Intimal thickening at coronary bifurcations in pediatric heart transplant recipients.

Heart transplant recipients are at increased risk for atherosclerosis and cardiac allograft vasculopathy, both initially presenting as intimal thickening. We aimed to determine the presence, extent, and anatomical characteristics of intimal thickness at coronary bifurcations in children using OCT. We measured the intimal thickness of coronary arteries in pediatric transplant recipients using OCT during routine cardiac catheterization. Intimal thickening was defined as (i) a percent change in contralateral intimal thickness greater than 50% when comparing the thickness at the bifurcation to the baseline thickness, and (ii) greater than 0.1 mm. We evaluated 153 unique coronary bifurcations in 31 children (58% boys, median 12.7 years). Intimal thickening was almost exclusively observed in the left coronary system (22 of 67 bifurcations) and rare in the right coronary system (2 of 86 bifurcations; P < .001). There was a positive association between the relative size of the side branch and contralateral intimal thickening at coronary bifurcations (P = .009). Intimal thickening at coronary bifurcations is already present in the left coronary system in many pediatric transplant recipients. The correlation between intimal thickening and side branch size suggests that low shear stress and oscillating shear stress may have an important role in the development of intimal thickening at coronary bifurcations.

Outcomes of Radiofrequency Perforation for Pulmonary Atresia and Intact Ventricular Septum: A Single-Centre Experience.

Percutaneous radiofrequency perforation (RFP) of the pulmonary valve is used as a primary therapy in neonates with pulmonary atresia and intact ventricular septum (PAIVS). We sought to determine the safety and efficacy of RFP for PAIVS in a single center and assess the pre-intervention anatomical parameters associated with a biventricular outcome. We retrospectively reviewed all cases of PAIVS treated with RFP at a single center from 1999 through 2012. We collected baseline imaging data, technical aspects of the procedure, adverse events and outcomes. RFP was attempted in 18 patients with 17 successful procedures. There was no mortality; one patient had an acute complication requiring surgical intervention. All were alive at the most recent follow-up (median 4.9 years; IQR = 2.0-6.8 years), 12/17 (71%) had a biventricular circulation, 2/17 (12%) had a 1½ ventricle repair, 2/17 (12%) had a univentricular repair and 1/17 was lost to follow-up. A biventricular outcome in patients with PAIVS was associated with the pre-intervention tricuspid valve/mitral valve (TV/MV) ratio and tricuspid valve (TV) z-score. The median TV/MV ratio for patients who underwent a biventricular repair and a non-biventricular repair was 0.82 (IQR = 0.71-0.90) and 0.59 (IQR = 0.39-0.76), P = 0.036, respectively. The median TV z-scores were -3.2 [(-4.9 to -2.6), and -6.8 (-9.7 to -4.8] P = 0.036 for the biventricular and non-biventricular groups, respectively. RFP is a safe primary therapy for PAIVS. With appropriate patient selection, RFP will often result in a biventricular circulation. Both the TV/MV and TV z-score were found to be a predictor of a biventricular outcome in our cohort.

Patterns of Early Coronary Artery Changes in Pediatric Heart Transplant Recipients Detected Using Optical Coherence Tomography.

BACKGROUND: Cardiac allograft vasculopathy, the leading cause of graft failure in pediatric heart transplant recipients, is characterized by diffuse and concentric coronary intimal thickening. Early treatment yields better outcomes. While coronary angiography is the standard for cardiac allograft vasculopathy screening and diagnosis, it only identifies luminal narrowing, which occurs in more severe disease. Coronary optical coherence tomography (OCT) is a high-definition intravascular imaging modality that may offer earlier diagnosis. We used OCT to investigate coronary intimal thickening in pediatric transplant recipients and examined its (1) location (ie, vessel type and location) and (2) nature (ie, characteristics of cross-sectional and longitudinal thickening). METHODS: Sites collected coronary angiography and OCT data from participants (N=258 vessel segments from 73 individuals; median age: 11.5 years [8.4-15.3]; 55% male). Images were collected from the left anterior descending, left circumflex, and right coronary arteries, and location (ie, proximal, middle, and distal) were classified using coronary angiography. RESULTS: OCT identified 32 vessel segments meeting criteria for significant thickening, 88% of which were angiographically silent. Longitudinal thickening was segmental rather than global in 88%, and cross-sectional thickening was 48% eccentric and 52% concentric. Intimal thickening prevalence and severity measures did not consistently differ between coronary artery type (P=1.000) or location (P=0.248) but increased with time since transplant and age at transplant and OCT procedure. CONCLUSIONS: In pediatric transplant recipients, we observed a surprisingly high prevalence of segmental and eccentric intimal thickening. Insights from intravascular imaging suggest these patterns of coronary vascular changes may precede overt cardiac allograft vasculopathy. Identifying early changes may offer opportunity for enhanced surveillance and earlier intervention.

