Carla Frescura

University of Padua

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Unicommissural aortic valve in neonates and its association with other congenital heart disease

Cardiology in the Young (1995)

Ana C. Durán Luciano Daliento Carla Frescura Giovanni Stellin Valentín Sans‐Coma

Abstract The morphology of the aortic valve was studied in 1,022 heart specimens belonging to the collection of the Institute of Pathological Anatomy, University of Padua. Twenty specimens were found to have a unicommissural aortic valve, characterized by the presence of a single leaflet with only one functional commissure; however, the presence of two raphes enabled the recognition of a basically three-sinus arrangement. Age and gender were known in 19 cases: 14 male and five female, mean age nine days. In 19 cases, the unicommissural valve was dysplastic and resulted in a severe congenital aortic valvar stenosis. Only in two hearts was the unicommissural nature of the aortic valve an isolated finding; among the remaining 18, left ventricular fibroelastosis in 11, malformation of the mitral valve in 11, hypoplasia of the left ventricle in eight, ventricular septal defect in four, mitral atresia in three, and subaortic fibrous diaphragm in one. Furthermore, seven (35%) of these 20 unicommissural aortic valves were associated with coarctation of the aorta. Statistical analysis shows that this association is not a random event. Our findings support the hypothesis that the unicommissural aortic valve originates from the early fusion of the three mesenchymal valvar cushions or leaflet primordia. Although the present data do not exclude the possibility that reduction of the blood flow through the aorta during fetal life may play a role in the formation of the unicommissural aortic valve, they rather point in the direction that another etiologic factor, such as an anomalous migration of neural crest cells, may be responsible for the fusion of the valvar cushions.

Atrioventricular valve

Commissure

10.1017/s1047951100011707

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Citations (8)

Postoperative pathology of congenital heart disease. I. Complete atrio-ventricular canal.

PubMed (1982)

Franceschini Grisolia E Carla Frescura Uberto Bortolotti A Mazzucco Gallucci

The postoperative pathology observed in 17 patients with complete atrioventricular canal, who died following total correction is described. In 3 patients, in whom valve replacement was necessary, the death was due to late prosthetic dysfunction. Out of 14 patients who underwent plastic repair, 4 died from undetected residual cardiac anomalies, which in 2 cases were associated with pulmonary obstructive vascular disease, 3 from unexplained myocardial infarction, 3 from inadequate postoperative care and 4 from pulmonary obstructive vascular disease which presented as an isolated finding. An analysis of the incidence of pulmonary obstructive vascular disease in these patients disclosed that it can develop before one year of age in some patients with Down syndrome.

Atrioventricular canal

Obstructive lung disease

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Small aortic root in neonates.

PubMed (1996)

Carla Frescura Gaetano Thiene

Small aortic root in neonates and infants is usually associated with unicommissural aortic valve, namely the presence of only one leaflet with eccentrically located commissure. This condition implies aortic stenosis because of: (i) a small aortic ring diameter; (ii) an intrinsically stenotic unicommissural orifice; and (iii) a dysplastic leaflet with myxoid nodular excrescences. The frequent association with hypoplastic left ventricle, fibroelastosis, dysplastic mitral valve and aortic arch obstructions prohibits a biventricular repair.

Commissure

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Citations (1)

List of Contributors

Elsevier eBooks (2017)

Rita Alaggio Annalisa Angelini Gianfranco Butera Biagio Castaldi Patrizia Dall’Igna

10.1016/b978-0-12-810406-4.01002-x

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The concept of double inlet-double outlet right ventricle: a distinct congenital heart disease

Cardiovascular Pathology (2016)

Veronica Spadotto Carla Frescura Siew Yen Ho Gaetano Thiene

Venous return curve

Atrioventricular canal

Atrioventricular valve

10.1016/j.carpath.2016.09.003

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Citations (11)

Endocarditis in an infant causing “tricuspid atresia”

International Journal of Cardiology (1989)

Siew Yen Ho Carla Frescura Gaetano Thiene

Tricuspid atresia

Right heart failure

10.1016/0167-5273(89)90282-9

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Citations (1)

[Segmental anatomic diagnosis in congenital cardiac anomalies. I. Anatomic criteria for the identification of the cardiac cavities (author's transl)].

PubMed (1978)

Carla Frescura R Bini Ornella Milanesi G. Alampi Gaetano Thiene

Anatomical features are illustrated for the identification of the cardiac chambers. Essential and accessory morphological structures are recognized. The identification of the atrial cavities is based upon the anatomy of the free walls (crista terminalis, size and shape of the atrial appendages). On the contrary, the identification of the ventricles is founded on the septal morphology (trabecula septo-marginalis on the morphological right side, smooth basal septum on the morphological left side, presence or absence of septal insertions of the atrio-ventricular value apparatus). Finally, no essential feature can be applied to the identification of the great arteries.

Crista terminalis

Cardiac Ventricle

Appendage

Trabecula (gastropod)

Identification

Atrial septum

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Non-atherosclerotic coronary artery disease and sudden death in the young

Heart (1992)

Domenico Corrado Gaetano Thiene P Cocco Carla Frescura

Objective—To assess prevalence and type of non-atherosclerotic coronary artery disease in young people (≤35 years) who died suddenly. Design—A necropsy study of 150 consecutive cases of sudden death (that is, within 6 h of the onset of symptoms). Results—Death was attributed to coronary artery disease in 48 cases: in 16 (33%) of them the disease was non-atherosclerotic. Twelve subjects (eight males and four females, age range 2–35 years, mean 24·2) had congenital anomalies: a deep intramyocardial course in six, origin from the wrong sinus in three, and ostial obstructions in three. Sudden death was the first manifestation of disease in six cases. The other six had a history of palpitation or syncope or both. An electrocardiogram was available in five cases and showed ventricular arrhythmias in four; none had angina pectoris. Stress testing was available in two cases: neither showed any effort-dependent ST-T abnormalities. In six cases sudden death was related to physical exercise. Acquired non-atherosclerotic coronary artery disease was found in four cases: spontaneous coronary dissection in three previously symptom free patients and Kawasaki coronary arteritis in one child who had had acute myocardial infarction. Conclusion—One third of the cases of fatal coronary artery disease were non-atherosclerotic with coronary artery anomalies being the most frequent form. Coronary artery anomalies should be suspected in young patients who have symptoms of ventricular arrhythmias without any overt signs and symptoms of ischaemia.

Sudden Death

Coronary disease

10.1136/hrt.68.12.601

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Citations (171)

Bone-marrow–derived CXCR4-positive tissue-committed stem cell recruitment in human right ventricular remodeling

Human Pathology (2010)

Chiara Castellani Massimo A. Padalino P China Marny Fedrigo Carla Frescura

Bone remodeling

Human bone

10.1016/j.humpath.2009.12.017

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