Video-assisted thoracoscopic versus open surgery for persistent ductus arteriosus.

BACKGROUND/PURPOSE: The aim of this study is to compare the experience with video-assisted thoracoscopic surgery (VATS) for patent ductus arteriosus (PDA) since 1995 with the results of conventional open surgery from the preceding 10 years. METHODS: The records of 60 children who underwent standard posterolateral muscle splitting thoracotomy and ligation of PDA in 1986-1995 were reviewed for the study. The data on 50 children who underwent VATS PDA ligation since 1995 were collected prospectively. RESULTS: All patients survived. Ductal bleeding requiring sutures with patches occurred once in the open surgery group. Two patients in the VATS group underwent immediate rethoracoscopy and clipping because of residual ductal flow in the postoperative echocardiography. Complications in the VATS group included 6 (12%) recurrent laryngeal nerve injuries (3 transient) and 2 chylothoraces. One patient in each group underwent open reoperation because of residual ductal flow 1 year after the initial operation. The operative time, duration of recovery room/neonatal intensive care unit care, duration of pleural drainage, and length of hospital stay were significantly shorter in the VATS group. CONCLUSIONS: VATS PDA ligation gave results equal to traditional open surgery with a shorter operative time, faster recovery, and shorter hospital stay. More complications, especially recurrent laryngeal nerve injuries, occurred in the VATS group.

Diastolic dysfunction without left ventricular hypertrophy is an early finding in children with hypertrophic cardiomyopathy-causing mutations in the beta-myosin heavy chain, alpha-tropomyosin, and myosin-binding protein C genes.

OBJECTIVES: We investigated the presence of left ventricular hypertrophy (LVH) and features of diastolic dysfunction in genotype-confirmed children from families with hypertrophic cardiomyopathy (HCM) and healthy control children. BACKGROUND: In subjects with HCM-causing mutations, LVH usually does not evolve until adolescence. Diastolic dysfunction has not been systematically evaluated in children carrying HCM-causing mutations. METHODS: All children (aged 1.5-16.7 years) from 14 HCM families with identified disease-causing mutations (the Arg719Trp mutation in the beta-myosin heavy chain gene [MYH7], the Asp175Asn mutation in the alpha-tropomyosin gene [TPM1], the Gln1061X mutation in the myosin-binding protein C gene [MYBPC3], and the IVS5-2A-->C mutation in the MYBPC3 gene) and 53 matched control children were examined with electrocardiography and 2- and 3-dimensional echocardiography (2DE and 3DE). Natriuretic peptides were measured in children from HCM families and 67 control children. RESULTS: Of 53 children from HCM families, 27 (51%) had a disease-causing mutation (G+). G+ children had slightly thicker septum on 2DE compared with the control children (P = .004), but only 3 (11%) of 27 G+ children exceeded the 95th percentile values of the body surface area-adjusted maximal LV thickness of healthy children (the major echocardiographic criterion for HCM). However, prolonged isovolumetric relaxation time, increased left atrial volume on 3DE, or increased levels of NT-proANP, all features suggestive of diastolic dysfunction, were found in 14 (52%) of 27 G+ children. CONCLUSIONS: In children with HCM-causing mutations, signs of diastolic dysfunction are found in about half of the cases, as LVH is present only in small percentage of these children.

Long-term results of pediatric cardiac surgery in Finland: education, employment, marital status, and parenthood.

OBJECTIVE: This population-based study was designed to examine the psychosocial outcome of Finnish patients who had been operated on for congenital heart disease during childhood. METHODS: A questionnaire was mailed to 3789 adult patients who had been operated on for congenital heart defects in Finland. Of these, 2896 (76%) answered the questionnaire. The mean age of patients was 33 years (range: 18-59 years), and they had had their first operation 9 to 46 years earlier. RESULTS: The patients had coped well with their defects when compared with the general Finnish population. The educational level of patients was comparable to and employment level was higher than expected (70% vs 66%, respectively). They were living in a steady relationship as often as the general population, but the number of parents among the patients was lower than that expected (47% vs 49%, respectively). The incidence of congenital heart disease among the 2697 children of the patients was 2.4%. CONCLUSION: Our results confirm that in addition to high survival rate, the long-term psychosocial outcome of patients with surgically treated congenital heart defects is good if they do not have any additional syndromes that cause mental retardation.

Normal aortic dimensions and flow in 168 children and young adults.

