Aortic atresia with normal left ventricle

A case of single-stage repair of aortic atresia with normal-sized left ventricle and ventricular septal defect in a neonate is reported. The surgical procedure included rerouting of the left ventricular bloodstream to the pulmonary artery through the ventricular septal defect and connection of the pulmonary trunk to the aortic arch. The right ventricular outflow tract was then reconstructed with an extracardiac valved conduit. Three years after the initial operation, replacement of the valved conduit was performed uneventfully. The clinical status of the child is very satisfactory.     

Aortic atresia: morphologic characteristics affecting survival and operative palliation

A quantitative anatomic study of 54 cardiac specimens with aortic atresia is related to surgical treatment of patients with aortic atresia and hypoplastic left heart syndrome. Coarctation of the aorta was present in 80% of patients with aortic atresia and should influence the extent of aortic arch reconstruction when present. Other associated cardiac defects were uncommon but, when present, may be contraindications to operative palliation because of added complexity of the operation. Anatomic abnormalities of the tricuspid or pulmonary valve were present in 7% of cases and could be important in the outcome of palliative procedures. Natural survival was related to thickness of the right ventricular wall so that selection of those infants with thicker and better functioning right ventricles may improve short-term and long-term operative results in aortic atresia.     

Aortic atresia with normal left ventricle: one-stage repair in early infancy

Successful one-stage repair of aortic atresia with a left ventricle was performed in a 6-week-old infant using a new technique. After patch enhancement of the hypoplastic aortic arch, the transected proximal pulmonary artery was directly anastomosed to the aortic arch. An intraventricular baffle established continuity between the left ventricle and neoaortic valve, and a homograft conduit was inserted between the right ventricle and pulmonary artery bifurcation.     

Aortic atresia with normal left ventricle. Single-stage repair in the neonate

Four to seven percent of infants born with aortic atresia have a normal-sized left ventricle in association with a ventricular septal defect and a normal mitral valve. In contrast to the more common group of infants with aortic atresia whose left ventricle is hypoplastic, this important subgroup has potential for complete operative correction involving both the right and left ventricles. Previous reports of surgical management of these infants have described early palliative procedures to control systemic and pulmonary blood flow. Our approach has been to perform complete repair at one stage in the neonate. Continuity between the proximal main pulmonary artery and aorta is established, a patch on the ventricular septal defect baffles left ventricular blood to the pulmonary valve, and a homograft conduit achieves right ventricular-pulmonary artery continuity. Three of four neonates who underwent single-stage repair were discharged from the hospital in good condition. Achieving a physiologically normal circulation simplifies the postoperative management of this condition and is to be preferred over neonatal palliation.     

Aortic atresia with aortico-left ventricular tunnel mimicking severe aortic incompetence in utero.

A fetus was diagnosed with regurgitation from the aorta into the left ventricle. A large aortico-left ventricular tunnel with aortic atresia was identified postnatally by echocardiography in a neonate suffering from severe heart failure. We successfully reconstructed the valve and closed the tunnel with a patch on his first day of life. At three-month follow-up, the baby is asymptomatic with mild aortic regurgitation. The combination of congenital aortico-left ventricular tunnel and aortic atresia can be misinterpreted in prenatal echocardiography as aortic regurgitation. This pathology may require urgent operative interventions.     

Aortic atresia with a ventricular septal defect: modified single-stage neonatal biventricular repair

BACKGROUND: The spectrum of hypoplastic left heart disease is diverse but the surgical repair is strictly dichotomous, culminating in either a univentricular or a biventricular surgical repair. Although aortic atresia with a ventricular septal defect historically has been managed by conversion to a univentricular physiology, a biventricular repair occasionally has been attempted in stages or in conjunction with the implantation of multiple allografts or prosthetic conduits. Our repair strategy recently has evolved to the use of a modified single-stage biventricular repair using only autologous tissues without conduits. METHODS: Retrospective analysis (1989 to 1997) of neonates with aortic atresia with a ventricular septal defect. RESULTS: Five neonates underwent repair of aortic atresia with a ventricular septal defect. One died in the hospital. The mean age and weight of the neonates who underwent repair were 7.8 days (range, 2 to 17 days) and 3.2 kg (range, 3 to 3.6 kg), respectively. Three neonates had a univentricular repair and 2 had a modified biventricular repair. The latter two procedures were successful and the patients were discharged from the hospital. CONCLUSIONS: Long-term results are lacking to attest to this surgical modification's superiority over either the standard multistage univentricular operation or the single-stage biventricular repair using multiple conduits. However, we are optimistic that routine use of this modification will enable a greater percentage of neonates to undergo a biventricular repair without the need for serial conduit revisions or future aortoplasty.     

