Sinus of Valsalva aneurysm or fistula: management and outcome.

BACKGROUND: Few large or long-term series exist regarding the management of patients with sinus of Valsalva aneurysms or fistulas (SVAFs). METHODS: Between 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 Marfan's 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%). RESULTS: There 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 Marfan's 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%). CONCLUSIONS: Resection 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.

The use of subcutaneous drains to manage subcutaneous emphysema.

Subcutaneous emphysema is a frequent complication of thoracic and cardiac surgical procedures, and emergency tracheostomy is often advocated as the treatment for this complication. However, we report the case of a patient in whom massive subcutaneous emphysema, which had developed after emergent replacement of the aortic root, was relieved using subcutaneous drains and suction, instead of a tracheostomy. We found that the subcutaneous drains provided effective decompression of the head and neck areas, and markedly reduced airway pressure and subcutaneous air. We recommend subcutaneous drains for safe, effective, and inexpensive management of massive subcutaneous emphysema.

Echocardiographic diagnosis of left ventricular outflow tract obstruction caused by an acquired subaortic membrane after mitral valve replacement.

Although an acquired subaortic membrane has been reported as a cause of left ventricular outflow tract (LVOT) obstruction in various clinical settings, it previously has not been reported after mitral valve surgery. We describe 3 cases of acquired LVOT obstruction that resulted from development of a subaortic membrane after mitral valve replacement. This report emphasizes the role of an acquired subaortic membrane in LVOT obstruction after mitral valve replacement, the use of echocardiography in diagnosing this condition, and the importance of early intervention.

Limited-access coronary artery bypass grafting. The Texas Heart Institute experience.

Limited-access coronary artery bypass grafting, without the aid of cardiopulmonary bypass, is being performed with increased frequency, but its indications are not well defined. To determine the outcome of, and indications for, this procedure, we analyzed our experience with limited-access coronary artery bypass grafting. Between February 1996 and June 1998, 84 patients underwent limited-access coronary artery bypass grafting at our institution. We retrospectively divided these patients into 2 groups: a high-risk group with complex disease and multiple comorbidities (n = 56), and a low-risk group with uncomplicated disease (n = 28). There were 2 perioperative deaths (2%), and both of them occurred in high-risk cases. Early and late complications included myocardial infarction (2 cases), recurrent angina necessitating revascularization (2 cases), and multisystem dysfunction (1 case). Compared with conventional bypass grafting, limited-access coronary artery bypass grafting offered a smaller skin incision, fewer arrhythmias, less blood loss, less need for inotropic drugs, shorter hospitalization, lower cost, and quicker recovery time. Limited-access coronary artery bypass grafting might have a role in treating high-risk patients who have complex disease and require single-vessel bypass. Anastomosis can be challenging, however, if the target coronary artery is small, calcific, or intramyocardial. Moreover, the long-term results are unknown. Therefore, nonselective use of limited-access coronary artery bypass grafting is unjustified.

Left ventricular performance following the arterial switch operation: use of noninvasive wall stress analysis in the postoperative period.

OBJECTIVE: To determine postoperative left ventricular mechanics following the arterial switch operation (ASO). DESIGN: Prospective, cohort study. SETTING: Pediatric cardiac recovery room. PATIENTS: Nine neonates with transposition of the great arteries undergoing the ASO within the first week of life. INTERVENTIONS: Noninvasive ejection phase indices: shortening fraction (% SF), corrected mean velocity of circumferential shortening (VCFc), and wall stress analysis were used to calculate indices of specific left ventricular systolic mechanics. The % SF and VCFc were respectively adjusted for left ventricular afterload (end-systolic wall stress) to derive an index for left ventricular performance (stress-shortening relation) and contractility (stress-velocity relation). Left ventricular preload was assessed as the variance between the performance and contractility indices. All indexed data are reported as mean Zscore (i.e., number of standard deviations from the mean of a normal age- and body surface area-adjusted population). A mean Zscore of < -2 or > 2 was regarded as a significant variance from normal. Transmitral Doppler flow patterns were recorded at each postoperative interval and analyzed for isovolumic relaxation time (IVRT) as an index of left ventricular compliance. MEASUREMENTS AND MAIN RESULTS: All nine patients did well clinically and completed the study. Noninvasive parameters were measured at mean intervals of 3 (early), 23 (intermediate), and 48 hrs (late postoperative) relative to the time of arrival in the cardiac recovery room. Postoperative left ventricular performance was decreased throughout the early (-4.0 +/- 1.5 SD), intermediate (-4.1 +/- 2.8), and late (-3.5 +/- 1.3) phases of recovery. In contrast, the overall left ventricular contractility remained normal throughout the three postoperative intervals (0.2 +/- 1.8, -1.2 +/- 1.9, and -1.0 +/- 1.6, respectively), although three of the nine patients had a diminished stress-velocity index during the study period. Left ventricular afterload was within normal range in the early (0.1 +/- 1.7) and intermediate (1.5 +/- 1.9) phases of recovery, but increased in the late postoperative period (2.5 +/- 2.9). Left ventricular preload was decreased significantly throughout the early (-4.2 +/- 1.3), intermediate (-2.8 +/- 2.0), and late (-2.5 +/- 1.0) postoperative phases. All nine patients demonstrated decreased preload during the recovery period. IVRT was decreased in the post-ASO patients at each phase of recovery compared with normal data (p < .001). CONCLUSIONS: Left ventricular performance is impaired in infants during the period immediately following the ASO. A persistent preload deficit closely matches the pattern of impaired ventricular performance. Decreased IVRT points to impaired ventricular compliance as the etiology of the altered preload. In contrast, left ventricular contractility remains normal in the majority of post-ASO patients. Decreased contractility may account for impaired ventricular performance in selected cases.

