George J. Reul

Congenital Malformations of the Coronary Arteries: The Texas Heart Institute Experience

Coronary artery anomalies, some of which are considered clinically insignificant, can be associated with other congenital heart defects, myocardial ischemia, and reduced life expectancy. We conducted a retrospective study to determine the efficacy of surgical treatment in 191 patients who had a total of 202 coronary artery anomalies, which were classified as anomalies of origin (88 patients), termination (93), or distribution (10). Of the 88 patients with anomalies of origin, 60 had a coronary artery arising from the pulmonary artery, 18 had a right coronary artery arising from the left anterior descending artery, and 10 had a coronary artery arising from the contralateral sinus of Valsalva. All patients with an anomaly of termination had a coronary arteriovenous fistula, and all patients with an anomaly of origin had a single coronary artery. The diagnostic and operative techniques for each of the defects are evaluated. Based on our experience, early diagnosis and surgical intervention can yield satisfactory results in patients with coronary artery anomalies, with most experiencing relief of symptoms. The operative procedures were associated with a low early and late mortality; in addition, few patients experienced complications.

Ischemic contracture of the heart: “Stone heart”

Open heart surgery using cardiopulmonary bypass has enjoyed an exciting 15 year history during which many technical modifications were made and baffling complications encountered. For the past 8 years we have routinely employed a simplified technique using simple intravenous solutions to “prime” the extracorporeal circuit, maintained the patient and his heart at normothermic levels and induced ischemic cardiac arrest to control or prevent myocardial injury during the period of bypass.1,2 Periods of induced ischemia have been tolerated by some patients for more than 2 hours without apparent myocardial damage. However, we have seen a relatively small number of patients who died in the operating room from an unusual type of myocardial failure. Rather than the poorly contracting, overdistended ventricle of myocardial failure, a small spastic heart, literally frozen in systole, develops. The ventricular chamber is decreased notably in volume because of the contracture, and even vigorous manual massage does not produce an adequate stroke volume. Changes in peripheral resistance, cardiotonic agents, electrolyte solutions, adrenergic. blocking and stimulating agents and assist devices have not altered its inevitable course. On palpation the heart is in a contracted state similar to the uterine contraction ring or the tetanic contraction of striated muscle as seen in the laboratory. This characteristic state is recognized readily at the time of surgery. Ultimately, cardiopulmonary bypass cannot be discontinued without the patient dying because of the maintained contractile state. We have named this irreversibly contracted ventricle the “stone heart.” Fortunately, the stone heart is rare. Of the 4,732 patients (1,407 for congenital lesions and 3,325 for acquired) who have undergone open heart surgery at the Texas Heart Institute during the 5 years from July 1966 to July 19’71, 51 patients (1 percent) died during operation from acute myocardial insufficiency. Of that group, less than a third (13 patients) experienced severe contracture of the heart and the criterion we recognize as stone heart. We have reviewed the clinical and patho-

Sinus of Valsalva aneurysm or fistula: management and outcome.

BACKGROUNDnFew large or long-term series exist regarding the management of patients with sinus of Valsalva aneurysms or fistulas (SVAFs).nnnMETHODSnBetween 1956 and 1997, 129 patients presented with a ruptured (64 cases; 49.6%) or nonruptured (65 cases; 50.4%) SVAF. The patients included 88 men and 41 women, with a mean age of 39.1 years. Associated findings included a history of endocarditis (42 cases; 32.6%), a bicuspid aortic valve (21 cases; 16.3%), a ventricular septal defect (15 cases; 11.6%), and Marfans syndrome (12 cases; 9.3%). Operative procedures included simple plication (61 cases; 47.3%), patch repair (52 cases; 40.3%), aortic root replacement (16 cases; 12.4%), and aortic valve replacement/repair (75 cases; 58.1%).nnnRESULTSnThere were five in-hospital deaths (3.9%): four due to preexisting sepsis and endocarditis and one that followed dehiscence of the repair in a patient with Marfans syndrome. Two patients (1.6%) had strokes during the early postoperative period. The survivors were followed up for 661.1 patient-years (5.3 years/patient). The following late complications occurred: prosthetic valve malfunction (5 cases; 3.9%), prosthetic valve endocarditis (3 cases; 2.3%), SVAF recurrence (2 cases; 1.6%), thrombosis (1 case; 0.8%), and anticoagulation-related bleeding (1 case; 0.8%).nnnCONCLUSIONSnResection and repair of SVAF entails an acceptably low operative risk and yields long-term freedom from symptoms. Early, aggressive treatment is recommended to prevent endocarditis or lesional enlargement, which causes worse symptoms and necessitates more extensive repair.

