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Biomedical subjects

J L Wilkinson

Publications and source records attributed to J L Wilkinson.

At least 73 records · Page 4Linked to original sources

Total anomalous pulmonary venous drainage. Seventeen-year surgical experience.

Between 1968 and 1985, 80 children underwent correction of total anomalous pulmonary venous drainage. There were 47 boys and 33 girls whose ages ranged from 3 days to 16 years (median 2 months, interquartile range 5 years). Seventy (87.5%) were less than 1 year of age at operation. Fifty-eight (72.5%) weighed less than 5 kg, the range being 1.6 to 42 kg (median 3.7 kg, interquartile range 2.4 kg). Forty-five (56%) patients had supracardiac, 14 (17.5%) cardiac, 15 (19%) infracardiac, and 6 (7.5%) had mixed total anomalous pulmonary venous drainage. Follow-up was complete in 78 (97.5%) and ranged from 6 to 189 months (median 58 months, interquartile range 59 months). There were 14 (17.5%) early and six (7.5%) late deaths. Analysis by various factors revealed year of operation as the only factor to affect survival at the 5% level of significance. Early mortality was 29% between 1968-1977 and 11% between 1978-1985 (p = 0.04). Postoperative pulmonary venous obstruction occurred in five (6%) patients between 6 weeks and 3 months after operation. All 5 died, three after reoperation. Five (6%) other children had reoperations, four for residual shunts and one for superior vena caval obstruction.

Adolescent↗

Captopril in treatment of infant heart failure: a preliminary report.

We have studied retrospectively 18 infants who have received captopril for treatment of severe heart failure due to left-to-right shunts with pulmonary hypertension. Captopril has been administered in doses of up to 3.5 mg/kg/day (mean 2.47 mg/kg/day). Maintenance treatment with digoxin and frusemide was continued but potassium-sparing diuretics were stopped in most patients. The mean period of assessment was 19 days before and 27 days after commencing captopril. The mean daily weight gain before captopril was -7 g and after its introduction was + 13 g (P less than 0.001). There were statistically significant (P less than 0.05) falls in mean heart rate and respiratory rate and rises in plasma sodium concentration and feeding score. Plasma urea concentration fell but this did not reach statistical significance. Two patients suffered hypotension after increments in captopril dosage and subsequently had a rise in plasma urea and creatinine values. This adverse reaction may be linked to the presence of hyponatraemia. This preliminary report shows captopril may be useful in the control of severe heart failure in infancy.

Body Weight↗

Disharmony between atrioventricular connections and segmental combinations: unusual variants of "crisscross" hearts.

Two congenitally malformed hearts are described, one studied in life and the other at autopsy, in which the topology of the ventricular mass was not as expected for the atrioventricular (AV) connection present. In Case 1, studied at autopsy, there was the usual atrial arrangement with a concordant AV connection. The morphologically right ventricle, however, was left-sided and the ventricular mass was of left-hand topology (l loop) rather than the expected right-hand pattern (d loop). In Case 2, studied during life, there was the usual atrial arrangement and a discordant AV connection. Rather than the anticipated left-hand topology (l loop), the ventricular mass was arranged in right-hand fashion (d loop) and the morphologically right ventricle was right-sided. The cases emphasize that for a full description of a congenitally malformed heart, it is often necessary to account for the topology of each segment as well as the connections (or alignment) among the segments.

Crisscross Heart↗

An anatomical study of the patterns of the coronary arteries and sinus nodal artery in complete transposition.

The origin and course of the main coronary arteries and the sinus nodal artery are relevant to both intra-atrial and arterial relocation operations for complete transposition (the combination of concordant atrioventricular and discordant ventriculo-arterial connexions). The patterns of the main coronary arteries in 148 hearts with complete transposition have been documented together with the patterns of the sinus nodal artery in 105 of these. The terminology which has been used to describe these arteries has been explained. The findings illustrate the wide variation which exists in the origin and course of all these arteries in complete transposition and indicate areas where they are most at risk.

Arteries↗

Misleading hyperoxia test.

A 2 day old infant with infracardiac total anomalous pulmonary venous connection achieved an arterial oxygen tension greater than 250 mmHg, breathing 100% oxygen. Diagnosis of cardiac disease was delayed several weeks. The anatomical basis for this phenomenon was streaming of systemic venous return across an inferior vena cava type atrial septal defect.

Coronary Vessel Anomalies↗

Double connections in total anomalous pulmonary venous connection.

