Aim: To measure quality of life in children with hypoplastic left heart syndrome and their families.
Methods: A questionnaire exploring socioeconomic status, structure and function of networks and ...psychological well‐being was completed by the families of all 18 patients older than 2 years (age range 2.7–10.6). The results were compared with those of 180 healthy Swedish children matched for age and sex.
Results: There were no significant differences between the groups in any of the aspects of socioeconomic status. Study group parents had not more available time for their child (p < 0.05) and more separations/divorces (p < 0.01). The patients had lower self‐esteem (p < 0.05), more psychosomatic symptoms (p < 0.001) and lower peer acceptance (p < 0.01) than control children.
Conclusion: With regard to psychological well‐being, quality of life was significantly lower in children with hypoplastic left heart syndrome than in healthy controls.
The objective of the study was to investigate the antigen specificity and occurrence of individual autoantibodies in mothers of children diagnosed with atrioventricular (AV) block in a nation‐wide ...setting. Patients with AV block detected before 15 years of age were identified using national quality registries as well as a network of pediatric and adult cardiologists and rheumatologists at the six university hospitals in Sweden. Patients with gross heart malformations, surgically or infectiously induced blocks were excluded. Blood samples were obtained from the mothers and maternal autoantibody profile, including the occurrence of antibodies against Ro52, Ro60, La, SmB, SmD, RNP‐70k, RNP‐A, RNP‐C, CENP‐C, Scl‐70, Jo‐1, ribosomal RNP and histones was investigated in 193 mothers of children with AV block by immunoblotting and ELISA. Autoantibody reactivity was detected in 48% (93/193) of the mothers of children with AV block. In autoantibody‐positive mothers, the vast majority, 95% (88/93), had antibodies against Ro52, while 63% (59/93) had autoantibodies to Ro60 and 58% (54/93) had autoantibodies to La. In addition, 13% (12/93) of the autoantibody‐positive mothers had antibodies to other investigated antigens besides Ro52, Ro60 and La, and of these anti‐histone antibodies were most commonly represented, detected in 8% (7/93) of the mothers. In conclusion, this Swedish population‐based study confirms that maternal autoantibodies may associate with heart block in the child. Further, our data demonstrate a dominant role of Ro52 antibodies in association with AV block.
Shunt occlusion is a major cause of death in children with single ventricle. We evaluated whether one daily measurement of oxygen saturation at home could detect life-threatening shunt dysfunction.
A ...total of 28 infants were included. Parents were instructed to measure saturation once daily and if less than or equal to 70% repeat the measurement. Home monitoring was defined as positive when a patient was admitted to Queen Silvia Children's Hospital because of saturation less than or equal to 70% on repeated measurement at home. A shunt complication was defined as arterial desaturation and a narrowing of the shunt that resulted in an intervention to relieve the obstruction or in death. Parents' attitude towards the method was investigated using a questionnaire.
A shunt complication occurred out of hospital eight times in eight patients. Home monitoring was positive in five out of eight patients. In two patients, home monitoring was probably life saving; in one of them, the shunt was replaced the same day and the other had an emergency balloon dilatation of the shunt. In three out of eight patients, home monitoring was negative; one had an earlier stage II and survived, but two died suddenly at home from thrombotic shunt occlusion. On seven occasions in three patients home monitoring was positive but there was no shunt complication. The method was well accepted by the parents according to the results of the questionnaire.
Home monitoring of oxygen saturation has the potential to detect some of the life-threatening shunt obstructions between stages I and II in infants with single-ventricle physiology.
Aim: To determine what proportion of newborns with critical heart defects are discharged undiagnosed from the maternity ward. Methods: Data were retrieved retrospectively for infants born in our ...referral area from 1993 to 2001 and undergoing surgical or catheter‐based intervention before 2 mo of age because of critical heart defects. Results: 259 full‐term infants had critical heart defects. Duct‐dependent systemic circulation was present in 129, duct‐dependent pulmonary circulation in 106, and 24 infants were not duct dependent but critically ill. In 51 infants (20%) the heart defect was not suspected before discharge from the maternity ward. Such late detection occurred more often in infants with duct‐dependent systemic circulation (30%) and in children with defects that were not duct dependent (38%) than in children with duct‐dependent pulmonary circulation (4%) (p<0.001). The proportion detected after discharge from the maternity ward increased from 13% in 1993–1995 and 21% in 1996–1998 to 26% in 1999–2001 (p<0.05). Conclusion: Many infants with critical congenital heart defects are not diagnosed before discharge from the neonatal maternity ward. The proportion discharged undiagnosed has increased. Neonatal screening fails mainly in children with duct‐dependent systemic circulation.
