Radiological case of the month. Coarctation of the aorta in Hurler syndrome.
Explore the source record for details and available documents.
Aortic Coarctation↗
Biomedical subjects
A P Rocchini
Publications and source records attributed to A P Rocchini.
Congenital Heart Surgery Nomenclature and Database Project: therapeutic cardiac catheter interventions.
The extant nomenclature for therapeutic cardiac catheter interventions is reviewed for the purpose of establishing a unified reporting system. The subject was debated and reviewed by members of the STS-Congenital Heart Surgery Database Committee and representatives from the European Association for Cardiothoracic Surgery. All efforts were made to include all relevant nomenclature categories using synonyms where appropriate. A comprehensive database set is presented which is based on a hierarchical scheme. Data are entered at various levels of complexity and detail which can be determined by the clinician. These data can lay the foundation for comprehensive risk stratification analyses. A minimum database set is also presented which will allow for data sharing and would lend itself to basic interpretation of trends. Outcome tables relating diagnoses, procedures, and various risk factors are presented.
Cardiac Catheterization↗
Impact of arrhythmia circuit cryoablation during Fontan conversion for refractory atrial tachycardia.
Refractory atrial arrhythmias in late postoperative Fontan patients are usually associated with residual hemodynamic abnormalities and result in significant morbidity and mortality. Surgical revision of the Fontan anastomosis may improve hemodynamics without eliminating tachycardia. This study sought to assess the impact of surgical cryoablation of the arrhythmia circuit at the time of Fontan conversion on the clinical recurrence of tachycardia. Sixteen consecutive atriopulmonary Fontan patients with refractory atrial arrhythmias underwent surgical conversion to lateral tunnel total cavopulmonary anastomosis (15) or Fontan revision (1 patient). The initial 4 patients underwent Fontan conversions alone, without specific arrhythmia surgery. The subsequent 12 patients underwent electrophysiologically guided cryoablation of the tachycardia circuits at the time of surgical conversion. The mean age at Fontan revision was 15.6 +/- 3.8 years. Cryoablation was directed to 3 identified major tachycardia circuits: the inferomedial right atrium, the superior rim of the prior atrial septal defect patch, and along the lateral right atrial wall. Transmural antitachycardia pacemakers were implanted in 11 of the 16 patients. There was no surgical mortality in either group, and all patients improved in functional classification. All patients not undergoing cryoablation experienced recurrent symptomatic tachycardia requiring antiarrhythmic therapy (median follow-up, 54 months) versus 2 of 12 patients receiving cryoablation (median follow-up, 25 months; p <0.02). Thus, surgical cryoablation of the arrhythmia circuit at the time of Fontan conversion is highly effective in the management of refractory atrial arrhythmias, and is superior to Fontan conversion alone.
Adolescent↗
Snare-assisted vascular access: a new technique.
Access to the central circulation can be difficult in small infants, particularly when normal anatomic landmarks have been altered. We describe a new technique that utilizes any existing central catheter to establish additional sites of vascular access. A 4 Fr end hole catheter is advanced under fluoroscopic guidance to the desired site of new vascular access. A 10-mm Amplatz snare catheter is advanced through the end hole catheter and the loop opened within the target vessel lumen. The snare is then used to guide percutaneous placement of a Cope wire through a 21-gauge needle and then to pull the wire into the central circulation. New access is then achieved by introducing an additional catheter over the guidewire. This technique has now been successfully utilized 16 times in 13 patients. Snare assistance is a safe and effective technique that provides a reliable means of establishing additional secure vascular access. Cathet. Cardiovasc. Intervent. 47:315-318, 1999.
Catheterization↗
Perventricular [correction of Periventricular] closure of ventricular septal defects without cardiopulmonary bypass.
BACKGROUND: Minimally invasive techniques are currently in use to close atrial and ventricular septal defects (VSD). Cardiopulmonary bypass (CPB) is instituted via the femoral vessels, which may cause injury to these vessels, especially in younger patients. The objectives of this study were to demonstrate the feasibility of perventricular [corrected] closure of muscular VSD (MVSD) and paramembranous VSD (PVSD) without CPB, using the Amplatz VSD device. METHODS: Five Yucatan pigs with naturally occurring PVSD (3- to 7-mm diameter) and 5 dogs with surgically created MVSD (6- to 14-mm diameter) were subjects of this study. The VSDs were closed intraoperatively with a 7-French delivery sheath inserted through the free wall of the right (n = 5) or left ventricle (n = 5), under epicardial echocardiogram guidance. The animals were followed for 3 months. RESULTS: There was no operative mortality. All MVSD closed after placement of the device. Closure rate of PVSD was 4 of 5 after placement and 3 of 5 after 3 months. One pig developed aortic incompetence at the last follow-up. CONCLUSIONS: Perventricular closure of MVSD and PVSD is feasible. Avoidance of CPB can decrease recovery time, its complications, and trauma to the femoral vessels.
