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Introduction: Anomalous origin of one pulmonary artery from the ascending aorta is a rare cardiac anomaly in which the pulmonary artery abnormally arises from the ascending aorta. Physiologically, most patients develop signs of cardiac failure due to high flow to both lungs, with systemic or supra-systemic pressures in the normally connected lung. The purpose of this study is to present our experience with this rare anomaly, in which early anatomic repair lead to rapid physiologic correction., Materials and Methods: Retrospective case review of all patients with anomalous origin of one pulmonary artery from the ascending aorta at Schneider Children's Medical center of Israel between 1986 and 2007. All clinical operative and echocardiographic charts were analysed., Results: Twelve patients were diagnosed as anomalous origin of one pulmonary artery from the ascending aorta. In 10 patients, the right pulmonary artery rose from the ascending aorta, while in two an anomalous origin of the left pulmonary artery was associated with a right aortic arch. Initial diagnoses was made with two-dimensional echocardiography in all patients. In six patients, diagnostic cardiac catheterisation was performed in order to confirm the diagnosis. Age at diagnosis ranged from 5 to 180 days with a median of 15 days, and patient weight ranged from 780 grams to 5 kilograms, with a median of 3 kilograms. Initial echocardiographic evaluation showed systemic (four patients) or supra-systemic (seven patients) pressures in the right ventricle and normally connected lung. All underwent surgical repair. There was no operative mortality. All reconstructed patients achieved normal right ventricular pressures within days after surgery. The flow pattern in both pulmonary arteries was normalised., Conclusions: Early surgical repair of anomalous origin of one pulmonary artery from the ascending aorta is feasible and safe even in newborn and premature babies with complete resolution of the pulmonary hypertension and normalisation of pulmonary vascular resistance.

Intellectual disability (ID) affects 1%-3% of the general population. We recently reported on a family with autosomal-recessive mental retardation with anterior maxillary protrusion and strabismus (MRAMS) syndrome. One of the reported patients with ID did not have dysmorphic features but did have temporal lobe epilepsy and psychosis. We report on the identification of a truncating mutation in the SOBP that is responsible for causing both syndromic and nonsyndromic ID in the same family. The protein encoded by the SOBP, sine oculis binding protein ortholog, is a nuclear zinc finger protein. In mice, Sobp (also known as Jxc1) is critical for patterning of the organ of Corti; one of our patients has a subclinical cochlear hearing loss but no gross cochlear abnormalities. In situ RNA expression studies in postnatal mouse brain showed strong expression in the limbic system at the time interval of active synaptogenesis. The limbic system regulates learning, memory, and affective behavior, but limbic circuitry expression of other genes mutated in ID is unusual. By comparing the protein content of the +/jc to jc/jc mice brains with the use of proteomics, we detected 24 proteins with greater than 1.5-fold differences in expression, including two interacting proteins, dynamin and pacsin1. This study shows mutated SOBP involvement in syndromic and nonsyndromic ID with psychosis in humans., (Copyright © 2010 The American Society of Human Genetics. Published by Elsevier Inc. All rights reserved.)

Myocarditis among pediatric patients varies in severity from mild disease to a fulminant course with overwhelming refractory shock and a high risk of death. Because the disease is potentially reversible, it is reasonable to deploy extracorporeal membranous oxygenation (ECMO) to bridge patients until recovery or transplantation. This study aimed to review the course and outcome of children with acute fulminant myocarditis diagnosed by clinical and echocardiographic data only who were managed by ECMO because of refractory circulatory collapse. A chart review of a single center identified 12 children hospitalized over an 8-year period who met the study criteria. Data were collected on demographics, diagnosis, disease course, and outcome. The patients ranged in age from 20 days to 8 years (25.5 ± 29.6 months). Echocardiography showed a severe global biventricular decrease in myocardial function, with a shortening fraction of 12% or less. Ten children (83.3%) were weaned off extracorporeal support after 100-408 h (mean, 209.9 ± 82.4 h) and discharged home. Two patients died: one due to multiorgan failure and one due to sustained refractory heart failure. During a long-term follow-up period, all survivors showed normal function in daily activities and normal myocardial function. The study showed that ECMO can be safely and successfully used for children with acute fulminant myocarditis diagnosed solely on clinical and radiographic grounds who need mechanical support. These patients usually have a favorable outcome, regaining normal or near normal heart function without a need for heart transplantation.

We compared ibuprofen and indomethacin for the treatment of patent ductus arteriosus (PDA) in preterm infants. A retrospective comparative study was conducted at a pediatric tertiary center in preterm infants diagnosed with PDA. Infants born from January 2000 to June 2003 were treated with indomethacin, whereas infants born from July 2003 to November 2005 were treated with ibuprofen. The two treatment groups were compared. Demographic data and clinical, laboratory, and outcome data were collected from the medical files. Seventy-three infants were included in the ibuprofen group and 46 in the indomethacin group. No significant difference in efficacy was found between indomethacin and ibuprofen. Compared with ibuprofen, indomethacin treatment was associated with significantly higher mean creatinine levels and a higher percent of infants with creatinine >1.2 mg/dL, hyponatremia <120 mmol/L, and platelet level <100,000 platelets/mL(3). There were no significant differences in bilirubin levels, incidence and grade of intraventricular hemorrhage, necrotizing enterocolitis, retinopathy of prematurity, rate of surgical duct ligation, sepsis, length of hospital stay, or mortality. Indomethacin and ibuprofen are equally effective for PDA closure in premature infants. Treatment with ibuprofen is safer, decreasing the risk of renal failure, thrombocytopenia, and hyponatremia., (Copyright Thieme Medical Publishers.)

