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This study compared the percent change in systolic blood pressure and the incidence of adverse cardiac events (ACEs; defined as cardiac arrest, cardiopulmonary resuscitation, arrhythmias, or ST-segment changes) during anesthesia induction in patients with Williams syndrome (WS) before and after implementation of a perioperative management strategy. Retrospective observational cohort study. Single quaternary academic referral center. The authors reviewed the records of all children with WS at the authors' institution who underwent general anesthesia for cardiac catheterization, diagnostic imaging, or any type of surgery between November 2008 and August 2019. The authors identified 142 patients with WS, 48 of whom underwent 118 general anesthesia administrations. A historic group (HG) was compared with the intervention group (IG). Change in perioperative management (three-stage risk stratification: preoperative intravenous hydration, intravenous anesthesia induction, and early use of vasoactives). The authors determined event rates within 60 minutes of anesthesia induction. Standardized mean difference (SMD) was calculated (SMD >0.2 suggests clinically meaningful difference). Sixty-seven general anesthesia encounters were recorded in the HG (mean age, 4.8 years; mean weight, 16.3 kg) and 51 in the IG (mean age, 6.0 years; mean weight, 18.2 kg). The change in systolic blood pressure was –17.5% (–30.0, –5.0) in the HG versus –9% (–18.0, 5.0) in the IG (p = 0.015; SMD = 0.419), and the incidence of ACEs was 6% in the HG and 2% in the IG (p = 0.542; SMD = 0.207). Preoperative risk stratification, preoperative intravenous hydration, intravenous induction, and early use of continuous vasoactives resulted in greater hemodynamic stability, with a 2% incidence of ACEs. [ABSTRACT FROM AUTHOR]

Patients with Williams and elastin arteriopathy syndromes often have similar cardiac phenotypes characterized by supravalvar aortic stenosis (SVAS), peripheral pulmonary artery stenosis (PPAS), and coronary artery ostial stenosis (CAOS). SVAS and/or PPAS result in a marked increase in myocardial oxygen demand, whereas CAOS may limit myocardial oxygen supply. This combination predisposes to myocardial ischemic events and sudden cardiac arrest. The purpose of this study was to review our experience with the surgical repair of CAOS in patients with Williams and elastin arteriopathy syndromes. This was a retrospective review of 16 patients with Williams (n = 11) or elastin arteriopathy (n = 5) who underwent surgical repair of CAOS as a concomitant procedure with SVAS and/or PPAS repair. Eleven patients had moderate or severe SVAS, and 10 had PPAS. The median age at surgery was 9 months (range, 3-108 months). Seven patients had repair of the left main and right coronary ostia, 6 the left main, and 3 the right coronary ostium. Median duration of aortic crossclamp was 56 minutes and cardiopulmonary bypass time was 454 minutes. The median SVAS gradient decreased from 70 to 12 mm Hg and pulmonary artery pressure decreased from 120 to 30 mm Hg. There was 1 operative mortality (6%). The remaining 15 patients were discharged from the hospital and are alive at a median of 17 months' follow-up. The data demonstrate that patients with Williams and elastin arteriopathy syndromes presenting with SVAS and/or PPAS plus CAOS can undergo successful repair of all hemodynamic issues simultaneously. [Display omitted] [ABSTRACT FROM AUTHOR]

