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ABSTRACT: Papillary fibroelastoma (PFE) is a rare and histologically benign tumor, but it may have malignant propensity for life-threatening complications. Herein are described four cases of PFE which reflect the clinical diversity of this lesion. The diagnostic and surgical approach utilized is also briefly reviewed.
The files of four patients with cardiac valvular PFE treated at the authors' center between January 2002 and November 2003 were reviewed. The diagnosis was strongly suggested by echocardiography. Tumors were noted in aortic (n = 2), mitral (n = 1) and tricuspid (n = 1) sites. Indications for surgery were myocardial infarction (both aortic tumors), previous stroke (mitral tumor), and preventive (tricuspid tumor).
Surgical excision with a conservative, valve-sparing approach was performed in all cases. For the first aortic tumor, the aortic valve was reconstructed with a patch of autologous pericardium. None of the patients had evidence of valvular regurgitation after excision on intraoperative transesophageal echocardiography, and all had an uneventful recovery. There were no cases of recurrence or regurgitation on follow up echocardiography.
PFE is an uncommon but increasingly recognized cause of embolic phenomena. Prompt identification allows for surgical excision, which seems to be curative, safe and well-tolerated. A conservative valve-sparing approach is recommended because of the absence of recurrence after total excision.
The Journal of heart valve disease 12/2005; 14(6):843-7. · 0.81 Impact Factor
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ABSTRACT: We present a 3-year-old boy born with anomalous hepatic venous drainage into the left atrium and a small sinus venosus atrial septum defect, in whom pulmonary arteriovenous malformations developed with progressive cyanosis. Surgical redirection of the hepatic venous drainage to the right atrium and closure of the atrial septal defect led to regression of the pulmonary arteriovenous malformations. However, in contrast to other reports, progressive pulmonary hypertension developed postoperatively.
The Annals of thoracic surgery 10/2005; 80(3):1113-5. · 3.74 Impact Factor
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ABSTRACT: Infections of short term, nontunneled, intravascular catheters are often caused by migration of organisms from the insertion site. The aim of this study was to evaluate the effectiveness and safety of a chlorhexidine gluconate-impregnated dressing for the reduction of central venous catheter (CVC) colonization and CVC-associated bloodstream infections in infants and children after cardiac surgery.
This prospective, randomized, controlled study was conducted in the pediatric cardiac intensive care unit of a tertiary care pediatric medical center. Patients 0-18 years of age who were admitted to the pediatric cardiac intensive care unit during a 14-month period and required a CVC for >48 hours were randomized to receive a transparent polyurethane insertion site dressing (control group) or a chlorhexidine gluconate-impregnated sponge (Biopatch) dressing covered by a transparent polyurethane dressing (study group). The main outcome measures were rates of bacterial colonization, rates of CVC-associated bloodstream infections and adverse events.
Seventy-one patients were randomized to the control group and 74 to the study group. There were no significant between group differences in age, sex, Pediatric Risk of Mortality score or cardiac severity score. CVC colonization occurred in 21 control patients (29%) and 11 (14.8%) study patients (P = 0.0446; relative risk, 0.6166; 95% confidence interval, 0.3716-1.023). Bloodstream infection occurred in 3 patients (4.2%) in the control group and 4 patients (5.4%) in the study group. Local redness was noted in 1 control patient and 4 study group patients.
The chlorhexidine gluconate-impregnated sponge is safe and significantly reduces the rates of CVC colonization in infants and children after cardiac surgery.
The Pediatric Infectious Disease Journal 08/2005; 24(8):676-9. · 3.58 Impact Factor
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ABSTRACT: A best evidence topic in cardiac surgery was written according to a structured protocol. The question addressed was whether pulmonary arteriovenous malformation resolves after redirection of the anomalous hepatic venous flow to the lungs. Altogether 714 papers were found using the reported search, of which only 13 papers presented the best evidence to answer the clinical question. The author, journal, date and country of publication, patient group studied, study type and relevant outcomes of these papers are tabulated. We conclude that the exclusion of hepatic venous blood from the lungs can predispose the patient to intrapulmonary shunt, which can be reversed surgically by diverting the hepatic venous drainage to the right atrium.