Objectives: To report on the methods and results of treatment of large congenital coronary-cameral fistulae by transcatheter closure with Amplatzer devices., Background: Large coronary-cameral fistulae cause a steal phenomenon from the normal coronary circulation. Surgical closure is an option. However, transcatheter methods allow for temporary occlusion, definition of anatomy, and online assessment of successful closure. Amplatzer devices are compact occluders that can be fully delivered, collapsed, and repositioned until a satisfactory position is attained., Methods: Coronary and fistula anatomy were defined by selective coronary angiography with or without temporary occlusion. Device closure of the fistula was performed at the most distal point accessible, often from the cameral side using an arteriovenous loop method., Results: Ten patients of median age 2.6 years (0.5-52.2) and weight 14.4 kg (6.1-67) underwent an attempt at transcatheter closure of a large fistula. In nine patients, the fistula was closed successfully with a device. There were no complications., Conclusions: Transcatheter closure of coronary-cameral fistula with Amplatzer devices is safe and effective., (Copyright 2010 Wiley-Liss, Inc.)

Objectives: To report on the early results of treatment of coarctation of the aorta by dilation with a new polytetrafluoroethylene covered stent., Background: Transcatheter dilation of aortic coarctation carries the risk of aneurysm or rupture. Covered stent implantation reduces this risk but requires a large delivery system. The Advanta V12 LD covered stent is premounted and requires a 9-11 Fr delivery system., Methods: Covered stents on balloons of a diameter sufficient to anchor the stent in the coarctation were implanted using the smallest available delivery system. Secondary dilation with larger diameter balloons was performed until the pressure gradient was <20 mm Hg and the stent was opposed to the aortic wall., Results: Twenty-five patients with aortic coarctation underwent stent implantation. Coarctation diameter increased from (6.3 + or - 3.5) mm to (14.4 + or - 2.3) mm (P < 0.0001). Peak pressure gradient decreased from (25.3 + or - 11.6) mm Hg to (2.5 + or - 3.0) mm Hg (P < 0.0001). The stent achieved the desired diameter in all cases. There were no complications. At short-term median follow-up of 4.9 months, all patients are alive and well with no evidence of recoarctation or aneurysm., Conclusions: These initial results show that the covered Advanta V12LD stent is safe and effective in the immediate treatment of coarctation of the aorta through a low profile delivery system of 8-11 Fr. Long term follow up is required.

Background: Selective serotonin reuptake inhibitors (SSRIs) have been suspected of cardiac teratogenicity, but reports have been inconsistent. Our aim was to investigate the rate of nonsyndromic congenital heart defects in newborns exposed in utero to SSRIs compared with unexposed controls., Methods: This prospective study of women who gave birth at our tertiary center from 2000 to 2007 yielded 235 women who reported first-trimester SSRI use during pregnancy. All newborns born during the study period and found to have a persistent cardiac murmur on day 2 or 3 of life were referred for examination by a pediatric cardiologist and by echocardiography. The findings were compared between the newborns who were exposed to SSRIs and those who were not., Results: Nonsyndromic congenital heart defects were identified by echocardiography in 8 of 235 (3.40%) newborns exposed in utero to SSRIs and in 1083 of 67,636 (1.60%) non-exposed newborns. The difference in prevalence between the two groups was significant (relative risk, 2.17; 95% confidence interval, 1.07-4.39). The prevalence rates for paroxetine and fluoxetine exposure were 4.3% and 3.0%, respectively. All cardiac defects in the study group were mild: ventricular septal defect (6), bicuspid aortic valve (1) and right superior vena cava to coronary sinus (1)., Conclusions: Newborns exposed in utero to SSRIs, have a twofold higher risk of mild nonsyndromic heart defects than unexposed infants. The data suggest that women who require SSRI treatment during pregnancy can be reassured that the fetal risk is low and possible cardiac malformations will probably be mild. Late-targeted ultrasound and fetal echocardiography at 22 to 23 weeks' gestation are recommended in this patient group.

Inherited disorders of elastic tissue represent a complex and heterogeneous group of diseases, characterized often by sagging skin and occasionally by life-threatening visceral complications. In the present study, we report on an autosomal-recessive disorder that we have termed MACS syndrome (macrocephaly, alopecia, cutis laxa, and scoliosis). The disorder was mapped to chromosome 20p11.21-p11.23, and a homozygous frameshift mutation in RIN2 was found to segregate with the disease phenotype in a large consanguineous kindred. The mutation identified results in decreased expression of RIN2, a ubiquitously expressed protein that interacts with Rab5 and is involved in the regulation of endocytic trafficking. RIN2 deficiency was found to be associated with paucity of dermal microfibrils and deficiency of fibulin-5, which may underlie the abnormal skin phenotype displayed by the patients.

