Enlarged bronchial arteries after early repair of transposition of the great arteries.

OBJECTIVES: This study was undertaken to define the incidence of enlarged bronchial arteries after early surgical repair of transposition of the great arteries by the arterial switch operation, and to report the results of catheter-directed therapy in five patients. BACKGROUND: Pathologic and angiographic studies have demonstrated enlarged bronchial arteries in patients with transposition of the great arteries. METHODS: A subjective 4-point scale was used to grade postoperative angiograms performed in 119 patients at our institution between January 1983 and December 1991. Grades 0 and 1 were designated if there was no opacification of the pulmonary arteries or veins, whereas grades 2 and 3 were assigned if there was such opacification. The median age at repair was 8 days (range 1 day to 13 months) and the median age at catheterization was 11.2 months (range 3.6 to 58.5). An intact ventricular septum was present in 84 (71%) of 119 patients. RESULTS: Significantly increased bronchial flow (grade 2 or 3) was present in 55 (46%) of 119 patients. Age at repair, age at catheterization and interval between repair and catheterization were not associated with significantly increased bronchial flow; however, an intact ventricular septum was weakly associated with increased flow (p = 0.04). Coil embolization was performed in five patients with complete occlusion of the vessels and no significant complications. CONCLUSIONS: Abnormally enlarged bronchial arteries are frequently identified at postoperative catheterization despite early repair and may explain continuous murmurs or persistent cardiomegaly in patients with otherwise normal noninvasive findings. When clinically indicated, catheter-directed therapy can be performed with good results.

Postmortem and intraoperative balloon valvuloplasty of calcific aortic stenosis in elderly patients: mechanisms of successful dilation.

Percutaneous balloon dilation of the aortic valve has recently been proposed as a palliative procedure for treating nonsurgical candidates with calcific aortic stenosis. To assess the safety, efficacy and mechanisms of successful balloon valvuloplasty, postmortem (n = 33) and intraoperative (n = 6) balloon aortic valvuloplasty was performed in the hearts of 39 elderly patients with calcific aortic stenosis. The cause of aortic stenosis was degenerative nodular calcification in 28 cases, calcific bicuspid aortic stenosis in 8 cases and rheumatic heart disease in 3 cases. Balloon dilation was performed with 15 to 25 mm balloons in the postmortem specimens, and with 18 to 20 mm balloons in the operating room immediately before aortic valve replacement. After balloon dilation, valve orifice dimensions and leaflet mobility increased in all patients. The mechanisms of successful dilation included fracture of calcified nodules in 16 aortic valves, separation of fused commissures in 5 valves, both in 6 valves and grossly inapparent microfractures in 12 valves. Valve leaflet avulsion occurred in one heart after inflation with a clearly oversized balloon. Liberation of calcific debris, valve ring disruption or midleaflet tears did not occur in any heart. In conclusion, there are at least three mechanisms of successful aortic valvuloplasty, depending on the origin of valvular stenosis. Embolic phenomena and acute valvular regurgitation do not appear to be likely events associated with this procedure.

Transcatheter umbrella closure of valvular and paravalvular leaks.

OBJECTIVES: Our aim was to adapt the technique of transcatheter umbrella closure of intracardiac defects for closure of valvular and paravalvular defects. BACKGROUND: The double-umbrella device developed by Rashkind and Cuaso has been safely and effectively delivered across a host of intracardiac defects, but transcatheter closure of valvular and paravalvular leaks has not been reported. METHODS: Between February 1987 and September 1990, eight patients who were believed to be poor operative candidates were taken to the catheterization laboratory for transcatheter double-umbrella closure of a valvular or a paravalvular leak. Four patients had a paravalvular leak around a prosthetic aortic valve. The other four patients had a valvular leak: one patient with a regurgitant native aortic valve after a Stansel procedure and three patients with a regurgitant porcine valve in a left ventricular apex to descending aorta conduit. RESULTS: Placement of a double-umbrella device was attempted in seven of the eight patients and was successful in all seven. Device placement was not attempted in one patient because of the crescentic shape of his defect. Two patients required two devices for each closure; the other five required only one device each. Angiography, performed on six patients after device closure, demonstrated that three patients had a completely occluded defect, two had trivial residual flow and one patient had mild residual flow through the device. All significant complications occurred in one patient who had hemolysis and oliguria that resolved when the initial umbrella was replaced by a larger device. In addition, two devices migrated to the patient's pulmonary arteries but were retrieved in the catheterization laboratory without difficulty. No other early or late complications occurred in 21 to 50 months of follow-up. Of the four patients with a paravalvular leak, the one who did not receive a device died at operation, one patient died at operation for an associated defect (in the operating room the umbrella was found securely in place across the paraaortic defect) and two patients are clinically well at home after 21 and 32 months, respectively. Of the four patients with closure of a valvular leak, one patient remains well at home 50 months later, one patient died at operation for associated defects and two patients had additional successful surgical treatment and remain well 29 months after device placement. CONCLUSIONS: Transcatheter umbrella closure appears to be a reasonable alternative for closure of a valvular or paravalvular leak in patients who are poor operative candidates.

