Quality of life 20 and 30 years after surgery in patients operated on for tetralogy of Fallot and for atrial septal defect.

Follow-up studies on patients operated on for congenital heart defects have shown good anatomical results and long-term survival. To date, there have been few studies on such patients with regard to long-term psychosocial outcome and quality of life. In this study, two cohorts of patients operated on before the age of 15 years, one for tetralogy of Fallot (TOF) and the other for atrial septal defect (ASD), were investigated 20 and 30 years after operation regarding quality of life. The combined cohort had a higher educational level than average. There was no connection between quality of life and physical health as judged from the New York Heart Association classification. The TOF group rated their quality of life higher than the ASD group, but both groups had lower figures at the 30-year than at the 20-year follow-up. Fewer patients in the TOF than in the ASD group considered that their lives were affected by the heart disease. It is concluded that the severity of the heart disease is not necessarily congruent with estimated quality of live and that mild heart defects, such as ASD, can have a considerable impact on later life quality. Surprisingly few TOF patients were affected negatively, a finding which might reflect development of a specific coping strategy in these patients during childhood.

A cohort study of childhood hypertrophic cardiomyopathy: improved survival following high-dose beta-adrenoceptor antagonist treatment.

OBJECTIVES: The study analyzed factors, including treatment, affecting disease-related death in patients with hypertrophic cardiomyopathy (HCM) presenting in childhood. BACKGROUND: Previous smaller studies suggest that mortality is higher in patients with HCM presenting in childhood compared with presentation in adulthood, but these studies have all originated from selected patient populations in tertiary referral centers, and reported no significant protection by treatment. METHODS: Retrospective comparisons of mortality were done in total cohort of patients presenting to three regional centers of pediatric cardiology. There were 66 patients (25 with Noonan's syndrome) with HCM presenting at age <19 years; mean follow-up was 12.0 years. RESULTS: Among risk factors for death were congestive heart failure (p = 0.008), large electrocardiogram voltages (Sokolow-Lyon index p = 0.0003), and degree of septal (p = 0.004) and left ventricular (p = 0.028) hypertrophy expressed as percent of 95th centile value. The only treatment that significantly reduced the risk of death on multifactorial analysis of variance was high-dose beta-adrenoceptor antagonist therapy (propranolol 5 to 23 mg/kg/day or equivalent; p = 0.0001). Nineteen out of 40 patients managed conventionally (no treatment, 0.8 to 4 mg/kg of propranolol, or verapamil) died, median survival 15.8 years, with no deaths among 26 patients on high-dose beta-blockers (p = 0.0004); survival proportions at 10 years were 0.65 (95% confidence interval 0.49-0.80) and 1.0, respectively (p = 0.0015). Survival time analysis shows better survival in the high-dose beta-blocker group compared with the "no specific therapy" group (p = 0.0009) and with the conventional-dose beta-blocker group (p = 0.002). Hazard ratio analysis suggests that high-dose beta-blocker therapy produces a 5-10-fold reduction in the risk of disease-related death. CONCLUSIONS: High-dose beta-blocker therapy improves survival in childhood HCM.

Short-term outcome of perinatal care in a Swedish county. Mortality, neonatal intensive care and overall evaluation of neuromotor function at 0-10 months of corrected age in preterm and term infants.

Improvements in obstetrical and neonatal care during the last decades have led to a marked increase in survival rate of preterm and term infants. In order to study the short- and long-term outcome in infants who survived neonatal intensive care (NIC) and were born in the county of Uppsala between January 1st 1986 and April 30th 1989, a prospective long-term follow-up study was conducted. Epidemiological data on all infants born in the county during the study period and the short-term outcome, measured as overall neuromotor function at term and at 2, 4, 6 and 10 months of corrected age in 245 infants surviving NIC and 72 healthy control infants are presented. The infants' neuromotor function was evaluated with different clinical neurological methods. In the study population of NIC infants 85.9% survived the neonatal period. The early infant mortality was high in this group 11.6% compared to that of all infants born in the county of Uppsala (0.30%). Only a minority of the infants showed abnormal neuromotor function. A comparison of the results of the overall evaluation of neuromotor function at 10 months of age with those of the examinations made at an earlier age showed poor correspondence in individual infants, especially in preterm and very preterm infants.

Natural history of congenital complete atrioventricular block.