Objectives: To report on the early results of treatment of native coarctation of the aorta by implantation and serial dilations of covered stents., Background: Transcatheter dilation of native coarctation of the aorta carries a risk of aneurysm or rupture. Covered stent implantation requires a large delivery system with the risk of vascular damage., Methods: Covered stents on balloons of diameter sufficient to anchor the stent in the coarctation were implanted using the smallest delivery system possible. Dilation with larger diameter balloons was performed until the pressure gradient was <20 mm Hg and the stent was opposed to the aortic wall., Results: Twenty-two patients with native coarctation underwent stent implantation. Coarctation diameter increased from 3.6 +/- 1.9 to 12.6 +/- 1.9 mm (P < 0.001). Peak pressure gradient decreased from 29.4 +/- 8.5 to 6.7 +/- 5.7 mm Hg (P < 0.001). Nine patients underwent further dilation on average 5 months later. Residual pressure gradient decreased from 12.3 +/- 5.8 to 2.1 +/- 2.9 mm Hg (P = 0.002). The stent achieved the diameter of the transverse arch in all cases. Complications included a small tear at further dilation treated with a second stent and a femoral pseudoaneurysm. At short-term follow-up of 18.5 months all patients are alive and well with no evidence of recoarctation or aneurysm., Conclusions: These initial results show that serial dilation of covered Cheatham-Platinum stents is feasible, safe, and an effective percutaneous method for the treatment of native coarctation of the aorta. However, long-term follow up is required., (Copyright 2009 Wiley-Liss, Inc.)

Background: Environmental physical activity is known to be associated with many factors of human homeostasis, such as fetal development, birth number, and some genetic abnormalities. This study sought to investigate possible temporal links between the occurrence of congenital heart disease and solar, geomagnetic, and cosmic ray activities., Patients and Methods: The study sample include 79,085 infants born live at a tertiary medical center in central Israel from 1995 to 2005, of whom 1739 were diagnosed with congenital heart disease, including 309 with patent ductus arteriosus (PDA). The number of infants with congenital heart disease (total and excluding PDA) was analyzed against the values of the physical parameters, as derived from international indices, by year of birth and 1 year before and by month of birth and 9 months before. Pearson correlation coefficients and their probabilities were calculated., Results: The number of cases of infantile congenital heart disease over the 132-month study period significantly correlated with solar activity (r=0.5, p<0.0001) and with cosmic ray activity (r=-0.45, p<0.0001). On analysis by year, correlations were as follows: with solar activity 1 year before delivery, r=0.71, p=0.014, n=11, and at time of delivery, r=0.66, p=0.026; with cosmic ray activity, 1 year before delivery, r=-0.66, p=0.03, and at time of delivery, r=-0.61, p=0.047, n=11. The levels of correlation and probability were higher for solar activity indices at conception (9 months or 1 year before delivery) than at birth. Significance was maintained when cases of PDA were excluded., Conclusion: The monthly number of infants born with congenital heart disease is directly correlated with the level of solar activity and inversely correlated with the level of cosmic ray activity during pregnancy, predominantly in the month of conception. The mechanism underlying the possible effect of solar activity on the occurrence of congenital heart disease warrants additional studies.

Objective: To evaluate endoscopic plantar fasciotomy for the treatment of recalcitrant heel pain., Method: We undertook a retrospective study of the use of endoscopic plantar fasciotomy in the treatment of chronic heel pain that was unresponsive to conservative treatment. Over a 10-year period, we reviewed the charts of 55 patients with a minimum 12-month history of heel pain that failed to respond to standard nonoperative methods and had undergone the procedure described. All patients were clinically reviewed and completed a questionnaire based on the American Orthopaedic Foot and Ankle Society (AOFAS) score for ankle and hindfoot., Results: The mean follow-up was 18 months. The mean preoperative AOFAS score was 66.5; the mean postoperative AOFAS score was 88.2. The mean preoperative pain score was 18.6; the mean postoperative pain score was 31.1. Complications were minimal (2 superficial wound infections). Overall, results were favourable in over 80% of patients., Conclusion: We conclude that endoscopic plantar fasciotomy is a reasonable option in the treatment of chronic heel pain that fails to respond to a trial of conservative treatment.

The shortage of available donor hearts limits the number of cardiac transplantations worldwide and in Israel as well. This organ shortage results in 15%-20% annual mortality of heart transplant candidates. For the sub-group of hospitalized decompensated heart failure patients depending on continuous inotropic support (Status I), the annual mortality is over 50%. Suboptimal utilization of donor hearts has been one of the reasons for the organ shortage. In 2004, only 42% of the potential donor hearts in Israel were eventually transplanted. The objective of this report is to define guidelines regarding the suitability of potential cardiac donors allowing more liberal criteria for accepting borderline donor hearts. Implementing the new guidelines will permit the utilization of organs that otherwise would have been discarded.

We have previously found that, following myocardial ischemia/reperfusion injury, isolated hearts from bax gene knockout mice [Bax(-/-)] exhibited higher cardioprotection than the wild-type. We here explore the effect of Bax(-/-), following myocardial infarction (MI) in vivo. Homozygotic Bax(-/-) and matched wild-type were studied. Mice underwent surgical ligation of the left anterior descending coronary artery (LAD). The progressive increase in left-ventricular end diastolic diameter, end systolic diameter, in Bax(-/-) was significantly smaller than in Bax(+/+) at 28 d following MI (p < 0.03) as seen by echocardiography. Concomitantly, fractional shortening was higher (35 +/- 4.1% and 27 +/- 2.5%, p < 0.001) and infarct size was smaller in Bax(-/-) compared to the wild-type at 28 days following MI (24 +/- 3.7 % and 37 +/- 3.3%, p < 0.001). Creatine kinase and lactate dehydrogenase release in serum were lower in Bax(-/-) than in Bax(+/+) 24 h following MI. Caspase 3 activity was elevated at 2 h after MI only in the wild-type, but reduced to baseline values at 1 and 28 d post-MI. Bax knockout mice hearts demonstrated reduced infarct size and improved myocardial function following permanent coronary artery occlusion. The Bax gene appears to play a significant role in the post-MI response that should be further investigated.

