Growth after neonatal arterial switch operation for D-transposition of the great arteries.

The objective of this study was to evaluate growth in infants with d-transposition of the great arteries (d-TGA) who underwent switch operation (anatomical correction) in the early neonatal period. Growth data (at birth and 1, 3, 6, 12, and 24 months of age) were retrieved in 23 infants with d-TGA who survived the operation without major complications. Measures were transformed into z scores and compared with normative data for age. Following surgery, infants showed low z scores for weight, height, and head circumference. Weight and height showed a trend to reach normal ranges between 6 and 12 months of age, and the z scores were positive at 24 months. z scores for head circumference were still negative at 24 months of age. No dietary interventions were implemented during this period, and the infants were fed their own mothers' milk or, before discharge, high-calorie, preterm formula. Following anatomical correction for d-TGA in the early neonatal period, infant growth shows a characteristic pattern, with parameters normalized by 12 months of age in most cases. Head growth shows a different pattern, although gross motor development seems unaffected.

Transcatheter closure of atrial septal defects with the STARFlex device: early results and follow-up.

BACKGROUND: The STARFlex (SF) device is a CardioSEAL (CS) double-umbrella device that has been modified by the addition of a self-centering mechanism comprised of nitinol springs connected between the two umbrellas and a flexible core wire with a pin-pivoting connection. This paper compares the results of atrial septal defect (ASD) closure with CardioSEAL and STARFlex devices. PATIENTS AND METHODS: Between December 1996 and March 2000, 117 patients underwent ASD closure with CardioSEAL (n = 79) and STARFlex (n = 38). The mean age (17 years), weight (49 kg), and ASD size (15 mm) were similar in the two groups. The procedures were performed under general anesthesia with both fluoroscopic and transesophageal echocardiographic monitoring. IMMEDIATE RESULTS: The devices were successfully implanted in all patients. Ten patients had multiple ASDs. A single device was used in four patients (CardioSEAL in three, STARFlex in one), while a simultaneous placement of two CardioSEAL (one patient) or two STARFlex (five patients) were performed in six patients. The results are summarized as follows: [table: see text] Follow-Up Results: On follow-up clinical exam, electrocardiograms, chest X-rays, and echocardiograms were obtained at 1, 6, and 12 months. [table: see text] During follow-up there were no deaths, endocarditis, rhythm disturbances, or other complications. Arm fractures were observed almost exclusively with large CardioSEAL devices (40 mm, less frequently with 33 mm), and only in one 33-mm STARFlex device. There were no clinical complications related to fractures. CONCLUSIONS: The STARFlex device seems to offer better results than the CardioSEAL, with significantly lower rates of residual shunts and arm fractures.

Interatrial septal aneurysm and genetic prothrombotic disorders: possible interaction in the pathogenesis of pediatric stroke.

Stroke in children is a rare condition and has a multifactorial etiology. The association between ischemic stroke in young adults and some minor cardiac abnormalities such as atrial septal aneurysm with or without interatrial shunting has recently been reported: however, the pathogenetic mechanism still remains unclear. Genetic and acquired prothrombotic disorders are also risk factors for cerebral ischemic events in children. We report a case of ischemic stroke in a 10-year-old female child who was heterozygous for the prothrombin G20210A variant and who presented with an atrial septal aneurysm associated with an interatrial shunt. We hypothesize that these risk factors play a synergic role but their relative importance and whether alone they can determine cerebral embolism remain to be determined.

[Timely detection of premature closure of the ductus arteriosus in a full-term fetus. Important role of fetal echocardiography]. / Tempestivo riconoscimento della chiusura prematura del dotto di Botallo in feto a termine. Ruolo cruciale dell'ecocardiografia fetale.

Patency of the ductus arteriosus (DA) is maintained during gestation by locally produced and circulating prostaglandins (PGE's). As gestation proceeds, the ductus becomes less sensitive to dilating prostaglandins and more sensitive to constricting factors such as PGE's synthetase inhibitors. This case report describes a fetus at term (38 weeks) with signs of severe right ventricular failure due to constriction of DA. Maternal history documented 5 day assumption of a non-steroid antiinflammatory agent to relieve skeletal-muscle pain. Careful echocardiogram ruled out a structural heart disease, such as coarctation of the aorta. A gradient of 41 mmHg across the ductus was recorded. A cesarean section delivery was immediately undertaken. The 3.5 kg newborn delivered appeared to be in good health, with Apgar score of 8/9 at 1 and 5'. There were no signs of congestive heart failure and mild respiratory distress. An echocardiogram showed a dilated, well contractile right ventricle, with a pressure of 50 mmHg. DA was already closed. The fetal echocardiogram was the most relevant investigation in the decision-making process of this case treatment. Any different evaluation of this fetal heart, delaying the delivery would have very seriously compromised the survival of the fetus. Fetal echocardiography is the most important diagnostic tool in the evaluation of the fetal heart; non steroid antiinflammatory drugs to mother at term should be avoided or given with close echocardiographic assessment of DA patency.