Three infants who underwent operation for total anomalous pulmonary venous connection had unobstructed drainage of all the pulmonary veins to both the coronary sinus and left vertical vein. In one patient the anatomy was demonstrated preoperatively and complete correction accomplished without difficulty, but in two patients the second connection was discovered only at postoperative reinvestigation. Arbitrary classification of total anomalous pulmonary venous connection into cardiac, supracardiac, infracardiac, and mixed types does not lend itself to description of such lesions and may obscure their clinical importance. For these less common variants of total anomalous pulmonary venous connection, a more detailed and precise definition of morphology facilitates accurate surgical repair.

Cardiac Catheterization↗

Architecture of the ventricular mass and atrioventricular valves in complete transposition with intact septum compared with the normal: I. The left ventricle, mitral valve, and interventricular septum.

Venous correction for complete transposition with intact ventricular septum remains the preferred surgical option in most centers (Mustard or Senning procedures). The long-term function of the morphologically right ventricle and tricuspid valve, which continue to sustain the systemic circulation thereafter, remains a matter of concern. Some centers have been performing arterial redirection ("arterial switch") as the surgical treatment of transposition. Consequently, the status of the left ventricle and mitral valve is of equal interest. Yet, detailed morphological studies of the ventricles, atrioventricular valves, and their tension apparatus in "simple" complete transposition are lacking. We have examined the morphology of the left ventricle and the spatial relationships of the component parts of the interventricular septum (part I) as well as the morphology of the right ventricle and the tricuspid valve (part II). A total of 77 specimens with complete transposition and intact ventricular septum were examined and compared with 50 normal hearts. Consistent deformities were noted in relation to the orientation of the ventricular septum. The ratios of ventricular outlet to the inlet lengths were increased in both ventricles and the posterior arterial valve was abnormally related to the atrioventricular valves. These anomalies are similar in some respects to those found in atrioventricular septal defects. Their significance in relation to long-term function remains unclear.

Child, Preschool↗

Architecture of the ventricular mass and atrioventricular valves in complete transposition with intact septum compared with the normal: II. The right ventricle and tricuspid valve.

After the Mustard or Senning procedure for surgical correction of complete transposition of the great arteries, the right ventricle and tricuspid valve have to support the systemic circulation for life. Consequently their function is of prime importance. In this (second) part, the detailed anatomy of the right ventricles and tricuspid valves is described in the same series of 50 normal hearts and a series of 77 with complete transposition and intact septum. 21% of the transposition specimens showed significant abnormalities of the tricuspid valve. Consistent deformities were observed in the structure of the trabecula septomarginalis and the membranous septum. The ratios of ventricular outlet to inlet lengths were increased.

Aorta↗

Aortic coarctation in the first three months of life. An anatomopathological study with respect to treatment.

Operation for coarctation in infancy is a lifesaving procedure, but it still carries a high mortality and is followed frequently by recurrence of stenosis. This is especially true when the procedure is performed in the first 3 months of life. To determine a correlation, if any, between anatomy of coarctation and surgical procedures, we have studied a series of 42 autopsy specimens from patients younger than 3 months who had aortic coarctation. We found that concepts of coarctation based solely on a discrete lesion or isthmic hypoplasia were simplistic. The anatomy was much more complex, the findings being relevant to surgical techniques currently in use (resection and end-to-end anastomosis, subclavian flap aortoplasty). Of equal significance in the cases we studied to the presence of a shelf of ductal tissue within the aortic lumen or hypoplasia of a segment of the arch was the finding of "waist" lesions. In these cases the wall of the aorta was constricted to form the obstruction. Such "waists" coexisted with discrete shelves or with shelves and isthmic narrowing. While we recognize the danger of extrapolating from autopsy findings to predict clinical results, our observations suggest that surgical procedures might best be tailored to the precise anatomy present. It seemed that in may cases a subclavian flap procedure offered the best chance of success. Cases with anatomy suitable for resection and end-to-end anastomosis were less frequent. Percutaneous angioplasty seemed at best to offer only palliative options in limited cases.

Age Factors↗

The univentricular atrioventricular connection: getting to the root of a thorny problem.

Most hearts described as "single ventricle" or "univentricular heart" possess 2 ventricular chambers, even though 1 is usually described as an "outlet chamber." This stems from the wide acceptance that the criterion of a single ventricle is the presence of a double-inlet atrioventricular (AV) connection. In recent years, using this criterion, an attempt was made to show how hearts with double-inlet right ventricle or "classic tricuspid atresia" were (in terms of ventricular morphology) just as univentricular as "single ventricle with outlet chamber." This attempt brought still further confusion to an already contentious topic. The root of the problem clearly is the injudicious use of the adjective "single" or "univentricular." Conventionally it is used to describe the ventricular mass. In most hearts with double-inlet connection it is not the ventricles that are univentricular; it is the AV connection. The concept of a univentricular AV connection, then, appropriately groups hearts with double-inlet along with those having absence of 1 AV connection. It distinguishes this entire group from those other hearts with biventricular AV connections (each atrium connected to its own ventricle). The term "univentricular AV connection" is thus a collective one for all those hearts in which the atria connect to only 1 ventricle. Confusion will be completely removed if individual hearts are described for what they are in terms of AV connection and ventricular morphology (for example, double-inlet left ventricle with rudimentary right ventricle rather than single ventricle with outlet chamber).

