Right pulmonary artery to left atrium fistula.

In this report, we describe a 25-year-old patient in whom a fistula between the right pulmonary artery and the left atrium was accurately diagnosed. The successful surgical management of this patient is described. Current diagnostic methods, evaluation, and treatment options for this uncommon cause of cyanosis in an adult patient are discussed.

Surgical management of lesions encountered in the setting of the retroaortic left brachiocephalic vein.

BACKGROUND AND AIM: Although the retroaortic left brachiocephalic vein in isolation is of no clinical importance, its recognition in the setting of associated lesions is important. We sought to address issues concerning the influence of isomerism, the establishment of diagnosis, and its importance in various surgical and interventional procedures. METHODS: A total of 80 published clinical and necropsy studies in the setting of a retroaortic left brachiocephalic vein described 250 patients. Clinical presentation, radiographic, ultrasonographic findings, contrast echocardiography, computed-tomographic angiocardiography, magnetic resonance imaging, and angiocardiography provided the diagnostic information prior to considering the surgical approach to the associated cardiac anomalies. RESULTS: Among 250 reported cases, three-quarters had associated congenitally malformed hearts. Of these 189 patients, all but seven had usual atrial arrangement. Right isomerism was reported in five patients and two patients having left isomerism. Almost two-thirds had tetralogy of Fallot or its variants, over four-fifths had malformations involving the outflow tract, two-thirds had a right aortic arch with two patients having a cervical aortic arch, and onepatient had double aortic arch. Various innovative individualized surgical procedures were employed with an overall perioperative mortality of 3.4%. CONCLUSIONS: Although the retroaortic left brachiocephalic vein is asymptomatic, its recognition during clinical investigation should raise the possibility of an association with other malformations, especially right aortic arch, ventricular septal defect, and anomalies of the outflow tracts. We submit that an increased appreciation of this venous anomaly may facilitate surgical planning, endovascular procedures, placement of central venous lines, and transvenous pacemakers.

Totally anomalous pulmonary venous connection with intact atrial septum.

BACKGROUND: Total anomalous pulmonary venous connection with intact interatrial septum is extremely rare. AIMS: In these patients, a right to left shunt through a ventricular septal defect or a patent ductus arteriosus is mandatory to maintain the systemic circulation. Mechanical or physiological restriction of shunting through these pathways results in rapid clinical deterioration. DISCUSSION: We describe the anatomical findings with surgical repair in one such 10-day-old baby.