Use of a Postoperative Care Management Pathway Reduces the Incidence of Chylothorax Post-Fontan Palliation.

Pleural effusions and chylothorax are challenging morbidities post-Fontan palliation. We sought to evaluate the efficacy of our Fontan Care Pathway (FCP) in reducing the incidence of post-operative chylothorax and Time to Chest Tube Removal (TTCTR), and to determine risk factors associated with longer TTCTR. Between 2016 and 2022 our institutional approach to post-Fontan care fell into three categories: Group 1 (n = 36): no standardized approach; Group 2 (n = 30): a prophylactic chylothorax diet (fat content < 5%); Group 3 (n = 57): the FCP (a chylothorax diet, fluid restriction, supplemental O2 and aggressive diuresis). The incidence of chylothorax and TTCTR was compared between groups. Predictors of TTCTR were analyzed using linear regression modelling, adjusting for covariates. Chylothorax rate decreased in Group 3 compared to Groups 1 and 2 (9% vs. 28% and 33% respectively, p = 0.011), without alteration in TTCTR. Univariate factors associated with median TTCTR included chylothorax (+ 13.7 days, p = 0.001), additional procedures at time of Fontan (+ 2.4 days per procedure p = 0.017), Fontan revision or takedown (+ 11.7 days, p = 0.018) and minor/major complications (+ 5.1, p = 0.01 and + 15.8, p < 0.001, respectively). On multivariable analysis, chylothorax (+ 6.5 days, p = 0.005) and major complications (+ 15.8 days, p = 0.001) were associated with increased TTCTR. When chylothorax was excluded from multivariable analysis, the FCP showed a significant decrease in TTCTR (- 3.3 days, p = 0.034). A bundled therapy approach was associated with reduced laboratory confirmed chylothorax post-Fontan, whereas diet change alone was not. Additional studies in this area, with larger sample sizes are warranted.

Use of the PK Papyrus covered coronary stent in the treatment of Kawasaki disease-associated giant coronary artery aneurysms.

Kawasaki disease (KD) is an acute vasculitis that can cause coronary artery inflammation and aneurysm formation leading to early obstructive disease. We describe the use of PK Papyrus covered stents (Biotronic, Inc.) in three pediatric patients to exclude coronary artery aneurysms (CAA) from the circulation and relieve aneurysm associated stenoses. Follow-up angiography at 11-17 months postprocedure demonstrated persistent exclusion of CAA and varying degrees of in-stent restenosis (ISR). Two patients required percutaneous coronary intervention with drug eluting stent (DES) implantation to relieve in-stent stenosis. Our findings suggest that CAA exclusion with the PK Papyrus stent is possible and may be a valuable tool in simultaneously treating stenotic and thrombogenic CAA in pediatric KD patients. ISR of these non-DES remains an issue and may require additional interventions within the short-term to maintain vessel patency.

The Gore Cardioform Atrial Septal Defect Occluder: A novel solution to the management of severe hemolysis following transcatheter septal defect closure.

Significant hemolysis is a recognized complication of transcatheter high-velocity shunt occlusion using some Amplatzer devices. We describe a case of severe hemolysis following occlusion of an iatrogenic Gerbode defect with an Amplatzer muscular ventricular septal defect occluder successfully managed by transcatheter device removal and reocclusion with a Gore Cardioform Atrial Septal Defect Occluder.

Long-Term Impact of Hospitalization for Kawasaki Disease on Health-Related Quality of Life.

OBJECTIVE: To prospectively evaluate the long-term impact of Kawasaki disease (KD) hospitalization on health-related quality of life (HRQoL). METHODS: We merged the Outcomes Assessment Program and KD databases and queried for KD admissions between 1 month and 18 years of age. Patients with a diagnosis of community-acquired pneumonia were included as a comparison group. HRQoL was evaluated with the parent proxy Pediatric Quality of Life Inventory (PedsQL). Long-term follow-up PedsQL surveys were performed at least 1 year after initial diagnosis and hospitalization. Results for the entire cohort adjusted for significant differences were calculated. Propensity score-matched cohorts were constructed from the unmatched cohorts of patients with long-term survey responses. Subgroup analysis for the KD group was performed. RESULTS: Patients with KD (n = 61) versus pneumonia (n = 80) had a lower PedsQL total score on admission and experienced a significantly greater HRQoL decline from baseline to admission. At long-term follow-up, no difference occurred in HRQoL between patients with KD and pneumonia, and 89% of patients with KD reached their baseline PedsQL scores. KD diagnostic subtype, coronary artery dilatation, and need for longer follow-up were not associated with HRQoL outcomes at any time point. Intravenous immunoglobulin nonresponders demonstrated lower HRQoL at admission, which did not persist at follow-up. CONCLUSIONS: Children with KD experience acute and significant HRQoL impairment exceeding that of children with newly diagnosed pneumonia, but the scores return to baseline at long-term follow-up. The recoveries at short- and long-term intervals are similar to patients with pneumonia.

Accuracy of Fetal Echocardiography in Defining Pulmonary Artery Anatomy and Source of Pulmonary Blood flow in Pulmonary Atresia with Ventricular Septal Defect (PA/VSD).

