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There are very few published reports of the transcatheter closure of perimembranous ventricular septal defects (PMVSDs) using the Amplatzer PMVSD occluder with encouraging initial results. This report presents initial and 1-year results from 54 patients with PMVSDs who underwent transcatheter closure at 5 different institutions with the Amplatzer PMVSD occluder. Sixty-five patients with PMVSDs were enrolled at 5 European centers. Eleven of the 65 patients did not fulfill the patient selection criteria at the initial echocardiographic evaluation or at cardiac catheterization. As a result, a total of 54 patients underwent attempted transcatheter closure using the Amplatzer PMVSD occluder. The median age of the patients was 5.1+/-3.6 years (range 0.3 to 13), and the median weight 18.5+/-10.3 kg (range 5 to 45). Devices were permanently implanted in 49 of 54 patients. Complete occlusion of the communication at 1-year follow-up was observed in 46 of 49 patients (94%). Main early procedural complications included (1) device embolization (2 patients), (2) severe bradycardia with hemodynamic compromise (2 patients), and (3) Mobitz II (2:1) heart block (1 patient). Late procedural complications included complete heart block (1 patient). No other complications were observed during follow-up. In conclusion, the Amplatzer PMVSD occluder is promising device that can be used for transcatheter closure in selected patients with PMVSDs. Further studies and long-term follow-up are required before this technique enters routine clinical practice.

The lower extremities of 28 unselected children with congenital heart disease were investigated and classified according to the criteria for postthrombotic syndrome five to ten years after their first cardiac catheterization. For the clinical criteria, all patients completed a questionnaire and underwent a standardized physical examination of both legs. For the pathophysiologic criteria, the presence of venous outflow obstruction and reflux was evaluated by color duplex sonography in 24 of the 28 patients. Mild postthrombotic syndrome was present in half the patients. Partial or complete occlusion of the investigated vein was found in four patients (17%). In all patients studied, the venous valves of the deep system were competent. Postthrombotic syndrome frequently occurs in children with congenital heart disease. Prospective studies seem to be justified to investigate the precise incidence and potential risk factors.

Objective The purpose of the present study was to determine the symptomatology, diagnostic procedures, and therapeutic strategies of patients with an isolated unilateral absence of a pulmonary artery (UAPA). Background Isolated UAPA is a rare anomaly. Some case reports exist, but the best diagnostic and therapeutic approaches to these patients remain unclear. Methods A retrospective analysis was made of 108 cases reported between 1978 and 2000. The database of the National Library of Medicine (MEDLINE) was used to identify cases that were published in any language from 1978 onward. Results Of the 108 patients identified, 14 were asymptomatic. The median age was 14 years (range, 0.1 to 58 years). Most patients had symptoms such as frequent pulmonary infections (37%), dyspnea or limited exercise tolerance (40%), or hemoptysis (20%). Pulmonary hypertension was present in 44% of the patients. Surgical procedures were performed in 17% of patients, and the overall mortality rate was 7%. Conclusion Only a few patients with isolated UAPA remain asymptomatic during follow-up. The diagnosis can be made by chest radiograph, echocardiography, CT scan, and MRI. Hilar arteries can be shown by cardiac catheterization and pulmonary venous wedge angiography. This is important since revascularization may improve pulmonary hypertension. The avoidance of high altitudes and pregnancy may further improve outcomes.

In clinical treatment of children with congenital heart disease (CHD) assessment of right ventricular (RV) function is important. Available imaging techniques have been of limited value because of technical factors and the complex geometry of the right ventricle. To validate magnetic resonance (MR) imaging measurements of RV function in children, gradient echo MR imaging of both ventricles and MR flow mapping of great vessel and tricuspid flow was performed in 20 children with CHD affecting the right ventricle and in 22 healthy children ranging in age from 5 to 16 years. Close correlation between RV versus LV stroke volumes ( r = 0.96) and RV stroke volume versus great artery ( r = 0.97) or tricuspid flow ( r = 0.97) was observed with small interobserver and intraobserver variability. Results of healthy children were end-diastolic volume: 70 ± 9 ml/m 2 , end-systolic volume: 21 ± 5 ml/m 2 , and ejection fraction: 70% ± 4%. In the patient groups clinically important differences were noted. We conclude that MR imaging provides accurate noninvasive measurements of RV function in healthy children and patients with (operated) CHD.

