Cause-Specific Mortality in Patients During Long-Term Follow-Up After Atrial Switch for Transposition of the Great Arteries.

Background Little is known about the cause of death (CoD) in patients with transposition of the great arteries palliated with a Mustard or Senning procedure. The aim was to describe the CoD for patients with the Mustard and Senning procedure during short- (<10 years), mid- (10-20 years), and long-term (>20 years) follow-up after the operation. Methods and Results This is a retrospective, descriptive multicenter cohort study including all Nordic patients (Denmark, Finland, Norway, and Sweden) who underwent a Mustard or Senning procedure between 1967 and 2003.

Procedural risk factors, incidence and timing of reintervention after treatment for native coarctation of the aorta in children: a population-based study†.

Objectives: The aim of the present study was to evaluate procedural risk factors, incidence and timing of reintervention because of recurrent aortic coarctation in children.

Methods: The study cohort consisted of 304 patients with isolated coarctation: 251 underwent surgery and 53 were treated percutaneously (40 balloon angioplasty, 13 stent) at the Helsinki Children's Hospital in 2000-2012. Characteristics, intervention and reintervention data were retrospectively collected from clinical records until 2014 (median follow-up 7.

Outcomes after the Mustard, Senning and arterial switch operation for treatment of transposition of the great arteries in Finland: a nationwide 4-decade perspective.

Objectives: We analysed nationwide early and late results after the Mustard, Senning and arterial switch operation.

Methods: We included all paediatric patients (<18 years) who underwent a Senning, a Mustard or an arterial switch operation for transposition of the great arteries from 1968 to 2009 in Finland. Data were obtained retrospectively from a paediatric cardiac surgical database and population data from the Finnish national registry.

Initial shunt type at the Norwood operation impacts myocardial function in hypoplastic left heart syndrome.

Objectives: We investigated the impact of initial shunt type, a Blalock-Taussig (BT) shunt versus a right ventricle to pulmonary artery conduit (RV-PA) on myocardial function at different stages of surgical palliation in patients with hypoplastic left heart syndrome (HLHS).

Methods: A population-based cohort of 63 Finnish children with HLHS (BT n  = 23, RV-PA n  = 40) born between 2003 and 2010 were studied retrospectively by echocardiography prior to Stages 1, 2 and 3 palliation and 0.5-3 years after Stage 3.

Late Causes of Death After Pediatric Cardiac Surgery: A 60-Year Population-Based Study.

Background: Comprehensive information regarding causes of late post-operative death following pediatric congenital cardiac surgery is lacking.

Objectives: The study sought to analyze late causes of death after congenital cardiac surgery by era and defect severity.

Methods: We obtained data from a nationwide pediatric cardiac surgery database and Finnish population registry regarding patients who underwent cardiac surgery at <15 years of age at 1 of 5 universities or 1 district hospital in Finland from 1953 to 2009.

Late outcome after paediatric heart transplantation in Finland.

Objectives: We studied the long-term survival and rejection episodes of paediatric heart transplant recipients.

Methods: We included all paediatric patients (≤18 years) who underwent heart transplantation during 1991-2014 in Finland. Data were obtained retrospectively from a paediatric cardiac surgery database.

Long-term results of the Ross procedure in a population-based follow-up.

Objectives: The purpose of this study was to evaluate the long-term outcomes of the Ross procedure in a nationwide follow-up.

Methods: This retrospective study involved all children treated with the Ross procedure in Finland between 1994 and 2009. The clinical records were reviewed for demographic and anatomical characteristics, Ross operation data, surgical history and status at the latest follow-up.

Progress in late results among pediatric cardiac surgery patients: a population-based 6-decade study with 98% follow-up.

Background: Surgical treatment of congenital cardiac defects in Finland started >60 years ago. We analyzed the survival of all the pediatric cardiac surgery patients operated on before 2010.

Methods And Results: Data were obtained retrospectively from a pediatric cardiac surgery database.

Need of transannular patch in tetralogy of Fallot surgery carries a higher risk of reoperation but has no impact on late survival: results of Fallot repair in Finland.

Objectives: Our study is a population-based evaluation of the long-term results after surgical repair for tetralogy of Fallot (TOF). All patients operated on in the country since the first procedure were identified via the Finnish research database of paediatric cardiac surgery and the Finnish population register. The follow-up was 99% completed due to comprehensive coverage of the registers.

Resection of the stenotic segment with individually tailored anastomosis for symptomatic congenital tracheal stenosis in infants.

Objectives: To analyse retrospectively population-based results of congenital tracheal stenosis (CTS) repair in infants in Finland.

Methods: Data on infants who were operated on for CTS in Helsinki Children's Hospital between August 1988 and May 2013 were analysed retrospectively. Fibreoptic bronchoscopy was performed perioperatively and in follow-up of all the surviving patients.

Late gadolinium enhancement (LGE) progresses with right ventricle volume in children after repair of tetralogy of fallot.

Background: Fibrosis after myocardial damage can be determined by cardiac magnetic resonance (CMR) with late gadolinium enhancement (LGE). We studied whether ventricular LGE is visible in the ventricles of pediatric and adolescent TOF (tetralogy of Fallot) patients by measuring LGE and investigating whether fibrosis correlated with right ventricular volume, pulmonary regurgitation, N-terminal pro-brain natriuretic peptide (NT-proBNP) or the aminoterminal propeptide of type III procollagen (PIIINP). We also studied if the patient's age, post-operative follow-up time or surgical history would affect LGE.

