Natural History of Postnatal Cardiopulmonary Adaptation in Infants Born Extremely Preterm and Risk for Death or Bronchopulmonary Dysplasia.

OBJECTIVE: To study the natural history of postnatal cardiopulmonary adaptation in infants born extremely preterm and establish its association with death or bronchopulmonary dysplasia (BPD). STUDY DESIGN: This was a prospective, observational, cohort study of infants born extremely preterm (<29 weeks). Initial echocardiogram was performed at <48 hours of life, followed by serial echocardiograms every 24-48 hours until 14 days of life. Resolution or no resolution of pulmonary hypertension (PH) at 72-96 hours was considered normal or delayed postnatal cardiopulmonary adaptation, respectively. PH between 96 hours and 14 days was defined as subsequent PH. Elevated pulmonary artery pressure throughout the 14 days of life was considered persistent PH. BPD was assessed at 36 weeks of postmenstrual age. RESULTS: Sixty infants were enrolled; 2 died before a sequential echocardiogram could be done at 72-96 hours. Normal and delayed cardiopulmonary adaptation were noted in 26 (45%) and 32 (55%) infants, respectively. Five patterns of postnatal cardiopulmonary adaptation were recognized: normal without subsequent PH (n = 20), normal with subsequent PH (n = 6), delayed adaptation without subsequent PH (n = 6), delayed adaptation with subsequent PH (n = 16), and persistent PH (n = 10). Infants with delayed cardiopulmonary adaptation were of lower gestation and birth weight and required prolonged ventilation and supplemental oxygen (P < .05). On multivariate analysis, the incidence of death or BPD was significantly greater among infants with delayed adaptation (P < .001). CONCLUSION: Infants born extremely preterm have normal or delayed postnatal cardiopulmonary adaptation that can be complicated by subsequent or persistent PH. Delayed cardiopulmonary adaptation is associated independently with death or BPD.

The use of a three-dimensional print model of an aortic arch to plan a complex percutaneous intervention in a patient with coarctation of the aorta.

Recently, three-dimensional printing of heart models is being used to plan percutaneous and surgical interventions in patients with CHD. We describe a case where we used a three-dimensional print model to plan a complex percutaneous intervention in a patient with coarctation of the aorta.

Pulmonary vascular resistance in repaired congenital diaphragmatic hernia vs. age-matched controls.

INTRODUCTION: Infants and children with repaired congenital diaphragmatic hernia (CDH) often continue to show delayed growth and development that may be, in part, secondary to unrecognized persistence of increased pulmonary vascular resistance (PVR). METHODS: Data were reviewed from all patients ages 6-36 mo with repaired CDH who underwent cardiac catheterization from 2007 to 2010 and were compared to data from a control population of patients undergoing percutaneous closure of a patent ductus arteriosus (PDA). Indexed pulmonary blood flow (Qp), mean pulmonary artery pressure (mPAP), pulmonary capillary wedge pressure (PCWP), and PVR were examined. RESULTS: Data from 8 CDH patients and 10 control patients were examined. The mPAP (22.5 ± 3.33 vs. 18.2 ± 4.13 mm Hg) and PVR (3.66 ± 0.79 vs. 1.22 ± 0.4 iwU (indexed Wood's units)) were both significantly elevated in the CDH population, whereas the Qp (4.08 ± 1.43 vs. 6.82 ± 1.46 l/min/m(2)) was significantly lower in this population. There was no significant difference in pulmonary capillary wedge pressure (PCWP). Less than half of the CDH patients had signs of pulmonary hypertension (PH) on echocardiogram. DISCUSSION: Our data suggest that children who are ages 6-36 mo with repaired CDH have significantly increased PVR compared with controls and early consideration of cardiac catheterization may be warranted.

Successful Surgical Management of Aortic Arch Thrombosis in the Neonate.

Spontaneous thrombosis in the aortic arch is a rare finding in the neonate. Often the thrombosis is initially interpreted as arch obstruction secondary to coarctation or interrupted aortic arch. Thus the obstruction is mechanical with no structural abnormality. We describe 2 newborns with coarctation and extensive thrombosis within the aortic arch. We report their successful surgical management, with a brief review of the literature and pertinent management principles.

Impact of Percutaneous Interventions for Pulmonary Artery Stenosis in Alagille Syndrome.

