Congenitally corrected transposition with pulmonary atresia and intact ventricular septum.

We describe a patient with the rare association of the heart in the left chest, congenitally corrected transposition, pulmonary atresia and an intact ventricular septum. There were associated fistulous communications between the morphologically left ventricle and the coronary arteries. Diagnosis was made by echocardiography, and subsequently confirmed by cardiac catheterization.

Pulmonary atresia with intact ventricular septum, right-sided aortic arch, and an aorto-pulmonary collateral artery.

Described is a rare association in a patient with the heart in the left chest, namely pulmonary atresia with intact ventricular septum, fistulous coronary arterial connections, a right-sided aortic arch and an aorto-pulmonary collateral artery feeding one lung. The pulmonary arteries were non-confluent, with the right lung supplied by the right arterial duct originating from the under surface of the right-sided aortic arch, and the left lung supplied through the aorto-pulmonary collateral artery arising from the descending aorta. The surgical management is different in the setting of non-confluent pulmonary arteries.

Costs and cost-effectiveness of routine transesophageal echocardiography in congenital heart surgery.

BACKGROUND: The safety and efficacy of transesophageal echocardiography (TEE) during congenital heart surgery is well established. The economic costs and benefits associated with its routine use in this setting are, however, uncertain. We sought to analyze the impact that routine intraoperative TEE had on echocardiographic costs in the setting of congenital heart surgery. METHODS AND RESULTS: A retrospective, case-controlled analysis of echocardiographic costs during the operative and postoperative periods was performed for 63 children undergoing elective, complex intracardiac repair. Similar analysis was performed for a smaller group of patients undergoing simple repairs. To ascertain whether any additional cost savings was realized through the use of TEE, we documented the impact that operative TEE had on altering surgical strategy as well as whether TEE use was associated with any intraoperative complications. Despite the additional expense, routine TEE, in the setting of complex repair, resulted in no significant increases in echocardiographic costs, suggesting the superior information provided may in fact reduce the need for additional postoperative studies in the intensive care setting. Although no child required a return to cardiopulmonary bypass, surgical therapy was altered by TEE findings in 2 (3%) of 63 patients. Complications were rare and self-limited, usually occurring with positioning of the probe in smaller patients. CONCLUSIONS: The findings of improved surgical outcomes in a percentage of patients, coupled with the lack of any significant increment in echocardiographic costs, confirm that intraoperative TEE is a beneficial and cost-effective intervention in children requiring complex cardiac repair.

Hypoplastic left heart syndrome with right aortic arch, bilateral arterial ducts and origin of the left subclavian artery from the left pulmonary artery.

The rare association, in a left-sided heart with hypoplastic left heart syndrome, of right aortic arch, bilateral patent arterial ducts and origin of the left subclavian artery from the left pulmonary artery are described. Cardiac catheterization was performed because of the abnormal anatomy of the arch noted at echocardiographic examination. This abnormality is of surgical importance when planning the Norwood operation.

Primary pediatric cardiac tumors: a 17 year experience.

We reviewed 22 cases of primary pediatric cardiac tumors followed at our institution from January 1981 through November 1997, analyzing them by subtype, age and manner of presentation, location, associated findings, interventions, and clinical course. Rhabdomyomas were the most common (11), followed by intrapericardial teratomas (2), myxomas (1), fibromas (1), hemangiomas (1), mesotheliomas (1), and rhabdomyosarcomas (1), with 4 undetermined tumors. The majority (77%) of tumors were diagnosed before the age of one year, including six prenatally. The most common presentations were murmurs (5), arrhythmias (5), and abnormal screening fetal ultrasound examinations (4). Tumors were located most frequently in the right ventricle (13) and left ventricle (7), with multiple tumors being present in 10 cases. Eight patients (36%) had associated arrhythmias or conduction abnormalities, and of the 11 patients with rhabdomyomas, tuberous sclerosis was diagnosed in 8. Eight patients underwent cardiac catheterization, including two for electrophysiologic study with radiofrequency ablation, and seven patients had complete or partial tumor resection. The follow-up period ranged from 2 months to 15 years, and there were 3 tumor-related deaths. Therefore, despite the benign histology of most primary pediatric cardiac tumors, there may be significant associated morbidity and occasional mortality. As echocardiographic techniques such as fetal ultrasonography have continued to improve, however, these cardiac tumors have increasingly been detected early before significant symptoms develop.

Cardiopulmonary responses to exercise in children with activity sensing rate responsive ventricular pacemakers.