[Omniplane transesophageal echocardiography: an improvement in the monitoring of percutaneous pulmonary valvuloplasty]. / L'ecocardiografia transesofagea omniplana: un'evoluzione nel monitoraggio della valvuloplastica polmonare percutanea.

A 16 year old girl underwent percutaneous balloon dilation of the pulmonary valve. The procedure was carried out in the catheterization laboratory under transesophageal echocardiographic guidance with the patient anesthetized (ketamine) without endotracheal intubation. A 64 elements phased array "omniplane" TEE probe connected to a Sonos 1500 Hewlett-Packard system was used. The transducer assembly encased within the tip of the endoscope can be rotated through a 180 degrees arc counterclockwise and clockwise, thus obtaining a multitude of imaging planes. The right ventricular outflow tract and pulmonary valve could be visualized very well and therefore monitored accurately during all phases of the procedure. In the future this technique will be able to reduce the need for X-ray exposure and dye injection during percutaneous pulmonary valvuloplasty especially in cases in which their use could be contraindicated.

[A transesophageal Doppler echo-color study of pulmonary venous flow before and after the correction of valvular defects]. / Studio mediante eco-color Doppler transesofageo del flusso venoso polmonare prima e dopo correzione di vizi valvolari.

AIM OF THE STUDY: To verify changes of pulmonary venous flow pattern before and after surgical or percutaneous correction of valvular heart disease. METHODS: The pulmonary venous flow pattern was studied by transesophageal echocardiography in 27 patients affected with heart valve disease (11 mitral insufficiency, 10 mitral stenosis, 2 aortic stenosis and 4 pulmonary stenosis), before and after surgical or percutaneous correction. Pulmonary venous flow velocity variables measured included peak systolic and diastolic flow velocities (VmaxS and VmaxD), systolic and diastolic velocity time integrals (IS and ID) and their respective ratios (VmaxS/VmaxD and IS/ID). Paired Student's t-test was used for analysis of data; a p value < 0.05 was considered statistically significant. RESULTS: In mitral stenosis and insufficiency, as well as in pulmonary stenosis, the VmaxS/VmaxD and IS/ID ratios were constantly < 1. Aortic stenosis, on the contrary, showed a normal preoperative pattern of pulmonary venous flow, which did not change after correction. All other successful corrections (17 surgeries, 4 angioplasties) were characterised by an increase of VmaxS/VmaxD and IS/ID ratios. (Mitral stenosis: VmaxS/VmaxD 0.80 +/- 0.31 vs 1.4 +/- 0.5, p = 0.006; IS/ID 0.86 +/- 0.77 vs 1.62 +/- 0.62, p = 0.016. Severe mitral insufficiency: VmaxS/VmaxD -0.71 +/- 0.32 vs 1.19 +/- 0.32, p < 0.0001; IS/ID 0.41 +/- 0.19 vs 1.04 +/- 0.31, p = 0.006. Moderate mitral insufficiency: VmaxS/Vmax D 0.38 +/- 0.04 vs 0.95 +/- 0.06, p = 0.001; IS/ID 0.32 +/- 0.05 vs 0.95 +/- 0.07, p = 0.02. Pulmonary stenosis: VmaxS/VmaxD 0.43 +/- 0.23 vs 1.09 +/- 0.35, n.s. e IS/ID 0.49 +/- 0.34 vs 0.92 +/- 0.65, n.s.). Failure to return to a normal pulmonary venous pattern was observed in the 2 cases of partially successful mitral valvuloplasty (one of which was subsequently transformed into a mitral valve replacement with immediate normalisation of the pattern) and in the 2 cases of incomplete relief of a pulmonary stenosis after pulmonary valvuloplasty. CONCLUSIONS: Though preliminary, these observations suggest a high sensitivity of this method and, therefore, a possible role of pulmonary venous pattern studies in the assessment of the efficacy of treatment in mitral and pulmonary valve disease.

Usefulness of transesophageal echocardiography in the pediatric catheterization laboratory.

