Results of surgery for congenital supravalvular aortic stenosis.

Of eight children aged 3 to 15 years with surgical correction of severe supravalvular aortic stenosis, 6 were evaluated 7 to 44 months later by repeat cardiac catheterization and aortography. Prosthetic patch angioplasty was performed in all cases. Preoperative systolic gradients ranged from 40 to 90 mm Hg (average 70); postoperative gradients ranged from 0 to 20 mm Hg (average 11). The postoperative anglographic appearance of the ascending aorta was near normal in all six patients, and none had new aortic valve insufficiency. These results of surgery for supravalvular aortic stenosis are judged to be excellent.

Considerations in the surgical management of infantile coarctation of aorta.

The results of a recent 5 year experience with resection of coarctation of the aorta in infants less than 1 year of age are compared with those of an earlier series from the same institution. The significant improvement in mortality and morbidity statistics is attributed to modifications in operative and postoperative care. Operative mortality has decreased from 38 to 17 percent and the incidence rate of significant restenosis has diminished from 60 to 33 percent. It is suggested that in patients with large associated intracardiac shunt banding of the main pulmonary artery should be performed before resection of the coarctation. Three of five patients have survived procedures performed in this sequence. Microsurgical techniques and careful approximation of the aortic lumen with interrupted sutures are the major factors responsible for the reduced incidence of recoarctation. Prolonged ventilatory support postoperatively with the occasional addition of controlled positive airway pressure and continued aggressive medical therapy for heart failure are recommended.

Treatment of d-transposition of the great arteries: management of hypoxemia after balloon atrial septostomy.

Between 1975 and 1979, a group of 43 patients with d-transposition of the great arteries were diagnosed and underwent Rashkind balloon atrial septostomy at the time of initial catheterization. Thirty-six (88 percent) survived to the time of intraatrial baffle repair, and 31 (72 percent) are long-term survivors, 2 of them now awaiting repair. Palliative operations were performed in nine patients before definitive surgery; four of these patients are long-term survivors. Prostaglandin E1 infusion improved oxygenation and relieved acidosis in four patients. It is concluded that most patients with d-transposition of the great arteries will survive to elective intraatrial baffle repair between 6 and 12 months without surgical palliation in spite of significant hypoxemia. Prostaglandin E1 infusion may be lifesaving and provide sufficient palliation in patients with persistent hypoxemia and acidosis after balloon atrial septostomy.

Dysrhythmias following the Senning operation for dextro-transposition of the great arteries.

Twenty-nine patients have undergone the Senning operation for repair of dextro-transposition of the great arteries (TGA) since 1979 at St. Louis Children's Hospital. The early mortality has been comparable to that reported for the Mustard operation (10%). Electrocardiograms (ECGs) following the Senning operation reveal abnormalities similar to those noted after the Mustard operation; 63% of survivors were in normal sinus rhythm 1 week postoperatively, and 86% demonstrated normal sinus rhythm at a mean of 15 months after operation. Twenty-four hour ambulatory monitoring revealed normal sinus rhythm in 35% and occasional premature atrial or ventricular contractions in another 30%. No cases of sudden unexpected death have been noted, and no pacemakers have been inserted. Possible mechanisms for rhythm disturbances following venous transposition operations are discussed.

Concordance of electrocardiographic and scintigraphic criteria of myocardial injury after cardiac surgery.

The concordance of transmural electrocardiographic (ECG) changes and myocardial infarct imaging in detecting myocardial injury in the perioperative period was evaluated in 50 patients undergoing coronary artery bypass surgery and in 6 patients without coronary artery disease undergoing valve replacement. Scintigraphy with technetium-99m (Sn) labeled pyrophosphate was performed 3 to 7 days after surgery. Plasma creatine phosphokinase (CPK) levels were determined preoperatively and daily for 7 days postoperatively. Plasma MB-CPK was assayed fluorometrically in samples obtained at 12 hour intervals for 36 hours. Total CPK and MB-CPK concentrations were normal in all patients preoperatively but increased in every patient postoperatively. A total of 8 patients (16%) had evidence of perioperative infarction. Six of these patients (12%) with coronary artery disease exhibited abnormal images after bypass surgery, associated with transmural ECG changes in each case. The other 2 patients (4%) with coronary artery disease who had abnormal images were among 4 patients who developed bundle branch block after the operation. Abnormal images did not occur in any patient undergoing valve replacement despite total CPK and MB-CPK elevations. These results indicate that total CPK and MB-CPK elevations occur consistently after cardiac surgery and cannot be relied upon for detection of transmural infarction. Furthermore, new conduction defects may not necessarily be a sign of perioperative infarction, and infarct imaging may be a useful means of establishing myocardial infarction in this group of patients.

