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

: 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),… Click to show full abstract

: 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 life-threatening 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.

Keywords: resource constrained; history; threatening complications; life threatening; constrained settings

Journal Title: Congenital Heart Disease
Year Published: 2024

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