Balloon dilation of mitral stenosis in adult patients: postmortem and percutaneous mitral valvuloplasty studies.

Preliminary reports have documented the utility of percutaneous balloon valvuloplasty of the mitral valve in adult patients with mitral stenosis, but the mechanism of successful valve dilation and the effect of mitral valvuloplasty on cardiac performance have not been studied in detail. Accordingly, mitral valvuloplasty was performed in five postmortem specimens and in 18 adult patients with rheumatic mitral stenosis, using either one (25 mm) or two (18 and 20 mm) dilation balloons. Postmortem balloon dilation resulted in increased valve orifice area in all five postmortem specimens, secondary to separation of fused commissures and fracture of nodular calcium within the mitral leaflets. In no case did balloon dilation result in tearing of valve leaflets, disruption of the mitral ring or liberation of potentially embolic debris. Percutaneous mitral valvuloplasty in 18 patients with severe mitral stenosis (including 9 with a heavily calcified valve) resulted in an increase in cardiac output (4.3 +/- 1.1 to 5.1 +/- 1.5 liters/min, p less than 0.01) and mitral valve area (0.9 +/- 0.2 to 1.6 +/- 0.4 cm2, p less than 0.0001), and a decrease in mean mitral pressure gradient (15 +/- 5 to 9 +/- 4 mm Hg, p less than 0.0001), pulmonary capillary wedge pressure (23 +/- 7 to 18 +/- 7 mm Hg, p less than 0.0001) and mean pulmonary artery pressure (36 +/- 12 to 33 +/- 12 mm Hg, p less than 0.01). Left ventriculography before and after valvuloplasty in 14 of the 18 patients showed a mild (less than or equal to 1+) increase in mitral regurgitation in five patients and no change in the remainder.(ABSTRACT TRUNCATED AT 250 WORDS)

The "laid-back" aortogram: an improved angiographic view for demonstration of coronary arteries in transposition of the great arteries.

Preoperative demonstration of coronary arterial anatomy may be important for babies undergoing the arterial switch operation. Echocardiography is clearly useful, but may not unequivocally show all coronary branches. Standard angiographic views can be confusing. An improved angiographic projection in which the frontal x-ray tube is caudally angled, resulting in a "laid-back" position of the image intensifier and cine camera, provides superior visualization of the coronary arteries and their relation to the aorta and the pulmonary artery. The balloon occlusion technique is used for opacification of the aortic root from the transvenous approach. Injection of 1 ml/kg of contrast delivered in 1/2 to 1 second provides the best images. The caudal aortogram is easier to interpret than standard views and facilitates description and recognition of various coronary patterns. The relation between the pulmonary artery and the aorta, the origins of the coronary arteries from the facing sinuses and their proximity to the intercoronary commissures, and the myocardial distribution of each coronary vessel are shown clearly. The caudal view therefore offers significant advantages over conventional projections for demonstration of coronary arterial anatomy in infants with transposition of the great arteries or double-outlet right ventricle.

Renal findings in 40 individuals with Williams syndrome.

