Giant Placental Chorangioma and Severe Ductal Arch Constriction: A Case Report With a Favorable Outcome.

Giant chorangiomas are uncommon yet frequently associated with adverse pregnancy outcomes. A 37-year-old female was referred due to findings of a placental mass during a second-trimester ultrasound. A fetal survey at 26 weeks revealed a 69×97×75 mm heterogenous placental tumor with two prominent feeding vessels. Her prenatal course was complicated by worsening polyhydramnios requiring amnioreduction, gestational diabetes, and transient severe ductal arch (DA) constriction. Placental pathology confirmed the diagnosis of giant chorioangioma following delivery at 36 weeks. To our knowledge, this represents the first case of DA constriction in the setting of a giant chorangioma.

Impact of initial shunt type on cardiac size and function in children with single right ventricle anomalies before the Fontan procedure: the single ventricle reconstruction extension trial.

BACKGROUND: In children with single right ventricular (RV) anomalies, changes in RV size and function may be influenced by shunt type chosen at the time of the Norwood procedure. OBJECTIVES: The study sought to identify shunt-related differences in echocardiographic findings at 14 months and ≤6 months pre-Fontan in survivors of the Norwood procedure. METHODS: We compared 2-dimensional and Doppler echocardiographic indices of RV size and function, neo-aortic and tricuspid valve annulus dimensions and function, and aortic size and patency at 14.1 ± 1.2 months and 33.6 ± 9.6 months in subjects randomized to a Norwood procedure using either the modified Blalock-Taussig shunt (MBTS) or right ventricle to pulmonary artery shunt (RVPAS). RESULTS: Acceptable echocardiograms were available at both time points in 240 subjects (114 MBTS, 126 RVPAS). At 14 months, all indices were similar between shunt groups. From the 14-month to pre-Fontan echocardiogram, the MBTS group had stable indexed RV volumes and ejection fraction, while the RVPAS group had increased RV end-systolic volume (p = 0.004) and decreased right ventricular ejection fraction (RVEF) (p = 0.004). From 14 months to pre-Fontan, the treatment groups were similar with respect to decline in indexed neo-aortic valve area, >mild neo-aortic valve regurgitation (<5% at each time), indexed tricuspid valve area, and ≥moderate tricuspid valve regurgitation (<20% at each time). CONCLUSIONS: Initial Norwood shunt type influences pre-Fontan RV remodeling during the second and third years of life in survivors with single RV anomalies, with greater RVEF deterioration after RVPAS. Encouragingly, other indices of RV function remain stable before Fontan regardless of shunt type. (Comparison of Two Types of Shunts in Infants with Single Ventricle Defect Undergoing Staged Reconstruction-Pediatric Heart Network; NCT00115934).

Upper body central venous catheters in pediatric cardiac surgery.

