[MRI for therapy control in patients with tetralogy of Fallot]. / MRT zur Therapiekontrolle bei Patienten mit Fallot-Tetralogie.

With prevalences ranging from 0.26 to 0.8‰ of all live births tetralogy of Fallot (TOF) is the most common congenital heart disease with primary cyanosis. Due to improvements in surgical techniques, nearly all patients can nowadays expect to reach adulthood. After surgical repair, pulmonary regurgitation (PR) occurs in almost every child and is an important contributing factor in long-term morbidity and mortality. Cardiac magnetic resonance imaging is well established for functional assessment and flow measurements and is an ideal tool for serial post-surgical follow-up examinations, as it is non-invasive and does not expose patients to ionizing radiation. The timing of pulmonary valve replacement is crucial as right ventricular (RV) volumes have only proven to normalize when preoperative end-diastolic volumes are <170 ml/m(2) and end-systolic volumes are <85 ml/m(2). After surgical repair up to 15% of patients have residual or recurrent pulmonary artery stenosis. Distal pulmonary branch stenosis can aggravate PR and lead to right heart failure due to combined pressure and volume overload. Therefore, it has to be diagnosed in time and treated by angioplasty with or without stenting.

Successful surgical treatment of a neonate with prenatal diagnosis of severe Ebstein's anomaly.

We present a case of a patient with a severe form of Ebstein's anomaly diagnosed in a 36th week of gestation through prenatal echocardiographic evaluation. The emergency surgery consisted of closure of the tricuspid valve and creation of a residual atriventricular communication, enlargement of interatrial communication, closure of the pulmonary artery, and construction of an aortopulmonary shunt. Subsequently, the patient underwent the hemiFontan and Fontan operation. We conclude that prenatal echocardiographic diagnosis is crucial for the survival of children with a severe form of Ebstein's anomaly, which is a neonatal emergency and requires prompt decision mating to initiate surgical management.

Successful surgical treatment of a mycotic pseudoaneurysm of the ascending aorta in an infant after hypoplastic left heart complex repair.

We describe a successfully treated pseudoaneurysm of the ascending aorta in a 2-month-old infant after a previous operation due to hypoplastic left heart complex. After a rapidly progressing respiratory tract obstruction as a first symptom, echocardiography revealed a huge aneurysmal formation originating from the anterior and right lateral wall of the ascending aorta. A patch aortoplasty was employed.

Surgical treatment of aortopulmonary window with tetralogy of Fallot.

An association between aortopulmonary window and tetralogy of Fallot is rare. We report a case of the combined anomalies in which, possibly for the first time, the diagnosis was made by echocardiography alone, and in which successful surgical treatment was performed in early infancy.

Surgical repair of anomalous origin of the coronary artery from the pulmonary artery in children.

OBJECTIVE: To evaluate surgical management of children with coronary artery anomalies, including patients with rare abnormalities (left anterior descending artery or right coronary artery originating from the pulmonary artery). METHODS: A retrospective review of eight patients (aged 2 months--10 yr; median 3.5 yr), treated for coronary artery anomalies between 1989 and 1999. Coronary vessels were reimplanted to the aorta in four children, while the remaining four were subjected to the Takeuchi procedure. RESULTS: All children survived. None required mechanical circulatory support. In six patients significant mitral valve insufficiency developed, nevertheless, no intraoperative mitral valvuloplasty was performed. Follow-up was 2 months--10 yr. All patients developed normally and are currently in NYHA class I. Echocardiography revealed normal FS and EF values within 1--17 months postoperatively. CONCLUSIONS: In children with coronary artery anomalies, the best results are achieved with an early diagnosis and aggressive management. In our opinion coronary vessel reimplantation and the Takeuchi procedure yields comparable results.

Hypoplastic left heart syndrome with an anomalous origin of the left coronary artery.

Abnormal origin of the coronary artery in children with hypoplastic left heart syndrome is an extremely rare defect. We describe a newborn with concomitant hypoplastic left heart syndrome and abnormal origin of the left coronary artery arising from the right pulmonary artery. A Norwood procedure and direct reimplantation of the left coronary artery to the ascending aorta was performed.

[Results of surgical treatment of congenital heart defects in infants below 2500 grams]. / Wyniki operacyjnego leczenia wrodzonych wad serca u dzieci z masa ciala ponizej 2500 g.

