Long-term follow-up and sex differences in adults operated for tetralogy of Fallot.

OBJECTIVE: Adults operated for tetralogy of Fallot (TOF) have high risk of ventricular arrhythmias (VA). QRS duration >180 ms is an established risk factor for VA. We aimed to investigate heart function, prevalence of arrhythmias and sex differences in patients with TOF at long-term follow-up. METHODS: We included TOF-operated patients≥18 years from our centre's registry. We reviewed medical records and the most recent echocardiographic exam. VA was recorded on ECGs, 24-hour Holter registrations and from implantable cardioverter defibrillator. RESULTS: We included 148 patients (age 37±10 years). Left ventricular global longitudinal strain (LV GLS, -15.8±3.1% vs -18.8±3.2%, p=0.001) and right ventricular (RV) GLS (-15.8±3.9% vs -19.1±4.1%, p=0.001) were lower in men at all ages compared with women. Higher RV D1 (4.3±0.5 cm vs 4.6±0.6 cm, p=0.01), lower ejection fraction (55%±8% vs 50%±9%, p=0.02), lower RV GLS (-18.1±4.0 ms vs -16.1±4.8 ms, p=0.04) and N-terminal pro-brain natriuretic peptide (NT-proBNP) over reference range (n=27 (23%) vs n=8 (77%), p<0.001) were associated with higher incidence of VA. QRS duration was longer in men (151±30 ms vs 128±25 ms, p<0.001). No patients had QRS duration >180 ms. QRS duration did not differ in those with and without VA (143±32 ms vs 137±28 ms, p=0.06). CONCLUSIONS: Our results confirmed reduced RV function in adults operated for TOF. Male patients had impaired LV and RV function expressed by lower LV and RV GLS values at all ages. Reduced cardiac function and elevated NT-proBNP were associated with higher incidence of VA and may be important in risk assessment.

Impact of pregnancy and risk factors for ventricular arrhythmias in women with tetralogy of Fallot.

OBJECTIVE: Patients with tetralogy of Fallot (TOF) have high survival rates 30 years after surgical repair. Many patients experience pregnancy; however, the effects of pregnancy on the long-term cardiovascular outcome are not well known. We investigated the association of pregnancy and cardiac function with occurrence of ventricular arrhythmia (VA) in women with TOF. METHODS: We recruited 80 women with repaired TOF from the national database. Holter monitoring or implanted devices detected VA, defined as non-sustained or sustained ventricular tachycardia or aborted cardiac arrest. All patients underwent echocardiography. Blood tests included NT-proBNP (N-terminal pro-brain natriuretic peptide). RESULTS: 55 (69%) women had experienced pregnancy. Mean age was lower in nulliparous compared with those with children (30±9 vs 40±9, p<0.01).VA had occurred in 17 (21%) women. Prevalence of VA was higher in women who had experienced pregnancy (n=16, 94%) compared with nulliparous (n=1, 6%) (p=0.02), also when adjusted for age (OR 12.9 (95% CI 1.5 to 113.2), p=0.02).Right ventricular mechanical dispersion was more pronounced in patients with VA (50±8 ms vs 39±14 ms, p=0.01, age-adjusted OR 2.1 (95% CI 1.3 to 7.5), p=0.01). NT-proBNP was also a marker of VA (211 ng/L (127 to 836) vs 139 ng/L (30 to 465), p=0.007). NT-proBNP >321 ng/L (normal values <170 ng/L) detected women with VA (p=0.019), also independent of age (OR 7.2 (95% CI 1.7 to 30.1), p=0.007). CONCLUSION: Pregnancy was associated with higher prevalence of VA among women with TOF. Right ventricular mechanical dispersion and NT-proBNP were age-independent markers of VA. These may have importance for pregnancy counselling and risk stratification.

Off-pump hepatic to azygos connection via thoracotomy for relief of fistulas after a Kawashima procedure: Ten-year results.

