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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.

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.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.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.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.III.

Background Congenital left ventricular aneurysm is a poorly understood and potentially lethal entity. Methods and Results In a clinicopathologic study of 7 new cases, the major presenting features in 6 patients were congestive heart failure in 4, ventricular arrhythmias in a 32-week fetus, and multiple congenital anomalies in a fetus with trisomy 13. Accurate diagnosis was achieved in all 3 living patients by echocardiography, angiocardiography, and magnetic resonance imaging. The aneurysm was predominantly apical in 3 and involved most of the left ventricular free wall in 4. Of the 3 living patients, medical management alone sufficed in 2. The third, a newborn boy, underwent a new and successful aneurysm-exclusion left ventriculoplasty. The mitral valve was abnormal in all 4 autopsied cases, the papillary muscles being short, thin, or absent. The aneurysm was thinner and its area was larger than that of the nonaneurysmal left ventricle in all necropsied patients. Conclusions Congenital left ventricular aneurysm appears to be a developmental anomaly, an idiopathic dysplasia of left ventricular endocardium and myocardium. No evidence of a viral etiology was found. Some neonates can be managed medically, but others require urgent surgical intervention. A new surgical operation is presented, a functional left ventricular aneurysmectomy that minimizes intraoperative and postoperative blood loss and that preserves the coronary arteries. (Am Heart J 2001;141:491-9.)

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.

This study tested the hypothesis that mean aortic velocity is relatively constant in children. Eighty-eight normal children (aged 1 month to 15 years) were studied prospectively. Ascending aortic flow velocities were obtained by pulse Doppler and mean aortic velocities calculated. Mean aortic flow velocity was relatively constant for all ages at 28.4 +/- 4.8 cm/s. As Doppler is easy to perform, mean aortic flow velocity may be an alternative approach to the assessment of cardiac output.

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.

In the surgical treatment of isolated non-atherosclerotic ostial stenosis of the left main coronary artery (LMCA), direct patch angioplasty may be superior to saphenous vein or internal thoracic artery grafts. We describe a case of a surgical ostial angioplasty of the LMCA using an anterior approach, employing transection of the aorta and main pulmonary artery. A 13-year-old boy with typical precordial anginal chest pain (New York Heart Association class III) whose coronary angiography demonstrated a discrete stenosis at the origin of the LMCA underwent direct angioplasty. The ascending aorta and main pulmonary artery were transected just above the commissures. An incision was made from the upper margin of the transected aorta, through the ostium of the left coronary artery (LCA), and into the left anterior desending (LAD) branch. A saphenous vein patch was sutured into the arteriotomy to create a wide triangular enlargement of the ostium and proximal LCA. The advantage of this technique is that it provides a perfect view of the LMCA from ostial lesion to bifurcation and may have a better long-term patency rate than intact vascular grafts.

Bacterial endocarditis is an uncommon diagnosis in childhood with significant morbidity and mortality. Aortic aneurysm as a complication is well described in adults but there are few reports in the paediatric literature. Two children with bacterial endocarditis are described, whose illnesses were complicated by aortic aneurysm formation requiring surgical intervention.

Prenatal administration of indomethacin for the treatment of polyhydramnios at 27 weeks' gestation resulted in the rapid restoration of normal amniotic fluid volume. However, after 16 days therapy, fetal echocardiography revealed constriction of the fetal ductus arteriosus which did not reverse during the 17 days after the therapy was discontinued. The constriction resulted in right heart failure but no long-term effects on the infant after birth. Indomethacin is a powerful treatment for polyhydramnios but its use requires close monitoring of the fetal heart.

The cardiovascular autonomic function of 13 boys with Duchenne muscular dystrophy was examined in order to test the hypothesis that the labile sinus tachycardia of that disorder is due to autonomic dysfunction. Five non-invasive procedures evaluated the responses of heart rate and blood pressure to postural change, the Valsalva maneuver, sustained hand grip and deep breathing. No individual had sufficient evidence of autonomic dysfunction. Most tests were normal (76%), 16% were borderline, and 8% were abnormal. None had more than one abnormal test. Although most had some difficulty with the Valsalva maneuver, the study demonstrated that simple clinical non-invasive testing of autonomic function can be carried out on a young severely handicapped population.