We tabulated the frequency of renal abnormalities in 40 Williams syndrome individuals presenting for medical and/or developmental assessment to a multi-disciplinary Williams syndrome program. The average age at time of assessment was 7 2/12 years. Seven individuals (7/40 = 18%) had abnormalities detected, including nephrocalcinosis = 2; marked asymmetry in kidney size = 2; small kidneys = 1; solitary kidney = 1; and pelvic kidney = 1. Renal function was also assessed. Two individuals had evidence of renal dysfunction, one secondary to nephrocalcinosis and the second due to hypercalcemia and interstitial nephritis of unclear pathogenesis. We examined the frequency of renal artery stenosis in 9 individuals who underwent abdominal angiography during cardiac catheterization. We found unilateral or bilateral mild renal artery narrowing in 4 individuals and normal renal arteries in the remaining 5. Persistent hypertension occurred in only 2 individuals and did not correlate with renal artery status. We conclude that intrinsic renal anomalies, as well as problems secondary to hypercalcemia, occur with sufficient frequency to warrant baseline renal screening in all individuals with Williams syndrome.

The dimensions of the right ventricular outflow tract and pulmonary arteries in tetralogy of Fallot and pulmonary stenosis.

Studies were undertaken of the cineangiograms in 196 consecutive patients entering two institutions with tetralogy of Fallot and pulmonary stenosis, none of whom had previously undergone a surgical procedure. The median age of the patients at the time of the study was 5.9 months. The diameters of the right ventricular infundibulum, pulmonary trunk, and the entirety of the right and left pulmonary arteries were measured (in millimeters), corrected for magnification, and expressed in standard deviation units (Z-values). The median values of the cineangiographically determined diameters of the right ventricular infundibulum and pulmonary trunk were smaller than those of 95% of normal individuals. The median values throughout the right and left pulmonary arteries were within the range of normal. Those of the distal branches of both the right and left pulmonary arteries were similar to the mean values in normal individuals. However, great variability of the dimensions between individuals, and along the pathway in individuals, characterized patients with tetralogy of Fallot. Diffuse narrowing of the pathways both proximally and distally was uncommon. The relation between the diameters of the pulmonary "anulus" and of the distal pulmonary trunk and origin of the left pulmonary artery explained the difficulty of extending an enlarging patch into a wide area distally in some patients.

Pediatric urologic radiology. Intervention and endourology.

Over the past 10 years new imaging and interventional techniques have drastically changed the ease and scope of urologic diagnosis and treatment. It is both rewarding and exciting to approach each clinical problem with a broad armamentarium of available studies, always seeking the most efficient and direct route to diagnosis. Similarly, radiologic interventional techniques are potentially applicable to a multitude of problems and should be innovatively considered in the urologic patient including patients in the pediatric age group.

Interventional procedures for congenital heart disease.

Interventional techniques available for use in treating congenital heart disease include balloon dilation of valves and vessels, stent placement and coil embolization of collaterals, patent ducts and other arterial fistulae. In addition, a variety of devices for closure of atrial and ventricular septal defects and patent ducts currently are under investigation. Radiofrequency ablation of arrhythmias also is applicable to the pediatric population.

Drainage of hepatic, intraabdominal, and mediastinal abscesses guided by computerized axial tomography. Successful alternative to open drainage.

Opens surgical drainage of intraabdominal, intrahepatic, and mediastinal abscess is well established. Although this may be the procedure of choice when there are indications for treatment of concurrent intraabdominal and mediastinal surgical disease, with the advent of computerized axial tomography and ultrasonography we have identified a patient population best served by percutaneous catheter drainage. The procedure involves precise localization using ultrasound or CAT scanning, fine needle aspiration for confirmation of diagnosis, and injection of radiopaque contrast medium with fluoroscopic observation to localize the abscess. Catheter placement is usually achieved by a Seldinger technique, although a trochar-cannula method is occasionally required. Our experience with 4 patients, as well as review of the world literature provides a cumulative experience of 252 patients, has led us to believe that this approach is successful 83 percent of the time with a minimal incidence of complications. Percutaneous catheter drainage of intrahepatic, intraabdominal, and mediastinal abscess guided by computerized axial tomography is the treatment of choice in patients who do not have other indications for exploration.

Interventional cardiac procedures in neonates and infants: state of the art.