Preoperative three-dimensional reconstruction of the heart and great vessels in patients with congenital heart disease. Technique and initial results.

Magnetic resonance images have been obtained preoperatively in six patients with congenital heart disease. Contiguous sequences of electrocardiogram-triggered spin-echo images have been reconstructed in three-dimensional form to define the size and anatomic relationships to the great vessels and internal cardiac structures. Findings of magnetic resonance imaging were corroborated by angiographic and sector-scan echocardiographic studies and at operation. Individual scan slices were manually edited to separate the heart and great vessels from the blood within them and from extracardiac structures. Surface reconstruction software originally developed for craniofacial and orthopedic surgical planning was adapted for processing of cardiac magnetic resonance image sequences. Preoperative three-dimensional magnetic resonance imaging reconstructions were obtained in patients with aortic coarctation with ventricular septal defect, hypoplastic left ventricle, pulmonary artery atresia with ventricular septal defect, atrial septal defect, partial atrioventricular canal defect with anomalous pulmonary venous drainage, and tetralogy of Fallot with peripheral pulmonary artery stenosis. The reconstructions showed anatomic findings consistent with two-dimensional magnetic resonance imaging, echocardiography, cineangiography, and intraoperative findings. The three-dimensional images have a format that is familiar and consistent with the gross intraoperative appearance of the heart and great vessels. These three-dimensional images can facilitate the interpretation of magnetic resonance scan findings for cardiac surgeons without the sacrifice of significant clinical information.

Perioperative mortality rate in patients with unstable angina selected by exclusion of myocardial infarction.

Coronary bypass surgery may be associated with an increased perioperative mortality rate in patients with unstable compared to stable angina. The mortality rate is excessively high when surgery is performed during evolving myocardial infarction. Elevated plasma MB CPK isoenzyme activity is a remarkably sensitive and specific marker of myocardial damage. Accordingly, we studied 111 patients with unstable angina to determine whether exclusion of patients with initially elevated MB CPK improves the perioperative mortality rate. Plasma MB CPK activity was assayed prior to catheterization and every 2 hours therafter. Of the 111 patients, 16, with initially elevated MB CPK activity, were excluded and managed medically. Catheterization was performed in 59 patients, and severe vessel obstruction was documented in 55. Coronary bypass surgery performed in 47 patients was associated with a mortality rate of 4 per cent. Thus, after exclusion of patients with evolving infarction by MB CPK isoenzyme analysis, catheterization and coronary bypass surgery in patients with unstable angina resulted in a mortality rate comparable to that in patients with stable angina.

Thymectomy for myasthenia gravis in the young adult. Long-term results.

An 8 year experience with a prospective treatment program for patients with myasthenia gravis is reviewed with particular focus on the results in patients less than 35 years of age. Twenty myasthenic young adults with an average age of 24 years and a duration of symptoms of 22 months, excluding two with the juvenile form, had thymectomy followed by short-term anticholinesterase and long-term prednisone therapy. All of the 18 patients with a short duration of symptoms are in markedly improved condition and 61% of them are in remission after a mean postoperative period of 32 months. The longest follow-up period is 7.3 years. Two patients have mild improvement. There was no correlation between thymic disease and clinical result. Complete en bloc extirpation of all thymic and adjacent tissue through a median sternotomy is advocated. The patients were treated postoperatively with prednisone, 100 mg/day, a regimen which gradually is changed to every other day medication and finally a gradual reduction of dosage. There have been no operative or late deaths and no serious complications of therapy. The effects have been long lasting, with not a single instance of significant recurrence of symptoms of myasthenia gravis 6 months after thymectomy.

Comparison of bubble and membrane oxygenators in short and long perfusions.

