Role of ischemia and infarction in late right ventricular dysfunction after atrial repair of transposition of the great arteries.

OBJECTIVES: This study was conducted to assess whether myocardial ischemia and/or infarction are involved in the pathogenesis of late right ventricular dysfunction in adult survivors of atrial baffle repair for transposition of the great arteries in infancy. BACKGROUND: The medium-term success of intraatrial baffle repair for transposition of the great arteries is good, with many patients surviving into adult life, but prognosis can be limited by progressive right ventricular dysfunction. We hypothesized that ongoing myocardial ischemia and/or infarction are important factors in the pathogenesis of this complication. Radionuclide techniques offer an opportunity to study both myocardial perfusion and concomitant ventricular wall motion. METHODS: Dipyridamole sestamibi single-photon emission computed tomography followed by rest sestamibi single-photon emission computed tomography was used to assess right ventricular myocardial perfusion, wall motion, wall thickening and ejection fraction in 22 adolescents/young adults who had undergone atrial baffle repair for simple transposition of the great arteries at median 6.7 (range 0.5 to 54) months of age. The patients were aged 10 to 25 (median 15.5) years; 19 in New York Heart Association class I, 2 in class II and 1 in class III. All were in a regular cardiac rhythm during the studies. The right ventricular tomographic images were examined in three parallel and two orthogonal planes, analyzed in 12 segments. RESULTS: Perfusion defects were evident in all patients in at least one segment, in either the rest or stress images. Twelve patients (55%) demonstrated fixed defects only, nine (41%) had fixed and reversible defects and one (4.5%) had reversible defects only. Concomitant wall-thickening abnormalities occurred in 83% of segments with fixed perfusion defects, mirrored by a reduction in wall motion in 91% of segments analyzed. Right ventricular ejection fraction was correlated with age (R = 0.62; p = 0.002), and with wall-thickening abnormalities (R = 0.60; p < 0.005). CONCLUSIONS: Reversible and fixed perfusion defects with concordant regional wall motion abnormalities occur in the right (systemic) ventricle 10 to 20 years after Mustard repair for transposition of the great arteries; this may be important in the pathogenesis of late right ventricular dysfunction in this group.

Minimally invasive management of transposition of the great arteries in the newborn period.

This study reports on a predominantly noninvasive management program for neonatal transposition of the great arteries [TGA] incorporating balloon atrial septostomy [BAS] under echocardiographic control. BAS was performed in 25 consecutive patients presenting with TGA between April 1988 and April 1990. Structural and coronary anatomy was evaluated echocardiographically with angiographic supplementation only when additional data were required. This information was correlated, where possible, with direct anatomic findings and subsequent course. BAS was performed through the umbilicus in 17 patients (85% of patients in whom this approach was attempted). Thirteen patients did not require ventilation during BAS. There were minimal complications and satisfactory septostomies in all cases. Coronary anatomy was correctly predicted in all patients where anatomic correlation was available. Without invasive investigation 9 patients underwent neonatal arterial switch procedures and 2 underwent palliative procedures. BAS under echocardiographic control proved safe, effective, minimally traumatic and mostly possible via the umbilical vein. The umbilical vein, where patent, permitted rapid safe access for BAS. Echocardiographic diagnosis of the coronary artery, and structural and functional anatomy was reliable and allowed minimally invasive preoperative management in many patients.

Right ventricular dysfunction in congenitally corrected transposition of the great arteries.

Although right ventricular (RV) dysfunction is an important complication in subjects with congenitally corrected transposition of the great arteries, its pathogenesis is poorly understood. We assessed the role of RV myocardial perfusion and found perfusion defects at rest in all 20 patients, involving 4.6 +/- 2.3 of a total of 12 segments; the extent of the resting perfusion defects correlated inversely with the RV ejection fraction.

Primary left ventricular rhabdomyosarcoma in a child: noninvasive assessment and successful resection of a rare tumor.

