The Impact of Prenatal Diagnosis on Clinical Outcomes of Isolated Vascular Rings From a Statewide Paediatric Cardiology Tertiary Service.

BACKGROUND: Vascular rings, including right aortic arch with aberrant left subclavian artery (RAA-ALSCA), double aortic arch (DAA) and pulmonary artery sling (PAS), are congenital anomalies that may cause airway and oesophageal compression. As prenatal detection has improved, literature comparing clinical outcomes of antenatally versus postnatally diagnosed cases continues to emerge. The aim is to define a statewide tertiary paediatric institution's clinical profile and outcomes of prenatal versus postnatally diagnosed isolated vascular rings. METHOD: A retrospective single-centre review of isolated RAA-ALSCA, DAA and PAS between 1 January 1999 and 31 December 2020 was conducted. Clinical characteristics, surgical and follow-up information were collected. Antenatal and postnatally diagnosed groups were compared. RESULTS: Out of 123 cases diagnosed with isolated vascular rings, 98 (79.7%) cases had RAA-ALSCA, 21 (17.1%) with DAA, 4 (3.3%) with PAS. The antenatal detection rate was 73.6% in the past decade; 20.3% had a genetic disorder, of which 48% had 22q11.21 microdeletion. Of prenatally diagnosed cases, 31.3% developed symptoms, commonly stridor and dysphagia, at a median age of 2.0 months (IQR 0.0-3.0), compared to a median age of diagnosis for the postnatal cohort of 9 months (IQR 1.0-40.7). Postnatally diagnosed cases were more likely to present with symptoms, primarily respiratory distress, than prenatally diagnosed cases (p=0.006). Fifty-nine (59) cases (50% antenatally diagnosed) required vascular ring division; 6.8% had residual symptoms following surgery. DISCUSSION: Antenatal diagnosis has improved and leads to better parental awareness and more timely, appropriate intervention. Postnatally diagnosed patients were older, more likely to be symptomatic, underwent more investigations and were commenced on more medications for symptom management prior to diagnosis. One in five cases of isolated vascular ring anomalies carried a genetic diagnosis, which has important implications on prenatal counselling and genetic testing.

Long-Term Outcomes of Total Anomalous Pulmonary Venous Drainage Repair in Neonates and Infants.

BACKGROUND: Outcomes of operations for total anomalous pulmonary venous drainage (TAPVD) have improved. However, operations in the neonatal period and the development of postoperative pulmonary venous obstruction are associated with a high mortality rate. METHODS: A retrospective review was conducted for all neonates and infants (n = 214) who underwent operations for isolated TAPVD (1973 to 2014). RESULTS: Median age was 18 days (1 day to 1 year). There were 17 (7.9%) early deaths. Risk factors for early death were prolonged cardiopulmonary bypass time (p = 0.005) and neonatal age at the operation (p = 0.048). Early mortality was 2.5% for infants (n = 81) and 11% for neonates (n = 133; p = 0.021) during the entire study period. Hospital deaths for neonates remained unchanged during the four eras of 1973 to 1988, 1989 to 1998, 1999 to 2008, and 2009 to 2014. Survival at 10 and 20 years was 88% ± 2.2% (95% confidence interval, 82% to 91%). Reoperation for postoperative pulmonary venous obstruction was required in 22 patients (10%). Risk factors for reoperation were prolonged cardiopulmonary bypass time (p = 0.015), lower operative weight (p = 0.003), and an episode of postoperative pulmonary hypertensive crisis (p = 0.005). Freedom from reoperation at 20 years was 86% ± 3.2% (95% confidence interval, 78% to 91%). All survivors were asymptomatic at a mean of 13 ± 9 years (range, 1 month to 42 years) after the operation. CONCLUSIONS: Although isolated TAPVD repair in infants can be performed without death, the operation is associated with a high mortality rate in neonates that remained unchanged during the long study period. Survival beyond 1 year after the operation is associated with excellent long-term outcomes.

Ross procedure in children: 17-year experience at a single institution.