Eighty patients had cardiopulmonary bypass (CPB), half having short (109 +/- 11 minutes) perfusions and half having long (188 +/- 14 min) perfusions. Twenty patients in each group were perfused with bubble oxygenators (Bentley, Harvey, or Galen) and 20 with membrane oxygenators (Modulung or Teflo). Hemodilution to a hematocrit value of 22.5% +/- 1.4% and hypothermia to 28 degrees +/- 2 degrees C were used in all patients. Complete hemograms, sequential multiple analyzer 18 tests, coagulation profiles, blood gases and pH, three immunoglobulins, and two complement fraction proteins were sampled as follows: three times before perfusion, one to ten times during perfusion, 1 hour immediately after perfusion, and 4, 24, and 48 hours postoperatively. Data in concentration terms were compared statistically and reported as mean and standard error for each subset. Additionally, rates of gain or loss were calculated in terms of quantity per liter of blood pumped per minute. During perfusion for both duration sets, use of a membrane oxygenator resulted in greater pump flows (4.55 +/- 0.15 L/min versus 3.75 +/- 0.11 L/min), lower total peripheral resistances (1,125 +/- 63 dynes.sec.cm-5 versus 1,652 +/- 115 dynes.sec.cm-5), and greater urinary outputs (9.4 +/- 1.1 ml/min versus 2.2 +/- 0.6 ml/min) than in the bubble oxygenator subsets. Comparisons of measured and calculated data in the immediate postperfusion interval showed no differences between bubble and membrane oxygenator subsets for short perfusions. In long perfusions, the membrane subset had lower plasma hemoglobin and white cell concentrations and generation rates, smaller (3 to 8 1/2 times) losses of IgG, IgM, C3 and shed blood necessitating less transfusion, and greater C4 losses. The membrane oxygenator systems used were more complex and costly and offered no advantages for short perfusion in adults. In anticipated long perfusions or where bleeding may be a problem, a membrane oxygenator appears more efficacious than bubble systems. For perfusions of less than 2 hours, membrane oxygenators had no biochemical or hematologic advantage over the bubble devices used in this study.

Infection control in cardiac surgery.

This report relates the results of a multifaceted, 4 year program directed toward reduction of infection in patients undergoing cardiac operations and extracorporeal circulation in a large teaching hospital. Retrospective analysis of all superficial and deep wound infections and prosthetic valve infections for the period of 1966 to 1970 and a prospective study of the period of 1970 to 1974 were made. The multifaceted program begun in 1970 consisted of (1) renovation of a cardiac operating room with incorporation of a high flow, vertical unidirectional ventilation system, (2) change in the gown and draping material for improvement of barriers to bacteriologic shedding, (3) frequent steam sterilization of prosthetic valves, (4) routine use of an antistaphylococcal agent in patients receiving valve replacement, and (5) an unannounced bacteriologic monitoring program of the cardiac operating room personnel. Studies of airborne particulates and bacteria and adequacy of skin preparation and hair removal also were conducted. The studies showed that (1) a high-flow HEPA filtered vertical ventilation system and altered operating room clothing reduced the concentration of airborne particles and the concentration of bacteria at the wound by a factor of 10 compared to conventional operating rooms, (2) the incidence of markedly contaminated scrubbed and unscrubbed hands decreased, (3) shedders and carriers were identified, and (4) current patient skin preparation and hair removal practices were satisfactory. The results of the program were a reduction of the deep wound infection rate from 2.9 to 0.6 percent (p less than 0.01) and a concomitant total wound infection decrease from 6.6 to 3.3 percent. Prosthetic valve infection rates decreased fourfold, from 5.6 to 1.4 percent. It is concluded that careful attention to possible endogenous sources of infection from the patient and a multifaceted program directed to exogenous sources of infection can lower infection rates in cardiac surgical patients.

Use of saphenous vein to extend and relocate coronary arteries: clinical experience during extensive reconstructive operations of the aortic root.

Eight patients with advanced disease of the aortic root involving the origins of the coronary arteries have been operated on with resection of the entire aortic root including the ostia of the coronary arteries. Reconstruction was performed by insertion of a composite prosthesis; extension and relocation of the proximal portion of the coronary arteries was accomplished with saphenous vein segments. Two patients had infected aortic valve prostheses, three patients had acute aortic dissection and three had annuloaortic ectasia. Seven of eight patients survived the operation, the only operative death occurring in a patient submitted to an operation in a shock state following rupture of the aorta. There was, however, a high incidence of subsequent late deaths resulting from continued infection and from central circulatory failure. An analysis of this series of patients suggests the possibility that a reconstruction that relocates and extends the proximal portion of the coronary circulation may provide a total blood flow that is inadequate or inappropriate, and further suggests that the methods used in the reconstruction of the proximal circulation may be critical to the prevention of myocardial hypoperfusion.