Objective: To review the incidence and complications of conservative management of bilateral diaphragm paralysis following pediatric cardiac surgery., Design and Setting: Retrospective clinical review based on computerized database with daily follow-up in a pediatric cardiac intensive care unit in a tertiary care center. PATIENT AND PARTICIPANTS: Were reviewed the data on nine patients with bilateral diaphragm paralysis from the 3,214 consecutive children (0.28%) after operations performed between 1995 and 2004., Measurements and Results: A fluoroscopy-confirmed diagnosis of bilateral diaphragm paralysis was made in all nine patients. Mechanical ventilation was required for 14-62 days; maximum time to recovery was 7 weeks. Three patients underwent unilateral plication. Patients with a complicated postoperative course required longer mechanical ventilation. All patients were managed with a nasotracheal tube. One patient had minor subglottic stenosis. All patients survived., Conclusions: Bilateral diaphragm paralysis can be managed conservatively with good prognosis and minor complications. The recovery time is relatively short, less than 7 weeks.

Candidal endocarditis is an uncommon and serious complication of invasive Candida infection in neonates. The aim of this study was to further characterise candidal endocarditis in neonates. Between 1995 and 2000, 56 patients were diagnosed with Candida bloodstream infections (CBSI) in the Neonatal Intensive Care Unit of Schneider Children's Medical Center of Israel. Five of them (9%) developed mycetoma of the right atrium. None of the patients had congenital heart disease or a central venous catheter in the right heart at the time of diagnosis. All were treated with amphotericin B alone or in combination with other antifungals, without surgical intervention. One patient died of the disease and one died later of polymicrobial sepsis and necrotizing enterocolitis. A review of the literature since 1980 yielded an additional 25 cases of candidal endocarditis. For the whole sample (n = 30) survival rate was 73.1%. Six of the 10 patients treated with antifungal agents and surgery survived (60%), compared with 13 of the 20 patients treated only medically (65%) (P = 1.0). Candida endocarditis in neonates differs from fungal endocarditis in adults in risk factors, clinical presentation and outcome. As the outcome of surgical and medical treatment are comparable, antifungal therapy alone may be a valid therapeutic option in high-risk cases.

Background and Aim of the Study: 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., Methods: 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)., Results: 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., Conclusion: 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.

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.

Background: 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., Methods: 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., Results: 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., Conclusions: The chlorhexidine gluconate-impregnated sponge is safe and significantly reduces the rates of CVC colonization in infants and children after cardiac surgery.

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.

Background: 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., Methods: 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., Results: 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., Conclusions: 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.

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.

Background: 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., Methods: 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., Results: 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., Conclusions: 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.

Background and Aim of the Study: 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., Methods: 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., Results: 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., Conclusion: 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.

Background: The mortality rate associated with congenital heart surgery is apparently related to caseload., Objective: To determine whether an increase in caseload over the long term at a single center affects management and outcome in children undergoing cardiac surgery., Methods: 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., Results: 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., Conclusions: 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.

This prospective study was undertaken to determine the spectrum, sites and main risk factors for hospital-acquired infections (HAI) in our paediatric cardiothoracic intensive care unit (PCICU), and to determine the main organisms causing bloodstream infection in this setting. All patients admitted between January and December 1999 were prospectively followed for the development of HAI. To define risk factors, patients were grouped by age, complexity score, length of stay in PCICU, and whether the patient's chest was open or closed postoperatively. Three hundred and thirty-five patients underwent cardiac surgery. Fifty-five patients acquired 69 HAIs (HAI patient rate 16.4%). The most common HAI were bloodstream and surgical wound infection in 10 and 8%, respectively. The main causative organisms were Klebsiella spp.,Enterobacter spp. and Pseudomonas spp. in 22, 17 and 16% of episodes, respectively. Staphylococcus spp. accounted for 16% of episodes. The main risk factors for developing HAI were: neonatal age [P < 0.05, odds ratio (OR): 5.89, 95% confidence interval (CI): 2.96-11.58] prolonged PCICU stay (P < 0.05, OR: 6.82, 95% CI: 3.37-14.48), open chest postoperatively (P < 0.05, OR: 3.44, 95% CI: 1.31-8.52) and high complexity score (P < 0.05, OR: 4.03 95% CI: 1.87-8.43). The main causative organisms of bloodstream infections in children hospitalized in the PCICU differ from those in adult and pediatric general intensive care units (ICUs) and include mainly Gram-negative bacilli. High complexity score, neonatal age, prolonged ICU stay, and open chest postoperatively are risk factors of HAI in this patient population., (Copyright 2003 The Hospital Infection Society)

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., (Copyright 2002 Wiley-Liss, Inc.)

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., (Copyright 2001 Wiley-Liss, Inc.)

