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Biomedical subjects

C E Duffy

Publications and source records attributed to C E Duffy.

At least 37 records · Page 2Linked to original sources

Comparison of single versus multidose blood cardioplegia in arterial switch procedures.

Fifty-three patients with transposition of the great arteries and Taussig-Bing anomaly undergoing an arterial switch procedure were divided into two groups. Group 1 (N = 32) received multidose cardioplegia injected initially into the aortic root and subsequently into the coronary artery orifices and Group 2 (N = 21), single-dose cardioplegia injected into the aortic root. The mean aortic cross-clamp and bypass times were generally longer in Group 1 compared with Group 2. Group 1 patients with simple transposition undergoing primary repair (N = 15) had an aortic cross-clamp time of 80 +/- 8 minutes and a bypass time of 203 +/- 27 minutes versus 64 +/- 6 minutes (p less than 0.001) and 170 +/- 15 minutes (p less than 0.01), respectively, for similar patients in Group 2 (N = 10). Group 1 patients with simple transposition undergoing staged repair (N = 7) had an aortic cross-clamp time of 71 +/- 6 minutes and a bypass time of 201 +/- 24 minutes versus 66 +/- 4 minutes (p = not significant [NS]) and 226 +/- 25 minutes (p = NS), respectively, for Group 2 (N = 6). In Group 1 patients with complex transposition (N = 10), the aortic bypass time was 79 +/- 12 minutes and the bypass time was 261 +/- 40 minutes versus 64 +/- 11 minutes (p less than 0.05) and 225 +/- 16 minutes (p less than 0.1), respectively, for Group 2 (N = 5). Early mortality was 16% (5/32) in Group 1; there were no early deaths in Group 2.(ABSTRACT TRUNCATED AT 250 WORDS)

Aorta↗

Prevention of giant coronary artery aneurysms in Kawasaki disease by intravenous gamma globulin therapy.

Previous studies have demonstrated the efficacy of intravenous gamma globulin in the prevention of coronary artery abnormalities in Kawasaki disease. We retrospectively reviewed our single-hospital experience with patients in whom Kawasaki disease was diagnosed from January 1979 to July 1987. Only 3 of 68 (4%) patients treated with intravenous gamma globulin in the first 10 days of illness developed coronary artery abnormalities (one of the three had abnormalities before gamma globulin therapy), in comparison with 39 of 119 (33%) patients not treated with gamma globulin (p less than 0.001). Giant coronary artery aneurysms, which are associated with the greatest morbidity and mortality rates in Kawasaki disease, developed in none of the 68 patients treated with gamma globulin but in 7 of 119 patients (6%) not treated with gamma globulin (p = 0.04). Intravenous gamma globulin appears to be effective not only in reducing the overall prevalence of coronary artery abnormalities in Kawasaki disease but, more important, in preventing the formation of giant aneurysms, the most serious form of coronary abnormality after this illness.

Child, Preschool↗

Transposition of the great arteries with intact ventricular septum. Arterial switch in the first month of life.

Twenty-three infants with simple transposition of the great arteries and intact ventricular septum were operated on from October 1983 to October 1986. The age at operation in 22 infants ranged from 2 to 21 days and in one was 35 days (mean 9.82 +/- 6.86 days). The infants were evaluated with cardiac catheterization at 1 to 27 days of age. Twenty-two infants had balloon atrial septostomy, and 22 received prostaglandin E1 infusion. The left ventricular diastolic wall thickness, assessed by M-mode echocardiograms, varied between 2.8 and 4 mm. There were two hospital deaths in this group of 23 infants (mortality 8.6%), and there were no late deaths. All surviving patients are doing well clinically. One patient had asymptomatic nonsustained ventricular tachycardia necessitating phenytoin. Postoperative echocardiographic assessment performed on 15 patients at 0.93 +/- 0.61 years of age and cardiac catheterization and angiographic studies on seven patients at 1.07 +/- 0.13 years after operation revealed excellent ventricular performance, good semilunar valve function, and mild gradient at the right ventricular outflow with a mean right ventricular pressure of 37.4 +/- 4.1 torr.

Aorta↗

Arterial switch in simple and complex transposition of the great arteries.

