Objective-To review the outcomes of transcatheter closure of atrial septal defects using the Card... more Objective-To review the outcomes of transcatheter closure of atrial septal defects using the Cardio-Seal implant. Design-A prospective interventional study. Setting-Tertiary referral centre. Patients-The first 50 patients (median age 9.7 years) who underwent attempted percutaneous occlusion. Interventions-Procedures were done under general anaesthesia and transoesophageal guidance between December 1996 and July 1998. Main outcome measures-Success of deployment, complications, and assessment of right ventricular end diastolic diameter, septal wall motion, and occlusion status by echocardiography. Results-The median balloon stretched diameter was 14 mm. Multiple atrial septal defects were present in 11 patients (22%) and a deficient atrial rim (< 4 mm) in 19 (38%). In four patients (8%), a second device was implanted after removal of an initially malpositioned first implant. There were no significant immediate complications. All patients except one were discharged within 24 hours. At the latest follow up (mean 9.9 months) a small shunt was present in 23 patients (46%), although right ventricular end diastolic dimensions (mean (SD)) corrected for age decreased from 137 (29)% to 105 (17)% of normal, and septal motion abnormalities normalised in all but one patient. No predictors for a residual shunt were identified. Supporting arm fractures were detected in seven patients (14%) and protrusion of one arm through the defect in 16 (32%), the latter being more common in those with smaller anterosuperior rims. No untoward eVects resulted from arm fractures or protrusion. There were no complications during follow up, although five patients (10%) experienced transient headaches. Conclusions-The implantation of the Cardio-Seal device corrects the haemodynamic disturbances secondary to the right ventricular volume overload, with good early outcome.
Background and Objectives. We evaluated and compared the influence of treatment for atrial septal... more Background and Objectives. We evaluated and compared the influence of treatment for atrial septal defect (ASD), patent ductus arteriosus (PDA), and coarctation of the aorta (CoA) on serum levels of N-terminal proatriopeptide and N-terminal probrain natriuretic peptide. Correlations between peptide levels and echocardiographic measurements were calculated. Patients and Methods. Peptide levels were measured and echocardiography performed before and 6-12 months after treatment in 21 children with ASD, 25 with PDA, 15 with CoA, and 76 control children. Results. ANPN levels were higher than in controls at baseline in all patient groups, and NT-proBNP in patients with ASD and PDA. Both peptide levels were elevated 6 months after treatment and decreased thereafter. Peptide levels were higher in patients with volume than pressure overload. They correlated with echocardiographic measurements. At the 6-month follow-up, dimensions of the originally overloaded ventricle had normalized only in patients with PDA. Conclusions. After intervention, peptide levels decrease but normalization takes over 6 months. The type of correlation between peptide levels and echocardiography varies according to the loading condition. Measurement of peptide levels can be used for monitoring the course of a patient's heart disease.
The study has been supported by the Foundation for Pediatric Research and the Sigrid Juselius Fou... more The study has been supported by the Foundation for Pediatric Research and the Sigrid Juselius Foundation Background: The appearance of human leukocyte antigen (HLA) antibodies after solid organ transplantation predisposes recipients to graft dysfunction. In theory, vascular homografts, which are widely used in children with congenital heart defects, may cause allosensitization. Material/Methods: In this single-center retrospective study, the presence of pre-existing HLA antibodies in pediatric heart transplant (HTx) recipients with a vascular homograft was evaluated in a cohort of 12 patients. HLA antibodies were screened before and after HTx and positive screening results were confirmed and identified using the Luminex ® single antigen bead method. Endomyocardial biopsies (EMB) and coronary angiography studies were re-evaluated to assess the prevalence of acute rejections and coronary artery change in these patients. Results: At the time of HTx, 8 patients (67%) had HLA antibodies detected by the Luminex assay, none of which were heart donor specific (DSA). All patients had negative leukocyte crossmatch. One patient developed DSAs against homograft donor prior to HTx. After the HTx, 5 patients (42%) developed DSAs against the heart donor and 4 patients (40%) against the homograft donor. In 2 patients (17%), the antibodies were against both heart and homograft donors. The rejection rate or prevalence of coronary artery vasculopathy did not differ significantly between the homograft cohort and our historical controls. Conclusions: Our results suggest that the prevalence of DSAs against homograft donor prior to HTx is relatively rare. However, almost half of the patients developed DSAs against homograft post-HTx. The clinical importance of these antibodies warrants further studies.