A total of 18 male patients with Duchenne muscular dystrophy (DMD), aged 8-29 years (mean, 15.7 years), were prospectively studied to assess the cardiomyopathy associated with DMD, using clinical parameters and noninvasive cardiovascular investigations: electrocardiogram (ECG), Holter monitoring, and echocardiography. In addition, five clinical tests of cardiovascular autonomic function were used to assess the role of the autonomic nervous system in the pathogenesis of dysrhythmias. The majority of subjects were asymptomatic, but four had abnormal physical findings. All had abnormal ECG, the commonest abnormality (in 16) being tall R waves or increased R/S ratios in the right precordial leads; 14 had abnormal findings on echocardiography, including three with poor left ventricular function and five with mitral valve prolapse (MVP). Labile abrupt sinus tachycardia was present in 11, and four had high-grade ventricular ectopy. None had definite clinical evidence of autonomic dysfunction. The cardiomyopathy of DMD appears to be unrelated to disease severity. However, abnormal Q waves or Q/R ratios in ECG leads I, aVL9 and V5-V6 are significantly related to young age (p less than 0.05), and high-grade ventricular ectopy occurred significantly more frequently (p less than 0.05) in older subjects (greater than 15 years). Dysrhythmias were not related to the presence of MVP, poor left ventricular function, or autonomic dysfunction.

The effectiveness of "bedside" balloon atrial septostomy via the umbilical vein using 2-dimensional echocardiography was compared to the traditional femoral vein approach using fluoroscopy in a series of neonates with transposition of great arteries from March, 1984 to April, 1987. There were 7 neonates who had balloon septostomy performed at the "bedside" (Group I) compared to 13 who had the procedure performed in the catheterization laboratory (Group II). Group II consisted of 7 newborns who had elective femoral vein catheterization under fluoroscopy (Group IIA) and 6 who failed "bedside" umbilical vein balloon septostomy and subsequently had the femoral vein approach under fluoroscopy (Group IIB). Results showed that adequacy of balloon septostomy was not related to the approach used, with 4 of 7 in Group I and 9 of 13 in Group II with an adequate atrial tear and clinical response. The Delay time to septostomy (i.e. time elapsed from initial assessment to commencement of balloon septostomy) and Procedure time (i.e. time taken to complete the balloon septostomy) was significantly shorter for Group I (mean time = 0.7 hours and 0.26 hours respectively) compared with Group IIA (mean time = 2.6 hours and 1.8 hours) and Group IIB (mean time = 2.4 hours and 1.4 hours). Of note, there was no significant increase in Delay time between Group IIA and IIB.(ABSTRACT TRUNCATED AT 250 WORDS)

The influence of echocardiography on pre-operative cardiac catheterization was assessed in a retrospective analysis comparing two 12-month periods. Of 245 operations in 1983, 200 (82%) had pre-operative cardiac catheterization when two-dimensional echocardiography only was used in the pre-operative assessment compared to 162 of 238 (68%) operations in July 1985-June 1986 when pulsed Doppler echocardiography also was used (P less than 0.001). Significant reductions in pre-operative cardiac catheterization were present in both open (87/87 vs 89/102, P less than 0.001) and closed (113/158 vs 73/136, P less than 0.005) heart procedures. The group with the most significant reduction in pre-operative cardiac catheterization before open heart surgery were children with atrial septal defects; of the closed heart surgical groups, the greatest reductions were in patent ductus arteriosus, coarctation of the aorta and tetralogy of Fallot. Echocardiogram diagnostic errors occurred in 22/245 (9%) in 1983 compared to 9/238 (4%) in 1985/86 (P less than 0.05). Echocardiography has resulted in a significant reduction in pre-operative cardiac catheterization and has become more accurate in diagnosis.

The influence of echocardiography on the number of cardiac catheterizations(CC) performed prior to cardiac surgery was assessed in a retrospective analysis comparing two 12 month periods(Jan.-Dec. 1983 and July1985-June 1986) when pulse doppler(PD) was added and a new pediatric tertiary centre was established. Of the 245 cardiac surgical procedures in 1983, 200(82%)had pre-operative CC compared to 163 catheterizations prior to 239 surgical procedures(68%) in 1985/86. This significant reduction in CC(p

Traditionally, the estimation of cardiac output by pulsed Doppler echocardiography (PDE) involves the computation of mean aortic flow velocity(MAFV) and aortic cross-sectional area(CSA); as CSA varies with surface area(BSA), we postulated that MAFV should be relatively constant. However, data on normal values of MAFV in infants and children is lacking. Accordingly, 51 normal children aged one month to 15.1 years(m=5.6 years)were prospectively studied by PDE via the suprasternal view using a 3MHz transducer with fast Fourier transform spectral analysis of the doppler shift frequency in the ascending aorta. MAFV was calculated by averaging the planimetered area under the spectral display of three consecutive beats using the outer edge of the display as the defining border. Results showed that MAFV=27.8 + 5.2 cm/sec. This was relatively constant decreasing slightly with age and BSA;MAFV at one year=30.3 cm/sec, and 23.4 cm/sec, at 15 years. MAFV=30.381-0.039 age(R2=0.123, p= 0.05)and MAFV=32.051-5.368 BSA(R2=0.097, p=0.05). Aortic root diameter (Ao.D.) progressively increased with age and BSA with Ao.D.= 1.327+0.06 age(R2=0.806, p