Rhabdomyosarcoma of the heart is a rare tumor, especially in childhood and particularly in the left ventricle. A primary tumor of this type was successfully resected after echocardiographic assessment alone in a young girl exposed prenatally to diphenylhydantoin. The patient is well and free of recurrence 2 1/2 years later.

Simplified single patch technique for the repair of atrioventricular septal defect.

OBJECTIVE: Because of the complexity of traditional 1- and 2-patch techniques for the repair of complete atrioventricular septal defect, we modified our repair technique to avoid the use of any ventricular septal patch material. We report our prospective experience with this simplified 1-patch technique. METHOD: Forty-seven consecutive patients between May 1995 and August 1998 underwent repair with the use of this technique without modification. Repair was done in all patients by direct suturing of the common atrioventricular valve leaflets to the crest of the ventricular septum. No division of valve leaflets was necessary. A single pericardial patch was used to close the defect in the atrial septal component. Follow-up included electrocardiography and echocardiographic assessment of ventricular function, atrioventricular valve function, and adequacy of the left ventricular outflow tract. RESULTS: There were 2 deaths (4%), only 1 cardiac related, in the series. There were 17 male patients and 30 female patients. Mean age at repair was 5.6 months (median, 3.4 months). Associated lesions were repaired in 19 patients (40%). Mean follow-up was 1.85 years (median, 1.9 years). There was no heart block. There were no significant residual ventricular septal defects detected and no left ventricular outflow tract obstruction seen on echocardiography in any patient to date. Mitral valve status after operation was assessed as no incompetence in 13 patients (28%), minimal in 19 patients (40%), mild in 12 patients (26%), and moderate in 3 patients (6%). CONCLUSION: The repair of complete atrioventricular septal defect by direct suturing of the atrioventricular valve leaflets to the crest of the ventricular septum with a single-patch technique greatly simplifies the repair and does not lead to left ventricular outflow tract obstruction nor interfere with valve function.

Myocardial perfusion defects and associated systemic ventricular dysfunction in congenitally corrected transposition of the great arteries.

BACKGROUND: Patients with systemic ventricles of right ventricular morphology are at high risk of contractile dysfunction, the cause of which has not been fully elucidated. OBJECTIVE: To assess whether ischaemia or infarction contributes to ventricular impairment in unoperated patients with uncomplicated congenitally corrected transposition of the great arteries (TGA) by studying myocardial perfusion and function. SETTING: Paediatric and adult congenital cardiac clinics of a tertiary referral centre. PATIENTS: Five patients with congenitally corrected TGA but without associated structural cardiac defects (aged 3.5 to 34 years). INTERVENTIONS: Maximal exercise stress testing using standard or modified Bruce protocols. Sestamibi (technetium-99m methoxy isobutyl isonitrile) scanning after isotope injection at maximal exercise and rest. MAIN OUTCOME MEASURES: Maximum exercise capacity; right ventricular myocardial perfusion, regional wall motion, and thickening; right ventricular ejection fraction. RESULTS: The two youngest patients (3.5 and 11 years) had normal exercise capacity for age, while the others had reduced exercise performance. Sestamibi scanning showed reversible myocardial ischaemia in four patients and fixed defects indicating infarction in five. Irreversible defects were mostly associated with impaired wall motion and thickening. The ejection fraction was normal (65%) in the youngest patient but < 55% in the others (mean (SD) 47(11)%). CONCLUSIONS: Patients with unoperated congenitally corrected TGA have a high prevalence of myocardial perfusion defects, with consequent abnormalities of regional wall motion and thickening, and impaired ventricular contractility. These data suggest that ischaemia and infarction are important in the pathogenesis of ventricular failure in this condition.

The surgical management of aortopulmonary window using the anterior sandwich patch closure technique.

