Ventricular septal defects: a surgical viewpoint.

Seventy-six cardiac autopsy specimens with ventricular septal defects were studied from a surgical viewpoint. The defects were classified as being conoventricular (n = 25), in the right ventricular outlet (n = 21), in the inlet septum (n = 11) or in the trabecular septum (n = 19) with each category having several subcategories. The left ventricular outflow tract relations of the defects are emphasized. The borders of the ventricular septal defects are described in detail, with use of the prefix "juxta" to indicate the immediate adjacency of the defect to a structure such as the tricuspid valve.

Outcome probabilities and life history after surgical mitral commissurotomy: implications for balloon commissurotomy.

From 1967 to 1988, 339 patients with mitral stenosis underwent surgical commissurotomy, 103 with a closed and 236 with an open technique. The 1 month and 1, 5, 10 and 20 year overall survival rate was 99.7%, 99%, 95%, 87% and 59%, respectively, and the technique (open versus closed) was not a risk factor. Technique was also not a risk factor for a second mitral commissurotomy, subsequent mitral valve replacement, thromboembolism or poor functional status. Risk factors were older age at commissurotomy, black race, higher pulmonary vascular resistance, mitral leaflet calcification, left ventricular enlargement and postcommissurotomy mitral incompetence. The closed technique was a risk factor for mitral incompetence immediately after commissurotomy, but important incompetence developed in only 2 of the 103 patients undergoing closed commissurotomy. Mitral valve replacement was not required within 10 years in 78% of patients and within 20 years in 47%. Despite some increased prevalence of postcommissurotomy mitral incompetence and particularly in view of the considerable long-term likelihood of mitral valve replacement, initial therapy for most patients with mitral stenosis should probably be surgical closed (or percutaneous balloon) mitral commissurotomy. The equations developed in the present study can be used to predict and compare outcome probability after percutaneous balloon commissurotomy with that after surgical commissurotomy and to compare these with outcome probability after mitral valve replacement.

Outcomes in critically ill neonates with pulmonary stenosis and intact ventricular septum: a multiinstitutional study. Congenital Heart Surgeons Society.

OBJECTIVE: This study attempted to determine the optimal therapeutic interventions by risk-adjusted comparisons of early and intermediate-term outcomes. BACKGROUND: The variety of interventions and the small case load at individual institutions have delayed the generation of reliable information concerning therapy for this condition. METHODS: In this prospective 27-institution study, 101 neonates were consecutively enrolled (between January 1, 1987 and January 1, 1991). Treatment was determined by the physicians. Demographic and morphologic details were tabulated. Dimensions of the pulmonary "anulus" and tricuspid anulus were measured on echocardiograms, and right ventricular cavity size was estimated. Right ventricular-pulmonary trunk pressure gradients were tabulated. Numerous analyses were made. RESULTS: Severe pulmonary valve stenosis and an intact ventricular septum were present in all patients. The right ventricular-pulmonary trunk junction ("anulus") was severely narrowed in 15%. Right ventricular cavity size was severely reduced in 4%. The tricuspid valve was small in 15% of patients; its diameter was poorly correlated with right ventricular cavity size. Eighty-nine percent and 81% of patients survived > or = 1 month and 4 years, respectively, after the initial procedure. Multivariable analysis identified no patient-specific risk factors for death. Only open pulmonary valvotomy without a support technique was uniformly a procedural risk factor; under some circumstances, transannular patching without a shunt was a risk factor. The right ventricular-pulmonary trunk gradient immediately after valvotomy was < 30 mm Hg in 81% of patients and was similar after surgical and balloon valvotomy. In 74% of patients, no intervention was required after the first accomplished intervention. CONCLUSIONS: Marked variation in morphology is uncommon in critical pulmonary stenosis in neonates. Percutaneous balloon valvotomy and certain types of surgical valvotomy are optimal initial procedures. The unusual situation of a small pulmonary "anulus" may initially require a transannular patch and a systemic-pulmonary artery shunt.

Current results of management in transposition of the great arteries, with special emphasis on patients with associated ventricular septal defect.