BACKGROUND: A central venous catheter located in the jugular or subclavian vein provides rapid, reliable vascular access for pediatric heart surgery. However, intravascular catheters are associated with vessel injury. Stenosis or thrombosis of central veins in the upper body can lead to 'superior vena cava syndrome' with markedly elevated venous pressures in the head and neck, causing facial swelling and headaches. This complication may be especially serious for patients with superior cavopulmonary (Glenn) or total cavopulmonary (Fontan) circulation. The authors hypothesized that upper body central line placement would be associated with a low risk of venous thrombosis or stenosis. METHODS: A three-year retrospective review of infant and univentricular cardiac procedures at a single institution was performed. Two hundred and thirty-five consecutive cardiac surgical patients <1 year of age or undergoing palliation for univentricular cardiac anatomy up to five years of age during January 2010 to December 2012 were included in this study. Upper body central lines are routinely placed by the anesthesiologist after induction of anesthesia for pediatric cardiac surgery at the study institution. The major exception is existing central venous access via an umbilical vein or femoral vein. Patients <2 years of age received a 4.0-French, 5-cm double-lumen central line [Cook Medical polyurethane, no antibiotic or heparin coating]. Those over two years of age received a 5.0-French, 8-cm triple lumen central line [Cook Medical polyurethane, no antibiotic or heparin coating]. A retrospective review of charts, hospital reports, echocardiographic studies, and cardiac catheterization studies was performed. RESULTS: The combined population of infants <1 year of age and patients <5 years of age with functional univentricular hearts totaled 235 patients who underwent 261 cardiac surgical operations. In this cohort of 261 cases, 171 size 4.0 or 5.0-French upper body central lines were inserted. A total of 158 right internal jugular vein catheters were placed. Two left internal jugular lines, two left subclavian lines, and nine right subclavian lines were placed in this population after failure to obtain right internal jugular access. Due to the small sample size (N = 13), the central lines not placed in the right internal jugular vein were excluded from further review. Two cases with right internal jugular venous lines were excluded due to death (without known stenosis or thrombosis) with the line in place. Twenty-three size 4.0- or 5.0-French right internal jugular central venous lines were placed in patients over one year of age (range 1.1-4.3 years) having modified Glenn- or Fontan-type surgery. The central lines were removed with a median of 1.4 days after insertion (range 0.7-8.2 days) for these older children, compared with a median of 4.2 days of age (range 0.3-19.3 days) for the 133 children <1 year of age. Retrospective chart review of nursing notes, progress notes, cardiology notes, discharge summaries, echocardiographic reports, and cardiac catheterization reports for all patients who received an upper body central venous line (internal jugular or subclavian) showed no definitive diagnosis of an upper body venous stenosis or thrombosis related to the central venous line. A further targeted review of echocardiographic and cardiac catheterization studies for univentricular cardiac patients failed to show stenosis or thrombosis of a vessel associated with upper body central line placement. CONCLUSIONS: This study describes one institution's experience with routine upper body central venous catheter placement for neonatal and infant cardiac surgery as well as univentricular cardiac palliation (Glenn and Fontan procedures) with minimal risk of clinically significant catheter-associated vessel thrombosis or stenosis. No upper body central venous stenosis or thrombosis was detected in association with perioperative catheter placement in the upper body central venous system, primarily the right internal jugular vein in 156 cases. Right internal jugular central line placement for infant cardiac surgery can be utilized with a low risk of direct venous thrombosis or stenosis.

Multicenter study comparing shunt type in the norwood procedure for single-ventricle lesions: three-dimensional echocardiographic analysis.

BACKGROUND: The Pediatric Heart Network's Single Ventricle Reconstruction (SVR) trial randomized infants with single right ventricles (RVs) undergoing a Norwood procedure to a modified Blalock-Taussig or RV-to-pulmonary artery shunt. This report compares RV parameters in the 2 groups using 3-dimensional echocardiography. METHODS AND RESULTS: Three-dimensional echocardiography studies were obtained at 10 of 15 SVR centers. Of the 549 subjects, 314 underwent 3-dimensional echocardiography studies at 1 to 4 time points (pre-Norwood, post-Norwood, pre-stage II, and 14 months) for a total of 757 3-dimensional echocardiography studies. Of these, 565 (75%) were acceptable for analysis. RV volume, mass, mass:volume ratio, ejection fraction, and severity of tricuspid regurgitation did not differ by shunt type. RV volumes and mass did not change after the Norwood, but increased from pre-Norwood to pre-stage II (end-diastolic volume [milliliters]/body surface area [BSA](1.3), end-systolic volume [milliliters]/BSA(1.3), and mass [grams]/BSA(1.3) mean difference [95% confidence interval]=25.0 [8.7-41.3], 19.3 [8.3-30.4], and 17.9 [7.3-28.5], then decreased by 14 months (end-diastolic volume/BSA(1.3), end-systolic volume/BSA(1.3), and mass/BSA(1.3) mean difference [95% confidence interval]=-24.4 [-35.0 to -13.7], -9.8 [-17.9 to -1.7], and -15.3 [-22.0 to -8.6]. Ejection fraction decreased from pre-Norwood to pre-stage II (mean difference [95% confidence interval]=-3.7 [-6.9 to -0.5]), but did not decrease further by 14 months. CONCLUSIONS: We found no statistically significant differences between study groups in 3-dimensional echocardiography measures of RV size and function, or magnitude of tricuspid regurgitation. Volume unloading was seen after stage II, as expected, but ejection fraction did not improve. This study provides insights into the remodeling of the operated univentricular RV in infancy.

