Biomedical subjects
Publications and source records attributed to P E Burrows.
One case of horseshoe lung with associated scimitar syndrome is presented. Unusual bronchial and pleural anomalies as delineated by CT and plain chest radiographic imaging are described. The presence of bilateral fissures led to a newly proposed classification of horseshoe lung based on pleural anatomy.
A heart specimen with double-orifice tricuspid valve is described. The tricuspid valve was divided into the anterior and posterior orifices by a bridge of leaflet tissue. The valve was stenotic because of the arcade deformity of the anterior papillary muscle to which the bridging leaflet tissue had short chordal insertions. The anterior orifice was regurgitant as a result of a deficient septal leaflet. Clinical correlation was obtained by magnetic resonance imaging of the specimen. The functional significance of the malformed valve was assessed by three-dimensional reconstruction of the two-dimensional magnetic resonance images.
OBJECTIVES: The objectives of this study were to determine the anatomic and physiological factors most responsible for the severe symptoms and poor prognosis of infants with scimitar syndrome. BACKGROUND: Whereas the diagnosis of scimitar syndrome is often made incidentally in older children and adults who undergo chest radiography for diverse reasons, infants in whom the diagnosis is made typically present with severe symptoms and have a poor prognosis. METHODS: The clinical, catheterization and imaging data of 13 consecutive infants with scimitar syndrome who underwent cardiac catheterization in the 1st 6 months of life were reviewed, with emphasis on the pulmonary artery pressure, pulmonary and cardiovascular anatomy, therapeutic interventions and outcome. RESULTS: Twelve of the 13 infants had pulmonary hypertension at the time of diagnosis. Six patients died despite specific treatment. Eleven of 13 infants had associated cardiac malformations and 9 had large systemic arterial collateral channels to the right lung. Seven patients had anomalies involving the left side of the heart, especially varying degrees of hypoplasia of the left heart or aorta, and six of these patients died. Ten patients underwent surgical or transcatheter therapy in the 1st year of life. Systemic arteries to the right lung were ligated in three patients and occluded by transcatheter embolization in four. Balloon angioplasty was carried out in two patients, one with stenosis of the left-sided pulmonary veins and one with stenosis of the anomalous right pulmonary vein. The latter had placement of a balloon-expandable stent. In both patients, pulmonary vein stenosis progressed. Six patients had surgical repair of associated cardiovascular anomalies, and two required repair of extracardiac congenital anomalies. Occlusion of the anomalous systemic arteries was generally associated with clinical improvement, but congestive heart failure and pulmonary hypertension recurred in those patients with associated cardiovascular anomalies, whose condition subsequently responded after correction of the shunt lesions. CONCLUSIONS: The severe symptoms and pulmonary hypertension found in infants with scimitar syndrome have many causes. Anomalous systemic arterial supply, pulmonary vein stenosis and associated cardiovascular anomalies play a significant role, and the ultimate outcome of individual infants depends on the feasibility of treating these anomalies in early infancy.
Magnetic resonance imaging (MRI) is an ideal imaging modality for investigating anomalies of the pediatric thoracic aorta. Its multiplanar capabilities allow imaging along anatomic or perpendicular planes, and clearly show the relationship between the great vessels and the airway structures. The most common pediatric aortic lesions investigated by MRI include vascular rings and coarctation of the descending aorta and its surgical complications. Other obstructive, aneurysmal, and positional anomalies of the aorta are readily demonstrated and quantified. However, precise image prescription is necessary to obtain optimal information. This review discusses and illustrates the common pediatric anomalies and current appropriate choices of imaging techniques.
