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The ascending aorta--right pulmonary artery anastomosis, originally introduced by Waterston as a palliative shunt to increase pulmonary blood flow in certain cyanotic congenital heart diseases, has been found to be associated with late complications in a significant number of cases. These complications include preferential distribution of most or all shunt flow to the right lung, narrowing or obstruction of the right pulmonary artery at the anastomotic site, increasing stenosis or atresia of the right ventricular outflow tract, hypoplasia of the left pulmonary artery, and obstruction of the shunt itself. A properly planned angiocardiographic study is the principal method of detection of these complications.

Several authors have reported an association between neuroblastoma and congenital heart disease; others contend that, unlike specific well-known associations between malignancy and congenital defects (Wilm's tumor and aniridia, leukemia and Down's syndrome), no real relationship exists. We present three cases of cyanotic congenital heart disease in which subclinical neuroblastoma was found. We speculate that abnormal neural crest cell migration and development may be a common link between cardiac malformations and congenital neuroblastoma.

Three children with repaired omphalocele underwent diagnostic cardiac catheterization. In addition to intracardiac shunts, each was found to have marked angulation at the junction of the inferior vena cava and the right atrium. After review of our autopsy material following omphalocele repair, it is suggested that the inferior vena cava - right atrial angulation is due to gradual abnormal fixation of this junction either in utero or related to the surgical repair. A change in current surgical therapy is NOT recommended. Angulation of the inferior vena cava - right atrial junction is potentially important because a) it may interfere with venous cardiac catheterization from "below", b) it poses a danger to inferior vena caval cannulation for cardio-pulmonary by-pass, and c) it may become obstructive with time producing portal hypertension.

A modified U-am with parallelographic motion is readily usuable for serialographic neurological, abdominal, and peripheral angiographic studies in infants and children. Clinical experience with the apparatus has demonstrated dependable and mechanically simple operation, and confirmed that undistorted rotated and sagittally angled views can be obtained without the need for repositioning sedated pediatric patients.

To assess the feasibility of quantitative central venous digital angiocardiography, right ventricular spatial orientation, size, and shape were compared with measurements based on conventional angiocardiography, both obtained during routine cardiac catheterization in 21 children with various congenital heart defects. Without systematic error, correlations for end-diastolic and end-systolic volumes were excellent, at r = 0.986 and 0.969, respectively. Ejection fractions were similarly closely correlated. Premature ventricular contractions were common in conventional angiocardiography and absent in digital and angiocardiography. The amount of contrast medium was 36% of that used for conventional angiocardiography. Digital angiocardiography using reduced amounts of contrast medium given through a central venous catheter allows accurate assessment of right ventricular size and function.

Four cases of cervical aortic arch are described and correlated with the 21 previously reproted cases. Five distinct forms of cervical aortic arch were found and classified according to configuration of the aorta, sequence of brachiocephalic branching, and embryogenesis. In brief, Type A has separate external and internal carotid artery branches from the aortic arch. Tye B is similar but has dual common carotid arteries. Type C is a left cervical arch with a right-sided descending aorta and bicarotid trunk. Type D is a left cervical arch with a normal branching pattern, redundant transverse aorta and left-sided (often hypoplastic) descending aorta. Type E is a right cervical aortic arch with a right descending aorta and an aberrant left subclavian artery.

Although bronchoscopy remains the best definitive method for localization of the site of hemorrhage in patients who have massive hemopytsis, angiography remains an important adjunct to this localization. When combined with embolization of the bleeding bronchial artery, it is an effective method of therapy for the management of massive hemoptysis in patients with cystic fibrosis.

