Ventricular septal defect associated with aneurysm of the membranous septum.

The most common variety of ventricular septal defect, a perimembranous defect, is frequently associated with a so-called aneurysm of the membranous septum. Previous studies have suggested that ventricular septal defects associated with an aneurysm of the membranous septum tend to spontaneously decrease in size or close more than defects without such an aneurysm. To better define the natural history of this entity, clinical and catheterization data from 87 patients with ventricular septal defect and aneurysm of the membranous septum were reviewed. The initial evaluation was made at a median age of 0.3 years (range 0.1 to 11), with the final evaluation at a median age of 10 years (range 1.5 to 20) and a median duration of follow-up of 8.6 years (range 1.2 to 18.8). Approximately 75% of the ventricular septal defects had a small or no left to right shunt at last evaluation. Overall, 48 patients (55%) had no significant change in the size of the defect, and 39 (45%) showed improvement during the period of observation. Only four patients (5%) had spontaneous closure of the defect. Of the 49 patients who presented with a large left to right shunt, with or without congestive heart failure, 23 (47%) had persistence of a shunt large enough to warrant surgery. When spontaneous improvement occurred, it did so by 6 years of age in all but one patient. Therefore, a continued tendency for a ventricular septal defect associated with an aneurysm of the membranous septum to spontaneously decrease in size or close after this age may be less likely than previously suggested.(ABSTRACT TRUNCATED AT 250 WORDS)

Arrhythmias and Stokes-Adams attacks in acute rheumatic fever.

In spite of general complacency about first-degree heart block in acute rheumatic fever, abnormal conduction with dysrhythmias, occasional complete heart block, and, rarely, Stokes-Adams attacks are important early signs of acute rheumatic fever and may precede other signs. Every person with episodic fainting is entitled to an ECG, and frequent ECGs are imperative in any case of rheumatic fever with signs of arrhythmias. Changing atrioventricular block necessitates continuous monitoring for dysrhythmias. A 13-year-old boy who appeared with Stokes-Adams attacks secondary to acute rheumatic fever was successfully treated by temporary pacing.

Mechanisms of closure of perimembranous ventricular septal defect.

Although more defects in the muscular ventricular septum close spontaneously than do defects abutting on the membranous septum (perimembranous defects), a good proportion of the latter show a tendency to diminish in size and to close. The mechanism responsible for this closure is usually described as an aneurysm of the membranous septum, although some doubt has been cast as to whether this is the precise mechanism. In this study, 69 unoperated hearts with ventricular septal defect were examined; 26 (38%) showed some evidence of partial closure and none extended exclusively into the outlet part of the septum. Two thirds of the trabecular perimembranous defects, almost half of the inlet defects, and one third of the confluent defects showed signs of partial occlusion. The anatomic structures most frequently responsible were reduplication of tricuspid valve tissue (16 cases), adhesion of tricuspid valve leaflets (6 cases), and prolapse of an aortic valve leaflet (3 cases). Only in 2 cases were subaortic tissue tags observed which might have originated from the remnant of the membranous septum. The present study shows that although anatomic evidence of partial closure was present in two fifths of the hearts studied, only rarely was it due to so-called aneurysm of the membranous septum.

Hemodynamic function after the Mustard operation for transposition of the great arteries.