OBJECTIVES: An almost universal incidence of developing pulmonary arteriovenous fistulas after the Kawashima operation has been reported. Exclusion of the hepatic venous flow from the pulmonary circulation causes the development of these malformations. Redirection of hepatic venous flow to the pulmonary circulation mostly leads to the regression of the arteriovenous fistulas. METHODS: We analyzed 11 patients with arteriovenous fistulas that developed after the Kawashima operation. The hepatic-to-azygos shunts were performed with an off-pump technique through a lateral thoracotomy in all but one. Operative and postoperative data were retrospectively collected. RESULTS: No intraoperative complications occurred, and no patient died in the hospital. Up to 10-year follow-up showed a significant postoperative improvement of patients' oxygen saturation and New York Heart Association class. Apart from 2 re-thoracotomies for bleeding in 1 patient, no complications occurred and no patient died during follow-up. Two other patients underwent reoperation for an undiagnosed additional hepatic vein. The improvement of patients' oxygen saturation and New York Heart Association class persisted during the follow-up period. CONCLUSIONS: The surgical connection can be performed safely with an off-pump technique that avoids the risks related to extracorporeal circulation and circulatory arrest. The results at 10 years follow-up confirmed the efficacy and safety of the surgical technique described.

Pulmonary valve replacement with a bovine pericardial valve: a five year follow-up study.

OBJECTIVES: From a population of 90 patients after pulmonary valve replacement with a biological valve (Carpentier-Edwards Perimount valve), 56 of 80 available patients were examined five years after surgery. BACKGROUND: Pulmonary valve replacement is needed in many patients with congenital heart disease. Homografts have limited availability and predictable degeneration, and mechanical valves require anticoagulation. No superiority of one kind of pulmonary valve replacement has been shown. Biological valves that are readily available are being used and evaluated in increasing numbers. METHODS: In this cross-sectional study, five years following surgery, data were gathered from hospital charts, echocardiography, stress echocardiography, magnetic resonance imaging, and exercise testing. RESULTS: In 90 patients, there were three new valve replacements, one early cardiac death, and four late noncardiac deaths. Echocardiographic assessment of the study group showed pulmonary Doppler velocities (m/s) before, after operation, and at five-year follow-up of 2.8±1.1, 1.6±0.4, and 2.3±0.7, respectively. The assessed insufficiencies (0-3) at the same times were 2.3±1.0, 0.3±0.4, and 1.1±0.8. Maximal oxygen uptake increased from 65.6%±10.1% to 77.1%±18.2% of predicted and QRS width increased by 7±23 ms. Valve degeneration could be associated with young age but not with diagnosis or valve size. CONCLUSION: In our study, the biological valve in the pulmonary position showed excellent mid-term results with few reoperations, low gradients, and mild to moderate insufficiency. Oversizing, in contrast to young age, was not a risk factor for valve degeneration. In younger patients, this allows later percutaneous replacement, reducing the need for further surgery. However, longer follow-up is needed.

Combined repair of upper sternal cleft and transposition of the great arteries in a newborn.

We report the case of a newborn with the unusual association of an upper sternal defect and transposition of the great arteries. Surgical correction of the cardiac disease consisted of the arterial switch procedure. The already less compliant bony thorax of the infant made direct approximation of the upper sternal defect only possible with adjuvant bilateral chondrotomy. Sternal cleft repair is advised during the very first weeks of life.

Single-center 50 years' experience with surgical management of tetralogy of Fallot.