We present a review of our recent experience of operating on infants below 2500 g suffering from congenital heart disease. A retrospective review was performed in 73 children who had undergone cardiac operations at our institution from 1990 to 1999. There were 43 (59%) females and 30 (41%) males; the mean age at operation was 28 days (range 4 to 92). The mean gestational age was 34.7 weeks (range 24 to 41), mean birth weight--1899 g (range 700 to 2450) and mean weight at operation--2013 g (range 640 to 2500). Cardiac diagnoses included patent ductus arteriosus (PDA) (N = 21, 28.7%), ventricular septal defect (VSD) (N = 9, 12.3%), transposition of great arteries (TGA) (N = 10, 13.7%), tetralogy of Fallot (TOF) (N = 7, 9.6%), double-outlet right ventricle (DORV) (N = 4, 5.5%), truncus arteriosus communis (TAC) (N = 4, 5.5%), hypoplastic left heart syndrome (HLHS) (N = 4, 5.5%), coarctation of aorta (CoAo) (N = 6, 8.2%), total anomalous pulmonary venous return (TAPVR) (N = 2, 2.7%), aortic stenosis (AoVS) (N = 3, 4.1%), interrupted aortic arch (IAA) (N = 1, 1.4%), pulmonary atresia (PA) (N = 1, 1.4%) and common atrioventricular canal (CAVC) (N = 1, 1.4%). Hospital mortality was 16.4%. There were 6 early deaths (8.2%) and 6 late deaths (8.2%). A higher mortality rate was noted in premature children and in children who had undergone palliative procedures. At a 1-116-month follow-up (mean 39 months), of 57 (93.4%) patients, 46 (80.7%) were in NYHA class I and 11 (19.3%) in NYHA class II. In conclusion the authors believe that early intervention can be performed in infants below 2500 g, and the associated mortality and morbidity rates are low.

Factors influencing early outcome of Norwood procedure for hypoplastic left heart syndrome.

OBJECTIVE: [corrected] The operative outcome of the Norwood procedure for hypoplastic left heart syndrome is still not satisfactory. Conflicting reports concern factors associated with early Norwood procedure mortality and the reasons for the instability after surgery are not sufficiently understood. The purpose of this study was to determine some perioperative factors influencing early (30 days) outcome. METHODS: We retrospectively analyzed a group of 30 consecutive children with hypoplastic left heart syndrome (aged 5-39 days) who underwent Norwood procedure in 1997 and 1998. The following factors were considered and statistically analyzed: operative age, birth weight, operative weight, serum level of bilirubin, aminotransferases, creatinine, urea, arterial blood gasses, anatomic subgroups, ascending aorta and arch size, systemic to pulmonary modified right Blalock-Taussig shunt size, cardiopulmonary circulatory arrest time, cardiopulmonary bypass time, and delayed chest closure. Eighteen patients underwent hemi-Fontan procedure with one late death and the modified Fontan operation was performed in 16 of them (one late death). RESULTS: The early mortality was 37%. Seven deaths (64%) occurred during the first 24 h after operation. There was a significant difference between survivals and non-survivals in: birth weight (P=0.047), operative age (P=0.016), preoperative serum level of bilirubin (P=0.044), and cardiopulmonary circulatory arrest time (P=0.006). The other assessed factors were not found to be predictors of early mortality. All 16 survivals followed up are in New York Heart Association class I or II. CONCLUSIONS: Anatomic and functional status of the patient, as well as procedural factors are related to Norwood operation early mortality. High mortality in hypoplastic left heart syndrome after stage I surgery indicates the necessity of assessing all factors which may determine further improvement in the outcome.

[Operative outcome in children with total anomalous pulmonary venous connection]. / Wyniki operacyjnego leczenia dzieci z calkowitym nieprawidlowym polaczeniem zyl plucnych.

The authors present their experience in surgical treatment of children with total anomalous pulmonary venous connection. A retrospective analysis was carried out in 42 patients operated on in the years 1979-1999. The group included 30 boys aged 7 days to 7 years (mean 170 +/- 480 days) and 12 girls aged 14 days to 7 months (mean 86 +/- 80 days). Diagnostic studies allowed to determine that 24 patients (57%) had supracardiac type (type I), nine (21%)--cardiac (type II), five (12%)--infracardiac (type III) and four (10%)--mixed (type IV). The diagnosis was based on clinical examinations and echocardiography, supplemented in 17 patients (40%) by hemodynamic studies and angiocardiography. Preoperative pulmonary hypertension was noted in 34 children (81%), including 15 patients (36%) diagnosed by hemodynamic studies and 19 children (45%) by echocardiography. All children were operated on in deep hypothermia with circulatory arrest. In the early postoperative period (30 days), seven children (17%) died, while four more (9.5%) died in the latter period. The former were characterized by significantly lower birth weight values (p = 0.023). The total mortality rate was higher in children with type II and III anomalies. No statistically significant association was found between preoperative pulmonary hypertension and early mortality. Thirty-one children (74%) remain in late follow-up. All of them are in NYHA (New York Heart Association) class I and develop normally. Total anomalous pulmonary venous connection is a rare congenital anomaly that can be fully repaired with improving early and late results.