Background: An accurate assessment of fluid status in haemodialysis patients presents a significant challenge especially in growing children. Clinical parameters of hydration are not always reliable, and invasive methods such as measurement of central venous pressure cannot be used routinely. We evaluated the usefulness of inferior vena cava diameter (IVCD) measured by echocardiography in the estimation of hydration in children on haemodialysis., Methods: Fifteen haemodialysis patients (mean age 14 years) were evaluated. Clinical assessment included patients' symptoms, weight, blood pressure, heart rate, presence of oedema and vascular congestion, before and after dialysis session. Dry weight was assessed based on the above parameters. Fifty-two echocardiographic studies immediately prior and 30-60 min following dialysis were performed. The anteroposterior IVCD was measured 1.5 cm below the diaphragm in the hepatic segment in supine position during normal inspiration and expiration. IVCD was expressed per body surface area., Results: Following haemodialysis mean IVCD (average of expiration and inspiration) decreased from 1.12+/-0.38 to 0.75+/-0.26 cm/m(2) (P<0.0001). Changes in IVCD were significantly correlated with alterations in body weight following dialysis (P<0.0001). The collapse index (per cent of change in IVCD in expiration vs inspiration) increased significantly after dialysis (P=0.035). IVCD clearly reflected alterations in fluid status. It did not vary significantly with changes in dry weight in a given patient., Conclusions: Our findings support the applicability of VCD measurement in the estimation of hydration status in paediatric haemodialysis patients. The combination of clinical parameters and measurement of IVCD may enable more accurate evaluation of hydration of children on haemodialysis.

We present a case of left ventricular thrombus in a child with a normal functioning left ventricle. The diagnosis was made by 2-dimensional echocardiography after 2 episodes of systemic emboli. Hereditary protein C deficiency diagnosed in the patient provides the probable pathogenesis of the thrombus formation. Systemic emboli necessitates cardiac examination, and in cases of unusual thrombi, hereditary or acquired thrombophilic risk factors should be considered.

OBJECTIVE: To review the incidence, diagnosis, and management of superior vena cava syndrome (SVCS) after surgery for congenital heart disease. DESIGN: Retrospective clinical review. All patients were computer registered. Our database includes daily follow-up. SETTING: Pediatric cardiac surgery intensive care unit in a university hospital. PATIENTS: A total of 1853 consecutive pediatric cardiac operations performed in 285 neonates and 1568 older children from 1993 to 1999 are reviewed. MEASUREMENTS AND MAIN RESULTS: The diagnosis of SVCS was suspected clinically: Color changes and swelling of the upper part of the body, confirmed by echo-Doppler, showed no or minimal flow in the superior vena cava at the beginning and collateral flow later on. Nine patients developed SVCS (0.5%). All the study patients were neonates. The prevalence of SVCS in our neonatal patients was 3.15% (nine of 285), with no SVCS in older children. Accompanying complications included chylothorax (five), hydrocephalous (four)-three of whom required ventriculoperitoneal shunt during follow-up. Thrombolytic therapy was used in five patients, and thrombectomy was used in one patient. The ventilation period ranged from 4 to 46 days (mean 20.1 days), and the length of hospital stay ranged from 37 to 120 days (mean 61.3 days). No mortality was observed during follow-up. CONCLUSIONS: SVCS is an uncommon, severe complication following neonatal cardiac surgery. It may cause chylothorax, hydrocephalus, and severe respiratory complications leading to high morbidity. Early diagnosis and thrombolytic therapy may prevent the progression of this syndrome to its subsequent sequels.

Background: Secundum atrial septal defect is a common congenital heart defect that causes right heart volume overload and produces symptoms usually after the third decade of life. Treatment until the last few years has been open heart surgery., Objectives: To review our early experience with transcatheter closure of ASD2 using the Amplatzer septal occluder., Methods: Between November 1999 and February 2000, 20 children and young adults with a median age of 9.1 years (4.2-35.1 years) were referred for transcatheter closure of ASD2. Diagnosis was established by transthoracic echocardiography. Implantation was performed under general anesthesia through the femoral vein with the guidance of transesophageal echocardiography and fluoroscopy. Femoral arterial puncture was performed for blood pressure monitoring during the procedure. The device size chosen was similar to the balloon-stretched diameter of the ASD2., Results: Implantation was completed successfully in 18 patients. Two patients were referred for elective surgery: one had an unsuitable anatomy for transcatheter closure by TEE in the catheterization laboratory and the device could not be implanted properly, the other patient had a large multiperforated septal aneurysm that was retrieved. Mean ASD2 diameter by TTE and TEE was similar (13.9 +/- 3 mm, 13.4 +/- 3.5 mm) and mean stretched diameter was 18.3 +/- 4.3 mm. Mean Qp:Qs (pulmonary flow:systemic flow) was 2.2 +/- 0.6. Mean fluoroscopy time for the procedure was 14.8 +/- 4.8 minutes. The patients were discharged the day after the procedure. Four patients had a tiny leak immediately post-procedure, and none had a leak at one month follow-up. The only complication was a small pseudoaneurysm of the femoral artery in one patient, that resolved spontaneously., Conclusions: Transcatheter closure of ASD2 with the Amplatzer septal occluder is a safe and effective alternative to surgical closure. Long-term outcome has to be evaluated.