Between 1969 and June, 1986, 13 patients with aortopulmonary window were evaluated and operated upon with eleven survivors. All were children with ages ranging from 2 weeks to 2 1/2 years and had a typical aortopulmonary window (Type 1) with a connection between the ascending aorta and main pulmonary artery. Six of them also had associated cardiac anomalies. Operative techniques included both closed and open procedures. Simple ligation was carried out in two, while the remaining 11 patients were operated upon with the aid of extracorporeal circulation. "Sandwich" patch closure was the preferred method which was employed in 7 patients.

Cardiac failure in infancy and childhood.

Cardiac failure in infants and children frequently results from pathophysiological mechanisms different from those of cardiac failure in adults. An age-based approach to aetiology is presented and attention drawn to details of management which should allow a high rate of salvage of patients who have reversible or correctable underlying conditions.

Intravenous verapamil in the treatment of paroxysmal supraventricular tachycardia in children.

Fifty-four spontaneous episodes of paroxysmal supraventricular tachycardia (PSVT) in 23 patients, varying in age from one day to fourteen years, were treated with intravenous verapamil according to a specific protocol. Stable sinus rhythm was obtained promptly with no side effects in 76% of episodes. There was no response in 9%; an unstable rhythm in 7.5%; and severe side effects (hypotension, sinus bradycardia and cardio-respiratory arrest) in 7.5%. All severe side effects were associated with larger than recommended doses of verapamil. Intravenous verapamil reverted 100% of all episodes of idiopathic PSVT in eight patients; 83% of episodes in five patients with congenital heart disease; and only 57% of episodes in nine patients with Wolff-Parkinson-White syndrome. It was ineffective in one neonate who had had intra-uterine SVT.

Late aortic root dilatation in tetralogy of Fallot may be prevented by early repair in infancy.

The objectives of this study were to examine the relative contributions of development and hemodynamics in aortic root dilatation of tetralogy of Fallot, to assess the impact of systemic to pulmonary artery shunt on aortic annular size, and to seek any relationship between the timing of corrective surgery and subsequent aortic root size. We performed a retrospective analytical study at a tertiary referral center of M-mode and two-dimensionol aortic root measurements in children with tetralogy of Fallot prior to any surgical or palliative intervention, after insertion of a surgical shunt, and on intermediate and long-term follow-up post-repair. The main outcome measures were aortic root diameter prior to correction, after palliative shunt insertion, and after definitive repair. The study found that the aortic root is enlarged in unrepaired tetralogy of Fallot irrespective of age, increased in the first 3-6 months after palliative shunt surgery, and persisted into adulthood in those repaired after 1 year of age. Age-corrected aortic root dimensions normalized by mid-childhood in those who were repaired in infancy. Preexisting aortic root dilatation normalizes by 7 years of age in patients with tetralogy of Fallot who have been repaired in infancy, whereas it persists into adulthood in the group repaired post-infancy.

Infective endocarditis in childhood.

A total of 37 cases of infective endocarditis, in children aged two days to 13 years, were seen from 1971 to 1983 at the Children's Hospital, Camperdown. Structural heart disease was known to preexist in 95%. Cyanotic congenital heart disease was present in 15 children, of whom 11 had been palliated by a systemic-to-pulmonary artery shunt. In five children, there was infection of a prosthetic valve. Identifiable precipitants of infection were rare. In particular, no child had had a preceding dental procedure within three months, while only 19% had had recent cardiac surgery. Organisms were identified by blood culture in 92%, the majority being streptococci (43% of cases) and staphylococci (27% of cases). While 59% of all organisms were penicillin sensitive, six of the seven early postoperative children were infected with organisms not sensitive to antibiotics, given for wound infection prophylaxis in the perioperative period. The overall death rate was 13.5%. Of the children infected with Staphylococcus aureus, 40% died, while no child with Streptococcus viridans endocarditis died. Of the survivors, four needed urgent valve replacement, and three had major central nervous system complications. Using these figures, a current profile of patients with infective endocarditis is presented.

Radiofrequency catheter ablation of right ventricular outflow tract tachycardia in a preschooler.