An update of studies on the natural history of congenital complete AV block is presented. A risk for heart failure, syncope, and sudden death is present at any age including fetal life. Unfavorable prognostic signs in utero are low and decreasing ventricular rate (VR), hydrops, AV valve regurgitation, and low aortic flow velocity. Indications for pacing in infancy are congestive heart failure, ventricular rate < 55 beats/min in isolated block and < 65 beats/min with associated disease, prolonged OTc, syncope attacks, frequent ventricular ectopic beats, and alternating ventricular pacemakers. Indications for immediate pacing in childhood and adult life are syncope, presyncope, VR rates lower than median for age, periods of junctional exit block, prolongation of QTc and mitral regurgitation, and change of ventricular pacemaker. Pacing is recommended to all patients older than 15 years.

Fetal tachycardia: intrauterine and postnatal course.

Eighteen consecutive cases of fetal tachycardia referred to the department of Pediatric Cardiology, Uppsala University, were studied retrospectively. All cases were detected at a routine visit at an antenatal clinic. None of the cases had a structural heart disease. Fetal supraventricular tachycardia was found in 8 cases and atrial flutter in 10 cases. In 7 cases, hydrops and heart failure were diagnosed. Antenatal treatment with digoxin, alone or in combination with other antiarrhythmic drugs, was needed in 15 cases. In 10 cases an obvious effect of the therapy was observed. No intrauterine deaths occurred. One infant died postnatally. At birth, 4 infants were in need of neonatal intensive care when delivered. Antiarrhythmic treatment was started in 13 cases postnatally. Late relapse of tachycardia was reported in 3 children. In 1 of these cases the prenatal tachycardia had resolved spontaneously and the infant was not treated antenatally nor during the neonatal period. Although fetal tachycardia is a serious condition, antenatal treatment in combination with careful monitoring and induction of delivery in cases with deteriorating fetal condition result in a satisfactory outcome for the majority of infants. However, there is a risk of late recurrence.

Irregular breathing in young lambs and newborn infants during heat stress.

Ten healthy unanaesthetized full-term lambs, aged 4-12 days, were studied during moderate radiant heat stress, and 21 full-term newborn infants were studied during moderate convective heat stress. The rate of breathing and the breathing pattern were recorded, using strain gauges made of mercury-filled rubber tubing placed around the thorax and abdomen. In both the lambs and the infants the respiratory rate increased during heat stress. When this increase began, both the lambs and infants had short periods of very rapid breathing followed by short apnoeas. The concentrations of carbon dioxide and water in a flow-through system collecting expired air increased during the short periods of rapid breathing and then decreased again during the subsequent short apnoeic period.

Intermediate-term outcome after intracardiac repair of associated cardiac defects in patients with atrioventricular and ventriculoarterial discordance.

BACKGROUND: Limited information is available concerning long-term results, especially systemic right ventricular (RV) or tricuspid valvular function, after intracardiac repair of anomalies associated with discordant atrioventricular (AV) and ventriculoarterial (VA) connections ("congenitally corrected transposition of the great arteries"). METHODS AND RESULTS: We retrospectively reviewed the intermediate-term follow-up of 28 patients, totaling 158 patient-years (median, 60 months), after intracardiac repair involving closure of a ventricular septal defect (VSD) with or without additional surgery. Seven patients had VSD closure alone, 5 had VSD repair with pulmonary stenosis relief, and 16 had VSD closure with conduit insertion between left ventricle and main pulmonary artery. Hospital mortality was 4% (1 of 28 patients; 70% confidence limits, 0.07% to 12%) and the 1-, 5-, and 10-year actuarial survival probabilities were 89%, 83%, and 83%, respectively. Twenty-one of 24 long-term survivors were in New York Heart Association functional class I and 3 were in class II. Sixteen of 24 patients showed increasing tricuspid regurgitation (TR) of more than moderate degree, which occurred within 3 years after surgery in 7 patients. Twelve of 22 patients showed deterioration of RV pump function, mainly (9 of 12 patients) within 3 years postoperatively. The pulmonary to systemic flow ratio at the preoperative cardiac catheter study was significantly (P < .05) higher in patients who developed RV dysfunction (2.3 +/- 1.0, mean +/- SD) than in those with well-maintained RV function (1.4 +/- 0.6). CONCLUSION: Intermediate-term results of intracardiac repair for AV and VA discordance were satisfactory in terms of survival and clinical functional status; however, there is concern about systemic RV dysfunction with development of TR relatively early after the operation. Alternative surgical approaches such as anatomic correction or Fontan repair for cases unsuitable for biventricular repair may improve the long-term results, including ventricular and valvular function.