Animals↗
Left ventricular growth in selected hypoplastic left ventricles: outcome after repair of coarctation of aorta.
BACKGROUND: Models that predict survival in neonates with left ventricular hypoplasia and critical aortic stenosis may not be applicable to neonates with left ventricular hypoplasia and coarctation. METHODS AND RESULTS: We report 8 infants with severe aortic coarctation and left ventricular hypoplasia. Mean age was 18 days (range 1-48 days), and mean weight was 3.5 kg (range 2.7-4.3 kg). Associated diagnoses included mild aortic stenosis (4), ventricular septal defect (2), and venous anomalies (2). All had coarctation repair as a primary procedure (3 of these had concomitant intracardiac procedures); 7 had subsequent operations. All are alive and well 1.1-6.7 years (mean 3.1 years) after the first surgery. Progressive increases were observed in aortic and mitral diameters, and in left ventricular dimensions, areas, and volumes when the preoperative, earliest postoperative, and most recent echocardiograms were compared. CONCLUSIONS: Despite severe left ventricular hypoplasia, a two-ventricle repair is possible in selected cases. The prognostic criteria for left ventricular hypoplasia in critical aortic stenosis may not be applicable to infant coarctation. Relief of coarctation may result in the growth of the very small left ventricle, especially when the aortic root and mitral diameters are satisfactory.
Aortic Coarctation↗
Lipoprotein (a): a controversial risk factor for atherosclerotic heart disease.
Explore the source record for details and available documents.
Coronary Artery Disease↗
Raising heart-healthy children.
The current article will describe how the cardiovascular risk factors of obesity, increased blood pressure, hyperlipidemia, cigarette smoking, poor physical fitness and prenatal environment impact the development of cardiovascular disease and what types of therapy can be used in the pediatric patient to modify them.
Cardiovascular Diseases↗
Clonidine prevents insulin resistance and hypertension in obese dogs.
The role that the central sympathetic nervous system plays in the development of obesity hypertension and insulin was evaluated by feeding dogs a high fat diet with or without clonidine treatment. Thirteen adult mongrel dogs were chronically instrumented and randomly assigned to receive either a high fat diet and no clonidine (n=6) or a high fat diet plus clonidine (n=7), 0.3 mg BID. Blood pressure, heart rate, plasma insulin, and electrolytes were measured daily. Insulin resistance was assessed with a multiple-dose euglycemic clamp (1, 2, and 30 mU. kg-1. min-1) before and after 1, 3, and 6 weeks of the high fat diet. Clonidine prevented the hypertension, tachycardia, and insulin resistance associated with feeding dogs the high fat diet but did not affect weight gain. The present study suggests that the central sympathetic nervous system plays a critical role in the development of both insulin resistance and hypertension associated with feeding dogs a high fat diet.
Animals↗
Repair of mitral valve and subaortic mycotic aneurysm in a child with endocarditis.
Endocarditis requiring surgical intervention in children is uncommon. Individualized operative therapy must be fashioned to the particular pathology of each case. In this case we describe mitral anterior leaflet homograft patch augmentation valvuloplasty, subaortic homograft patch closure of a large mycotic aneurysm, and homograft aortic root replacement in a 3-year-old patient with endocarditis after remote repair of complete atrioventricular canal.
Aneurysm, Infected↗
Surgical repair of the congenitally malformed mitral valve in infants and children.
BACKGROUND: Mitral valve remodeling techniques were applied to 26 infants and children (mean age, 6.0 years, range, 0.4 to 15.9 years) with various forms of congenital mitral valve disease over a 7-year period. Patients with atrioventricular canal, L-transposition and single ventricle were excluded. Intraoperative transesophageal echocardiography (TEE) was utilized to assess the repair and guide the need for immediate intervention. METHODS: Twenty-one patients had mitral regurgitation: 10 with cleft anterior mitral leaflet, 7 with annular dilatation, 1 with normal leaflets with an obstructing cord, 2 with prolapsed leaflets and elongated cords, and 1 with restricted leaflet motion, normal papillary muscles, and shortened cords. Of the 5 mitral stenosis patients, 3 had supravalvular mitral ring, 1 had midvalvular mitral ring, and 1 had a parachute valve. Three of the mitral stenosis patients had additional stenotic lesions. Concurrent repair of associated lesions was performed in 21 patients (78%). RESULTS: Operative mortality was 3.8% (n = 1). There were no late deaths. Immediate rerepair in 4 patients resulted in improved function. All mitral stenosis patients improved. A total of 20 mitral regurgitation patients (95%) improved; 1 required mitral valve replacement. Mean follow-up is 31 months (range, 2 to 81 months). All patients are in New York Heart Association functional class I or II. CONCLUSIONS: Mitral valve repair can be successfully performed in infants and children with excellent short- and midterm results. Assessment using transesophageal echocardiography can guide the necessity for immediate rerepair to achieve improved function.
