James W. Dushane

Relation between Structural Changes in the Small Pulmonary Arteries and the Immediate Reversibility of Pulmonary Hypertension Following Closure of Ventricular and Atrial Septal Defects

The relative importance of organic changes and vasoconstrictive influences in the production of increased pulmonary vascular resistance in congenital heart disease is problematic. With use of a grading system previously described, a study was made of the relation between each of the grades of hypertensive pulmonary vascular disease associated with ventricular or atrial septal defects and the change of pulmonary artery blood pressure immediately following closure of the abnormal communications. The immediate reversibility of the pulmonary hypertension varies inversely with the severity of the structural changes.

Graded Pulmonary Vascular Changes and Hemodynamic Findings in Cases of Atrial and Ventricular Septal Defect and Patent Ductus Arteriosus

With use of a previously described system of grading, a study was made of the relation between each of the grades of hypertensive pulmonary vascular disease and the hemodynamic findings in the lesser circulation in 15 cases of atrial septal defect, 19 cases of ventricular septal defect, and 6 cases of patent ductus arteriosus.

Prognosis for Patients with Ventricular Septal Defect and Severe Pulmonary Vascular Obstructive Disease

The status of 58 patients, 3 to 57 years of age, with ventricular septal defect and a marked increase in pulmonary vascular resistance (pulmonary obstructive disease) was determined 5 years or longer after diagnostic catheterization. Forty-six (80%) were alive 5 years after that study. The probability of living 5 years was 95% for patients aged 10 through 19 years and 56% for those 20 years of age or older. The dissimilarity of actuarial curves between these two groups is consistent with the hypothesis that pulmonary vascular obstructive disease develops under age 20. Calculated pulmonary resistance increased with age of patient on admission to the study, suggesting its progressive nature. Hemoptysis was rare under age 20, and its presence over this age indicated a poor prognosis. The most common causes of death were sudden or unknown. Most surviving patients were gainfully employed or attending school without severe symptoms.

Isolated Ventricular Septal Defect of the Persistent Common Atrioventricular Canal Type

Among 60 necropsy specimens of isolated ventricular septal defects and more than 300 cases of ventricular septal defect observed at operation, 15 cases demonstrating unusual anatomic positions of the defect were found. The ventricular defect differed from the ordinary ventricular septal defect in that it usually occupied the position of the ventricular component of the defect in persistent common atrioventricular canal. For this reason it was named ventricular septal defect of the persistent common atrioventricular canal type. Deformities of one or both atrioventricular valves were common (nine of 15 cases). No atrial septal defects of the ostium primum type were present. Anatomic studies of the conduction tissue revealed that this tissue skirted the posterior and inferior aspects of the ventricular septal defect and that, as in persistent common atrioventricular canal, the course taken by the conduction tissue was unusually long, as a result of the peculiar posteroinferior position of the lower edge of the defect. The electrocardiographic features were striking. In all cases the mean electrical axis of the QRS lay above the isoelectric point; the vector loop obtained in the frontal plane from the scalar electrocardiogram was directed counterclockwise, and its main mass was above the zero line. In addition, in all cases there were signs of right ventricular overload, and in some cases of left ventricular overload as well. Electrocardiographic findings of this pattern have been thought by some authors to be diagnostic of persistent common atrioventricular canal, but we observed that they also occurred in each of the cases of isolated ventricular septal defect of the variety described herein. We recognize that in the usual variety of ventricular septal defect this electrocardiographic pattern occurs, but it does so uncommonly. We have not studied its exact incidence. The anatomy, hemodynamics, and surgical considerations are different in cases with this defect from those with persistent common atrioventricular canal. The surgical risk in these cases has been higher than that in the usual type of ventricular septal defect. In the discussion of the electrophysiologic theories that seek to explain the unusual electrocardiographic patterns in this group of cases, a new theory is offered, based on studies of the conduction system. In our opinion, the different orientation of the advancing fronts of depolarization is the result of congenital displacement of the bundle of His in its relation to the ventricular septal defect.

Congenital Insufficiency of the Pulmonary Valve: Including a Case of Fetal Cardiac Failure

Congenital insufficiency of the pulmonary valve may result if the valvular cusps develop faultily or fail to develop. It is commonly associated with ventricular septal defect, with or without pulmonary stenosis. Under these conditions a continuous precordial murmur may be present. Less commonly, congenital pulmonary valvular insufficiency may appear as an isolated cardiac malformation.