Two hundred forty-five patients less than 15 days of age with transposition of the great arteries with or without a ventricular septal defect or pulmonary stenosis were entered into an ongoing 20 institution treatment study between January 1, 1985 and June 1, 1986. Complete follow-up is available on all patients. The ventricular septal defect narrowed in only 1 of 36 patients with combined transposition of the great arteries and ventricular septal defect; pulmonary stenosis developed or worsened in 3 of these 36 patients and in 3 of the 187 patients with simple transposition. Twelve month overall survival among the 245 patients was 80%. No morphologic feature of transposition was a risk factor for death but major associated cardiac and noncardiac anomalies (more common in patients with combined transposition and ventricular septal defect) and low birth weight were risk factors. Neither arterial switch repair (n = 86), atrial switch (Mustard) repair (n = 21) nor atrial switch (Senning) repair (n = 39) was a risk factor for death, but results in all surgical groups were better in the last part of the experience. Death before repair was less frequent late in the study. Possibly, in low birth weight infants, survival was better with the arterial than with the atrial switch repair. These data suggest that survival at 1 year is similar with either the arterial or the atrial switch repair. The early results of repair of combined transposition of the great arteries and ventricular septal defect are as good as those of simple transposition. Special institutional efforts are required to attain good results with the arterial switch repair and to prevent death before repair.

Investigation and management of primary cardiac tumors in infants and children.

Seven children with primary cardiac tumors are discussed. The diagnosis was achieved by cardiac catheterization in all seven patients. In four patients, surgical excision was undertaken with one long-term good result. One of the other three surgical patients died of acute heart failure at the discontinuation of cardiopulmonary bypass. One of the other two surgical patients died suddenly late postoperatively, presumably from arrhythmia. The other, affected by fibrosarcoma, also died suddenly while undergoing chemotherapy. The histologic findings on the tumor masses were available in six patients. There were three rhabdomyomas, one fibrosarcoma, one fibroma and one hamartomatous mass. In one patient with tuberous sclerosis, a presumptive diagnosis of rhabdomyoma was made clinically on the basis of multiple masses infiltrating the left ventricle. Two-dimensional echocardiography is the best noninvasive tool to determine the extent and location of the tumor and suitability for surgery. When surgery is indicated, cardiac catheterization for pressure data is also required. Surgery is recommended in symptomatic solitary lesions that most likely are fibromas and have an overall good surgical outcome.

Peroxynitrite irreversibly decreases diastolic and systolic function in cardiac muscle.

Much of the damaging action of nitric oxide in heart may be due to its diffusion-limited reaction with superoxide to form peroxynitrite. Direct infusion of peroxynitrite into isolated perfused hearts fails to model the effects of in situ formation because the bulk of peroxynitrite decomposes before reaching the myocytes. To examine the direct effects of peroxynitrite on the contractile apparatus of the heart, we exposed intact and skinned rat papillary muscles to a steady state concentration of 4-microM peroxynitrite for 5 min, followed by a 30-min recovery period to monitor irreversible effects. In intact muscles developed force fell immediately to 26% of initial force, recovering to 43% by 30 min. Resting tension increased by 600% immediately, and was still elevated 500% by 30 min. Nitrotyrosine immunochemistry showed that peroxynitrite can induce tyrosine nitration at low concentrations and is capable of penetrating 200-380 microm into the papillary muscle after a 5-min infusion. Decomposed peroxynitrite had no effect on either intact or skinned muscle developed force or resting tension. Our results show that peroxynitrite directly damages both developed force and resting tension of isolated heart muscle, which can be extrapolated to systolic and diastolic injury in intact hearts.

Indications for cardiac surgery in patients with active infective endocarditis.

Currently, absolute indications for valve replacement during active infective endocarditis include severe heart failure, the presence of an infecting microorganism that is not susceptible to available antimicrobial agents, and, in patients with an infected prosthetic valve, an unstable device. Relative indications include an etiologic microorganism other than a susceptible Streptococcus, relapse after presumed effective therapy, evidence of intracardiac extension of the infection, two or more systemic emboli, vegetations large enough to be demonstrated by echocardiography, and, in patients with an infected prosthetic device, early disease and periprosthetic leak. With use of data from the medical literature, a study generated by the cardiovascular surgical group at the University of Alabama School of Medicine, and a brief cost analysis, a point system was constructed to assist in decision-making concerning surgery in patients with active infective endocarditis. The usefulness of this system will depend on experience generated from its utilization in a larger number of patients as well as new data relative to a more complete understanding of the risks and benefits of surgery in this condition.

