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Biomedical subjects

W D Edwards

Publications and source records attributed to W D Edwards.

At least 235 records · Page 13Linked to original sources

Standardized nomenclature of the ventricular septum and ventricular septal defects, with applications for two-dimensional echocardiography.

The anatomy of the ventricular septum is reviewed, and ventricular septal defects are classified on the basis of their position and the presence or absence of septal malalignment. The standardized nomenclature for the various types of ventricular septal defects is presented, and analysis of these defects by two-dimensional echocardiography is described.

Echocardiography↗

Cardiac sarcoidosis: a potentially treatable form of myocarditis.

A 39-year-old woman had a 2-year history of heart block, which had necessitated pacemaker implantation, and a 6-month history of heart failure. Endomyocardial biopsy specimens initially revealed lymphocytic myocarditis but subsequently showed giant cell myocarditis. She died suddenly, and autopsy disclosed extensive cardiac sarcoidosis with minimal extracardiac involvement. Cardiac sarcoidosis may be difficult to diagnose clinically because the extent of cardiac and the extent of extracardiac involvement tend to be inversely related. Endomyocardial biopsy may be helpful in diagnosing such cases.

Adult↗

Primary pulmonary hypertension: a histopathologic study of 80 cases.

Among 80 cases with a clinical diagnosis of primary (unexplained) pulmonary hypertension, 45 (56%) had thromboembolic disease and 22 (28%) had plexogenic arteriopathy; the remaining 13 (16%) had pulmonary veno-occlusive disease, primary medial hypertrophy, primary pulmonary arteritis, or changes consistent with pulmonary venous hypertension. The mean age was 16 years for primary pulmonary arteritis, 21 to 34 years for plexogenic pulmonary arteriopathy, primary medial hypertrophy, and pulmonary veno-occlusive disease, and 41 and 45 years for thromboembolic disease and pulmonary venous hypertension, respectively. In all forms except pulmonary veno-occlusive disease and apparent pulmonary venous hypertension, female patients were involved twice as often as male patients. With the exception of apparent pulmonary venous hypertension, patients with plexogenic pulmonary arteriopathy had the longest survival (63 months). Sudden death, however, occurred most frequently in patients with plexogenic disease (45%) and occurred 2.5 times as often in this group as in patients with thromboembolic disease. Among our 80 cases, the most frequent histopathologic lesions were medial hypertrophy, intimal proliferation and fibrosis, fibrinoid degeneration and necrosis, and thrombosis. Thrombi were commonly observed and may have developed in situ or by embolization; they were often rich in platelets when they occurred in small pulmonary vessels. These histologic features may form the morphologic substrate for elevated pulmonary vascular resistance, and their recognition may provide the rationale for possible intervention with pulmonary vasodilators, anticoagulants, or platelet inhibitors.

Adolescent↗

Two-dimensional echocardiographic spectrum of univentricular atrioventricular connection.

The spectrum of anomalies in hearts having a univentricular atrioventricular (AV) connection was examined by two-dimensional echocardiography in 183 patients and the anatomic findings were compared with angiography. The mode of AV connection was found to be of three types: double inlet via two AV valves; single inlet via one AV valve with absence of the other (left or right AV valve atresia); and common inlet via a common AV valve. Identification of an accessory chamber by two-dimensional echocardiography was possible with 90% sensitivity, but it was limited compared with angiography in patients with severely hypoplastic anterior chambers and pulmonary valve atresia. All patients with subaortic outlet foramen obstruction were detected. Great artery position and the presence of obstruction to pulmonary flow were correctly predicted in all but one patient. Two-dimensional echocardiography was superior to angiography for the detection of AV valve abnormalities which were present in 27% and included abnormal chordae, hypoplasia or dysplasia of either valve. Two-dimensional echocardiography should play an essential role in the complete preoperative assessment of patients with univentricular AV connection.

Adolescent↗

Pulmonary blood supply in patients with pulmonary atresia and ventricular septal defect.

