Juha-Matti Happonen

Late results of senning operation

OBJECTIVES Few data exist for long-term results after the Senning operation for transposition of the great arteries. Sinus node dysfunction and systemic ventricular dysfunction have been the main problems. We evaluated risk factors for late death and the incidence of late death, sinus node dysfunction, and right ventricular dysfunction in 100 patients. METHODS The study was a retrospective analysis with a mean follow-up time of 12.8 +/- 3.1 years. No patients were lost to follow-up. Patients were divided in 2 groups according to ventricular septal defect (73 simple, 27 complex). The electrocardiogram, ambulatory electrocardiogram, echocardiogram, and chest radiograph were reviewed for each patient. RESULTS The overall mortality rate was 10%. The actuarial survival was 90% (simple) and 78% (complex); the probability of staying in sinus rhythm was 34% and 7%, and the probability of normal right ventricular function was 52% and 39%, respectively, 15 years after operation. The incidence of sinus node dysfunction increased gradually over time, although the incidence of right ventricular dysfunction increased rapidly after 10 years of follow-up. Late deaths, arrhythmias, and right ventricular dysfunction were significantly more frequent in the complex group. Right ventricular dysfunction and active arrhythmias were risk factors for late death. CONCLUSION Long-term follow-up after the Senning operation shows increasing incidence of sinus node dysfunction and right ventricular dysfunction over time. Deteriorating right ventricular function is a major concern. Its early recognition and initiation of appropriate management to preserve cardiac function is an important follow-up goal.

Junctional ectopic tachycardia after surgery for congenital heart disease: incidence, risk factors and outcome

OBJECTIVES Junctional ectopic tachycardia (JET) is a serious, haemodynamically compromising tachyarrhythmia associated with paediatric cardiac surgery, with a reported mortality up to 14%. The incidence, risk factors and outcome of this tachyarrhythmia were evaluated in this population-based, case-control patient cohort. METHODS A total of 1001 children, who underwent open-heart surgery during a 5-year period, were retrospectively analysed. The patients with haemodynamically significant tachycardia were identified, and their postoperative electrocardiograms were analysed. Three controls matched with the same type of surgery were selected for each patient with JET. RESULTS JET was diagnosed in 51 patients (5.0%). These patients had longer cardiopulmonary bypass time (138 vs 119 min, p=0.002), higher body temperature (38.0 vs 37.4 °C, p=0.013) and higher level of postoperative troponin-T (3.7 vs 2.1 μg l(-1), p<0.001) compared with controls. They also needed longer ventilatory support (3 vs 2 days, p=0.004) and intensive care stay (7 vs 5 days, p<0.001) as well as use of noradrenaline (23/51 vs 35/130, p=0.019). Ventricular septal defect (VSD) closure was part of the surgery in 33/51 (64.7%) of these patients. The mortality was 8% in the JET group and 5% in the controls (p=0.066). In the logistic regression model, JET was not an independent risk factor for death (p=0.557). CONCLUSIONS The incidence of JET was 5.0% in this large paediatric open-heart surgery patient group. Compared with controls, these patients had longer cardiopulmonary bypass time and higher level of troponin-T, possibly reflecting the extent of surgical trauma. However, the tachycardia was not an independent risk factor for death.

Hypertension, cardiac state, and the role of volume overload during peritoneal dialysis

Abstract The cardiac state and the prevalence of high blood pressure (BP) were analyzed in 21 pediatric patients (mean age 5.3±5.3 years) on chronic peritoneal dialysis (CPD), the aim being to specify the impact of hypervolemia in the etiology of hypertension. C- and N-terminal atrial natriuretic peptide (ANP-C, ANP-N) were measured as possible additional markers of hypervolemia. Baseline investigations were carried out 0.2 years after initiation of PD, and repeated after 0.9±0.2 years. Fifty-two percent of the patients had high BP, and in 40% the nocturnal BP decline was decreased. Left ventricular hypertrophy was present in 45%, but the systolic and diastolic functions of the heart were not impaired. Left ventricular mass correlated significantly with the severity of hypertension and with ANP-N (r=0.79, P<0.01 and r=0.66, P<0.01, Spearman rank correlation). Significant correlations were also found between the severity of hypertension and ANP-N and ANP-C (r=0.82, P<0.01 and r=0.66, P<0.01, Spearman rank correlation). High BP and cardiac impairment were more frequent in the younger and nephrectomized patients in whom volume overload seemed to be the most-important etiological factor. Our results suggest further that an ANP-N over 3.0 nmol/l combined with hypertension is strongly indicative of volume overload in patients on PD.

Intravenous epinephrine infusion test in diagnosis of catecholaminergic polymorphic ventricular tachycardia.

