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Pseudotruncus arteriosus: Its occurrence with acquired aortic stenosis in an adult.

Prolonged survival with pseudotruncus arteriosus, an extreme variant of Fallot's tetralogy, is unusual. We studied the clinical and pathological findings in an adult with uncorrected pseudotruncus. Longevity may have been related to the presence of markedly enlarged bronchial arteries that provided collateral circulation to the lungs. However, prolonged survival was accompanied by the development of severe degenerative changes of the aortic valve leaflets that resulted in acquired valvular stenosis.

Adult↗

[Plain roentgenography findings of congenital heart disease (author's transl)].

Plain roentgenography of congenital heart disease in newborn is subject to narrow diagnostic limitations. The cardiac abnormalities should preferably be classified according to haemodynamic criteria. In most cases the diagnostic information is restricted to the statement whether the malformation detected plain roentgenography is associated with normal, increased or decreased pulmonary perfusion. Most cardiac abnormalities occur in conjunction with a more or less pronounced cardiomegaly, and only the tetralogy of Fallot, tricuspid atresia, and total anomalous pulmonary venous drainage are associated with a heart of normal or only slightly enlarged size. Configurations which are considered as being typical of congenital heart disease are only seen in the tetralogy of Fallot and in tricuspid atresia (wooden shoe deformity) on the one hand, and in the transposition of the great arteries and the truncus arteriosus on the other eggshaped heart). Differentiation between these malformations of the heart is sometimes possible via the position of the aortic arch. The factors mainly responsible for the development of cardiac insufficiency during the first few days of life should be the coarctation of the aorta and the hypoplastic left-heart syndrome.

Aortic Coarctation↗

A radiographic and echocardiographic approach to cyanotic congenital heart disease.

An updated, systematic approach to the diagnosis of cyanosis in the infant or child has been presented. It involves the use of clinical data (to establish the presence of cyanosis), radiographic information (to assess lung parenchyma, pulmonary vascularity, and certain key, border-forming, cardiac structures), and M-mode and cross-sectional echocardiographic data (the source of detailed intracardiac anatomic information). Specific radiographic and echocardiographic features of the 10 most common cyanotic congenital heart lesions have been presented. Using this approach, one should be able to diagnose accurately the cyanotic infant or child. Further planning for cardiac catheterization and angiocardiography can then be accomplished with an informed and rational point of view.

Adolescent↗

Acute postoperative obstruction of extracardiac conduit due to separation of thin fibrous peel.

Late postoperative obstruction of extracardiac conduits may occur in some patients and may result from one of several mechanisms. Severe intraoperative or early postoperative obstruction of such conduits is very rare. Herein we describe a case of acute, severe, early postoperative obstruction of an extracardiac conduit; this followed partial excision and replacement of a Hancock conduit in which late postoperative calcific valvular stenosis had occurred. Unexpectedly elevated right ventricular pressure should suggest the possibility of acute conduit obstruction. In cases with partial conduit replacement, the remaining segment should be carefully inspected for the presence of a peel; if a peel is present, it should be removed from the conduit even if it is considered thin and nonobstructive.

Adolescent↗

[Complete transposition of the major vessels].

The study of 16 hearts with complete transposition of the main vessels shows that there is no correlation between the degree of subaortic cone and the forward displacement of the aorta. The anterior part of the interventricular septum is placed on the same plane with its posterior part. The conic part of the septum occupied antero-posterior position, which is explained by the absence of inversion of conus septum. The antero-posterior position of the main vessels is apparently due to a slight counterclockwise rotation of the truncus.

Heart Septal Defects↗

Correction of truncus arteriosus in the neonate using a nonvalved conduit.

Successful correction of truncus arteriosus in two neonates, both 4 days of age, is described. Nonvalved polytetrafluoroethylene (PTFE) conduits were used because of the small size of the infants. Both recovered from operation with no signs right ventricular failure and remain well. Catheterization data on one patient show satisfactory hemodynamics 1 year after operation with only residual branch pulmonary artery stenosis. These data suggest that a conduit valve is not essential in the correction of truncus arteriosus even in the neonate.

Blood Vessel Prosthesis↗

Postoperative hemolysis and its prevention in corrective surgery of truncus arteriosus.

Two cases of truncus arteriosus type I associated with truncal valve insufficiency were operated upon with the Rastelli's procedure. The first case of 3-year-old boy died from severe hemolysis which was caused by the regurgitant jet from the truncal valve running against the rough-surfaced Dacron patch for the VSD. The second case of 12-month-old girl was treated with a Dacron patch lined with an autologous pericardial pedicle. Her postoperative course was uneventful. The authors discuss the potential risk and prevention of postoperative hemolysis in corrective surgery of the truncus arteriosus with truncal valve insufficiency.

Child, Preschool↗