Ectopia cordis.

Ectopia cordis is a rare congenital defect in which the heart is placed externally on the surface of the chest. This article describes the embrylogic events that lead to the various classifications of the defect and how they possibly explain the process of its unusual occurrence in children. While survival in some cases is possible, the ultimate repair is difficult and survival is rare. The most extreme forms of ectopia cordis, especially those with intracardiac defects, have a poor prognosis. Several cases of this rare anomaly with primary repair and staged repair are discussed.

Removal of an infected ventricular septal defect patch after tetralogy repair.

An infected Dacron ventricular septal defect (VSD) patch was removed 15 years after repair of tetralogy of Fallot. Cardiopulmonary bypass was not utilized, and no significant hemorrhage was encountered. The patient recovered uneventfully and continues to do well 7 years after surgery without infection or residual VSD.

Psychological support to an autologous bone marrow transplant unit in a community hospital: a pilot experience.

Autologous Bone Marrow Transplantation (AuBMT) has emerged as an efficacious treatment for certain hematological and solid tumors. Some of the complications of allogeneic transplants are avoided with an autologous transplant. Due to the decreased toxicity of this procedure, AuBMT can be administered at a community hospital where the setting is less burdensome for patients and families. The latter can continue to provide support for the patient as relocation is not necessary and visiting is easier. This report is a case analysis of the first 30 patients to undergo an autologous transplant as a pilot study in such an environment. This pilot project--done in a community hospital in Connecticut--may provide cost effective, high quality care in a more personal environment in the patient's own community. The different neoplastic diagnoses are listed as well as the types of psychological interventions available for these patients. Two case studies are described for clinical illustration. A discussion presents how psycho-oncology can assist the multidisciplinary team.

Unique association of a rapidly growing right atrial myxoma in a child with double-outlet right ventricle.

We describe a patient with double-outlet right ventricle in whom a large right atrial myxoma developed over approximately 6 months. This patient represents the first case described of a right atrial myxoma occurring in an unoperated patient with congenital heart disease other than an isolated atrial septal defect. Because the child was followed with serial echocardiograms, we can document the rapid growth of the tumor.

Expanded PTFE membrane to prevent cardiac injury during resternotomy for congenital heart disease.

BACKGROUND: Resternotomy for repair of congenital cardiac defects can result in cardiac injury. Closure of the pericardium during the initial operation may prevent this, and several pericardial substitutes have been tried, with variable results, in patients in whom primary pericardial closure is not possible. We conducted a multicenter observational study of the use of the expanded polytetrafluoroethylene membrane (Preclude Pericardial Membrane, formerly called the Gore-Tex Surgical Membrane; W. L. Gore & Associates, Flagstaff, AZ) in patients likely to undergo reoperation for treatment of congenital heart disease. METHODS: Data were collected retrospectively on all patients in whom the expanded polytetrafluoroethylene membrane was inserted at the initial operation for congenital heart disease at 12 centers in 1984 to 1993. RESULTS: A total of 1,085 patients (mean age, 55 +/- 2.5 months) received the membrane. During follow-up ranging from 1.3 to 10.5 years, 105 reoperations were performed. Injury during resternotomy occurred in only 1 patient (1% of reoperations). There were no membrane-related deaths or complications in the entire series of 1,085 patients. CONCLUSIONS: The expanded polytetrafluoroethylene membrane was safe and effective in helping to prevent cardiac injury during resternotomy for treatment of congenital heart disease.

Single-stage repair of thoracic ectopia cordis.

Thoracic ectopia cordis is a rare congenital defect most often seen in association with sternal and congenital heart defects. Surgical correction of these defects is complex and generally requires a staged closure including (1) coverage of the "naked heart," (2) placement of the heart into the thoracic cavity, and (3) sternal or thoracic reconstruction. Survival past the perioperative period is rare, with only 2 reported cases in the English-language literature. As with our case, neither had any discernable intracardiac defect. We present a case report of a patient with thoracic ectopia cordis repaired in a single stage using polytetrafluoroethylene membrane and skin for coverage and closure of the heart and thoracic defect.

Pediatric intrathoracic large airway obstruction: diagnostic and therapeutic considerations.

We describe seven patients who had intrathoracic airway obstruction resulting in severe respiratory distress. The patients were a four month old with bronchial cartilage hypoplasia presenting as congenital lobar emphysema; a one month old with segmental bronchomalacia; a one month old with tracheal bronchus; a two day old and a seven month old with aneurysmal distension of the pulmonary arteries secondary to tetralogy of Fallot, with absent pulmonary valve and atrioventricular canal with pulmonary poststenotic dilatation, respectively; a three year old with double aortic arch forming a vascular ring; and a three month old with tracheomalacia from tracheoesophageal fistula. The patients had various associated congenital defects, and all of them required one or more surgical procedures to repair the defects and relieve the airway obstruction. Five patients received endotracheal intubation with mechanical ventilation on their first admission, and three of them died postoperatively. The diagnostic workup to delineate the anatomic factors causing the obstruction, as well as to determine the therapeutic medical and surgical strategies to relieve it, are described. We conclude that the course of illness in most pediatric patients with intrathoracic airway obstruction is complex, is associated with high morbidity and mortality, and entails extensive multidisciplinary diagnostic and therapeutic efforts.

