Pulmonary venous obstruction and atrioventricular canal anomalies: role of cor triatriatum and double outlet right atrium.

Two-dimensional echocardiographic findings and surgical repair in three infants with atrioventricular (AV) canal and pulmonary venous obstruction are described. The AV canal was complicated by cor triatriatum in two patients and by double outlet right atrium in the other. In patients with AV canal, anatomic obstruction of pulmonary venous return should be excluded by detailed cardiac ultrasound examination.

Long-term follow-up of two infants with an implanted cardiac pacemaker.

The clinical course of two patients with congenital heart block who had pacemaker implantation at age 7 and 8 months, respectively, is reviewed. One patient at age 10 years has had nine pulse generators inserted; the other has had six implantations, the most recent a lithium iodine pacemaker, during 8 1/2 years of observation. Both patients have shown normal physical development and emotional maturation despite multiple hospitalizations and pacemaker replacements, thus demonstrating that electrical pacing, initiated in infancy, can be maintained through childhood without adverse effects.

Postoperative assessment of porcine-valved right ventricular-pulmonary artery conduits.

Insertion of extracardiac right ventricular (RV)-pulmonary artery (PA) conduits for repair of severe forms of RV-PA discontinuity has become a widely used technique. During a 10-year period, 76 patients had open heart repair with a porcine-valved RV-PA external conduit. The most common diagnoses were truncus arteriosus (15%) and pulmonary atresia with ventricular septal defect (15%). The patients were 18 days to 37 years old (median 7 years). The postoperative median follow-up period was 4 years. Four patients (8%) have been lost to follow-up. Nineteen patients (25%) died in the early postoperative period and there were 10 late deaths (13%). Calculated probability of survival was 66% at 8 years. Thirty-six patients have undergone late hemodynamic studies. A residual shunt was present in 14% of the patients; moderate to severe PA hypertension was present in 25% and significant RV-PA gradients (greater than 30 mm Hg) in 50%. The mean RV pressure for the group was 78.5 +/- 38 mm Hg. The severity of the gradients appeared to be greater in patients who had been followed longer. Ten patients have undergone reoperation because of conduit obstruction or residual shunting; 6 are alive and well. Of the surviving patients, 95% are acyanotic and without significant exercise intolerance and only 5% have cardiovascular symptoms. Thus, open heart repair using an external RV-PA conduit provides a marked improvement in the quality of life in patients who survive.

Muscular ventricular septal defects repaired with left ventriculotomy.

Between 1974 and 1979 nine patients, aged 10 months to 4 years, underwent left ventriculotomy for closure of single or multiple defects in the muscular ventricular septum. The vertical incision paralleled the anterior descending branch of the left coronary artery near the apex of the left ventricle and ranged from 2.5 to 3.5 cm in length. Four patients also had a right ventriculotomy with closure of a high perimembranous ventricular defect in two. Serial electrocardiograms indicated changes of myocardial ischemia or necrosis. Left bundle branch block did not develop in any patient. Three patients died in the early postoperative period. The six surviving patients are living and well 2 to 7 years later. There is apparent complete closure of the ventricular defects, which was documented by cardiac catheterization in four cases. Two patients had cardiomegaly and left ventricular dysfunction as assessed with echocardiographic and angiographic study, whereas four displayed good cardiac function. In three of the latter patients, cardioplegia or deep hypothermia techniques were utilized intraoperatively. The observations indicate that left ventriculotomy of limited size is an acceptable approach to the difficult problem of repair of muscular ventricular defects but may involve some risk of compromise of the coronary circulation.

Primary definitive repair of type B interrupted aortic arch, ventricular septal defect, and patient ductus arteriosus. Early and late results.

Since March, 1974, eight patients, aged 7 days to 5 months, with type B interrupted aortic arch (IAA), ventricular septal defect (VSD), and patent ductus arteriosus (PDA) were treated at the Columbus-Presbyterian Medical Center and the University of Maryland Hospital. Six of these patients underwent definitive repair utilizing deep hypothermia and circulatory arrest. Correction involved resection of all ductal tissue, primary anastomosis of the aortic arch, closure of the foramen ovale, and patch closure of the VSD. In five patients, all arch vessels were preserved and no prosthetic material was used to reconstruct the aortic arch. One patient died 48 hours postoperatively of a coagulopathy. All others survived more than 30 days. One patient, 3 1/2 months old at repair, had undergone pulmonary artery banding at another institution at 11 days of age; he died of recurrent respiratory infections 8 months after correction. Three patients are alive and well 3 to 6 years after repair. Two have undergone repeat cardiac catheterization which demonstrated good growth of the anastomosis and no residual gradient. Primary definitive correction of type B IAA with VSD and PDA provides distinct advantages over palliative or other surgical procedures with excellent long-term results.