Angiocardiograms in 36 infants with tetrad of Fallot were reviewed to determine which projections best displayed the anatomy. The occurrence of associated cardiovascular anomalies was also recorded. An additional ventricular septal defect was found in 14 percent (5 of 36), peripheral pulmonary stenoses in 30 percent (10 of 36) and surgically important coronary arterial abnormalities in 8 percent (3 of 36). This retrospective study indicated that optimal biplane angiocardiography should include: (1) right ventriculography in a sitting or hepatoclavicular view for demonstration of the right ventricular, infundibular, and pulmonary artery anatomy, (2) left ventriculography in the long axial oblique (cranially angled oblique) view for display of ventricular septal defects and coronary arteries, and (3) and aortogram at the valve level (oblique view) if coronary arteries are not well seen in the left ventricular study.

Of 113 leg venograms performed in patients of all ages between 1969 and 1982, 68 were in children 16 years old or less. The patients were all studied on a tilt table (method of Rabinov and Paulin) in a head-up, 40-50 degrees incline without tourniquets, supporting their weight on the unaffected leg. Among the 68 venograms, 12 (18%) were positive for deep vein thrombosis. The clinical settings for thrombosis in children were post-catheterization (two patients), post surgery (two), tumor/tumor therapy (three), drug abuse (one), infection (one), and idiopathic (three). There were no long-term clinical sequelae in five patients. Pulmonary infarction occurred in three, and three patients required either long-term anticoagulation or IVC clipping. Clinical diagnosis is no more accurate for the diagnosis of deep vein thrombosis in children than it is in adults. Venography is the best method for making an accurate diagnosis and directing subsequent therapy.

Clinical and cellular differences between hemangiomas and vascular malformations in children have been defined. Hemangiomas are benign endothelial cell neoplasms that appear in infancy and usually have a natural history of proliferation and involution. Vascular malformations are errors of vascular morphogenesis that are present at birth, grow with the child, and never involute but often expand. The authors reviewed the preoperative angiograms of 14 children who had cellular analyses of resected vascular lesions. Hemangiomas could be distinguished from vascular malformations by the presence of a well circumscribed mass demonstrating intense tissue staining, usually organized in a lobular pattern. The vascular malformations, although angiographically variable depending on the predominant vascular channel type, were diffuse lesions consisting entirely of vessels without intervening tissue stain. These angiographic differences between hemangiomas and vascular malformations corroborate clinical and laboratory studies.

The excretory urograms of 14 children with ureteral notching were reviewed. In 10 of 14 cases the vascular impressions on the ureters were due to either venous obstruction and dilated collaterals or to dilated noncollateral veins. In two cases the notching was associated with arterial collaterals in renal ischemia and in two with dilated lymphatic channels. The implications of notching in children and the differences and similarities of this urographic sign between children and adults are discussed.

Nine patients, 6 weeks to 17 years of age with esophageal atresia (EA), developed esophageal strictures and were treated with 26 balloon catheter dilatations over a period of 3 years; 6 are now asymptomatic. Five of the 9 patients had suspected reflux esophagitis, confirmed in 3 by biopsy and treated medically prior to dilatation. Seven of the 9 patients had a primary anastomosis, 1 a gastric tube, and 1 a colonic interposition. Most dilatations in the group of 7 were performed with balloon (B) greater than or equal to the diameter of the distal esophagus (E) (B/E greater than or equal to 1). The 3 residually symptomatic patients include an infant dilated conservatively (B/E less than 1) to facilitate later bouginage, 1 patient with a recurrent stricture after stopping medical therapy and home bouginage, and 1 infant who had a persistent anastomotic stricture, suspected but untreated reflux esophagitis, and a perforation during the second balloon dilatation. Balloon catheter esophageal dilatation, as an alternative to bouginage, is usually a safe and effective procedure when reflux esophagitis is diagnosed and treated prior to dilatation.