Objective. The aim of the study was to evaluate the cardiopulmonary exercise tolerance in childre... more Objective. The aim of the study was to evaluate the cardiopulmonary exercise tolerance in children and adolescents after chest irradiation and anticancer chemotherapy. Methods. We studied 30 subjectively asymptomatic patients aged 8 to 25 years treated for pediatric malignancies with chest irradiation (XRT) ± chemotherapy. The median interval since XRT was 7 (range, 2 to 13) years. The median XRT dose for mediastinum and/or lungs was 2550 (range, 1000 to 5100) cGy. The median cumulative dose of anthracyclines was 250 (range, 0 to 480) mg/m2. Cardiac function and exercise tolerance were evaluated by electrocardiography, echocardiography, radionuclide cineangiography, and exercise test with gas exchange analysis. Results. The patients differed from normal controls in systolic indices of myocardial function. In echocardiography, the left ventricular contractility was abnormal in 14/30 patients. In radionuclide cineangiography, the left ventricular ejection fraction was subnormal in 6/3...
A 9-year-old boy developed progressive anthracycline-induced cardiomyopathy three months after co... more A 9-year-old boy developed progressive anthracycline-induced cardiomyopathy three months after completion of chemotherapy for osteosarcoma. Five months after completion of chemotherapy, at the age of 10 years, heart transplantation was performed. At 29 months since transplantation, the patient remains free of rejection and recurrence of osteosarcoma. (Level of Difficulty: Intermediate.)
Acta Obstetricia et Gynecologica Scandinavica, 2020
IntroductionNewborn infants with transposition of the great arteries (d‐TGA) need immediate care ... more IntroductionNewborn infants with transposition of the great arteries (d‐TGA) need immediate care for an optimal outcome. This study comprised a nationwide 11‐year population‐based cohort of d‐TGA infants, and assessed whether the implementation of a nationwide systematic fetal screening program, or other perinatal, or perioperative factors, are associated with mortality or an increased need for hospital care.Material and methodsThe national cohort consisted of all live‐born infants with simple d‐TGA (TGA ± small ventricular septal defect, n = 127) born in Finland during 2004‐2014. Data were collected from six national registries. Prenatal diagnosis and perinatal and perioperative factors associated with mortality and length of hospitalization were evaluated.ResultsPreoperative mortality was 7.9%, and the total mortality was 8.7%. The prenatal detection rate increased after introducing systematic fetal anomaly screening from 5.0% to 37.7% during the study period (P < .0001), but t...
OBJECTIVES: The aim of the present study was to evaluate procedural risk factors, incidence and t... more 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 7.9 years). Age-and sex-matched comparisons between the treatment groups were performed in 86 patients (surgery n = 43, percutaneous n = 43).
BJOG: An International Journal of Obstetrics & Gynaecology, 2019
ObjectiveTo evaluate whether a nationwide prenatal anomaly screening programme improves detection... more ObjectiveTo evaluate whether a nationwide prenatal anomaly screening programme improves detection rates of univentricular heart (UVH) and transposition of great arteries (TGA), and whether maternal risk factors for severe fetal heart disease affect prenatal detection.DesignPopulation‐based cohort study.SettingNationwide data from Finnish registries 2004–14.PopulationA total of 642 456 parturients and 3449 terminated pregnancies due to severe fetal anomaly.MethodsPrenatal detection rates were calculated in three time periods (prescreening, transition and screening phase). The effect of maternal risk factors (obesity, in vitro fertilisation, pregestational diabetes and smoking) was evaluated.Main outcome measuresChange in detection rates and impact of maternal risk factors on screening programme efficacy.ResultsIn total, 483 cases of UVH and 184 of TGA were detected. The prenatal detection rate of UVH increased from 50.4% to 82.8% and of TGA from 12.3% to 41.0% (P < 0.0001). Matern...
