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OBJECTIVES--A combined atrial and arterial switch procedure was performed in selected patients with congenitally corrected transposition to establish the morphological left ventricle as the systemic ventricle. Immediate and early follow up results are presented. BACKGROUND--Progressive right ventricular dysfunction and tricuspid regurgitation are common in patients with congenitally corrected transposition who undergo repair of associated lesions. A surgical procedure which re-establishes the left ventricle as the systemic ventricle should improve functional results. METHODS--Four symptomatic children aged from 9 months to 3 years 1 month (mean 2 years 3 months) with congenitally corrected transposition and ventricular septal defect underwent both an atrial and arterial switch procedure and were followed up for a mean of 12 months (range 6-21 months). RESULTS--There were no early or late deaths. Conduction abnormalities worsened in two patients. Hospital stay ranged from 8 to 17 days (mean 13 days). The cardiothoracic ratio decreased from a mean (range) of 0.65 (0.6 to 0.71) to 0.58 (0.52 to 0.6). Currently, three patients are in functional class I and one child is in functional class II. CONCLUSIONS--The combination of an atrial and an arterial switch procedure in symptomatic children with congenitally corrected transposition establishes the left ventricle as the systemic ventricle. The initial experience is encouraging with excellent immediate and early follow up results. [ABSTRACT FROM PUBLISHER]

Seventy three infants who underwent neonatal anatomical correction for transposition of the great arteries with or without a ventricular septal defect were reviewed for evidence of conduction and rhythm abnormalities on preoperative and postoperative 12 lead electrocardiograms and during 24 hour Holter monitoring. There was a partial right bundle branch block pattern in 47% (29/62) of all patients and in 60% (24/40) of those with simple transposition. Complete right bundle branch block was noted in 21% including 5% with simple transposition. Holter monitoring showed sinus rhythm in all patients except three: one had episodes of supraventricular tachycardia, another an intermittent second degree heart block, and a third a complete heart block. Atrial extrasystoles were noted in 47% (29/62) of patients but were frequent in only three patients. Occasional unifocal ventricular extrasystoles were encountered in 37% (23/62) of patients and were frequent in a further 3% (2/62). Only one patient (2%) developed multifocal ventricular extrasystoles. The frequency of important cardiac arrhythmias after neonatal anatomical correction of transposition of the great arteries was 5%, significantly less than that reported after atrial inflow diversion for the same malformation. [ABSTRACT FROM PUBLISHER]

The article presents an abstract of the study "Outcome of prenatally diagnosed hypoplastic left heart syndrome between 1994 and 1999." The study uses data of all cases of prenatally diagnosed hypoplastic left heart syndrome referred to Fetal Medicine Unit at Birmingham Women's Hospital between 1994 and 1999. The study finds that 36 out of 87 couples opted for abortion after diagnosis of hypoplastic left heart syndrome using fetal echocardiography.

Objective: The purpose of the study was two-fold: (1) to highlight the varied presentation of mediastinal tuberculous lymphadenitis (MTL) in children and (2) to identify parameters, that may help in the early diagnosis of this condition., Methods: Between January 1995 and December 2002, 13 children with histological diagnosis of MTL were retrospectively assessed for age at presentation, history of exposure to TB, presenting symptoms, investigations, initial diagnosis, surgical treatment and outcome. Stepwise multiple linear regression analysis was used to determine potential risk factors for early diagnosis of MTL., Results: Thirteen children presented with: (a) fever, night sweats and weight loss (4); (b) acute respiratory distress (2); (c) cough and shortness of breath (SOB) (5); (d) stridor (1); and (e) chest pain (1). TB was suspected only in 6 children (46%) at presentation. In the other 7 cases (54%) the presumed diagnoses were: neuroblastoma (n=1), metastatic malignancy (n=1), bronchial polyp (n=1), bronchogenic cyst (n=2), and presumed foreign body (n=2). Bronchoscopy was diagnostic in identifying cheesy material within the bronchus and organisms on lavage in 4 (30%) and in identifying external compression in 2 (15%). Thoracotomy and excision of the lymph node mass was necessary to treat the mediastinal compression and to ascertain the diagnosis of TB in 3 children (23%). All 13 children had complete resolution of tuberculous lymphadenitis following anti-tuberculous treatment. The diagnostic clues in this cohort of patients were cough and SOB with history of exposure to tuberculosis (P=0.0001) and bronchoscopy and lavage with positive staining for acid-fast bacilli (P=0.0001)., Conclusions: Tuberculosis was not suspected in 54% of children with MTL, and they posed diagnostic dilemma on admission. Bronchoscopy must be used as a diagnostic tool in children where tuberculosis cannot be excluded by radiology or specific skin tests. Thoracotomy and excision may be necessary to treat the obstructive symptoms.

