Common pulmonary vein atresia without anomalous pulmonary venous connection

Common pulmonary vein atresia without pulmonary venous connection is a rare form of congenital heart disease. No communication exists between the confluence of the pulmonary veins and the heart or a major systemic vessel. A case diagnosed antemortem is presented. Correct early diagnosis is imperative as surgery may be corrective.     

Correlation Between Peripheral and Central Venous Pressures in Children with Congenital Heart Disease

Central venous pressure (CVP) measurement is a reliable method for evaluating intravascular volume status and cardiac function, but it is an invasive method that results in some complications such as arterial puncture, pneumothorax, and development of infection. The current study was performed to compare CVP measurements between central and peripheral catheters in infants and children with congenital heart disease referred for right-sided heart catheterization. The CVP and peripheral venous pressure (PVP) in 45 patients were measured simultaneously. The mean difference between CVPs measured from the central and peripheral catheters was 8 ± 4 cm H₂O. The linear regression equation showed that CVP = 0.32 PVP + 3.8 (r = 0.67; p < 0.005). There was no difference in CVP measurements depending on the intravenous cannula and chest diameters, arm diameter, arm length, body surface area, patient’s age (≤10 years and >10 years), and type of congenital heart disease (cyanotic or noncyanotic). In conclusion, although CVP measured from a peripheral intravenous catheter in infants and children with congenital heart disease is not as accurate as the measurement in adults, the aforementioned linear regression equation based on measurement of PVP gives a reliable estimate of CVP.     

Low-dose warfarin for the prevention of central line-associated thromboses in children with malignancies--a randomized, controlled study

Background: Central venous lines (CVLs) are essential in the care of children with malignancies, but are associated with venous thromboembolism (VTE) and infections. Effective and safe prophylactic approaches are deficient. Aim: To perform a study of adjusted low-dose warfarin for the prevention of CVL-related VTE in children with malignancies. Methods: Children with newly diagnosed cancer, a CVL in a jugular vein and an expected treatment period of over 6 mo were eligible for the study. Participants were randomized to low-dose warfarin, with intended international normalized ratio (INR) 1.3-1.9, or to a control group. Primary outcome was VTE in a jugular vein diagnosed by ultrasonography at 1, 3 and 6 mo after inclusion. Secondary outcome was CVL-related infections, mainly measured as days on antibiotics or positive blood cultures. Results: The study enrolled 73 children, and 62 completed it fully. Asymptomatic CVL-related VTE was frequent (42%), but often transient. Regardless of severity, timing and duration, CVL-related VTE was equally frequent among children on warfarin as compared to controls (p=0.44). Low-dose warfarin (p=0.59) or jugular CVL-related VTE (p=0.91) did not have any impact on days on antibiotics, but we observed a tendency towards an association between CVL-related VTE and positive blood cultures (p=0.15).

Conclusion: Our randomized study of low-dose oral anticoagulation for the prevention of CVL-related asymptomatic VTE in children with cancer did not show any benefit of warfarin adjusted to maintain INR between 1.3 and 1.9.     

Late pulmonary venous obstruction after surgical repair of infradiaphragmatic total anomalous pulmonary venous return

Summary Twelve years after an apparently successful surgical correction of infradiaphragmatic (obstructed) total anomalous pulmonary venous drainage, a 12-year-old boy developed evidence of pulmonary artery hypertension secondary to pulmonary venous obstruction due to an apparent lack of growth at the anastomotic site.     

Congenital unilateral pulmonary venous atresia with pulmonary veno-occlusive disease in contralateral lung: An unusual association

Summary A patient presenting with the rare association of congenital unilateral pulmonary venous atresia in one lung and pulmonary veno-occlusive disease in the other is described. The patient first presented at the age of 3 &frac; years with anemia, hemoptysis, and pulmonary hypertension. After cardiac catheterization and angiocardiography, a diagnosis of atresia of the left pulmonary veins was made for which left pneumonectomy was done. Four years later the patient presented with right-sided congestive failure and radiologic evidence of right-sided pulmonary edema from which death resulted. At autopsy, the right lung showed changes of pulmonary veno-occlusive disease, while the major veins were not involved.     

Atypical total anomalous pulmonary venous connection: Two channels leading to infracardiac terminations

Summary Reported is a rare case of total anomalous pulmonary venous connection (TAPVC) where veins from each lung joined a homolateral confluence. From each confluence, a vein descended into the abdomen, the vein from the right lung joining the ductus venosus, while the vein from the left joined the portal vein.In TAPVC to systemic veins, multiple connections are rare. Multiple connections are most common at supracardiac and cardiac levels, less common at supra- and infracardiac levels, and rare at cardiac and infracardiac levels.From the literature, it is evident that multiple connections at one body level, as in our case, are rare.     