Abstract Objective. To evaluate treatment outcomes of pediatric valvar aortic stenosis (AS) in a ... more Abstract Objective. To evaluate treatment outcomes of pediatric valvar aortic stenosis (AS) in a nationwide follow-up. Design. Balloon aortic valvuloplasty (BAV) has been the preferred treatment for congenital AS in Finland since the year 2000. All children treated due to isolated AS during 2000–2014 were included in this retrospective study. Treatment outcomes were categorized into Optimal: residual gradient ≤35 mmHg and trivial or no aortic regurgitation (AR), Adequate: gradient ≤35 mmHg with mild AR, or Inadequate: gradient >35 mmHg and/or moderate to severe AR. Results. Sixty-one patients underwent either BAV (n = 54) or surgical valvuloplasty (n = 7) for valvar AS at a median age of 29 days (range 6 hours to 16.9 years). The proportion of patients not requiring reintervention at 1, 5, and 10 years was 61%, 50%, and 29% in neonates and 83%, 73%, and 44% in older patients, respectively (p = .02); without difference between treatment groups. Larger proportion of patients remained free from valve surgery after optimal BAV result than after adequate or inadequate result (p = .01). The reason for the first reintervention was AS in 50%, AR in 36%, and combined aortic valve disease in 16% of cases. Early mortality (before hospital discharge) was 4.9%, and associated with critical AS in neonates. There was no late mortality during the follow-up. Conclusions. Although majority of congenital AS patients require more than one intervention during childhood, an optimal BAV result improves long-term outcome by increasing the proportion of patients remaining free from valve surgery. High long-term freedom from reintervention is attainable also in the neonatal population.
Interactive cardiovascular and thoracic surgery, Jul 30, 2016
We studied the long-term survival and rejection episodes of paediatric heart transplant recipient... more We studied the long-term survival and rejection episodes of paediatric heart transplant recipients. 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. Patient status was received from the Finnish population registry. All patients underwent yearly routine postoperative endomyocardial biopsies and coronary angiographies. Between 1991 and 2014, 68 heart transplantations were performed. The early mortality (<30 days after surgery) rate was 10% and follow-up coverage was 100%. The 10- and 15-year survival rates for all patients were 68% (95% confidence internal, CI, 56-80%) and 65% (95% CI 53-78%), respectively, including early mortality. The 1-year survival rate was 100% when excluding early operative mortality. Indications for heart transplantation were cardiomyopathy in 57% and cardiac malformations in 43% of patients, with similar long-term s...
Treatment of a single-ventricle heart defect involves redirecting of systemic venous blood return... more Treatment of a single-ventricle heart defect involves redirecting of systemic venous blood returning to the heart to gradually flow directly into the pulmonary artery without the functional right side of the heart being involved. In developed countries, three out of four persons with this ailment will nowadays reach adulthood. Among these, at least one out of four presents significant associated problems and late complications. The most significant problems include heart failure, arrhythmias, cyanosis and thromboembolic complications that increase over time. In addition to these, neurological complications have an influence on the quality of life in some patients.
Of 41 pediatric patients currently alive after total body irradiation (TBI) and bone marrow trans... more Of 41 pediatric patients currently alive after total body irradiation (TBI) and bone marrow transplantation (BMT), 30 (allogeneic 20, autologous 10) participated in the study. Pre-transplant therapy included high-dose cyclophosphamide (CY) and TBI (n = 12), high-dose CY alone (n = 4), high-dose Ara C and TBI (n = 5), cisplatinum, high-dose melphalan, VP-16 and TBI (n = 9). Acute cardiotoxicity was suggested by a > 15% decrease in the QRS voltage sum of the limb leads in all patients. Late cardiotoxicity was evaluated 0.5-10 years (median 5 years) post-transplant by ECG, chest radiograph, radionuclide cineangiography (RNCA) and echocardiography (ECHO). Six patients had a persistent decrease in the QRS amplitudes. They were all asymptomatic but had abnormal systolic function at the time of the study. BMT patients differed from their controls in the mean values of both the systolic and diastolic indices of myocardial function shown by RNCA and ECHO. Treatment was associated with dec...