Background: Classic first-stage Norwood repair of hypoplastic left heart syndrome uses a homograft patch enlargement to obtain an unobstructed aorta and coronary arteries. Because of possible disadvantages of the homograft, such as lack of growth, degeneration and calcification, and availability, we have tried to repair the aorta without patch supplementation., Methods: Between February 1993 and September 1997, 120 patients, aged birth to 47 days (median 4 days) and weighing 1.7 to 4.4 kg (median 3.1 kg), underwent first-stage palliation for hypoplastic left heart syndrome. The diameter of the ascending aorta ranged from 1.5 to 8.0 mm (median 3.0 mm). Eight patients had an aberrant right subclavian artery arising from the descending thoracic aorta. In 95 patients (group I), all duct tissue was excised and the descending aorta was anastomosed to the aortic arch, which had been opened back into the ascending aorta. Then to this confluence was anastomosed the proximal main pulmonary artery. In the remaining 25 patients (group II), continuity of the aortic arch was maintained and the repair was performed with a Damus-Kaye-Stansel anastomosis. The size of the systemic-to-pulmonary shunt was 3 mm in 48 patients, 3.5 mm in 70, and 4.0 mm in 2., Results: Circulatory arrest time ranged from 19 to 105 minutes (median 54 minutes). A homograft patch was necessary for the arch reconstruction in 18 patients (15%); 9 group I patients (10%) and 9 group II (36%) (P =.001). There were 82 hospital survivors (68%); 69 group I patients (73%) and 13 group II (52%) (P =.04), 71 patients without a patch (70%) and 11 with a patch (61%) (P >.2). By multiple logistic regression, the aberrant right subclavian artery was a significant risk factor for hospital death (P =.008). There were 6 late deaths. Sixteen of 71 patients (23%) who underwent second-stage palliation had a neoaortic arch obstruction develop, with a peak gradient greater than 10 mm Hg; 14 group I patients (23%) and 2 group II (22%) ( P >.2), 15 without a patch (23%) and 1 with a patch (17%) (P >.2). Overall survivals were 57% at 1 year and 55% at 2 years., Conclusion: The modified Norwood procedure for first-stage palliation of hypoplastic left heart syndrome is possible in the majority of patients without the use of exogenous materials and does not result in an increased incidence of neoaortic arch obstruction. Repair of the aorta without patch supplementation may improve the potential for long-term growth of the new aorta.

Division of the morphologically left atrium is a rare abnormality, constituting around 0.1% of congenital heart disease. It may coexist with other cardiac abnormalities, and this association may obscure its recognition. We report an uncommon association with tricuspid atresia and discordant ventriculo-arterial connections, the latter dominating the clinical picture. Accurate diagnosis was made by transthoracic cross-sectional echocardiography, and the patient was referred to surgery without need for cardiac catheterisation.

