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Valve Disease
Published in Mary N. Sheppard, Practical Cardiovascular Pathology, 2022
Pulmonary atresia or stenosis is seen in the context of congenital stenosis as in Tetralogy of Fallot, when the valves cusps are often thickened and dysplastic. Bicuspid pulmonary valves (Fig. 3.75) occur but are much rarer than bicuspid aortic valves. Quadricuspid pulmonary valve (Fig. 3.76) is more frequent than aortic quadricuspid valve. Approximately 20% of newborns with CHD have anomalies in the pulmonary valve or right ventricular outflow tract (RVOT), such as Tetralogy of Fallot (TOF), truncus arteriosus, or pulmonary atresia. For these patients, surgical repair of the RVOT is important but these patients are subject to progressive RVOT dysfunction, with pulmonary valve regurgitation, pulmonary valve stenosis or both. Surgical pulmonary valve replacement is the treatment and can be performed with a low mortality rate. However, valved conduits have a limited lifespan with degenerative calcification often lasting less than 10 years, thus necessitating further replacement done percutaneously.
The cardiovascular system
Published in C. Simon Herrington, Muir's Textbook of Pathology, 2020
Mary N Sheppard, C. Simon Herrington
Pulmonary atresia or stenosis is seen in the context of Tetralogy of Fallot when the valve cusps are often thickened and dysplastic or simply replaced by a fibrous ridge in association with ventricular septal defect and right ventricular hypertrophy and anterior displacement of the aortic root.
Paediatrics
Published in Dave Maudgil, Anthony Watkinson, The Essential Guide to the New FRCR Part 2A and Radiology Boards, 2017
Dave Maudgil, Anthony Watkinson
RDS (hyaline membrane disease) may cause lobar/interstitial emphysema, or bronchopulmonary dysplasia. Congenital lymphangiectasia is a malformation characterised by anomalous dilatation of pulmonary lymph vessels and is invariably fatal before two months of age. Pulmonary atresia usually comprises an atretic pulmonary valve with underdevelopment of the pulmonary artery. The affected lung is small but otherwise normal initially, although pulmonary hypertension may ensue without treatment.
Clinical features and echocardiographic findings of isolated foramen ovale restriction in foetuses
Published in Journal of Obstetrics and Gynaecology, 2022
Tolga Akbaş, Fadli Demir, Sevcan Erdem, Berivan Çağnıs, Ferda Özlü, Selim Büyükkurt, Nazan Özbarlas
On postnatal echocardiographic examinations, 7 (77.7%) patients had normal or minor defects. Those with minor defects had patent ductus arteriosus, mild tricuspid regurgitation, and minimal pericardial effusion. Additionally, patient #1 was diagnosed with cardiomyopathy and patient #7 was diagnosed with functional pulmonary atresia. These two children needed treatment in the neonatal follow-up. The patient with an echocardiographic diagnosis of non-compaction cardiomyopathy was treated for heart failure due to mild systolic dysfunction. The other patient diagnosed with functional pulmonary atresia was born by elective caesarean section at 34 weeks. The echocardiography was performed immediately after delivery; there was significant tricuspid regurgitation, no antegrade flow was observed in the pulmonary artery, and started prostaglandin infusion treatment. This patient also had normal anterograde pulmonary artery flow and normal arterial oxygenation within one week. No death occurred in any foetus during follow-up. The patients' characteristics, pregnancy-related risk factors, foetal and postnatal echocardiographic findings were summarised in Tables 1 and 2.
Interventions in Congenital Heart Disease: A Review of Recent Developments: Part II
Published in Structural Heart, 2021
Similar to fetal AS, right ventricular outflow tract obstruction from critical pulmonary stenosis (CPS) or pulmonary atresia with an intact ventricular septum (PA/IVS) results in progression to right ventricular dysfunction and hypoplasia with or without co-existing coronary artery abnormalities. Despite improvements in postnatal management, PA/IVS carries a significant risk of morbidity and mortality.118–120 Fetal pulmonary valvuloplasty offers the potential for improved right ventricular growth, increasing the likelihood of biventricular outcome after birth. Published outcomes of pulmonary valvuloplasty for CPS or PA/IVS are comparatively fewer than for FAV. Data from the IFCIR (n = 16), Linz (n = 35), and Boston (n = 10) groups documented technical success from 60% to 69%, and a biventricular outcome was achieved in 38% to 65% of these cohorts.109,121,122 Results from the Linz group showed significantly increased RV dimensions and filling time acutely, and limited longitudinal data support continued RV growth until birth.121
Results of Pulmonary Valve Replacement with a Newly Introduced Bioprosthesis in Children and Young Adults with Congenital Heart Disease
Published in Structural Heart, 2021
Jose M. Arribas-Leal, Maria Garcia-Vieites, Antonio Jimenez-Aceituna, Sergio Canovas-Lopez, Francisco Gutierrez, Victor Bautista-Hernandez
The youngest patient had a history of pulmonary atresia and needed PVR at the age of 4 years. Eighteen patients (85.71%) had undergone previous sternotomies and 6 patients (28.57%) received concomitant procedures. Fourteen patients (66.66%) had the valve on the beating heart. Mean lengths of intensive care unit (ICU) and hospital stay were 2 ± 1.3 and 5 ± 1.96 days, respectively. One patient, a 43-year-old man with tetralogy of Fallot, 3 previous surgeries, and preoperative NYHA III–IV functional class and severe right ventricular failure, died after surgery from multiorgan failure despite extracorporeal membrane oxidation (ECMO) therapy for cardiogenic shock. No patient had a permanent pacemaker after surgery or other major complications. Early echocardiogram depicted a mean peak gradient across the valve of 17 ± 7.23 mm Hg. No paravalvular leaks were observed. With a mean follow up of 17.38 ± 8.70 months, all discharged patients were alive with no clinical 150 complications. Most recent echocardiograms show good hemodynamics with no paravalvular leaks and without structural valve deterioration (SVD). Peak gradients between echocardiograms are shown in Table 2.