Although therapeutic catheter procedures for congenital heart disease are still developing, the number of procedures being performed allows statistically relevant review of the acute complications. Between January 1, 1984, and February 1, 1987, 417 such procedures were performed at The Children's Hospital, Boston; the age range of the patients was 1 day to 51 years (median 4 years, 6 months). Catheter procedures consisted predominantly of vascular dilations (peripheral pulmonic stenosis, 97; valvular pulmonic stenosis, 67; valvular aortic stenosis, 62; recurrent coarctation, 49) and embolizations (double umbrella device, 36; steel coils, 45). Overall, there were 50 acute complications (12%); 24 (6%) were major and 26 (6%) were minor. The mortality rate was 3 of 417 (0.7%). Complication rates varied between 4% for dilation of recurrent coarctation and 40% for dilation of aortic stenosis. The age of the patients was a factor in complications of vascular access (11 patients), 8 of which occurred in patients younger than 6 months (median 5), and in cardiac arrest and ventricular fibrillation (4 patients, 3 of whom were younger than 6 months [median 3]). No statistically significant trend toward diminishing overall complication rates was discerned over the 37 months of this study.

Severe bronchial hemorrhage in 13 patients with cystic fibrosis was treated by catheter embolization of bronchial arteries. Indications were either excessive bleeding persisting for several days, or bleeding serious enough to interfere with pulmonary drainage and recurring over weeks or months. In follow-up ranging from one to 30 months, cessation of major bleeding was achieved in 12 of 13 patients (93%), although 5 of 13 patients (40%) did have recurrence of minor hemoptysis. No neurologic or other major complications were encountered. However, there are potential risks and this approach at present should be limited to patients with life-threatening bleeding and carried out only by experienced angiographers.

The development of systemic hypertension, in an eight-year-old girl, after resection of a large arteriovenous malformation, is described. The hypertension was related to an elevated plasma renin activity and was controlled with propranolol. A possible mechanism for the rise in plasma renin activity is postulated.

Gianturco stainless steel coils were discharged into clear plastic tubes of varying sizes and subjected to pulsatile flow in order to study their behavior in a controlled, in vitro environment. When coils are too small for a given vessel, they tend to migrate distally or even proximally. Coils that are too large tend not to reform completely, limiting their cross-sectional area and potentially affecting their occlusive potential. Thus, each coil was tested in tubes of varying sizes and evaluated with regard to positional stability and extent of reformation.

Exposure area product (EAP) and center field entrance exposure (free-in-air) were measured in seventeen pediatric patients undergoing cardiac catheterization. Exposures were recorded separately for biplane fluoroscopy and cine angiocardiography using flat-plate ionization chambers. In the posterior-anterior (PA) projections, median EAP was 425 Roentgen-square centimeter (R-cm2), with a range of 90.5-3,882 R-cm2;29-35% of this exposure occurred during cine filming. In the lateral projection, median EAP was 276 R-cm2 (range 117-1,173); 52-59% of this exposure was due to cine filming. Median center field entrance exposure in the PA view was 7.86 Roentgens (R) with a range 2.16-73.9 of and in the lateral projection 7.39 R (range 2.64-24.6). As much as 25% of the exposure from the entire examination was contributed by manual "test" exposures to set cine radiographic kVp. We recommend use of testing circuits, which determine cine radiographic factors automatically and thus should lower levels of exposure.

There is a large spectrum of interventional catheter procedures being performed presently in children. The procedures that offer a greater advantage than surgical techniques and that will continue to be established procedures include percutaneous angioplasty of the pulmonary valve, peripheral pulmonary artery and caval stenosis, embolizations of pulmonary collaterals and pulmonary arteriovenous malformations, angioplasty of vascular access shunt, embolization of bronchial arteries for hemoptysis, and percutaneous nephrostomy and abscess drainage procedures. Others that have been successful but not yet routinely established in children include thrombolysis, renal artery angioplasty, embolization of peripheral arteriovenous malformations, dilatation of the urinary and gastrointestinal tract stenosis, and percutaneous biopsy.

Twelve patients who had undergone complete repair of tetralogy of Fallot at ages ranging from 8 to 27 years were studied for exercise tolerance several years later. Work performance ranged from 58 to 130% of that predicted. Increased physiological dead space and alveolar-arterial Po2 difference at exercise suggested pulmonary vascular disease, which was confirmed in 1 patient by pulmonary angiography. Excercise tolerance decreased with increasing age at time of repair, underlining the desirability of early corrective operation.