Pre- and postoperative cardiac catheterization data and cinenangiocardiograms of 82 patients who survived the Mustard operation for transposition of the great arteries (TGA) were reviewed. The post-operative catheterizations were performed 20 days to 10 years after operation (mean 2.5 years). Forty-six patients (56%) had no or insignificant associated cardiac lesions, whereas 36 (44%) had ventricular septal defect, pulmonary stenosis, or both, and required surgical intervention at the time of the Mustard operation. Postoperatively, 11 patients (13%) had significant systemic venous obstruction. Of the 11 patients, 6 required reoperation, and 2 patients had evidence of restenosis or complete obstruction in the superior vena cava after reoperation. In most patients, superior vena caval obstruction was well tolerated even in the presence of high pressure in the superior vena cava. Pulmonary venous obstruction occurred in 5 patients (6%), 3 of whom had no clinical symptoms despite severe pulmonary venous obstruction, although all had radiographic evidence of pulmonary venous congestion. The incidence of obstruction was drastically reduced after the Mustard operation was modified to include routine enlargement of the pulmonary venous atrium. Tricuspid regurgitation was uncommon (10%), but did occur in patients who had transatrial closure of a ventricular septal defect. Preoperatively, left ventricular outflow obstruction occurred in 38%. In 12 patients an attempt was made to relieve the obstruction at surgery. The 6 patients who had localized obstruction had a good result, but patients with more diffuse narrowing of left ventricular outflow had little or no relief of obstruction. Mild to moderate left ventricular outflow gradients regressed spontaneously in most patients after the Mustard operation.

Arrhythmias in transposition of the great arteries after the Mustard operation.

Disorders of rhythm or conduction in patients with transposition of the great arteries (TGA) after the Mustard operation have been widely reported. This study provides a systematic evaluation of the electrophysiologic function of 87 survivors of the Mustard operation at a single institution. Surface electrocardiograms were reviewed in all 87 patients, Holter monitoring data in 26 patients, exercise electrocardiograms in 21 patients, and invasive electrophysiologic data in 61 patients. Surface electrocardiograms showed normal sinus rhythm in 52%, sinus node dysfunction in 27%, and atrioventricular block in 16%. Holter monitoring was obtained in an unselected subgroup of 26 patients who had a mean age of 12 years and a mean interval from operation of 9 years. Sinus node dysfunction was found in 58%, atrioventricular block in 27% ventricular ectopy in 50%, supraventricular ectopy in 27%, and no abnormalities in only 8%. Intracardiac electrophysiologic evaluation showed a high frequency of abnormal sinus node recovery times and suboptimal response of the atrioventricular-conduction system to rapid atrial pacing. When all modalities used in this study were considered, sinus node dysfunction occurred in 47%, ectopy in 34% and atrioventricular block in 23%. Although only 30% of patients had no evidence of arrhythmia, symptoms of rhythm or conduction disturbances were rare.

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.

Electrocardiogram in double chamber right ventricle.

Thirty patients with a double chamber right ventricle were seen during a 7 year period. The majority of patients had associated cardiac anomalies, most commonly a ventricular septal defect. In this series, as in others, the history, clinical examination, chest roentgenogram and echocardiogram were inconclusive for diagnosing double chamber right ventricle. Symptoms were generally related to the severity of the associated anomalies. In 40 percent of the patients reviewed, upright T waves were found in lead V3R as the only electrocardiographic finding suggestive of right ventricular hypertrophy. Because upright T waves in lead V3R in the absence of other evidence of right ventricular hypertrophy are not commonly seen in patients with an isolated ventricular septal defect or a defect associated with other forms of right ventricular outflow obstruction, this finding should alert the cardiologist to the possibility of the presence of double chamber right ventricle prior to cardiac catheterization.

Tetralogy of Fallot with absent pulmonic valve: analysis of 17 patients.

Seventeen patients with tetralogy of Fallot (TF) and absent pulmonic valve were seen at Children's Hospital of Pittsburgh from 1958 to 1981. In 7 patients, severe respiratory symptoms developed due to bronchial compression by an aneurysmal pulmonary artery, and 6 died. In 4 patients, moderate respiratory symptoms developed. Six patients remained free of respiratory distress. Medical and surgical management of patients with respiratory distress in infancy has been frustrating and frequently unsuccessful. A 4-month-old infant with respiratory distress underwent complete intracardiac repair with valve insertion and has subsequently survived with relief of symptoms. Considering the high mortality rate in this group of patients, further attempts at aggressive surgical repair in infancy for patients with severe respiratory symptoms is warranted. An anatomic review of pathologic specimens with this anomaly confirmed previous reports of the frequent association of absence of the ductus arteriosus in patients with tetralogy of Fallot and absent pulmonary valve. The exception is noted in patients with discontinuity between the right and left pulmonary arteries in which a ductus arteriosus may be present supplying the isolated left pulmonary artery.