Interactive CardioVascular and Thoracic Surgery, 2006
Less than 10% of cardiac myxomas are familial. These familial cases are related to Carney complex... more Less than 10% of cardiac myxomas are familial. These familial cases are related to Carney complex, a multiple neoplasia and lentiginosis syndrome. Mutations in the PRKAR1alpha gene are the cause of Carney complex in most patients. We report a boy, who had PRKAR1alpha gene mutation, and atrial myxoma that was diagnosed in a routine echocardiographic study at the age of four years. Surgical excision of myxoma was performed. This case demonstrates the benefit of screening genetically the kindreds of patients with familial myxomas, and the importance of close follow-up of individuals affected with this mutation irrespective of age.
To evaluate the influence of volume overload of the left (LV) and right ventricle (RV) and pressu... more To evaluate the influence of volume overload of the left (LV) and right ventricle (RV) and pressure overload of LV and restrictive physiology on levels of N-terminal proatriopeptide (ANPN) and N-terminal pro-brain natriuretic peptide (NT-proBNP). We studied 41 children with atrial septal defect (ASD), 35 with patent ductus arteriosus (PDA), 27 with coarctation of the aorta (CoA), 25 with restrictive physiology caused by Mulibrey nanism, and 64 control children. We measured serum concentrations of natriuretic peptides and evaluated ventricular size and function with echocardiography. In patients with ASD, PDA, and Mulibrey nanism, levels of both ANPN and NT-proBNP were higher than in controls but in children with CoA, only ANPN levels were higher. ANPN levels correlated with RV size in ASD and NT-proBNP levels with LV size in PDA. In patients with restriction, NT-proBNP levels correlated negatively with LV size. Correlation between echo measurements and levels of natriuretic peptides varied according to loading condition. Measurement of natriuretic peptide levels provides a supplemental method for non-invasive haemodynamic evaluation of children&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;#39;s heart disease.
To evaluate cardiac size and function in patients with coarctation of the aorta (CoA) before and ... more To evaluate cardiac size and function in patients with coarctation of the aorta (CoA) before and after treatment. Ventricular size and function were examined by 2- and 3-dimensional echocardiography, and concentrations of natriuretic peptides measured in 15 paediatric patients before repair, and one, 6, and 12 months thereafter. Controls comprised 15 children. Before repair, mitral inflow velocities and left ventricular (LV) size and wall thickness were higher in patients. Thicknesses of interventricular septum and LV posterior wall decreased after repair but increased to initial level one year thereafter. The LV end-diastolic diameter remained larger than in controls despite successful repair. The size of right ventricle increased and levels of natriuretic peptides decreased during follow-up. Levels of natriuretic peptides correlated with the smallest diameter of CoA segment and diastolic indices of LV function. LV hypertrophy persists and LV size remains larger than in controls after successful repair even in normotensive patients with normal growth of CoA segment. This may be due to remodelling of ventricles and the aorta caused by CoA.
To evaluate the prevalence of cardiac troponin I (cTnI) and autoantibodies to cTn in children wit... more To evaluate the prevalence of cardiac troponin I (cTnI) and autoantibodies to cTn in children with congenital heart defects with volume or pressure overload fulfilling the criteria for treatment, and in healthy children. The study groups comprised 78 children with volume overload caused by an atrial septal defect or a patent ductus arteriosus, and 60 children with pressure overload caused by coarctation of the aorta or stenosis of the aortic or the pulmonary valve, and 74 healthy controls. Serum levels of natriuretic peptides, cTnI, and autoantibodies to cTn were analyzed at baseline, prior to treatment and in 64 patients 6 months after treatment. At baseline, one child with volume overload, 12 children with pressure overload, and one healthy control had positive cTnI. Further analysis of the pressure overload subgroup revealed that the children with positive cTnI were younger than those with negative cTnI, and had higher levels of natriuretic peptides. The pressure gradient at the coarctation site or stenotic valve was higher in those with positive TnI. Six months after treatment, 63 of 64 children examined were cTnI negative. The cTnI release is more frequently associated with pressure than volume overload which resolves after treatment in most children.