OBJECTIVE: The aim of this study was to evaluate the long-term outcome of total repair for tetralogy of Fallot. We aimed to characterize late survival and the time-related risk of late reoperation. METHODS: Operative protocols, patient records, and the database of the department were evaluated from 1951 until 2008. The official death registry of Norway was used for follow-up. Of the patients identified, the follow-up was 99.6% complete. RESULTS: A total of 627 patients were studied. Of these, 570 could be identified for follow-up. There were a total of 41 early and 30 late deaths. The total early (including palliative procedures) mortality was 7.2% and total late mortality was 7.9%. However, during the last 10 years, no early mortality has been observed following repair. A total of 264 patients underwent some form of palliative procedure as their first treatment, and 541 patients had a reparative procedure performed, with an early mortality of 31 (5.7%). In patients subjected to a reparative procedure, there was no difference in freedom from death or reoperation following primary repair versus primary palliation. The use of transannular patch was associated with a highly significant risk of reoperation. CONCLUSIONS: Surgical treatment of the tetralogy of Fallot and related congenital cardiac malformations has good long-term prognosis. In this cohort of patients, more than one-third required additional procedures later on, and, in some cases, as many as four additional surgeries. Palliative procedures followed by repair do not influence survival or reoperation-free survival. There are no differences between transatrial versus transventricular repair on survival or re-repair. Any transannular incision increases the risk of re-repair, but does not influence long-time survival. There is an almost linear decrease in reoperation-free survival following any type of repair of tetralogy of Fallot, even for as long as 50 years since the first procedure.

Eight years of pulmonary valve replacement with a suggestion of a promising alternative.

UNLABELLED: In a retrospective study we assessed surgical results following right ventricular to pulmonary artery connection repair or replacement at a medium of 2.4 years (0-8) follow-up. Data were retrieved from hospital charts. RESULTS: Three hundred and sixty five operations were performed in 286 patients in eight years starting in 2000 using different surgical methods. Homografts and Monocusps had a more than 50% significantly lower risk for reoperation than Contegra or bicuspid valves (p < 0.01). Data for infants and older children and grown ups were analysed separately. In the infant group no significant difference between the different methods (homograft, Contegra and Monocusp) was detected. In older patients, the Perimount valves performed extremely well with no need for reoperation after 2.5 years of follow-up. Perimount valves and homografts performed better than other solutions (p = 0.01). CONCLUSION: Although the follow-up for the Perimount valves was short, they are promising and need to be followed long-term. The homograft and the Monocusp remain valuable choices.

A novel method of hybrid intraoperative catheter-based closure of ventricular septal defects using the Amplatzer® PDA occluder.

BACKGROUND: In five patients, an apical muscular septal defect was closed in a hybrid approach using the Amplatzer® duct occluder during open heart surgery, whereas concomitant defects were treated surgically. In addition to their different heart defects that needed surgery, all had a muscular ventricular septal defect in the apex of the heart, poorly accessible for traditional, surgical approach. We describe the method and outcome in these patients. METHODS: The tip of a forceps was advanced from the left into the right ventricle through the ventricular septal defect. The delivery sheath was caught under visual control in the right ventricle and pulled back into the left ventricle. The disc was developed and pulled back until it was felt tugging at the septum. Then the core was developed. The end of the device was visible in the right ventricle and was secured with a Prolene® suture. RESULTS: The procedures were event-free. During early follow-up there were either no or only insignificant shunts in the region of the prior trabecular defects in four patients. The unsatisfactory result in the last patient was caused by inaccurate preoperative assessment. CONCLUSION: The method seems valuable in patients in need of other intracardiac surgery.

Perfusion temperature, thyroid hormones and inflammation during pediatric cardiac surgery.

OBJECTIVES: During cardiopulmonary bypass (CPB) surgery there are several alterations in concentrations of thyroid hormones. Although hypothermia and inflammation have been implicated in the disturbed thyroid axis during CPB, these issues are far from clear. METHODS AND RESULTS: We measured serum/plasma concentrations of thyroid hormones and inflammatory mediators in children with body weight <10 kg, undergoing open heart surgery, randomized to mild (n=15, 32 degrees C) or moderate (n=15, 25 degrees C) hypothermia. During CPB there was a marked decrease in triiodothyronine (T3), free thyroxin (FT4) and thyroid-stimulating hormone (TSH), followed by a slight increase after 24 h, but without normalization 48 h after CPB. There was no difference in the thyroid response between the two hypothermia groups. During CPB the maximal changes in plasma levels of interleukin (IL)-6 and the chemokines, regulated on activation normal T cell expressed and secreted (RANTES) and monocyte chemoattractant protein (MCP)-1 were inversely correlated with the maximal changes in serum levels of T3. CONCLUSION: Our findings in this randomized trial do not support a role for hypothermia as a major cause of altered thyroxin responses in children undergoing CPB. Our finding may also suggest that in addition to IL-6, other inflammatory cytokines, such as chemokines, should be further investigated for their possible influence on the thyroid axis during CPB.