Heart Atria↗

Atrioventricular septal defect with intact atrial and ventricular septal structures.

A heart with an atrioventricular septal defect is characterized by absence of the atrioventricular muscular and membranous septa, a common atrioventricular junction, an unwedged position of the aortic valve annulus and disproportionate inlet--outlet dimensions of the ventricular septum. The clinical and post-mortem findings are described of a case which had intact atrial and ventricular septa but had all the other anatomical hallmarks of atrioventricular septal defect. The problems in clinical diagnosis may be overcome by cross-sectional echocardiography.

Cardiomegaly↗

Surgical anatomy of double-outlet right ventricle--a reappraisal.

In light of the recent developments in surgical treatment of double-outlet right ventricle, the anatomic observations on this lesion were reevaluated. For this review, double-outlet right ventricle was diagnosed when more than half of both arterial valves were connected to the same ventricle, although appreciating the reasons for using, in a clinical context, a "90% rule" rather than the "50% rule" used in this review. Although this ventriculo-arterial connection can exist with any segmental combination, most often it is found in the setting of usually arranged atrial chambers (solitus) and atrioventricular concordance. Categorization of this subset is then done on the basis of the relationships of the arterial trunks. Three main groups stand out: intertwining arterial trunks and "normally related" arterial valves, and parallel trunks, but with the aortic valve to the right side or left side. These groupings give information concerning the site of the ventricular septal defect, which in any group may be perimembranous, muscular or be doubly committed and subarterial. Infundibular morphology is also variable, and the proximity of the arterial valves to the roof of the defect is largely determined by the extent of the ventriculo-infundibular fold.

Heart Defects, Congenital↗

Double-inlet left ventricle with rudimentary right ventricle and ventriculoarterial concordance.

Seven hearts were studied that had in common the segmental combination of double-inlet left ventricle, rudimetary right ventricle and ventriculoarterial concordance. The prototype of these hearts has been described as the "Holmes heart" or "single ventricle type A1." Five of the hearts had 2 atrio-ventricular valves; in 1 of these the right valve was minimally straddling. The other 2 had a common valve. In 6 of the hearts the rudimentary right ventricle was obliquely situated on the anterior surface of the dominant left ventricle. The trabecular component was anterior and right-sided with the outlet components and pulmonary trunk in left-sided position. In the final case, both trabecular and outlet components of the rudimentary right ventricle were left-sided. Conduction tissue studies were performed in 2 hearts. In 1, the connecting atrioventricular node and bundle were in anomalous anterolateral position, as expected in double-inlet left ventricle. In the other, a ring-like, posterior, nonbranching bundle connected the regular node in the atrial septum to the branching bundle positioned on the anterior interventricular septum. The anatomic findings are related to the likely modes of presentation and differential diagnosis while the conduction tissue findings are discussed in the light of options for surgical repair. Thus, the term "single ventricle" is best avoided when describing hearts that unequivocally possess 2 ventricular chambers.

Heart Conduction System↗

Pulmonary artery growth during treatment with oral prostaglandin E2 in ductus dependent cyanotic congenital heart disease.

Growth of the pulmonary arteries was assessed in 11 patients with cyanotic congenital heart disease treated with oral prostaglandin E2. Each patient was dependent on patency of the ductus arteriosus for maintenance of the pulmonary circulation. Measurements were made initially from angiographic data obtained in the neonatal period. Subsequent assessments were made at the time of surgery after an interval of 2-8 months, during which time all the infants had gained weight normally. Comparisons were made with data obtained from 13 normal necropsy specimens. In only 4 of the 11 patients had the pulmonary arteries grown appreciably during the treatment period. In 2 patients no growth occurred, while in 5 patients the pulmonary arteries had actually become smaller. It had been hoped that the pulmonary arteries would grow in all 11 patients during this period, facilitating later surgical intervention. In patients with ductus-dependent cyanotic congenital heart disease, the prolonged use of oral prostaglandin E2 should be restricted to patients in whom the pulmonary arteries are too small to allow a palliative operation to be performed initially.

Body Weight↗