PubMed HealthSearch

Biomedical subjects

C Frescura

Publications and source records attributed to C Frescura.

14 recordsLinked to original sources

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

OBJECTIVE: To assess prevalence and type of non-atherosclerotic coronary artery disease in young people (< or = 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.

Adolescent

Is lung biopsy useful for surgical decision making in congenital heart disease?

Sixty patients with congenital heart disease (CHD), age range 3 months-45 years (median 2 years), underwent lung biopsy to exclude pulmonary vascular disease (PVD): 25 had an atrioventricular (AV) septal defect, 14 a ventricular septal defect (VSD), 7 a complete transposition of the great arteries (TGA) + VSD, 3 an atrial septal defect, and 2 a patent ductus arteriosus; 9 had other malformations. Scoring of histological section (0-4) according to a modified Heath-Edwards classification disclosed that 30 patients had severe "irreversible" PVD (greater than or equal to grade 3) (11 AV septal defect, 8 VSD, 6 TGA + VSD and 5 others); 8 patients were younger than 1 year. Pulmonary vascular resistance (PVR) was calculated in 51 patients and exceeded 7 U/m2 in 1 of 2 patients with grade 0, in 9 of 18 with grade 1, in 1 of 4 with grade 2, in 11 of 18 with grade 3, and in 8 of 9 with grade 4. PVR was also calculated after 100% oxygen administration in 19 patients, 14 of whom had a resting PVR greater than or equal to 7 U/m2. PVR persisted greater than or equal to 7 U/m2 in 5 patients: 4 had PVD greater than or equal to grade 3 and died after surgery. PVR fell to under 7 U/m2 in 14 patients, 3 of whom had PVD greater than or equal to grade 3:1 patient with grade 4 was not operated upon and 2 with grade 3 were operated upon: all are alive. Follow-up in discharged living patients was 100%, with a mean of 50 months. Fifty-five patients underwent surgery with 10 early and 2 late deaths. Among the 12 subjects with a fatal outcome, 10 had irreversible lesions. The 5 non-operated patients all had irreversible lesions at biopsy, and are still alive.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent

Isomerism of the left atrial appendage and left lung in conjoined twins.

A pair of thoracophagus twins with conjoined hearts and livers are described with emphasis on the cardiac anatomy. The heart of one twin had isomerism of the left atrial appendages in association with azygos continuation of the inferior caval vein. Aortic atresia and atrioventricular septal defect was present in the heart of the other twin.

Female

Coronary arterial wall and atherosclerosis in youth (1-20 years): a histologic study in a northern Italian population.

Based on the working hypothesis that coronary atherosclerosis begins in childhood, a histologic study was carried out on the subepicardial coronary arterial tree of 100 young persons (1-20 years), who had died from causes unrelated to the cardiovascular system. These subjects were natives of a well-defined geographic area in northern Italy, namely the region of Veneto. Intimal proliferations (musculo-elastic and fibro-elastic layers) were observed in 95.3% of the coronary arterial segments in the age group between one and five years. The more distal the coronary segments examined, the lesser was the intimal thickening. Raised mature fibrous plaques were detected in 23 segments from 15 patients (2 from subjects aged between six and 10 years; 4 between 11 and 15 years, and 9 between 16 and 20 years). Single vessel disease was present in 9, double vessel disease in 4 and triple vessel disease in 2 cases. The site most involved by plaques was the proximal part of the left anterior descending coronary artery. Only one plaque was of sufficient dimensions to be considered stenotic (50% luminal reduction). Plaques were rarely sudanophilic, and all seemed to arise in relation to previous intimal thickening. No qualitative nor quantitative sexual differences were observed. These data give rise to much concern, and one consistent with a recently observed occurrence of sudden coronary death in young people from the same geographic area.

Adolescent

Histology of pulmonary arterial supply in pulmonary atresia with ventricular septal defect.

A histologic study was performed on 22 specimens of pulmonary atresia with ventricular septal defect to 1) ascertain the existence of the main pulmonary artery; 2) distinguish the ductus arteriosus from the systemic collateral arteries (SCA); 3) establish the nutritive or functional nature of collateral circulation; and 4) evaluate the morphology of the distal pulmonary bed. Three cases had absent main pulmonary artery, one with and two without signs of infundibular septation. We suggest that absent main pulmonary artery may exist with both infundibular pulmonary atresia and persistent truncus arteriosus. SCAs have been found to have similar histological features as systemic muscular arteries of the same size--their medial muscular layer merges gradually into an elastic one at different depth inside the lungs. Injection of contrast material allowed us to demonstrate that these vessels are functional, since they inosculate into efficient pulmonary arteries ending in the respiratory units. When the distal pulmonary vascular bed is perfused by large SCAs, proliferative lesions like those found in large left-to-right shunts may occur. Early in infancy, banding of large, nonstenotic SCAs could protect the distal pulmonary vasculature. Moreover, total surgical repair should be associated with ligation of the SCA to avoid residual left-to-right shunt, if the pulmonary arteries can carry the full pulmonary blood flow.

Aorta, Thoracic

Complete atrioventricular canal associated with conotruncal malformations: anatomical observations in 13 specimens.