Endotracheal intubation influences respiratory water loss during heat stress in young lambs.

To study the effect of intubation on respiratory water loss (RWL) during heat stress, 10 young nonsedated lambs were exposed to radiative heat stress both when intubated and when not. RWL, oxygen consumption (VO2), and carbon dioxide production were monitored continuously by using a flow-through system with a mass spectrometer for gas analysis. When the lambs were not intubated, heat stress caused RWL to increase by 218%, whereas VO2 and body temperature remained unchanged. When the lambs were intubated, heat stress caused RWL to increase by 131% and VO2 to increase by 36%. On extubation during heat stress, RWL increased by 117 +/- 48% (standard error of the estimate) of the preextubation value and body temperature started to fall. This study shows that intubation reduces the ability of the lamb to increase RWL and heat loss during heat stress in a warm environment, possibly as an effect of exclusion of the nose and a reduction in dead space. After extubation, RWL increases markedly, a finding that might also be valid for intubated infants.

Respiratory water loss in relation to gestational age in infants on their first day after birth.

Respiratory water loss, oxygen consumption and carbon dioxide production were measured in 32 infants on their first day after birth. Gestational age was between 27 and 41 weeks. All infants were studied in incubators with 50% ambient relative humidity and an ambient temperature that allowed the infant to maintain a normal and stable body temperature. During the measurements the infants were usually asleep. Respiratory water loss was found to be highest in the most preterm infants and lower in more mature infants. Respiratory water loss per breath (mg/kg) was almost the same at all gestational ages and the higher respiratory water loss found in the most preterm as compared with the more mature infants is thus and increased with increasing gestational age. Thus, in full-term infants respiratory water loss and transepidermal water loss are of approximately equal magnitude at an ambient humidity of 50%, while respiratory water loss constitutes a smaller proportion than transepidermal water loss in very preterm infants. Respiratory water loss increases with the rate of breathing.

Isolated congenital complete atrioventricular block in adult life. A prospective study.

BACKGROUND: The prognosis of congenital complete atrioventricular block (CCHB) is usually considered favorable in adults. This belief is based on studies comprising a limited number of patients and with rather short observation times. In the present study, the natural history of the disease was investigated by a prospective follow-up through decades of adult life of patients with a large group having well-defined CCHB without structural heart disease. METHODS AND RESULTS: The diagnostic criteria of CCHB proposed by Yater were applied. Patients registered as having CCHB in 1964, supplemented by younger patients all without symptoms during their first 15 years of life, were selected. The study was limited to patients with isolated, complete, permanent block. An interview was conducted with all patients and clinical follow-up data obtained. There were finally 102 patients, 61 women and 41 men. In November 1994, the time of observation, after the age of 15 years in survivors, was between 7 and 30 years. The mean age at follow-up or at death was 38 years, median age 37 years, and range 16 to 66 years. Stokes-Adams (SA) attacks occurred in 27 patients, in 8 with a fatal outcome. The first attack was fatal in 6 of these 8 patients. Nineteen survived and a pacemaker (PM) was implanted thereafter. Another 8 patients received a PM because of repeated fainting spells, and 27 others have had a PM implanted for other reasons such as fatigue, effort dyspnea, dizziness, ectopies during exercise tests, mitral regurgitation, and a low ventricular rate (VR). VR decreased with age, with a mean rate at 15 years of 46 beats per minute (bpm), at 16 to 20 years of 43 bpm, at 21 to 30 years of 41 bpm, at 31 to 40 years of 40 bpm, and after 40 years of age of 39 bpm. SA attacks occurred in all 7 patients with prolonged QTc time. Low VR at rest or at work, presence of bundle-branch block pattern, low working capacity, and ectopies at rest and/or during effort were not statistically significant risk factors. SA attacks occurred in 6 patients without any of these signs. Mitral regurgitation developed in 16 patients and 4 died. A PM reduced the risk of death. A change to a lower degree of block occurred in 6 patients. CONCLUSIONS: Prophylactic PM treatment is recommended even for symptom-free adults with CCHB because of the high incidence of unpredictable SA attacks with considerable mortality from first attacks, a gradually decreasing VR, significant morbidity, and a high incidence of "acquired" mitral insufficiency.

Respiratory water loss and oxygen consumption in full-term infants exposed to cold air on the first day after birth.