Intraoperative unidirectional intra-aortic balloon pumping in the management of left ventricular power failure.

Unidirectional intra-aortic balloon pumping (IABP) was applied to 28 adult patients undergoing open-heart surgery over a 35 month period. The patients were divided into three groups according to the temporal sequence of initiating IABP. Group A consisted of 4 patients who were in a low output state or in cardiogenic shock prior to study. All patients survived cardiac catheterization and surgery, and 3 (75 per cent) were long-term survivors. Group B included 15 patients who could not be weaned from cardiopulmonary bypass with the usual supportive measures. Twelve patients (80 per cent) were weaned from bypass with IABP, and 11 (73 per cent) were discharged from the hospital. Group C was composed of 9 patients who manifested a low cardiac output syndrome within the first 24 hours following surgery. IABP was initiated in the recovery room. Six patients (67 per cent) were discharged. The total experience with these 28 patients therefore includes 24 patients (86 per cent) who were weaned from IABP, 20 (71 per cent) who were discharged, and 18 (64 per cent) who were long-term survivors. The present criteria for the use of IABP in the cardiac surgical patient are defined.

Surgical management of ventricular septal defects in infants.

Infants with ventricular septal defect (VSD) who are symptomatic despite intensive medical therapy require surgical intervention. Choice of treatment depends upon the cumulative mortality and morbidity rates of the two-stage approach of initial pulmonary artery banding followed by debanding and VSD closure as compared to the risk of primary intracardiac repair in infancy. Sixteen infants underwent pulmonary artery banding at Columbia-Presbyterian Medical Center between 1967 and 1976, with one operative death but with a significant incidence of morbidity and late death. Forty patients underwent pulmonary artery debanding and closure of VSD with three operative deaths. This second-stage procedure was frequently complicated by repair of acquired lesions. During the same 10 year period 37 infants underwent primary closure of VSD with eight operative deaths. The morbidity related to this procedure is low. With the use of profound hypothermia and circulatory arrest, results have significantly improved and the risk of early correction now compares favorably with the cumulative mortality rate of the two-stage approach. Primary intracardiac repair is the procedure of choice.

Signal enhancement for detection of specialized atrioventricular conduction system electrograms during open-heart surgery.

A method for signal enhancement of electrograms of the specialized atrioventricular conduction system is presented. This method allows a single electrogram to replace the tracings of bipolar electrograms previously required to ensure recognition of the specialezed conduction system and yet maintains the safety afforded by use of three pairs of recording electrodes. In addition, this new signal is enhanced in magnitude and is always unidirectional (positive). These features are desirable for automated signal recognition. This method therefore provides an important step toward future automation and signal recognition of specialized conduction system electrograms during open-heart surgery.

Cardiac output in infants and children after open-heart surgery.

Green dye measurements of cardiac index (in liters per minute per square meter) were compared in 32 infants and 58 children, 6 and 24 hours after normothermic cardiopulmonary bypass. Cardiac index was significantly lower in infants under one year of age, as compared to older infants and children. This parameter was also lower in patients with total anomalous pulmonary venous drainage, truncus arteriosus, and tetralogy of Fallot. Five infants who died had 6-hour cardiac index determinations of 2.0 L./min./sq. M. Only 2 of 27 survivors had cardiac indices below this level. Simultaneously recorded heart rate and central venous pressure did not correlate with cardiac index. Systolic blood pressure was significantly lower in infants in whom the cardiac index was less then 2.0 L./min./sq. M. (p less than 0.01). However, in individual situations, determination of systemic blood pressure lacked specificity in predicting outcome. Measurements of cardiac index are helpful in the management of infants and children following intracardiac repair by (1) assessing the hemodynamic status, (2) providing a useful indication of the cardiocirculatory effects of therapeutic interventions, and (3) predicting outcome.

Atrial morphologic features in tricuspid atresia.

We studied the atrial morphology in 110 hearts removed at autopsy from patients with tricuspid atresia. Ten of the patients had had a Fontan operation. We compared the findings in these hearts with those in 30 normal hearts. Prominent eustachian valves were common (40%). The tricuspid "dimple," present in 33 of 100 specimens, denoted the membranous atrioventricular septum and was never related to the right ventricle. Most interatrial communications (85/100) were nonobstructive. A minority of patients with obstructive interatrial communications (13/100 = small, 2/100 = severely obstructive) would be expected to benefit from balloon atrial septostomy early in life. Right atrial hypertrophy was greatest in specimens with restrictive interatrial communications (severely obstructive = 3.2 +/- 0.3 mm, small = 2.1 +/- 0.6 mm) and in those that had been subjected to a Fontan operation (3.2 +/- 0.7 mm), but thickness was also increased in hearts with nonobstructive interatrial communications (1.6 +/- 0.5 mm) compared with normal hearts (1.1 +/- 0.2 mm; p less than 0.001 versus all groups). Of the patients in the series, 11 of 100 had left juxtaposition of the atrial appendages, which may have important implications for the Fontan operation.