Conotruncal anomalies associated with atrioventricular (AV) canal defects are more common than is generally appreciated on clinical grounds. Among 39 specimens of AV canal malformations, 13 (33%) presented with conotruncal abnormalities: a complete form of AV canal has been observed in all. 5 cases exhibited visceral situs solitus, 5 situs ambiguus with asplenia and 3 situs ambiguus with polysplenia. In the first group, conotruncal anomalies were tetralogy of Fallot in 3 cases, bilateral conus with double outlet right ventricle (DORV) in 2, one with subpulmonary ventricular septal defect (VSD) and the other with doubly commited VSD. Survival in these patients was relatively longer (average 20 mth) and the clinical course was mainly determined by the degree of the pulmonary outflow obstruction: surgical correction should have been feasible in these cases. Patients with situs ambiguus, both with asplenia and polysplenia, had further severe cardiovascular malformations associated with AV canal which led to early death (average survival 12 days): anomalous pulmonary and systemic venous return and univentricular hearts. In the latter patients, tetralogy of Fallot, bilateral conus with DORV and pulmonary atresia were the conotruncal malformation. Retrospectively, in no case of the last category a complete repair had been accomplished. All but one specimen presented the complete form of AV canal with 'free floating anterior leaflet' and hypoplastic anterior tricuspid component. This hypoplasia could be interpreted as missing conal tissue in the development of the anterior tricuspid cusp. For this leaflet a dual embryological origin, both from the dextro-dorsal conal ridge and the right lateral AV cushion, is suggested.

Abnormalities, Multiple

[Transposition of the great arteries: anatomic types of left ventricular outflow tract obstruction (author's transl)].

A postmortem investigation has been carried out in 48 heart specimens with transposition of the great arteries (TGA) to evaluate incidence and type of left ventricular outflow tract obstruction. The frequence of pulmonary stenosis in our series (23%) is similar to those reported from previous studies. Cases have been divided in two groups according to presence or absence of ventricular septal defect. In cases with intact ventricular septum the obstruction was determined by asymmetric hypertrophy of the septum (1 observation) and by abnormal attachement of clefted anterior mitral leaflet to the interventricular septum (2 observations). In the specimens with ventricular septal defect, 4 presented a stenosis due to malalignment of the infundibular septum, associated with valvular stenosis in 1 case and with valvular stenosis and fibrous subvalvular ring in 2. Another case presented a stenotic pulmonary valve and a parachute mitral valve. The remaining 3 showed a cleft of the anterior leaflet of the mitral valve. The high incidence of infundibular malalignment obstruction is underlined together with its association with A-P or L-position of the aorta. This peculiar relation of the great arteries could be angiocardiographically employed as indicative for the presence of this type of left outflow tract obstruction in TGA.

Female

Systemic-pulmonary arterial supply in pulmonary atresia with ventricular septal defect: postmortem angiograms and histologic survey.

Postmortem angiographic and histologic studies of the pulmonary arterial circulation were performed in a patient with pulmonary atresia and a ventricular septal defect. While the left lung was supplied by a closing ductus arteriosus, the right lung was supplied by two systemic pulmonary arteries arising from the descending aorta. The examination disclosed that systemic pulmonary arteries lead into the pulmonary vascular bed and the capillaries of the alveolar walls. According to these observations, such collateral circulation is to be considered functional. The pulmonary vascular bed, supplied by the ductus arteriosus and the stenotic systemic pulmonary artery, showed a thin muscular layer in the small arteries and arterioles. On the contrary, medial hypertrophy and severe intimal proliferation were observed in the pulmonary segments perfused by the other large unobstructed systemic pulmonary artery, thus proving that asymmetric pulmonary vascular disease may complicate the natural history of this malformation.

Collateral Circulation

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

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.

Heart

[Segmental anatomic diagnosis in congenital cardiac anomalies. II: Sequential localization of the cardiac chambers (author's transl)].

The approach to the diagnosis of congenital heart disease, based upon the identification of the cardiac chambers, the reconstruction of their sequence and final recognition of the basic circulatory model is discussed. Three cardiac segments are recognizable embriologically, anatomically and functionally: atria, ventricles and great arteries. Connexion is the sequential link of these segments, independently from their spatial relationship. Situs of the atria can be: solitus, inversus or ambiguus, and is determined by the thoracic situs which can be assumed from the bronchial anatomy. Atrio-ventricular connexion can be concordant, absent or double inlet ventricle. Ventricular-arterial connexion can be concordant, discordant, double outlet ventricle or single outlet heart. Since this approach aims to the reconstruction of the connexion among the cardiac segments, heart position and the infundibular anatomy are not relevant to the recognition of the circulatory model.

Heart Atria

[Subaortic stenosis due to antero-lateral muscular band, associated with ventricular septal defect and underdevelopment of the aortic arch. Anatomical features in three cases (author's transl)].

The pathological findings in three cases with ventricular septal defect and severe coarctation or tubular hypoplasia of the aortic arch are reported. The left ventricular outflow tract was partially obstructed by muscle situated on its antero-lateral wall. The haemodynamic relationship between this type of subaortic stenosis and aortic arch underdevelopment, due to unequal partitioning of the blood flow between the ascending aorta and the pulmonary artery during foetal life, is emphasized. The normal anatomy of the left ventricular infundibulum is described and some embryological hypotheses to explain this antero-lateral muscular band are forwarded.

Aortic Coarctation