The heart and lung specimens in 31 cases of pulmonary value atresia and ventricular septal defect were studied at autopsy. Three types of natural arterial blood supply to the lungs were identified: 1) ductus arteriosus (patient or ligamentous) (12 cases); 2) major collateral arteries (20 cases); and 3) diffuse small pleural arterial plexus coexisting with either ductus arteriosus or major collateral arteries (17 cases). The ductus arteriosus and major collateral arteries did not coexist in the same lung in these cases. Confluent central pulmonary arteries were present in 22 (71%) of the 31 cases, involving 7 (58%) of the 12 cases of ductus arteriosus, 14 (70%) of the 20 cases with major collateral arteries and 1 case with an aorticopulmonary window. The pulmonary trunk (atretic or patent) was identifiable in 24 (77%) of the 31 cases. A lung or lungs that connected to a ductus (or ligamentum) had a complete and unifocal intrapulmonary arterial distribution (without arborization abnormalities). Major collateral blood supply was frequently multifocal and associated with arborization abnormalities. The size of the central pulmonary arteries was not related to the type of arterial blood source but seemed to be related to the amount of blood flow actually reaching the vessels, This study demonstrated a complex systemic arterial system supplying the lungs in these cases. The size, sources and relation among the ductus, the pulmonary artery confluence, the large and small collateral vessels and the intrapulmonary system are far more varied than has ever been reported previously. Careful and thorough premortem studies are crucial if surgical intervention is contemplated.

Adolescent↗

Diagnosis and classification of atrial septal aneurysm by two-dimensional echocardiography: report of 80 consecutive cases.

Atrial septal aneurysms have been related (either by association or as potential causes) to systolic clicks, atrial arrhythmias, systemic and pulmonary embolism, atrioventricular valve prolapse and atrial septal defect. To study these associations and the incidence of atrial septal aneurysm, we reviewed 80 consecutive patients (female to male ratio 1.9:1, mean age 47 years, range 1 day to 89 years) who had been identified prospectively as having an atrial septal aneurysm. These were found in 36,200 two-dimensional echocardiographic studies (incidence: 0.22% overall; 0.29% in the last year of the study done between 1978 and 1984). Three types of fossa ovalis aneurysm and one type of aneurysm involving the entire atrial septum were observed; a fossa ovalis aneurysm with leftward projection and excursion of less than 5 mm or an aneurysm involving the entire atrial septum with rightward projection was not observed. Atrial septal aneurysm occurred more often as an isolated abnormality than in association with other cardiac malformations, although all patients with an aneurysm involving the entire atrial septum had complex congenital cardiac anomalies of the hypoplastic right heart type. The reported associations between atrial septal aneurysms and atrial septal defect, atrioventricular valve prolapse, midsystolic clicks, atrial arrhythmias and cerebral ischemic events were examined. A hypothesis based on interatrial pressure gradients is proposed to explain the different motions and configurational characteristics of fossa ovalis aneurysms observed in these patients. All patients in whom atrial septal aneurysm is demonstrated should undergo examination for atrial septal defect. Atrial septal aneurysm should be specifically looked for in patients who have these associations and who undergo two-dimensional echocardiography, especially if these abnormalities are unexplained.

Adolescent↗

Primary cardiac tumors: experience with 30 consecutive patients since the introduction of two-dimensional echocardiography.

Experience with 30 consecutive patients who had a total of 32 primary cardiac tumors and who underwent two-dimensional echocardiographic examinations between January 1977 and June 1983 was reviewed. Most of the tumors were atrial myxomas (20 left and 4 right), and 30 were identified on echocardiography. Twenty-five patients, including 21 of 22 with atrial myxoma, underwent surgical resection on the basis of the echocardiographic examination, without preoperative angiocardiography. When the morphologic characteristics of the left atrial myxomas were studied statistically in relation to clinical abnormalities, large tumor size was most closely related to the number and type of associated clinical and laboratory abnormalities. The single exception was embolization, which correlated with echocardiographic tumor consistency. Since the introduction of two-dimensional echocardiography, the yearly incidence of cardiac tumor diagnosis at this clinic has increased several fold and the incidence of unexpected intraoperative diagnosis has been very low (one case). Echocardiography is the method of choice for clinical diagnosis. It has replaced angiocardiography for routine preoperative assessment, permits early diagnosis of cardiac neoplasms and provides insight into the pathophysiology of primary cardiac tumors.

Adolescent↗

Echocardiographic findings in systemic amyloidosis: spectrum of cardiac involvement and relation to survival.