Absent right superior vena cava with persistent left superior vena cava: implications and management.

Seven cases of absent right superior vena cava with persistent left superior vena cava and normal situs were diagnosed at Children's Hospital of Pittsburgh. All patients had associated cardiac defects. In two cases the diagnosis was made at autopsy, the first in 1957 and in a 26 day old infant with multiple cogenital defects and the second in 1965 in a 22 day old infant who had pulmonary atresia with ventricular septal defect and patent ductus arteriosus. Since 1966 absent right superior vena cava has been diagnosed at cardiac catheterization in five children. Three of these children have had surgery, two for subaortic stenosis and one for an atrial septal defect. One has an insignificant atrial septal defect and the fifth has a ventricular septal defect. The electrocardiogram of four reveals s short P-R interval and a leftward frontal plane axis of the P wave, suggesting a low atrial focus. None has had any significant conduction problem. All five children are living and well, the oldest has survived 13 years postoperatively. Certain precautions are necessary should corrective cardiac surgery or transvenous pacemaker insertion be necessary.

Exercise studies after the Mustard operation in transposition of the great arteries.

Twenty-one postoperative patients with transposition of the great arteries (TGA) underwent graded exercise testing 4 to 15 years (mean 9) after the Mustard operation. No patient had subjective exercise intolerance before testing, although some had symptomatic resting arrhythmias. Correlations were made between cardiac catheterization data and 24-hour ambulatory monitoring. Exercise tolerance was diminished in nearly half of the patients. No statistically significant differences were found in heart rate or blood pressure responses, but maximal oxygen consumption values were lower than the control values (p less than 0.001) in the larger patients. Arrhythmias were present or provoked in most patients during exercise testing. Only 28% remained in normal sinus rhythm during and after exercise. Multifocal premature contractions were the most serious arrhythmias demonstrated. Some long-term survivors of the Mustard operation may have abnormal exercise dynamics, even though they may be asymptomatic and have normal physical activities and endurance. Mean maximal systolic blood pressure, heart rate, oxygen consumption, and maximal treadmill times were consistently in the low-normal range or were statistically lower than normal.

The role of coronary artery abnormalities in the prognosis of truncus arteriosus.

A high incidence of coronary ostial and arterial abnormalities was found in a study of 30 pathologic specimens of classic truncus arteriosus at Children's Hospital of Pittsburgh. The following were of special note: (1) left coronary ostium in a posterior and high position; (2) close relation of the left coronary ostium to the pulmonary artery segment in three-leaflet truncal valves; (3) stenosis of the coronary ostium caused by small size, slitlike shape, or the location of the ostium above or in a commissure; (4) the acute angle takeoff of the coronary artery; (5) the position of the left anterior descending artery as it courses posteriorly and close to the truncal wall, and then to the left of the interventricular septum; (6) the size and course of the conal and diagonal arteries from the right coronary artery across the right ventricular outflow area; (7) other coronary abnormalities, including a single coronary artery or ostium with branches crossing the right ventricle below the truncus, the circumflex arising from the right coronary artery and coursing behind the truncus, and the right coronary artery originating from the left anterior descending artery and vice versa. Eight heart specimens with conduit repair were reviewed, and all had injury to coronary arteries, possibly responsible for or contributing to the deaths of six of the eight patients. Coronary abnormalities, often several occurring in combination, may contribute to high operative mortality rate and may be a cause of late sudden death in truncus arteriosus. Surgical procedures should be planned with a view to protecting coronary arteries in the region of the right ventricular outflow tract below the truncus. Coronary artery obstruction (ostial or luminal) can occur and may need to be addressed as a separate issue during surgical procedures.