Arterial switch for repair of transposition of the great arteries was performed on 53 patients since October 1983. These patients were divided into three groups: group I, 25 infants with an intact ventricular septum who had primary repair in the first month of life (2 to 34 days of age, mean 9.7 +/- 6.6); group II, 13 patients with an intact ventricular septum who had anatomic repair after a preliminary procedure (pulmonary artery banding in 13, shunt in 10, atrial septectomy in 1); and group III, 15 infants with transposition of the great arteries and ventricular septal defect. In group III, six patients had Taussig-Bing abnormality, nine had previous pulmonary artery banding, three had coarctation of the aorta repaired earlier in life, and four were less than 2 weeks old. Overall early mortality was 9.4% (5/53: group I 8%, group II 7.6%, group III 13.3%). Two late deaths occurred in group II 10 and 12 weeks postoperatively after infection and high fever. A third late death 18 weeks postoperatively was due to aspiration in an infant with Goldenhar's syndrome. Mortality and morbidity decreased significantly after an initial learning period (no deaths from July 1985 to March 1987 overall, and none in the last 15 infants operated on in group I). The surviving 45 patients are doing well. All have normal sinus rhythm. Two had transient asymptomatic arrhythmias. Left and right ventricular function assessed by echocardiogram and postoperative cardiac catheterization were within normal ranges in all but two patients, one with pulmonary artery stenosis and one (Taussig-Bing abnormality with two large ventricular septal defects) with severe pulmonary vascular disease (9.6 units) observed before anatomic repair. The right ventricular pressure at catheterization ranged from 27 to 42 mm Hg in 12 patients and was 55 mm Hg in two. There was no aortic stenosis. Aortic insufficiency was trivial in three patients and mild in one. We conclude that excellent results can be obtained with arterial switch for transposition of the great arteries with or without ventricular septal defect, especially in neonates.

Cardiac Catheterization↗

Usefulness of echocardiographic evidence of pericardial effusion and mitral regurgitation during the acute stage in predicting development of coronary arterial aneurysms in the late stage of Kawasaki disease.

In 28 patients with Kawasaki disease, the relation of specific echocardiographic findings identified during the acute study of the illness, including valvular regurgitation, to development of coronary aneurysms was evaluated. Initial studies were performed at the time of clinical presentation, 5 to 10 days after the onset of fever, and follow-up studies were performed 1 to 2 months later. Patients in whom coronary aneurysms developed were more likely to have pericardial effusion (p = 0.0006) or mitral regurgitation (MR) (p = 0.014) at initial echocardiographic study than those without aneurysms. Presence of either mitral regurgitation (MR) or pericardial effusion had a positive predictive value of 0.84 for aneurysm development. Twenty-three percent of patients had MR, and it was associated with mild LV dilatation (35 +/- 3 vs 32 +/- 5 mm, p less than 0.05). Insufficiency of other valves was rare. Thus, MR and pericardial effusion on acute phase echocardiographic examination may predict development of coronary aneurysms in Kawasaki disease. Mild MR occurs frequently in acute Kawasaki disease and is associated with mild LV dilation.

Child↗

Valve replacement in children: guidelines for selection of prosthesis and timing of surgical intervention.

One hundred fifty-nine patients ranging from 3 months to 18 years old (mean, 8.1 +/- 3.7 years) underwent 162 primary valve implantations. A porcine valve was used in 104 patients, a St. Jude Medical valve in 40, and a Björk-Shiley valve in 18. The valve replaced was the aortic in 25 patients, the mitral (systemic atrioventricular [AV] valve) in 43, the pulmonary in 71, and the tricuspid (pulmonary AV valve) in 23. Hospital mortality was 6%. Patients with a Björk-Shiley valve received warfarin sodium anticoagulation, and those with a St. Jude Medical valve were given salicylates and dipyridamole. Follow-up is available on all patients 0.6 to 12 years postoperatively (mean, 6.3 +/- 2.6 years). New York Heart Association Functional Class improved in 62% and remained unchanged in 38% of the patients. Thromboembolic complications occurred in only 8 (57%) of 14 patients with a St. Jude Medical valve in the right (pulmonary) side and in 3 (12%) of 26 with the valve in the left (systemic) side of the circulation. Bacterial endocarditis developed in 3 patients, all with porcine valves. Early valve replacement, less than 2 years after detection of hemodynamic deterioration, resulted in improvement in the ventricular ejection fraction in 25 of 29 patients (from 81 +/- 14% to 90 +/- 12% of normal; p less than 0.05). In contrast, the ejection fraction remained abnormal in all 22 patients with delayed valve insertion (more than 2 years) (81 +/- 16% of normal preoperatively and 80 +/- 10% of normal following operation; p = not significant).