Interactive cardiovascular and thoracic surgery 07/2005; 4(3):227-31.
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ABSTRACT: Postoperative bleeding and blood product requirements can be substantial in children undergoing open-heart surgery, and reexploration is required in 1% of cases. Recombinant activated factor VII (rFVIIa, NovoSeven, NovoNordisk, Denmark) is a hemostatic agent approved for the treatment of hemophilic patients with inhibitors to factor VIII or factor IX. It has also been used with success in other conditions. We present our experience with rFVIIa treatment for uncontrolled bleeding after open-heart surgery in five pediatric patients.
The study group consisted of five patients after open-heart surgery with excessive blood loss. The patients were treated with rFVIIa after failure of conventional treatment to control the bleeding. Blood loss, blood product consumption, and coagulation test results were recorded before and after rFVIIa administration.
In all cases, blood loss decreased considerably after rFVIIa administration (mean 7.8 ml x kg(-1) x h(-1)), almost eliminating the need for additional blood products, and the prolonged prothrombin time normalized. In two patients with thrombocytopathy, rFVIIa helped to discriminate surgical bleeding from bleeding caused by a defect in hemostasis. No side effects of rFVIIa treatment were noted.
These cases support the impression that RFVIIa is efficient and safe in correcting hemostasis in children after cardiopulmonary bypass when other means fail. However, the data are still limited, and more extensive research is needed.
Pediatric Anesthesia 04/2005; 15(3):235-40. · 2.10 Impact Factor
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ABSTRACT: This case demonstrates a novel use of stent implantation for relief of coarctation of the aorta caused by protrusion of a Rashkind patent ductus arteriosus umbrella. Follow-up 3 years after stent implantation shows complete relief of obstruction.
Catheterization and Cardiovascular Interventions 10/2004; 63(1):80-2. · 2.29 Impact Factor
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ABSTRACT: Aortopulmonary window is a rare congenital heart defect commonly associated with other cardiac anomalies. Although single institutional experience is low, several surgical techniques have been reported. The purpose of this study is to describe our approach to the management of aortopulmonary window and its associated lesions.
Between February 1996 and November 2002, 13 patients underwent repair of aortopulmonary window. The age range went from 4 days to 5.5 months (mean 42 +/- 52 days), with 9 patients younger than 1 month old. The weight range was from 1.9 to 6.7 kg (mean 3.5 +/- 1.2 kg). Concomitant cardiac anomalies were present in 11 patients. The major additional anomalies were interruption of aortic arch in 4 patients and tracheal stenosis in 1 patient. Initial diagnoses were made using two-dimensional echocardiography only.
There was one postoperative death. In general, patients with aortopulmonary window and additional major defects had a prolonged intensive care unit and hospital stay when compared with the other patients. Follow-up time ranged from 2 months to 6.8 years (mean of 2.5 +/- 2.2 years). There were no reoperations and no late deaths. Transcatheter balloon dilatation of the repaired aortic arch was required in 1 patient and of the right pulmonary artery in another. All other patients had good flow to both pulmonary arteries. No residual shunts were detected at the aortopulmonary window site, and pulmonary pressures were normal.
Aortopulmonary window may be effectively diagnosed with echocardiography. Early surgical treatment (neonatal period, if possible) is safe and associated with the best long-term results, even in the presence of other cardiac anomalies. Complete separation and reconstruction of both aorta and pulmonary arteries under direct vision may prevent recurrence and distortion of adjacent structures.