Surgical anatomy of tetralogy of Fallot.

On the basis of previous experience, we have analyzed the anatomy of a series of 53 hearts catalogued as having tetralogy of Fallot in the museum of the Children's Hospital of Pittsburgh. All hearts had a ventricular septal defect, aortic overriding, infundibular pulmonary obstruction, and right ventricular hypertrophy. None of the hearts had been operated upon during life. We paid particular attention to features of surgical importance. Thus the ventricular septal defect was found to be of variable form. It was perimembranous in 42 hearts. In 11 hearts it had entirely muscular rims and in the remaining heart it was roofed by the conjoined aortic and pulmonary valve rings (subarterial). From our previous histologic experience, it was evident that this varying morphology significantly affected the surgical anatomy of the atrioventricular conduction tissues. We endeavored to display this disposition as might be viewed by the surgeon. The degree of aortic override was variable, the aortic valve being connected by 15% to 95% to the right ventricle. In 17 hearts more than half the aortic valve was attached to right ventricular musculature. The nature of the infundibular obstruction also varied markedly. In all cases (except the one with absent infundibular septum) the infundibular septum was deviated in cephalad and anterior direction so as to produce obstruction. In the majority of hearts further anatomic structures also contributed to the obstruction. In some hearts there was hypertrophy of the infundibular septum, particularly at its junction with the trabecular septum. In others there was hypertrophy of the trabecula septomarginalis, and in many hearts there was hypertrophy of additional anterior infundibular trabeculations. The pulmonary valve was the narrowest point of the outflow tract in only six hearts, but in many of the others, it was abnormal. The results emphasize that while hearts may exhibit the classical features of tetralogy of Fallot, there is usually considerable individual variation in each of these features which is of major surgical significance.

Double-outlet right ventricle with tunnel from left ventricle to aorta.

The successful surgical correction of an unusual form of double-outlet right ventricle (DORV) in a 4-year-old boy is described. A long tunnel-like structure was present between the left ventricle and the aorta and also communicated with the right ventricle. hemodynamic and angiographic evaluation demonstrated restrictive communications between the tunnel, the aorta, and each of the ventricles. A pathological concept explaining the anatomic abnormality seen in this patient is discussed.

Left aortic arch with right descending aorta and right ligamentum arteriosum. A rare form of vascular ring.

The combination of left aortic arch, right descending aorta, and right patent ductus arteriosus is a rare form of aortic arch anomaly. Only 11 such cases have been previously reported. A 6-month-old infant with this anomaly manifested severe respiratory difficulty and feeding problems in the early newborn period. The aortic arch abnormalities were confirmed by angiocardiography, and operative correction was successful. This is the first case reported in which the patient developed severe respiratory and feeding difficulties due to this type of a vascular ring.

Bilateral ductus arteriosus in d-transposition of the great arteries with right aortic arch.

The rare anomaly of a right aortic arch, distal origin of the left subclavian artery, and posterior left ductus arteriosus (forming a vascular ring) plus a right ductus arteriosus is described in an infant with d-transposition of the great arteries with an intact septum. The presence of a right aortic arch and distal left subclavian artery with bilateral ductus arteriosus has not been described previously.

Pneumonectomy for intractable left bronchial compression in d-transposition of the great arteries.

In infants with transposition of the great arteries (TGA) tracheobronchial obstruction may occur as a result of compression by a dilated posterior pulmonary artery, especially if there is a large interventricular septal defect with its attendant large pulmonary blood flow and high pressure. Banding of the pulmonary artery may give temporary relief. A patient with TGA had collapse of the left lung at 2 months of age, following atrial septectomy. Bronchoscopy and bronchography revealed extrinsic compression of the left main bronchus. Banding of the pulmonary artery at 6 months of age relieved the respiratory distress. At 8 years of age atelectasis of the left lung recurred following a Mustard procedure. Two additional procedures were attempted to decrease the pressure of the pulmonary artery on the bronchus but these were unsuccessful. At age 9 years, pneumonectomy, her sixth thoracotomy, was performed as a final attempt to relieve the respiratory problem caused by persistent atelectasis. She is now 17 years of age and has remained asymptomatic during the last 8 years.