Resection of Aortic Arch Aneurysms: A Comparison of Hypothermic Techniques in 60 Patients

Hypothermic circulatory arrest has been used to facilitate resection of aneurysms of the aortic arch. During a five-year period, two methods of hypothermic arrest were compared in 60 patients. In Group 1, 20 patients underwent deep hypothermia (14 degrees to 18 degrees C) and circulatory arrest to allow repair of the transverse arch under optimal conditions. A hospital mortality of 50% occurred and was attributed to uncontrolled hemorrhage and cerebral or cardiac complications. In Group 2, modified techniques were employed in 40 patients and included moderate levels of hypothermia (22 degrees to 26 degrees C) and simplified operative methods, which reduced the duration of circulatory arrest and shortened the length of perfusion. Pretreatment of plasma-soaked Dacron grafts by autoclaving eliminated serious bleeding problems. A marked improvement in patient survival (90%) and reduction in postoperative complications were observed after adoption of these modifications. The improved results in the present series have reconfirmed our belief that this type of intervention is the preferred approach to aneurysms of the aortic arch.

Is an Integrated Approach Warranted for Concomitant Carotid and Coronary Artery Disease

BACKGROUNDnThe management of patients with severe, concomitant coronary and carotid artery occlusive disease is controversial.nnnMETHODSnBetween 1975 and 1996, 512 patients (mean age, 64.9 years; 70% male) were admitted for coronary revascularization; 316 (61.7%) had asymptomatic, severe carotid disease (stenosis > 70%) and 196 (38.3%) had symptomatic carotid disease (159 [31.1%] with transient ischemia and 37 [7.2%] with completed stroke). In group 1, coronary revascularization and carotid endarterectomy were simultaneously performed in 255 patients (49.8%) with unstable angina. In group 2 (staged approach), carotid endarterectomy was performed before coronary revascularization in 257 patients (50.2%) without unstable angina.nnnRESULTSnBefore 1986, the incidence of stroke and death was greater in group 1 (n = 149) than in group 2 (n = 156) (14 [9.4%] versus 4 [2.6%]; p < 0.01). Since 1986, outcomes in group 1 (n = 106) and group 2 (n = 101) have been similar for stroke (2 [1.9%] versus 2 [2.0%]), death (4 [3.8%] versus 3 [3.0%]), and myocardial infarction (4 [3.8%] versus 5 [5.0%]). Significant univariate and multivariate predictors of adverse outcome were primarily heart-related (reoperation, intraaortic balloon use, ejection fraction < 0.50, and angina grade 4 for death; age > 70 years and congestive heart failure for stroke).nnnCONCLUSIONSnDespite highly selected populations, contemporary surgical results do not indicate that staged treatment of severe, concomitant coronary and carotid artery occlusive disease has an advantage over simultaneous treatment. Advances in myocardial protection and perioperative hemodynamic management may account for the low incidences of stroke and death in these operations.

Ascending Aorta-Abdominal Aorta Bypass: Indications, Technique, and Report of 12 Patients

Use of the supraceliac segment of the abdominal aorta for ascending aorta-abdominal aorta bypass (AAAAB) offers a new technique for management of certain difficult surgical problems. Since 1973, we have performed AAAAB in 12 patients: 4 with recurrent coarctation of the thoracic aorta; 4 with coarctation of the thoracic aorta and associated cardiac lesions requiring a concomitant intracardiac procedure; 2 with recurrent aortoiliac occlusive disease (AIOD); 1 with interruption of the aortic arch requiring concomitant pulmonary artery banding; and 1 with coarctation of the abdominal aorta. In 3 of these patients (2 with recurrent AIOD and 1 with coarctation of the abdominal aorta) the distal anastomosis was made to the distal abdominal aorta or femoral arteries. Ten patients (83.3%) experienced satisfactory results; 2 patients (16.6%) died. The technique of AAAAB provides a practical solution to complex situations in which previous procedures preclude a standard operative approach, or when necessary concomitant procedures would otherwise require a two-stage operation.