BACKGROUND: The Ross procedure in children carries substantial mortality and reoperation rate. Aortic root dilatation is of concern. To prevent dilatation of the neoaortic root, but permit normal growth, we began to apply an absorbable poly-(p-dioxanone)-filaments (PDS) band at the sino-tubular (ST)-junction. METHODS AND RESULTS: All children (n=100) who underwent Ross procedure during 1995-2012 were studied. Mean age at operation was 8.6±6.1 years (median 8.3 years, range 3 days to 18 years); 19 patients were younger than 1 year of age. The root replacement (n=91, Ross-Konno procedure in 29 patients), root inclusion (n=6), and subcoronary implantation (n=3) techniques were used. Operative mortality was 6% (6/100, 4 neonates, 2 infants). Age of <1-year at time of operation was a risk factor for early death (P<0.001). Mean follow-up time was 7.0±4.8 years (median 7.4 years, range 5 days to 16 years). Late mortality was 4.3% (4/94). Freedom from moderate or greater neoaortic valve insufficiency (AI) at 5 and 10 years was 89% and 83%, respectively. Freedom from neoaortic valve reoperation at 5 and 10 years was 96% and 86%, respectively. Aortic dilatation to Z-score >4 was greatest at the ST-junction (23%, 11/48) compared to the aortic annulus (17%, 11/66) and sinuses (14%, 7/50). Since 2001, a PDS band was placed around the ST-junction in 19 patients. Survivors with the PDS band had less AI (0 versus 20%, P=0.043) compared to survivors (n=35) without the PDS at 4.1±3 years. CONCLUSIONS: The Ross procedure in children can be performed with acceptable results. Children younger than 1 year of age have higher mortality, but not an increased autograft reoperation rate. Stabilization of the ST-junction may reduce AI.

Childhood cardiac function after severe maternal red cell isoimmunization.

OBJECTIVE: To estimate the long-term effects of anemia on the fetal heart by echocardiography of children who received intrauterine blood transfusions for red cell isoimmunization. METHODS: Surviving children who received intrauterine transfusions during the period from 1992 to 2003 were identified. Children matched for age and sex were chosen for the control group to create a 1:1 case-control study design. A clinical interview, physical examination, and echocardiography assessment (corrected for body surface area) were performed. RESULTS: Twenty-five children were recruited for the case group and matched to 25 healthy children for the control group. Children in the case group had received a median of four intrauterine transfusion procedures (range 1-7), with a median gestation at initial intrauterine transfusion of 28 weeks (range 22-34 weeks). Hydrops was present in 32%. Median initial hemoglobin was 76 g/L (range 25-133 g/L). Median gestation at delivery was 36 weeks (range 29-38 weeks). The median age of children in the case group was 10.1 years (range 3.6-15.8 years) and of those in the control group was 10.5 years (range 3.8-16.4 years; P=.122). There was no difference in body surface area, baseline heart rate, systolic blood pressure, or diastolic blood pressure between children in the case group and those in the control group. Echocardiography demonstrated three main differences: children in the case group had 9% less left atrial area (95% confidence interval [CI] 2-16% less; P=.02), 10% less ventricular mass (95% CI 1-19% less; P=.039), and an average 11 ms less mitral valve atrial duration (95% CI 3-19 ms less; P=.009) than did those in the control group. These results did not alter when adjusted for isoimmunization severity. CONCLUSION: Fetal anemia secondary to red cell isoimmunization is associated with a reduction in left ventricular mass and left atrial area in childhood, although resting ventricular function is maintained. We speculate this may be secondary to the prenatal effects of anemia on cardiomyocyte proliferation and differentiation. LEVEL OF EVIDENCE: III.

Occlusion of a large pulmonary arteriovenous malformation in a paediatric patient using multiple vascular plugs.

Pulmonary arteriovenous malformations (PAVMs) are caused by an abnormal vascular communication between a pulmonary artery and vein. They are usually congenital in nature and are associated with considerable morbidity and mortality if left untreated. Transcatheter techniques are the favoured option for PAVM occlusion in children, and large lesions can have multiple feeder vessels that may not be appreciated until selective angiography is performed. We describe the successful occlusion of a large PAVM in a paediatric patient using multiple Amplatzer vascular plugs.

Refractory fetal supraventricular tachycardia and obstetric cholestasis.

OBJECTIVE: Supraventricular tachycardia (SVT) is the most common form of sustained fetal tachyarrhythmia in pregnancy. The development of hydrops with SVT is associated with significant worsening of prognosis. CASE: We report a case of fetal SVT with hydrops at 28 weeks' gestation that required both transplacental and direct fetal treatment in a woman with concurrent obstetric cholestasis. Antiarrhythmic therapy and control of the maternal liver function and bile acid levels with ursodeoxycholic acid was associated with a fetal response to treatment resulting in a healthy term live birth. CONCLUSION: Fetal SVT and hydrops fetalis are associated with significant perinatal morbidity and mortality. Resistance to intensive antiarrhythmic therapies may be augmented by the myocardial effects of obstetric cholestasis and remains a challenge for management.