Pulmonary atresia with intact ventricular septum. Sixteen-year experience.

Infants with pulmonary atresia and intact ventricular septum (PA-IVS) usually require urgent surgical intervention. Thirty patients with this anomaly, seen at the Columbia-Presbyterian Medical Center between 1962 and 1978, had palliative operations, 26 within the first 3 days of life. Six underwent a closed pulmonary valvotomy alone, with no survivors; six had only a systemic--pulmonary artery shunt, with three early survivors. Because of this experience, 17 had a combined procedure of valvotomy and shunt, with 14 early survivors. One patient recently underwent a definitive right ventricular outflow patch procedure with cardiopulmonary bypass. Eight patients subsequently have had corrective open-heart procedures, with five patients surviving from 2 to 10 years. A unicusp aortic homograft was used for repair in five and a Hancock valved conduit in three. Four patients are presently awaiting operation. We conclude that the initial surgical management of these critically ill infants must not only increase pulmonary blood flow but in addition provide an opportunity for right ventricular growth. Thus we continue to advocate the combined procedure of a valvotomy plus a shunt to provide adequate palliation. Repeat catheterization should be performed within a year to confirm the adequacy of the valvotomy, since this is essential to maximal right ventricular enlargement and to allow for definitive correction at a later date.

Comparison of intraoperative nitroprusside unloading in mitral and aortic regurgitation.

To compare the hemodynamic effect of vasodilator therapy on different regurgitant lesions, we infused sodium nitroprusside intraooperatively in 12 patients with mitral regurgitation and 15 with aortic regurgitation. During the critical period preceding establishment of cardiopulmonary bypass, both groups had developed intense vasoconstriction and cardiac decompensation. All demonstrated improved cardiac function with vasodilator therapy; however, the degree of improvement with nitroprusside differed in the two groups. Stroke volume increased 10 ml. per beat per meter squared in those patients with aortic regurgitation and only 6 ml. per beat per meter squared in those with mitral regurgitation (p less than 0.05). The percent increase in stoke volume induced by nitroprusside was inversely correlated to the preoperative left ventricular ejection fraction (r = 0.44, p less than 0.02). Patients with aortic regurgitation had lower preoperative left ventricular ejection fractions than those with mitral regurgitation (0.53 versus 0.63, p less than 0.02). Therefore, we conclude that patients with aortic regurgitation derived greater intraoperative hemodynamic benefit from unloading with nitroprusside, because they came to surgery with greater impairment of left ventricular contractility. Although nitroprusside improved cardiac function in both groups, only the patients with aortic regurgitation achieved normal pulmonary artery pressure (17 torr) and pulmonary vascular resistance (2.1 units) as a result of unloading. Those with mitral regurgitation continued to have pulmonary hypertension (28 torr) and increased pulmonary vascular resistance (3.9 units) despite vasodilator therapy. Thus the data suggest that patients with mitral regurgitation derived less hemodynamic benefit from intraoperative nitroprusside therapy because they were also limited by right ventricular dysfunction and a less responsive pulmonary vasculature.

Preparation of fibrin glue from single-donor fresh-frozen plasma.

Fibrin glue is used widely in Europe as a tissue sealant and hemostatic agent. The European glue is prepared commercially from pooled human blood. It is not available in this country because of the risk of transmission of hepatitis B, acquired immune deficiency syndrome, and other blood-transmitted diseases. We describe a cryoprecipitation technique for preparation of fibrin glue from single-donor fresh-frozen plasma. This technique enables the glue to be made in large quantities with no greater risk of disease transmission than with that from the transfusion of single-unit fresh-frozen plasma. We have found that the glue is a useful tool in surgery. By helping to control difficult bleeding, its use can decrease the need for blood transfusions and shorten operating room time. It also is effective as a means to pretreat highly porous vascular prostheses that currently are used infrequently because of bleeding. These porous grafts offer potential advantages in handling, suturing, and long-term patency. This new technique of fibrin glue preparation may make this useful surgical adjunct as readily available in this country as it is in Europe.

Cardiac valve replacement in children: a twenty-year series.