Management of 239 patients with hypoplastic left heart syndrome and related malformations from 1993 to 2007.

BACKGROUND: We reviewed our entire programmatic operative experience with children with hypoplastic left heart syndrome (HLHS) and related malformations. METHODS: As of October 1, 2007, 239 patients with HLHS and related malformations underwent surgical treatment at the Congenital Heart Institute of Florida. This manuscript focuses on the 199 initially treated with Norwood stage 1. RESULTS: One hundred and ninety-nine patients were initially treated with Norwood stage 1. Univariate analysis demonstrated the following significant predictors of mortality: right ventricular dominance (p = 0.0023), mechanical circulatory support before stage 1 (p = 0.0192), and significant noncardiac abnormality or syndrome, including Down syndrome, Turner syndrome, heterotaxy, asplenia, polysplenia, biliary atresia, or other chromosomal abnormality (p < 0.0001). Multivariable logistic regression analysis revealed the presence of a significant noncardiac abnormality or syndrome or prematurity less than 35 weeks or mechanical circulatory support before stage 1 to be a significant predictor of mortality (p < 0.0001). Over the 14 years of this patient series, survival for the 157 "low-risk" patients managed with Norwood staged palliation (those patients without significant noncardiac abnormality or syndrome or prematurity less than 35 weeks or mechanical circulatory support prior to Stage 1) was 86%, 80%, and 69% at 30 days after Stage 1, hospital discharge after Stage 1, and 1 year of after Stage 1, respectively. CONCLUSIONS: Several treatment options are available for HLHS and related malformations. The appropriate treatment strategy must be matched to the individual patient, taking into consideration anatomic variables as well as other patient-specific characteristics. The majority of patients with HLHS and related malformations can undergo successful staged palliation with risk that varies according to several documented anatomic and patient-specific variables.

Effect of perfusion temperature on the inflammatory response during pediatric cardiac surgery.

BACKGROUND: Cardiopulmonary bypass (CPB) triggers the whole body inflammatory response, and it has been suggested that the degree of hypothermia may influence these responses. The aim of this prospective study was to compare the inflammatory response in children undergoing CPB for repair of congenital heart defects, randomized to mild or moderate hypothermia. METHODS: We measured inflammatory markers in blood samples of thirty children with body weight less than 10 kg undergoing open heart surgery randomized to surgery at either mild (32 degrees C) or moderate (25 degrees C) hypothermia. Blood was sampled after induction of anesthesia, at skin closure, 2 hours, 24 hours, and 48 hours postoperatively. RESULTS: Except for an enhanced interleukin-8 response in the moderate hypothermia group, there were no differences in levels of inflammatory mediators between those with mild and those with moderate hypothermia. In contrast to the modest influence of the degree of hypothermia, long CPB time and long aortic cross-clamp time were accompanied by enhanced inflammation involving raised levels of interleukin-8 and myeloperoxidase, as well as increased leukocyte counts. CONCLUSIONS: Only minor differences in cytokine levels were detected between those with moderate and those with mild hypothermia during CPB. Ischemic aortic cross-clamp time and time on CBP should be as short as possible to avoid an excessive inflammatory response and possibly adverse clinical effects.

Successful reconstruction of traumatic carinal tissue loss using the esophagus in an infant.

We present a case report of an infant who underwent successful reconstruction of a traumatic tracheal and carinal chemically induced corrosive injury using an esophageal flap to reconstruct the trachea and subsequently re-establishing gastrointestinal continuity with a colon interposition.

