ABSTRACT
Truncus arteriosus (TA) is a rare congenital heart disease defined as a single arterial vessel arising from the heart that gives origin to the systemic, pulmonary and coronary circulations. The truncal valve in majority of the cases is tricuspid though quadricuspid and bicuspid valves have been reported. Patients with TA typically have a large nonrestrictive sub truncal ventricular septal defect. Survival of these infants beyond 1‑year is uncommon. Here, we report a unique case of 12‑year‑old female patient with persistent TA who underwent surgical repair by using transesophageal echocardiography as a monitoring device during the perioperative management.
Subject(s)
Child , Echocardiography, Transesophageal/methods , Echocardiography, Transesophageal/therapeutic use , Female , Heart Defects, Congenital/surgery , Heart Defects, Congenital/therapy , Humans , Perioperative Period/therapy , Truncus Arteriosus/surgery , Truncus Arteriosus/therapyABSTRACT
Descrevemos um caso de adulto de 28 anos com suspeita de cardiopatia congênita desde o nascimento, não tratada na infância por opção da família. Aos 27 anos, foi feito diagnóstico de atresia pulmonar com comunicação interventricular e colaterais sistêmico-pulmonares, sendo contraindicada a cirurgia. Uma nova reavaliação em nosso serviço demonstrou tratar-se de um truncus arteriosus atípico. O fato de um tronco arterial comum com shunt esquerda-direita ter sido visualizado ao ecocardiograma foi um dado crucial para a indicação de novo cateterismo, abrindo perspectiva de correção cirúrgica. No momento, o paciente encontra-se bem, com 7 anos de evolução pós-operatória.
This is the case of 28 year-old adult with suspected congenital heart disease since birth, not treated in childhood at the his family's choice. At 27 years old, he was diagnosed with pulmonary atresia with ventricular septal defect and systemic-pulmonary collaterals, where surgery was contraindicated. A new review in our department showed that it was an atypical form of truncus arteriosus. The fact that a common arterial trunk with left-right shunt was viewed by echocardiography was a crucial fact for the indication of new catheterization, opening the prospect of surgical correction. Currently, the patient is well, with 7 years of postoperative outcome.
Describimos un caso de adulto de 28 años con sospecha de cardiopatía congénita desde el nacimiento, no tratada en la niñez por opción de la familia. A los 27 años, se hizo diagnóstico de atresia pulmonar con comunicación interventricular y colaterales sistémico-pulmonares, y se contraindicó la cirugía. Una nueva reevaluación en nuestro servicio reveló tratarse de un truncus arteriosus atípico. El hecho de un tronco arterial común con shunt izquierdaderecha haber sido visualizado al ecocardiograma fue un dato crucial para la indicación de nuevo cateterismo, abriendo perspectiva de corrección quirúrgica. Al momento, el paciente se encuentra bien, con 7 años de evolución posoperatoria.
Subject(s)
Adult , Humans , Male , Heart Defects, Congenital/complications , Truncus Arteriosus/surgery , Age Factors , Diagnosis, Differential , Truncus ArteriosusABSTRACT
Most commonly encountered cyanotic cardiac lesions in children, namely, tetralogy of Fallot, transposition of the great arteries and tricuspid atresia are reviewed. Pathology, pathophysiology, clinical features, non-invasive and invasive laboratory studies and management are discussed. The clinical and non-invasive laboratory features are sufficiently characteristic for making the diagnosis and invasive cardiac catheterization and angiographic studies are not routinely required and are needed either to define features, not clearly defined by non-invasive studies or as a part of catheter-based intervention. Surgical correction or effective palliation can be undertaken with relatively low risk. However, residual defects, some requiring repeat catheter or surgical intervention, are present in a significant percentage of patients and therefore, continued follow-up after surgery is mandatory.