The Annals of Thoracic Surgery 03/2004; 77(2):484-7. · 3.74 Impact Factor
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ABSTRACT: The authors' experience is reported of aortic valve replacement (AVR) using the pulmonary autograft in patients with active aortic valve endocarditis, including an urgent Ross procedure in infants with the acute condition.
Nine patients aged between 8 months and 38 years, with a diagnosis of aortic valve endocarditis, have undergone AVR using the Ross procedure at the authors' institution since October 1997. The diagnosis was established by clinical and echocardiographic findings. Indications for surgery were severe aortic insufficiency and congestive heart failure in all patients, with the addition of thromboembolic events (n = 3), persistent hyperpyrexia (n = 3) and vegetations (n = 5). Four infants with no history of congenital cardiac malformation underwent urgent surgery because of acute bacterial endocarditis and rapid hemodynamic deterioration. Blood cultures were positive for Streptococcus pneumoniae in three patients, and Kingella kingi and Staphylococcus aureus in one patient each. Four patients were culture-negative. All patients were treated with intravenous antibiotics for four to six weeks postoperatively.
There were no perioperative or late deaths, and no recurrent endocarditis at the implanted valves. Echocardiographic evaluation at discharge showed trivial to mild aortic insufficiency, with no stenosis at the left ventricular outflow tract. Similar findings were found across the right ventricular outflow tract. At follow up (range: 4 months to 5.5 years), none of the patients showed progression of aortic valve insufficiency or developed stenosis; three had mild and moderate homograft stenosis (Doppler gradient 20-40 mmHg), and all children had moderate homograft insufficiency.
The Ross procedure is an excellent therapeutic option for active aortic valve endocarditis in young patients, and demonstrates low morbidity and mortality. Early surgery may be indicated in patients with acute aortic valve endocarditis because of the rapidly progressive nature of this disease.
The Journal of heart valve disease 02/2004; 13(1):73-7. · 0.81 Impact Factor
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ABSTRACT: The mortality rate associated with congenital heart surgery is apparently related to caseload.
To determine whether an increase in caseload over the long term at a single center affects management and outcome in children undergoing cardiac surgery.
Data were collected prospectively over a 4 year period from the computerized registry of the hospital's pediatric intensive care unit. Five parameters were analyzed: age at surgery, type of surgery, preventive measures (open chest), surgery-related and other complications (diaphragm paralysis and acute renal failure, respectively), and mortality. The data of a single-type surgery (arterial switch) were analyzed for bypass time and mechanical ventilation on an annual basis.
The age distribution changed over the years, with more children under 1 year of age (20% newborns) undergoing surgery by the fourth year of the study. The caseload increased from 216 in the first year to 330 in the fourth, with a concomitant decrease in mortality rate from 4.9% to 3.2%. The chest was left open in 3.2% of patients in the first year and in 9.2% in the fourth year. The rate of diaphragm paralysis decreased from 6% to 2.4%. Death due to acute renal failure in patients requiring dialysis decreased from more than 80% in the first 2 years to 36% in the last two. These changes show an improvement but failed to reach statistical significance. Regarding the arterial switch operation, there was a significant improvement in pump time and duration of mechanical ventilation.
The increase in caseload in pediatric cardiac surgery was accompanied by improved management, with a lower complications-related mortality rate. We suggest that for optimal care of children with congenital heart disorders, quality management resources should be concentrated in centers with high caseloads.
The Israel Medical Association journal: IMAJ 08/2003; 5(7):471-4. · 1.02 Impact Factor
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Circulation 05/2003; 107(14):e92-3. · 14.74 Impact Factor
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ABSTRACT: We report a case of an infant with an extensive hemangioma encompassing the thoracic aorta, associated with complex coarctation. Surgical approach was abandoned for fear of bleeding. The complexity of the coarctation made it unsuitable for balloon dilation. We implanted a stent with significant angiographic improvement and resolution of systemic hypertension.
