ABSTRACT
Coronary Artery Fistulae (CAFs) are cardiac congenital anomalies consisting of an abnormal communication of a coronary artery with either a cardiac chamber or another cardiac vessel. In humans, these congenital anomalies can lead to complications such as myocardial hypertrophy, endocarditis, heart dilatation, and failure. Unfortunately, despite their clinical relevance, the aetiology of CAFs remains unknown. In this work, we have used two different species (mouse and avian embryos) to experimentally model CAFs morphogenesis. Both conditional Itga4 (alpha 4 integrin) epicardial deletion in mice and cryocauterisation of chick embryonic hearts disrupted epicardial development and ventricular wall growth, two essential events in coronary embryogenesis. Our results suggest that myocardial discontinuities in the embryonic ventricular wall promote the early contact of the endocardium with epicardial-derived coronary progenitors at the cardiac surface, leading to ventricular endocardial extrusion, precocious differentiation of coronary smooth muscle cells, and the formation of pouch-like aberrant coronary-like structures in direct connection with the ventricular lumen. The structure of these CAF-like anomalies was compared with histopathological data from a human CAF. Our results provide relevant information for the early diagnosis of these congenital anomalies and the molecular mechanisms that regulate their embryogenesis.
Subject(s)
Heart Defects, Congenital , Heart , Mice , Humans , Animals , Myocardium , Coronary Vessels/pathology , Heart VentriclesABSTRACT
La esternotomía media es el acceso habitual para la corrección quirúrgica de las cardiopatías congénitas. Sin embargo, la cicatriz visible recuerda siempre que se trata de un cardiópata. Diversos abordajes alternativos han sido explorados en afecciones simples con objeto de ocultar la cicatriz. Presentamos una serie de 26 pacientes intervenidos de comunicación interauricular por vía axilar. La media de edad fue de 5,45 años (rango 3-13) y de peso 19,84 kg (rango 13-37). En 13 casos el defecto se cerró directamente (sutura) y en los 13 restantes, mediante parche de pericardio propio. Los pasos y los tiempos quirúrgicos son superponibles a la técnica clásica. La recuperación funcional, estancia en cuidados intensivos y planta hospitalaria hasta el alta han sido estándar. El resultado estético, valorado por pacientes y familiares, ha sido excelente (AU)
Mid-line sternotomy is the routine approach for surgical repair of congenital heart diseases. However, its noticeable scar is a constant reminder of having undergone heart surgery. Several alternative approaches have been developed for simple cardiac conditions to hide the scar. Our series, consisting of 26 patients with axillary closure of atrial septal defect, is presented. The median age was 5.45 years (range 3-13), and median weight was 19.84 Kg. (range 13-37). The defect was closed directly in 13 cases, and with an autologous pericardial patch in the other 13. The number of surgical steps and time taken were the same as in median sternotomy. Functional recovery, intensive care unit stay, and hospital discharge were also standard. The cosmetic result, assessed both by patients and relatives, was excellent (AU)
Subject(s)
Humans , Male , Female , Child, Preschool , Child , Adolescent , Axilla/surgery , Heart Septal Defects, Atrial/surgery , Extracorporeal Circulation/methods , Minimally Invasive Surgical Procedures/methods , SternotomyABSTRACT
Mid-line sternotomy is the routine approach for surgical repair of congenital heart diseases. However, its noticeable scar is a constant reminder of having undergone heart surgery. Several alternative approaches have been developed for simple cardiac conditions to hide the scar. Our series, consisting of 26 patients with axillary closure of atrial septal defect, is presented. The median age was 5.45 years (range 3-13), and median weight was 19.84 Kg. (range 13-37). The defect was closed directly in 13 cases, and with an autologous pericardial patch in the other 13. The number of surgical steps and time taken were the same as in median sternotomy. Functional recovery, intensive care unit stay, and hospital discharge were also standard. The cosmetic result, assessed both by patients and relatives, was excellent.
Subject(s)
Heart Septal Defects, Atrial/surgery , Adolescent , Axilla , Cardiac Surgical Procedures/methods , Child , Child, Preschool , HumansABSTRACT
Carney's triad is defined by the coexistence of at least two of three rare disorders, including gastric epithelioid leiomyosarcoma (malignant leiomyoblastoma), pulmonary chondroma, and paraganglioma, most often extra-adrenal and functioning. We report a new case in a 10-year-old girl. The paraganglioma, although nonfunctioning, was detected after it was searched for, as Carney's triad was suspected. Unrelated seems the development of breast fibroadenomas in the same patient. Whenever a patient with one component of the triad is encountered, the possibility of this syndrome should be considered and the other two components sought.