Reconstructive valve surgery.

Despite continued refinement of heart valve prostheses, valve replacement carries risks of thromboembolic, mechanical, and infectious complications, and long-term success is further limited by the eventual wear of prosthetic parts. In many patients with congenital or acquired valve diseases, valve function may be improved, if not restored, by reconstructive techniques, prosthetic replacement being thereby avoided or delayed. This review examines the current status of reconstructive procedures for management of diseased valves, with emphasis on long-term results and post-operative hemodynamic studies. In many instances the choice between reconstruction and replacement of a diseased valve remains controversial. The documented success of selectively applied reconstructive techniques, however, weighs against expedient decisions for prosthetic replacement and supports a continuing search for new techniques.

Current management of transposition of the great arteries: immediate septostomy, occasional prostaglandin infusion, and early Senning operations.

Between January, 1979, and September, 1982, 30 infants with dextro(D)-transposition of the great arteries were managed with the Senning procedure for transposition of ventricular inflow. In 11 infants under 6 months of age, there were no associated cardiac malformations and no hospital deaths. Among 17 infants operated on between the ages of 6 and 12 months, 6 had associated cardiac malformations, and there were 2 hospital deaths. Two infants in the series were over 12 months of age; 1 had an associated malformation, and there were no hospital deaths. Analysis of cardiac rhythms in the postoperative period demonstrates that the first 2 patients operated on continue to have persistent junctional escape rhythm, while the remaining 26 survivors are in sinus rhythm. Twenty-four-hour Holter monitoring performed in 24 patients showed only 9 patients to be in sinus rhythm throughout the entire recording period. Seven patients had occasional atrial and ventricular premature contractions; the remainder had episodes of sinus arrest with junctional escape rhythm. Evidence of pulmonary caval or pulmonary venous obstruction has not appeared in any patient. Recently introduced technical modifications to the Mustard procedure have improved the results of that operation in regard to rhythm disturbances and baffle obstruction to venous return. This series, therefore, does not demonstrate superiority of the Senning procedure over the Mustard procedure. However, since results comparable to those of the Mustard procedure can be obtained in very young infants using the Senning operation along with deep hypothermia and circulatory arrest, the Senning procedure is deemed preferable to the Mustard procedure for this age group because of the ease with which it can be performed and because the procedure eliminates surgical judgment, and thereby surgical error, in the location of suture lines.

Surgical management of hypoplastic right ventricle with pulmonary atresia or critical pulmonary stenosis and intact ventricular septum.

Our experience with the surgical management of hypoplastic right ventricle with intact ventricular septum includes 26 patients with pulmonary atresia and 4 with critical pulmonary stenosis. Group 1 consisted of 8 neonates managed initially by transventricular valvotomy; 6 later required a secondary procedure, with 100% survival. Group 2 had 11 neonates managed by aorta-pulmonary artery shunting without operative death. However, only 3 have survived over the long term and 1 has required an additional shunt procedure. Group 3 had 9 infants who underwent concomitant valvotomy and shunting. There were 4 operative deaths and 1 late death. Finally, Group 4 included 2 infants managed by primary repair at 3 days and 6 days old with prosthetic enlargement of the right ventricle; 1 required the addition of a shunt. Both are alive. Seven of the 15 patients in Groups 1, 2, and 3 who survived neonatal palliative procedures have undergone reparative operations. Two had no growth of the right ventricle and underwent repair after conversion to tricuspid atresia, by a Fontan procedure. Five had prosthetic enlargement of the right ventricle in childhood with 1 late death. Findings of this review were as follows: (1) effective palliation of pulmonary atresia and intact ventricular septum or critical pulmonary stenosis with cavitary hypoplasia of the right ventricle is rare unless transventricular flow can be established; (2) establishment of transventricular flow produces a high incidence of cavitary "growth," which permits later repair; (3) the Fontan operation is available for repair in patients who have no cavitary growth; and (4) when all three portions of the right ventricular cavity can be identified by angiography, a primary repair can be performed in the neonatal period with a good long-term prognosis.

