Outcome following tricuspid valve detachment for ventricular septal defects closure.

OBJECTIVES: Detachment of the septal leaflet of the tricuspid valve from the annulus (TVD) has been used to improve visualization of ventricular septal defects (VSDs), but may be associated with increased operative time, heart block, and the development of tricuspid regurgitation (TR). METHODS: Patients undergoing VSD closure between 1/1/96 and 31/12/99 were retrospectively reviewed. Follow-up was obtained from the patients' cardiologists. RESULTS: Transatrial VSD closure was performed in 172 patients with TVD in 36 (21%) at the surgeon's discretion. The leaflet incision was repaired with a separate suture (22) or with the VSD patch suture (14). Additional procedures including arch augmentation, closure of atrial septal defects, and closure of additional VSDs were performed in 93 (68%) non-TVD patients and 20 (56%) TVD patients. The median age was 6.2 months (range 1 day to 46 years) and the median weight was 5.9 kg (range 1.5-71.5 kg). Cardiopulmonary bypass (CPB) time was 64+/-24 min and cross-clamp time was 34+/-16 min. One hospital death occurred in an infant with tracheal stenosis. No child in either group developed complete heart block. The median duration of postoperative stay was 4 days (range 2-49 days). There were no differences in CPB time, cross-clamp time or postoperative stay between the TVD and non-TVD groups (P>0.1 for all). At a mean follow-up of 17+/-15 months, there have been two late deaths unrelated to cardiac disease. No child in the TVD group required reoperation for residual VSD, compared to three in the non-TVD group. No child in the TVD group has greater than mild TR, but six in the non-TVD group have greater than mild TR. No child in either group has undergone reoperation for TR. CONCLUSIONS: TVD is a safe, effective technique to improve visualization of VSD and is not associated with heart block, increased operative time, or TR. TVD may result in improved preservation of tricuspid valve architecture and decrease the incidence of significant postoperative TR.

Ectopic automatic atrial tachycardia in children: clinical characteristics, management and follow-up.

Ectopic automatic atrial tachycardia, an uncommon type of supraventricular tachycardia in children and adults, has been reported to be resistant to medical therapy, and surgical or cryoblation has been recommended. This report describes 10 infants and children (median age 6 months; range birth to 7.5 years) with automatic atrial tachycardia and their management and follow-up. Digoxin alone was unsuccessful in controlling tachycardia in all 10 patients but decreased the tachycardia rate by 5 to 20% in 8. Intravenous (0.1 mg/kg body weight per dose) and oral propranolol successfully suppressed tachycardia in three of five patients and oral propranolol successfully controlled tachycardia in two of five other patients. Class I antiarrhythmic agents--quinidine (three patients), procainamide (four patients) and phenytoin (three patients)--did not control tachycardia in any patients but made the tachycardia rate worse in three patients. Intravenous (5 mg/kg per dose) and oral amiodarone suppressed tachycardia in three of four patients and oral amiodarone suppressed it in another patient. Thus, intravenous propranolol and amiodarone were effective in acutely suppressing automatic ectopic atrial tachycardia and predicted the response to long-term oral therapy. One patient had persistent tachycardia after surgical ablation of the high right atrial ectopic focus, and another patient had unsuccessful catheter ablation of the high right atrial ectopic focus (25 J). During follow-up (10 to 28 months), ectopic atrial tachycardia resolved completely in four patients and was well controlled in four patients.

Oncocytic cardiomyopathy of infancy with Wolff-Parkinson-White syndrome and ectopic foci causing tachydysrhythmias in children.

Two female infants, ages 6 months and 13 months, were first seen in the newborn period with supraventricular tachycardia associated with Wolff-Parkinson-White syndrome. One infant had echocardiographic and angiographic evidence of diffuse cardiomyopathy and died suddenly at home. The other infant was seen initially at 13 months of age with refractory ventricular tachycardia and died following surgical resection of arrhythmogenic foci on the left and right ventricles. Autopsy showed diffuse patchy oncocytic cardiomyopathy in both instances. Serial histologic sections of the cardiac conduction system showed oncocytic involvement of the atrioventricular (AV) node, His bundle, and bundle branches. Both infants had interruption of the anulus fibrosus by oncocytic cells at several sites, resulting in multiple accessory AV and nodoventricular connections. Additionally, patient No. 1 had an accessory AV connection by oncocytic cells in the fatty fibrous tissue of the left AV sulcus. To our knowledge, this is the first report of multiple accessory AV connections of oncocytic cells seen during histologic study. In addition, both infants had oncocytic involvement of the exocrine and endocrine glands. This report discusses the clinicopathologic correlations in these two patients, the literature on oncocytic cardiomyopathy, and the types of dysrhythmias found in these patients and their management.

Recurrent obstruction after subclavian flap repair of coarctation of the aorta in infants. Can it be predicted or prevented?