Operative treatment of truncus arteriosus communis coexisting with tricuspid atresia.

The coexistence of tricuspid atresia and common arterial trunk is extremely rare. We present a successful three-stage surgical treatment of this defect. The first stage included disconnection of the pulmonary arteries from the common trunk, atrial septectomy and systemic-to-pulmonary shunt; the second stage, a hemi-Fontan procedure; and the third stage, a fenestrated Fontan completion. The child is now 8 years old and is developing well.

Primary reconstruction of interrupted aortic arch--surgical management and results.

Type B interrupted aortic arch associated with other extracardiac and intracardiac congenital lesions is a lethal defect. Surgical management with a one- or two-stage approach is complex, difficult and associated with high morbidity and mortality. Between January 1996 and December 1999, 5 consecutive patients with type B interrupted aortic arch were operated on. The group included 3 girls and 2 boys weighing from 2.4 kg to 3.5 kg (mean 2.84 kg) and aged from 4 to 35 days (mean 15 days). All patients underwent a primary reconstruction of the aortic arch and one-stage repair of the associated heart defects. Two patients developed symptoms of mild aortic arch narrowing 7-12 months postoperatively. Balloon angioplasty of the obstructed site was successfully carried out in one of the patients. In the second child, angioplasty was not successful and surgery was used. All the patients survived and are being followed-up. This experience supports a strategy of primary one-stage repair of IAA type B, including direct anastomosis for aortic arch reconstruction, and all associated heart anomalies in the neonatal period.

Results of surgical treatment of congenital heart defects in children with Down's syndrome.

We analyzed early and late results of surgical treatment of 100 consecutive children with Down's syndrome (DS) and congenital heart defect (CHD) who were operated on between 1990 and 1997. Fifty had common atrioventricular canal (CAVC), 24 ventricular septal defect, 8 the ostium primum atrial septal defect, 8 tetralogy of Fallot (TOF), 3 patent ductus arteriosus, 3 the ostium secundum atrial septal defect, and 4 CAVC coexisting with TOF. In 93 patients total correction was performed. The total death rate was 6%. Death in the CAVC group was 8%, but it decreased to 2.7% during the past 3 years. The children who were followed up (from 7 months to 6 years; mean, 39 months) are in NYHA class I or II. There were no reoperations. The postoperative course was complicated by pulmonary infections in 38% of patients, which converted to generalized infection in 10% and was the cause of death in 8% of patients. These results indicate that CHD in DS children can be repaired with a low death rate and low incidence of severe mitral atrioventricular valve regurgitation in the CAVC group. A high incidence of severe infections can influence the final results. Repair of CHD in infancy helps to eliminate problems connected with congestive heart failure and pulmonary hypertension.

Efficacy of buspirone in alcohol dependence: a review.

The five published controlled studies on the effects of buspirone in alcoholism treatment are reviewed. They have been conducted mostly in alcoholics with comorbid anxiety. Significant differences in favor of the medication were observed in several psychopathological measures (anxiety, depression, hostility, interpersonal sensitivity, and global psychopathology). In only two studies were alcohol craving and consumption found influenced. Metaanalysis showed positive effects of buspirone on treatment retention, as well as on anxiety. It can be concluded that the main effect of buspirone in the treatment of alcoholism is not on ethanol consumption per se, but on associated psychopathological symptoms. A favorable safety profile and a lack of interaction with alcohol make buspirone a useful pharmacological adjunct in the treatment of alcoholism.

Buspirone in the treatment of alcohol dependence: a placebo-controlled trial.

A double-blind controlled study of the effects of buspirone on alcohol dependence and associated symptoms in ambulatory alcoholics showed a marked improvement in both treatment and control groups. However, significant differences in favor of the medication were observed in several psychopathological measures, but not in measures of alcohol consumption. Contrary to a majority of prior controlled trials of buspirone in alcoholics, subjects were not selected on the basis of comorbid generalized anxiety; rather, the study tested the hypothesis of a direct effect on craving and consumption, independent from an anxiolytic effect. This hypothesis was not confirmed.