Adolescent↗

Incomplete Kawasaki disease with coronary artery involvement.

We report four patients with Kawasaki disease in whom characteristic coronary artery abnormalities developed after illnesses that did not meet diagnostic criteria. An additional patient lacked a history of acute manifestations of Kawasaki disease, but severe Kawasaki-like arterial changes were noted at autopsy. Fever was present in four of the five patients, in three lasting from 7 to 14 days. Despite manifestation of few classic acute clinical features of Kawasaki disease, three of four patients had desquamation of the fingers and toes 10 to 14 days after onset of illness, and the fifth had desquamation several months prior to death. These patients were seen over a 2-year period during which 22 other children were seen with Kawasaki disease with coronary artery abnormalities. Thus, strict adherence to currently accepted criteria for diagnosis of Kawasaki disease may lead to failure to recognize incomplete forms of this illness, with potential sequelae of myocardial infarction or sudden death. This finding suggests that children with prolonged unexplained febrile illnesses, especially those associated with subsequent peripheral desquamation, should undergo echocardiography 3 to 4 weeks after onset of the illness. This practice would help to identify those patients with illnesses characterized by incomplete diagnostic criteria but in whom significant coronary abnormalities develop.

Coronary Aneurysm↗

Preparation of the left ventricle for anatomical correction in patients with simple transposition of the great arteries. Surgical guidelines.

Pulmonary artery banding in combination with an aortopulmonary shunt was performed on 16 patients with simple transposition of the great arteries to prepare the left ventricle for anatomical correction. Three groups were identified after operation: Group I (four patients) had increased pulmonary blood flow and tight pulmonary artery banding; Group II (four patients) had increased pulmonary blood flow and moderate pulmonary artery banding; Group III (eight patients) had normal pulmonary blood flow and moderate pulmonary artery banding. Postoperative low cardiac output was present in all patients in Group I, whereas mild heart failure was present in two patients in Group II and in two in Group III. There was one hospital death (6%). The follow-up period was 125 patient-months. Left ventricular systolic pressure rose from 63 +/- 11 torr before the operation to 101 +/- 35 torr after the procedure in Group I (p less than 0.05), from 59 +/- 10 to 93 +/- 33 torr in Group II (p less than 0.05), and from 55 +/- 10 to 84 +/- 16 torr in Group III (p less than 0.005). The increase in left ventricular muscle mass was from 44 +/- 2 gm/m2 preoperatively to 108 +/- 12 gm/m2 after operation in Group I (p less than 0.01), from 43 +/- 3 to 93 +/- 8 gm/m2 in Group II (p less than 0.02), and from 46 +/- 3 to 55 +/- 14 gm/m2 in Group III (p = no statistically significant difference). The postoperative change in left ventricular end-diastolic volume was from 100% +/- 17% to 133% +/- 23% of normal in Groups I and II (p less than 0.05) and from 123% +/- 29% to 107% +/- 36% of normal in Group III (p = no statistically significant difference). In preparing the left ventricle for anatomical correction, avoidance of severe pulmonary artery banding decreases the incidence of postoperative myocardial dysfunction, a moderate degree of volume overload and pulmonary artery banding provides the most effective stimulus for ventricular growth, and a small to moderate atrial septal defect is advantageous because it ensures the volume preload necessary for the development of the left ventricle.

Arteriovenous Shunt, Surgical↗

Experience with St. Jude Medical valve prosthesis in children. A word of caution regarding right-sided placement.