Transesophageal echocardiography was performed in 51 children (aged 2 to 14 years, mean 4; weight 9 to 50 kg, mean 21) undergoing elective diagnostic or therapeutic cardiac catheterization. The interventional procedures were percutaneous balloon dilation of pulmonary (n = 8) and aortic (n = 2) valve stenosis, percutaneous closure of patent ductus arteriosus (n = 8), and attempted occlusion of Pott's anastomosis by the double umbrella device (n = 1). The diagnostic catheterizations were performed on preoperative children of whom 5 had undergone previous palliative procedures. Precise placement of the balloon across the valve, timing of balloon inflation and deflation according to real-time monitoring of ventricular function and immediate evaluation of results and complications were accomplished with transesophageal monitoring. The exact position of distal and proximal umbrellas of patent ductus occlusive devices was checked on transesophageal imaging and completeness of occlusion controlled on color Doppler. The only relevant information in the preoperative cases was the detection of a septic thrombus in a severely ill patient. With more experience and smaller probes, transesophageal echocardiography may become a new method of monitoring cardiac catheterization also in smaller children where it may reduce duration of the procedure and amount of contrast material.

Late left atrioventricular valve insufficiency after repair of partial atrioventricular septal defects: anatomical and surgical determinants.

Risk factors for late left atrioventricular (AV) valve insufficiency, which occurred in 16 (18%) of 90 patients evaluated after repair of partial AV septal defect, were examined. The operative findings in 9 patients undergoing reoperation were also examined. Preoperative left AV valve insufficiency was significantly more common in the group with late left AV valve incompetence, as were associated valvular malformations as a whole and fenestrations of valve leaflets in particular. Conversely, the higher incidence of malformed or malpositioned papillary muscles, accessory clefts, and double-orifice left AV valves in the group with late left AV valve insufficiency did not reach significance. The method of surgical treatment of the septal commissure was not a significant factor. In the group having reoperation, additional valvular malformations were found in association with inappropriate treatment of the septal commissure in 7 patients. The 2 remaining patients had either a directly sutured ostium primum or dilatation of the annulus. Three re-repairs were successful. Five patients required prosthetic valve replacement. Preoperative left AV valve insufficiency and associated valvular malformations are major determinants of late left AV valve insufficiency in partial AV septal defect.

[Anomalous origin of the left coronary artery from the aorta. The surgical implications in a case]. / Origine anomala dell'arteria coronaria sinistra dall'aorta. Implicazioni chirurgiche in un caso.

A case of high take-off of the left coronary artery from the aorta in a patient with a large ventricular septal defect (VSD) is described. The preoperative detection of this coronary anomaly, enabled the surgeon to modify the technique of aortic cannulation in such a way that the anomalous left coronary artery could be adequately perfused with the cardioplegic solution. Furthermore, injury to the coronary artery during the operative procedure was carefully avoided by knowing the exact location and course of the left main coronary trunk, from the angiographic pictures. Other unfavorable pathophysiologic consequences of this anomaly may derive from the acute angle of origin of the coronary ostium relative to the aortic wall. This could limit coronary blood flow especially during exercise. It seems, therefore, reasonable to moderately restrict the physical activity of such individuals and to advise regular cardiological follow-up.

Pericardial cyst diagnosed by two-dimensional echocardiography and computed tomography in a newborn.

A one-day-old full-term female infant with massive "cardiomegaly" developed marked respiratory distress soon after birth. The two-dimensional (2D) echocardiogram demonstrated a 4 X 5-cm cystic mass adjacent to the left ventricular free wall. Computed tomography (CT) confirmed the presence and dimension of the cyst and CT number indicated the fluid within the cyst to be serous. A pericardial cyst was successfully removed at thoracotomy. Two-dimensional echocardiography and CT, both noninvasive procedures, are fully adequate tools in the diagnosis of pericardial cysts in the neonate, so that invasive investigations may be avoided.

Staged septation of double inlet left ventricle.

Complete septation of a double inlet left ventricle with left anterior subaortic outlet chamber was carried out successfully as a staged procedure during the first two years of life in a patient with severe pulmonary hypertension and an abnormal left atrioventricular valve. In contrast with isolated pulmonary artery banding, which rarely has led to a satisfactory septation or modified Fontan operation, this technique achieved good initial palliation and uncomplicated intracardiac repair. It should be considered for infants with univentricular atrioventricular connection, two atrioventricular valves, and excessive pulmonary blood flow.

Spectrum of hearts with one underdeveloped and one dominant ventricle.