Objective-To review the outcomes of transcatheter closure of atrial septal defects using the Card... more Objective-To review the outcomes of transcatheter closure of atrial septal defects using the Cardio-Seal implant. Design-A prospective interventional study. Setting-Tertiary referral centre. Patients-The first 50 patients (median age 9.7 years) who underwent attempted percutaneous occlusion. Interventions-Procedures were done under general anaesthesia and transoesophageal guidance between December 1996 and July 1998. Main outcome measures-Success of deployment, complications, and assessment of right ventricular end diastolic diameter, septal wall motion, and occlusion status by echocardiography. Results-The median balloon stretched diameter was 14 mm. Multiple atrial septal defects were present in 11 patients (22%) and a deficient atrial rim (< 4 mm) in 19 (38%). In four patients (8%), a second device was implanted after removal of an initially malpositioned first implant. There were no significant immediate complications. All patients except one were discharged within 24 hours. At the latest follow up (mean 9.9 months) a small shunt was present in 23 patients (46%), although right ventricular end diastolic dimensions (mean (SD)) corrected for age decreased from 137 (29)% to 105 (17)% of normal, and septal motion abnormalities normalised in all but one patient. No predictors for a residual shunt were identified. Supporting arm fractures were detected in seven patients (14%) and protrusion of one arm through the defect in 16 (32%), the latter being more common in those with smaller anterosuperior rims. No untoward eVects resulted from arm fractures or protrusion. There were no complications during follow up, although five patients (10%) experienced transient headaches. Conclusions-The implantation of the Cardio-Seal device corrects the haemodynamic disturbances secondary to the right ventricular volume overload, with good early outcome.
Background and Objectives. We evaluated and compared the influence of treatment for atrial septal... more Background and Objectives. We evaluated and compared the influence of treatment for atrial septal defect (ASD), patent ductus arteriosus (PDA), and coarctation of the aorta (CoA) on serum levels of N-terminal proatriopeptide and N-terminal probrain natriuretic peptide. Correlations between peptide levels and echocardiographic measurements were calculated. Patients and Methods. Peptide levels were measured and echocardiography performed before and 6-12 months after treatment in 21 children with ASD, 25 with PDA, 15 with CoA, and 76 control children. Results. ANPN levels were higher than in controls at baseline in all patient groups, and NT-proBNP in patients with ASD and PDA. Both peptide levels were elevated 6 months after treatment and decreased thereafter. Peptide levels were higher in patients with volume than pressure overload. They correlated with echocardiographic measurements. At the 6-month follow-up, dimensions of the originally overloaded ventricle had normalized only in patients with PDA. Conclusions. After intervention, peptide levels decrease but normalization takes over 6 months. The type of correlation between peptide levels and echocardiography varies according to the loading condition. Measurement of peptide levels can be used for monitoring the course of a patient's heart disease.
The study has been supported by the Foundation for Pediatric Research and the Sigrid Juselius Fou... more The study has been supported by the Foundation for Pediatric Research and the Sigrid Juselius Foundation Background: The appearance of human leukocyte antigen (HLA) antibodies after solid organ transplantation predisposes recipients to graft dysfunction. In theory, vascular homografts, which are widely used in children with congenital heart defects, may cause allosensitization. Material/Methods: In this single-center retrospective study, the presence of pre-existing HLA antibodies in pediatric heart transplant (HTx) recipients with a vascular homograft was evaluated in a cohort of 12 patients. HLA antibodies were screened before and after HTx and positive screening results were confirmed and identified using the Luminex ® single antigen bead method. Endomyocardial biopsies (EMB) and coronary angiography studies were re-evaluated to assess the prevalence of acute rejections and coronary artery change in these patients. Results: At the time of HTx, 8 patients (67%) had HLA antibodies detected by the Luminex assay, none of which were heart donor specific (DSA). All patients had negative leukocyte crossmatch. One patient developed DSAs against homograft donor prior to HTx. After the HTx, 5 patients (42%) developed DSAs against the heart donor and 4 patients (40%) against the homograft donor. In 2 patients (17%), the antibodies were against both heart and homograft donors. The rejection rate or prevalence of coronary artery vasculopathy did not differ significantly between the homograft cohort and our historical controls. Conclusions: Our results suggest that the prevalence of DSAs against homograft donor prior to HTx is relatively rare. However, almost half of the patients developed DSAs against homograft post-HTx. The clinical importance of these antibodies warrants further studies.
