James Manning

Early and Late Results of Repair of Partial Anomalous Pulmonary Venous Connection to the Superior Vena Cava with a Pericardial Baffle

The early and late results of repair of partial anomalous pulmonary venous connection to the superior vena cava with a simple pericardial baffle without enlargement of the superior vena cava were examined. Fifteen consecutive patients received operations between 1973 and 1983, and all survived. They have been followed for a mean of 6 years and a minimum of 2 years. Nodal rhythm and atrial dysrhythmias were present in 6 patients (40%) early after operation. However, every patient resumed normal sinus rhythm prior to hospital discharge except 1 adult who remained in the preoperative rhythm of atrial flutter-fibrillation. No patient has clinical evidence of a residual atrial level shunt or superior vena cava obstruction. All have received an excellent clinical result, and none, except the patient in chronic atrial fibrillation, require cardiac medication. These results are comparable or superior to those obtained with more intricate procedures that use either complex atrial flaps or translocation of the superior vena cava to the atrial appendage.

Echocardiography of the tricuspid valve in congenital left ventricular-riht atrial communication.

Two patients, ages 14 and 18, with congenital left ventricular-right atrial communication through a defect in the tricuspid valve were studied by echocardiography. Proof was obtained by angiocardiography and surgery in one and ultrasonic contrast injection and angiocardiography in the other. Both presented clinically as uncomplicated ventricular septal defects. Echocardiography consistently demonstrated a high frequency, low amplitude flutter of the tricuspid valve in systole and none in diastole. Following surgical correction of the defect in one patient, there was complete disappearance of the systolic flutter. Systolic flutter has not been observed with tricuspid incompetence nor with other forms of ventricular septal defects. Fistulous communication from the aorta to the right atrium just above the tricuspid valve did not demonstrate systolic flutter in one patient studied. Tricuspid valve systolic flutter appears to be caused by the passage of the left ventricular jet of blood into the right atrium through a defect in the tricuspid valve. Echocardiographic study of the tricuspid valve is of value in the recognition of the congenital left ventricular-right atrial communication.

Echocardiography in Coexisting Hypertrophic Subaortic Stenosis and Fixed Left Ventricular Outflow Obstruction

Echocardiographic study was done on four pediatric patients with coexisting hypertrophic subaortic stenosis (HSS) and fixed left ventricular outflow (LVO) obstruction. One had valvar aortic stenosis, two had discrete subaortic stenosis and one had coarctation of the aorta. Preoperatively, HSS was not suspected in any of these patients because of the classical findings of fixed LVO obstruction. The echocardiographic examination revealed an abnormal systolic anterior movement (SAM) of the anterior mitral leaflet with ventricular septal hypertrophy in all, but these findings were overlooked or thought to be related to a subvalvar diaphragm. One or two years after the surgical removal of their fixed obstruction, all showed clinical pictures of HSS. Postoperative echocardiographic examination showed an abnormal SAM with asymmetrical septal hypertrophy, and cardiac catheterization revealed mild to severe degrees of resting pressure gradients across LVO tracts. Amyl nitrite inhalation produced a more prominent SAM with increasing pressure gradient, and angiotensin infusion abolished the SAM and reduced the pressure gradient.From our present study, echocardiography has the potential for identification of HSS coexisting with a fixed LVO obstruction. We recommend that all patients with LVO obstruction have an echocardiographic examination along with pharmacological testing. The demonstration of an unusually thickened ventricular septum and especially, abnormal SAM of the mitral valve would require careful evaluation of left heart hemodynamics and the determination of the level of the major obstruction during cardiac catheterization for pre and postoperative management.

Echocardiography of the intra-atrial baffle in dextro-transposition of the great vessels.

Twelve patients with dextro-transposition of the great vessels (age eight months to four years) were studied by echocardiography following Mustards procedure. Nine of them had also been studied preoperatively. Postoperatively all patients demonstrated structural echoes in the atrial cavity behind the pulmonary root. In ten, the motion pattern generally resembled that of a stenotic atrioventricular valve iwth a sharp anterior movement followed by flattening in diastole and rapid posterior excursion in systole. The maximum amplitude of motion ranged from 4 to 9 mm (average 6.6 mm). In the remaining two cases, the anterior diastolic movement was attenuated. Similar moving, linear echoes with larger amplitudes of motion (10-14 mm) were observed behind the tricuspid valve in four patients while poorly moving, multiple or thick conglomerate echoes (2-11 mm wide) were detected in seven cases. Echocardiographic contrast studies performed by injecting indocyanine green via catheters placed on either side of the intra-atrial baffle identified it as the source of these echoes. Following operation, coarse diastolic undulations of the mitral valve (ten cases) and the tricuspid valve (nine cases) were noted. Also, fine flutter of both atrioventricular valves, not present before, appeared after operation in three patients. These findings may be related to the altered pathway of blood flow and turbulence resulting from the insertion of the baffle in the atria. Echocardiography appears useful in delineating the character and movement pattern of the intra-atrial baffle and this may have potential in evaluating its long-term functional status.