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The clinical impact of percutaneous balloon angioplasty on the management of patients with native or postoperative pulmonary arterial stenosis was reviewed. Seventy-four patients underwent 110 angioplasty procedures. Mean age at dilation was 6.7 +/- 5.3 years (range 0.2 to 18.1), 17 patients were aged less than 1 year, mean follow-up was 37.7 +/- 22.8 months (range 16 to 96), and 34 patients (44%) had follow-up angiography. Pulmonary artery dilation was acutely successful in 53% of patients, 17% had recurrent stenosis, and 5% had complications. The impact on subsequent care was favorably influenced in 26 of 74 patients (35%) with either complete resolution of stenosis (n = 7), optimizing future surgical conditions (n = 14), reduction in right ventricular pressure by greater than 20% (n = 3), or improvement of ipsilateral lung perfusion (n = 2). No patient previously considered inoperable was subsequently considered suitable for surgical repair owing to the intervention. No correlation was found between success and cardiac diagnosis (p = 0.48), site of stenosis (p = 0.78), balloon-vessel ratio (p = 0.42), or whether the stenotic area consisted of native or synthetic material (p = 0.22). No predictive factors for success could be defined, and often there was only a transient clinical impact. Due to the low complication risk and potential for a beneficial result, it still appears prudent to offer angioplasty as an initial therapeutic modality in this setting.
Transjugular liver biopsy is widely used in adult patients with liver disease when transthoracic needle liver biopsy is contraindicated by severe coagulopathy or ascites. It has not been used extensively in children. We report our experience with 30 consecutive transjugular liver biopsy procedures performed in 27 young patients at the Hospital for Sick Children in Toronto. Patient weights ranged from 14 to 91 kg (median = 42 kg); most patients had not yet attained adult size. A 9F catheter was used except in very small children, for whom we developed a 7F biopsy needle catheter. Most procedures were done with patients under general anesthesia. Specimens were obtained in all patients, and the procedure was tolerated well. A major complication occurred only once: perforation of the inferior vena cava, which was later repaired at liver transplantation. Minor complications included subcapsular extravasation of contrast agent in five patients and a small intrahepatic hematoma in one. Results of transjugular liver biopsy changed the diagnosis in 30% of patients and added valuable information about the disease process in most patients. Transjugular liver biopsy was an important component of emergency assessment for liver transplantation. Our results indicate that transjugular liver biopsy can be performed safely in children with liver disease if a skilled interventional radiologist and a well-equipped angiography suite are available. Histological studies in these patients enhance our understanding of the natural history of pediatric liver diseases.
This report describes a case of fistula between the left circumflex coronary artery and the left atrium, which was identified by color flow mapping. This finding was confirmed by selective coronary arteriography.
OBJECTIVES: This study was designed to assess the impact of implantation of balloon-expandable stents on right ventricular outflow obstruction in children with congenital heart disease. BACKGROUND: Intravascular stenting has been established as a useful treatment in adults with coronary and peripheral vascular disease. Its application in the treatment of infants and children with pulmonary, systemic and right ventricular conduit obstruction resistant to balloon angioplasty is limited. METHODS: A total of 24 stainless steel stents were implanted in 17 patients. Five stents were placed within right ventricular to pulmonary artery conduits, 17 in branch pulmonary arteries and 1 in an aortopulmonary collateral vessel. Follow-up time has ranged from 1 to 14 months, with 6 patients having hemodynamic and angiographic studies greater than 1 year after stent placement. The mean age at implantation was 7.4 +/- 5.6 years and the mean weight 33 +/- 16 kg. RESULTS: Optimal stent position was obtained in 22 of 24 implantations. In one patient the stent slipped from the delivery balloon and was left positioned in the inferior vena cava. No embolization or thrombotic event has been documented. Among patients with right ventricular to pulmonary artery conduit obstruction, the gradient was immediately reduced from 85 +/- 30 mm Hg to 35 +/- 20 mm Hg after stent implantation; however, three patients required conduit replacement because of persistent obstruction with elevated right ventricular pressures (82 +/- 16 mm Hg). In 10 of 11 patients with pulmonary artery stenosis, clinical improvement was noted in association with enlargement of vessel diameter by 92% +/- 90% (range 17% to 355%) and the gradient reduction of 22 +/- 24 mm Hg to 3 +/- 4 mm Hg. CONCLUSIONS: These data support the view that intravascular stenting will become an important adjunct in the management of children with congenital heart disease.