Doppler flow patterns in the right ventricle-to-pulmonary artery shunt and neo-aorta in infants with single right ventricle anomalies: impact on outcome after initial staged palliations.

BACKGROUND: A Pediatric Heart Network trial compared outcomes in infants with single right ventricle anomalies undergoing Norwood procedures randomized to modified Blalock-Taussig shunt (MBTS) or right ventricle-to-pulmonary artery shunt (RVPAS). Doppler patterns in the neo-aorta and RVPAS may characterize physiologic changes after staged palliations that affect outcomes and right ventricular (RV) function. METHODS: Neo-aortic cardiac index (CI), retrograde fraction (RF) in the descending aorta and RVPAS conduit, RVPAS/neo-aortic systolic ejection time ratio, and systolic/diastolic (S/D) ratio were measured early after Norwood, before stage II palliation, and at 14 months. These parameters were compared with transplantation-free survival, length of hospital stay, and RV functional indices. RESULTS: In 529 subjects (mean follow-up period, 3.0 ± 2.1 years), neo-aortic CI and descending aortic RF were significantly higher in the MBTS cohort after Norwood. The RVPAS RF averaged <25% at both interstage intervals. Higher pre-stage II descending aortic RF was correlated with lower RV ejection fraction (R = -0.24; P = .032) at 14 months for the MBTS cohort. Higher post-Norwood CI (5.6 vs 4.4 L/min/m(2), P = .04) and lower S/D ratio (1.40 vs 1.68, P = .01) were correlated with better interstage transplantation-free survival for the RVPAS cohort. No other Doppler flow patterns were correlated with outcomes. CONCLUSIONS: After the Norwood procedure, infants tolerated significant descending aortic RF (MBTS) and conduit RF (RVPAS), with little correlation with clinical outcomes or RV function. Neo-aortic CI, ejection time, and S/D ratios also had limited correlations with outcomes or RV function, but higher post-Norwood neo-aortic CI and lower S/D ratio were correlated with better interstage survival in those with RVPAS.

Does initial shunt type for the Norwood procedure affect echocardiographic measures of cardiac size and function during infancy?: the Single Vventricle Reconstruction trial.

BACKGROUND: The Pediatric Heart Network trial comparing outcomes in 549 infants with single right ventricle undergoing a Norwood procedure randomized to modified Blalock-Taussig shunt or right ventricle-pulmonary artery shunt (RVPAS) found better 1-year transplant-free survival in those who received RVPAS. We sought to compare the impact of shunt type on echocardiographic indices of cardiac size and function up to 14 months of age. METHODS AND RESULTS: A core laboratory measured indices of cardiac size and function from protocol exams: early after Norwood procedure (age 22.5 ± 13.4 days), before stage II procedure (age 4.8 ± 1.8 months), and at 14 months (age 14.3 ± 1.2 months). Mean right ventricular ejection fraction was <50% at all intervals for both groups and was higher in the RVPAS group after Norwood procedure (49 ± 7% versus 44 ± 8%; P<0.001) but was similar by 14 months. Tricuspid and neoaortic regurgitation, diastolic function, and pulmonary artery and arch dimensions were similar in the 2 groups at all intervals. Neoaortic annulus area (4.2 ± 1.2 versus 4.9 ± 1.2 cm(2)/m(2)), systolic ejection times (214.0 ± 29.4 versus 231.3 ± 28.6 ms), neoaortic flow (6.2 ± 2.4 versus 9.4 ± 3.4 L/min per square meter), and peak arch velocity (1.9 ± 0.7 versus 2.2 ± 0.7 m/s) were lower at both interstage examinations in the RVPAS compared with the modified Blalock-Taussig shunt group (P<0.001 for all), but all were similar at 14 months. CONCLUSIONS: Indices of cardiac size and function after the Norwood procedure are similar for modified Blalock-Taussig shunt and RVPAS by 14 months of age. Interstage differences between shunt types can likely be explained by the physiology created when the shunts are in place rather than by intrinsic differences in cardiac function. CLINICAL TRIAL REGISTRATION: URL: http://www.clinicaltrials.gov. Unique identifier: NCT00115934.