Computed tomographic parenchymal lung findings in premature infants with pulmonary vein stenosis.

BACKGROUND: Developmental pulmonary vein pulmonary vein stenosis in the setting of prematurity is a rare and poorly understood condition. Diagnosis can be challenging in the setting of chronic lung disease of prematurity. High-resolution non-contrast chest computed tomography (CT) is the conventional method of evaluating neonates for potential structural changes contributing to severe lung dysfunction and pulmonary hypertension but may miss pulmonary venous stenosis due to the absence of contrast and potential overlap in findings between developmental pulmonary vein pulmonary vein stenosis and lung disease of prematurity. OBJECTIVE: To describe the parenchymal changes of pediatric patients with both prematurity and pulmonary vein stenosis, correlate them with venous disease and to describe the phenotypes associated with this disease. MATERIALS AND METHODS: A 5-year retrospective review of chest CT angiography (CTA) imaging in patients with catheterization-confirmed pulmonary vein stenosis was performed to identify pediatric patients (< 18 years) who had a history of prematurity (< 35 weeks gestation). Demographic and clinical data associated with each patient were collected, and the patients' CTAs were re-reviewed to evaluate pulmonary veins and parenchyma. Patients with post-operative pulmonary vein stenosis and those with congenital heart disease were excluded. Data was analyzed and correlated for descriptive purposes. RESULTS: A total of 17 patients met the inclusion criteria (12 female, 5 male). All had pulmonary hypertension. There was no correlation between mild, moderate, and severe grades of bronchopulmonary dysplasia and the degree of pulmonary vein stenosis. There was a median of 2 (range 1-4) diseased pulmonary veins per patient. In total, 41% of the diseased pulmonary veins were atretic. The right upper and left upper lobe pulmonary veins were the most frequently diseased (n = 13/17, 35%, n = 10/17, 27%, respectively). Focal ground glass opacification, interlobular septal thickening, and hilar soft tissue enlargement were always associated with the atresia of an ipsilateral vein. CONCLUSION: Recognition of the focal parenchymal changes that imply pulmonary vein stenosis, rather than chronic lung disease of prematurity changes, may improve the detection of a potentially treatable source of pulmonary hypertension, particularly where nonangiographic studies result in a limited direct venous assessment.

Simultaneous deployment of a covered stent and a Sapien S3 as a bridge to surgical valve replacement in acute infective endocarditis.

A 25-year-old male presented to our institution with acute right ventricular (RV) failure secondary to an infected pulmonary prosthetic valve (Melody® Valve, Medtronic Inc., Minneapolis, MN, USA), 3 months after percutaneous placement. Due to his decompensated state, his surgical risk was determined to be very high during his acute illness. As a bridge to surgical valve replacement, we elected to percutaneously implant a Sapien S3 valve (Edwards Lifesciences, Irvine, CA, USA), mounted within a covered stent and deployed simultaneously to exclude the mass of infected tissue within the RV outflow tract, and improve his hemodynamics. Our patient tolerated the procedure well; recovered fully and returned 2 months later for planned surgical valve replacement. .

Technical Feasibility on the Use of Optical Coherence Tomography in the Evaluation of Pediatric Pulmonary Venous Stenosis.

Pulmonary vein stenosis (PVS) in children is a morbid disease and limited progress has been made in improving outcomes for this heterogenous group of patients. Evaluation is currently limited to imaging techniques that fail to provide an adequate overview of the intraluminal and luminal pathology perpetuating our limited understanding of this condition. Optical coherence tomography (OCT) is an imaging modality which provides intraluminal profiling with microstructural detail through optical reflective technology. We sought to evaluate whether its use was technically feasible in pediatric PVS and whether the imaging data provided potentially useful outputs for clinical utility. Eleven patients were prospectively selected from our cardiac catheterization for OCT evaluation of their pulmonary veins (PV) during elective catheterization for PVS. Measurements were taken both pre and post intervention using both manual and automated tools. Stent morphology was characterized. Eleven patients had evaluation of 34 pulmonary veins, with 7 patients having more than one assessment, for a total of 25 overall catheterizations. Most patients were female (75%). Median age at cardiac catheterization was 35 months (range 5-45 months). Median weight of subjects was 10.6 kg (3.7-14.2) with a median BSA documented at 0.505 m2 (0.21-0.57). Median number of pulmonary veins involved was 3, (range 1-5 veins) and median contrast volume of 2.9 mL/kg (0.7-3.7) given. Median radiation dose (DAP) was 6095 µGy·cm2 (1670-12,400). Median number of previous cardiac catheterizations was 7 (range 1-11). All of the vessels with a diameter < 5 mm were adequately visualized. Of all the OCT images acquired, in 15 vessels (44%) contrast was used to clear the vessels from blood as an angiogram was required at the time, in the other 19 vessels (56%), saline was used with adequate imaging. There were no complications related to OCT. OCT is technically feasible to use in pediatric patients without any directly related complications. It provides intraluminal anatomy in children with both native and treated pulmonary venous stenosis when vessel size is less than 5 mm.