Role of extended aortoplasty related to the definition of coarctation of the aorta.

One hundred thirty-nine patients underwent operation for coarctation of the aorta. Age ranged from 1 day to 21 years and weight, from 1.5 to 70.4 kg. Numerous methods of repair were used. The operative mortality was low (1.3%), and 17 patients (11.3%) died late. Recoarctation occurred in 13 patients (9.4%). We attempted to correlate mortality and recoarctation with the surgical procedure. A review of the literature revealed no classifications of coarctation that applied to the anatomical and pathological variations we found at the time of operation. Therefore, we devised a surgical classification to separate the various entities in the spectrum of coarctation: type I = primary coarctation; type II = coarctation with isthmus hypoplasia; and type III = coarctation with tubular hypoplasia involving the isthmus and segment between the left carotid and left subclavian arteries. Each of these types has subtypes: A = with ventricular septal defect and B = with other major cardiac defects. We believe that rather than labeling one procedure as "the procedure of choice," providing this classification will allow the surgeon to use a method of repair that is suited to the anatomical variation.

Neonatal aortic stenosis.

Aortic stenosis in the neonate has been associated in the past with a high operative mortality. As a result, in the current era of percutaneous balloon dilatation, the optimal mode of therapy remains controversial. An approach of stabilization with cardiopulmonary bypass, followed by relief of left ventricular outflow tract obstruction, was used at three institutions, and the results are presented. During the period 1983 to 1989, 40 neonates with isolated aortic stenosis and patent ductus arteriosus or coarctation of the aorta, or both, underwent operative therapy. Ages ranged from 1 to 30 days, median of 12 days, including 17 patients in the first week of life. There were 30 boys and 10 girls; weights ranged from 2.5 to 5.5 kg with a mean of 3.6 kg. Perioperative conditions included congestive heart failure in 38 and mitral regurgitation in 16; left ventricular-aortic gradients ranged from 15 to 130 mm Hg, with a mean of 67 mm Hg. There were 30 open valvotomies and 10 transventricular dilatations. The hospital survival rate was 87.5% (35/40) with no significant difference between the methods of valvotomy (9/10 in the transventricular dilatation group, 90%; 26/30 in the open valvotomy group, 87%). Although multiple methods of perfusion and valvotomy were used, the single unifying factor of cardiopulmonary bypass stabilization was present in all 40 patients. No significant difference in survival was noted between institutions, methods of cardiopulmonary bypass, cardiopulmonary bypass times, crossclamp times, or method of valvotomy. There have been five reoperations, with one late death in a patient requiring mitral valve replacement and an apical-aortic conduit. One sudden death occurred; autopsy revealed endocardial fibroelastosis. Results demonstrate that in the three institutions using the methods described, a high operative and late survival rate is possible. The results of this technique, against which percutaneous dilatation should be compared, are standard in the current era.

New outlook on pericardial substitution after open heart operations.

The difficulties of reoperation owing to adhesions are well known. Clinical attempts to solve this problem using synthetic materials or glutaraldehyde-fixed pericardial xenograft have been less than satisfactory. Although experimental animal results have been good, they have not considered the influence of cardiopulmonary bypass (CPB) on adhesion formation. This study addressed the influence of CPB on the formation of adhesions and evaluated biodegradable polyglycolic acid as a material to reduce adhesions and as a pericardial substitute. Forty-five weanling sheep received implants of pericardial xenograft and polyglycolic acid with and without CPB. The pericardial xenograft showed no adhesions when implanted without CPB, but severe adhesions formed and a thick fibrinous layer covered the heart when CPB was used, making identification of coronary arteries at reoperation very difficult. The polyglycolic acid mesh implanted without CPB was absorbed and replaced with newly formed host collagen. With CPB, the polyglycolic acid was more rapidly absorbed, and a thinner layer of host collagen formed. Therefore, future animal studies must include CPB. In agreement with reported clinical results, glutaraldehyde-fixed pericardial tissue implanted with CPB resulted in severe epicardial reaction and therefore is not an ideal pericardial substitute.

Experience with the polytetrafluoroethylene surgical membrane for pericardial closure in operations for congenital cardiac defects.

