Cardiac transplantation in adults with congenital heart disease.

BACKGROUND: Due to increasing success with repair or palliation in childhood, there is a rapidly growing population of adult patients with complex congenital heart disease who may require transplantation. There remains little data on outcomes of cardiac transplantation in this group. METHODS: 38 orthotopic cardiac transplants were performed in 37 patients (18 men) > or =18 years of age with congenital heart disease (CHD) from 1988 to 2009 in our institution. Outcomes were reviewed using medical records and transplant databases. RESULTS: 15 patients (41%) had univentricular and 22 (59%) biventricular physiology. The biggest group was transposition of the great arteries following atrial switch in eight patients (22%). Six (16%) had no previous surgical intervention. Mean age at transplant was 33.5 years (range 19.1-59.9 years). 11 patients (30%) required additional surgical procedures at transplant. 16 (43%) died, 12 early and 4 late deaths (1.8, 2.4, 2.7 and 7 years). Survival was 70% at 30 days, 68% at 1 year, 58% at 5 years and 53% at 10 and 15 years. Outcome improved in later eras with reduction in 30-day mortality from 50% to 18% and increase in 5-year survival from 50% to 69%. Two patients developed post-transplant lymphoproliferative disease. None required long-term renal replacement therapy. One patient was re-transplanted for cardiac allograft vasculopathy. CONCLUSIONS: While operative mortality following cardiac transplantation for adult congenital heart disease is higher than for other diagnostic groups, long-term survival is good and comparable to patients without CHD. Disappointing early results are improved with increasing experience.

Initial results of primary device closure of large muscular ventricular septal defects in early infancy using perventricular access.

OBJECTIVES: To report our experience with the use of the Amplatzer muscular ventricular septal defect (VSD) occluder, using direct right ventricle free wall puncture for primary closure of muscular VSDs in infants. BACKGROUND: Young infants with heart failure due to large or multiple muscular VSDs often require intervention at a stage when percutaneous device closure is impractical due to delivery system limitations. There are considerable benefits to avoiding bypass in these infants. METHODS: Patients with suitable muscular VSDs, considered too small for transvenous closure, underwent periventricular muscular VSD closure under transesophageal echo (TEE) guidance. RESULTS: Eight infants underwent the procedure. The median age was 14 weeks (2-41) with median weight of 4 kg (3-6.6). Four patients had additional VSDs and one patient had previous repair of coarctation. One patient had dextrocardia and severe left ventricular impairment due to an associated cardiomyopathy. The median VSD size was 8 mm (range, 4-10 mm). A single device (6-12 mm) was deployed in each patient, the size chosen being 1-2 mm larger than the TEE measurement. Two patients had subsequent pulmonary artery banding, with absorbable bands, because of moderate residual shunting through additional VSDs. The latest echo study at a median of 7.2 weeks (0.5-66) post device implantation revealed no patients with a hemodynamically significant shunt. CONCLUSION: Perventricular muscular VSD closure can be safely and effectively undertaken in small infants with suitable muscular defects, and this strategy can avoid the need for bypass.

Weight change in the term baby in the first 2 weeks of life.

BACKGROUND: Midwives once used serial weighing to highlight lactation problems, but this is now discouraged for the fear of undermining maternal confidence. AIM: To explore weight changes in healthy newborn term babies, to gain information to aid interpretation of such measurements and to construct a centile chart for those exclusively breastfed during the first 2 weeks. METHOD: Two hundred ninety-nine mothers weighed their baby daily using the same electronic scales. In 46 cases, three or more consecutive measurements were omitted leaving 253 series to evaluate, of which 111 were exclusively breastfed. RESULTS: Breastfed babies lost a mean 6.4% of birthweight (95% CI: 5.5-7.3%) before starting to gain, and 54% took more than 8 days to regain birthweight. Artificially fed babies lost less (3.7%, 95% CI: 2.7-4.7%), but 39% had not regained their birthweight by 8 days. Once birthweight was regained, average gain was about 1% of birthweight per day in both breast- and artificially-fed babies. Measurements less than 5 days apart predicted average weight gain poorly. CONCLUSIONS: Feeding problems should be considered if weight is not increasing by 6 days, but some healthy babies take 17 days to regain their birthweight.

Patient attitudes to sternotomy and thoracotomy scars.