Epinephrine Infusion in Diagnosis of CPVT. Introduction: A test involving intravenous infusion of epinephrine has been proposed as a method alternative to exercise stress test in diagnosis of catecholaminergic polymorphic ventricular tachycardia (CPVT). We aimed at estimating the predictive value of intravenous epinephrine administration in CPVT patients with frequent exercise‐induced ventricular ectopy.

Excellent functional result in children after correction of anomalous origin of left coronary artery from the pulmonary artery – a population-based complete follow-up study☆

Surgical strategy to construct a two-coronary system for a patient with anomalous origin of left coronary artery from pulmonary artery (ALCAPA) has evolved with time. Limited long-term follow-up data are available on these children. We report population-based follow-up in children operated on for ALCAPA. In total, 29 patients underwent aortic reimplantation of ALCAPA between 1979 and 2006. Twenty (69%) children were repaired with direct aortic implantation, five (17%) with a modified tubular extension technique, and four (14%) patients with an intrapulmonary baffling technique. Early postoperative mortality (<30 days) was 17%. No late mortality (>30 days) was detected. The median length of follow-up was 11 years (range 10 months-27 years). Global left ventricular function by echocardiography (M-mode) was within normal limits (>30%) in all patients one year after operation. Functionally, 80% of patients were classified in NYHA class I, 20% in NYHA II, and 0% in NYHA classes III/IV at the time of the last examination. Excellent results with good long-term outcome can be achieved in infants with ALCAPA using reimplantation techniques. Normalization of cardiac function is expected within the first year in all operative survivors with a patent coronary system.

Amrinone Versus Dopamine-Nitroglycerin After Reconstructive Surgery for Complete Atrioventricular Septal Defect

OBJECTIVES To compare the effectiveness and safety of amrinone and a combination of dopamine and nitroglycerin in infants after reconstructive surgery for congenital heart disease. DESIGN A prospective, randomized, double-blind study. SETTING Pediatric intensive care unit in a university hospital. PARTICIPANTS Thirty-two infants with complete atrioventricular septal defect. INTERVENTIONS Amrinone loading dose, 2 mg/kg, followed by a maintenance infusion, 7.5 micrograms/kg/min, was given to 17 infants before separation from cardiopulmonary bypass. The remaining 15 patients received a combination of dopamine, 5 micrograms/kg/min, and nitroglycerin, 1 microgram/kg/min. MEASUREMENTS AND MAIN RESULTS The circulatory state of the patients was evaluated from 4 to 18 hours after cardiopulmonary bypass. The systemic blood flow index, calculated using the Fick principle, was higher in the amrinone group (2.5 +/- 0.7 L/min/m2) compared with the dopamine-nitroglycerin group (2.0 +/- 0.6 L/min/m2, mean +/- SD). The pulmonary blood flow index in the amrinone group was higher (2.9 +/- 0.6 L/min/m2) than in the dopamine-nitroglycerin group (2.2 +/- 0.6 L/min/m2); no significant difference was noted in the mean pulmonary artery pressure. The oxygen extraction ratio was higher in the dopamine-nitroglycerin group (0.41 +/- 0.07) compared with the amrinone group (0.34 +/- 0.08). Despite lower platelet counts in the amrinone group, no hemorrhagic complications were seen in any patient. CONCLUSIONS With this dosage regimen, amrinone provides a higher cardiac output, more favorable oxygen dynamics, and lower pulmonary vascular resistance than dopamine and nitroglycerin.

Amrinone versus dopamine and nitroglycerin in neonates after arterial switch operation for transposition of the great arteries.

OBJECTIVE To compare the efficacy and safety of amrinone and a combination of dopamine and nitroglycerin in neonates after reconstructive surgery for transposition of the great arteries. DESIGN A prospective, randomized, double-blind study. SETTING Pediatric intensive care unit in a university hospital. PARTICIPANTS Thirty-five neonates with transposition of the great arteries. INTERVENTIONS A loading dose of amrinone, 2 mg/kg, followed by a maintenance infusion of 7.5 microg/kg/min, were administered to 16 neonates before separation from cardiopulmonary bypass. The remaining 19 patients were administered a combination of dopamine, 5 microg/kg/min, and nitroglycerin, 1 microg/kg/min. An open-label epinephrine infusion was administered in both groups as required. MEASUREMENTS AND MAIN RESULTS The circulatory state of the patients was evaluated from 4 to 18 hours after cardiopulmonary bypass. The systemic blood flow index, calculated using the Fick principle, was higher in the amrinone group (1.7+/-0.5 L/min/m2 [mean +/- SD]) compared with the dopamine-nitroglycerin group (1.4+/-0.4 L/min/m2; p < 0.04). The systemic vascular resistance in the amrinone group was lower (26+/-8 Wood units x m2) than in the dopamine-nitroglycerin group (35+/-12 Wood units x m2; p < 0.02). The oxygen extraction ratio was higher in the dopamine-nitroglycerin group (0.34+/-0.08) compared with the amrinone group (0.28+/-0.06; p < 0.02). Lower platelet counts were observed in the amrinone group, but no difference in hemorrhagic complications was seen between the groups. CONCLUSION With the dosage regimen used, supplemented with epinephrine, amrinone provides a higher cardiac output and more favorable oxygen dynamics than a combination of dopamine and nitroglycerin.