Background: Less than perfect coronary artery translocation accounts for the majority of perioperative deaths after the arterial switch procedure for transposition of the great arteries. Some types of coronary arterial anatomy are associated with a higher risk of death., Methods: Prospective epicardial ultrasound examination of all neonates with failing left ventricle or difficulty in weaning off cardiopulmonary bypass was performed after completion of the arterial switch operation during a 2-year period from March 1994 to February 1996. The aim was to identify any mechanical, and potentially remediable, factors accounting for ventricular failure., Results: Four neonates fulfilling the above criteria were identified during a 2-year period when epicardial echocardiography was routinely applied. In 2 patients coronary arterial problems in the form of kinking of the proximal left coronary artery (1 patient) and extrinsic compression of the artery by the neo-pulmonary trunk (1 patient) were identified and successfully corrected. In 2 other patients, supravalvar aortic stenosis was recognized, leading to prompt revision., Conclusions: Epicardial echocardiography has an important "troubleshooting" role in the subgroup of patients with a suboptimal hemodynamic result after the arterial switch operation. Patients with unusual coronary anatomy should routinely be candidates for such studies.

Reconstruction of the right ventricular outflow tract with a valved conduit is often necessary to repair some forms of congenital heart disease. We have used a 12- to 13-mm sized glutaraldehyde-preserved lamb's (ovine) valve for this purpose. The valves were harvested from the local abattoir, dissected, and stored in glutaraldehyde at 4 degrees C for 2 weeks before use. The valve was placed in four infants, two with truncus arteriosus, one with aortic valve atresia and ventricular septal defect, and one with absent pulmonary valve syndrome. In three patients, the valve had to be replaced in the second year of life, and in one patient in the fourth year of life. All patients are alive and well at follow-up. In the short-term, this valve has functioned well and could be considered where commercially available conduits or homografts are not routinely available.

In 24 consecutive infants (19 male and 5 female) with complex forms of single-ventricle physiology and systemic outflow obstruction, a modified Damus operation without the use of exogenous material was undertaken in conjunction with creation of an aortopulmonary shunt 3.5 mm in diameter. The median age at operation was 6 days (range, 1 to 170 days) and the median weight, 3.4 kg (range, 2.6 to 4.6 kg). There were nine early deaths. All 15 survivors (median follow-up, 6.5 months) were clinically well without major systemic ventricular dysfunction or atrioventricular or arterial valve regurgitation. Ten of them have undergone a superior vena cava-pulmonary shunt (one death), and 1 has required patch angioplasty of the aortic arch and innominate artery with revision of the aortopulmonary shunt. The 4 other survivors are awaiting a cavopulmonary shunt. Univariate analysis yielded the chronologic rank for an individual procedure (higher risk of death early in the series), presence of aortic arch atresia, and presence or absence of transposition of the great arteries as predictors of death. This aggressive surgical approach provides excellent early palliation, and because the operation prevents abnormal ventricular hypertrophy from pressure or volume overload, systemic ventricular function is optimally conserved for a future Fontan-type procedure.

An 8-year-old boy who suffered from Hirschsprung's disease had development of tricuspid valve endocarditis that progressed to aortic root abscess formation, development of a fistulous communication between aorta and right atrium, atrial and ventricular septal defects, and a left ventricle to right atrium defect. Several surgical procedures were required. Operation consisted initially of closure of the septal defects and aortic valve repair. This was followed by homograft replacement of the aortic valve for persistent infection, and further closure of a left ventricle to right atrium fistula.

Significant post-operative bleeding may occur following cardio-pulmonary bypass, sometimes necessitating early re-exploration. Two questions were asked by this study. Firstly, in children undergoing cardio-pulmonary bypass, can pre-operative, operative and immediately post-operative variables be used to predict post-operative blood loss? Secondly, in the same patient group, can the same variables be used to predict the surgical intervention of re-exploration for excessive bleeding within 12 h of surgery? In a prospective study of patients operated on in a 9-month period, bleeding fell rapidly in the first 3 h after surgery and early re-exploration for bleeding was necessary in 8 of the 206 patients. The only significant predictor of post-operative blood loss was minimum oesophageal temperature, low values being associated with greater blood loss (correlation r = -0.19 (P < 0.05)). With regard to re-exploration for bleeding, a long bypass time (P < 0.05) and abnormal pre-operative coagulation indices (P < 0.01) were significant and independent predictors. The data were also used to create a practical nomogram relating re-exploration for bleeding to hourly post-operative blood loss.