BACKGROUND: There are no data comparing patient attitudes to sternotomy and thoracotomy scars following surgery for congenital heart disease (CHD). METHODS: Two hundred and one patients with a scar from CHD surgery (105 sternotomy, 36 thoracotomy, and 60 both scars) had a structured interview to explore attitudes to their scar. RESULTS: Comparable proportions of each group reported that they did not like or hated their scar (23/105 [22 %] sternotomy, 9/36 [25 %] thoracotomy, 17/60 [28 %] both scars). Significantly more patients stated that they where embarrassed by and/or their choice of clothing was affected by a thoracotomy scar (20/36, 56 %) than those with a sternotomy scar (36/105, 34 %), p = 0.04. This was also seen when comparing sternotomy alone with both scars (36/105 [34 %] vs. 34/60 [57 %], p = 0.008). CONCLUSIONS: Adults who have undergone surgery for CHD are more likely to have a negative attitude to a thoracotomy than a sternotomy scar. Before a change in surgical approach is considered based on patient preferences, the acceptability and psychological impact of the different scars following surgery needs formal study.

Variability of four limb blood pressure in normal neonates.

AIMS: To assess the variation in blood pressure (BP) between limbs in normal neonates. To assess whether comparison of arm and leg BP in neonates is reproducible enough to allow the difference to raise suspicion of coarctation of the aorta. METHODS: Infants recruited from the postnatal wards and the postnatal murmur clinic underwent echocardiography and BP measurement in each limb using a Dinamap Compact T 482210. The method of BP measurement was guided by a telephone survey of 40 UK neonatal units. RESULTS: Forty healthy neonates underwent echocardiography and all had a normal aortic arch. BP was measured in 39. In three, BP in the arms was 20 mm Hg higher than in the legs. This gave a specificity of comparison of the upper and lower limb BPs of 92 (36/39) or a false positive rate of 8% (3/39). The standard deviation in BPs was 15.7 mm Hg between arms, 14.5 mm Hg between legs, and 11 mm Hg when the nearest arm and leg were compared. CONCLUSIONS: With current measurement techniques, normal neonates may have a wide variation in BP between limbs. A difference of 20 mm Hg in isolation is more likely to be due to random variability in measurement than to coarctation of the aorta. If coarctation of the aorta is suspected, it can only be excluded or confirmed by echocardiography.

Life insurance and mortgage application in adults with congenital heart disease.

OBJECTIVE: To compare the outcome of life insurance and mortgage applications of adults with congenital heart disease (CHD) with controls and at different severities of CHD. METHODS: Two hundred and ninety-nine adult CHD patients underwent a questionnaire-based interview by a trained nurse. They were asked to give an identical questionnaire to a friend to act as a control. One hundred and seventy-seven controls replied. CHD patients were classified into three categories based on severity. Comparisons were made between matched controls and between different severities of CHD. RESULTS: Similar proportions of the CHD group (59%) had applied for life insurance as matched controls (56%). Compared to controls, significantly more of the adults with CHD who had applied for life insurance have been refused (34 vs 4%, P < 0.0001) or asked to pay extra (37 vs 6%, P = 0.0002). Mortgage application rate was also similar in both groups with more of the CHD patients refused than matched controls (20 vs 3%, P = 0.0004). These differences in both life insurance and mortgage remain significant when the cases and controls are matched by employment status and NYHA functional class. There was no significant difference in life insurance and mortgage application outcome between the groups of mild, significant and complex CHD. CONCLUSIONS: Adults with CHD are significantly more likely to have difficulty obtaining life insurance or a mortgage than controls. Refusal rates appear to be independent of the severity of CHD. This suggests that the label of CHD may have a negative impact despite the lesion being minor and that the outcome of an individual application is difficult to predict based on the severity of the CHD. The increasing numbers of adults with CHD suggest that this problem is likely to increase and needs to be addressed as it can have a major impact on the patient's quality of life.

Prevalence and distribution of petechiae in well babies.

AIMS: To determine the prevalence of petechial spots in well babies. METHODS: A total of 116 babies under the age of 12 months were fully examined at child health surveillance clinics. The number and site of petechiae were recorded together with details of possible causes. RESULTS: A total of 27.6% of babies had one or more petechiae, 8.6% had two or more petechiae, and 2.6% had more than two. None of these babies subsequently developed sepsis. CONCLUSIONS: Many well infants examined in the community are likely to have petechial spots. In this setting one or two petechiae are common and their presence should not be taken as pathological without other clinical signs. Recognition of this fact may also be helpful when examining otherwise well infants with petechiae in a secondary care setting.