Objective. The aim of the study was to evaluate the cardiopulmonary exercise tolerance in childre... more Objective. The aim of the study was to evaluate the cardiopulmonary exercise tolerance in children and adolescents after chest irradiation and anticancer chemotherapy. Methods. We studied 30 subjectively asymptomatic patients aged 8 to 25 years treated for pediatric malignancies with chest irradiation (XRT) ± chemotherapy. The median interval since XRT was 7 (range, 2 to 13) years. The median XRT dose for mediastinum and/or lungs was 2550 (range, 1000 to 5100) cGy. The median cumulative dose of anthracyclines was 250 (range, 0 to 480) mg/m2. Cardiac function and exercise tolerance were evaluated by electrocardiography, echocardiography, radionuclide cineangiography, and exercise test with gas exchange analysis. Results. The patients differed from normal controls in systolic indices of myocardial function. In echocardiography, the left ventricular contractility was abnormal in 14/30 patients. In radionuclide cineangiography, the left ventricular ejection fraction was subnormal in 6/3...
A 9-year-old boy developed progressive anthracycline-induced cardiomyopathy three months after co... more A 9-year-old boy developed progressive anthracycline-induced cardiomyopathy three months after completion of chemotherapy for osteosarcoma. Five months after completion of chemotherapy, at the age of 10 years, heart transplantation was performed. At 29 months since transplantation, the patient remains free of rejection and recurrence of osteosarcoma. (Level of Difficulty: Intermediate.)
Acta Obstetricia et Gynecologica Scandinavica, 2020
IntroductionNewborn infants with transposition of the great arteries (d‐TGA) need immediate care ... more IntroductionNewborn infants with transposition of the great arteries (d‐TGA) need immediate care for an optimal outcome. This study comprised a nationwide 11‐year population‐based cohort of d‐TGA infants, and assessed whether the implementation of a nationwide systematic fetal screening program, or other perinatal, or perioperative factors, are associated with mortality or an increased need for hospital care.Material and methodsThe national cohort consisted of all live‐born infants with simple d‐TGA (TGA ± small ventricular septal defect, n = 127) born in Finland during 2004‐2014. Data were collected from six national registries. Prenatal diagnosis and perinatal and perioperative factors associated with mortality and length of hospitalization were evaluated.ResultsPreoperative mortality was 7.9%, and the total mortality was 8.7%. The prenatal detection rate increased after introducing systematic fetal anomaly screening from 5.0% to 37.7% during the study period (P < .0001), but t...
OBJECTIVES: The aim of the present study was to evaluate procedural risk factors, incidence and t... more 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 7.9 years). Age-and sex-matched comparisons between the treatment groups were performed in 86 patients (surgery n = 43, percutaneous n = 43).
BJOG: An International Journal of Obstetrics & Gynaecology, 2019
ObjectiveTo evaluate whether a nationwide prenatal anomaly screening programme improves detection... more ObjectiveTo evaluate whether a nationwide prenatal anomaly screening programme improves detection rates of univentricular heart (UVH) and transposition of great arteries (TGA), and whether maternal risk factors for severe fetal heart disease affect prenatal detection.DesignPopulation‐based cohort study.SettingNationwide data from Finnish registries 2004–14.PopulationA total of 642 456 parturients and 3449 terminated pregnancies due to severe fetal anomaly.MethodsPrenatal detection rates were calculated in three time periods (prescreening, transition and screening phase). The effect of maternal risk factors (obesity, in vitro fertilisation, pregestational diabetes and smoking) was evaluated.Main outcome measuresChange in detection rates and impact of maternal risk factors on screening programme efficacy.ResultsIn total, 483 cases of UVH and 184 of TGA were detected. The prenatal detection rate of UVH increased from 50.4% to 82.8% and of TGA from 12.3% to 41.0% (P < 0.0001). Matern...