Early risks of open heart surgery for mitral valve disease.

During 1972 and 1973, a total of 235 patients had open heart surgery for mitral valve disease unassociated with significant aortic or rheumatic tricuspid valve disease. Thirty-one underwent closed heart mitral commissurotomy, without mortality. Of the 204 patients undergoing open operation, 125 had sequential measurement of cardiac output and mixed venous oxygen pressure. The hospital mortality rate was 6.4 percent in the larger group of 204 patients and in the 125 with cardiac output measurements. The rate was greater in those with class IV disability (New York Heart Association criteria) preoperatively than in those with class III or II disability. The mean +/- standard deviation of the average cardiac index early postoperatively was 2.05 +/- 0.579 liters-min--1-m--2. Cardiac index was lower in the patients who died early postoperatively than in those who did not. The probability of hospital death was a significant function of cardiac index. The predicted probability of death was 10 percent with an average cardiac index of 1.42 liters-min--1-m--2 and increased sharply with lower indexes. Cardiac index was lower early postoperatively than preoperatively, and was lower in patients in class IV than in those in class III. There was no significant difference in cardiac index between patients with mitral valve replacement and those in repair. A history of closed commissurotomy, age, duration of cardiopulmonary bypass, duration of cardiac ischemia and method of myocardial preservation did not significantly influence cardiac index or hospital mortality rate. There was no significant relation between mixed venous oxygen pressure and hospital death. Further improvement in results of mitral valve surgery requires adequate preservation of left ventricular performance before, during and after operation.

Afterload reduction and cardiac ouptut in infants early after intracardiac surgery.

Sixteen infants less than 18 months old were studied within 3 hours of intracardiac surgery. Initial mean arterial pressure and systemic vascular resistance levels were higher than normal in each infant (99 +/- 17.2 mm Hg and 48 +/- 18.1 units'm2', respectively [mean +/- standard deviation]); cardiac index was low (1.9 +/- 0.48 liters'min-1-2) and mean left and right atrial pressures were 11.4 +/- 2.39 and 12.5 +/- 3.10 mm Hg, respectively. Mean pulmonary arterial pressure was 29 mm Hg; pulmonary vascular resistance was 8.6 units'm2'. When nitroprusside was infused to reduce mean arterial pressure to about normal, cardiac index increased 17 percent and mean left and right atrial pressures decreased 25 and 22 percent, respectively. Mean pulmonary arterial pressure decreased 31 percent. When atrial pressures returned to initial values after infusion of blood with continued infusion of nitroprusside, cardiac index increased another 24 percent. When administration of nitroprusside was discontinued, cardiac index decreased to 116 percent of the initial value and mean atrial pressure increased to 90 percent of the initial value. The study demonstrated the favorable effect on cardiac output of vasodilator therapy in combination with blood volume expansion in hypertensive infants early after intracardiac surgery. An empirical equation interrelating atrial and arterial pressures and cardiac index was derived from the data.

Long-term evaluation (12 to 22 years) of open heart surgery for tetralogy of Fallot.

Four hundred seventy-five patients underwent repair of tetralogy of Fallot from 1955 to 1964; 396 of these were hospital survivors and were followed up for 12 to 22 years. An excellent late clinical result was maintained by 87 percent of the 396 hospital survivors. A less than excellent result in the remaining 13 percent of hospital survivors was caused by late mortality in 7 percent (sudden death in 3 percent, death due to cardiac causes in 2 percnt and death due to noncardiac causes in 2 percent), required reoperation in 4 percent (mainly because of residual ventricular septal defect) and development of symptoms in 2 percent. Postoperative cardiomegaly (cardiothoracic ratio greater than 0.55) was observed in 60 (25 percent) of 246 patients who had a follow-up chest roentgenogram, and was more common among those who died late or remained symptomatic. Among the few patients with inadequate surgical relief of right ventricular hypertension who did not have transanular patch repair, the hypertension did not tend to decrease progressively, whereas it did decrease in patients who had patch repair. No late sudden deaths were encountered in 20 patients shown to have postoperative right bundle branch block plus left axis deviation (bifascicular block pattern). Pulmonary valve incompetence appeared to have relatively little harmful influence on the late result, causng cardiac disability in 1 percent of the patients and appeared to be the main contributing factor of postoperative cardiomegaly in 13 (5 percent) of the 246 patients who had a follow-up chest roentgenogram. Most late deaths and complicatins appeared within 2 years of operation, and accelerating deterioration in late results did not occur as the follow-up extended beyond 2 decades.

