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Cardiovascular system
Published in Jagdish M. Gupta, John Beveridge, MCQs in Paediatrics, 2020
Jagdish M. Gupta, John Beveridge
8.28. Which of the following statements is/are true regarding Williams' syndrome?Supravulvar aortic stenosis is usually progressive.Peripheral pulmonary artery stenosis is a well-recognized association.Distal arch anomalies are more common than in the general population.Neonates and infants are irritable and poor feeders.It is autosomal recessive.
Cardiovascular system
Published in A Stewart Whitley, Jan Dodgeon, Angela Meadows, Jane Cullingworth, Ken Holmes, Marcus Jackson, Graham Hoadley, Randeep Kumar Kulshrestha, Clark’s Procedures in Diagnostic Imaging: A System-Based Approach, 2020
A Stewart Whitley, Jan Dodgeon, Angela Meadows, Jane Cullingworth, Ken Holmes, Marcus Jackson, Graham Hoadley, Randeep Kumar Kulshrestha
Two more relatively common congenital cardiology treatments are for coarctation of the aorta and pulmonary artery stenosis. Each of these is treated by placing a (relatively large) stent within the obstruction. An aortic coarctation is often a very significant stenosis, around the superior aspect of the descending thoracic aorta. This can be so severe as to make passage of a catheter quite challenging. Once a stiff wire has been passed via the femoral artery and through the stenosis, pressure measurements are taken on either side of the coarctation. The difference in peak proximal to distal pressure is known as a pressure gradient. Once an appropriately sized stent has been deployed, the pressure gradient is again measured to check the value of the treatment. If not significantly reduced, the stent may be dilated using a larger balloon.
Management of surgical complications
Published in Wickii T. Vigneswaran, Edward R. Garrity, John A. Odell, LUNG Transplantation, 2016
Severe venous stenosis may appear as pulmonary congestion and edema soon after the transplanted lung has been reperfused, thus leading to cardiopulmonary instability. The transplanted lung may appear dusky and lose its elasticity, becoming heavy and firm to the touch. In cases of moderate to mild stenosis, findings may be delayed for a few hours or days and often include persistent radiographic opacification of the affected lobe or lung and clinical signs of pulmonary edema.6 In addition to frothy secretions, an excessive amount of clear chest tube drainage (>1000 mL/ day) was noted in a patient of ours with severe venous stenosis (Figure 27.1). Pulmonary artery stenosis may be associated with persistent pulmonary hypertension, hypoxia, or unsuccessful weaning from the ventilator. Anastomotic stenosis can result in a predisposition to thrombus formation in response to turbulent flow patterns (Figure 27.2).
Next Generation Sequencing in a Case of Early Onset Hydrops: Closing the Loop on the Diagnostic Odyssey!
Published in Fetal and Pediatric Pathology, 2023
Priya Ranganath, Vineeth VS, Ikromi Rungsung, Ashwin Dalal, Shagun Aggarwal
Fetal anthropometry corresponded to 50th centile for 13–14 weeks of gestational age. Internal examination showed normal abdominal viscera and structures. Thoracic cavity showed an anteriorly placed cardiac apex with rudimentary right ventricle. Pulmonary artery was 1 mm in diameter and aorta was 2 mm in diameter, communication between pulmonary artery and right ventricle could not be established hence a diagnosis of possible pulmonary valve atresia with pulmonary artery stenosis was made. A nubbin like tissue was seen on the surface of the right ventricle not communicating with the cavity, suspected to be a part of developing right ventricle (Figure 2f and g). Inter-atrial septum, bilateral atrium, left ventricle, inter-ventricular septum and aorta were normal in their structure and orientation. Lungs, trachea, and esophagus appeared normal. A final diagnosis of fetal hydrops with cardiac abnormality (hypoplastic/rudimentary right ventricle with outflow tract defect) and nonspecific dysmorphism was made.
Progression of a supra-valvular aortic stenosis in adulthood during 13 years
Published in Acta Cardiologica, 2021
G. Clermont, A. Friart, C.-H. Huynh, M. Antoine
A 27-year-old woman presented for the first time to the Cardiology department in 2006 for an asymptomatic heart murmur since the childhood and familial hypercholesterolaemia. Cholesterol level was 161 mg/dL under rosuvastatin 40 mg, compared to 317 mg/dL before treatment. Physical examination revealed an aortic midsystolic heart murmur of 2/6 intensity. No signs of hypercholesterolaemia were identified. The EKG was normal. The transthoracic echocardiography (TTE) showed a tricuspid aortic valve, with a mean and maximum transaortic gradient of 16/20 mmHg respectively and a maximal velocity (Vmax) of 2.73 m/s. A supra-valvular aortic narrowing is seen at the sino-tubular junction, reducing the diameter of the aorta to 1.5 cm (Figure 1). The diagnosis of supra-valvular stenosis is established and further confirmed by cardiac magnetic resonance (cMR) (Figure 2) and cardiac scan (cCT). Other heart defects, such as pulmonary artery stenosis which is frequently associated, have been excluded. Stress echography showed no increase in the mean gradient. CoroCT revealed normal coronary arteries.
Interventional Cardiology at a Pivot Point
Published in Structural Heart, 2018
Interventional procedures have been applied for a variety of congenital heart diseases for many years. In fact, catheter closure of atrial septal defects was the first non-invasive technique directed to the treatment of structural heart disease. A variety of devices have been developed to close atrial and ventricular septal defects as well as patent ductus arteriosus. In addition, balloon dilation of pulmonic stenosis, coarctation of the aorta, and pulmonary artery stenosis have become accepted clinical procedures. Transcatheter valves are being deployed even as technological innovation continues. It is likely that such procedures will increase in the future as patients with congenital heart disease increasingly survive into adulthood. In terms of the future, perhaps the greatest growth will be in closure of the patent foramen ovale (PFO). Three recent studies in the New England Journal of Medicine and a recent meta-analysis all attested to the value of catheter closure of a patent foramen in patients with prior cryptogenic stroke or transient ischemic attack.9 Since a PFO has been found to be present in approximately 20% of the population, closure of such lesions is likely to substantially increase.