ALCAPA, a possible reason for mitral insufficiency and heart failure in young patients.

OBJECTIVES: ALCAPA, anomalous origin of the left coronary artery from the pulmonary artery is a rare congenital disease. We have from 1990 to 2003 operated on seven patients in our clinic. The objective of this study was to describe our experience of treating the condition and to remind other doctors of it as a possible diagnosis. DESIGN: The patients were identified by a retrospective review of our clinical records. RESULTS: Symptoms and signs varied from discomfort and pathologic heart murmur to ventricular fibrillation. All patients were operated on by use of cardiopulmonal bypass. Two died postoperatively within one week, two were reoperated later on because of pulmonary artery stenosis. CONCLUSION: ALCAPA should be suspected if a young patient with no previous history of heart failure presents with dyspnoe, chest pain or dysrhythmia. ALCAPA must be excluded by coronary angiography in younger patients with mitral insufficiency and no other morphological findings. Patients diagnosed early and operated on have a good prognosis.

Mentorship, learning curves, and balance.

Professionals working in the arena of health care face a variety of challenges as their careers evolve and develop. In this review, we analyze the role of mentorship, learning curves, and balance in overcoming challenges that all such professionals are likely to encounter. These challenges can exist both in professional and personal life. As any professional involved in health care matures, complex professional skills must be mastered, and new professional skills must be acquired. These skills are both technical and judgmental. In most circumstances, these skills must be learned. In 2007, despite the continued need for obtaining new knowledge and learning new skills, the professional and public tolerance for a "learning curve" is much less than in previous decades. Mentorship is the key to success in these endeavours. The success of mentorship is two-sided, with responsibilities for both the mentor and the mentee. The benefits of this relationship must be bidirectional. It is the responsibility of both the student and the mentor to assure this bidirectional exchange of benefit. This relationship requires time, patience, dedication, and to some degree selflessness. This mentorship will ultimately be the best tool for mastering complex professional skills and maturing through various learning curves. Professional mentorship also requires that mentors identify and explicitly teach their mentees the relational skills and abilities inherent in learning the management of the triad of self, relationships with others, and professional responsibilities.Up to two decades ago, a learning curve was tolerated, and even expected, while professionals involved in healthcare developed the techniques that allowed for the treatment of previously untreatable diseases. Outcomes have now improved to the point that this type of learning curve is no longer acceptable to the public. Still, professionals must learn to perform and develop independence and confidence. The responsibility to meet this challenge without a painful learning curve belongs to both the younger professionals, who must progress through the learning curve, and the more mature professionals who must create an appropriate environment for learning. In addition to mentorship, the detailed tracking of outcomes is an essential tool for mastering any learning curve. It is crucial to utilize a detailed database to track outcomes, to learn, and to protect both yourself and your patients. It is our professional responsibility to engage in self-evaluation, in part employing voluntary sharing of data. For cardiac surgical subspecialties, the databases now existing for The European Association for CardioThoracic Surgery and The Society of Thoracic Surgeons represent the ideal tool for monitoring outcomes. Evolving initiatives in the fields of paediatric cardiology, paediatric critical care, and paediatric cardiac anaesthesia will play similar roles.A variety of professional and personal challenges must be met by all those working in health care. The acquisition of learned skills, and the use of special tools, will facilitate the process of conquering these challenges. Choosing appropriate role models and mentors can help progression through any learning curve in a controlled and protected fashion. Professional and personal satisfaction are both necessities. Finding the satisfactory balance between work and home life is difficult, but possible with the right tools, organization skills, and support system at work and at home. The concepts of mentorship, learning curves and balance cannot be underappreciated.

Risk of surgery for congenital heart disease in the adult: a multicentered European study.