Cardiac troponin I, cardiac troponin-specific autoantibodies and natriuretic peptides in children with hypoplastic left heart syndrome.

Objectives: To evaluate serum levels of cardiac troponin I (cTnI), autoantibodies against cardiac troponin (cTnAAbs) and natriuretic peptides during the treatment protocol in children with hypoplastic left heart syndrome (HLHS).

Methods: In a prospective study, we had 18 consecutive children with HLHS, for whom serum samples were analysed before the Norwood operation, before the bidirectional Glenn (BDG) operation, at the age of one year and before total cavo-pulmonary connection (TCPC). In addition, we performed a cross-sectional study in 22 children examined before TCPC.

Methylprednisolone in neonatal cardiac surgery: reduced inflammation without improved clinical outcome.

Background: Corticosteroids are widely used in pediatric open-heart surgery to reduce systemic inflammatory response and to mediate possible cardioprotective effects. However, the optimal dosing of corticosteroids is unknown and their administration varies considerably between different institutions.

Methods: Forty neonates undergoing open-heart surgery were randomized in a double-blind fashion equally into 2 groups.

Outcome of pediatric heart transplantation recipients treated with ventricular assist device.

This study was conducted to evaluate the long-term prognosis of pediatric HTx patients treated with VAD before transplantation. The clinical data of six patients bridged to HTx with Berlin Heart EXCOR pediatric device were analyzed retrospectively. Information about graft function, CA results, and EMB findings as well as appearance DSA was collected.

The outcome of patients with right atrial isomerism is poor.

Right-atrial isomerism (RAI) is a heterotaxy syndrome with disturbances of left-right axis development resulting in complex heart malformations and anomalies of the thoracic and abdominal organs. To study the outcome of RAI, all data from patients diagnosed with this syndrome at Helsinki University Hospital between January 1976 and December of 2010 were reviewed. The outcomes were studied for 32 patients (38 % girls).

Surgery for primary cardiac tumors in children: early and late results in a multicenter European Congenital Heart Surgeons Association study.

Background: To evaluate indications and results of surgery for primary cardiac tumors in children.

Methods And Results: Eighty-nine patients aged ≤18 years undergoing surgery for cardiac tumor between 1990 and 2005 from 16 centers were included retrospectively (M/F=41/48; median age 4.3 months, range 1 day to 18 years).

Outcome of left atrial isomerism at a single institution.

Left atrial isomerism includes a complex spectrum of cardiac and extracardiac anomalies. The records of all patients with left isomerism born during the period of 1973-2010 and treated at the Children's Hospital, Helsinki were reviewed. The short- and long-term outcomes were studied.

Prevention of postoperative pericardial adhesions in children with hypoplastic left heart syndrome.

Reoperations for congenital cardiac defects are associated with an increased surgical risk due to adhesions. We compared the capability of a polytetrafluoroethylene (PTFE) membrane, synthetic polyethyleneglycol hydrogel (PEG), and a combination of them to prevent postoperative pericardial adhesions in patients with hypoplastic left heart syndrome (HLHS). Eighteen consecutive patients with HLHS were included.

Outcome of drowned hypothermic children with cardiac arrest treated with cardiopulmonary bypass.

Background: There is a lack of data on the outcome of cardiopulmonary bypass (CPB) rewarming of hypothermic children with cardiac arrest following drowning.

Aim Of The Study: To retrospectively analyze single-center outcome of drowning victims treated with CPB.

Materials And Methods: This retrospective study included all hypothermic drowning victims admitted to the Hospital for Children and Adolescents with attempted resuscitation on CPB between 1994 and 2008 inclusive.

Regulatory T cell defect in APECED patients is associated with loss of naive FOXP3(+) precursors and impaired activated population.

The pathogenetic mechanisms of organ-specific autoimmune diseases remain obscured by the complexity of the genetic and environmental factors participating in the breakdown of tolerance. A unique opportunity to study the pathogenesis of human autoimmunity is provided by autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED), a rare inherited autoimmune disease caused by mutations in Autoimmune Regulator (AIRE) gene. Loss of AIRE function disrupts the deletion of autoreactive T cells and impairs the suppressive function of regulatory T (Treg) cells.

Morbidity after paediatric cardiac surgery assessed with usage of medicines: a population-based registry study.

Objective: To examine the overall morbidity of patients who underwent surgery for congenital cardiac defect during childhood.

Background: A congenital cardiac defect treated with surgery is seldom totally cured. The incidence of residua, sequelae, and comorbidity is quite high.

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.

During treatment protocol for univentricular heart serum levels of natriuretic peptides decrease.

Objective: In children treated for univentricular heart (UVH), prospective evaluation of serum levels of N-terminal proatriopeptide (ANPN) and N-terminal pro-brain natriuretic peptide (NT-proBNP) was performed.

Methods: Serum samples were analysed in 19 children before the first operation, before the bi-directional Glenn (BDG) operation, at age 1 year and before total cavopulmonary connection (TCPC). In addition, we performed cross-sectional measurement of peptide levels in 32 children: 22 hypoplastic left ventricle (LV), 10 hypoplastic right ventricle (RV) before; and in 12 children: nine hypoplastic LV, three hypoplastic RV, 2 (range: 0.