Few data exists on the differences in long-term outcome between Mustard and Senning operations. We reviewed available data of all hospital survivors of these operations and assessed risk factors for late death and sinus node dysfunction. Of those patients undergoing the Mustard operation, 60 were hospital survivors (46 simple transposition, 14 complex); of those patients undergoing the Senning operation, 62 were hospital survivors (43 simple, 19 complex). Median duration of follow-up was 16 years (maximum 25 years) for Mustard operation, 11 years (maximum 20 years) for Senning operation. No reoperations were done, except for pacemaker implantation. No differences were found between the two groups with regard to baffle-associated problems, right ventricular failure, sudden death (6% in both groups), and functional status at final follow-up (New York Heart Association class I or II, except for four patients). For patients undergoing the Mustard operation, survival at 16-year follow-up was 91% with simple transposition and 60% with complex transposition ( p = 0.027); for both groups of patients undergoing the Senning operation, survival at 16-year follow-up was 78%. Survival in the absence of rhythm disturbance at 16-year follow-up was 18% for Mustard operation and 53% for Senning operation ( p

Aortic valve insufficiency has been described to occur after the arterial switch operation. Valve insufficiency is mild in most cases. In some patients valve repair or root reconstruction may solve the problem, but in others the valve will have to be replaced. Inasmuch as the number of patients surviving the arterial switch operation is growing, valve insufficiency may be seen more often. In infants the ideal way to replace the aortic valve is by using a pulmonary autograft. Our experience with aortic valve replacement using a pulmonary autograft (former aortic valve) in one infant with a history of arterial switch operation is described. Clinical summary. A little girl, 3 years 4 months old, was referred to us from another institution. At the age of 2 days an arterial switch operation was performed for simple transposition of the great arteries. The coronary anatomy was normal (1LCx-2R, Leiden classification). 1 The aorta was anterior to the pulmonary artery. Both semilunar valves were tricuspid and normal. The coronary arteries were excised together with U-shaped aortic wall buttons and implanted in the pulmonary artery base by means of the trapdoor technique. The neopulmonary root was reconstructed with a pantaloon-shaped patch of autologous pericardium. A Lecompte maneuver was part of the procedure. The postoperative course was complicated by ischemia, frequent ventricular extrasystoles, and periods of ventricular fibrillation. Left hemidiaphragmatic paresis necessitated later diaphragmatic plication. After an extended period of intensive care, she gradually recovered and finally was discharged from the hospital. Progressive dilatation of the aortic root with valvular insufficiency was observed during follow-up. Insufficiency was considered to be significant (2/4) when she was 1 year old. The left ventricle was dilated. Her condition deteriorated gradually, leading to fatigue on slight exertion.

A case is reported in which is described a modification of staged septation for univentricular atrioventricular connection with double-inlet left ventricle and left-sided rudimentary outlet chamber beneath the aorta. This modification diminishes the risk of atrioventricular conduction disturbances by gluing the perforated septation patch on the myocardium in the area overlying the central conduction system. Fixation of the patch was facilitated by exact fitting of the patch in this area, by ample contact of the patch and the myocardium in this area, and by immobilization of the patch because of equal pressures on both sides because of the perforation of the patch. In addition, this modification facilitates evenly drawing in of the ventricular free wall because already, in the first stage, the complete circumference of the patch is fixed, defining the maximal contribution of prosthetic material to the newly created septum.

In 10 out of 31 cases of tricuspid atresia (absent atrioventricular connexion), gross examination of the morphologically right ventricle suggested the presence of a tiny inlet portion in addition to the trabecular portion. Serial sections of two of these hearts have proven this diagnosis: a fibrous remnant in continuity with the central fibrous body was present not only above the left ventricle but also above the right ventricle. We interpreted this as a remnant of the atrioventricular valve and we also observed that the ventricle had a hypoplastic inlet portion. This inlet portion was bounded by a microscopically small septomarginal trabeculation containing the right bundle branch. Therefore, the diagnosis of absent connexion, which after clinical or gross anatomical investigation remains an appropriate one, cannot always be maintained after microscopical study if it is considered that the fibrous remnant represents evidence of a connexion initially present between the right ventricle and the right atrium.