BACKGROUND: Surgery for congenital heart disease (CHD) has changed considerably during the last three decades. The results of primary repair have steadily improved, to allow treating almost all patients within the pediatric age; nonetheless an increasing population of adult patients requires surgical treatment. The objective of this study is to present the early surgical results of patients who require surgery for CHD in the adult population within a multicentered European study population. METHODS: Data relative to the hospital course of 2,012 adult patients (age > or = 18 years) who required surgical treatment for CHD from January 1, 1997 through December 31, 2004 were reviewed. Nineteen cardiothoracic centers from 13 European countries contributed to the data collection. RESULTS: Mean age at surgery was 34.4 +/- 14.53 years. Most of the operations were corrective procedures (1,509 patients, 75%), followed by reoperations (464 patients, 23.1%) and palliative procedures (39 patients, 1.9%). Six hundred forty-nine patients (32.2%) required surgical closure of an isolated ostium secundum atrial septal defect. Overall hospital mortality was 2%. Preoperative cyanosis, arrhythmias, and NYHA class III-IV, proved significant risk factors for hospital mortality. Follow-up data were available in 1,342 of 1,972 patients (68%) who were discharged home. Late deaths occurred in 6 patients (0.5%). Overall survival probability was 97% at 60 months, which is higher for corrective procedures (98.2%) if compared with reoperations (94.1%) and palliations (86.1%). CONCLUSIONS: Surgical treatment of CHD in adult patients, in specialized cardiac units, proved quite safe, beneficial, and low-risk.

Rescue cardiac transplantation for failing staged palliation in patients with hypoplastic left heart syndrome.

OBJECTIVE: Orthotopic heart transplantation is considered a rescue option for children with failing staged palliation or repair of hypoplastic left heart syndrome. We present our strategy for management, and outcomes, for these complex patients. METHODS: We transplanted 68 consecutive children, with diagnoses of hypoplastic left heart syndrome in 31, cardiomyopathy in 20, and post-operative complex congenital heart disease in 17. Of these patients, 9 (13.2%) were neonates, and 46 (67.6%) were infants. Median age was 118.5 days. Operative technique involves bicaval cannulation and anastamoses with continuous low flow bypass, and either short periods of circulatory arrest or continuous low flow antegrade cerebral perfusion for reconstruction of the aortic arch. Initial reperfusion of the donor heart utilizes glutamate and aspartate substrate enriched white blood cell filtered cardioplegia. Immunosuppressive therapy includes induction (pulse steroids, gamma globulin, and polyclonal rabbit antithymocyte globulin) and initial maintenance (calcineurin inhibitor, an anti-proliferative agent, and a weaning steroid protocol). Of the 31 patients with hypoplastic left heart syndrome, 23 underwent primary transplantation, and 8 underwent rescue transplantation from failing staged palliation in seven, or attempted biventricular repair in one. Of the seven patients who had failing staged palliation, three had undergone only the Norwood Stage 1 operation, 2 had undergone a Norwood Stage 1 operation and a Glenn superior cavopulmonary anastomosis and two had undergone a Norwood Stage 1 operation, a Glenn superior cavopulmonary anastomosis, and a completion Fontan operation. RESULTS: The group undergoing primary transplantation was younger (p equals 0.007), weighed less (p equals 0.003), and waited longer for an appropriate donor heart (p equals 0.021) compared to those requiring rescue transplantation. No significant difference exists between the groups with regards to donor heart ischaemic time or post-transplant length of hospital stay. Thirty day survival (p equals 0.156) and overall survival (p equals 0.053) was better in those having primary transplantation, although these differences were not statistically significant when a p value of less than 0.05 is considered to be significant. In those having primary transplantation, no patients had elevated panel reactive antibody greater than 10%. Half of the 8 requiring rescue transplantation had panel reactive antibody greater than 10%, and this subgroup did especially poorly. CONCLUSION: Cardiac transplantation can offer children with failing staged palliation their only chance of survival. Transplantation, however, carries a high risk in this subgroup, especially in the setting of elevated panel reactive antibody.

The arterial switch operation in Europe for transposition of the great arteries: a multi-institutional study from the European Congenital Heart Surgeons Association.

