Connective tissue disorders are known to cause cardiac and vascular complications. We present the case of a 37-year-old female patient with a rare Ehlers Danlos/Osteogenesis Imperfecta Overlap Syndrome, referred to cardiac surgery with aortic valve regurgitation, who underwent a successful Biological Surgical Aortic Valve Replacement (SAVR). A multidisciplinary, patient-centered, heart-team approach is essential in managing patients with rare genetic disorders to optimize postoperative outcomes. Adult cardiac surgeons must become familiar with genetic syndromes and their implications for improving perioperative outcomes.
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http://dx.doi.org/10.3389/fcvm.2025.1480363 | DOI Listing |
ESC Heart Fail
March 2025
Department of Cardiology, Oslo University Hospital, Rikshospitalet, Oslo, Norway.
Aims: The prevalences of aortic stenosis (AS) and transthyretin amyloid cardiomyopathy (ATTR-CM) increase with age. Identification of occult ATTR-CM in patients with AS can help explain out-of-proportion myocardial dysfunction, aid in prognostication and prompt initiation of disease-modifying treatment. Studies have suggested that many patients referred for transcatheter aortic valve implantation (TAVI) have concomitant ATTR-CM, but some have included unverified ATTR-CM in patients with ambiguous scintigrams.
View Article and Find Full Text PDFFront Cardiovasc Med
February 2025
Department of Cardiovascular Surgery, Fundacion Cardioinfantil-Instituto de Cardiologia, Bogotá, Colombia.
Connective tissue disorders are known to cause cardiac and vascular complications. We present the case of a 37-year-old female patient with a rare Ehlers Danlos/Osteogenesis Imperfecta Overlap Syndrome, referred to cardiac surgery with aortic valve regurgitation, who underwent a successful Biological Surgical Aortic Valve Replacement (SAVR). A multidisciplinary, patient-centered, heart-team approach is essential in managing patients with rare genetic disorders to optimize postoperative outcomes.
View Article and Find Full Text PDFCureus
March 2025
Department of Cardiovascular Surgery, Japanese Red Cross Society Kyoto Daiichi Hospital, Kyoto, JPN.
Pheochromocytomas present with paroxysmal hypertension due to a sudden release of catecholamines stimulated by radiological contrast media, surgery, or anesthetic agents. This often complicates the maintenance of patient hemodynamics during surgery. A 55-year-old man with a high fever was admitted to a hospital.
View Article and Find Full Text PDFCureus
February 2025
Cardiology, Unidade Local de Saúde (ULS) São João, Porto, PRT.
A 53-year-old female with a medical history of multiple cardiovascular risk factors, alcoholic chronic hepatic disease (Child-Pugh B) with thrombocytopenia, and severe calcified aortic stenosis was admitted for an elective transcatheter aortic valve implantation (TAVI). After the procedure, the patient was hypotensive and unresponsive to fluid challenge, and there was a significant difference in blood pressure between the two arms. The echocardiogram confirmed the normal position and function of the prosthetic valve but was suggestive of aortic dissection.
View Article and Find Full Text PDFInterdiscip Cardiovasc Thorac Surg
March 2025
Cardiac Surgery Department. Hospital de la Santa Creu i Sant Pau (HSCSP), IIB Sant Pau. Universitat Autónoma de Barcelona. Barcelona, Spain.
Objectives: Interest in Del Nido solution is increasing in adult cardiac surgery. This study compared Del Nido with Buckberg cardioplegia in patients undergoing isolated aortic valve replacement.
Methods: A prospective, two-center, randomized trial was conducted from July 2019 to August 2023, with adult patients undergoing first-time isolated aortic valve replacement, and were randomized to receive Buckberg (n = 159) or Del Nido (n = 152) solution.
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