Cor Triatriatum: Operative Results in 25 Patients

Twenty-five patients with cor triatriatum underwent surgical correction at the Texas Heart Institute during a 21-year period from 1959 to 1980. Patients ranged in age from 4 months to 38 years. Diagnosis was established preoperatively in 14 patients (56%), at the time of operation for correction of associated lesions in 10 patients (40%), and during reoperation in 1 (4%). In the earlier part of this series, diagnosis was more common at the time of operation. Associated cardiovascular anomalies were present in 20 patients (80%), major anomalies in 14 others (56%). Simultaneous correction or palliation of associated lesions and total excision of the anomalous membrane were done in 18 patients utilizing temporary cardiopulmonary bypass. Cor triatriatum alone was repaired in 5 patients (20%) with the aid of extracorporeal circulation. Two patients underwent reoperation: 1 because of incomplete excision of the septum and the other because the condition had not been diagnosed during a first operation for correction of total anomalous pulmonary venous return. Excision of the membrane was accomplished utilizing the left atrium in 10 patients (40%), the right atrium in 12 (48%), or both in 3 (12%). Four patients (16%) died early after operation; all were infants who had severe associated cardiac anomalies. Among the 21 surviving patients, results were excellent in 20.

Sinus Venosus Atrial Septal Defect: Early and Late Results Following Closure in 109 Patients

The clinical course of 109 patients who underwent closure of a sinus venosus atrial septal defect is reviewed, with emphasis on the incidence, type, and severity of arrhythmias before and after operation. There were no operative deaths and only 1 late death. No instances of obstruction of the superior vena cava were detected clinically. One patient had a probable hemorrhagic pulmonary infarction that cleared; another may have a persistent left-to-right shunt. Excellent results were achieved in 72% of the patients, good results in 5%, and poor results in 3%. The type of arrhythmia, both before and after operation, varied with the age of the patient: younger patients had bradyarrhythmias, and older patients had tachyarrhythmias. Ten patients experienced persistent new arrhythmias postoperatively, but none were disabled, required a pacemaker, or died. The arrhythmias in all severely symptomatic patients were supraventricular tachycardias that had occurred before operation. Although functional classification after operation was clearly related to age at the time of the procedure (with younger patients having the best functional results), 19 of 21 patients over age 40 were noticeably improved after surgical closure of the sinus venosus atrial septal defect.

Primary cardiac tumors in infants and children: Immediate and long-term operative results

BACKGROUNDnThe literature contains few large or long-term series involving infants and children with primary cardiac tumors. This article summarizes our 35-year experience with such lesions.nnnMETHODSnBetween January 1961 and January 1996, 40 infants and children (mean age, 3.3 years; range, 2 days to 17 years; 65% female) were diagnosed at our institution with primary cardiac tumors. Of these tumors, 37 (92%) were benign and 3 (8%) were malignant. Tumors were resected in 38 patients (95%). In 2 patients (5%), biopsy confirmed rhabdomyoma; however, presenting symptoms spontaneously resolved, so these patients did not undergo tumor resection. Follow-up echocardiographic studies showed a diminishing tumor mass in each of these patients.nnnRESULTSnImmediate, symptom-free status was achieved in all patients. There were two early deaths, for an operative mortality of 5%. Three late postoperative deaths (7.5%) occurred as follows: 1 patient with a myocardial hamartoma died at 3 months of congestive heart failure. Another patient with a recurrent rhabdomyosarcoma died at 6 months, and a third patient with a recurrent fibrosarcoma died at 28 months. Long-term follow-up was available for 34 survivors (97% complete) and totaled 240.2 patient-years (mean, 7.1 years/patient). All remaining survivors were without evidence of presenting symptoms and tumor recurrence or progression.nnnCONCLUSIONSnThe data suggest that an aggressive operative approach is warranted for benign symptomatic and malignant tumors. This aggressive approach has resulted in extended symptom-free status in patients with benign lesions, and significant palliation and longer survival in patients with malignant lesions, with acceptably low operative risk.

Coronary artery bypass in patients over 70 years of age: Indications and results

With recent advances in all phases of coronary care and the increasing success of coronary arterial surgery, operative treatment of coronary artery disease is more readily recommended, even for patients over 70 years of age. In a series of 3,730 patients who underwent aortocoronary bypass from November 1969 through June 1974, there were 95 patients who were 70 years of age or older. The primary indication for surgery was severe angina, which was present in 88 patients. The mean coronary arterial score was 9.51. Associated valvular lesions were treated surgically in 21 patients, and a left ventricular aneurysm was resected in 7. Improvements in surgical technique and postoperative care are responsible for the hospital mortality rate of only 4.8 percent in the 21 patients operated upon during the first 6 months of 1974 compared with the overall mortality rate of 22.1 percent in all 95 patients. Long-term follow-up among the 95 patients includes data from 33 patients: 9 patients whose condition improved, 21 who were asymptomatic and 1 coronary death.