OBJECTIVE: The study aims to examine acute and midterm outcomes after percutaneous interventions for treatment of pulmonary artery stenosis (PAS) in patients with Alagille Syndrome (ALGS). BACKGROUND: PAS affects up to two thirds of ALGS patients. Responsiveness to transcatheter therapies may differ from other causes of PAS. To date, there has been no study to evaluate outcomes of transcatheter interventions on PAS exclusively in ALGS. METHODS: In this single-center series, we reviewed procedural, hemodynamic, and angiographic data from patients with ALGS and PAS from 2007 to 2011 who underwent an interventional catheterization. Minimal luminal diameter (MLD) was assessed pre- and postintervention, and at follow-up catheterization(s) when available. Acute and midterm response to high-pressure balloon angioplasty (HBA), bare metal stent (BMS) placement, and cutting balloon angioplasty (CBA) were assessed. RESULTS: Nine patients (median age 9.1 years) underwent 16 cardiac catheterizations with 34 interventions performed (20 HBA, 11 BMS, 3 CBA). There was a significant acute increase in MLD for all three modalities (42% HBA, P < .01; 91% BMS, P < .01; 58% CBA, P = .04). Follow-up data were available for 19 treated lesions at a median of 11 months. There was no significant difference in the improvement of MLD from baseline between the HBA and BMS groups, although in contrast to the BMS group, the HBA group showed continued interval vessel growth. CONCLUSIONS: Transcatheter intervention for PAS in ALGS is generally safe and acutely effective. Although BMS implantation was associated with the greatest immediate improvement in MLD, HBA-treated vessels demonstrate interval growth, whereas BMS-treated lesions do not.

Percutaneous balloon-expandable covered stent implantation for treatment of traumatic aortic injury in children and adolescents.

Surgical treatment of pediatric acute traumatic aortic injury (TAI) after blunt chest trauma is standard of care. Use of endovascular stent grafts for treatment of TAI in adults is common but has important limitations in children. We sought to describe the use of balloon-expandable covered endovascular stents for treatment of TAI in children and adolescents. Participants of the multicenter Coarctation of the Aorta Stent Trial (COAST) had access to investigational large-diameter, balloon-expandable, covered stents (covered Cheatham-platinum stents; NuMed, Inc., Hopkinton, New York) on an emergency-use basis. From 2008 through 2011, 6 covered stents were implanted in 4 patients at 3 COAST centers for treatment of TAI. Median patient age was 13.5 years (range 11 to 14) and weight was 58 kg (40 to 130). All patients sustained severe extracardiac injuries that were judged to preclude safe open surgical repair of TAI. Median aortic isthmus and stent implantation balloon diameters were 16.4 mm (13.2 to 19) and 19 mm (16 to 20), respectively. Stent implantation was technically successful in all attempts. Complete exclusion of aortic wall injury was achieved in all cases. There were no access site complications. At a median follow-up of 24 months, there was 1 early death (related to underlying head trauma) and 1 patient with recurrent aortic aneurysm who required additional stent implantation. In conclusion, balloon-expandable covered-stent implantation for treatment of pediatric TAI after blunt trauma is generally safe and effective. Availability of this equipment may alter the standard approach to treatment of pediatric TAI.

Fenestrated ASD closure in a child with idiopathic pulmonary hypertension and exercise desaturation.

Pulmonary arterial hypertension is a progressive disorder that may result in right heart failure and death. Atrial level shunts in the presence of pulmonary hypertension may allow right-to-left mixing with maintenance of cardiac output and improved survival. However, excessive mixing at the atrial level can cause undue systemic desaturation, increased fatigue and decreased exercise tolerance even in the presence of adequate cardiac output. A 5½-year-old was diagnosed with pulmonary hypertension, a large atrial septal defect and right-to-left shunting. Medical therapy over an 18-month period was successful in decreasing pulmonary artery pressure and pulmonary vascular resistance. However, because of the size and position of the intracardiac defect, symptoms of fatigue, and severe systemic desaturation with only minor activities persisted. Fenestrated surgical closure of the defect was thus undertaken to decrease the degree of atrial mixing, but still allow atrial decompression if necessary. Subsequent hemodynamic evaluation has demonstrated continued improvement, and all previous symptoms have resolved. Repeated echocardiography has confirmed patency of the atrial fenestration with left-to-right atrial flow.