{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,9]],"date-time":"2026-10-09T16:51:54Z","timestamp":1791564714059,"version":"4.3.4"},"reference-count":24,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2026,2,12]],"date-time":"2026-02-12T00:00:00Z","timestamp":1770854400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2026,2,12]],"date-time":"2026-02-12T00:00:00Z","timestamp":1770854400000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["obgyn.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Ultrasound in Obstet &amp;amp; Gyne"],"published-print":{"date-parts":[[2026,3]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Objective<\/jats:title>\n                    <jats:p>To document the natural progression of fetal cardiac rhabdomyomas and evaluate the impact of prenatal sirolimus (PNS) on tumor size, cardiac complications and brain\u2010tuber size.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>This was a single\u2010center retrospective cohort study of pregnancies with suspected fetal cardiac rhabdomyoma referred to our center\u00a0from April 2013 to May 2024. Serial ultrasound and echocardiography reports were reviewed to obtain tumor characteristics, such as diameter and location, and cardiac complications, including inflow or outflow obstruction, arrhythmia and hydrops. The tumor\u2010to\u2010femur length (TFL) ratio was calculated to correct for fetal growth. Prenatal neurosonography reports were collected from clinical records. Pre\u2010 and postnatal brain magnetic resonance imaging (MRI) scans were reviewed by two observers who were blinded to PNS treatment. Brain\u2010lesion severity was assessed based on the presence of subependymal nodules, the EPISTOP score, the maximum diameter of the largest subcortical tuber, the maximum diameter of the largest subependymal giant cell astrocytoma (SEGA) and the diameter of the largest lateral ventricle. Prenatal or postnatal genetic testing results were documented when available.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      Twenty\u2010seven pregnancies were included in the study, seven of which received PNS. Prior to initiation of treatment, the diameter of the largest cardiac tumor\u00a0at diagnosis was similar between the non\u2010PNS and PNS groups (mean, 15.2\u2009mm\n                      <jats:italic>vs<\/jats:italic>\n                      14.3\u2009mm;\n                      <jats:italic>P<\/jats:italic>\n                      \u2009=\u20090.72), with the left ventricle the most frequently affected location. Without treatment, rhabdomyomas grew rapidly from 20\u2009+\u20090 to 27\u2009+\u20096\u2009weeks' gestation (mean, 2.58\u2009mm\/week) but growth slowed after 28\u2009weeks (mean, 0.44\u2009mm\/week). Hydrops was reported in four cases and occurred at a mean tumor diameter of 31.7\u2009\u00b1\u20096.2\u2009mm, mean TFL ratio of 0.68\u2009\u00b1\u20090.15 and at a mean growth rate of 4.3\u2009\u00b1\u20091.7\u2009mm\/week. In the seven women treated with PNS, treatment for &gt;\u20097\u2009days (\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u20093) resulted in tumor regression and\/or resolution of outflow obstruction, and a reduction in\u00a0TFL ratio; however, prenatal cessation of treatment resulted in rebound growth (\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u20092). Treatment for \u2264\u20097\u2009days (\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u20094) did not impact tumor size or resolve existing cardiac complications. Among the 12 cases that underwent prenatal MRI, the median EPISTOP score was 7 (interquartile range (IQR), 1\u201315) and the median largest lateral ventricle diameter was 8.9 (IQR, 7.0\u20139.7)\u2009mm; subcortical tubers and subependymal nodules were each identified in 67% of cases, and SEGAs were identified in 58%. Among the 13 cases that underwent\u00a0postnatal MRI, the median EPISTOP score was 14 (IQR, 3\u201316) and the median largest lateral ventricle diameter was 7 (IQR, 7\u20138)\u2009mm; brain tubers were identified in 92% of cases. In cases with both pre\u2010 and postnatal MRI findings who received PNS for &gt;\u20097\u2009days (\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u20093), on postnatal MRI compared with prenatal MRI, one patient showed no change in findings, one demonstrated a mild increase in the largest subcortical tuber diameter and one had no detectable brain tubers.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>\n                      Early and sustained PNS treatment was associated with reduced cardiac rhabdomyoma size and\/or resolution of cardiac complications. Rebound tumor growth was observed after discontinuation of treatment. Brain tubers appeared unchanged with PNS treatment, although the sample size was too small to draw a definitive conclusion. \u00a9 2026 The Author(s).\n                      <jats:italic>Ultrasound in Obstetrics &amp; Gynecology<\/jats:italic>\n                      published by John Wiley &amp; Sons Ltd on behalf of International Society of Ultrasound in Obstetrics and Gynecology.\n                    <\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/uog.70185","type":"journal-article","created":{"date-parts":[[2026,2,12]],"date-time":"2026-02-12T09:11:18Z","timestamp":1770887478000},"page":"350-359","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Impact of prenatal sirolimus on cardiac rhabdomyomas and brain tubers"],"prefix":"10.1002","volume":"67","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-8372-6434","authenticated-orcid":false,"given":"S.","family":"Vergote","sequence":"first","affiliation":[{"name":"Fetal Medicine Unit, Department of Obstetrics &amp; Gynaecology Mount Sinai Hospital and University of Toronto  Toronto ON Canada"},{"name":"Department of Development and Regeneration KU Leuven  Leuven Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6551-661X","authenticated-orcid":false,"given":"L.","family":"Van der Veeken","sequence":"additional","affiliation":[{"name":"Department of Development and Regeneration KU Leuven  Leuven Belgium"},{"name":"Department of Obstetrics and Gynaecology University Hospitals Antwerp  Antwerp Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"D.","family":"Chitayat","sequence":"additional","affiliation":[{"name":"Fetal Medicine Unit, Department of Obstetrics &amp; Gynaecology Mount Sinai Hospital and University of Toronto  Toronto ON Canada"},{"name":"The Prenatal Diagnosis and Medical Genetics Program, Department of Obstetrics and Gynecology, Mount Sinai Hospital University of Toronto  Toronto ON Canada"},{"name":"Division of Clinical and Metabolic Genetics, Department of Pediatrics, The Hospital for Sick Children University of Toronto  Toronto ON Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"E.","family":"Jaeggi","sequence":"additional","affiliation":[{"name":"Fetal Medicine Unit, Department of Obstetrics &amp; Gynaecology Mount Sinai Hospital and University of Toronto  Toronto ON Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"G.","family":"Ryan","sequence":"additional","affiliation":[{"name":"Fetal Medicine Unit, Department of Obstetrics &amp; Gynaecology Mount Sinai Hospital and University of Toronto  Toronto ON Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"E.","family":"Miller","sequence":"additional","affiliation":[{"name":"Fetal Medicine Unit, Department of Obstetrics &amp; Gynaecology Mount Sinai Hospital and University of Toronto  Toronto ON Canada"},{"name":"Department of Diagnostic Imaging and Interventional Radiology, The Hospital for Sick Children University of Toronto  Toronto ON Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6352-4846","authenticated-orcid":false,"given":"S.","family":"Shinar","sequence":"additional","affiliation":[{"name":"Fetal Medicine Unit, Department of Obstetrics &amp; 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