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Paper 1
Published in Amanda Rabone, Benedict Thomson, Nicky Dineen, Vincent Helyar, Aidan Shaw, The Final FRCR, 2020
Amanda Rabone, Benedict Thomson, Nicky Dineen, Vincent Helyar, Aidan Shaw
Other conditions associated with aortic coarctation include ventricular septal defect, hypoplastic aortic arch, truncus arteriosus, scoliosis and Turner syndrome. Congenital truncus arteriosus and ventricular septal defect would become symptomatic much earlier in the patient’s life. Pulmonary artery aneurysms are not associated with aortic coarctation.
Congenital Heart Disease in Pregnancy
Published in Afshan B. Hameed, Diana S. Wolfe, Cardio-Obstetrics, 2020
Truncus arteriosus, also known as persistent truncus arteriosus, is an uncommon cyanotic congenital heart defect, accounting for 1%–4% of cardiac heart defects in a large autopsy series [15] and 0.6–1.4 per 10,000 live births [16]. In truncus arteriosus, the main pulmonary artery or branch pulmonary arteries arise from the aorta, typically the ascending aorta. Patients nearly always have a large nonrestrictive ventricular septal defect. The truncal (aortic) valve has normal trileaflet morphology in 69%, is quadricuspid in 22%, and bicuspid in 9%. The abnormality, as well as dilation of the aorta and valve annulus, predisposes patients with truncus arteriosus to aortic regurgitation.
The cardiovascular system
Published in C. Simon Herrington, Muir's Textbook of Pathology, 2020
Mary N Sheppard, C. Simon Herrington
In persistent truncus arteriosus the aorta and pulmonary artery arise from a common stem vessel, the truncus arising from both ventricles and overriding a ventricular septal defect. If the septum fails to develop, a single ventricular cavity results. Interatrial septal defects are also common.
Prenatal sonographic findings in a cohort of foetuses with a confirmed 22q11.2 microdeletion at a single Chinese Tertiary Centre
Published in Journal of Obstetrics and Gynaecology, 2022
Xiang-Yi Jing, Yong-Ling Zhang, Li Zhen, Yan-Lin Li, Dong-Zhi Li
In the study period, a total of 77 foetuses with del22q11.2 were detected by CMA. One case was excluded because of the loss to follow-up, and the remaining 76 cases were included in this study (shown in Figure 1). The median maternal age was 28 (19–39) years. The median gestational age at prenatal diagnosis was 25 (12–32) weeks. CHD was the most common indication (47/76, 61.8%) for prenatal testing and was isolated in 52.6% (40/76) (Table 1). The constitution of CHD comprised predominantly of conotruncal defects (61.7%; 29/47), in which tetralogy of Fallot (TOF) was the most common one (23.4%; 11/47), followed by interrupted aortic arch (14.9%; 7/47), truncus arteriosus (10.6%; 5/47), coarctation of aorta (6.4%; 3/47), pulmonary stenosis (4.3%; 2/47) and double-outlet right ventricle (DORV) (2.1%; 1/47), respectively. Other heart anomalies were encountered in 38.3% (18/47) of cases; among these, right-sided aortic arch (RAA) was found in 11 (23.4%; 11/47) cases, and as an isolated cardiac finding in 9 cases (19.1%; 9/47).
First Trimester Prenatal Diagnosis of a Conotruncal Anomaly Using Spatiotemporal Image Correlation Imaging Confirmed by Conventional Autopsy
Published in Fetal and Pediatric Pathology, 2022
Balaganesh Karmegaraj, Vani Udhayakumar, Gigi Selvan
Truncus arteriosus is characterized by a single arterial trunk which gives origin to the systemic, coronary and pulmonary circulations. A large VSD is almost always present. It is classified into four types based on the anatomic origin of the pulmonary arteries. Ductus arteriosus is absent in >50% of cases. It is highly associated with 22q11 microdeletion (30-40%). When diagnosed prenatally, the outcome is poorer when compared with postnatal diagnosis. Hence this anomaly is associated with high termination rates [6]. Four-dimensional STIC imaging provides incremental benefits in evaluation of fetal cardiac anomalies, and confirmation by autopsy findings allows further refinement of the diagnosis. Detection of severe CHD before the period of fetal viability can give parents the option to consider termination of pregnancy. Conventional autopsy is quite feasible especially in early trimester. Postmortem examination plays an important role in confirming the antenatal diagnosis, which is crucial with regard to counseling future pregnancies [7].
Early Experience with the INSPIRIS RESILIA Valve in Pulmonary Valve Replacement for Congenital Heart Disease
Published in Structural Heart, 2020
Sreekumar Subramanian, Daniel Nento, Karl Reyes
Results: A total of 8 patients (4 male/4 female) were identified. Mean age was 29 (range 6 - 62 yrs). 6/8 patients had tetralogy of Fallot, and 2/8 had complete repair of truncus arteriosus previously. The size of the INSPIRIS valves implanted were 25 mm (3/8), 27 mm (2/8) and 29 mm (2/8) and the large implant sizes were associated with superb hemodynamic outcomes (mean gradient 3-9 mm). Concomitant operations performed included beating heart PFO closure (2/8), MPA plasty in 7/8 patients and RV-PA conduit using a 25 mm INSPIRIS valve in a 28 mm Dacron graft (1 patient), as well as branch PA plasty in 3/8 patients and AVR in 1/8 patients. Hospital length of stay was 5.5 days (range 3-7 days) and there was no 30-day mortality. At a mean follow-up time of 6 months, all patients are doing well, NYHA Class I status.