Background: The need for aortic valve replacement in children and young adults poses a special problem to cardiologists and surgeons. Replacing the sick aortic valve with the patient's pulmonary valve as described by Ross has proven to be a good option in this special age group., Objective: To review our initial experience in order to assess the short-term results., Methods: From January 1996 to June 1999, 40 patients (age 8 months to 41 years) underwent aortic valve replacement with pulmonary autograft. Indications for surgery were congenital aortic valve disease in 30 patients, bacterial endocarditis in 5, rheumatic fever in 3, and complex left ventricular outflow tract obstruction in 3. Trans-esophageal echocardiography was performed preoperatively and post-bypass in all patients, and transthoracic echocardiography was done prior to discharge and on follow-up., Results: There was no preoperative or late mortality. All patients remain in functional class I (New York Heart Association) and are free of complications and medication. None showed progression of autograft insufficiency or LVOT obstruction. Homograft insufficiency in the pulmonary position has progressed from mild to moderate in one patient, and three developed mild homograft stenosis., Conclusions: The Ross procedure can be performed with good results in the young population and is considered an elegant surgical alternative to prosthetic values and homografts.

Background: Anomalous origin of the left coronary artery from the pulmonary artery is a rare congenital malformation that presents a diagnostic challenge to the pediatrician and pediatric cardiologist. Although surgical repair is always indicated, the optimal technique has yet to be determined., Objectives: To review our experience with the diagnosis of children with ALCAPA and to assess short to midterm surgical results., Methods: Between 1992 and 1998, 13 infants and children (2 months to 15 years) were treated for ALCAPA at our medical center. Eight were diagnosed during the first year of life; all were symptomatic and had severe dysfunction of the left ventricle. The five patients diagnosed at an older age had normal myocardial function. Diagnosis was established by echocardiography alone in seven patients; six required catheterization (one infant and all older patients). Surgery was performed in 12 patients to establish dual coronary artery system: 7 underwent the Takeuchi procedure and 5 had re-implantation of the anomalous left coronary artery., Results: One infant died shortly after diagnosis before surgical repair was attempted, and one died postoperatively. Four patients required additional surgery: three for late complications of the Takeuchi procedure and one valve replacement for mitral insufficiency. Recent evaluation revealed good global left ventricle function in all patients except for one, who is still within the recovery phase and shows gradual improvement. However, most patients who presented with severe myocardial dysfunction upon diagnosis still display abnormal features such as echo-dense papillary muscles or evidence of small akinetic segments. In this group, early repair was associated with faster myocardial recovery., Conclusions: The diagnosis of ALCAPA remains a clinical challenge to the pediatrician and cardiologist. Diagnosis can be established echocardiographically, and early diagnosis and treatment may lead to faster myocardial recovery. The preferred surgical method appears to be re-implantation of the ALCA. The chance for good recovery of global ventricular function is high even in the sickest patients, nonetheless abnormal myocardial features can be identified even years after surgery.

Background: CKMB, the cardiac-specific heterodimer of cytosolic creatine-kinase (CK), is developmentally and physiologically regulated, tissue hypoxia being a proposed regulator. In patients with cyanotic heart disease the myocardium is perfused with partially saturated blood. We questioned whether the myocardium of cyanotic subjects contains higher proportions of CKMB., Materials and Methods: CK activity, the distribution of cytosolic CK isozymes, activity of lactic dehydrogenase (LDH), and tissue protein content were determined in obstructive tissues removed at corrective surgery of patients with congenital heart defects. Cyanotic (n = 13) and acyanotic (n = 12) subjects were compared., Results: In cyanotic and acyanotic patients, CK activity was 8.4 +/- 0.6 and 7.6 +/- 0.6 IU/mg protein and the proportion of CKMB was 21 +/- 1.4 and 22 +/- 2. 0% (mean +/- S.E.M), respectively. In the two groups of patients, the activity related to the B subunit corresponded to the steady-state level of the CKBmRNA. The tissue content of protein and the activities of CK and LDH were similar in cyanotic and acyanotic subjects and increased with the age., Conclusions: The lack of difference in CKMB distribution between the cyanotic and acyanotic patients may either indicate that hypooxygenation is not a regulator of CK isozyme expression, or may be attributed to the already high proportion of this isozyme in hypertrophied, obstructive tissues. Recruitment of additional CKMB, in the cyanotic hearts, may thus not be required.

The incidence of congenital heart defects is 0.8% of liveborn infants. Of these 13% are ostium secundum atrial septal defects (ASD) which can be successfully repaired by open heart surgery with less than 1% mortality. However, morbidity associated with cardiac surgery is universal. Transcatheter closure of ASDs was introduced 2 decades ago to decrease surgical complications, hospital stay and cost. Few devices have undergone clinical trials but all of them have been associated with instances of failure and complications. The most important mechanism for acute failure was selection of cases with too large a defect or selection of a defective device. In the past decade great progress has been made with the aid of transesophageal echocardiography (TEE) and modifications of the device. We present our experience in our first 5 patients. They ranged in age from 4 to 27 years; the ASD diameter was 11-15 mm and the device diameter was 17-33 mm. All transcatheter closures were performed on the same day without any complications, and all patients were discharged home after 2 days of hospitalization. We conclude that transcatheter occlusion of ASDs up to 15 mm is feasible, relatively safe and effective. This transcatheter method appears to be a viable alternative to surgery for some patients with secundum atrial septal defects.