Adolescent↗
Intraoperative closure of muscular ventricular septal defect in a canine model and application of the technique in a baby.
Explore the source record for details and available documents.
Animals↗
Coronary artery fistulas in infants and children: a surgical review and discussion of coil embolization.
BACKGROUND: Coronary artery fistula (CAF) is a rare congenital anomaly that can be complicated by intracardiac shunts, endocarditis, myocardial infarction, or coronary aneurysms. Recent reports have emphasized the efficacy of percutaneous transcatheter techniques. The purpose of this article is to review a 28-year surgical experience with CAF as a standard for comparison and to discuss the emergence and efficacy of transcutaneous catheter coil embolization as an alternative form of therapy. METHODS: From 1968 to 1996, 17 patients (age, 6 weeks to 16.5 years; mean age, 5.5 years) were diagnosed with CAF: 8 of 12 by echocardiography and 17 of 17 by cardiac catheterization. All patients with isolated CAF (n = 13) were asymptomatic despite significant clinical, electrocardiographic, and chest roentgenographic findings in 10. Sixteen had congenital CAF and 1 had acquired CAF after tetralogy of Fallot repair with injury of the anomalous left anterior descending coronary artery. Associated anomalies included tetralogy of Fallot (2), atrial septal defect (1), and patent ductus arteriosus (1). Nine fistulas originated from the right coronary artery and eight from the left. Drainage was to the right ventricle (9), right atrium (4), pulmonary artery (3), and left atrium (1). RESULTS: All patients had a median sternotomy with epicardial or endocardial ligation. Cardiopulmonary bypass was used in 8; 1 of these (iatrogenic CAF) required distal internal mammary artery bypass graft. There were no operative or late deaths. Follow-up evaluation by physical examination (17), echocardiography (8), and catheterization (2) showed no evidence of recurrent or residual CAF. A retrospective review of the 16 available cine cardioangiograms showed that coil embolization was possible in, at most, 6 patients. CONCLUSIONS: Early surgical management of CAF is a safe and effective treatment resulting in 100% survival and 100% closure rate. Transcatheter embolization is a reasonable alternative to standard surgical closure in only a very small, select group of patients. These surgical results should be considered the standard against which transcatheter techniques are compared.
Adolescent↗
Time course of insulin resistance associated with feeding dogs a high-fat diet.
The current study evaluated both the time course of insulin resistance associated with feeding dogs a high-fat diet and the relationship between the development of insulin resistance and the increase in blood pressure that also occurs. Twelve adult mongrel dogs were chronically instrumented and randomly assigned to either a control diet group (n = 4) or a high-fat diet group (n = 8). Insulin resistance was assessed by a weekly, single-dose (2 mU.kg-1.min-1) euglycemic-hyperinsulinemic clamp on all dogs. Feeding dogs a high-fat diet was associated with a 3.7 +/- 0.5 kg increase in body weight, a 20 +/- 4 mmHg increase in mean blood pressure, a reduction in insulin-mediated glucose uptake [(in mumol-kg-1.min-1) decreasing from 72 +/- 6 before to 49 +/- 7 at 1 wk, 29 +/- 3 at 3 wk, and 30 +/- 2 at 6 wk of the high-fat diet, P < 0.01]. and a reduced insulin-mediated increase in cardiac output. In eight dogs (4 high fat and 4 control), the dose-response relationship of insulin-induced glucose uptake also was studied. The whole body glucose uptake dose-response curve was shifted to the right, and the rate of maximal whole body glucose uptake was significantly decreased (P < 0.001). Finally, we observed a direct relationship between the high-fat diet-induced weekly increase in mean arterial pressure and the degree to which insulin resistance developed. In summary, the current study documents that feeding dogs a high-fat diet causes the rapid development of insulin resistance that is the result of both a reduced sensitivity and a reduced responsiveness to insulin.
Animals↗
Taussig-Bing anomaly: arterial switch versus Kawashima intraventricular repair.