Thirty-six children aged 6 months to 18 years, underwent insertion of 37 St. Jude Medical cardiac prostheses. In 20, the valve was placed in the aortic or mitral position, and in 16 in the pulmonary or tricuspid position. There was one (2.8%) hospital death. All patients received maintenance doses of salicylates and dipyridamole after the operation. Follow-up data are available for all patients for 12 to 24 postoperative months. There was no incidence of valve dysfunction or thromboembolic complication in any of the 20 patients with valves in the systemic (left) side of the circulation, and all manifested improvement in their functional class. In contrast, six (37%) of the 16 patients with valves in the pulmonary (right) side of the circulation developed dysfunction of the prosthesis 1 to 6 months after insertion. Prosthesis failure was associated with fibrous tissue growing into the struts, leading to leaflet immobilization. At 2 years, the actuarial functional life was 100% for mitral and aortic valves and 70% for pulmonary and tricuspid valves. The data illustrate the excellent hemodynamic function of the St. Jude Medical valve in children. The absence of thromboembolic complications warrant continued implantation of the prosthesis in the left side without warfarin anticoagulation therapy, but the high incidence of valve dysfunction in the pulmonary position does not justify its continued use in the right side.

Adolescent↗

The treatment of Kawasaki syndrome with intravenous gamma globulin.

We compared the efficacy of intravenous gamma globulin plus aspirin with that of aspirin alone in reducing the frequency of coronary-artery abnormalities in children with acute Kawasaki syndrome in a multicenter, randomized trial. Children randomly assigned to the gamma globulin group received intravenous gamma globulin, 400 mg per kilogram of body weight per day, for four consecutive days; both treatment groups received aspirin, 100 mg per kilogram per day, through the 14th day of illness, then 3 to 5 mg per kilogram per day. Two-dimensional echocardiograms were interpreted blindly and independently by two or more readers. Two weeks after enrollment, coronary-artery abnormalities were present in 18 of 78 children (23 percent) in the aspirin group, as compared with 6 of 75 (8 percent) in the gamma globulin group (P = 0.01). Seven weeks after enrollment, abnormalities were present in 14 of 79 children (18 percent) in the aspirin group and in 3 of 79 (4 percent) in the gamma globulin group (P = 0.005). No child had serious adverse effects from receiving gamma globulin. We conclude that high-dose intravenous gamma globulin is safe and effective in reducing the prevalence of coronary-artery abnormalities when administered early in the course of Kawasaki syndrome.

Aneurysm↗

Mucocutaneous lymph node syndrome (Kawasaki disease): delayed aortic and mitral insufficiency secondary to active valvulitis.

A 2 8/12 year old girl with acute mucocutaneous lymph node syndrome (Kawasaki disease), presented 17 months later with a new onset of mitral and aortic insufficiency. Congestive heart failure rapidly developed and double valve replacement was performed. Pathologic study of the excised valve tissue disclosed active valvulitis. This is the first report of late onset aortic and mitral valvulitis associated with Kawasaki disease.

Aortic Valve↗

The Damus-Stansel-Kaye procedure. Should the aortic valve or subaortic valve region be closed?

Two patients (one with transposition of the great arteries and another with Taussig-Bing anomaly) underwent the Damus-Stansel-Kaye procedure (Group I). Significant aortic valve insufficiency developed postoperatively in both patients. In contrast, seven patients with a univentricular heart and subaortic stenosis from a variety of reasons underwent creation of an aortopulmonary window (Group II), a procedure very similar to the proximal main pulmonary artery-aortic root anastomosis of the Damus-Stansel-Kaye procedure. Aortic valve insufficiency had not developed after up to 7 years of follow-up in this group (average 43 months). Postoperative angiograms suggest that aortic valve incompetence in Group I may have been caused by prolapse of the aortic valve. The valvular structures are subjected to high systolic pressures and face a dilated, low-pressure right ventricle. Aortic root distortion may have contributed, as well. In Group II patients, the aortic valve structures face a small, thick-walled chamber. The orientation of the aortic valve vis-a-vis the right ventricle changed postoperatively in Group I but not in Group II patients. Our experience suggests that the aortic valve or subaortic valve region should be closed at the initial repair in patients with low pulmonary vascular resistance who are undergoing the Damus-Stansel-Kaye procedure, to minimize the need for reoperation for aortic valve insufficiency.

Aorta↗

Effects of elevated coronary sinus pressure on left ventricular function after the Fontan operation. An experimental and clinical correlation.