Unusual complications of central venous catheters in two infants with congenital venous anomalies associated with congenital heart disease

We report two infants with congenital heart disease who had unusual complications of indwelling central venous catheters related to anomalies of systemic and pulmonary venous drainage. Correspondence to: B. J. Pettitt     

Venous patency after open central-venous cannulation

To investigate the value of Doppler ultrasound scan (USS) assessment of internal jugular vein (IJV) patency after previous open central-venous cannulation (CVC), a prospective study of 66 consecutive children (median age 4.5 years; range 4 months–17 years) who had previously undergone open insertion of at least one indwelling IJV line and required further CVC for completion of therapy was undertaken. All underwent Doppler USS examination prior to surgery. Where patency of the previously cannulated vein was suggested ultrasonographically, the accuracy of this finding was confined at open surgical exploration. Initial CVCs were in situ for a median of 9 months (1 month–4 years) prior to removal. The median interval to repeated CVC was 11 months (3 weeks–45 months). In 79 Doppler USS, 70 (88.6%) veins appeared patent, 3 (4.2%) stenosed, and 6 (7.6%) obliterated. Of the 70 “USS patent” veins, 66 were explored. Patency was confirmed surgically in 59 (89.4%) and a new CVC successfully inserted. Seven (10.6%) apparently patent veins on USS were found to be obliterated at open exploration. Review of USS images in these cases suggested that enlarged collateral veins were usually responsible. Overall, successful recannulation was possible in 74.6% of all previously accessed veins. In children requiring repeated CVC, Doppler USS of neck veins is a valuable but not entirely reliable guide to the presence of underlying vessel patency and should be interpreted with caution. At least three-fourths of previously cannulated IJVs remain patent after catheter removal and can be reused for CVC. Accepted: 30 November 1998     

Biatrial or Left Atrial Drainage of the Right Superior Vena Cava: Anatomic,Morphogenetic, and Surgical Considerations Report of Three New Cases and Literature Review

Since the posterior wall of the right superior vena cava (RSVC) is contiguous with the anterior wall of the right upper pulmonary veins, a localized defect in this common wall may create a cavopulmonary venous confluence without eliminating the normal connection of the same right pulmonary veins with the left atrium (LA). Through this defect, blood of the unroofed right pulmonary veins will drain into the RSVC and right atrium (RA), and blood from the RSVC may shunt into the right pulmonary veins and LA. Hemodynamically, the RSVC will become biatrial. If the RSVC blood flows preferentially into the LA, its right atrial orifice will become stenotic or even atretic. If atretic, the normally positioned RSVC will drain entirely into the LA. In this report, we present the clinical and anatomical findings of two postmortem cases with biatrial drainage of the RSVC. We also document the clinical, echocardiographic, angiocardiographic, and surgical data of a living patient with left atrial drainage of the RSVC and tetralogy of Fallot with pulmonary atresia. The relevant literature and surgical treatment are reviewed, and the morphogenesis of the biatrial and left atrial RSVC is considered.     

Radiographic inguinal curl may indicate paraspinal misplacement of percutaneously inserted central venous catheters: report of three cases

Background: Misplacement of percutaneously inserted central venous catheters (PCVCs) into the paraspinal venous plexus can result in devastating outcomes. Several cases have been reported in the literature together with an explanation of the mechanism. Objective: To describe three premature babies with their PCVCs inserted through the left saphenous vein that ended up in the lumbar spinal dural venous plexus. Results: Plain radiographs obtained to check positions showed an unusual 360° curl of the PCVC in the left inguinal area. Conclusion: We believe that misplacement of the catheter into the paraspinal venous plexus could be diagnosed with great accuracy if such a curl is seen.     

The bowed catheter sign: a risk for pericardial tamponade

The use of a central venous catheter (CVC) has become commonplace in the care of children with a wide variety of medical and surgical problems. Complications resulting from the insertion of these catheters are well recognized and can be life-threatening. When a temporary CVC or other catheter is inserted into the central venous system it is secured to the skin with a combination of sutures and sterile dressing. This fixes the catheter in place and does not allow it to retract, thereby putting pressure on the right atrial wall via the catheter tip if it is too long. The probability of wall penetration is increased if a catheter or device is tapered at the point of contact. The purpose of this case report is to present the bowed catheter sign and to review the anatomy of the cavotricuspid isthmus, a possible predisposing factor to cardiac perforation and tamponade.     