Abstract Objective. To evaluate treatment outcomes of pediatric valvar aortic stenosis (AS) in a ... more Abstract Objective. To evaluate treatment outcomes of pediatric valvar aortic stenosis (AS) in a nationwide follow-up. Design. Balloon aortic valvuloplasty (BAV) has been the preferred treatment for congenital AS in Finland since the year 2000. All children treated due to isolated AS during 2000–2014 were included in this retrospective study. Treatment outcomes were categorized into Optimal: residual gradient ≤35 mmHg and trivial or no aortic regurgitation (AR), Adequate: gradient ≤35 mmHg with mild AR, or Inadequate: gradient >35 mmHg and/or moderate to severe AR. Results. Sixty-one patients underwent either BAV (n = 54) or surgical valvuloplasty (n = 7) for valvar AS at a median age of 29 days (range 6 hours to 16.9 years). The proportion of patients not requiring reintervention at 1, 5, and 10 years was 61%, 50%, and 29% in neonates and 83%, 73%, and 44% in older patients, respectively (p = .02); without difference between treatment groups. Larger proportion of patients remained free from valve surgery after optimal BAV result than after adequate or inadequate result (p = .01). The reason for the first reintervention was AS in 50%, AR in 36%, and combined aortic valve disease in 16% of cases. Early mortality (before hospital discharge) was 4.9%, and associated with critical AS in neonates. There was no late mortality during the follow-up. Conclusions. Although majority of congenital AS patients require more than one intervention during childhood, an optimal BAV result improves long-term outcome by increasing the proportion of patients remaining free from valve surgery. High long-term freedom from reintervention is attainable also in the neonatal population.
Interactive cardiovascular and thoracic surgery, Jul 30, 2016
We studied the long-term survival and rejection episodes of paediatric heart transplant recipient... more We studied the long-term survival and rejection episodes of paediatric heart transplant recipients. 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. Patient status was received from the Finnish population registry. All patients underwent yearly routine postoperative endomyocardial biopsies and coronary angiographies. Between 1991 and 2014, 68 heart transplantations were performed. The early mortality (<30 days after surgery) rate was 10% and follow-up coverage was 100%. The 10- and 15-year survival rates for all patients were 68% (95% confidence internal, CI, 56-80%) and 65% (95% CI 53-78%), respectively, including early mortality. The 1-year survival rate was 100% when excluding early operative mortality. Indications for heart transplantation were cardiomyopathy in 57% and cardiac malformations in 43% of patients, with similar long-term s...
Treatment of a single-ventricle heart defect involves redirecting of systemic venous blood return... more Treatment of a single-ventricle heart defect involves redirecting of systemic venous blood returning to the heart to gradually flow directly into the pulmonary artery without the functional right side of the heart being involved. In developed countries, three out of four persons with this ailment will nowadays reach adulthood. Among these, at least one out of four presents significant associated problems and late complications. The most significant problems include heart failure, arrhythmias, cyanosis and thromboembolic complications that increase over time. In addition to these, neurological complications have an influence on the quality of life in some patients.
Of 41 pediatric patients currently alive after total body irradiation (TBI) and bone marrow trans... more Of 41 pediatric patients currently alive after total body irradiation (TBI) and bone marrow transplantation (BMT), 30 (allogeneic 20, autologous 10) participated in the study. Pre-transplant therapy included high-dose cyclophosphamide (CY) and TBI (n = 12), high-dose CY alone (n = 4), high-dose Ara C and TBI (n = 5), cisplatinum, high-dose melphalan, VP-16 and TBI (n = 9). Acute cardiotoxicity was suggested by a > 15% decrease in the QRS voltage sum of the limb leads in all patients. Late cardiotoxicity was evaluated 0.5-10 years (median 5 years) post-transplant by ECG, chest radiograph, radionuclide cineangiography (RNCA) and echocardiography (ECHO). Six patients had a persistent decrease in the QRS amplitudes. They were all asymptomatic but had abnormal systolic function at the time of the study. BMT patients differed from their controls in the mean values of both the systolic and diastolic indices of myocardial function shown by RNCA and ECHO. Treatment was associated with dec...