Concomitant occlusive disease of the coronary arteries and great vessels.

BACKGROUND: Although an increasing number of elderly and high-risk patients, including those with generalized atherosclerosis, are undergoing coronary revascularization, few reports exist regarding the management of patients who have both occlusive disease of the great vessels and coronary artery disease. METHODS: Between 1972 and 1996, 31 consecutive patients (mean age, 56.5 years; 74% men) with multivessel coronary artery disease and symptomatic occlusive disease of the great vessels (25 single-vessel, 80.6%; 6 multiple-vessel, 19.4%) had 40 great vessels reconstructed by transthoracic bypass (n = 17, 42.5%), transthoracic endarterectomy (n = 8, 20%), or extrathoracic bypass (n = 15, 37.5%). All patients had simultaneous coronary artery bypass grafting (mean, 2.6 grafts per patient), and 8 patients had 10 distal carotid bifurcation endarterectomies (6 staged, 4 simultaneous). RESULTS: The early primary patency rate was 100%, and symptoms resolved completely in all 31 patients. There was 1 in-hospital death (3.2%) in a patient who had a respiratory arrest 11 days after operation. Perioperative morbidity included two myocardial infarctions (6.5%) and one opposite-hemisphere, embolic stroke (3.2%). Long-term follow-up of the 30 survivors (167.4 patient-years; mean, 5.6 years per patient) documented 5- and 10-year actuarial survival rates of 88.6% and 60.4%, respectively, with a 100% late brachiocephalic primary patency rate. Ten-year actuarial rates of freedom from the following events were as follows: death, 60.4%; myocardial infarction, 82.5%; stroke, 90.9%; percutaneous transluminal coronary angioplasty or redo coronary artery bypass grafting, 95.2%; and vascular operation or amputation, 78.4%. CONCLUSIONS: Depending on the anatomic distribution of the disease, an integrated approach to great vessel reconstruction that incorporated transthoracic and extrathoracic approaches and techniques of endarterectomy and bypass resulted in few adverse outcomes and excellent long-term patency. Simultaneous revascularization of the great vessels and coronary arteries can produce immediate and long-term, symptom-free outcome with acceptably low operative risk.

Who does the family court refer for psychiatric services?

Demographic differences between adolescents referred for psychiatric services by the Family Court and by facility staff at a state-run juvenile justice evaluation center are examined. Those groups are then compared to the facility's general population. It is concluded that race, gender, age, and judicial discretion are the factors that distinguish court-referred adolescents from their counterparts referred by facility staff and in the general population.

Risk factors for life-threatening cavopulmonary thrombosis in patients undergoing bidirectional superior cavopulmonary shunt: an exploratory study.

We have observed six patients with life-threatening superior vena caval or pulmonary thrombosis after bidirectional superior cavopulmonary shunt. With the use of a case control study we sought to identify perioperative risk factors for this thrombotic complication. Medical records of six patients with cavopulmonary thrombosis and those of 24 patients in a control group were reviewed to abstract data for potential risk factors. Contingency tables and univariate logistic regression were used to determine associations between various perioperative parameters and occurrence of cavopulmonary thrombosis. Preoperative variables associated with thrombosis included bilateral superior vena cavae, odds ratio: 23, p = 0.02, increased age at surgery (p = 0.05), and female sex (odds ratio: 7, p = 0.05). The McGoon Ratio (index of relative pulmonary artery branch diameter) was inversely related to thrombosis risk (p = 0.08). Two torr increases in mean right atrial (p = 0.08) or ventricular end-diastolic (p = 0.05) pressures were associated with approximately 70% increases in thrombosis risk. Intraoperative prolongation of aortic cross-clamp time related directly to thrombosis risk (p = 0.06). Postoperative variables associated with thrombosis included increased superior vena caval pressure within 12 hours after surgery (odds ratio > or = 10 for 5 torr increase in pressure, p = 0.02) and poor ventricular function (odds ratio: 9, p = 0.06) We conclude that high risk variables for patients undergoing a cavopulmonary shunt include bilateral superior vena cavae, female sex, increasing age, decreased McGoon Ratio, and elevated right atrial and ventricular end-diastolic pressure (before surgery), patients with prolonged aortic cross-clamp time (during surgery), and patients with elevated superior vena caval pressure and poor ventricular function (after surgery).

