Efficient approach to superior vena cava baffle stenosis following the Mustard procedure: Expanding the role of a mechanical rotating dilator sheath for lead extraction.

INTRODUCTION: In adult congenital patients with transposition of the great arteries originally treated with the Mustard (atrial switch) procedure, the most common reason for re-intervention is baffle stenosis. This may be exacerbated by permanent transvenous pacemaker lead placement across the baffle. CASE REPORT: A 47-year-old female status post Mustard procedure performed at 15 months old presented with a high-grade stenosis of the superior vena cava (SVC) baffle from the SVC to the left atrium, with a nonfunctional permanent pacemaker lead passing through the baffle. A mechanical rotating dilator sheath was used for attempted lead extraction, relieving the baffle stenosis almost completely as a secondary effect, before the placement of a 10 × 27 mm Visipro balloon-expandable stent in the SVC baffle. CONCLUSIONS: Use of the mechanical rotating dilator sheath is an evolving treatment strategy in adult congenital heart disease to minimize the risk of bleeding, trauma to surrounding structures, and death. Its ability to fully alleviate baffle stenosis even when full lead extraction is not feasible or is associated with significant procedural risk, further demonstrates its expanded role in this patient population. A multidisciplinary approach and great diligence must be employed to avoid potential complications.

Factors associated with development of early and late pulmonary hypertension in preterm infants with bronchopulmonary dysplasia.

OBJECTIVE: To investigate factors associated with development of early and late pulmonary hypertension (E/LPH) in preterm infants with bronchopulmonary dysplasia (BPD). STUDY DESIGN: A retrospective case-control observational study of preterm infants with BPD admitted to a level IV referral neonatal intensive care unit over 5 years. We compared pre- and postnatal characteristics between infants with or without BPD-associated EPH and LPH. RESULTS: Fifty-nine out of 220 infants (26.8%) had LPH, while 85 out of 193 neonates (44%) had EPH. On multiple logistic regression, novel factors associated with development of BPD-LPH included presence of maternal diabetes, EPH, tracheostomy, tracheitis, intraventricular hemorrhage (IVH, grade ≥3) and systemic steroid use. For EPH, these were maternal diabetes, IVH grade ≥3, high frequency ventilator use, and absence of maternal antibiotics use. CONCLUSION: We identified novel factors and confirmed previously established factors with development of LPH and EPH, which can help develop a screening strategy in BPD patients.

Rare case of a superior sinus venosus atrial septal defect presenting with cyanosis.

Cyanosis in a newborn is commonly due to intracardiac shunts or duct-dependent lesions. Systemic venous anomalies are uncommon and sinus venous atrial septal defects do not usually present with cyanosis. We report the case of a 4-month-old female with persistent hypoxemia due to a right superior vena cava overriding a superior sinus venous atrial septal defect resulting in a right to left shunt.

Removal of a retained intracardiac radiolucent guidewire fragment using an Atrieve™ vascular snare using combined fluoroscopy and transesophageal echocardiography guidance in an infant.

Retained intravascular foreign body is a well-known complication of central venous access placement in children as well as adults. Most of these foreign bodies are radio-opaque and hence are removed under fluoroscopy guidance. In our case, we describe the removal of an intracardiac radiolucent foreign body in an infant utilizing a combination technique - transesophageal echocardiogram and fluoroscopy.

Early catheterization after initiation of extracorporeal membrane oxygenation support in children is associated with improved survival.

OBJECTIVES: The study evaluated the institutional experience with cardiac catheterization on extracorporeal membrane oxygenation (ECMO) support. BACKGROUND: There is scant literature on the outcomes of catheterization on ECMO. METHODS: A retrospective review was performed of all children who underwent catheterization on ECMO from 2003 to 2013. Patients were categorized as cardiomyopathy (CM) or congenital heart disease (CHD). RESULTS: During the study period, 215 children were placed on cardiac ECMO. Of these, 29.8% underwent 75 catheterization procedures while on ECMO support. The median age of the cohort was 1.5 months (range 0 days -16.7 years) and the median weight was 3.9 kg (2.2-63.1 kg). CM patients constituted 18.8% of the cohort and all of them underwent atrial septoplasty (an atrial septal stent in 7/12 and balloon atrial septoplasty or septostomy in 5). The survival to hospital discharge rate was 83% and the transplant-free survival rate was 58.3%. CHD patients constituted 81.2% of the cohort. In this group, transcatheter interventions were performed in 40.4% and subsequent surgical interventions in 40.4%. Survival to hospital discharge rate was 34.6% and transplant free survival rate was 32.7%. Overall, 76.7% underwent transcatheter or surgical interventions. The major catheterization complication rate was 6.7%. The mean ECMO-to-catheterization time was 1.6 days for survivors and 3.5 days for non-survivors (P = 0.034). Survival to discharge was better for the CM group compared to the CHD group (P = 0.01). Among CHD, survival was better with transcatheter interventions compared to no interventions or surgical interventions (P < 0.001). CONCLUSIONS: Cardiac catheterization and transcatheter interventions on ECMO can be performed with low rate of complications. Catheterization was associated with high rate of interventions. Better survival to hospital discharge was associated with transcatheter interventions, earlier performance of catheterization after ECMO and diagnosis of CM. © 2016 Wiley Periodicals, Inc.