Pulmonary arterial compression syndrome caused by false aneurysm of left ventricular outflow tract.

A 29 year old man experienced exertional dyspnea and coughing 3 1/2 years after insertion of a Brauwald-Cutter aortic valve prosthesis. Clinical examination suggested pulmonary arterial hypertension, and cardiac catheterization revealed a saccular lesion apparently arising from the left ventricular outflow tract and producing compression of the right pulmonary artery. Origin from the left ventricular outflow tract just under the aortic ring was confirmed at operation. The lesion apparently arose from an anular excavation related to previous endocarditis with abscess formation. Reported cases of similar aneurysmal lesions are briefly reviewed, and other known causes of the pulmonary arterial compression syndrome are discussed.

Angiography of atrioventricular canal defects.

The axial angiograms of 20 patients with an atrioventricular (A-V) canal defect were carefully examined and compared with anatomic observations made at operation. A common A-V valve was present in 15 patients and two separate valves in 5. The atrial septal defect, the anatomy of the ventricular septum and the morphology and functional status of the A-V valves were well demonstrated. There ws consistent angiographic deformation of the normally straight contour extending from the noncoronary aortic cusp to the crux cordis observed in the four chamber and elongated right anterior oblique projections of the left ventricular angiogram of each patient. This deformity is caused by the deficiency or absence of the A-V septum and is the best angiographic sign of the malformation. The differentiation of two separate valves from a common A-V valve was uniformly made. When a common A-V valve was present, three of the five leaflets were usually identified but distinction of the free floating from the tethered anterior leaflet was not always possible. It is concluded that axial techniques provide more complete angiographic definition of the anatomic abnormalities of A-V canal defects than are demonstrated from frontal and lateral views.

Primary repair of complete atrioventricular canal in patients less than 2 years old.

Twenty-seven consecutive patients less than 2 years of age underwent primary intracardiac repair of complete atrioventricular (A-V) canal. Three (19 percent) of the 16 operated on after January 1, 1975 died in the hospital, a smaller proportion than the 8 of 11 patients who died in the hospital after operation between 1972 and 1975 (P = 0.005). The date of operation as a continuous variable is also related to the probability of hospital death (P = 0.016). Age at operation was not related to hospital mortality among the total group of 27 infants, nor were the anatomic characteristics of the anterior and posterior bridging leaflets, the location and size of the interventricular communications or the duration or technique of profound hypothermia (total circulatory arrest versus low perfusion flow rate). The improved results in the 16 patients operated on since January 1, 1975 are believed to be primarily the result of an improved ability to construct "mitral" and "tricuspid" valves from the common A-V valve. Fourteen of the 16 hospital survivors are alive and well 5 to 60 months after operation. These results and the natural history of patients with this malformation indicate that there should be no change in the policy of performing elective intracardiac repair before age 2 years and primary repair rather than pulmonary arterial banding when operation is required in the early months of life.

Life expectancy without surgery in tetralogy of Fallot.

All published autopsy cases of patients with tetralogy of Fallot who died without surgical treatment were studied to determine the life expectancy of such persons. In addition, the data from a study of persons with tetralogy alive in Denmark in 1949 were reanalyzed. The survival data from these two sources were remarkably similar, indicating that 66 percent of persons with tetralogy of Fallot not treated surgically live to age 1 year, 49 percent to age 3 years and 24 percent to age 10 years; thereafter, the hazard function (or instantaneous risk of death) remains constant. The chance of survival is significantly less when pulmonary atresia, rather than stenosis, is present.

Intracardiac surgery in infants under age 3 months: predictors of postoperative in-hospital cardiac death.

Data are reported on 142 infants less than 3 months old who left the operating room alive after an open intracardiac operation during the 13 years from January 1967 to July 1980. The probability of postoperative in-hospital cardiac death for acute postoperative heart failure (the most common mode of death in these infants) was found by multivariate logistic analysis to be significantly related only to the strength of pedal pulses, the pedal skin temperature and the cardiac index in the first 5 postoperative hours. When cardiac index was not analyzed and cold cardioplegic myocardial preservation methods used, only pedal pulses and pedal skin temperature were significant predictors of hospital death. Blood pressure and heart rate were not related to this mode of hospital death. Oliguria occurred in 23 percent of patients; it was related primarily to inadequate cardiac performance and increased the probability of hospital death. Treatment protocols are derived based on these facts.