Is an integrated approach warranted for concomitant carotid and coronary artery disease?

BACKGROUND: The management of patients with severe, concomitant coronary and carotid artery occlusive disease is controversial. METHODS: Between 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. RESULTS: Before 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). CONCLUSIONS: Despite 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.

Congenital heart surgery in Houston. The early years.

During the 1950s and 1960s, major advances in medicine significantly influenced the development and application of surgery as treatment for congenital heart disease. The Texas Medical Center in Houston was at the forefront of these pioneering efforts and thus played an important role in the development of the art and science of congenital heart surgery.

Influence of age on the effect of bidirectional cavopulmonary anastomosis on left ventricular volume, mass and ejection fraction.

OBJECTIVES: We sought to identify age-related differences in the ventricular response of patients after bidirectional cavopulmonary anastomosis (CPA) and to compare changes in the ventricular response among children < 3 years of age who underwent CPA with that of age-matched control subjects who had a systemic to pulmonary artery shunt alone. BACKGROUND: Pre-Fontan CPA has been advocated over a systemic to pulmonary artery shunt alone in patients with a single ventricle to facilitate ventricular volume unloading and minimize risk of the Fontan operation. METHODS: Our study evaluated 23 patients who initially received a systemic to pulmonary artery shunt as an initial procedure before subsequent Fontan palliation. In eight of these patients (group I), bidirectional CPA was performed before age 3 years, and in four (group II), it was performed after age 10 years. The remaining 11 patients (group III, age and weight control group for group I) were maintained with their initial shunt until they underwent Fontan palliation. Serial echocardiographic analysis was used retrospectively to evaluate left ventricular volume and mass and systolic pump function (ejection fraction) before and after bidirectional CPA. RESULTS: Through 10 months of follow-up, group I patients showed significant decreases in indexed end-diastolic volume both after CPA (120 ml/m1.5 body surface area vs. 78 ml/m1.5, p = 0.001) and in comparison with values in patients in group II and III, who showed no changes in end-diastolic volume (p < 0.001). Indexed ventricular mass decreased moderately after bidirectional CPA in group I (from 228 g/m1.5 body surface area to 148 g/m1.5) but remained unchanged in groups II and III. The differences in trends between groups I and III were significant (p = 0.03). Ejection fraction decreased significantly in group II versus group I patients (0.48 to 0.27 vs. 0.51 to 0.52, p < 0.05) after CPA. Oxygen saturation measurements before and after bidirectional CPA revealed a significant increase in group I (73% to 86%, p < 0.001) and a decrease in group II (82% to 73%, p < 0.01). CONCLUSIONS: Bidirectional CPA facilitates ventricular volume unloading and promotes regression of left ventricular mass in younger children (< 3 years) in preparation for a Fontan operation. In contrast, bidirectional CPA is of questionable value in older children as a staging procedure for Fontan palliation.

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

BACKGROUND: The 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. METHODS: Between 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. RESULTS: Immediate, 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. CONCLUSIONS: The 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.

Left thoracotomy for distal tracheal repair.

When consulted emergently by another surgeon in the operating room, we accomplished repair of a major laceration of the posterior wall of the distal trachea with associated avulsion of the left upper-lobe bronchus via the existing left thoracotomy exposure in a 7-year-old girl. Mobilization of the descending aorta anteriorly provided adequate exposure of the tracheal injury.

Severe carbon monoxide poisoning in the pediatric patient: a case report.

A 10-yr-old female presented at Deaconess Medical Center, Spokane WA, comatose after being rescued from a house fire. Her carboxyhemoglobin was 48%. An 11-yr-old playmate presented with a carboxyhemoglobin level of 51% and later expired. Our patient was treated with hyperbaric oxygen therapy and manual artificial ventilation for 2.5 h. She recovered fully, and 7 mo later has had no neurologic deficits. Carbon monoxide bonds to the hemoglobin more tightly than oxygen, displacing the oxygen hemoglobin dissociation curve to the left and resulting in tissue hypoxia and hypotension. Carbon monoxide also exerts a negative influence on the electron transport chain, may lead to delayed neurologic sequelae because of free radical formation, and produces profound changes in the myocardium. Once seen as a capricious treatment for many symptoms, hyperbaric oxygen therapy is now an accepted treatment for carbon monoxide poisoning. The outcome suggests that in a patient whose prognosis is grave, a good outcome may be achieved with aggressive HBO therapy.

