Pulmonary vascular disease in children with truncus arteriosus (original) (raw)
Related papers
Paediatric pulmonary vascular disease
Paediatric Respiratory Reviews, 2004
BACKGROUND The pulmonary circulation is a low-pressure, high-flow system with a great capacity for recruitment of normally non-perfused vessels. As a consequence, the walls of pulmonary arteries are thin, in keeping with their low transmural pressure. Pulmonary vascular disease (PVD) covers any congenital or acquired pathology of the intrinsic pulmonary vessels with the unique feature of the pulmonary arteries carrying unsaturated blood and the pulmonary veins carrying oxygenated blood. During the second half of gestation, the absolute pulmonary blood flow increases as the lungs grow. 1 This is accompanied by a 10-fold increase in the number of pulmonary vessels between mid gestation and term. However, the blood flow per gram of lung tissue remains relatively constant. Thus, the increase in pulmonary arterial pressure towards term is due to a gradual increase in
Journal of Ultrasound in Medicine, 2004
truncus arteriosus is a solitary great artery arising from the base of the heart and supplying the systemic, pulmonary, and coronary circulation. A truncus arteriosus is a rare cardiac defect, accounting for less than 1% of structural heart defects. 1 The single great artery usually arises above a malalignment ventricular septal defect (VSD) and straddles the ventricular septum. There is variability in the origins of the pulmonary branch and whether the aortic arch is interrupted. Van Praagh and Van Praagh 1 suggested classifying trunci arteriosi with VSD into 4 types. In type I, a main pulmonary artery arises from the truncal root and bifurcates into the branch pulmonary arteries. In type II, the branch pulmonary arteries are separate from the truncal root. In type III, the left pulmonary artery is supplied by a collateral from the aortic arch and does not arise from the truncal root. In type IV, the aortic arch is interrupted. A truncus arteriosus may rarely occur without a VSD. Although prenatal diagnosis of a truncus arteriosus has been reported several times, 2-4 to our knowledge, the rare variant with an atretic main pulmonary trunk and normal pulmonary circulation such as the case presented here has not been described prenatally. The objective of this report was to show abnormal fetal circulation associated with a truncus arteriosus with pulmonary atresia but with well-developed pulmonary circulation derived from retrograde flow through the ductus arteriosus.
8th International Conference on Neonatal and Childhood Pulmonary Vascular Disease
Pulmonary Circulation, 2015
Real-time three-dimensional echocardiography (RT3DE) evaluation of the right ventricle (RV) decreases geometric assumptions from conventional echocardiography. RV strain has been demonstrated to be more sensitive in evaluation of RV function than conventional echocardiography. TomTec 4D RV-Function 2 software is vendor neutral and can generate RV size and functional parameters. We compared 4D RV-Function 2 data between controls and pediatric pulmonary hypertension (PH) patients. We also compared tricuspid annular plane excursion (TAPSE) and fractional area change (FAC) measured by 4D RV-Function 2 and by traditional two-dimensional echocardiography. We enrolled 145 children with PH with 161 visits (mean age: 11.5 ± 6.9 years; 47% male) and 13 controls (mean age: 8.6 ± 2.5 years; 66% male) who underwent RT3DE evaluation of the RV in 2014-2015. Offline analysis of the 4D RV-Function 2 data of these patients generated RV indices: 3D ejection fraction (EF), RV septal and free-wall longitudinal strain (RVLS), TAPSE, and FAC. Differences in RV indices between PH patients and controls were assessed with linear mixed models, adjusting for age and accounting for repeated measures. A t test was performed on the TAPSE and FAC from traditional two-dimensional echocardiography and compared to the TAPSE and FAC generated from the 4D RV-Function 2 software in pediatric PH patients. TAPSE is measured in 4D RV-Function 2 by taking the difference between the length from the apex to the tricuspid valve annulus in systole and that in diastole. Intraclass correlations (ICCs) between two users were tested. There were significant differences in RV indices between PH patients and controls, with the exception of TAPSE. There were also significant differences in TAPSE and FAC between the two-dimensional echocardiography and 4D RV-Function 2. The ICCs showed good agreement for EF and RV free-wall longitudinal strain (0.92 and 0.82, respectively). The ICCs showed moderate agreement in RV septal longitudinal strain, TASPE, and FAC (0.62, 0.66, and 0.64, respectively). Pediatric PH patients have impaired RV function compared to normal children. The 4D RV-Function 2 software is feasible and generates RV functional parameters from one RT3DE acquisition, making it easier for clinical use. Differences between the values of TAPSE and FAC from the two methods will need to be evaluated further to define the normal values gen
