Intimal sarcoma (IS) is an extremely rare but aggressive malignant tumor and is now recognized as the most frequent primary malignancy of the heart and great vessels. Its clinical presentation is often non-specific, frequently mimicking common conditions such as pulmonary embolism or cardiac myxoma, leading to significant diagnostic delays. This case series describes four women with IS in various locations: two in the left atrium with mitral valve involvement, one in the main pulmonary artery, and one presenting as a pulmonary mass. Diagnosis relied on multimodal imaging, including echocardiography, computed tomography, and magnetic resonance imaging, followed by histopathological confirmation. Microscopic analysis revealed marked cellular pleomorphism and high mitotic activity. Immunohistochemistry (IHC) was essential for the final diagnosis, as all cases demonstrated nuclear mouse double minute 2 (MDM2) expression, a defining marker associated with gene amplification. Other markers, such as h-caldesmon and diffuse cluster of differentiation 31, were negative, effectively excluding leiomyosarcoma and angiosarcoma. Given its poor prognosis and high embolic potential, early recognition and radical surgical resection remain crucial for patient survival. This series emphasizes the clinical relevance of considering IS in the differential diagnosis of intracardiac and endovascular masses, highlighting MDM2 IHC as a reliable diagnostic tool.
Background:Patients with single-ventricle physiology present one of the most complex congenital heart diseases, with implications that extend beyond strictly cardiologic aspects. Survival has improved due to new technologies, but comprehensive, effective, and sustainable follow-up requires innovative care models. Objective:To describe the implementation and outcomes of an interdisciplinary clinic in a high-complexity public hospital in Argentina. Method:This is a retrospective, longitudinal, descriptive study from the initiation of the interdisciplinary clinic in April 2023 through August 2025. A monthly multidisciplinary care setting was established. The strategy included an organized pre-clinic evaluation, sequential clinical consultations, psychoeducational workshops for families, and therapeutic play sessions. A formalized roadmap and interdisciplinary communication structure were implemented. Results:From April 2023 to August 2025, 81 patients were incorporated into this care model. The median age at the time of first surgery was 16 days. Attendance rates for scheduled visits increased from 70 to 92%. Significant improvements were achieved in care coordination, early detection of cardiac and extracardiac complications, access to complementary studies, and adherence to follow-up. More than 60% of families participated in workshops, creating spaces for support and shared experiences. Conclusion:The interdisciplinary single-ventricle clinic at Hospital de Pediatría SAMIC Juan P. Garrahan represents an innovative and unique model in Argentina, with a positive impact on adherence, early identification of complications, and family engagement.
Carcinoid syndrome is characterized by the presence of a carcinoid tumor that can release chemical mediators that, if not adequately controlled, can even lead to carcinoid crisis. In this condition occurs hemodynamic instability, with distributive shock, bronchospasm, and cardiac arrhythmias. Carcinoid tumors have an incidence of 1-2 cases per 100,000 patients, and 20-30% of them present episodic vasomotor symptoms such as bronchospasm, hypotension and diarrhea secondary to therelease of vasoactive amines. Because it is an infrequent situation, we present the anesthetic management of a patient withcarcinoid syndrome undergoing surgery for tricuspid and pulmonary valve replacement, where we indicate the procedure forinduction, maintenance and termination of anesthesia without any reported events. The risk of a carcinoid crisis must beconsidered, in conjunction with a multidisciplinary approach to ensure effective surgery.