Validating a risk assessment tool in United Kingdom and Irish paediatric cardiac catheterisation practice.

BACKGROUND: No established risk prediction tool exists in United Kingdom and Irish Paediatric Cardiology practice for patients undergoing cardiac catheterisation. The Catheterisation RISk score for Paediatrics is used primarily in North American practice to assess risk prior to cardiac catheterisation. Validating the utility and transferability of such a tool in practice provides the opportunity to employ an already established risk assessment tool in everyday practice. AIMS: To ascertain whether the Catheterisation RISk score for Paediatrics assessment tool can accurately predict complications within United Kingdom and Irish congenital catheterisation practice. METHODS: Clinical and procedural data including National Institute for Cardiovascular Outcomes Research derived outcome data from 1500 patients across five large congenital cardiology centres in the United Kingdom and Ireland were retrospectively collected. Catheterisation RISk score for Paediatrics were then calculated for each case and compared with the observed procedural outcomes. Chi-square analysis was used to determine the relationship between observed and predicted events. RESULTS: Ninety-eight (6.6%) patients in this study experienced a significant complication as qualified by National Institute for Cardiovascular Outcomes Research classification. 4% experienced a moderate complication, 2.3% experienced a major complication and 0.3% experienced a catastrophic complication resulting in death. Calculated Catheterisation RISk score for Paediatrics scores correlated well with all observed adverse events for paediatric patients across all CRISP categories. The association was also transferable to adult congenital heart disease patients in lower Catheterisation RISk score for Paediatrics categories (CRISP 1-3). CONCLUSION: The Catheterisation RISk score for Paediatrics score accurately predicts significant complications in congenital catheterisation practice in the United Kingdom and Ireland. Our data validated the Catheterisation RISk score for Paediatrics assessment tool in five congenital centres using National Institute for Cardiovascular Outcomes Research-derived outcome data.

Contrast-free percutaneous pulmonary valve replacement: a safe approach for valve-in-valve procedures.

INTRODUCTION: Percutaneous pulmonary valve replacement (PPVI) continues to gather pace in pediatric and adult congenital practice. This is fueled by an expanding repertoire of devices, techniques and equipment to suit the heterogenous anatomical landscape of patients with lesions of the right ventricular outflow tract (RVOT). Contrast-induced nephropathy is a real risk for teenagers and adults with congenital heart disease (CHD). AIM: To present a series of patients who underwent PPVI without formal RVOT angiography and propose case selection criteria for patients who may safely benefit from this approach. MATERIAL AND METHODS: We retrospectively collected PPVI data from the preceding 2 years at our institution identifying patients who had been listed as suitable for consideration for contrast-free PPVI from our multidisciplinary team (MDT) meeting based on predefined criteria. Demographic, clinical, imaging and hemodynamic data were collected. Data were analyzed using SPSS. RESULTS: Twenty-one patients were identified. All patients had a technically successful implantation with improvements seen in invasive and echocardiographic hemodynamic measurements. 90% of patients had a bio-prosthetic valve (BPV) in situ prior to PPVI. One patient had a complication which may have been recognized earlier with post-intervention RVOT contrast injection. CONCLUSIONS: Zero-contrast PPVI is technically feasible and the suitability criteria for those who might benefit are potentially straightforward. The advent of fusion and 3D imaging in cardiac catheterization laboratories is likely to expand our capacity to perform more procedures with less contrast. Patients with bio-prosthetic valves in the pulmonary position may benefit from contrast-free percutaneous pulmonary valve implantation.

Stent implantation to relieve native obstructed left partial anomalous pulmonary venous connections.

Obstructed partial anomalous pulmonary venous connections (APVC) are rare but may be associated with severe pulmonary hypertension (PHTN) and warrant urgent relief. There are a number of case reports of successful catheter intervention for obstructed total APVC. We present the first reported case of catheter intervention to relieve obstructed, left sided PAPVC in a neonate with Turner syndrome. © 2016 Wiley Periodicals, Inc.