Follow-up of 316 Molecularly Defined Pediatric Long QT Syndrome Patients - Clinical Course, Treatments and Side Effects

Background—Inherited long-QT syndrome (LQTS) is associated with risk of sudden death. We assessed the clinical course and the fulfillment of current treatment strategies in molecularly defined pediatric LQTS type 1 and (LQT1) and type 2 (LQT2) patients. Methods and Results—Follow-up data covering a mean of 12 years were collected for 316 genotyped LQT1 and LQT2 patients aged 0 to 18 years. No arrhythmic deaths occurred during the follow-up. Finnish KCNQ1 and KCNH2 founder mutations were associated with fewer cardiac events than other KCNQ1 and KCNH2 mutations (hazard ratio [HR], 0.33; P=0.03 and HR, 0.16; P=0.01, respectively). QTc interval ≥500 ms increased the risk of cardiac events compared with QTc <470 ms (HR, 3.32; P=0.001). Treatment with &bgr;-blocker medication was associated with reduced risk of first cardiac event (HR, 0.23; P=0.001). Noncompliant LQT2 patients were more often symptomatic than compliant LQT2 patients (18% and 0%, respectively; P=0.03). Treatment with implantable cardioverter defibrillator was rare (3%) and resulted in reinterventions in 44% of cases. Conclusions—Severe cardiac events are uncommon in molecularly defined and appropriately treated pediatric LQTS mutation carriers. &bgr;-Blocker medication reduces the risk of cardiac events and is generally well tolerated in this age group of LQTS patients.

Population‐Based Single‐Center Outcome for Pediatric Catheter Ablation of Common Supraventricular Tachycardias

Catheter ablation has become the preferred treatment for common supraventricular tachycardia (SVT) in children and adolescents, but long‐term follow‐up data on pediatric patients remain limited.

Long-term outcome after treatment of pulmonary atresia with ventricular septal defect: nationwide study of 109 patients born in 1970–2007

OBJECTIVES Treatment of pulmonary atresia with ventricular septal defect (PA + VSD) has evolved during recent decades, but it still remains challenging. This study evaluated 41-year experience of outcome, survival and treatment of PA + VSD patients. METHODS Patient records and angiograms of 109 patients with PA + VSD born in Finland between 1970 and 2007, and treated at the Childrens Hospital, Helsinki University Central Hospital, were retrospectively analysed in this nationwide study. RESULTS Of the 109 patients, 66 (61%) had simple PA + VSD without major aortopulmonary collateral arteries (MAPCAs). Although we observed no difference in overall survival between those with or without MAPCAs, the patients without MAPCAs had better probability to achieve repair (64 vs 28%, P < 0.0003). Only 3 patients were treated by compassionate care. Overall survival was affected by the size of true central pulmonary arteries on the first angiogram (P = 0.001) and whether repair was achieved (P < 0.0001). After successful repair, the survival rate was 93% at 1 year, 91% from the second year, and functional capacity as assessed by New York Heart Association (NYHA) I-II remained in 85% of patients alive at the end of follow-up. Palliated patients at 1, 5, 10 and 20 years of age had Kaplan-Meier estimated survival rates of 55, 42, 34 and 20%, respectively. Patients who underwent repair attempts but were left palliated with right ventricle (RV)-pulmonary artery connection and septal fenestration had better survival than the rest of the palliated patients (P = 0.001). Further, the McGoon index improved after implementation of a systemic-pulmonary artery shunt in the overall PA + VSD population (P < 0.0001). CONCLUSIONS These findings show that achievement of repair and initial size of true central pulmonary arteries affect survival of patients with PA + VSD. Although the overall survival of patients with MAPCAs showed no difference compared with simple PA + VSD patients, they had a higher risk of remaining palliated. However, palliative surgery may have a role in treatment of PA + VSD because the size of pulmonary arteries increased after placement of systemic-pulmonary artery shunt. In addition, subtotal repair by a RV-pulmonary artery connection and septal fenestration improved survival over extracardiac palliation.