One hundred thirty-two patients with biopsy-proven systemic amyloidosis underwent echocardiographic examination to define the spectrum of cardiac involvement. Echocardiographic abnormalities were then correlated with clinical variables and survival at follow-up. Patients were subgrouped by left ventricular wall thickness: Group I, mean wall thickness 12 mm or less; Group II, mean wall thickness greater than 12 mm but less than 15 mm; Group III, mean wall thickness 15 mm or greater; or Group IV, atypical features such as wall motion abnormalities or left ventricular dilation. Patients with greater wall thickness had a higher frequency of associated echocardiographic abnormalities such as left atrial enlargement or granular sparkling appearance on two-dimensional examination and, more commonly, reduced systolic function. The occurrence of clinical congestive heart failure was strongly correlated with greater wall thickness and multiple other echocardiographic abnormalities. Survival was negatively influenced both by greater wall thickness and reduced systolic function. The median survival of the entire group was 1.1 years. Echocardiographic examination is an important tool for establishing the presence of cardiac amyloid involvement and may be useful in estimating prognosis in such patients.

Aged↗

Sudden unexpected death in children and adolescents.

To determine the incidence and clinicopathologic spectrum of sudden unexpected death, we reviewed the death certificate of all residents of Olmsted County, Minnesota who were between 1 and 22 years of age when they died during the period from January 1950 to October 1982. Of 515 death certificates reviewed, 12 (2.3%) recorded sudden unexpected death, resulting in an incidence of 1.3 per 100,000 patient-years. The subjects ranged in age from 3 to 20 years (median 13); 8 of the 12 were male. Of the 12 deaths, 4 were definitely cardiac-related and 3 were probably cardiac-related. In the five other cases, the cause of death was unknown. Three of the 12 subjects had a history of syncope; 2 of the 3 had syncope associated with exercise, and both died while exercising. The relative rarity of sudden unexpected death in children and adolescents probably precludes population screening techniques to identify subjects at risk. However, a subset of subjects with 1) exercise-associated syncope, 2) nonvasodepressor syncope, 3) a family history of sudden unexpected death, or 4) a family history of hypertrophic cardiomyopathy deserves extensive and thorough evaluation.

Adolescent↗

Fifteen-year experience with surgical repair of truncus arteriosus.

From 1965 until March, 1982, 167 patients underwent surgical repair of truncus arteriosus. The age at operation ranged from 18 days to 33 years (mean 6 years). There were 48 hospital deaths (28.7%). The following factors had a positive correlation with the possibility of a surgical death: age at operation less than 2 years (p less than 0.001), a postrepair pulmonary arterial/left ventricular pressure ratio greater than 0.5 for patients with two pulmonary arteries (p less than 0.001) and greater than 0.6 for patients with unilateral absence of a pulmonary artery (p less than 0.02), and a postrepair right ventricular/left ventricular pressure ratio greater than 0.8 (p less than 0.008). The 119 hospital survivors were followed up for a total of 829 person-years. Late survival rate at 5 years was 84.4% and at 10 years, 68.8%. Preoperative factors that correlated with a reduced long-term survival rate were as follows: increasing age at operation (p = 0.004), the presence of moderate or severe truncal valve insufficiency (p = 0.008), lower pulmonary/systemic flow ratio (p = 0.04), and unilateral absence of a pulmonary artery (p less than 0.001). Thirty-six patients required reoperation during the follow-up period (30%) primarily for replacement of the right ventricular-pulmonary arterial conduit and/or for truncal valve replacement. The long-term results obtained in these patients support the need for early repair of the anomaly, improvement in the methods for control or repair of the truncal valve insufficiency, and the continued search for better extracardiac valved conduits.

Adolescent↗

Replacement of obstructed right ventricular-pulmonary arterial valved conduits with nonvalved conduits in children.

Six to thirty percent of right ventricular-to-pulmonary arterial (RV-PA) valved conduits in children fail within 5 years. Experience with correction of tetralogy of Fallot has shown that a competent pulmonary valve is not essential for excellent late results in most patients. Between March 1980 and November 1984, 26 patients who were 12.5 +/- 3.3 years old underwent conduit replacement 6.7 +/- 2.4 years after definitive repair of congenital heart defects in which a xenograft RV-PA valved conduit had been used. A new Dacron tube graft (n = 15) or pericardial (n = 8), dura mater (n = 2), or Dacron patch (n = 1) over the previous conduit bed was used as replacement. Preoperative mean right ventricular systolic pressure was 90.5 +/- 20 mm Hg and mean gradient across the conduit was 67.9 +/- 24.5 mm Hg. After replacement with a nonvalved conduit, right ventricular systolic pressure was reduced to 45.2 +/- 10.8 mm Hg (p less than .001), with a gradient of 8.9 +/- 7.6 mm Hg (p less than .001). There were no operative deaths. Follow-up at 19.5 +/- 14.9 months (range = 0 to 53) showed that all patients were in New York Heart Association class I (n = 21) or class II (n = 5). In the absence of pulmonary hypertension, hypoplastic pulmonary arteries, significant right ventricular dysfunction, or unrepaired tricuspid regurgitation, replacement of an obstructed conduit with a nonvalved conduit gives excellent early results and may diminish the need for late reoperation.