Priapism may be classified as the classical veno-occlusive form or the more recently described high flow, arterial type. We report a case of posttraumatic arterial priapism in a 7-year-old boy. The successful management with percutaneous transcatheter embolization of the internal pudendal artery is described.
This report describes four infants with hepatic hemangioendotheliomas and cardiac failure who had extensive portal venous and systemic collateral arterial supply complicating hepatic arterial embolization. One patient with diffuse hepatic hemangioendothelioma and extensive portal vein supply but minimal systemic collateral arteries showed no improvement after technically successful hepatic artery embolization and died with disseminated intravascular coagulation and sepsis. A second infant with extensive portal vein and collateral supply died without undergoing embolization. Two patients had portal vein-hepatic vein fistulas as well as an extensive systemic arterial supply. Both infants tolerated staged hepatic and collateral artery embolization, although one patient died of congestive heart failure, probably related to pulmonary hemangiomas. The authors conclude that angiographic study of the potential collateral vessels and portal venous circulation should be performed before embolization. Patients with shunting from the portal vein to the hepatic vein and minimal systemic arterial collateral circulation should not undergo hepatic artery embolization.
Twelve children were identified with subaortic stenosis after Fontan's operation. All had absent resting and isoproterenol-provoked pressure gradient before the Fontan procedure. Six had a univentricular heart of left ventricular morphology, three had a single ventricle of right ventricular morphology, one had tricuspid atresia with transposition of the great arteries, one had pulmonary atresia, intact ventricular septum, and hypoplastic right ventricle, and one had corrected transposition with hypoplastic systemic ventricle. The median interval between the Fontan operation and the recognition of subaortic stenosis was 2.5 years. Ten patients underwent surgical treatment after a prior Fontan operation: Five had myectomy and enlargement of ventricular septal defect with two operative deaths; two had placement of a valved conduit from the ventricular apex to the descending aorta, and both died postoperatively; two with single ventricle had subaortic myectomy, and one had enlargement of ventricular septal defect and pulmonary aortic connection. Complete heart block developed in only one patient. Postoperative testing with Doppler echocardiography with color flow imaging demonstrated good relief of subaortic stenosis. All six children who survived the operation are well 4 months to 4 years later. Subaortic stenosis is a progressive lesion that may develop after a Fontan operation. Its surgical treatment continues to carry a significant mortality. Myectomy and enlargement of ventricular septal defect achieve direct relief of the obstruction with minimal risk of heart block.
This study reviews the clinical course of 104 consecutive patients with pulmonic valve atresia and ventricular septal (VSD) defect who were diagnosed in the first year of life and followed for a mean period of 4.95 years (range 2 days to 13.75 years). Specific attention was paid to the nature of the pulmonary blood supply and to its influence on patient outcome. Confluent pulmonary arteries supplied by a single ductus arteriosus were present in 72 patients (69%, group I), whereas 32 patients (31%, group II) had a pulmonary blood supply that was partially or exclusively dependent on systemic collateral arteries. An estimate of the probability of survival for 10 years was 69% in the entire cohort, with no different between patients in group I and group II. Definitive surgical repair was performed in 33 of 72 group I patients (46%), compared with 5 of 32 group II patients (16%). Arborization and distribution abnormalities of the pulmonary arteries as well as intrapulmonary stenoses that were exclusively present in patients with systemic collateral arteries (p less than 0.00001) accounted for the significantly lower probability of undergoing corrective surgery in group II patients.
The clinicopathological features of five fatal cases of diffuse haemangiomatosis presenting in neonatal life or early infancy are presented. The infants all had multiple skin haemangiomas as well as deep-seated lesions in many different tissues that caused protean clinical manifestations and management problems. Because the outlook may be improved by early diagnosis and application of new modes of treatment, any infant with multiple cutaneous haemangiomas should be closely assessed for possible visceral involvement. Development of hepatomegaly, high-output cardiac failure, unexplained anaemia or thrombocytopenia in these infants should immediately suggest disseminated disease. Early recognition with implementation of steroid and/or antiangiogenic therapy, embolization and/or surgery is essential to improve the chances of survival.