Obstruction of Left Ventricular Outlet in Association with Ventricular Septal Defect

In a pathologic collection of 722 congenital heart lesions we have observed 10 cases in which a ventricular septal defect was associated with obstruction to normal left ventricular outflow. These cases are presented in the framework of a classification relating hemodynamics to the position of the obstructing lesion and the size of the ventricular septal defect. One additional case observed clinically and at operation is also reported to make more nearly complete the classification of the anatomic arrangements in which the common denominators are ventricular septal defect and obstruction to normal ventricular outflow.

Aortic Sinus Fistula—Surgical Considerations and Results of Operation

Abstract From 1956 to 1971, 21 Mayo Clinic patients underwent surgical correction of aortic sinus fistula. The 12 male and 9 female patients were from 3 to 52 years old (median 27 years). In 16 patients the right aortic sinus had ruptured into the right ventricle, and in 1, into the right atrium; in 4 patients a noncoronary sinus fistula communicated with the right atrium. The appearance of 4 fistulas was related to trauma, but all 21 fistulas were basically congenital in origin. Associated lesions were common; 15 patients had one or more of the following: ventricular septal defect (VSD) (14 patients), aortic insufficiency (7 patients), and pulmonary stenosis (3 patients). All patients survived operation. Three required reoperation: 1 for closure of a recurrent VSD (deterioration of an Ivalon sponge patch), 1 for recurrence of the fistula and VSD, and 1 to replace a malfunctioning Teflon cusp with a prosthetic valve. Follow-up has been carried out for as long as fourteen years (median seven years) after operation. One patient has moderate aortic insufficiency but remains well compensated; the remaining patients are free of symptoms and clinical signs of cardiac dysfunction. Currently, an aortic sinus fistula should be repaired when it is diagnosed because the disability is progressive, operative risk is negligible, and long-term results are excellent.

Heart Block after Repair of Ventricular Septal Defect in Children

From March 1955 to April 1959, 174 patients with ventricular septal defect and 124 patients with ventricular septal defect associated with pulmonic stenosis were referred from the Section of Pediatrics of the Mayo Clinic for open intracardiac repair. Forty-eight of these 298 patients had complete heart block at some time following operation. Eighteen of the patients with heart block died in the immediate postoperative period, 18 had reversion to normal sinus rhythm, and 12 were discharged from the hospital with complete heart block. Three of these 12 patients died subsequently. Two died presumably as a direct result of complete heart block and a third died following a second open cardiac operation. Of the 9 surviving patients, 3 have had serious bradycardia or periods of asystole and 6 have no symptoms from heart block. One of 3 surgical technics was employed for all the patients in this study with variation in incidence of block with the use of each technic. Other factors that might relate to the incidence of heart block are discussed. The management of the patient with complete heart block both in the immediate post-operative period and after dismissal from the hospital is described.

Differentiation of Interatrial Communications by Clinical Methods Ostium Secundum, Ostium Primum, Common Atrium, and Total Anomalous Pulmonary Venous Connection

Of 128 children with anatomically confirmed interatrial communications 74 had ostium secundum, 37 had ostium primum defects, 5 had common atrium, and 12 had total anomalous pulmonary venous connection. Analysis of the medical history, physical examination, roentgenogram, and electrocardiogram resulted in accurate diagnosis in most instances. In 6 of the 128 patients the proper diagnosis was not made: 3 infants with total anomalous pulmonary venous connection died within a few hours after admission to the hospital and did not have proper diagnostic evaluation; the condition of 3 other patients with ostium secundum defects was misdiagnosed as ostium primum defect before surgical treatment. In patients with ostium secundum and ostium primum defects the symptoms, signs, roentgenologic data, and findings at cardiac catheterization are similar, but differences in respect to the electrocardiograms are striking when the latter are subjected to vectorial analysis. The QRS loop is clockwise in the presence of ostium secundum defect and counterclockwise when ostium primum defect is at hand. Clinical and roentgenologic manifestations also are noted among patients with common atrium and total anomalous pulmonary venous connection, and the findings at cardiac catheterization likewise may be similar, but the electrocardiographic differences are significant. The QRS loop is counterclockwise in patients with common atrium and clockwise in patients with total anomalous pulmonary venous connection.

Tetralogy of Fallot with Anomalous Tricuspid Valve Simulating Pulmonary Stenosis with Intact Septum

An anatomicopathologic entity of the tetralogy of Fallot with an additional finding of an accessory flap of tricuspid valvular tissue is described. In the 3 cases reported in this paper, the outstanding hemodynamic finding was a right ventricular systolic pressure that was significantly greater than the systemic systolic pressure. The hemodynamic findings were explained by the fact that the accessory tricuspid flap partially closed the large ventricular septal defect. The clinical picture was similar to that of either severe pulmonary stenosis with an intact ventricular septum or severe pulmonary stenosis with a small ventricular septal defect and a right-to-left shunt at the ventricular level.