Posterior ventricular aneurysm, severe mitral regurgitation, and rupture of the left ventricle after acute myocardial infarction: six-year survival after surgical correction.

Posterior ventricular aneurysm and severe mitral regurgitation due to acute myocardial infarction are rarely recognized during life. This report describes the successful surgical treatment of a patient with this combination of lesions who at operation was found to have rupture of the left ventricle as well. Aneurysmectomy, mitral valve replacement, and coronary artery bypass were performed with a gratifying late result. Aggressive investigation of patients with hemodynamic deterioration after posterior myocardial infarction may identify surgically correctable mechanical complications.

Massive pulmonary embolism permitting paradoxical systemic arterial embolism: successful surgical management.

The case of a young woman, receiving oral contraceptives, who developed massive pulmonary embolism producing circulatory collapse and paradoxical arterial embolism through a patent foramen ovale is documented.. Limb viability was threatened. Emergency management included removal of arterial and pulmonary emboli, surgical closure of the patent foramen ovale, inferior caval partitioning, ovarian vein ligation, and short-term anticoagulation. Recovery was rapid and complete.

Experimentally reversed pulmonary blood flow. Implications for the surgical management of complex forms of congenital heart disease.

Blood flow to the right lung was reversed in 28 dogs, 14 of which survived more than 1 month. Four successful long-term survivors (1 years to 4 years 7 months) were repeatedly studied by cardiac catheterization, angiography, and ventilation/perfusion scans as well as hemodynamically when they were killed. Hemodynamics and gas transfer were normal up to 4 1/2 years after the procedure, and the histology of the right lung was essentially normal. The application of reversed pulmonary blood flow is proposed in the surgical management of forms of congenital heart disease in which the right ventricle is diminutive. The procedure consists of repartitioning of the atria to allow venous blood to flow into the lung through the pulmonary veins and exit to the left atrium through the pulmonary artery.

Left ventricular aneurysm resection: indications and long-term follow-up.

Forty-four patients had resection of a chronic postinfarction left ventricular aneurysm. Operative indications were heart failure, angina, and ventricular arrhythmias. Twenty-six patients (59%) had coronary grafting in addition to aneurysmectomy. The operative mortality rate was 4.5% (2/44), and late mortality (mean follow-up, 31 months) was 17.9% (7/39). Preoperatively all patients were in New York Heart Association Functional Class III or IV; 91% were Class I or II postoperatively. Coronary bypass grafting did not increase the operative mortality rate, and long-term survival was similar between those receiving coronary grafts and those not receiving grafts. Postoperative ventriculograms were evaluated in 10 patients by means of a system of internal grids. Amount of regional myocardial contraction correlated well with the patient's postoperative functional capacity. It is concluded that ventricular aneurysmectomy in combination with coronary bypass grafting is safe and effective, resulting in marked improvement in the patients' functional capacity and longevity.

A new operation for far-advanced cystic medial necrosis of the aortic root.

Three patients with far-advanced cystic medial necrosis of the aorta, which had produced giant bulbous enlargement of the aortic root and severe aortic regurgitation, were operated on using a procedure not previously described. Measurements of the aortic valve annulus and ascending aorta were made from aortograms. A knitted polyester arterial prosthesis was sewn together to form a circle. This circular prosthesis was sewn to a 31 mm Björk-Shiley aortic valve prosthesis in the way that a tire is fitted onto a wheel. The resulting composite prosthesis, which had the same diameter as the aortic root, was used to replace the excised valve. In all cases a composite prosthesis measuring greater than 50 mm in diameter was used. In 2 of the 3 patients the ascending aorta was replaced with a tubular graft reshaped as a truncated cone. This reshaping was done by inserting multiple gussets into one end of the aortic prosthesis so that the flanged end fit precisely to the transverse aortic arch. Two patients are asymptomatic more than two years following operation. The third patient died suddenly of a ventricular arrhythmia on the twenty-third postoperative day.

Multiple valve replacement: changing status.

Review of our experience with multiple valve replacement over a 6-year-period revealed a recent marked reduction in operative risk. Operative mortality for 62 patients operated on before 1974 was 34%; for 44 patients operated on since 1974, mortality was only 11%. In both groups, mortality was clearly related to the preoperative cardiac functional status. Several recent technical advances including myocardial protection by topical hypothermia, shortened perfusion time, and the intraaortic balloon pump may all have contributed to the reduced operative risk.