Interactive CardioVascular and Thoracic Surgery, 2006
Less than 10% of cardiac myxomas are familial. These familial cases are related to Carney complex... more Less than 10% of cardiac myxomas are familial. These familial cases are related to Carney complex, a multiple neoplasia and lentiginosis syndrome. Mutations in the PRKAR1alpha gene are the cause of Carney complex in most patients. We report a boy, who had PRKAR1alpha gene mutation, and atrial myxoma that was diagnosed in a routine echocardiographic study at the age of four years. Surgical excision of myxoma was performed. This case demonstrates the benefit of screening genetically the kindreds of patients with familial myxomas, and the importance of close follow-up of individuals affected with this mutation irrespective of age.
To evaluate the influence of volume overload of the left (LV) and right ventricle (RV) and pressu... more To evaluate the influence of volume overload of the left (LV) and right ventricle (RV) and pressure overload of LV and restrictive physiology on levels of N-terminal proatriopeptide (ANPN) and N-terminal pro-brain natriuretic peptide (NT-proBNP). We studied 41 children with atrial septal defect (ASD), 35 with patent ductus arteriosus (PDA), 27 with coarctation of the aorta (CoA), 25 with restrictive physiology caused by Mulibrey nanism, and 64 control children. We measured serum concentrations of natriuretic peptides and evaluated ventricular size and function with echocardiography. In patients with ASD, PDA, and Mulibrey nanism, levels of both ANPN and NT-proBNP were higher than in controls but in children with CoA, only ANPN levels were higher. ANPN levels correlated with RV size in ASD and NT-proBNP levels with LV size in PDA. In patients with restriction, NT-proBNP levels correlated negatively with LV size. Correlation between echo measurements and levels of natriuretic peptides varied according to loading condition. Measurement of natriuretic peptide levels provides a supplemental method for non-invasive haemodynamic evaluation of children&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;#39;s heart disease.
To evaluate cardiac size and function in patients with coarctation of the aorta (CoA) before and ... more To evaluate cardiac size and function in patients with coarctation of the aorta (CoA) before and after treatment. Ventricular size and function were examined by 2- and 3-dimensional echocardiography, and concentrations of natriuretic peptides measured in 15 paediatric patients before repair, and one, 6, and 12 months thereafter. Controls comprised 15 children. Before repair, mitral inflow velocities and left ventricular (LV) size and wall thickness were higher in patients. Thicknesses of interventricular septum and LV posterior wall decreased after repair but increased to initial level one year thereafter. The LV end-diastolic diameter remained larger than in controls despite successful repair. The size of right ventricle increased and levels of natriuretic peptides decreased during follow-up. Levels of natriuretic peptides correlated with the smallest diameter of CoA segment and diastolic indices of LV function. LV hypertrophy persists and LV size remains larger than in controls after successful repair even in normotensive patients with normal growth of CoA segment. This may be due to remodelling of ventricles and the aorta caused by CoA.
To evaluate the prevalence of cardiac troponin I (cTnI) and autoantibodies to cTn in children wit... more To evaluate the prevalence of cardiac troponin I (cTnI) and autoantibodies to cTn in children with congenital heart defects with volume or pressure overload fulfilling the criteria for treatment, and in healthy children. The study groups comprised 78 children with volume overload caused by an atrial septal defect or a patent ductus arteriosus, and 60 children with pressure overload caused by coarctation of the aorta or stenosis of the aortic or the pulmonary valve, and 74 healthy controls. Serum levels of natriuretic peptides, cTnI, and autoantibodies to cTn were analyzed at baseline, prior to treatment and in 64 patients 6 months after treatment. At baseline, one child with volume overload, 12 children with pressure overload, and one healthy control had positive cTnI. Further analysis of the pressure overload subgroup revealed that the children with positive cTnI were younger than those with negative cTnI, and had higher levels of natriuretic peptides. The pressure gradient at the coarctation site or stenotic valve was higher in those with positive TnI. Six months after treatment, 63 of 64 children examined were cTnI negative. The cTnI release is more frequently associated with pressure than volume overload which resolves after treatment in most children.
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