Respiratory water loss, oxygen consumption, carbon dioxide production and skin blood flow were measured continuously in nine full-term infants on the first day after birth. After at least 18 min of measurements with the infant asleep in an incubator, with an air temperature of 33 degrees C and a relative humidity of 50%, the temperature of the incubator air was lowered to less than 27.5 degrees C. This resulted in a significant decrease in skin temperature and peripheral skin blood flow, while the infant's core temperature remained unchanged. At the same time, mean respiratory water loss increased from 3.7 to 6.1 mg/kg.min, which can be explained partially by the decrease in ambient humidity that accompanied the decrease in air temperature. In addition, mean oxygen consumption increased from 5.3 to 7.9 ml/kg.min and mean carbon dioxide production increased from 3.8 to 5.9 ml/kg.min. There was no concomitant increase in motor activity. Thus, when the newborn infants were exposed to cool air, they reacted with an increase in respiratory water loss, oxygen consumption and carbon dioxide production before their core temperature was affected and without increasing their motor activity.

The temperature of inspired air influences respiratory water loss in young lambs.

The temperature of inspired air influences respiratory water loss (RWL) in young lambs. Water loss from the airways, oxygen consumption and carbon dioxide production were measured using an open flow-through system with a mass spectrometer, specially equipped with a water channel, for gas analysis. Measurements were made in 9 newborn lambs at 3 different inspired air temperatures keeping all other environmental factors stable, including the ambient air temperature. The water content of the inspired air was also kept constant. RWL was found to be 9.9 +/- 3.9 (SD) mg/kg/min when the temperature of the inspired air was 30 degrees C and its humidity 30%. At 40 degrees C this loss increased to 11.5 +/- 3.6 mg/kg/min, and at about 60 degrees C it increased further to 26.0 +/- 8.2 mg/kg/min. The oxygen consumption was 10.0 +/- 0.8 (SD) ml/kg/min at 30 degrees C and 10.4 +/- 2.0 ml/kg/min at 60 degrees C, a change which is not significant. Thus RWL is influenced by the temperature of the inspired air, with greater loss at higher temperatures.

Respiratory water loss and oxygen consumption in newborn infants during phototherapy.

Respiratory water loss was measured together with oxygen consumption (VO2) and carbon dioxide production (VCO2) in 11 full-term and eight preterm infants (mean gestational age 34 weeks, range 31-36 weeks) before and during 1 h of phototherapy. The method for determination of respiratory water loss, VO2 and VCO2 was based on an open flow-through system with a mass spectrometer for measurement of gas concentrations. All infants were studied naked in an incubator with an ambient relative humidity of 50% and with a controlled environment with respect to temperature and air velocity. The infants were calm during the measurements. Before phototherapy, in term infants respiratory water loss was 4.4 (SD 0.7) mg/kg min and VO2 5.9 (0.9) ml/kg min and in preterm infants respiratory water loss was 4.7 (0.8) mg/kg min and VO2 6.1 (0.8) ml/kg min. No significant difference was found between values obtained during or after 1 h of phototherapy and those obtained before.

The effect of a warm environment on respiratory water loss in fullterm newborn infants on their first day after birth.

Continuous measurements of respiratory water loss (RWL), oxygen consumption and carbon dioxide production were made in 21 fullterm infants on their first day after birth. The infants were first studied in incubators with a temperature of 32.5 degrees C and an ambient humidity of 50%. After an interval with stable conditions the incubator temperature was raised to 36.5 degrees C while the water vapour pressure was kept constant. When the rectal temperature had increased to 37.8 degrees C or when the infant had started to sweat, the relative humidity in the incubator was increased to 50%. At the start of the measurements mean RWL was 4.9 mg/kg min. On the average, RWL increased to a maximal value of 7.0 mg/kg min in the warm environment. Mean oxygen consumption only increased from 5.3 to 5.8 ml/kg min. This meant that when nursed in this warm environment the infants were able to increase their respiratory water loss by nearly 50% without a significant change in oxygen consumption.

Deficient herpes simplex virus-induced interferon-alpha production by blood leukocytes of preterm and term newborn infants.