A retrospective review was undertaken to determine the incidence of, and radiologic findings associated with aortic rupture resulting from blunt chest trauma in children. Records and imaging data of 54 consecutive pediatric patients admitted over a 2 year period to a pediatric trauma center after sustaining blunt chest trauma were reviewed. Four of 54 (7.4%) had a documented aortic tear. Plain films were evaluated for 7 radiographic signs described in the adult literature as sensitive indicators of aortic rupture, including abnormal aortic contour and mediastinal widening. Two groups of patients were defined: Group 1 (n = 4) had aortic rupture confirmed by angiography or operation and Group 2 (n = 50) with no angiographic investigation. All patients in Group 1 demonstrated mediastinal widening and abnormal aortic contour; however, 50 percent of patients in Group 2 had similar findings. Computed tomograms of the thorax where obtained were reviewed, including 1 patient from Group 1 and 6 patients from Group 2. The aortic tear was well demonstrated in the one patient from Group 1; however, the remaining computed tomograms were deemed inadequate for reliable exclusion of significant aortic injury. Plain chest radiographic findings in 5 consecutive children who underwent aortography in the two years subsequent to this series, including 2 additional patients with aortic rupture, were also reviewed, with similar results. In conclusion, traumatic aortic rupture in the pediatric population may be more common than previously reported. Plain film findings of aortic rupture in children are similar to those in adults, and are sensitive but non-specific. Currently, at least in our institution, this injury may be underinvestigated. Angiography remains the modality of choice in the diagnosis of aortic tears in children.(ABSTRACT TRUNCATED AT 400 WORDS)
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We report our experience with the use of tissue plasminogen activator to treat 12 infants and children with various thromboembolic states after conventional thrombolytic agents had failed. The dosage range was between 0.1 to 0.5 mg/kg per hour. Complete clot dissolution occurred in seven cases after 2 hours to 3 days of therapy. Partial clot dissolution and clinical improvement were noted in another four patients. Bleeding complications were noted in 6 of the 12 patients and included bruising, oozing from various venipuncture sites, and bleeding; these complications were controlled by clinically available means. In all cases with bleeding the dose rate was in the higher range (0.46 to 0.50 mg/kg per hour). In one patient, restlessness, agitation, and screaming were noted during administration of tissue plasminogen activator and when it was reinstituted. We conclude that tissue plasminogen activator is effective in inducing clot lysis in children. Because the effective dose appears to overlap with those causing bleeding, we recommend that a dose of 0.1 mg/kg per hour be started and increased gradually if clot dissolution does not occur, with close monitoring for bleeding.
To determine the morphologic sequelae after surgical repair of coarctation of the aorta, the authors retrospectively reviewed angiograms and hemodynamic and clinical data on 215 patients who underwent cardiac catheterization after surgical repair of coarctation of the aorta during a 13-year period. Ninety-seven patients (45%) underwent coarctation resection with end-to-end anastomosis; 92 (43%), subclavian-flap angioplasties; and 26 (12%), synthetic-patch repairs. Sixty-four patients (30%) had an "aneurysm," defined as a measurement ratio of repair site to diaphragmatic aorta greater than 1.5. Transverse-arch or isthmic hypoplasia or recoarctation was detected in 86 patients (40%) and was most commonly associated with septal defects or obstruction of the left ventricular outflow tract. Pullback systolic pressure gradients at catheterization were significantly higher (P = .0001) in the patients with transverse-arch hypoplasia and recoarctation than in those with ratios of transverse arch to diaphragmatic aorta greater than 0.9. Significant postoperative arch obstructions can be predicted with measurement ratios on the basis of the diameter of the abdominal aorta.