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We present the case of a 1-month-old infant with right atrial isomerism and complex functionally univentricular heart disease, in whom totally anomalous pulmonary venous return drained below the diaphragm via two separate vertical veins. One of the descending vertical veins drained the entire right lung and the left upper lobe, while the other drained the remainder of the left lung. Only one similar case has been described previously.

Background: Abnormalities of brachiocephalic arterial branching and arch laterality are common in patients with a cervical aortic arch. In addition, structural anomalies of the arch such as obstruction, aneurysms, and tortuosity are found in a significant number of cases., Methods: Between 1990 and 1998, 6 patients underwent surgery for an obstructed right cervical arch. A significant obstruction was present at the transverse or distal arch in all patients, and was recurrent after previous repair in 2. In 1 patient, there was also a multi-lobed aneurysm of the aortic segment contiguous to the obstruction, and in 2 there was marked tortuosity of the arch. In all cases, the order of origin of the head and neck vessels was abnormal, and obstruction of 1 or more brachiocephalic vessels was found in 3. A vascular ring was present in all patients, with a right aortic arch and aberrant left subclavian artery in 4 patients and a double aortic arch with a dominant right cervical arch in 2. The descending aorta was circumflex (left-sided) in 3 patients. Three patients were repaired through a standard right posterolateral thoracotomy, and 3 through a median sternotomy. Patch augmentation aortoplasty was used in 2 patients, a tube graft from the ascending to descending aorta in 2, end to side anastomosis of the descending aorta to the proximal arch in 1, and direct anastomosis to reconstruct an atretic left-sided component of a double arch in 1., Results: Repair was successful in all cases, with no perioperative complications. At follow-up ranging from 1 to 9 years, all patients were alive and well, with no recurrence of arch obstruction or other significant complications. Fluorescent in situ hybridization revealed microdeletion of chromosome 22q11 in 1 patient (not performed in the others)., Conclusions: Structural anomalies of the arch are relatively common in patients with a cervical aortic arch. Such abnormalities may be the result of hemodynamic conditions and/or abnormal vascular tissue related either to the cervical position of the arch or its embryologic precursors. Given the highly variable anatomy of patients with a complicated cervical aortic arch, surgical considerations will vary in kind.

Background: Gastroesophageal reflux and dysmotility are common in children with trisomy 21. Children with trisomy 21 and congenital heart disease are at increased risk for complications of gastroesophageal reflux even after repair of their cardiac abnormalities. The optimal management of reflux in these patients is not known., Methods: The authors studied 24 consecutive infants (5.3+/-3.1 months) with trisomy 21 and atrioventricular septal defect who had symptoms or signs of gastroesophageal reflux and a positive esophageal pH study finding early after repair of their cardiac anomaly. Ten patients were given standardized medical therapy with upright positioning during and after feedings, thickening of feedings, metoclopramide, and an H2-receptor antagonist. The other 14 underwent primary surgical management consisting of Nissen fundoplication through a minilaparotomy., Results: All 10 medically treated patients required readmission within 2 weeks for complications related to reflux, including aspiration or pneumonia (n = 6), persistent failure to thrive (n = 2), and frequent apneic episodes (n = 2). No surgically treated patients had reflux-related complications requiring readmission. The total duration of hospitalization in the medically treated patients, including the initial hospitalization and the rehospitalization, was significantly longer than in patients who underwent fundoplication (35.8+/-9.8 v. 10.4+/-2.2 days, P<.001). At follow-up (24 to 56 months), all patients were alive except for 1 medically treated patient who died of aspiration pneumonia 28 days after readmission. Two medically treated patients required a Nissen, and 3 patients in the surgical group underwent redo fundoplication, all within 1 year. Three other patients in the medically treated group required a total of 8 hospitalizations for complications of reflux. No patient in either group required placement of a gastrostomy tube. Weight percentile for age was higher in surgical than medical patients., Conclusions: Infants with trisomy 21 and atrioventricular septal defect who undergo fundoplication are less likely to experience major complications of reflux early after cardiac surgery than those treated with a medical regimen of upright posture, thickened feedings, metoclopramide, and H2-receptor blockade.

Subannular aneurysms of the left ventricle are rare aneurysms originating adjacent to the aortic or mitral valve. Mitral subannular aneurysms are more common and have been well described. Two cases of aortic subannular aneurysm are reported, and an additional 20 cases were found in the literature. These aneurysms are usually found in young adults who may be seen with congestive heart failure, cardiac murmurs, angina pectoris, myocardial infarction, or even rupture. The aneurysms are often associated with aortic valve anomalies or subaortic stenosis. Diagnosis can be made by echocardiography and cardiac catheterization, and surgical repair is indicated.

A rare case is reported of premature closure of the foramen ovale in d-transposition of the great vessels with intact ventricular septum and normal development of the left side of the heart. Asphyxia neoatorum and cardiac failure were the manifestations in the early neonatal period. Balloon atrial septostomy during cardiac catheterization would be unsuccessful in infants with this condition, and closed atrial septectomy (Blalock-Hanlon procedure) would be necessary for survival.

A total of 130 cases of pulmonary vascular sling, including seven new cases in our collection, were studied. The sex distribution was 60% male and 40% female. The age at presentation was in the first year of life in approximately 90% of cases. Barium-esophagraphy showing anterior indentation was diagnostic in most of the cases. Bronchoscopy and tracheobronchography were useful in detecting associated tracheobronchial anomalies preoperatively. The analysis of 68 autopsied cases revealed associated tracheobronchial anomalies in 40% of the cases. Anomalies of the tracheobronchial tree took three major forms: abnormal distribution of cartilage in the walls of the trachea and major bronchi, intrinsic stenosis, and abnormal branching, the latter being that of bronchus suis. Acquired changes secondary in the sling resulted in compression of the major respiratory pathway by the anomalous left pulmonary artery. Major associated cardiovascular anomalies were present in 30% of the cases. These were represented by ventricular septal defect, atrial septal defect, patent ductus arteriosus, tetralogy of Fallot, common ventricle, and coarctation of the aorta.