The ability of peripheral blood mononuclear cells (PBMC) from newborn infants, gestational age 24-42 wk, to produce interferon-alpha (IFN-alpha) on the first day after birth was studied in vitro. Human amnion cells (WISH) coated with herpes simplex virus type I and fixed by glutaraldehyde were used as IFN-alpha inducers. Individual IFN-alpha producing cells (IPC) among PBMC were determined by an immunoplaque assay. The frequency of IPC was low in all premature (less than or equal to 36 wk) infants (median 0.3 IPC/10(4) PBMC, range 0.0-2.6), and significantly higher (median 2.0 IPC/10(4) PBMC, range 0.0-16.4) in term infants (greater than 37 wk). The frequencies were lower in both groups of infants than in adults (7.3 IPC/10(4) PBMC, range 2.0-23.7). When a conditioned medium from cultures of herpes simplex virus type I-stimulated PBMC from adults was added to the IFN induction cultures, the frequencies of IPC increased in PBMC from both preterm and term infants, and in the latter group did not differ significantly from adult levels. The median production of IFN-alpha per IPC was 1.1 U (range 0.0-2.8) in premature infants, 1.0 U (range 0.0-8.8) in term infants and 3.2 U (range 1.5-8.0) in adults. When concentrations of PBMC in the cultures [corrected] were decreased, a decline of IPC frequencies occurred. This decline was more marked and started at higher PBMC concentrations in infants than in adults, and was prevented by addition of conditioned medium from herpes simplex virus type I-stimulated cultures of PBMC from adults.(ABSTRACT TRUNCATED AT 250 WORDS)

Influence of radiant heat stress on respiratory water loss in new-born lambs.

Respiratory water loss, oxygen consumption and carbon dioxide production were measured during rest and during moderate radiant heat stress in 9 healthy unanaesthetized lambs, age 4-15 days. The method used is based on an open flow-through system with a mass spectrometer for gas analyses. Respiratory water loss was 12.7 mg/kg min when the lamb was at rest and increased to 24.7 mg/kg min during heat stress. Oxygen consumption was 13.9 ml/kg min at rest and remained unchanged during heat stress. The mean rate of breathing increased from 63 to 125 breaths/min.

Respiratory water loss in relation to activity in fullterm infants on their first day after birth.

Respiratory water loss (RWL), oxygen consumption and carbon dioxide production were measured in ten fullterm infants on their first day after birth, using an open flow-through system with a mass spectrometer, specially equipped with a water channel, for analysis of gas concentrations. Measurements were made both with the infant asleep and during different levels of motor activity. The ambient temperature was maintained at approximately 32.5 degrees C and the ambient relative humidity at 50%. RWL increased from 4.2 +/- 0.7 (SD) mg/kg min when the infant was asleep to 6.3 +/- 1.0 mg/kg min when he or she was awake but calm; with increasing activity there was a further increase in RWL. The oxygen consumption increased from 5.4 +/- 0.9 (SD) ml/kg min during sleep to 6.9 +/- 0.8 (SD) ml/kg min when awake, and also increased further with increasing activity.

Respiratory water loss in fullterm infants on their first day after birth.

Measurements of water loss from the airways, oxygen consumption and carbon dioxide production were made in 12 fullterm, newborn infants on their first day after birth, using an open flow-through system. The system includes a mass spectrometer, specially equipped with a water channel, for analysis of gas concentrations. To avoid condensation of water vapour, the tubing in the flow-through system is heated. Respiratory water loss was 4.9 +/- 1.2 (SD) mg/kg min, which meant an insensible water loss from the respiratory tract of 25.4 +/- 6.9 (SD) g per infant and 24 h at rest, at an ambient temperature of 32.2 degrees C and with an ambient humidity of 50%. Oxygen consumption was 6.0 +/- 0.8 (SD) ml/kg min. An inverse relationship was found between respiratory water loss and ambient humidity, with higher losses at a low than at a high humidity. Oxygen consumption did not change very much with ambient humidity.

Measurement of respiratory water loss in newborn lambs.

A flow-through system for measurement of respiratory water loss, providing data on oxygen consumption and carbon dioxide production, is described. The system uses a mass spectrometer, specially equipped with a water channel, for analysis of gas concentrations. To avoid condensation of water vapour, the tubing in the flow-through system is heated. The method was tested by evaporating known amounts of water, and was found to be very accurate with a correlation coefficient of 0.999. Measurements were made in 10 fullterm lambs of varying postnatal age (4-15 days) and a mean respiratory water loss (RWL) of 11.7 mg kg-1 min-1 was found at rest. The RWL decreased with increasing postnatal age, as did oxygen consumption. The RWL at rest was influenced by the ambient relative humidity, with higher values at lower humidities.