Echocardiography in Truncus Arteriosus The Value of Pulmonic Valve Detection

Nine patients with a clinical presentation suggestive of truncus arteriosus were examined by echocardiography. Subsequent intracardiac studies including cardiac catheterization and angiocardiography divided this group into five patients with truncus arteriosus and four patients with other congenital heart diseases. In all patients with proven truncus arteriosus, it was possible to identify only one semilunar valve with a large truncal vessel overriding the ventricular septum. In none of these patients could a second semilunar valve be found.This study indicates that echocardiography may provide a direct, yet noninvasive method to exclude the diagnosis of truncus arteriosus by finding the pulmonic valve. However, the diagnosis of truncus arteriosus is not tenable when based solely on the inability to demonstrate the pulmonic valve. The importance of the clinical application of this method is discussed.

Extended Aortoplasty to Relieve Supravalvular Aortic Stenosis

An extended aortoplasty was used to relieve severe supravalvular aortic stenosis in 5 patients whose preoperative left ventricular-aortic gradient ranged from 85 to 140 mm Hg (median, 120 mm Hg). The stenotic ring above the commissures was divided in two places by an inverted U incision extending into the right and noncoronary sinuses. A distal vertical incision in the ascending aorta converted this into an inverted Y. The ridge above the left coronary sinus was excised. The aortic incision was repaired with an inverted Y-shaped Dacron gusset. The postoperative gradient ranged from 0 to 30 mm Hg (median, 15 mm Hg). The extended aortoplasty provides excellent relief of supravalvular aortic stenosis and, in addition, restores the aortic root geometry to a much more anatomical configuration than is achieved with the simple patch technique.

Ventricular septal defect associated with aortic insufficiency: Medical and surgical management

Abstract We have described seven patients with aortic insufficiency and ventricular septal defect (classified by Tatsuno and associates 6 as supracristal and infracristal). Four patients (Group I) had supracristal VSD and three (Goup II) had infracristal) VSD. All underwent VSD repair only, without operation on the aortic valve. In three patients from Group I the AI disappeared two to three years after VSD repair. From our present study and reports of others, it is suggested that, if the VSD is supracristal, appearance of AI is an indication for early surgical repair of the VSD prior to development of signs and symptoms of left ventricular failure, with the expectation that AI will disappear or be minimized.

Partial atrioventricular canal defect: the early and late results of operation.

Thirty-five patients underwent repair of a partial atrioventricular canal defect and have been followed for a mean of 8 years. Seventeen patients had either moderate or severe mitral incompetence prior to operation. The mitral valve was treated as a bileaflet structure. The cleft in its anterior leaflet was closed in each patient in whom the valve was incompetent. The septal defect was closed with pericardium, and the coronary sinus was left on the right atrium. Two patients required reoperation, 1 to close a recurrent septal defect and 1 to replace a mitral valve that had been competent for the first several years after the initial repair. All 33 survivors remain in New York Heart Association Functional Class I. Nineteen patients have no mitral incompetence, and the remaining 14 have only mild incompetence. These data demonstrate that excellent late results from repair of partial atrioventricular canal are possible when the mitral valve is managed as a bileaflet structure.

Maturation of Small Pulmonary Arteries in Puppies

The histologic pulmonary vascular changes and the pulmonary arterial pressure changes which occur during the first 13 weeks of life have been studied in mongrel puppies. The histologic changes were based on measurements of the average lumen to wall ratios of arterial vessels 50 to 200 μ in diameter. The pulmonary arterial pressure changes were recorded by direct needle puncture of the main pulmonary artery in anesthetized thoracotomized animals. The average L/W ratios of all neonatal mongrel puppies from separate litters were less than 4.0, the boundary chosen to distinguish neonatal and adult pulmonary arterial vasculature. All puppies younger than 1 month had average L/W ratios below 4.0. Although average mean pulmonary arterial pressures were found to be near adult levels by age 1 week, the ratio of pulmonary to systemic arterial pressure, expressed as a percentage, did not consistently approach adult levels until 1 month of age. The dimensional evolution of small pulmonary arteries and the gradual decline in the ratio of pulmonary to systemic pressure toward near adult levels at 4 weeks suggested a cause-and-effect relationship between these 2 phenomena. Major operative procedures have been performed on puppies 3 to 5 weeks of age with survival. It is proposed that experimental pulmonary hypertension produced in 4-week-old mongrel puppies would closely approximate the situation of a high pressure congenital cardiac shunt in humans without excessive operative mortality.

Current Results with Construction and Interruption of the Waterston Anastomosis

The Waterston anastomosis was constructed in 21 infants and neonates between 1973 and 1977. Sixteen neonates were 1 week old or less and 8 were less than 24 hours old. There were 2 operative deaths, giving a hospital survival of 90%. There were 3 late deaths. All surviving infants received satisfactory palliation except 1 who required a Potts anastomosis one year later. During the same time interval, 9 other patients who had had a Waterston anastomosis underwent complete intracardiac repair. Seven of them had significant angulation of the right pulmonary artery necessitating patch reconstruction. All patients survived operation, and follow-up pulmonary angiograms demonstrated only a slight persistent narrowing of the right pulmonary artery in 2 patients. We conclude that the Waterston anastomosis can be constructed with a low operative mortality even in the severely cyanotic neonate and that it can be taken down at the time of complete repair with minimal morbidity and no mortality even if it has significantly angulated the right pulmonary artery.