Hearts with one underdeveloped and one dominant ventricle form a spectrum of anomalies extending from the heart with two clearly adequate chambers to those with a true single ventricle. An angiographic concept of grouping such hearts is presented. 129 patients with unequal ventricles underwent catheterization and cineangiography between 1974 and 1983. The age at first catheterization ranged from one day to 24 years (mean 3.9 years). Male-female ratio was 2:1. Five groups of hearts (with their relative frequencies in the spectrum) were established: dominant left ventricle (53%); dominant right ventricle (20%), each with normally related chambers; dominant left ventricle (20%); dominant right ventricle (3%), each with ventricular inversion; true single ventricle (5%). The incidence of atrial anatomy, venous return, intracardiac connections and associated lesions within each group was assessed. From the standpoint of deranged physiology as well as surgical implications there are more similarities than differences among these hearts. The fact that one ventricle will not generate an adequate stroke volume after repair is overwhelmingly more important than most other considerations. For the diagnostic and surgical approach, we believe that the system offers many advantages.

Overriding atrioventricular valves. An angiographic-anatomical correlate.

An analysis of 6 patients with overriding atrioventricular valve was performed in order to correlate the anatomic and angiographic features of this malformation. The atrial arrangement was usual (situs solitus) in each patient. The ventricular chambers were normally related in 4 and the mirror image of normal in 2. The atrioventricular connexion was concordant in parallel fashion in 4, discordant in 1 and double inlet left ventricle in 1 heart. In 2 of 4 hearts with concordant atrioventricular connexion, the right atrioventricular valve (tricuspid) overrode the septum and connected partially to the left ventricle. In 2, the left atrioventricular valve (mitral) overrode the septum and partially connected with the right ventricle. One heart with discordant atrioventricular connexion had its right atrioventricular valve (mitral) overriding the septum. The remaining heart with double inlet left ventricle had an overriding left atrioventricular valve. Each overriding atrioventricular valve had its "septal" leaflet (the one committed to the contralateral ventricle) divided into 2 segments by a large cleft. Ventricular septal defects at different locations were present in all cases. The ventriculoarterial connexion was double outlet right ventricle in 5 patients and was discordant in 1. Malalignment of the atrial and ventricular septa was present to some degree in all cases. This was well depicted on ventriculograms in four chamber view in those with an overriding tricuspid valve. It was not detected in those with an overriding mitral valve. The displaced leaflets of the overriding and/or stradding valve had a vertical motion on cineangiograms similar to those observed in atrioventricular septal defects. Abnormal morphology and motion of the septal leaflet of the overriding atrioventricular valve and malalignment of the atrial and ventricular septal structures were the most important angiographic findings in this series.

Repair of congenital pulmonary venous stenosis with living autologous atrial tissue.

Repair of localized bilateral congenital pulmonary venous stenosis with living autologous atrial tissue was accomplished in two patients, 3 and 4 months of age. The tip of the left atrial appendage was opened and used as an onlay patch to widen the stenotic area of the left pulmonary veins, leaving a double pathway for venous return. A flap of interatrial septum was evaginated through an incision in the right side of the left atrium and used as an onlay patch to widen the stenotic area of the right pulmonary veins. Effective relief of obstruction was demonstrated by nearly normal postrepair pulmonary artery pressure in each and early postoperative angiography in one. Although we believe this method of repair will increase the probability of a lasting result, further experience and subsequent late reevaluation are needed to demonstrate its long-term effectiveness.

Congenital pulmonary vein stenosis.

Congenital pulmonary vein stenosis is a rare and serious form of congenital heart disease. Between 1969 and 1982 10 patients with this lesion were studied. In 2 patients the condition was diagnosed at autopsy; these patients died before the presence of congenital heart disease was suspected. Of the 8 in whom the condition was diagnosed during life, it was suspected clinically in 6 and found unexpectedly at cardiac catheterization in 2. All underwent operation, and 5 were hospital survivors. In all survivors rapid and progressive restenosis of the pulmonary veins occurred over the next several months. Three of the 5 underwent reoperation, but progressive restenosis recurred and all eventually died of this condition. Thus, despite partial surgical relief of pulmonary vein stenosis, the lesion is apparently one of relentless progression. No surgical repair has been successful in the cure or long-term palliation of this lethal lesion.

Tetralogy of Fallot with atrioventricular canal. An angiographic study.

Tetralogy of Fallot associated with atrioventricular (AV) canal is a well-recognized cardiac malformation; its surgical repair carries a high mortality, which is related partly to the complexity of the defect and partly to incomplete diagnosis. Hence an accurate preoperative diagnosis is essential. This communication describes the angiographic features observed in 17 patients with tetralogy of Fallot and AV canal with emphasis on the morphology of the right AV valve as seen during the right ventriculogram. A complete anatomic diagnosis can be consistently made from right and left ventriculograms performed in axial projections.