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. The median follow-up time was 7 (range 1-20) years. RESULTS: Thirteen infants were operated on for CTS. Resection of the stenotic segment with individually tailored anastomosis was used in 12 patients and slide tracheoplasty in 1 patient. The median age at the operation was 2.9 (range 0.2-19) months. Eight (62%) patients had associated cardiovascular defects, which were corrected during the same operation. The median length of stenosis was 35% (range 25-60%) of the total length of the trachea. The median length of time of postoperative mechanical ventilation was 10 (range 5-19) days. The median length of time of intensive care treatment was 15 (range 7-40) days. One patient died from hypoplastic lung tissue and fibrosis, and multiorgan failure. One patient required reoperation, and 3 other patients received balloon bronchodilatations postoperatively. There was no late mortality. All of the 12 survivors had a good outcome. CONCLUSION: Resection with individually tailored anastomosis with up to 55% of the stenotic segment of the trachea presented a good long-term outcome.

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). The overall survival was 22 % at a median follow-up time of 13.8 years (range 0.1-33). Extracardiac malformations, mostly asplenic, occurred in 91 % of patients. Cardiac defects included dextrocardia in 44 % and common atrioventricular valve in 100 % of patients. Ventriculoarterial discordance or double-outlet ventricle was seen in 56 and 44 % of patients, respectively. Total anomalous pulmonary venous drainage occurred in 75 % and partially anomalous venous drainage in 13 % of patients. Pulmonary outflow-tract obstruction was identified in 91 % of patients. Cardiac arrhythmias were noted in nine patients (28 %), two of them with atrioventricular block. Cardiovascular surgery was performed in 71 % patients (N = 25), seven patients were inoperable. Biventricular repair was not possible in any of the patients. During long-term follow-up there was no significant difference between the patients with total, normal, or partially anomalous pulmonary venous drainage (P = 0.5). In conclusion, RAI is one of the most severe forms of congenital cardiac diseases. The prognosis remains poor despite modern surgical techniques. When RAI is identified during pregnancy, prenatal counseling, termination, or planning for prompt cardiac treatment after the birth is necessary.

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. The review included 38 patients (50% females). The overall survival with left atrial isomerism was 63% during a median follow-up time of 16 years (range, 4-30 years). Extracardiac anomalies were noted in 14 (37%) of 38 cases. Cardiac defects included dextrocardia in 26%, partially or totally anomalous pulmonary venous return in 29%, common atrium in 50%, atrioventriculoseptal defect in 73%, single ventricle in 40%, ventriculoseptal defect without atrioventricular defect in 11%, transposition in 21%, double outlet of the right ventricle in 26%, pulmonary stenosis or atresia in 61%, and left ventricular outflow obstruction in 24% of the cases. Cardiac arrhythmias were presented in 71% and pacemaker treatment in 29% of the cases. Of the 38 patients, 33 had cardiac surgery. Simple palliative methods were used in 11 cases, single-ventricle palliation in 12 cases, and operation with a biventricular track in 10 cases. In the groups that had surgery, 3 of 11 patients, 3 of 12 patients, and 3 of 10 patients died, respectively. In this review, 14 deaths occurred, associated with extracardiac anomalies in five cases and with cardiac arrhythmia in four cases. Five postoperative deaths occurred. At this writing, all three patients who had heart transplantation are alive. Complicated heart defects associated with severe arrhythmias and extracardiac anomalies contribute to a high mortality rate with left isomerism. Cardiac transplantation was considered a good option for selected patients.

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. At the end of the Norwood I operation the cranial and the caudal half of the heart of each patient was randomized to receive a PTFE membrane, a synthetic PEG, a combination of them, or no treatment (control). Tenacity and density of adhesions, epicardial visibility, and adhesions between the heart and the sternum were analyzed semiquantitatively at a subsequent bidirectional Glenn operation. The PTFE membrane significantly decreased adhesion formation between the heart and the sternum (P<0.001). However, the PTFE membrane, with or without synthetic PEG, impaired epicardial visibility (P<0.05) when compared to synthetic PEG or controls. Synthetic PEG alone did not significantly reduce the formation of pericardial adhesions. Tenacity and density of adhesions were not affected by any of the treatment modalities. The PTFE membrane significantly decreases postoperative adhesions between the heart and the sternum, but impairs epicardial visibility. Synthetic PEG does not prevent formation of pericardial adhesions.