Management of bilateral ductus arteriosus in complex cyanotic heart disease.

Bilateral ductus arteriosus (BDA) usually is associated with complex cyanotic heart disease. Since pulmonary valve atresia often is part of the complex, hypoxia may necessitate emergency cardiac catheterization and surgery for these critically ill newborn infants. Optimum management depends on accurate delineation of the intracardiac and great vessel anatomy. Since the ductus arteriosus has a tendency to close spontaneously, the true anatomy of the fourth to sixth aortic arch connections should be determined on the first catheterization. An over-all plan for future care by the medical-surgical team should have been made at the time of the initial surgical procedure. The case histories of four newborn infants with BDA associated with cyanotic heart diseases are reported. The anatomy and basic embryology of the fourth to sixth arch system is reviewed and recommendations for long-term management are given.

Systemic-pulmonary artery anastomoses in infancy.

Over the last 10 years, 142 systemic-pulmonary artery anastomoses were performed in 134 infants under 1 year of age. These included shunts from the subclavian artery (46 per cent), ascending aorta (41 per cent), descending aorta (9 per cent), and superior vena cava (1 per cent) to the pulmonary artery. Ninety-six (72 per cent) of the infants were less than 4 months of age at the time of the initial procedure, and one half were less than 1 month old. When groups of patients were compared according to diagnosis and age at the time of operation, the immediate and long-term results of the subclavian-pulmonary artery (Blalock-Taussig) anastomosis were better than the results with the other procedures. In view of the ease of closure of this anastomosis at the time of corrective surgery, as well as the recent further improvement of vascular surgical techniques, the Blalock-Taussig shunt is the procedure of choice in most infants requiring a systemic-pulmonary artery anastomosis, even during the early months of life.

Valve replacement in children and adolescents.

Replacement of diseased heart valves has become the treatment of choice in many adults. Valve replacement in older children and adolescents has been done in our institution only when other procedures are ineffective. Over the past 10 years, 24 patients have received 28 artificial valves. Thirteen (54 per cent) had rheumatic heart disease; the remainder had congenital or acquired valvular abnormalities. The Björk-Shiley valve was most commonly used in the aortic position and the Beall prosthesis in the mitral position. Sixteen patients (67 per cent) survived operation and have been followed from 3 to 79 months, averaging 27 months. All are free of cardiac symptoms. Hemarthrosis occurred once in 2 survivors (13 per cent). Eight patients died (33 per cent), 5 immediately postoperatively and 3 within a year after the operation. The mortality rate has declined to 21 percent during the past 3 years. Our experience in the pediatric age group suggests that valve replacement is a serious undertaking with higher mortality rate than in adults. The operation should be reserved for those patients in whom valvuloplasty or valvulotomy is expected to be ineffective.

Conduction tissue anomalies in absence of the right superior caval vein.

The incidence of electrical instability of the heart is high in patients with absence of the right and persistence of the left superior caval vein when the latter connects to the coronary sinus. It has been suggested that a large coronary sinus may influence the susceptibility to arrhythmias. we studied the conduction tissues of 8 hearts from the cardiopathological collection of Children's Hospital of Pittsburgh. Six of these specimens had a persistent left superior caval vein connecting to the coronary sinus. There were 4 with absence of the right superior caval vein, 3 of which were in the group with persistent left veins. We evaluated the sinus node and the specialized atrioventricular junctional area, comparing them with known normals. The coronary sinus varied in size from through it. The size did not alter the histology of the adjacent conduction tissue. In contrast, the sinus node was abnormal in 3 of the 4 hearts with absent right superior caval vein. This may be the key factor in the development of arrhythmias when the right superior caval vein is absent or abnormal.