cardiac tamponade. Emergency pericardiocentesis was performed. Confusingly, only 3 mL of bloody pericardial effusion was drained in total, and subsequently, the patient's symptoms rapidly improved with stable haemodynamics. Repeat echocardiography showed that the pericardial effusion had disappeared. Urgent computed tomography pulmonary angiography demonstrated localized effusion, which was not seen the previous computed tomography results and was noted around the left ventricle in the mediastinal apace. No intervention was performed, given that there was no bleeding tendency or further adverse events related to the mediastinal effusion. The patient was subsequently discharged in a stable condition a few days later, and outpatient follow-up was advised.ConclusionsMediastinal effusion is a rare complication of pericardiocentesis. In the case described herein, the most likely cause was pericardial effusion extravasated into the mediastinum through the needle insertion site in the puncture process due to large pressure variations in the intrapericardial space with tamponade, differing from cases of over-anticoagulation reported in the previous literature. Just as our case demonstrates that conservative treatment of an hemodynamic insignificant mediastinal effusion may be appropriate. Echocardiography is useful and effective to minimize complication rates.

Dataset Information

Prenatal Pericardiocentesis and Postnatal Sirolimus for a Giant Inoperable Cardiac Rhabdomyoma.

Publications

Prenatal Pericardiocentesis and Postnatal Sirolimus for a Giant Inoperable Cardiac Rhabdomyoma.

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