Case Report:Laubry-Pezzi Syndrome:Confronting the Lethal Nexus of Life-Threatening Complications in Resource Constrained Settings  

作　　者：Hayatu Uma Abdulaziz Aminu Raghu Cherukupalli Femi Akindotun Akintomide Abdul Habu Aisha Aminu Lawal Adamu Mohammad 

机构地区：[1]Department of Internal Medicine,Usmanu Danfodiyo University Teaching Hospital,Sokoto,23270,Nigeria [2]Cardiovascular Division,Aster Prime Hospitals,Hyderabad,500038,India [3]Department of Internal Medicine,Federal Medical Center Nguru,Nguru,630261,Yobe,Nigeria

出　　处：《Congenital Heart Disease》2024年第6期635-645,共11页先天性心脏病(英文)

摘　　要：Laubry-Pezzi syndrome(L-PS)is a rare congenital heart disease characterized by a ventricular septal defect(VSD)and aortic valve prolapse.These cardiac lesions predispose individuals to infective endocarditis(IE),a lifethreatening complication,especially in resource-constrained settings.A 17-year-old male presented with a three-week history of fever and headache,and a one-week history of abdominal pain,vomiting,and diarrhea.On presentation,he appeared toxic,was febrile,tachypneic,tachycardic,and blood pressure of 120/30 mmHg,and heart sounds were S1,S2.Abdominal examination revealed generalized tenderness.A provisional diagnosis of typhoid sepsis with intestinal perforation was considered.However,a thorough clinical evaluation led to the definitive diagnosis of L-PS complicated by right-sided IE,sepsis,acute kidney injury,acute heart failure,and haemoptysis.Despite significant improvement with appropriate antibiotics and adjunctive therapy,the patient’s inability to afford surgical correction and subsequent non-adherence to medical advice he resorted to traditional medicine this led to readmission for heart failure.This case highlights the diagnostic and treatment challenges associated with L-PS with life-threatening complications,particularly in resource-constrained settings,where it may be misdiagnosed as common infections like typhoid sepsis with intestinal perforation.It emphasizes the importance of a comprehensive clinical approach,including a detailed history,meticulous physical examination,and targeted investigations,to ensure accurate diagnosis and timely intervention in patients with L-PS and its life-threatening complications.

关 键 词：Laubry-Pezzi syndrome ventricular septal defect aortic valve prolapse infective endocarditis SEPSIS 

分 类 号：R54[医药卫生—心血管疾病]