Aortic atresia and type B interrupted aortic arch: diagnosis by physiologic cerebral monitoring

Physiologic cerebral monitoring has become an important part of our cardiovascular surgical unit. We recently encountered an unusual variant of aortic atresia that was first suggested by physiologic cerebral monitoring and required modification of our operative technique. We describe and discuss the anatomy, its translation into cerebral monitor findings, and how we modified our operative technique.     

Aortic atresia and aortico-left ventricular tunnel: successful surgical management by Konno aortoventriculoplasty in a neonate

A newborn infant with aortic atresia and aortico-left ventricular tunnel was successfully treated by Konno aortoventriculoplasty. A 15 mm Ionescu-Shiley pericardial xenograft was inserted when the patient was 5 days old and it was replaced with a 19 mm St. Jude Medical prosthesis at 2 years of age.     

Esophageal atresia, choanal atresia, and dysautonomia

Patients with esophageal atresia (EA) or choanal atresia (CA) manifest similar clinical and pathophysiological features. To determine the significance of this observation, the clinical records of 80 patients with EA and 57 with CA were reviewed. This survey showed that similarities between the two conditions included inspiratory and expiratory dyspnea, episodes of reflex apnea and/or bradycardia, oropharyngeal dysphagia, vomiting, convulsions, hyperhydrosis, hyperthermia, sialorrhea, and sudden death. After the second year of life most symptoms disappeared spontaneously. In both conditions, respiratory effort resulted in partial or complete obstruction affecting both the inspiratory and expiratory phases of the respiratory cycle. Support for this finding was obtained by studying the breathing pattern of 3 patients with EA and 3 with CA, before and during postural respiratory loading. The data suggest that patients with EA are similar to those with CA, having upper airway instability that may result in obstructive hypopnea or apnea associated with expiratory grunting. It is possible that this upper airway instability is a manifestation of more general maturational dysautonomia previously not recognised in patients with EA.     

Ileocaecal atresia

Meconium ileus is one of the commonest causes of distal small bowel obstruction in neonates. Within the last six years at our hospital two newborns presented with identical signs and symptoms of meconium ileus. Abdominal x-rays showed distal small bowel obstruction with ground glass opacity in the right lower quadrant. Gastrografin enema in both cases demonstrated a microcolon, but there was no reflux of this contrast material into the terminal ileum. Laparotomy in both babies revealed atresia of the ileocaecal valve with dilatation of the terminal ileum loops which contained normal meconium. Ileocolic resection was carried out in both neonates. Sweat chlorides and eventual recovery were normal. Pathology in one of the specimens showed muscularis passing across the atretic area with a telescoped pattern of the muscularis propria suggesting intussusception in utero. Although the presentation of a newborn may be that of meconium ileus, the fact that the Gastrografin enema is unable to enter the terminal ileum in a retrograde fashion should be an indication for immediate laparotomy rather than persisting in this mode of treatment. It is obvious that occasionally the radiological diagnosis of meconium ileus is incorrect.     

Bronchial atresia

Bronchial atresia is a rare congenital anomaly usually producing an extrahilar mass and associated distal hyperinflation on chest roentogenogram. Bronchography reveals nonfilling of the atretic bronchus and displacement of neighboring normal bronchi. Characteristically, the patient is young and asymptomatic but has an abnormal chest roentgenogram. The only physical finding may be decreased breath sounds over the affected parenchyma. Treatment is surgical excision to prevent recurrent or persistent infection and encroachment on normal tissues by the hyperinflated lung. A case report and a review of 34 patients are presented.     

Pyloric atresia

We present a case of a female newborn with a single air bubble on abdominal x-ray by pyloric atresia and a short review of the literature.     

Esophageal atresia

Esophageal anomalies present a challenge to the pediatric surgeon and demand close attention and care from the nursing staff as well as the surgical staff. We have encountered a survival rate of 96 per cent for all infants undergoing operation for esophageal atresia. An emergency gastrostomy is recommended as an immediate procedure when the diagnosis is established. In many instances, it can be performed with local anesthesia if the infant is particularly small or has developed aspiration pneumonia. The definitive operation is delayed until the infant is in optimum condition to permit a general anesthetic and thoracotomy. Staging is recommended for all infants with a birth weight of less than 2000 gm. Once the infant has recovered from the operative correction of the malformation, significant late complications are rare, and the great majority of individuals lead completely normal lives.