Peripheral pulmonary artery stenosis is a relatively rare form of congenital heart disease typically associated with genetic syndromes, such as Williams or Alagille syndromes. However, some patients present with severe stenosis without associated syndromes. The purpose of the study was to review our surgical experience in such patients. This was a retrospective review of 30 patients who underwent surgical repair for peripheral pulmonary artery stenosis. Concomitant anatomical diagnoses in 20 patients (67%) included supravalvar aortic stenosis (n = 8), tetralogy of Fallot (n = 4), d-transposition of the great arteries (n = 2), truncus arteriosus (n = 2), hypoplastic left heart syndrome (n = 2), ventricular septal defect (n = 1), and patent ductus arteriosus (n = 1). Additional medical diagnoses in 15 patients (50%) included elastin arteriopathy (n = 9), pulmonary artery calcinosis (n = 1), arterial tortuosity syndrome (n = 1), DiGeorge syndrome (n = 1), and Noonan syndrome (n = 1). Median age at surgery was 3.6 years (interquartile range 1.6-7.4 years). Seventeen patients (57%) had prior cardiac operations, and 16 patients (53%) previously underwent percutaneous intervention. With surgery, mean right ventricle-to-aortic systolic pressure ratio decreased from 0.95 ± 0.2 to 0.28 ± 0.08 (P< 0.0001). Median duration of cardiopulmonary bypass was 369 minutes. There was 100% survival to hospital discharge, with no mortality at mean follow-up of 2.3 years. No patient required reoperation, while 4 underwent balloon dilation. Freedom from pulmonary artery catheter-based reintervention was 95% and 80% at 12 and 36 months, respectively. Patients with peripheral pulmonary artery stenosis without either Williams or Alagille syndrome can successfully undergo surgical repair with a significant reduction in right ventricle-to-aortic pressure ratios. [ABSTRACT FROM AUTHOR]

The study sought to evaluate the short-term and midterm outcomes of surgical pulmonary artery reconstruction in patients with Williams syndrome (WS). We performed a retrospective cohort study of all patients with WS who underwent surgical pulmonary artery reconstruction at Lucile Packard Children's Hospital between January 2001 and May 2018. There were 25 WS patients (52% female) who underwent pulmonary artery reconstruction during the study period. Median age at surgery was 2.4 (interquartile range [IQR], 0.9 to 4.5) years. Median preoperative right ventricular (RV) pressure was 80 (IQR, 70 to 90) mm Hg and aortic pressure was 96 (IQR, 90 to 107) mm Hg, with an RV-to-aortic pressure ratio of 0.8 (IQR, 0.7 to 1.0). The median number of pulmonary arterioplasty patches was 16.5 (IQR, 6.5 to 24). Median postoperative RV pressure was 27 (IQR 20 to 31) mm Hg and aortic pressure was 90 (IQR, 87 to 105) mm Hg, with an RV-to-aortic pressure ratio of 0.27 (IQR, 0.22 to 0.35). The postoperative RV pressure and RV-to-aortic pressure ratio were significantly lower than preoperative RV pressure and RV-to-aortic pressure ratio (p < 0.0001 for both). There was 1 (4%) postoperative death. In a median follow-up of 2.6 (IQR, 0.94 to 3.4) years, 1 (4.2%) patient has undergone RV outflow tract aneurysm repair and 2 (8.3%) patients have undergone balloon dilation of the pulmonary arteries. Multilevel, surgical pulmonary artery reconstruction addressing severe extrapericardial stenoses is highly effective in patients with WS. This technique results in immediate normalization of RV pressure and has a low rate of reintervention in midterm follow-up. [ABSTRACT FROM AUTHOR]

Objectives Angiotensin converting enzyme inhibitors (ACEI) have been shown to decrease aortic growth velocity (AGV) in Marfan syndrome (MFS). We sought to compare the effect of β-blockers and ACEI on AGV in MFS. Study design We retrospectively reviewed all data from all patients with MFS seen at Arkansas Children's Hospital between January 1, 1976 and January 1, 2013. Generalized least squares were used to evaluate AGV over time as a function of age, medication group, and the interaction between the 2. A mixed model was used to compare AGV between medication groups as a function of age, medication group (none, β-blocker, ACEI), and the interaction between the 2. Results A total of 67 patients with confirmed MFS were identified (34/67, 51% female). Mean age at first encounter was 13 ± 10 years, with mean follow-up of 7.6 ± 5.8 years. There were 839 patient encounters with a median of 10 (range 2-42) encounters per patient. AGV was nearly normal in the β-blocker group, and was less than either the ACEI or untreated groups. The AGV was higher than normal in ACEI and untreated groups (P < .001 for both). Conclusions β-blocker therapy results in near-normalization of AGV in MFS. ACEI did not decrease AGV in a clinically significant manner. [ABSTRACT FROM AUTHOR]