To evaluate the feasibility of detecting duct-dependent congenital heart disease before hospital discharge by using pulse oximetry.
Case-control study.
A supra-regional referral centre for paediatric ...cardiac surgery in Sweden.
200 normal term newborns with echocardiographically normal hearts (median age 1.0 d) and 66 infants with critical congenital heart disease (CCHD; median age 3 d).
Pulse oximetry was performed in the right hand and one foot using a new-generation pulse oximeter (NGoxi) and a conventional-technology oximeter (CToxi).
With the NGoxi, normal newborns showed a median postductal saturation of 99% (range 94-100%); intra-observer variability showed a mean difference of 0% (SD 1.3%), and inter-observer variability was 0% (SD 1.5%). The CToxi recorded a significantly greater proportion of postductal values below 95% (41% vs 1%) in the normal newborns compared with NGoxi (p<0.0001). The CCHD group showed a median postductal saturation of 90% (45-99%) with the NGoxi. Analysis of distributions suggested a screening cut-off of <95%; however, this still gave 7/66 false-negative patients, all with aortic arch obstruction. Best sensitivity was obtained by adding one further criterion: saturation of <95% in both hand and foot or a difference of >+/-3% between hand and foot. These combined criteria gave a sensitivity of 98.5%, specificity of 96.0%, positive predictive value of 89.0% and negative predictive value of 99.5%.
Systematic screening for CCHD with high accuracy requires a new-generation oximeter, and comparison of saturation values from the right hand and one foot substantially improves the detection of CCHD.
Aim: To study the outcome of staged palliation for classic hypoplastic left heart syndrome. Methods: Retrospective chart review. Risk factors for mortality were analysed using Cox's proportional ...hazard regression modelling. Results: From 1993 to 2004, 55 infants underwent Norwood stage I procedure at a median age of 8 d (range 1–19 d). Hospital survival was 39/55 (71%), and there were six late deaths (before stage II). Birthweight, circulatory arrest time and cardiopulmonary bypass time were independent risk factors for stage I hospital mortality (p=0.029, p=0.001 and p=0.003, respectively). Poor right ventricular function prior to stage I was a significant predictor for interstage mortality (p=0.02). Thirty‐two patients underwent bidirectional cavopulmonary anastomosis, at a median age of 6.5 mo (range 2.0–9.5 mo), with seven late deaths. Two patients had a heart transplant after stage II. Total cavopulmonary connection (TCPC) was performed in 13 patients, at a median age of 33 mo (range 21–45 mo), without mortality. Kaplan‐Meier survival was 58%, 52% and 45% at 6, 12 and 48 mo, respectively.
Conclusion: Low birthweight, long time on circulatory arrest and cardiopulmonary bypass were risk factors for stage I mortality. Poor right ventricular function was detrimental to intermediate outcome.
Aim: To evaluate the feasibility of detecting duct‐dependent congenital heart disease before hospital discharge by using pulse oximetry. Design: Case‐control study. Setting: A supra‐regional referral ...centre for paediatric cardiac surgery in Sweden. Patients: 200 normal term newborns with echocardiographically normal hearts (median age 1.0 d) and 66 infants with critical congenital heart disease (CCHD; median age 3 d).
Methods: Pulse oximetry was performed in the right hand and one foot using a new‐generation pulse oximeter (NGoxi) and a conventional‐technology oximeter (CToxi).
Results: With the NGoxi, normal newborns showed a median postductal saturation of 99% (range 94–100%); intra‐observer variability showed a mean difference of 0% (SD 1.3%), and inter‐observer variability was 0% (SD 1.5%). The CToxi recorded a significantly greater proportion of postductal values below 95% (41% vs 1%) in the normal newborns compared with NGoxi (p < 0.0001). The CCHD group showed a median postductal saturation of 90% (45–99%) with the NGoxi. Analysis of distributions suggested a screening cut‐off of < 95%; however, this still gave 7/66 false‐negative patients, all with aortic arch obstruction. Best sensitivity was obtained by adding one further criterion: saturation of < 95% in both hand and foot or a difference of > ± 3% between hand and foot. These combined criteria gave a sensitivity of 98.5%, specificity of 96.0%, positive predictive value of 89.0% and negative predictive value of 99.5%.
Conclusion: Systematic screening for CCHD with high accuracy requires a new‐generation oximeter, and comparison of saturation values from the right hand and one foot substantially improves the detection of CCHD.
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