Acute respiratory distress secondary to severe compression of the left main bronchus by the ductus arteriosus.

Two newborn infants presented with acute respiratory distress. In both cases, the left lung was opaque, hyperinflated, and associated with a rightward shift of the mediastinum. A diagnosis of retained fetal fluid secondary to vascular compression of the left bronchus by the ductus arteriosus was made by combining various imaging methods including chest radiograph, computed tomography (CT), and echocardiography. Although the initial chest radiographs were similar, the mechanisms of obstruction were different. The imaging emphasizes the importance of CT angiography to understanding the three-dimensional relationships resulting in bronchial compression.

Optical Coherence Tomography for the Early Detection of Coronary Vascular Changes in Children and Adolescents After Cardiac Transplantation: Findings From the International Pediatric OCT Registry.

OBJECTIVES: This study sought to describe the initial findings from the International Pediatric Optical Coherence Tomography (OCT) registry in pediatric heart transplant recipients. BACKGROUND: Cardiac allograft vasculopathy (CAV) is a common cause of late graft failure and mortality in pediatric heart transplant recipients. Early diagnosis may improve outcomes. OCT is a high-resolution intravascular imaging technique that has the potential to identify CAV earlier than angiography. METHODS: OCT and angiography of the coronary arteries were performed in pediatric heart transplant recipients at participating centers. Demographics, clinical data, medications, episodes of rejection, and angiographically confirmed CAV were collected for each case. OCT and angiography images were analyzed in a central core imaging laboratory. Intimal thickness and intima/media cross sectional area (I/M CSA) ratios were calculated for each case. Intimal thickness ≥0.25 mm was defined as abnormal and ≥0.4 mm as severe intima thickening. I/M CSA ratio of ≥1 was defined as abnormal. OCT findings were compared to angiographic findings for each case. RESULTS: Across 3 centers, 110 cases were analyzed from 76 patients. Intimal thickening was present in 26 of 110 cases. Eleven of these cases had severe intima thickening (≥0.4 mm) and notably, angiography results were normal in 8 cases. All 5 cases with a median I/M CSA ratio of ≥2 had normal angiography. The maximal intima thickness was ≥0.25 mm in 24% and ≥0.4 mm in 10% of cases. Median I/M CSA ratio was ≥1 for 80% of cases. I/M CSA ratio was significantly higher in cases with concurrent CAV (p = 0.03). Maximal intima thickness was significantly greater in cases with current or previous rejection (p = 0.01). I/M CSA ratio was significantly lower in patients treated with statins (p = 0.01). OCT findings alone prompted a change to medical management in 17% of cases. CONCLUSIONS: OCT provides important insights into coronary vascular changes not detected by angiography in pediatric transplant recipients. The use of OCT for pediatric heart transplant recipients should be further investigated, given its potential to impact the management of CAV.

Physiological Responses to Exercise in Pediatric Heart Transplant Recipients.

INTRODUCTION: Pediatric heart transplant (HTx) recipients have reduced exercise capacity typically two-thirds of predicted values, the mechanisms of which are not fully understood. We sought to assess the cardiorespiratory responses to progressive exercise in HTx relative to controls matched for age, sex, body size, and work rate. METHODS: Fourteen HTx recipients and matched controls underwent exercise stress echocardiography on a semisupine cycle ergometer. Hemodynamics, left ventricular (LV) dimensions, and volumes were obtained and indexed to body surface area. Oxygen consumption (V˙O2) was measured, and arteriovenous oxygen difference was estimated using the Fick Principle. RESULTS: At rest, LV mass index (P = 0.03) and volumes (P < 0.001) were significantly smaller in HTx, whereas wall thickness (P < 0.01) and LV mass-to-volume ratio (P = 0.01) were greater. Differences in LV dimensions and stroke volume persisted throughout exercise, but the pattern of response was similar between groups as HR increased. As exercise progressed, heart rate and cardiac index increased to a lesser extent in HTx. Despite this, V˙O2 was similar (P = 0.82) at equivalent work rates as HTx had a greater change in arteriovenous oxygen difference (P < 0.01). CONCLUSIONS: When matched for work rate, HTx had similar metabolic responses to controls despite having smaller LV chambers and an attenuated increase in hemodynamic responses. These findings suggest that HTx may increase peripheral O2 extraction as a compensatory mechanism in response to reduced cardiovascular function.