In atrioventricular (AV) septal defect, the architecture of the left ventricular (LV) outflow tract differs consistently from the normal tract. In patients with separate AV valves and in those with a common valve with septal attachment of the superior bridging leaflet (Rastelli type A), the LV outflow tract below the anteriorly positioned aorta is long and narrow. Possible obstruction of this outflow tract has been attributed to abnormal papillary muscles 1,2 and to an "atrial fold" that is the anterior wall of the left atrium reinforcing the aortic-left AV fibrous continuity. 3 Less known is the anterolateral muscle bundle, 4 often very prominent in AV septal defect. 5 This muscle bundle that replaces the left coronary-AV fibrous continuity could well contribute to a possible stenosis of the LV outflow tract. We carried out Doppler echocardiography and magnetic resonance imaging studies to evaluate the role of the anterolateral muscle bundle in the possible systolic narrowing of the subaortic outflow tract.

An anatomical study of 34 specimens presenting with tricuspid atresia, in which particular emphasis was devoted to the atrioventricular junctional area, revealed 5 cases with an imperforate valve. In 1 heart the valve was completely committed to the right ventricle. In the other 4, however, the membrane was related not only to the right ventricle but also to the left ventricle. A hypoplastic right ventricular inlet portion was found in these 4 specimens. In 2 cases the tricuspid membrane overrode an inconspicuous inlet septal defect. In the other 2 hearts, as studied macroscopically, no defect was present underneath the membrane, which was connected to both right and left ventricles. These 4 specimens also had other ventricular septal defects. In classical tricuspid atresia, characterized by absence of the inlet portion of the right ventricle, the inlet septum is absent. In contrast, these 4 hearts did have an hypoplastic inlet septum.

Sixty-six patients (23 neonates with transposition of the great arteries and intact ventricular septum, 33 infants and children with transposition and a large ventricular septal defect, and 10 with double-outlet right ventricle with a subpulmonary ventricular septal defect have received an arterial switch repair since 1977. Eight (one with transposition and intact ventricular septum, six with transposition and ventricular septal defect, and one with double-outlet right ventricle with subpulmonary ventricular septal defect) died in the hospital. All other patients have had follow-up as of June, 1985. Including the hospital deaths, the 11 month actuarial survival rate for the entire group was 81%, and no deaths have occurred among the 33 patients alive at that time and traced as long as 8 years. The hazard function for death has only a single early phase, and its 70% confidence limits overlap the hazard function of a matched general population by 12 months after the operation. Incremental risk factors for death included low birth weight (but not weight or age at operation), transposition of the great arteries with large ventricular septal defect, double-outlet right ventricle with subpulmonary ventricular septal defect, and presence of a patent ductus arteriosus. Coronary artery morphology and position of the great arteries were not risk factors. Long aortic cross-clamp time was possibly (p = 0.11) a risk factor. Early date of operation was a risk factor (p = 0.004); thus, predicted 1 year survival rate, including hospital deaths, after the arterial switch operation in 1985 is 99.9% (70% confidence limits 99.0%-100%) for neonates with transposition and intact ventricular septum and 99.7% (98.4%-99.9%) for those with transposition and a large ventricular septal defect or double-outlet right ventricle. The late functional status was excellent, and the rhythm was sinus in 96% of the 55 surviving patients. A formal comparison with the results of the atrial switch repair indicates that the arterial switch repair is superior.

An anatomic study of 28 heart specimens with tricuspid atresia showed 15 hearts with ventriculoarterial concordance and a right-sided anterior outlet chamber. Of these 15 hearts, 12 showed evidence of restricted pulmonary blood flow. In eight of these 12 specimens, an anterior deviation of the outlet septum, relative to the primary septum, was present. This deviation caused a narrowing inside the outlet chamber and functioned as infundibular pulmonary stenosis. The outlet chambers of the 15 hearts with ventriculoarterial concordance were compared with those of 13 hearts with tricuspid atresia and ventriculoarterial discordance and also a right-sided anterior outlet chamber. In the latter group of 13 hearts, five showed evidence of restricted pulmonary blood flow, at least partially caused by posterior deviation of the outlet septum relative to the primary septum. A clear difference in outlet chamber morphology was found without exception between specimens with and without ventriculoarterial concordance. Clinical data in three long-term survivors of the Fontan procedure demonstrate that the special morphology of the outlet chamber can pose surgical problems. Especially in patients with ventriculoarterial concordance, when the outlet chamber is incorporated into the pulmonary circuit, the surgeon must give special attention to the specific morphology to prevent undesirable sequelae.