From 1984 through 1987, pericardial closure for 96 patients who underwent repair of congenital heart defects was completed with placement of a polytetrafluorethylene surgical membrane. Ages ranged from 1 day to 20 years (median age 2.1 years), and weight ranged from 2.7 to 44.6 kg (median weight 10.7 kg). Use of the membrane increased over the 4-year period. The patients were divided into three groups according to probability of reoperation: group I, low probability (35 patients); group II, intermediate probability (41 patients); and group III, high probability (20 patients). Early postoperatively there were no infections in any group and no complications attributable to the polytetrafluoroethylene membrane. No increase in pericardial drainage was found when the studied patients were compared to similar groups without the membrane. There were a total of 17 deaths (17.7%): group I, 1/35 (2.8%); group II, 9/41 (21.9%); and group III, 7/20 (35%). None of the deaths could be attributed to placement of the polytetrafluorethylene membrane. Seven patients had a second operation, which made possible the in vivo evaluation of the membrane. In addition, there was one late death, which allowed an autopsy. Findings were similar in all eight. There were no adhesions between the chest wall and the membrane. The latter had basically become transparent and was loosely attached to the epicardium. On the epicardial surface there was a thin layer of collagenous fibrous tissue that did not interfere with the visualization of the heart anatomy, including the coronary vessels. At early postoperative follow-up, difficulty in evaluation of the patient by two-dimensional echocardiography was encountered only in the parasternal view, but not in the other scans. In the parasternal view, the membrane is between the echo transducer and the heart and there is moderate blurring of finer details of the cardiac architecture. At late postoperative ultrasound study, resolution of the images in the parasternal views is much better. The following conclusions were reached: (1) Polytetrafluorethylene surgical membrane can be used for pericardial closure; (2) reoperation shows no adhesions to the chest wall or epicardium, and visualization of the cardiac surface, especially of the coronary vessels, is not obscured; (3) the membrane causes some difficulty in the echocardiographic examination of patients in the parasternal views, but other views allow for a complete postoperative evaluation of the heart.

Bone as a secondary missile: an experimental study in the fragmenting of bone by high-velocity missiles.

Destruction to soft tissues by bone fragments has been described in the literature. Civilian and military injuries have been reported in which bone fragments have acted as secondary missiles. This experimental study demonstrates, in detail, the formation of the temporary cavity within bone and the forward motion of these bone spicules. The knowledge of the ballistics will assist in the treatment of wounds caused by high-velocity missiles.

Systemic-pulmonary polytetrafluoroethylene shunts in palliative operations for congenital heart disease. Revival of the central shunt.

The concept of central shunting in smaller children with the Waterston shunt was initially well accepted. It has been abandoned because of the difficult estimation of lumen size, preferential flow to the right side, and difficulty in the take-down of the shunt. We have replaced the Waterston shunt with a short segment of polytetrafluoroethylene between the ascending aorta and the main pulmonary artery. From January 1979 to December 1986, 190 shunt operations were performed in 157 patients, with the use of 26 classic Blalock-Taussig shunts (13.7%), six Waterston shunts (3.1%), nine Glenn shunts (4.7%), 80 central aortopulmonary polytetrafluoroethylene shunts (42.2%), and 69 modified Blalock-Taussig shunts (36.3%). Polytetrafluoroethylene grafts were used for 149 of the 190 (78.4%) shunts. Overall mortality was 15.2%, with nine early deaths (4.7%) and 20 late deaths (10.5%). Deaths were due to the complex nature of the congenital anomaly or definitive surgical repair. The patients weighed from 1.6 to 48 kg and ages ranged from 1 day to 22 years. We have modified our technique so that (1) graft length is less than 0.5 cm and both ends are beveled, (2) the aortotomy is fashioned with a punch, (3) the center of the polytetrafluoroethylene graft is never clamped, (4) heparin is given during the construction of the shunt, and (5) aspirin (10 mg/kg/day) is administered daily. Patency ranges from 1 to 4 years. We conclude that the polytetrafluoroethylene shunt provides excellent palliation and that the central shunt, in the smaller child and infant, offers the benefits of shunting without distortion of the peripheral pulmonary arteries.

Cystic lung disease in Down's syndrome: a report of two cases.

Previously unreported lung disease found at autopsy in 2 young infants with Down's syndrome and congenital heart disease (complete atrioventricular canal malformation with left-to-right shunt) is described. The perinatal and neonatal period was unremarkable, and there was no history of mechanical ventilation or administration of high concentration of oxygen for extended periods. In 1 of the cases respiratory symptoms and hyperinflation with focal cystic changes in the lung fields on chest X-ray were noted at 5-7 months of age. Pathologically there was cystic dilatation of alveoli with focal cuboidal metaplasia of alveolar epithelium and mild to moderate focal alveolar septal fibrosis. Wilson-Mikity syndrome, congenital pulmonary lymphagiectasia, bronchopulmonary dysplasia, and idiopathic interstitial fibrosis of lungs were ruled out on clinical and/or pathologic grounds. Factors such as compression of bronchi by enlarged pulmonary arteries or cardiac chambers, peribronchiolar accumulation of fluid, pulmonary hypoplasia occurring in Down's syndrome, and episodes of pulmonary arterial hypoperfusion associated with severe congenital heart disease may be related to the pathogenesis of the lesion.