A Systematic Review of Infective Endocarditis in Patients With Bovine Jugular Vein Valves Compared With Other Valve Types.

OBJECTIVES: The aim of this study was to systematically evaluate the incidence of infective endocarditis (IE) in right ventricle-to-pulmonary artery conduits and valves, comparing bovine jugular vein (BJV) valves with all others. BACKGROUND: Recent evidence suggests that the incidence of IE is higher in patients with congenital heart disease who have undergone implantation of BJV valves in the pulmonary position compared with other valves. METHODS: Systematic searches of published research were conducted using electronic databases (MEDLINE, Embase, and CINAHL) and citations cross-referenced current to April 2016. Included studies met the following criteria: patients had undergone right ventricle-to-pulmonary artery conduit or percutaneous pulmonary valve implantation, and investigators reported on the type of conduit or valve implanted, method of intervention (surgery or catheter based), IE incidence, and follow-up time. RESULTS: Fifty studies (Levels of Evidence: 2 to 4) were identified involving 7,063 patients. The median cumulative incidence of IE was higher for BJV compared with other valves (5.4% vs. 1.2%; p < 0.0001) during a median follow-up period of 24.0 and 35.5 months, respectively (p = 0.03). For patients with BJV valves, the incidence of IE was not different between surgical and catheter-based valve implantation (p = 0.83). CONCLUSIONS: There was a higher incidence of endocarditis with BJV valves than other types of right ventricle-to-pulmonary artery conduits. There was no difference in the incidence of endocarditis between catheter-based bovine valves and surgically implanted bovine valves, suggesting that the substrate for future infection is related to the tissue rather than the method of implantation.

Feasibility of optical coherence tomography in children with Kawasaki disease and pediatric heart transplant recipients.

BACKGROUND: Optical coherence tomography (OCT) is a high-resolution intravascular imaging technique used in adults. We tested the hypothesis that OCT could identify coronary abnormalities not seen by angiography in children with a history of Kawasaki disease (KD) and pediatric heart transplant (TX) recipients. METHODS AND RESULTS: Patients with KD and TX recipients were evaluated between December 2012 and October 2013 with angiography and OCT (Ilumien System, LightLabs, St Jude Medical, Westford, MA). Modifications were made to the adult OCT protocol to adapt this technique for children. Serial cross-sectional area measurements of the lumen, intima, and media were made. Entire imaging data were analyzed for the presence of qualitative changes. Seventeen children were evaluated (5 patients with KD; 12 TX recipients). In patients with KD, angiography was normal. However, OCT imaging revealed that significant vessel wall abnormalities were present in all children including intimal thickening (intima/lumen cross-sectional area ratio>0.4), loss of the normal layered structure of the vessel wall, white thrombus, calcification, and neovascularization. There was extensive destruction of the internal elastic lamina. In TX recipients, angiography was normal; however, intimal thickening (intima/media cross-sectional area ratio>1) was seen in 9 of 12 patients. The median intima/media cross-sectional area ratio was 1.18. CONCLUSIONS: In this initial experience with OCT in children, we have identified significant coronary abnormalities with OCT that are angiographically silent in children with a history of coronary aneurysms because of KD and in pediatric TX recipients.

Tetralogy of Fallot with absent pulmonary valve and obstructed totally anomalous pulmonary venous connection.

We describe a combination, unique as far as we are aware, of tetralogy of Fallot with so-called "absent" pulmonary valve and supracardiac totally anomalous pulmonary venous connection. The vertical vein was obstructed in a neonate born at term who presented with respiratory compromise.

Congenital right atrial diverticulum, atrial septal defect within the oval fossa, and complex pulmonary valvar obstruction in an infant with chromosome 8(p23.1) deletion.

We describe an unusual right atrial aneurysm, occurring with progressive obstruction of the right ventricular outflow tract and increasing cyanosis, in a nine-month-old boy with chromosome 8(p23.1) deletion. Surgical resection of the diverticulum, and relief of the right ventricular obstruction, was successful, although impairment of right ventricular compliance persists after 19 months follow-up.