Adolescent↗

Ventricular septal rupture complicating acute myocardial infarction: identification of simple and complex types in 53 autopsied hearts.

Fifty-three hearts with rupture of the ventricular septum complicating acute myocardial infarction (AMI) were studied. Thirty-three of the hearts were from men (average age 76 years) and 20 were from women (average age 73 years). The study showed 2 types of rupture of the ventricular septum: simple (28 patients) and complex (25 patients). Simple ruptures were direct through-and-through defects. Complex ruptures were associated with serpiginous dissection tracts remote from the primary site of tear of the ventricular septum. Specimens were classified as to the location of the underlying AMI and the level of the septum (apex to base) at which the rupture occurred. Twenty-nine hearts had an inferior AMI and 24 an anterior AMI. Complex ruptures occurred in 20 of the inferior AMIs (69%) and in 5 of the anterior AMIs (21%) (p less than 0.001). Ruptures that involved the inferobasal portion of the septum were much more likely to be complex (94%) than ruptures in all other locations (27%, p less than 0.001). Significant 3-vessel obstructive coronary arterial atherosclerosis was present in 48 hearts. Rupture of a second structure in addition to the ventricular septum was observed in 11 hearts (left ventricular free wall in 9 cases and papillary muscle in 2). The interval from the onset of the AMI to rupture of the septum could be estimated in 22 patients and averaged 4 days (median 2.5 days). Complete heart block reportedly occurred in 6 patients during hospitalization.

Adult↗

Risk factors for aortic dissection: a necropsy study of 161 cases.

Among 161 necropsy cases of aortic dissection, 87 (54%) were type I, 34 (21%) type II, and 40 (25%) type III, and an intimal tear was identified in each. Systemic hypertension had been present in 63 of 121 cases (52%) with type I or II dissection and in 30 of 40 (75%) with type III dissection. Aortic dissection involved 7 of 16 cases (44%) with the Marfan syndrome. In the 154 cases without the Marfan syndrome, grade 3 or 4 medial degeneration (cystic medial necrosis) was observed in the ascending aorta in only 27 (18%). The risk of aortic dissection in persons with congenitally bicuspid and unicommissural aortic valves, respectively, was 9 and 18 times that in subjects with tricuspid aortic valves. The mean age of those with aortic dissection and tricuspid, bicuspid and unicommissural aortic valves was 63, 55 and 40 years, respectively, and aortic dissection was more common in men than in women. Grade 3 or 4 atherosclerosis involved the intimal tear in only 11 of 121 type I or II dissections (9%) but 32 of 40 type III dissections (80%). Accordingly, the major risk factors for aortic dissection were systemic hypertension, the Marfan syndrome, and, for type I and II dissections, congenitally bicuspid or unicommissural aortic valves. Aortic medial degeneration was a less important risk factor. Rupture of ulcerocalcific aortic atheromas may have initiated the intimal tear in some type III dissections.

Adult↗

Incidence and size of patent foramen ovale during the first 10 decades of life: an autopsy study of 965 normal hearts.

The incidence and size of the patent foramen ovale were studied in 965 autopsy specimens of human hearts, which were from subjects who were evenly distributed by sex and age. Neither incidence nor size of the defect was significantly different between male and female subjects. The overall incidence was 27.3%, but it progressively declined with increasing age from 34.3% during the first three decades of life to 25.4% during the 4th through 8th decades and to 20.2% during the 9th and 10th decades. Among the 263 specimens that exhibited patency in our study, the foramen ovale ranged from 1 to 19 mm in maximal potential diameter (mean, 4.9 mm). In 98% of these cases, the foramen ovale was 1 to 10 mm in diameter. The size tended to increase with increasing age, from a mean of 3.4 mm in the first decade to 5.8 mm in the 10th decade of life.

Adolescent↗

Mitral stenosis associated with valvular tophi.

Chronic tophaceous gout has become less common since the introduction of allopurinol and probenecid. Cardiac tophi have rarely been reported. In this article, we report a case of severe mitral stenosis in which valvular tophi played a major role in the pathogenesis. The case was well substantiated by echocardiography, surgical pathology, and chemical analysis.

Aged↗