Role of Transcatheter patent ductus arteriosus closure in extremely low birth weight infants.

BACKGROUND: Patent ductus arteriosus (PDA) is common in extremely low birth weight (ELBW) infants. The objectives of this study were to describe our early clinical experience of transcatheter PDA closure (TCPC) in ELBW infants, compare outcomes with surgical ligation of PDA (SLP), and identify risk factors for prolonged respiratory support. METHODS: A retrospective review was performed comparing infants born <27 weeks, weighing <1 kg at birth and < 2 kg during TCPC with 2:1 propensity-score matched group of infants that underwent SLP. Change in respiratory severity scores (RSS) immediately post-procedure and the time taken for return to pre-procedure RSS for TCPC versus SLP was compared. Factors contributing to prolonged elevation of RSS were identified. RESULTS: Eighty ELBW infants (median procedure weight: 1060 [range 640-2000] grams) that underwent successful TCPC were compared with 40 infants that underwent SLP (procedure weight 650-1760 g). There was greater increase in RSS following SLP compared to TCPC (76% vs. 18%; P < 0.01). It took longer for RSS to return to pre-procedural scores post-SLP compared to post-TCPC (28 vs. 8.4 hr; P < 0.01). Elevated pulmonary artery pressure (PAP) and TCPC at >8 weeks of age were associated with prolonged (>30-days) elevation of RSS ≥ 1 (OR = 5.4, 95%CI: 2.2-9.4, P < 0.01 and OR = 2.86, 95%CI: 1.5-4.2, P = 0.05 respectively). Overall complication rate for TCPC was 3.7%. CONCLUSIONS: TCPC is feasible in infants as small as 640-2000 g and can be performed safely in the majority. TCPC may offer faster weaning of respiratory support compared to SLP when performed earlier in life, and before the onset of elevated PAP.

Improved Outcomes in Management of Hypoplastic Left Heart Syndrome Associated With Congenital Diaphragmatic Hernia: an Algorithmic Approach.

Hypoplastic left heart syndrome (HLHS) is the second most common congenital heart disease associated with congenital diaphragmatic hernia (CDH). The reported survival rate of neonates with CDH and HLHS is only 1%-5%. We review our experience with CDH and HLHS and compare our outcomes with published literature. Retrospective review of all neonates with CDH and HLHS at our institution over a 10-year period was performed. The morphology of cardiac and diaphragm defects, clinical course, treatment strategies, and outcomes were reviewed, and an algorithmic approach was proposed. Five patients with CDH and HLHS were treated between 2006 and 2016. All had mitral stenosis with aortic stenosis. Four patients had a left-sided Bochdalek diaphragmatic hernia and 1 patient had a large bilateral Morgagni hernia. Two (2/4) of the Bochdalek hernias were associated with significant pulmonary hypoplasia and required patch closure of the CDH; both were palliated with percutaneous ductal stents and both died. Three patients underwent primary Norwood operation followed by repair of less severe CDH defect. All 3 patients are currently well and have survived bidirectional Glenn anastomosis; one patient is well after Fontan operation. Successful palliation of neonates with HLHS and associated CDH is possible in the current era. Outcome is determined primarily by the severity of the CDH and the degree of associated pulmonary hypoplasia. An algorithmic team approach is helpful in management of this difficult group of patients.

Diaphragmatic fenestration for refractory chylothorax after congenital cardiac surgery in infants.

OBJECTIVE: Medically refractory chylous pleural effusion after congenital heart surgery is associated with significant morbidity and mortality, especially in infants. We reviewed our experience with diaphragmatic fenestration procedure in this group of patients. METHODS: A retrospective chart review of all patients who had diaphragmatic fenestrations for chylous effusion at our institution over a 2-year period was performed. RESULTS: A total of 9 diaphragmatic fenestration procedures were performed in 8 patients who had failed medical management of chylous pleural effusions. All procedures except 1 were performed on the right side. The median age at time of procedure was 4.6 months (range, 3 weeks to 14 months). The average time between primary congenital cardiac surgery and fenestration was 26 days (range, 4-53 days). Three patients had single ventricle repair. Average time of chest tube removal after procedure was 4 days. Average time to extubation was 3 days. All patients but 1 were able to advance to full feedings without reaccumulation of chylous effusion within 12 days. No complications developed in the patients. Recurrent effusion over a median follow-up period of 19 months did not develop in the patients. CONCLUSIONS: Diaphragmatic fenestration is an effective and safe strategy for management of persistent chylous effusions after congenital cardiac surgery.