{"id":2076,"date":"2025-06-26T14:27:55","date_gmt":"2025-06-26T14:27:55","guid":{"rendered":"https:\/\/blogs.bmj.com\/jmg\/?p=2076"},"modified":"2026-02-22T01:06:33","modified_gmt":"2026-02-22T01:06:33","slug":"shprintzen-goldberg-syndrome-follow-up-of-the-cardiovascular-features-in-an-international-cohort-of-29-patients-with-sgs","status":"publish","type":"post","link":"https:\/\/blogs.bmj.com\/jmg\/2025\/06\/26\/shprintzen-goldberg-syndrome-follow-up-of-the-cardiovascular-features-in-an-international-cohort-of-29-patients-with-sgs\/","title":{"rendered":"Shprintzen-Goldberg syndrome: follow-up of the cardiovascular features in an international cohort of 29 patients with SGS (Contributed by Dr Yordi-Micha\u00ebl BOUHATOUS and Pr Laurence FAIVRE)"},"content":{"rendered":"<p>This study sheds new light on the long-term heart issues linked to Shprintzen-Goldberg syndrome (SGS), a rare connective tissue disorder in the same family as Marfan syndrome. Unlike previous literature with limited data, this analysis of 29 international patients reveals that nearly half develop mitral regurgitation, and over a third experience thoracic aortic aneurysms. Importantly, no cases of aortic dissection were observed, and preventive treatments like beta blockers were rarely used. The study emphasizes the need for systematic cardiovascular monitoring in SGS, similar to Marfan syndrome, especially given the genetic link between certain variants and aortic risks\u2014an underexplored area in prior research. (<a href=\"https:\/\/jmg.bmj.com\/content\/early\/2025\/06\/25\/jmg-2024-110341\">https:\/\/jmg.bmj.com\/content\/early\/2025\/06\/25\/jmg-2024-110341<\/a> )<!--TrendMD v2.4.8--><\/p>\n","protected":false},"excerpt":{"rendered":"<p>This study sheds new light on the long-term heart issues linked to Shprintzen-Goldberg syndrome (SGS), a rare connective tissue disorder in the same family as Marfan syndrome. Unlike previous literature with limited data, this analysis of 29 international patients reveals that nearly half develop mitral regurgitation, and over a third experience thoracic aortic aneurysms. Importantly, [&#8230;]<\/p>\n<p><a class=\"btn btn-secondary understrap-read-more-link\" href=\"https:\/\/blogs.bmj.com\/jmg\/2025\/06\/26\/shprintzen-goldberg-syndrome-follow-up-of-the-cardiovascular-features-in-an-international-cohort-of-29-patients-with-sgs\/\">Read More&#8230;<\/a><\/p>\n","protected":false},"author":123,"featured_media":0,"comment_status":"open","ping_status":"closed","sticky":false,"template":"","format":"standard","meta":{"footnotes":""},"categories":[1],"tags":[],"class_list":["post-2076","post","type-post","status-publish","format-standard","hentry","category-uncategorized"],"yoast_head":"<!-- This site is optimized with the Yoast SEO plugin v27.6 - https:\/\/yoast.com\/product\/yoast-seo-wordpress\/ -->\n<title>Shprintzen-Goldberg syndrome: follow-up of the cardiovascular features in an international cohort of 29 patients with SGS (Contributed by Dr Yordi-Micha\u00ebl BOUHATOUS and Pr Laurence FAIVRE) - JMG Contact blog<\/title>\n<meta name=\"robots\" content=\"index, follow, max-snippet:-1, max-image-preview:large, max-video-preview:-1\" \/>\n<link rel=\"canonical\" href=\"https:\/\/blogs.bmj.com\/jmg\/2025\/06\/26\/shprintzen-goldberg-syndrome-follow-up-of-the-cardiovascular-features-in-an-international-cohort-of-29-patients-with-sgs\/\" \/>\n<meta property=\"og:locale\" content=\"en_US\" \/>\n<meta property=\"og:type\" content=\"article\" \/>\n<meta property=\"og:title\" content=\"Shprintzen-Goldberg syndrome: follow-up of the cardiovascular features in an international cohort of 29 patients with SGS (Contributed by Dr Yordi-Micha\u00ebl BOUHATOUS and Pr Laurence FAIVRE) - JMG Contact blog\" \/>\n<meta property=\"og:description\" content=\"This study sheds new light on the long-term heart issues linked to Shprintzen-Goldberg syndrome (SGS), a rare connective tissue disorder in the same family as Marfan syndrome. Unlike previous literature with limited data, this analysis of 29 international patients reveals that nearly half develop mitral regurgitation, and over a third experience thoracic aortic aneurysms. Importantly, [...]Read More...