Early and intermediate-term (10-year) results of surgery for univentricular atrioventricular connection ("single ventricle").

One hundred sixteen patients with "single ventricle" underwent 147 operations between 1967 and July 1982, with an actuarial survival rate (hospital deaths are included in all actuarials) at 10 years of 66%. One hospital death (2%) occurred after 45 primary "classic" shunting operations, and the 10-year actuarial survival was 85%. There were no hospital deaths after atrial septectomy or pulmonary artery banding, and the 10-year actuarial survival rate after these as initial procedures was 76% and 74%, respectively. Thirteen of 36 patients (36%) who underwent ventricular septation between 1967 and November 1, 1983, died in hospital. Among the 13 with the ideal morphologic characteristics of double inlet left ventricle without major associated cardiac anomalies, and without the need for valve replacement or a valved extracardiac conduit, there were no hospital deaths and a late survival rate of 77% (confidence limits 59 to 90%). Sixteen of 73 patients (22%), including those with tricuspid atresia, undergoing the Fontan-type procedure between 1967 and November 1, 1983, died in hospital. Multivariate analysis indicates that cardiac morphologic patterns other than tricuspid atresia are risk factors for hospital death in this group. The 10-year actuarial survival rate among the 73 patients was 71%, but only 1 death (associated with reoperation) occurred more than 2 months after operation. Ninety-seven percent of all surviving patients are in New York Heart Association functional class I or II, as are 100% of those who underwent septation and 96% of those who underwent the Fontan-type procedure.

Congenital pulmonary vein stenosis.

Congenital pulmonary vein stenosis is a rare and serious form of congenital heart disease. Between 1969 and 1982 10 patients with this lesion were studied. In 2 patients the condition was diagnosed at autopsy; these patients died before the presence of congenital heart disease was suspected. Of the 8 in whom the condition was diagnosed during life, it was suspected clinically in 6 and found unexpectedly at cardiac catheterization in 2. All underwent operation, and 5 were hospital survivors. In all survivors rapid and progressive restenosis of the pulmonary veins occurred over the next several months. Three of the 5 underwent reoperation, but progressive restenosis recurred and all eventually died of this condition. Thus, despite partial surgical relief of pulmonary vein stenosis, the lesion is apparently one of relentless progression. No surgical repair has been successful in the cure or long-term palliation of this lethal lesion.

Take-down of cava-pulmonary artery anastomosis (Gleen) during repair of congenital cardiac malformations: report of 5 cases.

The dismantling of previously made anastomses between the superior vena cava and right pulmonary artery (Glenn shunts) and reconstruction of superior vena cava-right artial continuity have been accomplished in 5 patients at the time of intracardiac repair of congenital heart defects. All patients survived the hospitalization period, but 2 died late postoperatively of causes apparently not related to the cava-atrial reconstruction. Experience with these 5 patients and the intraoperative techniques employed are presented.

Tetralogy of Fallot with aorticopulmonary window. Report of two surgical cases.

The association of aorticopulmonary window and tetralogy of Fallot is rare. In this report, we describe two cases in which this combination of anomalies was repaired successfully. Both patients made uneventful recoveries.

Surgical treatment for closed thoracic aortic injuries.

Eighteen patients with acute aortic tear secondary to nonpenetrating thoracic trauma and seven with chronic tears have been operated upon. Of the patients with acute injury, 28% had normal chest x-ray on admission. The diagnosis of acute aortic tear was made by aortography in all patients. Two patients died from causes unrelated to the aortic injury. In the group of 18 patients in which some method of perfusing the distal aorta was used, two developed paraplegia; in the group of 7 treated with simple aortic cross-clamping none occurred. The average aortic cross-clamp time 69 and 23 minutes, respectively, and average blood administration 3,139 and 1,700 ml., respectively. We conclude that the diagnosis of acute aortic tear cannot be reliably made on clinical grounds, and that with properly trained personnel the method of choice is usually simple aortic cross-clamping during repair.