OBJECTIVES: This study analyzes the results of the arterial switch operation for transposition of the great arteries in member institutions of the European Congenital Heart Surgeons Association. METHODS: The records of 613 patients who underwent primary arterial switch operations in each of 19 participating institutions in the period from January 1998 through December 2000 were reviewed retrospectively. RESULTS: A ventricular septal defect was present in 186 (30%) patients. Coronary anatomy was type A in 69% of the patients, and aortic arch pathology was present in 20% of patients with ventricular septal defect. Rashkind septostomy was performed in 75% of the patients, and 69% received prostaglandin. There were 37 hospital deaths (operative mortality, 6%), 13 (3%) for patients with an intact ventricular septum and 24 (13%) for those with a ventricular septal defect (P < .001). In 36% delayed sternal closure was performed, 8% required peritoneal dialysis, and 2% required mechanical circulatory support. Median ventilation time was 58 hours, and intensive care and hospital stay were 6 and 14 days, respectively. Although of various preoperative risk factors the presence of a ventricular septal defect, arch pathology, and coronary anomalies were univariate predictors of operative mortality, only the presence of a ventricular septal defect approached statistical significance (P = .06) on multivariable analysis. Of various operative parameters, aortic crossclamp time and delayed sternal closure were also univariate predictors; however, only the latter was an independent statistically significant predictor of death. CONCLUSIONS: Results of the procedure in European centers are compatible with those in the literature. The presence of a ventricular septal defect is the clinically most important preoperative risk factor for operative death, approaching statistical significance on multivariable analysis.

Repair of complex left ventricular outflow tract obstruction in children.

BACKGROUND: Complex obstruction of the left ventricular outflow tract (LVOTO) in children may be treated with surgical procedures like the Ross-Konno or Koncz-Konno procedures, or modifications thereof. We present our results from the last 10 years. MATERIAL AND METHOD: During the period from December 1991 to April 2002, 17 patients/children with complex LVOTO were operated on. Patients treated with balloon valvuloplasty, open commisurotomy or simple valve replacement are excluded. The procedures performed were: Koncz-Konno (KK) or modified KK (n = 10), Ross-Konno (RK) (n = 2) and Ross (RO) (n = 5). Concomitant procedures included mitral valve replacement (n = 1), aortic arc plasty (n = 1), subvalvular myotomi (n = 1), resection of subvalvular membrane (n = 1) and aorto-coronary bypass (n = 1). Patients' ages ranged from 1 to 178 months, median 71 months. Body weights ranged from 3.8 to 50.0 kg, median 19.4 kg. Eight cases were redo procedures. RESULTS: Three early deaths occurred. One patient in the KK group, one in the modified KK group and one in the RK group. One patient died 8 years postoperatively of unknown reasons; no autopsy was performed. The rest of our patients who survived the initial major surgical treatment show normal physical performance and their quality of life seems to be good. CONCLUSION: Patients with complex LVOTO can be operated with biventricular repair in selected cases. Early and late mortality are acceptable.

[Transcatheter closure of atrial septal defects in the oval fossa]. / Kateterlukking av atrieseptumdefekter.

BACKGROUND: We report five years' experience with transcatheter closure of defects in the oval fossa at Rikshospitalet University Hospital in Oslo, Norway. MATERIAL AND METHODS: We reviewed the results in 69 patients between 1.3 and 74 years of age (median 5.3), weighing median 20 kg (7.8-109), with pulmonary blood flow 2.40.74 (1.3-4.8) times systemic flow, and median diameter of the defect 18 mm (9-34). The patients were followed clinically for one year. RESULTS: In 69/77 (90%) the defect could be closed. None had a significant residual shunt. One patient showed a very small residual leak after one year. Two children, not yet seen after one year, had a small shunt after one month. The complication rate has been low, and we have not been able to demonstrate any sequelae. The hospital stay is two nights without any convalescence period. The results compare favourably to surgery. INTERPRETATION: The method was efficient and safe in all age and weight groups. The very limited total volume of such procedures indicates they should be centralised and only carried out by a small number of physicians.