\" \/>\n<meta property=\"og:url\" content=\"https:\/\/blogs.bmj.com\/jmg\/2025\/06\/26\/shprintzen-goldberg-syndrome-follow-up-of-the-cardiovascular-features-in-an-international-cohort-of-29-patients-with-sgs\/\" \/>\n<meta property=\"og:site_name\" content=\"JMG Contact blog\" \/>\n<meta property=\"article:published_time\" content=\"2025-06-26T14:27:55+00:00\" \/>\n<meta property=\"article:modified_time\" content=\"2026-02-22T01:06:33+00:00\" \/>\n<meta name=\"author\" content=\"hqqu\" \/>\n<meta name=\"twitter:card\" content=\"summary_large_image\" \/>\n<meta name=\"twitter:creator\" content=\"@HuiQiQu\" \/>\n<meta name=\"twitter:label1\" content=\"Written by\" \/>\n\t<meta name=\"twitter:data1\" content=\"hqqu\" \/>\n\t<meta name=\"twitter:label2\" content=\"Est. reading time\" \/>\n\t<meta name=\"twitter:data2\" content=\"1 minute\" \/>\n<script type=\"application\/ld+json\" class=\"yoast-schema-graph\">{\"@context\":\"https:\\\/\\\/schema.org\",\"@graph\":[{\"@type\":\"Article\",\"@id\":\"https:\\\/\\\/blogs.bmj.com\\\/jmg\\\/2025\\\/06\\\/26\\\/shprintzen-goldberg-syndrome-follow-up-of-the-cardiovascular-features-in-an-international-cohort-of-29-patients-with-sgs\\\/#article\",\"isPartOf\":{\"@id\":\"https:\\\/\\\/blogs.bmj.com\\\/jmg\\\/2025\\\/06\\\/26\\\/shprintzen-goldberg-syndrome-follow-up-of-the-cardiovascular-features-in-an-international-cohort-of-29-patients-with-sgs\\\/\"},\"author\":{\"name\":\"hqqu\",\"@id\":\"https:\\\/\\\/blogs.bmj.com\\\/jmg\\\/#\\\/schema\\\/person\\\/be0250f8d5b52412c3e7c222dabd591b\"},\"headline\":\"Shprintzen-Goldberg syndrome: follow-up of the cardiovascular features in an international cohort of 29 patients with SGS (Contributed by Dr Yordi-Micha\u00ebl BOUHATOUS and Pr Laurence FAIVRE)\",\"datePublished\":\"2025-06-26T14:27:55+00:00\",\"dateModified\":\"2026-02-22T01:06:33+00:00\",\"mainEntityOfPage\":{\"@id\":\"https:\\\/\\\/blogs.bmj.com\\\/jmg\\\/2025\\\/06\\\/26\\\/shprintzen-goldberg-syndrome-follow-up-of-the-cardiovascular-features-in-an-international-cohort-of-29-patients-with-sgs\\\/\"},\"wordCount\":135,\"commentCount\":0,\"publisher\":{\"@id\":\"https:\\\/\\\/blogs.bmj.com\\\/jmg\\\/#organization\"},\"inLanguage\":\"en-US\",\"potentialAction\":[{\"@type\":\"CommentAction\",\"name\":\"Comment\",\"target\":[\"https:\\\/\\\/blogs.bmj.com\\\/jmg\\\/2025\\\/06\\\/26\\\/shprintzen-goldberg-syndrome-follow-up-of-the-cardiovascular-features-in-an-international-cohort-of-29-patients-with-sgs\\\/#respond\"]}]},{\"@type\":\"WebPage\",\"@id\":\"https:\\\/\\\/blogs.bmj.com\\\/jmg\\\/2025\\\/06\\\/26\\\/shprintzen-goldberg-syndrome-follow-up-of-the-cardiovascular-features-in-an-international-cohort-of-29-patients-with-sgs\\\/\",\"url\":\"https:\\\/\\\/blogs.bmj.com\\\/jmg\\\/2025\\\/06\\\/26\\\/shprintzen-goldberg-syndrome-follow-up-of-the-cardiovascular-features-in-an-international-cohort-of-29-patients-with-sgs\\\/\",\"name\":\"Shprintzen-Goldberg syndrome: follow-up of the cardiovascular features in an international cohort of 29 patients with SGS (Contributed by Dr Yordi-Micha\u00ebl BOUHATOUS and Pr Laurence FAIVRE) - 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