Recoarctation is a problem in some patients after subclavian flap aortoplasty. To investigate the reason for recoarctation, we reviewed the records of 26 infants who underwent subclavian flap repair for symptomatic coarctation of the aorta at less than 3 months of age between June, 1979, and December, 1983. Age at repair ranged from 2 to 65 days (median 16 days) and weight from 2.1 to 4.9 kg (median 3.4 kg). In 14 patients the coarctation was associated with significant intracardiac defects (complex in six). There were two intraoperative deaths and one early death (surgical mortality 12%). The survivors were followed from 6 weeks to 66 months (median 12 months). Five survivors (22%), all operated on at less than 14 days of age, developed severe recoarctation 6 weeks to 6 months (median 5 months) after repair. The obstruction appeared to be due to lumen obliteration by shelf-life posterior wall tissue. Morphometric analysis of preoperative angiograms showed no correlation between recoarctation and distance between the left subclavian artery and the site of coarctation, length of the isthmus, diameter of the isthmus, combined cross-sectional area of the left subclavian artery and isthmus, or the ratio of the combined cross-sectional area of the left subclavian artery and isthmus to the cross-sectional area of the descending thoracic aorta. Recoarctation did not correlate with weight at operation, but it correlated significantly with age at aortoplasty (p = 0.02). The results suggest that intrinsic abnormalities of the periductal aortic wall are responsible for recoarctation after subclavian flap aortoplasty. Particular attention to this abnormal tissue at repair may prevent early recurrence in young infants.

Aneurysm of main pulmonary artery in a neonate with airway obstruction and heart failure: long-term survival after pulmonary artery aneurysmectomy and patent ductus arteriosus ligation.

A neonate was seen with complete atelectasis of the left lung secondary to compression of the left main bronchus by a congenital aneurysmal main pulmonary artery. Operation consisted of pulmonary artery aneurysmectomy and ligation of an associated patent ductus arteriosus. Follow-up (3 years after operation) demonstrated complete resolution of the atelectasis and congestive heart failure.

Successful percutaneous balloon valvuloplasty of the aortic valve in an infant.

Percutaneous balloon aortic valvuloplasty was attempted in an eight-month-old infant with severe aortic valve stenosis. The procedure resulted in a fall in the resting transvalvular systolic pressure gradient from 106 mmHg to 40 mmHg and no aortic regurgitation. Clinical and Doppler echocardiography findings suggest persistent improved status 3 1/2 months after the procedure.

Blood pressure response to dynamic exercise in adolescents before and after percutaneous transluminal renal artery angioplasty.

The blood pressure response to maximal bicycle exercise was studied using the James protocol in two adolescents with renal artery stenosis before and after percutaneous transluminal renal artery angioplasty. Prior to angioplasty, one patient (pt A) had persistent and one patient (pt B) had labile elevation of the resting blood pressure above the 95th percentile for age; both patients had elevated systolic blood pressure at maximal exercise (pt A: 215 mm Hg; James expected 170 +/- 20; pt B: 228 mm Hg; James expected 156 +/- 15). Following angioplasty, both patients had resting blood pressures below the 95th percentile for age; patient A had improved systolic pressure at maximal exercise (195 mm Hg) while patient B had persistent elevation in the exercise systolic blood pressure (215 mm Hg). Angiography confirmed the presence of residual/recurrent renal artery stenosis in patient B. We conclude that measurement of blood pressure during exercise may reveal residual abnormalities not apparent at rest and thus may be useful in assessing the result of renal artery angioplasty in children with renal artery stenosis.

Congestive heart failure and absent femoral pulses in newborns without coarctation of the aorta.

Two infants with thrombosis of the abdominal aorta are discussed. In each case the presentation was indistinguishable from that in coarctation of the aorta, with heart failure and absent femoral pulses. Surgery in one infant successfully relieved the obstruction. The diagnosis may not be suspected from the history. Aggressive management is indicated.

Myocardial aneurysm in association with disseminated cytomegalovirus infection.

An infant with disseminated cytomegalovirus infection and apical aneurysm of the left ventricle died. At autopsy the coronary arteries were anatomically normal, but there was occlusion of the left anterior descending artery with an inflammatory lesion and corresponding organized thrombus. It seemed likely that cytomegalovirus infection acquired in utero may have induced an endothelial lesion, leading to thrombosis, occlusion, apical myocardial infarction, and eventual aneurysm formation.

Relative frequency of different types of epilepsy: a study employing the classification of the International League Against Epilepsy.

Approximately three-quarters of epileptic patients seen in consultation by specialists can easily be assigned to one of the diagnostic categories of the International League against Epilepsy. When patient age is taken into account, such a distribution shows that partial epilepsy is more common than generalized epilepsy (62% versus 38%) in patients of all ages, especially in those over 15 years of age (78% versus 22%). In contrast, generalized epilepsy is more frequent in subjects under 15 than partial epilepsy (55% versus 45%). In this younger age group the rarity of partial epilepsy is primarily due to the infrequency of temporal lobe epilepsy (21% of cases, as opposed to 56% in subjects over 15), whereas both primary and secondary generalized epilepsy contribute to the frequency of generalized epilepsy in this group.