BACKGROUND: Knowledge of normal aortic dimensions is important while evaluating children with aortic root dilatation. OBJECTIVE: The purpose of the study was to create normal values for aortic dimensions with two-dimensional echocardiography and for aortic flow velocities with Doppler echocardiography in healthy children and young adults. DESIGN AND PATIENTS: One hundred and sixty-eight healthy children were studied by a single observer using digitized two-dimensional (2DE) and Doppler echocardiography. METHODS: The 2DE measurements were obtained at the level of aortic annulus, sinus, sinotubular junction, before the origin of innominate artery, before and after the origin of left carotid artery, after left subclavian artery and descending aorta at the level of the diaphragm. Doppler measurements were made from ascending aorta and from descending aorta. RESULTS: For the analysis the subjects were divided into five groups according to body surface area (BSA): 0.5-0.75 m2, 0.75-1.0 m2, 1.0-1.25 m2, 1.25-1.5 m2 and over 1.5 m2. Aortic dimensions normalized to BSA were greater in smaller children at all levels. All diameters correlated closely with age, BSA, height and weight (for each r>0.75, P<0.001). The best predictor of aortic dimensions was BSA with r values over 0.84 for all estimates (P<0.001). The diameters of ascending and descending aorta were similar in both genders when indexed to BSA. Flow velocities in descending aorta were greater than those measured in ascending aorta (P<0.001 for all measurements). There were significant inverse correlations with heart rate and velocity time integral in ascending and descending aorta (r=-0.32 and -0.53, P<0.001, respectively). CONCLUSIONS: The presented aortic dimensions at eight levels from the valve annulus to the descending thoracic aorta by 2DE in conjunction with Doppler measurements of ascending and descending aorta in 168 healthy subjects will serve as reference data for further studies and clinical use in patients with various cardiac abnormalities.

Long-term prospective follow-up study of cardiac function after cardiotoxic therapy for malignancy in children.

PURPOSE: To evaluate cardiac function by means of conventional and three-dimensional echocardiography (3DE) and measurement of natriuretic peptides in children and adolescents previously treated for childhood malignancy using individual follow-up data and matched control children as reference criteria. PATIENTS AND METHODS: Thirty-nine survivors of childhood malignancy were examined in 1994 and 1998. The mean time from the diagnosis was 8.6 (3.9 to 16.8) years and between cardiac evaluations was 4.1 (3.3 to 5.1) years. Patients were divided into two groups according to therapies given (group I (n = 30): no cardiac irradiation, median cumulative anthracycline dose 210 mg/m2; group II (n = 9): irradiation in the cardiac region, median cumulative anthracycline dose 180 mg/m2). RESULTS: Fractional shortening (FS) in 1994 was higher than in 1998 (32.5 +/- 4.3 vs. 30.3% +/- 3.3%, P =.009). 33% of patients in group I and 56% in group II in 1994 and 30% of patients in group I and 67% in group II in 1998 had N-terminal of the propeptide-atrial natriuretic peptide (NT-proANP) levels exceeding the 90th percentile of controls. In 1998, both groups (I and II) had lower ejection fraction (EF) measured by 3DE than their matched controls (52.9 +/- 5.2 vs. 58.8% +/- 3.1%, P <.001 and 50.0 +/- 6.6 vs. 60.8% +/- 3.2%, P =.024, respectively). Left atrial maximum volumes/body surface area were smaller in the patients than in controls. B-Type natriuretic peptide values did not differ significantly in either group. CONCLUSION: Left ventricular contractility decreases slowly even years after cardiotoxic cancer therapy in children. 3DE and NT-proANP measurements are effective methods to evaluate the cardiac function in these patients.

Ambulatory blood pressure in 12-year-old children born small for gestational age.

An association between low birth weight and subsequent elevated blood pressure has been demonstrated in a large number of studies, but the number of subjects born small for gestational age in these studies has been negligible. The inverse relationship between birth weight and blood pressure in children has been evaluated previously with an ambulatory blood pressure device, but only in children with normal birth weights. In this prospective case-control study from birth to the age of 12, we evaluated the ambulatory blood pressures in 50 children born at term but small for gestational age and in 50 full-term children born appropriate for gestational age. Children born small for gestational age had similar mean+/-SD systolic (117.5+/-8.5 mm Hg versus 115.3+/-7.4 mm Hg, P=0.221), and diastolic (69.2+/-5.3 mm Hg versus 67.3+/-4.4 mm Hg, P=0.075) 24-hour ambulatory blood pressure compared with the values of the children born appropriate for gestational age. However, 24-hour systolic blood pressure in the small-for-gestational-age children was higher (3.90 mm Hg; 95% confidence interval, 0.65 to 7.15) after adjusting for current body mass index. The difference in current body mass index was the only determinant for the difference in systolic blood pressure between the groups. Birth weight had no direct association with the blood pressure values. Impaired fetal growth may have a relationship with higher later blood pressure, but in 12-year-old children, blood pressure differences between small for gestational age and appropriate for gestational age children are much more dependent on current body size.