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Norwood surgery has been available in Sweden since 1993. In this national cohort study, we analysed transplantation-free survival after Norwood surgery for hypoplastic left heart syndrome with aortic atresia.
Patients were identified from the complete national cohort of live-born with hypoplastic left heart syndrome/aortic atresia 1993–2010. Analysis of survival after surgery was performed using Cox proportional hazards models for the total cohort and for birth period and gender separately. Thirty-day mortality and inter-stage mortality were analysed. Patients were followed until September 2016.
The 1993–2010 cohort consisted of 208 live-born infants. Norwood surgery was performed in 121/208 (58%). The overall transplantation-free survival was 61/121 (50%). The survival was higher in the late period (10-year survival 63%) than in the early period (10-year survival 40%) (p = 0.010) and lower for female (10-year survival 34%) than for male patients (10-year survival 59%) (p = 0.002). Inter-stage mortality between stages I and II decreased from 23 to 8% (p = 0.008). For male patients, low birthweight in relation to gestational age was a factor associated with poor outcome.
The survival after Norwood surgery for hypoplastic left heart syndrome/aortic atresia improved by era of surgery, mainly explained by improved survival between stages I and II. Female gender was a significant risk factor for death or transplantation. For male patients, there was an increased risk of death when birthweight was lower than expected in relation to gestational age.
A diagnosis of congenital heart disease (CHD) in offspring triggers psychological distress in parents. Results of previous studies have been inconsistent regarding the psychological impact of a prenatal versus a postnatal diagnosis. The aim of this study was to evaluate the influence of the time of diagnosis on levels of parental distress.
Pregnant women and their partners with a fetus diagnosed with complex CHD, parents of children with postnatally diagnosed CHD, and pregnant women and their partners with uncomplicated pregnancies were invited to participate. Data were collected during pregnancy and 2–6 months after delivery using the Hospital Anxiety and Depression Scale, sense of coherence, life satisfaction, and Dyadic Adjustment Scale.
During pregnancy, the prenatal group scored lower sense of coherence compared to controls (p=0.044). Postnatally the prenatal group scored lower on sense of coherence compared to the postnatal group and controls (p=0.001; p=0.001). Postnatally, the prenatal and postnatal groups had higher levels of anxiety compared to controls (p=0.025; p=0.0003). Life satisfaction was lower in the prenatal group compared to that in the postnatal group and in controls (p=0.000; p=0.0004).
Parents with a prenatal diagnosis of CHD in offspring report a low sense of coherence already during pregnancy which decreased further at follow-up. The same group reported a lower satisfaction with life compared to parents of a child with postnatal diagnosis of CHD and parents of a healthy child. This motivates further efforts to improve counselling and support during pregnancy and for parents after a prenatal diagnosis.
The update course in foetal cardiology held by the Fetal Working Group of the Association for European Paediatric and Congenital Cardiology in Istanbul in May 2012 included a session on foetal cardiac therapy. In the introductory overview to this symposium, we critically examine the level of evidence supporting or refuting proposed foetal cardiac therapies including transplacental treatment of foetal tachyarrhythmias, steroid treatment in foetal atrioventricular block, and foetal aortic valvuloplasty. In summary, the evidence for the efficiency and safety of currently available foetal cardiac therapies is low, with no therapy based on a randomised controlled trial. Transplacental treatment of foetal tachycardia is generally accepted as effective and safe, based on extensive and widespread clinical experience; however, there is no consensus on which drugs are the most effective in different electrophysiological situations. Randomised studies may be able to resolve this, but this is complicated because tachyarrhythmias are relatively rare conditions, the foetus is not accessible for direct treatment, and it is the healthy mother who accepts treatment she does not need on behalf of her foetus. The indications for steroid treatment in foetal atrioventricular block and for foetal valvuloplasty are even more controversial. Although randomised trials would be desirable, the practical issues of recruiting sufficient sample sizes and controlling for variation in practice across multiple sites is not to be underestimated. Multicentre registries, analysed free of bias, may be an alternative way to improve the evidence base of foetal cardiac therapy.
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.
We studied echocardiographically 17 children with depletion of carnitine due to treatment over 1–3 years with antibiotics containing pivalic acid. When the children were depleted of carnitine, the left ventriclar posterior wall was significantly thickened in diastole when compared to a reference group. Six months later, after normalisation of the concentrations of carnitine, the left ventricular posterior wall was significantly thinner and did not differ from the reference group. We conclude that drugs containing pivalic acid, when given for several months, decrease the stores of carnitine to levels where cardiac involvement occurs.
The development of pulmonary arteriovenous malformations is a well-known complication after Fontan operations, and may result in significant morbidity due to increasing arterial desaturation. We compared the use of bubble contrast echocardiography and pulmonary angiography in detecting such malformations. We also examined which anatomical and haemodynamic variables were associated with their development. Our study includes 20 patients who had undergone modified Fontan procedures, 10 with atriopulmonary and 10 with total cavopulmonary connections, in Gothenburg between 1980 and 1991. All patients underwent cardiac catheterisation and pulmonary angiography. Bubble contrast echocardiography was performed at the same time, with injection of agitated polygelin colloid solution (Haemaccel, Hoechst) into the right and left pulmonary arteries, respectively. Transoesophageal echocardiography was used to detect the appearance of bubble contrast in the pulmonary venous atrium. The aim was also to evaluate the role of hepatic venous blood. Of the 20 patients, 9 (45%) had a positive contrast echocardiography study, compared with only 2 (10%) detected by pulmonary angiography. Patients with positive contrast echocardiography had a significantly lower arterial oxygen saturation than those with negative studies, both at rest (88% vs 95%, p < 0.01) and during exercise testing (78% vs 89%, p = 0.01). Bubble contrast echocardiography is much more sensitive in detecting pulmonary arteriovenous malformations than pulmonary angiography. By injecting echo contrast into the right and left pulmonary arteries, the method can be made highly selective. Pulmonary arteriovenous malformations develop much more frequently in patients with the Fontan circulation than previously reported.