Objective: To evaluate whether the presence of patent ductus arteriosus (PDA) in preterm infants worsens long-term neurodevelopmental outcomes.Study Design: This was a secondary observational analysis of data from 1090 preterm low-birthweight infants in the Infant Health and Development Program (IHDP), a multicenter longitudinal cohort study of outcomes assessed from 3 to 18 years of age. Multivariable analysis was adjusted for IHDP treatment group (intervention or follow-up), birth weight, maternal race, maternal education, infant sex, maternal preconception weight, Home Observation Measurement of the Environment (HOME) total score at 12 months, neonatal health index, and gestational age.Results: Of the 1090 patients (49% male) included in the analysis, 135 had a PDA. Mean birth weight (1322 g vs 1871 g; P < .0001) and gestational age (30.2 weeks vs 33.4 weeks, P < .0001) were lower and mean ventilator days (11.8 vs 1.3; P < .0001), vasopressor use (12.6% vs 1.2%; P < .0001), and congestive heart failure (8.9% vs 0.1%; P < .0001) were higher in the PDA group. There were no differences between the PDA and no-PDA groups in maternal education level and HOME total score at age 12 months. Multivariable analysis demonstrated no between-group differences in cognitive development or behavioral competence at age 3, 8, and 18 years.Conclusions: The presence of a PDA in moderately preterm, low-birthweight infants does not impact long-term neurodevelopmental outcomes. [ABSTRACT FROM AUTHOR]

Cardiovascular abnormalities in Williams syndrome (WS) are largely attributable to elastin haploinsufficiency resulting from a large deletion of the elastin-containing region on chromosome 7q11.23. The risk of sudden death in patients withWS is 25- to 100-fold greater than that in the general population. The corrected QT (QTc) interval is prolonged in 14% of patients with WS. Patients with nonsyndromic supravalvar aortic stenosis (NSVAS) have elastin mutations resulting in elastin haploinsufficiency and a vascular phenotype nearly identical to that of WS. No previous studies have evaluated the QTc duration in NSVAS. A retrospective review of all electrocardiograms (ECGs) performed on consecutive patients with NSVAS at Arkansas Children's Hospital from January 1, 1985 to January 1, 2012 was completed.ECGs with nonsinus rhythm or unmeasurable intervals were excluded. TheECGs were read by 1 reader who was unaware of previous readings. A QTc interval of ‡460 ms was defined as prolonged. The NSVAS cohort was compared to previously published WS and control groups using the mixed model for continuous electrocardiographic variables and the generalized estimating equation for binary indicators for prolonged QTc. The generalized estimating equation used bootstrapping with 1,000 replicates. A total of 300 ECGs (median 6, range 1 to 27) from the 35 identified patients with NSVAS met the inclusion criteria. A total of 482ECGs from patients withWSand 1,522ECGs from controls were included. The mean age of the patients with NSVAS at ECG was 7.3 - 6.9 years; 64% were male. The mean QTc duration was 409 - 20 ms in the NSVAS group, 418 - 17 ms in the control group (p <0.001), and 436 - 27 ms in theWSgroup (p <0.001 compared to the control group). The prevalence of QTc prolongation was 0.3% in the NSVAS group, 2.0% in the control group (p <0.001), and 14.8% in the WS group (p <0.001 compared to controls). No patients with NSVAS died. In conclusion, cardiac repolarization is normal in patients with NSVAS. Elastin haploinsufficiency does not appear to be the etiology of QTc prolongation in patients with WS. The possible contribution of other genes on 7q11.23 to QTc prolongation in WS should be investigated. [ABSTRACT FROM AUTHOR]