Ninety-four children ranging from 3 months to 19 years of age underwent cardiac valve replacement at Columbia Presbyterian Medical Center from 1965 to 1985. The overall operative mortality was 12%, but mortality was higher among patients less than 2 years of age, patients who had had previous cardiac operations, and patients requiring double-valve replacement. Seven of 11 patients who received mechanical valves and no anticoagulation experienced major thromboembolic events. An episode of gastrointestinal hemorrhage that was easily controlled represents the only bleeding complication in the entire series. Valve replacement in children continues to be a high-risk procedure, and efforts to preserve native valve function should be attempted when technically feasible. Our data also suggest that anticoagulation can be safely accomplished in the pediatric age group and should be employed in patients requiring placement of a mechanical prosthesis, especially in the mitral position.

Traumatic rupture of extracardiac valved conduit: unusual late complication producing outflow tract obstruction.

A 22-year-old woman died suddenly 15 years after successful repair of truncus arteriosus with a valved Dacron conduit. At autopsy there was complete obstruction of the right ventricular outflow tract by a large organizing thrombus between the outer aspect of the conduit and the adherent pericardial tissue. This rare late complication may have resulted from an unrecognized deceleration injury occurring at the time of a serious automobile accident 5 months before death.

Hemostatic effectiveness of fibrin glue derived from single-donor fresh frozen plasma.

Fibrin glue derived from pooled human blood is an effective sealant for high-porosity vascular grafts and a valuable topical hemostatic agent in heparinized patients. Use of this agent in the United States is prohibited because of potential transmission of hepatitis B, acquired immunodeficiency syndrome, and other serologically transmitted illnesses. We have developed a cryoprecipitation technique that allows preparation of fibrin glue from single-donor fresh frozen plasma. Use of this agent presumably entails no greater risk of disease transmission than intravenous administration of single-unit fresh frozen plasma. This report describes our early clinical experience with this material. Fibrin glue was used as a sealant for porous woven Dacron tubular prostheses and cardiovascular patches in 19 patients. The fibrin glue sealant has also been employed to control bleeding from needle holes and small anastomotic tears in 22 patients. No patient in this series had a bleeding complication from a suture line or graft treated with fibrin glue. This experience indicates that like fibrin glue from pooled blood, fibrin glue from single-donor plasma is effective as a graft sealant and topical hemostatic agent. Preparation of fibrin glue from single-donor plasma is simple and economical, and may provide cardiothoracic surgeons in the United States with a widely available, valuable hemostatic adjunct.

A pulsatile assist device (PAD) for use during cardiopulmonary bypass.

A pulsatile assist device (PAD) has been developed to convert roller pump flow to pulsatile flow in a simple fashion. The device can also be used as an arterial counterpulsator before and after cardiopulmonary bypass. The PAD has been used in 125 adult patients undergoing open-heart operations for coronary artery or valvular heart disease or the combination. Ninety-two patients were in New York Heart Association Functional Class III or IV or had ejection fractions of less than 0.3. The PAD functioned as a hemodynamically effective arterial counterpulsator before and after perfusion. All patients were successfully weaned from bypass with the PAD. There has been 1 intraoperative death and 2 late deaths. Only 1 patient had a perioperative myocardial infarction, and this person was successfully treated with intraaortic balloon pumping. We believe the PAD is a simple and reliable device for intraoperative counterpulsation and for the creation of pulsatile cardiopulmonary bypass. More important, use of the PAD may decrease both the incidence of perioperative myocardial infarction and the need for postoperative intraaortic balloon pumping.

Open-heart experience in infants using normothermia and deep hypothermia.

During the 9-year period from 1967 through 1975, 124 open-heart operations were performed on infants less than 1 year of age with 35 operative deaths (28%). Ninety-seven of these procedures used continuous cardiopulmonary bypass with normothermia or mild hypothermia, and 27 were done under deep hypothermia and circulatory arrest. Mortality and morbidity were similar regardless of the operative technique, although deep hypothermia facilitated the repair of complex lesions. The highest mortality occurred in infants less than 3 months of age. Respiratory insufficiency, usually requiring prolonged ventilatory support, occurred only among infants who had pulmonary overcirculation or congestion prior to operation. Adequacy of intraoperative repair and postoperative care were the major determinants of survival.

[Counterpulsation by means of a new device with a pulsatile blood flow in open heart operations (author's transl)]. / Kontrpul'satsiia pri pomoshchi novogo ustroistva s pul'siruiushchim krovotokom pri operatsiiakh na otkrytom serdtse.

A new counterpulsation method in operations on an open heart is described. Clinical experience in the use of the new device in 124 patients, which also makes it possible to produce a pulsatile flow of blood in the system of extracorporeal circulation, testifies to its effectiveness. The authors claim that the positive changes in cardiac output, diastolic and systolic pressure, and coronary circulation are among the factors which cause an improvement in the final result of the operation in patients with severe valvular diseases and ischemic heart disease.