Gianturco coils were used to embolize 77 vessels in 54 patients: 58 aortopulmonary collateral vessels, 14 Blalock-Taussig shunts, 3 arteries involved in pulmonary sequestrations and 2 venae cavae. Embolization resulted in total occlusion in 53 (69%), subtotal occlusion in 19 (25%) and partial occlusion in 3 (1 intentional). Two embolizations failed to reduce flow. Thus, 72 (95%) of 76 embolizations in which complete occlusion was the intended result resulted in total or subtotal occlusion. Analysis of the results demonstrates that completely occluded collateral vessels were longer and had a smaller diameter than did incompletely occluded vessels. Complications included six cases of inadvertent embolization to the pulmonary arteries (n = 5) or the aorta (n = 1); three were retrieved and three were left without symptoms. In addition, there was a case of severe hemolysis after intentional partial occlusion of a Blalock-Taussig shunt. The results demonstrate that coil embolization can be an effective procedure for managing a wide variety of aorto-pulmonary collateral vessels and shunts in children with congenital heart disease.

Between 1956 and 1976, 18 patients underwent surgery for supravar aortic stenosis at the The Children's Hospital Medical Center, Boston. Discrete obstruction, present in 11, was treated by insertion of a prosthetic gusset placed across the area of narrowing and extending into the noncoronary sinus of Valsalva. There was one operative death. Residual gradients (measured in five patients) ranged from 4-55 mm Hg, one of which was supravalvar in location. Significant aortic regurgitation was not common preoperatively. The diffuse form of supravalvar obstruction, a more difficult surgical problem, was present in seven patients. There were three operative deaths. Complete relief of the pressure gradient was achieved only in one instance by insertion of a left ventricular-aortic bypass shunt diverting the majority of the cardiac output into the descending thoracic aorta. This patient is now asymptomatic 20 months following operation. On the basis of this experience, it is suggested that patients with the diffuse form of supravalvar obstruction, and perhaps even those with a hypoplastic annulus alone, would benefit from a left ventricular-aortic bypass shunt.

A case of pseudostenosis of the right pulmonary artery caused by a catheter in the azygos vein is presented. The recognition of this pseudolesion occurred when a repeat catheterization prior to angioplasty, demonstrated a normal right pulmonary artery.

The charts of 79 patients who required femoral arterial (FA) thrombectomy after cardiac catheterization were reviewed. Fifteen patients (19%) had poor pulses after thrombectomy and 2 had an extremity amputated. One thousand consecutive patients undergoing cardiac catheterization were also studied to prospectively determine the safety and efficacy of systemic fibrinolytic therapy for treatment of FA thrombosis. Among these, 771 patients underwent retrograde arterial catheterization, including 31 patients with left-sided obstructive lesions who had undergone transarterial balloon dilation procedures with large catheters. All patients were given heparin at the time of arterial cannulation. Patients who had a pulseless extremity 4 hours after catheterization continued to receive heparin therapy for 24 to 48 hours. If the extremity continued to have no palpable pulse and the systolic blood pressure was less than 50% of that in the contralateral leg, intravenous streptokinase infusion was begun. The overall incidence of FA thrombosis was 3.6% (28 of 771), including 39% (12 of 31) of all patients undergoing transarterial balloon dilation procedures; 97% (27 of 28) of patients weighed less than 14 kg and the majority weighed less than 10 kg. After an average treatment period of 33 hours, 16 patients continued to have a pulseless extremity and were treated with streptokinase for an average duration of 13 hours. Normal pulses and systolic blood pressure returned in 14 (88%) and were nearly normal in 1 other patient (6%). The incidence of bleeding at the arterial puncture site was 25% and was highest in the patients who had a transarterial balloon dilation procedure. No serious complications occurred.

Three children with typhilitis complicating acute leukemia and presenting with severe intestinal hemorrhage are described. The arteriographic findings were hypervascularity of the cecum with intense mucosal staining, opacification of superficial ulcers, and arteriovenous shunting into dilated mesenteric veins. The three children were managed successfully with medical, surgical, and embolic therapies, respectively.