In the past 10 years there has been a growing preference for early, complete correction of congenital heart disease. The first year of operation of this cardiac unit is described. 216 operations were performed: 15% in the neonatal age group and 35% in the newborn to 1-year-old groups; 2% were palliative procedures. Mortality was 4.9%. Average stay in the ICU was 3.2 days, with a median of 2.25. Average length of ventilation was 35 hours, with a median of 17.5. Complications were: diaphragm paralysis in 13 (6%), 2/3 of which were recurrent operations; in 2 patients (0.9%) we had to plicate the diaphragm. There was severe neurological damage in 2 which deteriorated to brain death in 1. There was peripheral, reversible neurological damage in 4 (1.8%), and acute renal failure in 3%, with half of them requiring dialysis. 75% of these children died and there was superficial infection in 4.1%, deep wound infection in 1.3%, bacteremia in 4.1%, superior vena cava syndrome in 3 (1.3%) and chylothorax in 2 of them (0.9%). 1 patient (0.45%) required a ventricle-peritoneal shunt after acute viral meningitis. We are encouraged by our results to offer early complete correction to all children with congenital heart disease.

The use of cardiopulmonary bypass (CPB) in children is associated with significant morbidity due to the accumulation of an excessive amount of water. This can be decreased by massive diuresis, peritoneal dialysis, or conventional ultrafiltration technique (CUF) during bypass. However, we were dissatisfied with their effect on the outcome of our young patients, and recently began to use the modified technique of ultrafiltration (MUF) with good results. MUF was carried out for 15 min after completion of CPB to a hematocrit of 40%. Eighty patients were equally divided into two groups--MUF (group A) and control (group B)--and prospectively studied. There was one death in group A and two deaths in group B. The chest was left open in one patient in group A and in three patients in group B. At the end of MUF, the cardiac size was smaller, performance was better, and systemic blood pressure was higher in group A. Hematocrit levels in the two groups were similar during preoperative and CPB time, but post-CPB hematocrit in group A was significantly higher, 39.4% mean (range 35-50) vs. 28.5 (range 22-38) in group B (P <0.05). Blood loss was 10.56 ml/kg per 24 h (2-48.7) in group A vs. 20.8 (4.5-105.6) in group B (P <0.05). Blood transfused was 7.2 ml/kg per 24 h (0-29) in group A vs. 17.3 (3.1-49) in group B (P <0.05). Colloids transfused were 16.7 ml/kg per 24 h (0-64) in group A vs. 27.5 (0-58.6) in group B (P <0.05). No significant difference was found in urine output or the use of diuretics between the two groups. MUF results in better hemodynamic status in children, with the added advantage of less transfused blood and blood products. We believe that the use of accepted surgical techniques in combination with MUF will further improve the outcome of complex cardiac malformations.

Background: There is growing recognition that postoperative pulmonary regurgitation may result in early or late progressive right heart failure., Method: A technique for fashioning an autologous monocusp pulmonary valve from the wall of the pulmonary artery was developed. The monocusp valve was fashioned from the anterior wall of the main pulmonary artery, and the remaining defect was filled with autologous pericardium. The procedure was performed in 8 dogs and 5 children., Results: Early follow-up and serial echocardiographic assessment in both dogs and children proved the functionality of this monocusp pulmonary valve. All valves were pliable and demonstrated mild to moderate pulmonary stenosis and insufficiency., Conclusions: Construction of the autologous monocusp pulmonary valve is a feasible technique, and the valve performs efficiently. The acute performance in the canine model was excellent, and preliminary midterm results in the clinical study are reasonable. It is logical to assume that the monocusp, being an integral part of the arterial wall, will retain its viability and share in the subsequent growth of the pulmonary artery. Should follow-up studies demonstrate its long-term competence, this autologous valve may provide a good solution for various forms of pulmonary regurgitation and be useful in pulmonary autograft replacement of the aortic valve.

Patients who undergo surgical repair of congenital heart defects, characterized by a hypoplastic right ventricle or high pulmonary vascular resistance, are at high risk for the development of postoperative right heart failure. This risk may discourage the surgical team from carrying out a biventricular or complete repair in such patients. To reduce the risk for right heart failure, we developed a one-way, valved, atrial septal patch to serve as an artificial one-way foramen ovale and tested it in an animal model. By permitting right-to-left shunt, this device decompresses the failing right ventricle and maintains systemic cardiac output. The device has been used in 15 patients divided into three different groups: group 1 (n = 8), patients with a hypoplastic right ventricle and pulmonic stenosis or atresia, seven of whom underwent a biventricular repair; group 2 (n = 5), patients with evidence of pulmonary disease after longstanding left-to-right shunt caused by a correctable atrial or ventricular septal defect, all of whom had a complete repair; group 3, two patients with acute right heart failure in whom the device was used as a last option of treatment to wean them from cardiopulmonary bypass. This article presents our data in regard to the use of the one-way, valved, atrial septal patch and the indications for its clinical use.

Interrupted right sided aortic arch is a very rare anomaly, and to our knowledge only one such case in which the patient underwent complete repair has been recorded in the literature. Twelve additional cases were found on autopsy or underwent palliative surgery. We present the case of a 25-day-old infant with an interrupted right sided aortic arch. The anomaly was repaired by a one-stage surgical approach, and the patient discharged from the hospital. Surgical technique considerations are discussed.