BACKGROUND: Current corrective surgical approaches for the Taussig-Bing heart include arterial switch with ventricular septal defect (VSD) closure and intraventricular repair as described by Kawashima. METHODS: Between 1983 and 1994, 20 children underwent intracardiac repair of Taussig-Bing anomaly. Mean age at operation was 17 months (range, 1 week to 9 years). Prior palliation included pulmonary artery band (15) with coarctation repair (8) and atrial septectomy (1). Arterial switch with VSD closure was performed in 16 patients, 10 with anteroposterior great arteries. Kawashima repair was performed in 4 patients, all with side-by-side great arteries. RESULTS: After arterial switch, there was one operative death (6.2%) due to myocardial ischemia and three late deaths (18.7%) due to pulmonary hypertension, gastrointestinal bleeding, and acute lymphocytic leukemia. In the Kawashima repair group there have been no deaths. After arterial switch, 9 patients underwent 11 reoperations for residual coarctation (3), residual pulmonary artery stenosis (2), aortic valve replacement, aortic valvuloplasty, unrecognized VSD, mitral valvuloplasty, mediastinitis, and pacemaker insertion. After Kawashima repair, 1 patient underwent reoperation for baffle stenosis and 1 for an unrecognized VSD. CONCLUSIONS: For children with Taussig-Bing anomaly, the Kawashima intraventricular repair (for side-by-side great arteries) preserves the native aortic valve and avoids coronary dissection. The arterial switch operation with VSD closure can be applied without ventriculotomy to all great artery relationships and allows neonatal repair with or without concomitant coarctation repair. Both techniques yield excellent early and intermediate-term results despite the high rates of prerepair palliation and postrepair reoperation for both groups.
Aortic Coarctation↗
Metabolic and hemodynamic effects of a graded intracoronary insulin infusion in normal and fat anesthetized dogs: a preliminary study.
This study evaluated both cardiac hemodynamic and metabolic effects of a graded intracoronary artery infusion of insulin in four normal and five obese anesthetized dogs. Dogs were anesthetized with isoflurane, a catheter was advanced under fluoroscopic guidance into the coronary sinus and great cardiac vein, and a 20-MHz 3F coronary Doppler catheter was advanced into either the mid left anterior descending or circumflex coronary artery. A graded intracoronary insulin infusion was administered (starting at 0.3 mU/min and doubling every 40 minutes until a maximum dose of 2.4 mU/min was achieved). Coronary glucose extraction, coronary blood flow velocity, and coronary artery size were measured at each infusion rate. An intracoronary artery infusion of insulin stimulated myocardial glucose uptake in normal dogs. However, in high-fat-fed dogs, weight gain was associated with a reduction in the ability of insulin to promote glucose uptake by cardiac muscle and a rightward shift in the dose-response curve. In normal dogs, an intracoronary insulin infusion resulted in an increase in coronary blood flow and coronary vasodilation (with insulin coronary vascular resistance index decreases to 0.72 +/- 0.06, P<.01), whereas with weight gain the vasodilator response to insulin was lost. The loss of coronary artery vasodilation to local hyperinsulinemia in fat-fed dogs is consistent with other reports in obese or hypertensive humans that document an impairment in the action of insulin to increase skeletal muscle blood flow.
Animals↗
Relationship between insulin resistance and abnormal lipid profile in obese adolescents.
OBJECTIVE: To determine whether the lipid abnormalities observed in obese adolescents are associated with insulin resistance. METHODS: We evaluated the relationship between lipid levels and insulin resistance in 82 obese adolescents. Insulin resistance was assessed by fasting insulin level and sum of the insulin values after an oral glucose tolerance test in all 82, and were compared with data from 40 nonobese adolescents. Whole-body glucose uptake during euglycemic hyperinsulinemia (M value) was performed in 19 of the obese adolescents and compared with that of 24 nonobese young adults. RESULTS: The obese adolescents had significantly elevated low-density lipoprotein cholesterol (LDL-C) (3.09 +/- 0.73 mmol/L; 119 +/- 28.2 mg/dl) and triglycerides (1.22 +/- 0.62 mmol/L; 108 +/- 54.6 mg/dl) and low high-density lipoprotein cholesterol (HDL-C) levels (0.94 +/- 0.24 mmol/L; 36 +/- 9.1 mg/dl) when compared with values in the nonobese subjects. M values were significantly depressed in the obese compared with the nonobese subjects. Adiposity significantly correlated with low HDL-C and elevated triglyceride values. From the variables representing insulin resistance, the strongest correlation with the abnormal lipid profile was found for the M value. A stepwise multiple regression analysis revealed that the M value was the only step entered into the relationship for triglycerides and LDL-C, and both M value and fasting insulin were entered for HDL-C. CONCLUSION: In obese adolescents the degree of insulin resistance explains a significant portion of the variance in the levels of triglycerides, LDL-C, and HDL-C.
Adolescent↗
Insulin resistance, obesity and hypertension.
Although controversy exists as to the role that insulin resistance and hyperinsulinemia play in the pathogenesis of hypertension, data are presented that strongly suggest that selective insulin resistance and hypertension are directly related. This paper summarizes how insulin resistance may be linked to obesity hypertension and describes some of the other cardiovascular implications that insulin resistance may have in the hypertensive obese individual.
Animals↗