Right coronary sinus fistula for ruptured aneurysm to the right atrium in a patient with Noonan syndrome
Vega-Nañez, Oziel; Calderón-Osorio, Fernando Manuel; Muruato-Ontiveros, Eleazar; Vázquez-Torres, José Luis; López-Rodríguez, Efrén
ABSTRACT
Introduction: an aneurysm at the level of the sinuses of Valsalva is a pathology of congenital or acquired origin. The presence of a fistula can also be asymptomatic or express itself at any age with different manifestations.
Case presentation: 34-year-old female patient carrier of Noonan syndrome (NS); surgical history of ventricular septal defect closure. Since the patient was 27 years old, she presented episodes of dyspnea. Cardiopulmonary auscultation revealed the presence of a continuous murmur in the second right parasternal intercostal space. A transthoracic echocardiogram was performed with findings of a fistulized right coronary aneurysm. Surgery was performed where a fistula from the right coronary sinus to the right atrium was observed, proceeding to closure.
Conclusions: a fistula from a coronary sinus to a cardiac cavity is a rare presentation of a ruptured aneurysm, so it highlights the presentation of this pathology in a patient with a RASopathy related to cardiac defects. Diagnosis requires an adequate approach. However, in some cases, the exact location of the defect can be determined during surgery. Early diagnosis and treatment are crucial.