The currently available interventional cardiac procedures in neonates and infants are at various stages of development. We currently dilate neonates and infants with critical valvular pulmonary and aortic stenosis and postoperative aortic obstruction. We do not routinely dilate native coarctation of the aorta because of the possibility of aneurysm formation, unless the neonate is very sick and acidotic and an operative approach is considered to be high risk. Balloon and blade atrial septostomy are done routinely whenever indicated with a low incidence of morbidity. Coil embolization, endomyocardial biopsy, foreign body retrieval and percutaneous pericardial drainage are relatively safe, and with the currently available instruments these techniques can be performed safely in neonates and infants with the same indications as for older patients. We currently consider stenotic pulmonary veins to be an undilatable lesion and an optimal therapy remains to be defined. Transcatheter closure of PDA and intracardiac shunts is presently limited to older patients, due to the large size of the delivery system devices and cannot currently be used in neonates. Dilation of the pulmonary valve in cyanotic congenital heart disease appears useful, but further experience is needed.

Chronic posttraumatic aortic pseudoaneurysm. Recognition before rupture.

Few patients survive transection of the aorta caused by blunt trauma. However, among those who do are a small number who go on to live with an unrecognized pseudoaneurysm that may rupture at any time. Because these aneurysms may be mistaken for more common disease processes, such as hilar adenopathy, atherosclerotic aneurysm, or neoplasia, the authors describe radiographic findings that suggest the correct diagnosis.

Aneurysm rupture secondary to transcatheter embolization.

False aneurysms of the pancreatic and peripancreatic arteries are a well recognized complication of chronic pancreatitis due to proteolytic enzymatic digestion of the arterial wall. These false aneurysms can be a source of life-threatening hemorrhage. Three cases are reported in which attempted embolizations of these bleeding aneurysms resulted in rupture into the gastrointestinal tract. Special precautions should be taken in such a procedure because of the inherent weakness of the aneurysmal wall.

Devices for transcatheter closure of intracardiac defects.

Transcatheter closure of intracardiac defects is an investigational procedure that is in use at a number of centers in North America and Europe. A radiologist should be able to recognize these devices on a chest radiograph, understand their actual physical appearance, and be able to recognize their expected location. This essay summarizes the indications for, technical aspects of, and radiologic appearance of these devices.

Gas within hemophiliac pelvic pseudotumors: CT demonstration.

Three hemophiliac patients are presented who had gas within their pelvic pseudotumors demonstrated by computed tomography. Etiologies of the gas included iatrogenically introduced gas from infection, failed tube drainage of the pseudotumor, and a communication between the pseudotumor and the bladder containing a Foley catheter.

Persistent sciatic artery: clinical, embryologic, and angiographic features.

The persistent sciatic artery is a rare but interesting and clinically pertinent vascular anomaly that may present as a buttock aneurysm or as ischemic or embolic disease. Its correct angiographic diagnosis depends on recognition of an abnormally large internal iliac artery, appropriate injection and adequate timing to fill and follow flow into the large vessel, and recognition and differentiation of the tapering superficial femoral artery from routine occlusive disease so that an accurate picture of lower leg runoff is provided.

Right ventricular decompression and left ventricular function in pulmonary atresia with intact ventricular septum. The influence of less extensive coronary anomalies.

BACKGROUND: Right ventricular decompression (RVD) may cause myocardial ischemia in patients with pulmonary atresia with intact ventricular septum and associated coronary artery abnormalities. Although we have previously shown that mortality is very high when two or more coronary arteries are obstructed, the effects of lesser degrees of coronary abnormalities are unknown. We therefore evaluated the effect of RVD on left ventricular (LV) function in those with less extensive coronary artery abnormalities. METHODS AND RESULTS: Preoperative cineangiograms demonstrated fistulas with or without one coronary artery stenosis in 12 of 24 patients aged 2 days to 33 months at the time of RVD. Preoperative and postoperative two-dimensional echocardiograms were analyzed for global and regional LV function. One infant with fistulas involving two coronary arteries and stenosis of the right coronary artery died from severe global LV dysfunction after RVD. Despite this, mean LV end-diastolic volume (66 +/- 17 mL/m2) and mean LV ejection fraction (60 +/- 9%) were similar in patients with and without coronary artery abnormalities before and after RVD. Before RVD, regional LV dysfunction was seen in 8 of 132 (6%) regions in those with coronary artery abnormalities and in 3 of 132 (2%) in those without coronary artery abnormalities. After RVD, there were 16 of 132 (12%) abnormal regions in those with coronary artery abnormalities and 1 of 132 (< 1%) in those without coronary artery abnormalities. In regions with normal wall motion before RVD, the presence of coronary artery abnormalities was related to regional LV dysfunction after RVD (P < .001). CONCLUSIONS: Regional LV dysfunction was rare in patients without coronary artery abnormalities. In those with less extensive coronary artery abnormalities not involving obstruction to multiple coronary arteries, regional LV dysfunction was common before and increased after RVD, but severe global LV dysfunction was unusual.