Precise delineation of central and branch pulmonary artery anatomy, patent ductus arteriosus, and major aorto-pulmonary collateral artery anatomy in the fetal diagnosis of pulmonary atresia with ventricular septal defect is challenging but important to prenatal counseling and postnatal management. We aimed to evaluate the accuracy of fetal echocardiography to determine these anatomical nuances in pulmonary atresia with ventricular septal defect. This was a retrospective, single-institution, 10-year chart review of consecutive prenatal diagnosis of pulmonary atresia with ventricular septal defect for assessment of pulmonary artery, patent ductus arteriosus, and major aorto-pulmonary collateral artery anatomy and comparison with postnatal imaging including echocardiography, cardiac catheterization, and computerized tomography angiography. Twenty-six fetuses were diagnosed with pulmonary atresia with ventricular septal defect during the review period and complete postnatal follow-up was available in 18, all confirming the basic prenatal diagnosis. Fetal echocardiography accurately predicted central and branch pulmonary artery anatomy in 16 (89%) [confluent in 14, discontinuous in 2], patent ductus arteriosus status in 15 (83%) [present in 10, absent in 5], and major aorto-pulmonary collateral arteries in 17 (94%) [present in 9, absent in 8]. Accuracy increased to 100% for pulmonary artery anatomy (16/16) and major aorto-pulmonary collateral artery (17/17) when excluding patients whose anatomy was reported as uncertain on fetal echocardiography. Fetal echocardiography can provide accurate anatomical details in the vast majority of fetuses with pulmonary atresia with ventricular septal defect. This allows for more anatomy-specific counseling, prognostication, and improved selection of postnatally available management options.

Preoperative Physiology, Imaging, and Management of Coarctation of Aorta in Children.

Coarctation of the aorta (CoA) is a narrowing of the proximal thoracic aorta typically located at the junction of the aorta with the ductus arteriosus. While it is a simple lesion to understand, considerable variation exists in the anatomy and pathophysiology, leading to varied clinical presentation, management options, and prognosis. On the one hand critical CoA manifests in the neonatal period as a duct-dependent lesion, while less severe forms of obstruction present later in childhood or adulthood as hypertension or incidentally noted precordial murmurs. While transthoracic echocardiography is usually adequate, older children and adults may need more advanced imaging modalities like computed tomography and magnetic resonance imaging prior to intervention. Depending on the type of lesion, management options currently available include surgery and percutaneous balloon angioplasty and stenting. Even after successful interventions, these patients need life-long surveillance for residual aortic obstruction and systemic hypertension with variable long-term clinical outcomes.

Long-term outcomes up to 25 years following balloon pulmonary valvuloplasty: A multicenter study.

OBJECTIVE: Evaluate long-term outcomes following balloon pulmonary valvuloplasty (BPV) for pulmonary stenosis (PS). BACKGROUND: Long-term data following BPV is limited to small, single center studies. METHODS: BPV from April 12, 1985 to January 7, 2015 from three centers were included. Outcomes studied were ≥ moderate PI by echocardiogram and residual PS ≥ 40 mm Hg. Risk factors for ≥ moderate PI, residual PS, and repeat intervention were assessed by univariate and multivariate analysis. RESULTS: Among 254 patients, mean age at BPV was 3.8 years (range 1 day-67 years), initial PS catheter gradient was 56 mm Hg (IQR 40-70), 19% had critical PS, and 9% had genetic syndromes. Mean follow-up duration was 7.5 years (maximum 25 years). Sixty-nine (29%) had ≥ moderate PI, 41 patients (17%) had residual PS > 40 mm Hg, and 31 (13%) had re-intervention. In univariate analysis, younger age, lower weight, greater initial PS gradient, greater initial RV/systemic pressure ratio, critical PS, and longer follow-up duration were associated with ≥ moderate PI. Greater initial PS gradient was associated with long-term residual PS or repeat intervention. In multivariate analysis, greater initial gradient and lower weight were independently associated with > moderate PI and greater initial PS gradient and genetic abnormality were independently associated with residual PS and repeat intervention. CONCLUSION: Smaller patients with greater initial PS were more likely to develop significant long-term PI. Patients with greater initial PS and genetic abnormalities were more likely to have residual PS or require repeat intervention following BPV.

Sphincter of Oddi Dysfunction: A Perplexing Presentation.

Sphincter of Oddi dysfunction is caused by stenosis or dyskinesia of the sphincter of Oddi, leading to blockage of bile drainage from the common bile duct. We present the case of a 16-year-old female with chronic abdominal pain who underwent laparoscopic cholecystectomy for cholelithiasis but continued to experience abdominal pain, nausea, and vomiting along with persistently elevated ALT and AST levels. Postoperative abdominal ultrasound was nondiagnostic. Esophagogastroduodenoscopy showed mild reflux esophagitis and mild chronic Helicobacter pylori-negative gastritis. Omeprazole was started, but it did not decrease the frequency and severity of the abdominal symptoms. Magnetic resonance cholangiopancreatography did not reveal any pathology. Endoscopic retrograde cholangiopancreatography with manometry confirmed an elevated biliary sphincter pressure. Biliary sphincterotomy was performed, and the symptoms improved.