New transplant technique with hemiazygos continuation and interrupted inferior vena cava.

In the presence of left superior vena cava, cardiac transplantation is performed at the risk of left superior vena cava flow obstruction. In patients with hemiazygos continuation with interrupted inferior vena cava, the patency of left superior vena cava is vital. We introduced a new surgical technique to use a Fontan connection including the native central pulmonary artery as the systemic venous return. The pulmonary artery anastomosis was performed distal to the superior vena cava anastomosis sites. We used this treatment approach successfully to perform cardiac transplantation in two patients who developed severe heart failure after Fontan completion.

An unusual presentation of necrotizing enterocolitis on an echocardiogram.

This report describes the spontaneous intracardiac air contrast found on the echocardiogram of a 5 day-old term neonate with Down syndrome and a complete atrioventricular septal defect who had experienced sudden-onset tachypnea and systemic desaturation. The stream of air contrast was tracked coming from the hepatic veins, and a diagnosis of necrotizing enterocolitis was suspected. An abdominal radiograph and ultrasound confirmed the diagnosis.

Cystatin C and neutrophil gelatinase-associated lipocalin as markers of renal function in pediatric heart transplant recipients.

We hypothesized that use of Schwartz formula underestimates the prevalence of CKD in PHT recipients. This study determined the prevalence and risk factors for CKD in PHT using novel methods-serum cystatin C, CKiD formula, Revised Schwartz formula, s- and u-NGAL. Serum BUN, creatinine, cystatin C and s- and u-NGAL were measured after prospective enrollment. Schwartz formula GFR was compared with novel methods. CKD was defined as CKiD GFR < 90 mL/min/1.73 m(2) . The s- and u-NGAL were compared between those with and without CKD. Potential risk factors for CKD were analyzed. Seventy-nine patients (46 male children or boys), mean age 9.9 ± 5.8 yr formed the study cohort. The prevalence of mild and moderate CKD was 2- to 3-fold higher using novel methods compared to Schwartz formula. u-NGAL and u-NGAL/Cr were significantly higher in patients with CKD. u- and s-NGAL had negative correlation with estimates of GFR. Women were at a higher risk for CKD (odds ratio 8.7) as was longer duration since transplant (p = 0.009). In conclusion, use of novel methods of GFR estimation unmasked 2- to 3-fold increased prevalence of CKD in PHT. Women and those with longer duration since transplant are at higher risk for CKD.

Cardiopulmonary bypass flow rate: a risk factor for hyperlactatemia after surgical repair of secundum atrial septal defect in children.

OBJECTIVE: Early postoperative hyperlactatemia is seen in some children after surgical repair of secundum atrial septal defect despite apparently normal cardiac output. The objective of the study was to investigate the intraoperative risk factors for hyperlactatemia in patients undergoing atrial septal defect repair. METHODS AND RESULTS: A retrospective review of 68 consecutive patients who underwent isolated atrial septal defect repair at Arkansas Children's Hospital between January 2001 and March 2006 was performed. Perioperative factors in the high lactate group (lactate >3 mmol/L, n = 26) were compared with those in the low lactate group (n = 42). Early hyperlactatemia was seen in 38% of the cohort. The high lactate group showed significantly lower weight-indexed cardiopulmonary bypass flow rate (101 + or - 6.5 mL/kg(-1)/min(-1) vs 131 + or - 6.0 mL/kg(-1)/min(-1), P = .0013), oxygen delivery during cardiopulmonary bypass (mean 12.7 + or - 0. 7 mL/kg(-1)/min(-1) vs 17.0 + or - 1 mL/kg(-1)/min(-1), P = .0009), and higher postoperative glucose (191 + or - 8.6 mg/dL vs 151 + or - 5.4 mg/dL, P = .003) compared with the LL group. Multivariate logistic regression analysis showed that weight-indexed cardiopulmonary bypass flow rate (P = .007) and average mean arterial blood pressure during cardiopulmonary bypass (P = .009) were independent risk factors for postoperative hyperlactatemia. Cardiopulmonary bypass flow rate less than 100 mL/kg(-1)/min(-1) was associated with an odds ratio of 7.67 (95% confidence interval, 1.28-45.86; P = .026) for postoperative hyperlactatemia. CONCLUSION: Lower weight-indexed cardiopulmonary bypass flow rate is an independent risk factor for early postoperative hyperlactatemia in children after atrial septal defect repair.

Pathologic aneurysmal dilation of the ascending aorta and dilation of the main pulmonary artery in patients with Kabuki syndrome: valve-sparing aortic root replacement.

We report the aneurysmal dilation of the ascending aorta and the main pulmonary artery in 2 children with Kabuki syndrome. In 1 patient, there was progressive aneurysmal dilation of the ascending aorta necessitating aortoplasty. Histologic examination of the resected aorta revealed disrupted and fragmented elastic fibers in the medial layer, along with mucinous degeneration of the aortic wall. This is the first recognition and report of these findings as part of the Kabuki syndrome.