A 3-year-old girl presented with a febrile illness complicated by right ventricular outflow tract tachycardia, which persisted after resolution of the presumed viral infection. The tachycardia was intermittent, but was significantly exacerbated by exercise. Radiofrequency ablation (RFA) of the ectopic focus was successfully performed and the child remains free of tachycardia 12 months later. A review of the literature suggests that RFA in children is increasingly seen as a safe and convenient option for the treatment of supraventricular tachycardia, whereas the management of ectopic ventricular tachycardia in pediatric practice has not been well defined.

Neonatal ductus arteriosus aneurysm causing nerve palsies and airway compression: surgical treatment by decompression without excision.

A 4-kg male child, born at 34 weeks to a gestational diabetic mother, had a large ductus arteriosus aneurysm causing phrenic and recurrent laryngeal nerve palsies and large airway compression. The right and left atrial appendages and distal descending aorta were cannulated, allowing left heart partial or complete cardiopulmonary bypass as necessary. On bypass the ductus was ligated, decompressed, and oversewn but not excised. Examination 1 month later suggested resolution of the recurrent laryngeal palsy and echocardiography showed regression of the aneurysm. Ductus ligation and decompression was an effective surgical treatment, which is less likely to cause complications than resection.

Generalized arterial calcification of infancy: three case reports, including spontaneous regression with long-term survival.

Generalized arterial calcification in infancy is a rare disorder in which death usually occurs in infancy, the diagnosis generally being made at autopsy. Three patients are reported. The diagnosis was made during life in two, enabling new information to be collected. Cardiac catheterization in one provided evidence suggesting stiffness of pulmonary and systemic arterial walls. Another is a long-term survivor with spontaneous regression of calcification. The third case, diagnosed at necropsy, was associated with endocardial fibroelastosis.

Indicator dilution curves in the diagnosis of D-transposition of the great arteries in infancy.

Paired right and left atrial indicator dilution curves performed in a series of 17 patients with D-transposition of the great arteries (D-TGA) were reviewed. Taken together, the patterns obtained from patients who had trivial mixing between the pulmonary and systemic circulations were distinctive and easily recognizable. In the presence of substantial degrees of intracardiac mixing, however, the paired curves recorded become virtually identical and cannot be distinguished from curves recorded from patients with other forms of cyanotic congenital heart disease characterized by obligatory admixture. Thus paired atrial dye curves demonstrate a high degree of diagnostic specificity for the infant with D-TGA at greatest risk, normally those with a small intracardiac shunt. Such a procedure should facilitate early balloon atrial septostomy and obviate deterioration in the infant's condition due to prolonged catheter manipulation and/or angiography.

Angiographic assessment of anomalous origin of the left coronary from the pulmonary artery in infancy and childhood.

Angiographic findings in five paediatric patients with anomalous left coronary artery arising from the pulmonary artery are presented. An attempt is made to provide an angiographic rationale of some aspects of the pathophysiology and electrocardiography in this condition, and the vital importance of angiography in selecting the appropriate surgical approach is underlined.

Accessory mitral valve tissue causing left ventricular outflow tract obstruction.

Although left ventricular outflow tract obstruction is commonly associated with congenitally corrected transposition of the great vessels, this obstruction is seldom caused by accessory mitral valve tissue. Three cases in which accessory mitral valve tissue caused left ventricular outflow tract obstruction in children are described. Two had congenitally corrected transposition and one had normally connected great vessels. The accessory leaflet tissue, which was identified by echocardiography and angiography, was attached by chordae tendineae to normally sited papillary muscles and herniated into the left ventricular outflow tract during systole. Operation was successful in these patients. The accessory valve tissue was excised via an arteriotomy in the great vessel that arose from the left ventricle. The obstructive tissue was excised close to its peripheral attachments in the outflow tract and its chordae tendineae were divided. Resection was performed without injury to the abnormally placed conduction system or to the normal valve structures.