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. The morbidity has not been thoroughly examined. METHODS AND PATIENTS: Medication was used as an indicator of morbidity. Data from the Finnish Research Registry of Paediatric Cardiac Surgery were linked to data from the medication registry of Finland's Social Insurance Institution. This study includes 5116 patients with a mean age of 33.5 (ranged from 14.7 to 64.8) years, who had undergone surgery for congenital cardiac defect between 1953 and 1989. The use of medicines among patients in 2004 was compared with 10232 age- and sex-matched control subjects. RESULTS: The overall use of medicines was frequent; 62% of patients and 53% of controls had purchased at least one prescribed medicine (risk ratio: 1.2, 95% confidence interval: 1.1-1.2). The number of patients using cardiovascular medicines (17%) and anti-thrombotic agents (5%) was higher than that of control subjects (risk ratio: 2.2 and 8.4). In addition, the patients needed medicinal care for epilepsy (3%), asthma (7%), and psychiatric diseases (10%) more often than did controls (risk ratio: 2.2, 1.5, and 1.3, respectively). CONCLUSION: Patients operated on for congenital cardiac defect had more chronic diseases and used more medicines than did controls.

Causes of late deaths after pediatric cardiac surgery: a population-based study.

OBJECTIVES: We examined the causes and modes of late death after pediatric cardiac surgery. BACKGROUND: The late mortality of patients operated on for congenital heart defect (CHD) is comprehensively unexamined. In this study, the causes of death were examined to obtain further knowledge of the morbidity of the patients. METHODS: We studied all late deaths of patients operated on for CHD in Finland during the years 1953 to 1989. We calculated the survival of patients, identified the causes of deaths from death certificates, and examined the modes of CHD-related deaths. We compared the survival and the causes of non-CHD-related deaths to those of the general population. RESULTS: Of the 6,024 patients who survived their first operation, 592 (9%) died during the 45-year follow-up period. The progress of treatment was seen in the survival of the patients operated on in different decades. The cause of death was confirmed with postmortem examination in 474 (81%) cases. The majority of patients (397, 67%) died owing to the CHD. Furthermore, non-CHD-related mortality was twice as high (risk ratio 1.9, 95% confidence interval 1.5 to 2.4) as expected. The main mode for CHD-related death was heart failure (40%). Other modes included perioperative (26%), sudden (22%), and cardiovascular (12%) deaths. The number of deaths caused by neurological and respiratory diseases was higher and the number of accidental deaths was lower than expected. CONCLUSIONS: The survival of patients was lower than that of the general population (relative 45-year survival 89%). Most patients died owing to CHDs, but non-CHD-related mortality was also high.

Cardiac troponin T levels for risk stratification in pediatric open heart surgery.

BACKGROUND: Cardiac troponin T has been found to be accurate predictor of complications and adverse clinical events after pediatric cardiac surgery. Contrary to adult cardiac surgery, the relationship of troponin T to patient survival after pediatric heart surgery has not been previously studied. The purpose of this study was to determine whether troponin T could predict death after pediatric open cardiac surgery. METHODS: This was a retrospective cohort study in which data from 1001 consecutive children having cardiac surgery during a 5-year period were studied. Perioperative variables that could influence death at 30 postoperative days were evaluated. RESULTS: Multivariate analysis, using a forward stepwise logistic regression, showed that troponin T measured on the first postoperative day was a strong independent predictor of death at 30 days. Level of troponin T greater than 5.9 microg/L on the first postoperative day predicted death (odds ratio, 10.7; 95% confidence interval: 5.2 to 22.1) as did admission lactate level greater than 5.2 mmol/L (odds ratio, 22.2; 95% confidence interval: 9.7 to 50.8) No other variable, including postoperative creatine kinase-MB mass concentration, age, diagnosis, surgical procedure, presence of cyanosis, chromosomal anomaly or ventriculotomy, duration of cardiopulmonary bypass, or aortic cross-clamp, had any independent effect on 30-day survival. CONCLUSIONS: Cardiac troponin T level on the first postoperative day is a powerful independent risk marker of death in pediatric cardiac surgery.

Late results and quality of life after pediatric cardiac surgery in Finland: a population-based study of 6,461 patients with follow-up extending up to 45 years.

This population-based study characterizes the history and progress of pediatric cardiac surgery in Finland. For the first time in the literature, all defects and procedures are included in an outcome study, reflecting the true effectiveness of operative treatment. All the patients operated on since the beginning of pediatric cardiac surgery in Finland in 1953 and through the end of 1989 were included in this population-based, long-term outcome study. The survival was defined from the Finnish Population Registry Center. The survival rates were compared with those of an age- and sex-matched general population. During the study period, 6,461 patients underwent surgery; 96% of them were traced. A questionnaire was used to study the quality of life. The 45-year survival of patients was 15% less than that of the general population. The survival and the number of necessary operations varied widely with the defect. The patients coped well with their defects when compared with the general population. The education level was similar, and the employment level was higher than expected. Patients were living in a steady relationship as often as the general population, but parenthood was less frequent than expected. The incidence of congenital heart disease among the children of the patients was 2.4%. The overall survival of children operated on for heart defects is fairly good. The patients, especially those with simpler defects, do not have increased risk of death years after successful operation. The majority of patients are feeling well, and their life situation is similar to the general population.