Anomalous origin of the left coronary artery (ALCA) from the pulmonary artery is a rare cardiac anomaly. It can result in decreased myocardial perfusion and impaired left-ventricular function, which can lead to congestive heart failure or even myocardial infarction. Reconstructive surgery of the left coronary artery is the desirable measure in the management of this anomaly. Since July 1992, five patients with ALCA from the pulmonary artery were treated surgically. Age at operation ranged from 3 months to 11 years. Three patients had congestive heart failure and one was in cardiogenic shock. Operative techniques included "tunnel type" surgery in three cases and aortic reimplantation in two. Two operative variations performed successfully in this series are described. There were no postoperative deaths. At the latest follow-up (mean 14 months), all anastomoses were patent and showed antegrade flow. For those patients with ALCA from the pulmonary artery, direct reimplantation of the ALCA to the aorta is the most physiologically appropriate reconstructive solution, and offers good early and late results. Intrapulmonary tunnel from aortopulmonary window to coronary artery is recommended for children in whom aortic reimplantation is not anatomically feasible.

Aortic valve prolapse is found in over 5% of children with ventricular septal defect (VSD). Although this association occurs mostly with doubly committed subarterial VSDs, in this study the predominant type of VSD was perimembranous. In order to determine the need and timing for surgery and whether the anatomical features of septal defect may influence clinical management and outcome in this lesion, we reviewed our experience with 28 consecutive patients, operated on for VSD with prolapsed aortic valve cusp, with or without aortic regurgitation. Twenty-two patients had a perimembranous VSD and six had doubly committed VSD. Aortic regurgitation was trivial or absent in nine patients, mild in ten and moderate to severe in nine. Associated cardiac anomalies were present in 18 patients, all having perimembranous VSD, and included right ventricular outflow tract (RVOT) obstruction (n = 6), discrete subaortic membrane (n = 4) or both (n = 8). None of these patients had more than moderate aortic regurgitation. The patients underwent surgical closure of the septal defect between the ages of 1.5 and 34 years of age (median = 7). Sixteen patients having mild or trivial aortic regurgitation underwent closure of the VSD only, and 12 patients underwent VSD closure with aortic valvuloplasty. Valvuloplasty was required more often in doubly committed VSDs (66%) and in the perimembranous type without associated anomalies (100%), and significantly less often in the presence of RVOT obstruction, subaortic membrane or both (22%). At follow-up (up to 5 years, mean 18 months), the grade of aortic regurgitation was unchanged in 11 and decreased in 5 patients undergoing closure of the VSD only.(ABSTRACT TRUNCATED AT 250 WORDS)

Definitive repair of tetralogy of Fallot was performed on 104 children, including 42 younger than 2 years (24 < 1 year and 7 < 6 months). Twenty-two had previous shunt. Transannular patching was required in 60 cases and conduit grafts in two. During 60-day postoperative observation, five patients died. Of the 99 survivors, 93 recovered without complications, three required prolonged mechanical ventilation, two reoperation and one balloon dilatation for residual left pulmonary artery stenosis. Morbidity and mortality were not significantly higher in the patients younger than 12 months, although transannular patching was more common in that age group. Previous shunt operations entailed higher prevalence of distal stenosis and distortion of the pulmonary arteries, which often necessitated surgical repair, and also considerably increased postoperative morbidity and mortality. As the outcome in definitive repair of Fallot's tetralogy is favourable, even in patients younger than 12 months, and as preliminary shunt operations are associated with heightened perioperative morbidity and mortality rates in definitive repair, we advocate the definitive operation for all young patients with severe cyanosis or cyanotic spells.

Eleven patients underwent late repeated correction of tetralogy of Fallot in 1991-1993. The previous operation was repair of simple Fallot's tetralogy in seven cases, repair plus transannular patch in one case and repair of tetralogy and pulmonic atresia in three cases. The indications for reoperation were residual ventricular septal defect, right ventricular outflow tract (R.V.O.T.) obstraction, residual branch pulmonary artery stenosis, aneurysmal dilatation of R.V.O.T. Patch or combination of any of the above. At reoperation these defects were corrected. The post operative course was uneventful in eight patients. Two required mechanical ventilation for 2-3 days, and one underwent another operation for residual branch pulmonary artery stenosis. The functional and haemodynamic results were good in ten patients, and one had residual distal pulmonary artery stenosis. There were no death during 2 years of follow-up. Repeated correction of tetralogy of Fallot thus had low postoperative morbidity and good haemodynamic results. For the relatively few patients initially found to have tetralogy of Fallot and pulmonic atresia, the outcome may be less favorable.

We reviewed our experience with 40 patients who had undergone total cavopulmonary connection (TCPC) during the past three years. Thirty-one patients had functional single ventricle; only 8 of these with tricuspid atresia, five patients had complex forms of double outlet right ventricle (DORV), and four complex A-V canal. Previous palliative procedures, mostly systemic-pulmonic shunts, were performed in 34 patients. Concomitant procedures were required in 18 patients, mainly reconstruction of distorted pulmonary arteries. A subgroup of 14 high risk patients, that did not fulfil the classical Fontan criteria, underwent 4 mm fenestration of the intra-atrial baffle. There were three (7.5%) early postoperative deaths which occurred in the higher risk group (fenestrated group). However, the remaining patients were all in functional class I or II. Total cavopulmonary connection provides reasonably good definitive palliation for patients with single ventricle physiology. Fenestration of the intra-atrial baffle increases the number of candidates suitable for the Fontan procedure, although the exact inclusion criteria for these patients has yet to be defined.