Advancing technology in catheterization techniques have allowed the development of several therapeutic procedures in patients with congenital heart disease. Some of these, such as atrial balloon septostomy, are well established. Others, such as pulmonary valve dilatation, patent ductus closure, and systemic-pulmonary collateral obliteration are currently being tried with success in many institutions. In the future, certain forms of coarctation, some intracardiac shunts and perhaps other anomalies may be suitable for therapy by catheter techniques.

Twenty-seven children aged 1 day to 16 years studied arteriographically for acute or chronic gastrointestinal bleeding were reviewed. Children with known esophageal varices and portal hypertension were excluded. Final diagnoses were made in 25 patients by means of surgery, endoscopy, biopsy, laboratory data, and clinical follow-up. Of these 25 cases, arteriography gave a correct diagnosis in 64% and was falsely negative in 36%. In acute gastrointestinal bleeding, arteriographic diagnosis was correct in 71% and falsely negative in 29%. In chronic or recurrent gastrointestinal bleeding, arteriographic diagnosis was correct in 55% and falsely negative in 45%. The common causes of bleeding in this study were gastric and duodenal ulcers, gastritis, vascular malformations, and typhlitis. Transcatheter therapy was attempted in six acute bleeders, with success in three (50%).

Although balloon dilation of valvular pulmonary stenosis is established in infants and children, the techniques for and results of balloon dilation in neonates with critical pulmonary stenosis remain largely unreported. Since January 1, 1985, six successive neonates with critical pulmonary stenosis (aged 1 to 6 days) underwent attempted balloon dilation. Each was cyanotic and three of the six were on prostaglandin E1 therapy and three required tracheal intubation and ventilation. All had suprasystemic right ventricular pressures (mean 122.8 +/- 6.8 mm Hg). After hemodynamic evaluation and right ventricular angiography, the valve was crossed in five patients, and was first dilated with a low profile, 5 or 6 mm diameter, 2 cm long balloon. At least one more balloon was used in each patient, the largest being 95 to 133% of the diameter of the pulmonary valve anulus. The anulus size was 6.8 +/- 1.1 mm and the largest balloon size used was 6 to 10 mm. Right ventricular pressure decreased to nearly systemic level or less in five of five patients (58.8 +/- 6.7 mm Hg). Pressure gradients, measured in four infants, were 7, 12, 16 and 35 mm Hg, respectively, but were unreliable indicators of obstruction because of a patent ductus arteriosus. The five patients were discharged 3 to 8 days after balloon dilation. All are currently symptom free 10.6 +/- 11.7 months later, and all but one are believed clinically to have mild obstruction. Complications included iliac vein occlusion (n = 1) and complete right bundle branch block (n = 1). Although follow-up has been brief, neonates with critical pulmonary stenosis can safely undergo balloon dilation, usually with good short-term results.

Eight cases are presented in which the diagnosis of overriding of the tricuspid valve was made during life and the electrocardiographic, echocardiographic and angiographic features of the defect are presented. Four of the patients had dextrotransposition of the great arteries, three had normally related great arteries and one had corrected transposition. In each case there was hypoplasia of the right ventricle and a ventricular septal defect of the atrioventricular (A-V) canal type. The electrocardiogram in the cases with d-transposition was characterized by diminished right ventricular forces, left ventricular forces, left ventricular hypertrophy and a superior leftward frontal plane axis. The echocardiogram in seven cases demonstrated a septal leaflet of the tricuspid valve opening posterior to the septum into the left ventricle. In four cases the anterior leaflet of the tricuspid valve was shown crossing the plane of the interventricular septum as it opened in diastole. The diagnosis in five cases was made angiographically by a left ventricular injection in the left anterior oblique projection. In this view the septum was viewed tangentially and in diastole the negative silhouette of the tricuspid valve was seen straddling the interventricular septum. The presence of an overriding tricuspid valve can greatly complicate repair of intracardiac defects. The diagnosis of this A-V valve anomaly can be accurately made with the use of echocardiography and selective left ventricular angiography.