Pictorial review of the pulmonary vasculature: from arteries to veins
Insights into Imaging, 2018
Pathology of the pulmonary vasculature involves an impressive array of both congenital and acquired conditions. While some of these disorders are benign, disruption of the pulmonary vasculature is often incompatible with life, making these conditions critical to identify on imaging. Many reviews of pulmonary vascular pathology approach the pulmonary arteries, pulmonary veins and bronchial arteries as individual topics. The goal of this review is to provide an integrated overview of the high-yield features of all major disorders of the pulmonary vasculature. This approach provides a more cohesive and comprehensive conceptualisation of respiratory pathology. In this review, we present both the salient clinical and imaging features of congenital and acquired disorders of the pulmonary vasculature, to assist the radiologist in identifying pathology and forming a robust differential diagnosis tailored to the presenting patient. Teaching Points • Abnormalities of the pulmonary vasculature are both congenital and acquired. • Pathology of a single pulmonary vascular territory often affects the entire pulmonary vasculature. • Anomalous pulmonary venous flow is named as a function of its location and severity. • Bronchial arteries often undergo dilatation secondary to cardio-respiratory pathology.
Diameters of pulmonary veins in normal children—an angiocardiographic study
1989
The diameters of the pulmonary veins at their atrial junction were measured from cineangiocardiographic films in 27 children (mean height 128.5 cm) without heart disease. Right upper pulmonary veins and right lower pulmonary veins were measured in all 27 films. The left upper pulmonary veins could be measured in 23 children (mean height 127.0 cm), and the left lower pulmonary veins only in 15 (mean height 135.0 cm) because their atrial junctions were obscured by other structures. Measurements were obtained in frorrtal projection of levophase of pulmonary trunk angiograms. Calipers and a micrometer were used and the values were expressed to the nearest 0.5 mm. The measured size of the intracardiac catheter was used for calibration. The diameter (in mm) for right upper pulmonary veins was 0.08 x height (in cm) + 1.4 (r = 0.96); for right lower pulmonary veins, 0.07 x height + 2.1 (r = 0.94); for left upper pulmonary veins, 0.06 x height + 3.1 (r = 0.87); and for left lower pulmonary veins it was the same as for left upper, but with r of 0.94.
The Turkish journal of pediatrics, 2016
Congenital pulmonary vascular abnormalities arise from several etiologies. These anomalies are difficult to categorize and sorted into distinct classifications. Major pulmonary vascular abnormalities can be ranked as interruption of the main pulmonary artery or its absence, emergence of the left pulmonary artery in the right pulmonary artery, pulmonary venous drainage abnormalities, and pulmonary arteriovenous malformations (PAVMs). Some of the cases are asymptomatic and diagnosed by coincidence, whereas a few of them are diagnosed by typical findings in the newborn and infancy period, symptoms, and radiological appearances. Early diagnosis is important, since death may occur as a result of pulmonary and cardiac pathologies developed in patients with pulmonary vascular anomalies. In this case presentation, the clinical and radiological findings of patients that presented with different complaints and were diagnosed with pulmonary vascular anomalies were introduced.
Anomalous Origin of the Left Pulmonary Artery: Hemi-Truncus Arteriosus
APSP journal of case reports
Hemi-truncus arteriosus is a rare congenital cardiovascular malformation. It usually presents in infancy and leads to development of progressive pulmonary vascular disease, heart failure, and death. We report a case of hemi truncus arteriosus in a 12-day-old neonate who was successfully managed at our institute.
CHEST Journal, 1990
http://chestjournal.chestpubs.org/site/misc/reprints.xhtml Communications to the Editor accurate and reproducible. We cannot explain the discrepant hemodynamic measurements obtained in the one patient reported in the present letter which contradicts our findings, except to say that hemodynamic measurements may lag behind changes in the respiratory cycle during CSB. Receive free e-mail alerts when new articles cite this article. To sign up, select the "Services" link to Citation Alerts slide format. See any online figure for directions. articles can be downloaded for teaching purposes in PowerPoint CHEST Figures that appear in Images in PowerPoint format