Edward Malec

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

Abstract. We analyzed early and late results of surgical treatment of 100 consecutive children with Downs 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.

Extracorporeal life support in severe propranolol and verapamil intoxication.

Combined poisoning with calcium-channel blockers and β-blockers is usually associated with severe heart failure. This report shows the effectiveness of emergency extracorporeal life support in treating life-threatening simultaneous propranolol and verapamil intoxication. A 15-year-old girl presented in cardiogenic shock after alcohol consumption and a propranolol and verapamil overdose; plasma concentrations: propranolol, 0.53 m/mL; verapamil, 1.06 mg/mL. She was successfully resuscitated with extracorporeal life support. Therapeutic plasma exchange was initiated. Extracorporeal support was discontinued 70 hours later. The patient made a full recovery. Simultaneous verapamil and propranolol overdoses can cause severe hemodynamic compromise and arrest of electrical and mechanical function of the heart. Emergency extracorporeal life support can successfully maintain vital organ blood flow and allows time for drug metabolism, redistribution, and removal. Therapeutic plasma exchange may reduce the time of emergency extracorporeal life support. Emergency extracorporeal life support should be considered early in cases of near-fatal intoxications with cardiodepressive drugs.

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

OBJECTIVEn[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.nnnMETHODSnWe 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).nnnRESULTSnThe 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.nnnCONCLUSIONSnAnatomic 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.

Cardiorespiratory Response to Exercise in Children after Modified Fontan Operation

Objective : Examination of exercise function of Fontan patients and comparison with healthy control subjects. Design : Fourteen patients (6 males, 8 females; age: 5.7-17 years, mean 8.1 years) after Fontan repair in New York Heart Association (NYHA) class I with rest O 2 sat > 85% requiring no cardiovascular medications performed graded exercises on a treadmill 0.5-3.2 years postoperatively (mean 1.8 years). During the tests the heart and respiratory rate, blood pressure, oxygen uptake, carbon dioxide production, minute ventilation, tidal volume and O 2 sat were recorded. Spirometry was performed before and during exercise. Results : The peak VO 2 max in Fontan patients was significantly reduced compared with controls ( p = 0.0002). Other parameters: anaerobic threshold ( p = 0.0001); pulsO 2 ( p = 0.00005); peak minute ventilation ( p = 0.0014); physiological dead space to tidal volume ratio at peak exercise ( p = 0.0004); maximal work rate ( p = 0.00008); exercise time ( p = 0.00003) were significantly reduced in univentricular patients. The heart rate at peak exercise was lower in the patients ( p = 0.0003) and O 2 sat dropped significantly ( p = 0.003). Conclusion : The aerobic capacity, work and ventilatory parameters in Fontan patients are markedly reduced compared with controls. The anaerobic threshold was significantly lower. The decreased O 2 sat at peak exercise may suggest intrapulmonary shunting.

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.

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.

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

OBJECTIVEnTo 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).nnnMETHODSnA 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.nnnRESULTSnAll 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.nnnCONCLUSIONSnIn 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.

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.

Anatomical correction of complex forms of transposition of the great arteries in neonates.

Objective—The arterial switch operation has become the procedure of choice for the simple transposition of the great arteries (TGA) while in the complex forms of the defect the staged approach is frequently implemented. The aim of this study is to compare groups of patients with simple transposition and children with the complex form of the defect and identify factors affecting early and late outcome. Design—From 1997 to 2003, 135 consecutive neonates with TGA underwent arterial switch operation and simultaneous reparation of all associated defects. Univariate and multivariate analysis of perioperative variables and follow‐up data was performed. Patients were divided into two groups. Group I (n=84, 62.2%) included neonates with simple transposition (TGA/IVS), Group II (n=51, 37.8%) included children with complex transposition (TGA/VSD). Results—Overall early mortality was 8.1% and there was one late death (0.7%). One‐month, 1‐year and 5‐year actuarial survival rates were 91.8, 91.1 and 91.1%, respectively. There were no differences in the early and late survival rate between groups. Reintervention rate for right ventricular outflow tract obstruction (RVOTO) was 13.3% (balloon plasty or reoperation). The freedom from reintervention at 1, 3 and 5 years was 98.4, 87.9 and 85.4%, respectively. There were no differences in the need for reintervention between groups. The significant differences between groups concerned: age at operation (pu2005<u20050.001), associated anomalies (p=0.002) including aortic arch anomalies (p=0.002) and coronary artery anomalies (p=0.02), application of delayed chest closure (p=0.015), and occurrence of sepsis (p=0.032). Risk factors for early death were: left ventricule dysfunction related to age at operation (p=0.016) and resternotomy in intensive care unit (pu2005<u20050.001). There were no differences between groups concerning these risk factors as far as circulatory arrest time, aorta clamping time, and early and late morbidity. Conclusions—The arterial switch operation can be the treatment of choice for various forms of TGA with low early and late mortality and morbidity rates. The main cause of early death is still left ventricular dysfunction. Such well‐known predictors of poor outcome as presence of ventricular septal defect, coronary artery anomalies and aortic arch anomalies did not affect early and late findings. The presented approach of early simultaneous anatomical correction of TGA and all associated anomalies ensures good condition of children with low necessity for reintervention.

Successful repair of aorto-left ventricular tunnel diagnosed prenatally

Aorto-left ventricular tunnels are anomalous communications between the ascending aorta and the left ventricle. Prenatal diagnosis, followed by successful surgical management, is rare. We describe a fetus diagnosed with aorto-left ventricular tunnel at 35 weeks who achieved a favourable outcome after immediate postnatal surgical repair.