The physiological benefits of activity sensing rate responsive ventricular pacing (VVIR) over fixed rate pacing (VVI) were investigated in 14 children during incremental cycle exercise. Based on their heart rhythm response during exercise, children were divided into two groups. Group I patients (13 +/- 4 years) remained in a paced-only rhythm when exercised. Group II patients (16 +/- 7 years) were paced at rest but converted to sinus rhythm with exercise. In Group I patients, the significant physiological benefits of VVIR over VVI pacing were evidenced by a 51% increase in peak heart rate (HRmax) and a 16% increase in exercise duration and maximum oxygen uptake (VO2max). Additionally, a 27% reduction in peak oxygen pulse (O2Pmax) was found, reflecting a similar decrease in stroke volume. The cardiorespiratory responses of Group I and II patients were compared in terms of percent of predicted normal values. Although Group I patients in the VVIR mode attained a better exercise performance than in the VVI mode and a normal O2Pmax (108% pred), their HRmax (62% pred) and VO2max (70% pred) fell far below normal values. In comparison, Group II patients, who went into sinus rhythm, achieved normal values for HRmax (84% pred), VO2max (90% pred), and O2Pmax (97% pred). The higher pacing rates attained by Group I patients in the VVIR mode may have allowed them to reach not only a higher cardiac output but also a more normal stroke volume at peak exercise than in the VVI mode.(ABSTRACT TRUNCATED AT 250 WORDS)

Management of nutritional and infectious complications of postoperative chylothorax in children.

Postoperative chylothorax may occur following any intrathoracic surgical procedure in children. From January 1979 through February 1987, 18 children aged 1 month to 9 years had chylothorax listed among their discharge diagnoses. Initial therapy for all patients consisted of chest tube placement for pleural space drainage and nutritional support with either enteral formulas enriched with medium-chain triglycerides or total parenteral nutrition. Both total parenteral nutrition and medium-chain triglyceride-enriched enteral regimens provided adequate nutritional support in these children. Five of the 18 experienced lymphopenia secondary to chylous lymphocyte loss. Infections were diagnosed in 5 patients during hospitalization; one was a fatal viral pneumonitis. No correlation between infectious complications and lymphocyte count could be demonstrated.

Carotid reconstruction in the neonate following ECMO.

Permanent ligation of the carotid artery remains a major objection to the use of extra corporeal membrane oxygenation (ECMO) in infants with severe cardiorespiratory disorders. Because reconstruction of the carotid artery is highly desirable, we began a study to evaluate the feasibility and risks of carotid artery repair following decannulation. All infants placed on ECMO from December 1988 to January 1990 were evaluated for carotid artery reconstruction. During this period 18 infants underwent carotid reconstruction and 8 infants were deemed unsuitable candidates. Patency of the right common carotid artery was demonstrated in 14 of the 18 infants with good bilateral anterior and middle cerebral artery flow. Seven infants have had MRA evaluation at 6 months and have demonstrated no significant change from their discharge study. These preliminary findings suggest that carotid reconstruction can be performed safely with no apparent morbidity in the majority of infants placed on ECMO, but long-term follow-up data concerning patency rate and neurological status must be obtained before this technique is applied to all infants with this problem.

Pneumothorax in cystic fibrosis: a 26-year experience.

We reviewed the records of all patients with cystic fibrosis and radiologically demonstrated pneumothorax at Rainbow Babies and Childrens Hospital between 1959 and 1987. There occurred 144 pneumothoraces, 71 right and 73 left, in 99 patients, 48 female and 51 male. The median survival from the date of the first pneumothorax was 29.9 months. Primary therapy included the following: observation; tube thoracostomy; tube thoracostomy with instillation of quinacrine hydrochloride, tetracycline, silver nitrate, or talc; or partial pleurectomy. Complications were minimal in each group. The surgical group did significantly better than all other groups except the group given talc. We conclude that pneumothorax is a late and ominous complication of cystic fibrosis. The primary treatment of pneumothorax should be partial pleurectomy. Talc instillation should be reserved for patients in respiratory failure who are too ill to undergo operation and for the occasional patient in whom surgical intervention fails.

Mitochondrial and myocardial performance. Response to ischemia and reperfusion.

The relationships between cardiac bioenergy metabolism and myocardial function were examined in a model of global myocardial ischemia and reperfusion. The respiratory activity of distinct populations of subsarcolemmal and interfibrillar mitochondria was correlated with max dP/dt (an index of myocardial contractility with respect to time). Max dP/dt was significantly reduced to 27% of the preischemic value following two hours of cardioplegia-protected, warm, global, ischemia in dogs during the cardiopulmonary bypass period. Reperfusion resulted in improved myocardial function such that by 60 minutes of reperfusion, max dP/dt returned to baseline. Significant declines in both state 3 respiratory rates and respiratory control indexes for subsarcolemmal and interfibrillar mitochondria were noted following the ischemic interval. Mitochondrial function similarly returned to baseline values following 60 minutes of reperfusion. These data demonstrate a close association between mitochondrial and myocardial activity.