Catheterization and Cardiovascular Interventions 05/2002; 55(4):510-2. · 2.29 Impact Factor
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ABSTRACT: Congenital heart defects (CHDs) appear in greater frequency among relatives of patients and in individuals with DiGeorge syndrome (DGS) or velo-cardio-facial syndrome (VCFS). A majority of these patients and part of the apparently nonsyndromic CHD patients with conotruncal defects manifest hemizygous deletions within chromosome 22q11.2 (del22q11). We tested myocardial tissues of 31 CHD patients, 21 with tetralogy of Fallot (TOF) and 10 with a double-chamber right ventricle (DCRV). DNA isolated from tissues removed at corrective surgery was analyzed for homo- or heterozygosity of nine polymorphic short tandem repeat (STR) markers along the 22q11.2 region. DNA from the blood of 45 healthy individuals represented the general population. Ten of the 21 TOF patients (48%) showed homozygosity for three or more consecutive markers, indicating deletions of various sizes. No such indication was found for DCRV patients. Heterozygosity for markers D22S1648, D22S941, and D22S944 was lower in the TOF group than in normal controls, defining a minimal critical region (MCR) for the deletion. Our findings support an association between TOF and hemizygosity in 22q11.2, suggesting a distinct region, between markers D22S1638 and COMT, that may harbor TOF susceptibility genes.
American Journal of Medical Genetics 03/2002; 107(4):294-8.
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ABSTRACT: Definitive repair of tetralogy of Fallot was performed on 74 patients under two years of age, including 40 under the age of one year and 13 less than 6 months of age. In six patients, there had been previous construction of a systemic-to-pulmonary arterial shunt. Patching across the ventriculo-pulmonary junction was required in 46 patients, placement of conduits in six, and procedures producing unifocaliza-tion in three. Three patients died during the 60-day postoperative period of observation. Of the 71 survivors, 64 recovered without complications. One patient had neurologic sequels, one required prolonged mechanical ventilation, two reoperation, and one balloon dilation of residual left pulmonary arterial stenosis. Morbidity and mortality were not significantly higher in those patients undergoing surgery at earlier than 6 months of age, although transjunctional patching was more common in that age group. Previous construction of a shunt was associated with a higher prevalence of distal stenosis and distortion of the pulmonary arteries, which needed surgical repair in two out of six patients (33%). Since the outcome of definitive repair is favourable in patients with tetralogy of Fallot younger than six months of age, we now advocate definitive surgery for all young symptomatic patients by the age of six months.
Cardiology in the Young 06/1997; 7(03):254 - 257. · 0.76 Impact Factor
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ABSTRACT: We describe a neonate who presented with aortic atresia, a hypoplastic left ventricle and aorto-left ventricular tunnel. These findings, recognized by transthoracic and transesophageal cross-sectional echocardiography complemented by Doppler color flow imaging, were confirmed at surgery by direct inspection. This complex of associations has not been described before and suggests a primary abnormality of the left ventricle because diastolic filling from the aorto-left venticular tunnel and mitral flow should tend to ensure fetal left ventricular growth.
Cardiology in the Young 03/1995; 5(02):190 - 193. · 0.76 Impact Factor
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ABSTRACT: In the United States, duloxetine has been approved for the treatment of major depressive disorder, diabetic peripheral neuropathic pain and fibromyalgia in the adult population. Data regarding the use of duloxetine in the pediatric population, however, are very limited. Femoral nerve injury is a rare complication of cardiac catheterization. In the case described, duloxetine contributed to a successful multimodal treatment program for peripheral neuropathic pain due to femoral neuropathy in an adolescent with 'reactive depression' and conversion symptoms. To the best of the authors' knowledge, the present article is only the third such report on this dual use of duloxetine in children and adolescents, and the first report of such treatment following femoral neuropathy induced by cardiac catheterization.
Pain research & management: the journal of the Canadian Pain Society = journal de la societe canadienne pour le traitement de la douleur 16(6):457-9. · 1.97 Impact Factor
