Investigation and management of primary cardiac tumors in infants and children.

Seven children with primary cardiac tumors are discussed. The diagnosis was achieved by cardiac catheterization in all seven patients. In four patients, surgical excision was undertaken with one long-term good result. One of the other three surgical patients died of acute heart failure at the discontinuation of cardiopulmonary bypass. One of the other two surgical patients died suddenly late postoperatively, presumably from arrhythmia. The other, affected by fibrosarcoma, also died suddenly while undergoing chemotherapy. The histologic findings on the tumor masses were available in six patients. There were three rhabdomyomas, one fibrosarcoma, one fibroma and one hamartomatous mass. In one patient with tuberous sclerosis, a presumptive diagnosis of rhabdomyoma was made clinically on the basis of multiple masses infiltrating the left ventricle. Two-dimensional echocardiography is the best noninvasive tool to determine the extent and location of the tumor and suitability for surgery. When surgery is indicated, cardiac catheterization for pressure data is also required. Surgery is recommended in symptomatic solitary lesions that most likely are fibromas and have an overall good surgical outcome.

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.

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.

Tricuspid atresia with double-outlet left atrium.

Clinical and pathologic findings of an unusual case of cardiac malformation are presented. The main features were those of atresia of the right atrioventricular valve associated with two distinct atrioventricular orifices connecting the morphologically left atrium to the underlying morphologically left ventricle. Other distinguishing characteristics were ostium secundum atrial septal defect, normally related great arteries, with the aorta arising from the main ventricular chamber and the pulmonary artery from the anterior and right-sided outlet chamber, and infundibular and valvular pulmonary atresia.

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.

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.

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.

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.

[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.

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.

Visualization of pulmonary vein obstruction by pulmonary artery wedge injection.

One of the major postoperative complications of total anomalous pulmonary venous connection is obstruction of the pulmonary veins. This event may be apparent immediately after surgery when it is usually due to congenitally obstructed veins or later when it may be the result of failure of commensurate growth of the left atrium-common vein anastomosis or of scarring at the anastomotic site. The exact location of the obstruction becomes, then, of paramount importance in deciding if the patient can be helped by further surgery. At cardiac catheterization, pulmonary artery wedge injection was the only angiographic technique that visualized the site and degree of pulmonary venous obstruction in the case reported here.

[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.

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.

[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.

Histology of pulmonary arterial supply in pulmonary atresia with ventricular septal defect.

A histologic study was performed on 22 specimens of pulmonary atresia with ventricular septal defect to 1) ascertain the existence of the main pulmonary artery; 2) distinguish the ductus arteriosus from the systemic collateral arteries (SCA); 3) establish the nutritive or functional nature of collateral circulation; and 4) evaluate the morphology of the distal pulmonary bed. Three cases had absent main pulmonary artery, one with and two without signs of infundibular septation. We suggest that absent main pulmonary artery may exist with both infundibular pulmonary atresia and persistent truncus arteriosus. SCAs have been found to have similar histological features as systemic muscular arteries of the same size--their medial muscular layer merges gradually into an elastic one at different depth inside the lungs. Injection of contrast material allowed us to demonstrate that these vessels are functional, since they inosculate into efficient pulmonary arteries ending in the respiratory units. When the distal pulmonary vascular bed is perfused by large SCAs, proliferative lesions like those found in large left-to-right shunts may occur. Early in infancy, banding of large, nonstenotic SCAs could protect the distal pulmonary vasculature. Moreover, total surgical repair should be associated with ligation of the SCA to avoid residual left-to-right shunt, if the pulmonary arteries can carry the full pulmonary blood flow.

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.

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.