Fluoroscopic placement of jejunal feeding tubes.

Over a two-and-one-half year period, 50 children underwent placements of jejunal tubes through a nasal route (NJ, n = 47) or through an existing gastrostomy site (GJ, n = 119). There were four attempted placements (98% success rate). The NJ tubes remained in place an average of 13 days, and the GJ tubes remained in place an average of 37 days. Fluoroscopic time for placement of an NJ tube averaged 6 min (29 cases), and for a GJ tube 8 minutes (91 cases). In spite of the limited retention time, fluoroscopic time, and availability of alternative methods, fluoroscopically placed jejunal feeding tubes are still playing an active role in this institution.

Diagnosis and management of right ventricle-dependent coronary circulation in pulmonary atresia with intact ventricular septum.

BACKGROUND: Coronary artery anomalies including 1) right ventricle (RV)-to-coronary artery fistulas, 2) coronary artery stenoses, and 3) coronary occlusions occur in patients with pulmonary atresia with intact ventricular septum (PA-IVS). In some, a large part of the coronary blood supply may depend on the RV. This RV-dependent coronary circulation may determine survival after right ventricular decompression (RVD): RVD may cause RV "steal" in the presence of fistulas alone and ischemia, coronary isolation, or myocardial infarction in the presence of coronary stenoses. METHODS AND RESULTS: Eighty-two patients with PA-IVS who presented between January 1979 and January 1990 were reviewed; 26 (32%) had RV-to-coronary artery fistulas. Of these 26, 23 had adequate preoperative coronary angiograms for analysis. RVD was achieved in 16. Seven of 16 had fistulas only; each survived RVD. Six of 16 had stenosis of a single coronary artery [left anterior descending coronary artery (LAD), four; right coronary artery (RCA), two]; four of six survived RVD. Three of 16 had stenoses and/or occlusion of both the RCA and LAD; all three died shortly after RVD of acute left ventricular dysfunction. CONCLUSIONS: 1) Potential RV steal alone does not preclude successful RVD. 2) Fistulas with stenoses to a single coronary artery may not preclude successful RVD. 3) RVD appears to be contraindicated in the presence of stenoses and/or occlusion involving both the right and left coronary systems. Nonsurvival after RVD seems to depend on the amount of the left ventricular myocardium at risk, i.e., that which is distal to coronary artery stenoses, especially when involvement of both coronary arteries limits effective collateralization. Precise definition of coronary arterial anatomy is mandatory in neonates with PA-IVS.

Management of failing prosthetic bypass grafts with metallic stent placement.

PURPOSE: To evaluate the role of metallic stents in treating stenoses involving prosthetic arterial bypass grafts. METHODS: Patients undergoing stent placement within a failing prosthetic bypass graft, during a 41-month period, were reviewed for treatment outcome and complications. The indications for stent placement in 15 patients included severe claudication (n = 3), rest pain (n = 9), and minor or major tissue loss (n = 3). Lesions were at the proximal anastomosis (n = 6), the distal anastomosis (n = 3), or within the graft (n = 6). RESULTS: Treatment with metallic stents was successful in all patients. There was one acute stent thrombosis, successfully treated with thrombolytic therapy. Follow-up data are available for a mean duration of 12.3 months. The mean duration of primary patency was 9.4 months with 6- and 12-month primary patency rates of 51.9% and 37.0%, respectively. The mean duration of secondary patency was 12.1 months with 6- and 12-month secondary patency rates of 80.0% and 72.7%, respectively. Two patients with discontinuous runoff and preexisting gangrene required a below-knee amputation. Six patients were revised surgically after stent placement (at a mean of 10.8 months). Three late deaths occurred during follow-up. CONCLUSION: Given the mortality risks of surgical revision and the reduced life expectancy of this patient population, metallic stent placement represents a viable, short-term treatment option for stenoses within or at the anastomoses of prosthetic grafts. Further evaluation is warranted to compare intragraft stent placement with surgical graft revision.