Percutaneous balloon valvotomy was attempted in 27 patients (aged 6 days to 19 years, median 2 years, 11 months) with unoperated typical valvular pulmonary stenosis using a balloon 7 to 60% (mean 30%) larger than the valve anulus. One patient had undergone a previous balloon valvotomy elsewhere. To achieve an oversized dilation diameter in three larger patients, two balloons were inflated side by side. Their "effective dilation diameter" was determined by the diameter of the circle with the same area as that of the oval enveloping the two balloons. A significant reduction of the transvalvular gradient occurred in all patients (mean +/- SD = 74.3 +/- 14.7%, range 33 to 100%). The average gradient of 65.0 +/- 19.0 mm Hg (mean +/- SD) fell to 15.9 +/- 7.6 mm Hg (0 to 30 mm Hg). Twenty-five of 27 patients had a residual transvalvular gradient of less than 25 mm Hg. The calculated valve orifice area increased by an average of 183 +/- 80%. No significant complications occurred. It is concluded that percutaneous balloon valvotomy with a balloon 20 to 40% larger than the valve anulus is the treatment of choice for typical congenital valvular pulmonary stenosis.

Cineurography is often performed after cineangiocardiography to look for occult congenital urinary tract disease. The accuracy of cineurography was investigated in 171 patients by comparing cineurograms with renal sonograms. One hundred fifteen cineurograms (67%) showed both kidneys well enough to allow assessment of renal structure and function and the results were confirmed in 112 by ultrasonography; 3 cineurograms yielded false-positive results. Limited or no information was obtained from cineurograms of 56 patients (33%) because of nonvisualization or poor visualization of 1 or both kidneys. Of the 11 patients (6%) with urinary tract disease, only 3 were correctly assessed by cineurography. Ultrasonography discovered all 11 renal abnormalities and produced only 1 false-positive result. These data indicate that cineurography is a poor screening test and should be abandoned. When uroradiologic screening is necessary for high-risk patients, sonography is recommended.

Hemodynamically significant primary anomalies of the coronary arteries are those which alter myocardial perfusion. There are four major types: coronary artery fistulae, origin of the left coronary artery from the pulmonary artery, congenital coronary stenosis or atresia, and origin of the left coronary artery from the right sinus of Valsalva, with subsequent passage of the vessel between the aorta and right ventricular infundibulum. The angiographic features of these lesions are discussed.

Simple and compound sagittally angled views of the heart obtained cineangiocardiographically with a parallelographic U-arm device were evaluated clinically in 132 patients with congenital heart disease. The principle advantage of the apparatus was the case with which axial and oblique projections were obtained without repositioning of the patient, although rotation and angulation of the image intensifier-X-ray tube unit resulted in some increase in scattered radiation. Certain angled views, particularly the 40 degree cranial and the compound 25 degrees cranial/70 degrees left oblique projections, were better than standard frontal and lateral views for visualization of pulmonary arteries in the mediastinum, in ventricular septal defects and anomalies of the left ventricular outflow tract.

The excretion urogram-nephrotomogram is the most important imaging study in the diagnosis of renal parenchymal infection in children. If renal parenchymal infections are discovered before significant necrosis has occurred, vigorous treatment with appropriate parenteral antibiotics may result in resolution without the need for surgical drainage.

The patency of vascular access shunts and fistulae has been prolonged by a combined surgical and radiological approach that includes percutaneous transluminal angioplasty (PTA), surgical revision, thrombectomy, and thrombolysis. Over the last 3 years, 35 vascular accesses in 27 patients were found to have angiographic abnormality. PTA was performed 32 times on 19 accesses and 7 PTAs resulted in patent accesses by the end of the study. Surgical revision was performed 9 times on 8 accesses and 2 of the surgical revisions resulted in a patent access by the end of the study. Concerning Thomas femoral shunts, PTA prolonged the patency by 2.2 months and surgical revision by 3.8 months per procedure. Concerning arteriovenous (AV) fistulae, PTA prolonged the patency by 4.3 months and surgical revision by 3.5 months per procedure. A combination of procedures effectively doubles the duration of patency of Thomas femoral shunts and almost triples the duration of patency of AV fistulae in children. Forty-one percent of these accesses remain open 1 year following the initiation of these procedures.

