Anomalous systemic arterial supply to normal basal segment of the left lung.

BACKGROUND: Anomalous systemic arterial supply to normal segments of the lung is an unusual anomaly. It represents part of a spectrum of bronchovascular abnormalities which have various anatomical and clinical manifestations. METHODS: We retrospectively analysed cases from January 2007 to April 2010 from two institutions diagnosed with an anomalous systemic arterial supply to a normal lung segment. RESULTS: Three infants were found to have anomalous systemic arterial supply to normal segments of the lung. One patient was from The Children's Hospital at Westmead, Australia and two cases from Amrita Institute of Medical Sciences, Kochi, India. The mean age at diagnosis was 65 days (range 30-120 days) and mean weight was 3.05 kg (range 1.9-4.4 kg). All babies presented with tachypnoea. The diagnosis was suspected on echocardiography and confirmed by computerised tomography scan (CT scan) in one and by angiography in two cases. The preterm baby underwent ligation of the anomalous vessel by thoracotomy and other two infants had transcatheter occlusion of the collateral. There was no residual flow on echocardiography in any of the three cases and all have done well on follow up. CONCLUSION: Anomalous systemic arterial supply to normal lung segments is a very rare anomaly. A high index of suspicion is needed to expedite diagnosis. Transcatheter embolisation or surgical ligation of the collateral proved effective therapeutic approaches in young infants without a need for surgical lobectomy.

Evaluation of the extracardiac conduit modification of the Fontan operation for thrombus formation using magnetic resonance imaging.

BACKGROUND: The prevalence of thrombosis after the Fontan procedure depends upon the surgical technique used and the method of detection employed. Current investigations for thrombosis lack sensitivity and specificity or, in the paediatric population, require a general anaesthetic. We undertook a study to examine the feasibility of using magnetic resonance imaging (MRI) to detect thrombosis within the conduit, cardiac chambers and pulmonary arteries after the extracardiac conduit modification of the Fontan procedure. METHODS: Of the 50 children who had undergone this procedure at our institution between 1997 and 2002, 26 were eligible for, and 13 underwent, MRI study. The mean age was 10.2 years (range 8.2-16.8 years, median 9.5 years) and the average time from operation was 63 months (range 29-79 months, median 68 months). The mean age at Fontan operation was 4.9 years (range 2.1-10.5 years). Ten were on low dose aspirin, two were on warfarin and one was not anti-coagulated. In all cases, satisfactory imaging of the venous pathways and pulmonary arteries was obtained and there were no thrombi detected. CONCLUSIONS: We conclude that MRI is a potentially useful tool for the detection of thrombus in patients who have undergone the Fontan operation.

Outcomes following surgery for congenital heart disease in low-birthweight infants.

AIM: To describe cardiac surgery, survival and outcomes for low-birthweight (< or = 2500 g) infants undergoing surgery for congenital heart disease. METHODS: Using data from a prospectively collected population-based database of admissions to neonatal intensive care units in New South Wales and the Australian Capital Territory, we identified all low-birthweight infants undergoing cardiac surgery between 1992 and 2001. Infants with only a persistent ductus arteriosus were excluded. Two-year cardiac and neurodevelopmental outcome data were sought from hospital medical records. RESULTS: A total of 121 low-birthweight infants underwent cardiac surgery, of whom 34% had a congenital syndrome or non-cardiac birth defect. Most (81%) underwent a palliative surgical procedure in the neonatal period. There were 19 early (15.7%) and 19 late deaths giving a 2-year mortality of 31%. Factors associated with mortality included birthweight below 1500 g (P = 0.006), low weight at surgery (P = 0.028) and Apgar score at 1 min (P = 0.019). No single factor predicted 30-day mortality. By 2 years of age, 27 (33% of survivors) were known to have neurodevelopmental delay. Although 22 children are known to be developing normally, the neurodevelopmental status of 34 children was not known. CONCLUSIONS: These surgical data were comparable to previous single-institution studies. This group had a high risk of disability due to prematurity, low birthweight and associated conditions. There is a need to prospectively assess and manage neurodevelopmental outcomes in this group.

Abnormal right ventricular tissue velocities after repair of congenital heart disease--implications for late outcomes.