Venous thromboembolism

Incidence of venous thromboembolism (VTE) in children is rapidly rising. Frequent use of central venous lines (CVLs) in children with a variety of disease processes has contributed to increased incidence of VTE. In addition, increased detection of VTE has been possible due to the availability of sensitive imaging studies such as Color Duplex/Doppler ultrasound, lineogram and venogram. Heightened awareness of congenital prethrombotic disorders may help identify children at higher risk of thrombosis. Anticoagulation with heparin, low molecular weight heparin (LMWH) and oral anticoagulant such as warfarin are used for the treatment of VTE. Thrombolytic therapy may be indicated in some cases. Clinical presentation, management and pathogenesis including role of congenital prethrombotic disorders in pediatric VTE are discussed.     

Incidence and characteristics of venous thrombotic events in pediatric cancer patients: A 20-year experience in the Maritimes,Canada

Objective: Venous thrombotic events (VTE) are a well-recognized complication in pediatric cancer patients. Population-based data on the incidence and characteristics of VTE in all pediatric cancer patients are limited. This information is crucial to identify patients at high risk and design targeted interventions accordingly. The present study was designed to determine the incidence and characteristics of VTE in the pediatric oncology population. Participants: We conducted a retrospective, population-based, cohort study of patients treated in the Maritimes, Canada between 1995 and 2015. Results: There were 1210 pediatric hematology/oncology patients from the Maritimes, Canada, treated at the IWK Health Centre between 1995 and 2015. Fifty-eight (4.8%) experienced at least 1 VTE and the majority of patients experienced it within 6 months of cancer diagnosis. The median age of patients who experienced VTE was 10.7 years (SD = 6.0). The most common presenting symptom of thrombosis was central venous line dysfunction, and the most common location for thrombosis was within the upper venous system. We observed that 65.6% of the patients with VTE required >1 central venous catheters (CVC). The presence of a VTE increased the odds of requiring >1 CVC to 3.6 (95% confidence interval: 1.76–7.3). Conclusion: Thus, in this large, population-based study, we present the incidence and characteristics of VTE in the pediatric oncology population and demonstrate the clinical impact of VTE in terms of loss of CVC. Larger, prospective studies are required to confirm these findings and to develop a risk model for managing and preventing VTE in this patient population.     

Obstructed total anomalous pulmonary venous connection

Summary With the advent of echocardiography, total anomalous pulmonary venous connection (TAPVC) can be readily diagnosed without much difficulty. However, noninvasive detection of the presence of pulmonary venous obstruction in TAPVC remains a difficult issue. During a 5.5-year period, 42 patients were found to have TAPVC by catheterization, surgery, and/or autopsy: 17 had supracardiac drainage, 13 paracardiac drainage, nine infracardiac drain-age, and three mixed drainage. Obstruction to pulmonary venous drainage was found in 24 patients (57%). Patients with right isomerism tended to have a higher incidence of pulmonary venous obstruction than those with the usual atrial arrangement (80% vs. 44%,p<0.05). Color Doppler combined with cross-sectional echocardiography provided accurate delineation of drainage sites in 93% cases (39 of 42). Among the 39 cases with correct echocardiographic delineation of the drainage site, obstruction was detected by echocardiography in 22 cases with a sensitivity of 100% (22 of 22) and a specificity of 85% (17 of 20). Therefore, complete echocardiography, including cross-sectional images and color Doppler proved to be a reliable tool in the detection of drainage sites and pulmonary venous obstruction in TAPVC.     

A novel mutation in the SerpinC1 gene presenting as unprovoked neonatal cerebral sinus venous thrombosis in a kindred

Antithrombin (AT) deficiency has been associated with an increased risk of pediatric cerebral sinus venous thrombosis (CSVT); but few cases of neonatal CSVT have been reported. We describe two half‐siblings who presented with seizures in the first week of life and were found to have extensive CSVT with associated parenchymal and intraventricular hemorrhage. Both infants were found to have type 1 AT deficiency. Sequencing of the SerpinC1 gene revealed a novel heterozygous mutation on exon 5 (c.1009C > T p.Q337X). Both infants were treated with anticoagulation and had recanalization of the dural sinuses on follow up imaging. Pediatr Blood Cancer 2013; 60: 133–136. © 2012 Wiley Periodicals, Inc.