This communication provides a description and update of the three adjuvant regimens utilized at the Sidney Farber Cancer Institute. The disease-free survivals are: Study I--42% at 4 years; Study II--58% at 3 years; and Study III--currently 78%. In the limb salvage experience, among the evaluable patients, 6 of 12 treated with preoperative chemotherapy are disease free (median 32 months) and 12 of 14 (86%) treated without preoperative chemotherapy are disease free (median 13 months).

Between October 1984 and September 1986, we attempted transcatheter umbrella closure, using the Rashkind PDA occluder, of 12 congenital or postoperative cardiovascular defects (other than patent ductus arteriosus [PDA]) in 11 patients. In each, we used the umbrella for closure because the defect was too short and/or too large to close with conventional transcatheter methods. The defects included three post-Glenn venous communications (superior vena cava-right atrium, n = 2; azygos vein to inferior vena cava), four congenital "interatrial defects" producing cyanosis ("coronary sinus" septal defect, left superior vena cava to left atrium, patent foramen ovale, left inferior vena cava to left atrium), and five non-PDA systemic-to-pulmonary arterial communications (two congenital and three postoperative). Ten of 12 defects were embolized successfully; nine had complete or subtotal closure, and one was partially closed. The first attempted closure resulted in embolization of a 12 mm device to a lower-lobe pulmonary artery, without clinical sequelae. No other complications occurred. Clinical improvement was most dramatic in those patients whose cyanosis was relieved and less obvious when pulmonary blood flow was reduced. The Rashkind umbrella device, originally designed for closure of PDA, considerably expands the list of congenital or operative defects that can be closed nonsurgically.

Extravascular lung water (EVLW) was measured in 17 patients with congenital heart disease by the cold-green-dye, double indicator-dilution technique. In 5 control patients, EVLW was 4.7 +/- 0.5 ml/kg (111 +/- 13 ml/m2) (mean +/- standard deviation). Twelve patients were studied immediately after correction of their heart defects. In 6 patients with normal or decreased pulmonary blood flow preoperatively (Group I), EVLW was 6.2 +/- 1.9 ml/kg (122 +/- 46 ml/m2). This value is not significantly different from that of the control patients. In 6 patients with increased pulmonary blood flow and congestive heart failure preoperatively (Group II), EVLW was 15.7 +/- 3.8 ml/kg (270 +/- 60 ml/m2), which is significantly different from both control and Group I patients (p less than 0.01). There was no correlation of EVLW with pre- or postoperative left atrial pressure, length of cardiopulmonary bypass or deep hypothermic circulatory arrest, postoperative serum protein, albumin, hematocrit or cardiac index. Thus, EVLW in the immediate postoperative period is determined by preoperative pathophysiologic characteristics rather than by intraoperative management, and patients with congestive heart failure resulting from left-to-right shunts have increased EVLW despite normal left atrial pressures.

Portal hypertension in Gaucher disease is unusual; the seventh known patient with this complication is reported. Prior to portacaval shunting in this child, a localized obstruction of the inferior vena cava at the subdiaphragmatic level was demonstrated by caval manometry and inferior vena cavography. At autopsy, centrilobular hepatic fibrosis seemed to be responsible for the portal hypertension. Nodular enlargement of the right and caudate lobes of the liver was the cause of the caval obstruction; elevated caval resistance may have contributed to the portal hypertension and possibly was responsible for failure of a portacaval anastomosis. The value of preoperative inferior vena cavography in addition to arterial portography in children with portal hypertension is stressed.