Bennani Ghali, Zahri Soukaina, Khaldi Mohamed, Benouna Ghali, Drighil Abdenasser, Habbal Rachida
Department of Cardiology, University Hospital of Ibn Rochd, Lotissement Lina Villa 46 Sidi Maarouf, Casablanca, Morocco.
Egypt Heart J. 2024 Apr 18;76(1):50. doi: 10.1186/s43044-024-00479-1.
Kallmann-Morsier syndrome is a rare disease characterized by the association of congenital gonadotropic deficiency and anosmia or hyposmia. The cardiac manifestations associated with this syndrome are little known. Through this case, we will characterize the cardiac involvement of this disease in the light of what is already described in the literature.
We report the case of a young patient who presented with a picture of cardiac decompensation revealing restrictive heart disease. In her exploration, she was found to have primary amenorrhea, leading to the diagnosis of Kallmann syndrome. Medical treatment was optimized for the management of her cardiac decompensation as well as hormonal replacement treatment for her delayed puberty and growth.
Cardiac manifestations in Kallmann-Morsier syndrome are few reported in the literature, and restrictive heart disease is uncommon with no cases report till now. This association suggests a possible common genetic origin that should be explored in the future.
卡尔曼 - 莫西尔综合征是一种罕见疾病，其特征为先天性促性腺激素缺乏与嗅觉缺失或嗅觉减退相关联。与该综合征相关的心脏表现鲜为人知。通过本病例，我们将根据文献中已描述的内容来阐述该疾病的心脏受累情况。
我们报告了一名年轻患者的病例，该患者出现心脏失代偿表现，提示限制性心脏病。在检查过程中，发现她有原发性闭经，从而诊断为卡尔曼综合征。针对其心脏失代偿进行了优化的药物治疗，并对其青春期延迟和生长进行了激素替代治疗。
卡尔曼 - 莫西尔综合征的心脏表现文献报道较少，限制性心脏病并不常见，目前尚无病例报告。这种关联提示可能存在共同的遗传起源，未来应进行探索。