BACKGROUND: Although repair of Tetralogy of Fallot (TOF) and transposition of the great arteries (TGA) has facilitated survival into adulthood, many survivors have residual haemodynamic abnormalities, including exercise intolerance and late right ventricular (RV) failure. METHODS: We studied 40 asymptomatic adult subjects (31.3+/-1.5 years) after congenital heart disease (CHD) surgery during childhood, using tissue Doppler echocardiography (TDE). We compared systolic (S') and diastolic (E' for early filling) RV and LV velocities, with 40 age matched controls (29.5+/-1.0 years). RESULTS: Both RV S' and E' velocities were significantly slower in the CHD group compared to controls (6.3+/-0.4 cm/s vs. 9.3+/-0.3 cm/s; 8.5+/-0.5 cm/s vs. 10.9+/-0.4 cm/s, respectively, p<0.001 for both). By contrast, LV S' and E' velocities were similar in both groups. Interestingly, in 50% of CHD subjects where RV function was reported as 'normal', both RV S' and E' velocities were significantly slower compared with controls (6.5+/-0.6 cm/s vs. 9.3+/-0.3 cm/s, p<0.0001 and 9.4+/-0.7 cm/s vs. 10.9+/-0.4 cm/s, p<0.05 respectively). CONCLUSIONS: RV S' and E' velocities are frequently abnormal in asymptomatic survivors of TOF and TGA repair, even where RV function appears 'normal'. Hence TDE during follow up may be a sensitive means of detecting pre-clinical abnormalities in RV performance.

The medium-to-long-term outcome of Papua New Guinean children after cardiac surgery.

This study reports the medium-to-long-term outcome in Papua New Guinean (PNG) children selected to undergo cardiac surgery at the Royal Alexandra Hospital for Children in Sydney, Australia between 1978 and 1994. Follow-up ranged from 4 to 20 (median 11) years. The cohort comprised 125 children who had surgery and 31 who were initially selected in PNG for surgery but who on further investigation were found to be unsuitable. Through strenuous attempts, local health workers, the media and village and church leaders traced 122 (98%) of the operated and 29 (94%) of the non-operated children. One of the operated children and six of the non-operated children had died, giving respective survival rates among those traced of 99% and 79%. Altogether, 106 (88%) of the 121 operated and 20 (87%) of the 23 non-operated survivors were reviewed. Ninety-nine (93%) of the surgical patients were asymptomatic and all fulfilled the New York Heart Association criteria (NYHAC) class I or II. Mild pulmonary hypertension or residual defects of no haemodynamic significance were present in 47 (44%). In contrast, all 11 survivors from the 18 children originally classified as having inoperable lesions were symptomatic, all in NYHAC classes III or IV, six were on cardiac medication and four had been admitted at least once in the previous year. Ninety-two of 96 (96%) of the surgical group had a normal exercise test and 75 of 96 (78%) had normal chest X-rays. Thirty-nine of 99 had a normal electrocardiogram whilst the remainder had changes related to the underlying lesion and the surgery performed. This study shows that the PNG children who had cardiac surgery at RAHC between 1978 and 1994 had good medium-to-long-term survival.

Gregg's congenital rubella patients 60 years later.

BACKGROUND: In 1941, a Sydney ophthalmologist, Norman McAlister Gregg, correctly identified the link between congenital cataracts in infants and maternal rubella early in pregnancy. Fifty of Gregg's subjects with congenital rubella, born in 1939-1944, were reviewed in 1967 and again in 1991. We reviewed this cohort in 2000-2001, 60 years after their intrauterine infection. METHODS: The subjects underwent full clinical assessment, plus pathology tests, an ophthalmological and cardiological review (including electrocardiography and echocardiography) and HLA histocompatibility testing. RESULTS: Since they were first seen in 1967, 10 have died (cardiovascular causes [4], malignant disease [4], AIDS [1], and hepatitis C-related cirrhosis [1]). All surviving men came for review (19) and 13 women (eight women declined). Echocardiography showed mild aortic valve sclerosis in 68%. The prevalence of diabetes (22%), thyroid disorders (19%), early menopause (73%) and osteoporosis (12.5%) was increased compared with the Australian population; 41% had undetectable levels of rubella antibodies. The frequency of HLA-A1 (44%) and HLA-B8 (34%) antigens was increased, and the haplotype HLA-A1, B8, DR3, said to be highly associated with many autoimmune conditions, was present in 25%. CONCLUSIONS: This cohort of people with congenital rubella has illuminated our understanding of viral teratogenesis.