Carotid endarterectomy. Results in asymptomatic and symptomatic patients.

Objective evidence of the benefit of carotid endarterectomy in preventing stroke and its significant sequelae has recently been demonstrated by prospective trials. The salutary results depend on meeting strict operative outcome criteria as established by the American Heart Association. We retrospectively analyzed 265 consecutive carotid endarterectomies performed in 248 patients during 1 year at our institution. The perioperative mortality rate was 0; late mortality occurred 6 months postoperatively in 1 of 2 patients who experienced a perioperative stroke. The combined perioperative mortality and stroke rate was 0.8%. The combined mortality and stroke rate in patient subgroups was 0.7% (1/151) for asymptomatic patients, 1.6% (1/64) for symptomatic patients who had presented with a transient ischemic attack, and 0% (0/50) for symptomatic patients who had presented with a completed stroke. We conclude that the objective postoperative benefits of carotid endarterectomy in treating extracranial cerebrovascular disease can be achieved with low perioperative patient morbidity.

Critical decision analysis for extracranial cerebrovascular disease.

Results from 6 major prospective studies that have recently been either completed, or terminated prematurely, provide compelling evidence of the benefit of carotid endarterectomy in treating certain groups of patients who have carotid stenosis. Results of these studies show that symptomatic patients (those experiencing transient ischemic attack, amaurosis, or completed mild stroke) with a 70% ipsilateral carotid stenosis have an absolute risk reduction of 39% to 65% for stroke or death when treated with carotid endarterectomy as opposed to medical therapy alone. Asymptomatic patients with a 60% ipsilateral carotid stenosis have 53% absolute risk reduction for stroke or death when treated with carotid endarterectomy, rather than medical therapy alone. Combined neurologic morbidity and perioperative mortality rates for treating carotid stenosis should not exceed 3% in the asymptomatic patient or 5% to 7% in the symptomatic patient, on the basis of criteria established by the American Heart Association. These studies show that prophylactic carotid endarterectomy can effectively reduce the risk of stroke in both asymptomatic and symptomatic patients. Centers specializing in vascular surgery can benefit patients by minimizing the operative risk to levels well below those established by the American Heart Association.

Concomitant repair of complete atrioventricular canal defect and transposition of the great arteries in an infant.

The case of an infant who was born with the rare presentation of complete atrioventricular canal defect and transposition of the great arteries is described. The patient underwent pulmonary banding at the age of 2 months, followed by concomitant arterial switch and repair of the atrioventricular canal defect at 20 months. Palliative banding allowed for the infant to grow and thus facilitated the later successful repair of these congenital defects.

Amphetamine sensitivity in open-field activity vs. the prepulse inhibition paradigm.

Amphetamine-induced mesolimbic dopamine release has been reported to reduce prepulse inhibition of the acoustic startle response. In addition, it is well known that mesolimbic dopamine stimulation leads to hyperactivity. The present study was undertaken to explore the possibility that one or the other measure may be a more sensitive in vivo indicator of dopamine release in the nucleus accumbens by determining if the amphetamine dose-response curves for these two behavioral measures were different. The data indicate that the dose-response curves obtained for the different behavioral measures are identical. These data are consistent with the idea that the same dopamine terminal field supports both prepulse inhibiton of the acoustic startle response and dopamine-stimulated hyperactivity.

Lifelong management of patients with a single functional ventricle: a protocol.

Of the wide variety of congenital heart defects, the single functional ventricle continues to be one of the most enigmatic. Compared with patients who have complex congenital heart defects and 2 functional ventricles, patients with a single functional ventricle have greater surgical and long-term morbidity and mortality, and use more medical resources. Recent investigations have shown that patients with a single functional ventricle often have very satisfactory hemodynamics soon after a modified Fontan repair but then develop dilated cardiomyopathy during the subsequent decade. The cause and pathogenesis of the cardiomyopathy have not yet been determined. Many publications have examined individual aspects of the care of these patients based on retrospective reviews, but very few have provided comprehensive prospective methods for managing patients with a single functional ventricle. We hypothesized that a comprehensive management protocol with regular review of the results would provide a better understanding of the problems encountered in these patients and thus improve long-term outcome. Herein, we present our initial protocol for the lifelong management of patients with a single functional ventricle.