Incidence of aneurysm formation after Dacron patch aortoplasty repair for coarctation of the aorta: long-term results and assessment utilizing magnetic resonance angiography with three-dimensional surface rendering.

OBJECTIVES: Magnetic resonance angiography with three-dimensional surface rendering was performed to determine its value in assessing anatomic detail in patients with suspected aortic aneurysms. BACKGROUND: Dacron patch aortoplasty repair of coarctation of the aorta carries an inherent risk of aneurysm development. Sudden death from aortic rupture prompted discontinuing this operation and evaluating 39 patients (16 girls; mean age 6.3 years, range 10 days to 14.5 years) undergoing repair between January 1976 and October 1987. The aorta ruptured in 10 patients; 6 died at a mean interval of 8.1 years (range 0.75 to 12.4) after repair. All 33 survivors were interviewed and examined. METHODS: Conventional magnetic resonance imaging was performed in 26 patients, magnetic resonance angiography in 18. Angiographic slices were used to reconstruct three-dimensional images. No catheterization or contrast angiography was performed. Surgical intervention was based on clinical findings and magnetic resonance images. RESULTS: Twenty patients (11 girls) developed aneurysms, of which nine were detected in patients studied by magnetic resonance. Ruptures occurred in eight female patients, three of whom were pregnant. Surface renderings accurately defined aortic anatomy or aneurysms in all patients. On follow-up, no aneurysms have been detected in patients with negative magnetic resonance study results. Precise anatomic correlation with operative findings was reported. CONCLUSIONS: Magnetic resonance angiography with three-dimensional surface rendering provides noninvasive, radiation-free and contrast agent-free high resolution images of the thoracic aorta. These images can be reviewed and have three-dimensional form and perspective. These techniques were preferred over invasive angiography by surgeons and clinicians as definitive, risk-free procedures before surgical intervention.

Systolic honks in young children.

Three children with loud systolic honks were studied noninvasively with phonocardiography and echocardiography. It was shown that the precordial honk, like the late systolic mitral murmur and the clicking apical systolic sound, is part of a continuum of auscultatory sounds that result from a defect of mitral valve support and are classified under the general heading of mitral valve prolapse syndrome. Prolapse of one or both of the mitral valve leaflets is believed to cause the characteristic auscultatory findings of click, murmur or honk. The timing of these sounds in systole varies with different physiologic or pharmacologic maneuvers. Variations in the onset of prolapse are associated with changes in left ventricular end-diastolic dimensions.

A palliative operation for all types of aortic arch interruption in the neonate.

A palliative operation is described for the treatment of infants with all types of aortic arch interruption without regard to anatomic variation. It consists of establishment of continuity between the main pulmonary artery and descending thoracic aorta with a prosthetic graft, pulmonary artery banding distal to the graft, and ligation of the closing ductus arteriosus. Infant mortality rate in the past has been high, rega-dless of the type of operation used. The method of treatment described in this report is one in which an initial palliative operation is employed in the newborn period with total correction planned at a later date, when the risk of morbidity and death will be less.

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.

Hemangioma of the right ventricle causing outflow tract obstruction.

A 3 1/2-year-old child had a murmur of pulmonary stenosis. Echocardiography and cardiac catheterization revealed a pulmonary infundibular obstruction. Magnetic resonance imaging of the heart demonstrated a mass in the interventricular septum. The mass was successfully resected and a pathologic diagnosis of capillary hemangioma was made. Only two previous cases of hemangioma causing right ventricular outflow obstruction have been reported; both of these cases involved adults. This case represents the first report of a hemangioma causing right ventricular outflow tract obstruction in a child. An exploratory operation with resection is the treatment of choice.

Individualized surgical management of complete atrioventricular canal.

In a 5 year interval 46 children with complete atrioventricular canal (CAVC) required 51 operations. Thirty-two children underwent correction; nine received surgical palliation. The ages of the children undergoing correction ranged from 4 months to 14.6 years (mean 4.2 years); eight were less than 1 year old. Weights ranged from 4.1 kg to 39 kg (mean 13.5 kg); 15 weighted less than 10 kg. Ten had undergone previous palliation (seven by pulmonary artery band; three by shunt). There were no early deaths and two late deaths. One infant required mitral replacement at correction; two required subsequent mitral replacement. The ages of the children undergoing palliation ranged from 8 days to 1.34 years (mean 5.8 months); 16 were less than 1 year old. Weights ranged from 2.5 kg to 8.5 kg (mean 4.4 kg); 14 weighted less than 5 kg. Operations included pulmonary artery banding in 14, shunt creation in four, and pericardial enlargement of the right ventricular outflow tract in one. One death occurred 5 days after pulmonary artery banding in an infant with unrecognized coarctation. One late death occurred several months after the creation of a second shunt in a child with severe tetralogy of Fallot and hypoplastic pulmonary arteries. Forty-two (91%) of these children were alive at the time of this review. The outcome in these 46 patients supports individualized choice of initial operation (palliation versus correction) based upon clinical condition, weight, and associated anomalies. The pulmonary artery is banded in infants less than 4 to 5 kg; larger infants and children undergo correction primarily.

Prostaglandin-induced hyperostosis. A case report.

The use of prostaglandin-E1 (PGE1) to maintain patency of the ductus arteriosus in infants with ductal-dependent congenital heart disease is now well established. A 2.5-month-old child with cyanotic heart disease who required long-term PGE1 infusions; developed widespread periosteal reactions during the course of therapy. Prostaglandin-induced subperiosteal hyperostosis should now be considered in the differential diagnosis of neonatal cortical proliferation.