An experimental model was devised to evaluate the effects of elevated coronary sinus pressure on left ventricular performance. Thirteen mongrel dogs were used. The coronary sinus was cannulated and its entire blood flow diverted into a reservoir. The pressure in the coronary sinus was increased from 5 to 25 torr by elevating the drainage reservoir in a stepwise fashion. Cardiac index, coronary arteriovenous difference, rate of rise of left ventricular pressure, left ventricular systolic time intervals, and coronary blood flow were measured. When the coronary sinus pressure reached 15 torr, there was a significant decrease in cardiac index (3.60 +/- 0.5 to 2.70 +/- 0.6 L/min/m2, p less than 0.001), coronary blood flow (13.7 +/- 3.1 to 7.0 +/- 2.1 ml/min, p less than 0.001), rate of rise of left ventricular pressure (1,567 +/- 275 to 1,331 +/- 314, p less than 0.05), and an increase in coronary arteriovenous difference (62.8% +/- 9.3% to 70.5% +/- 5.4% saturation, p less than 0.03). These experimental results were correlated with postoperative catheterization findings in 24 patients with the Fontan procedure. Patients with a mean right atrial pressure less than 15 torr had a left ventricular ejection fraction of 93% +/- 6% of predicted, whereas patients with a right atrial pressure of 15 torr or more had a left ventricular ejection fraction of 75% +/- 13% of predicted (p less than 0.001). These experimental and clinical data strongly suggest that elevated coronary sinus pressure has deleterious effects on ventricular function after the Fontan procedure. Modifications of the procedure, such as using the rudimentary right ventricle when feasible or diverting coronary sinus flow to the pulmonary venous atrium, might decrease coronary sinus hypertension and improve long-term results.

Animals↗

Hemodynamic effects of intravenous nitroglycerin in pediatric patients after heart surgery.

Twenty patients with congenital heart disease, ages 8 months to 6 years, received 1 to 5 micrograms/kg/min iv nitroglycerin after intracardiac repair to assess the hemodynamic effects of, and the pulmonary vascular response to, the drug in pediatric cardiac patients. Before surgery all patients had systemic-to-pulmonary blood flow ratios equal to or greater than 2.5:1. The pulmonary arterial pressure (PAP) was equal to aortic pressure in 14 patients and normal in six. The pulmonary vascular resistance (PVR) was elevated (more than 4 units) in five patients. After surgery, the right and left atrial pressures, PAP, and cardiac index were measured by thermodilution, and the systemic vascular resistance (SVR) and PVR were calculated. Intravenous nitroglycerin produced a significant drop in SVR at higher doses only (1602 +/- 359 to 1590 +/- 324 dynes X sec X cm-5, p = NS, at 2 micrograms/kg/min vs 1602 +/- 359 to 1247 +/- 417 dynes X sec X cm-5, p less than 0.01, at 5 micrograms/kg/min). Similarly, improvement in cardiac index was apparent only at higher doses (3.33 +/- 1.25 to 3.49 +/- 0.90 liters/min/m2, p = NS, at 2 micrograms/kg/min vs 3.33 +/- 1.25 to 3.82 +/- 0.98 liters/min/m2, p = NS, at 5 micrograms/kg/min). Also, the effect on the pulmonary vascular bed was most marked at higher doses and in patients with elevated PAP (175 +/- 27 to 148 +/- 28 dynes X sec X cm-5, p = NS, in patients with normal PAP vs 305 +/- 78 to 214 +/- 76 dynes X sec X cm-5, p less than .001, in patients with elevated PAP).(ABSTRACT TRUNCATED AT 250 WORDS)

Cardiac Output↗

Successful surgical repair of aortic atresia associated with normal left ventricle.

Surgical repair of aortic atresia with a large ventricular septal defect and a normally developed left ventricle was accomplished in a 14 month old infant. Palliative surgery at age 23 days consisted of bilateral banding of pulmonary artery branches and replacement of the ductus arteriosus with a Goretex conduit. Corrective surgery was accomplished by closure of the ventricular septal defect, insertion of a valved conduit between the apex of the left ventricle and the subdiaphragmatic aorta, removal of the pulmonary artery bands and division of the pulmonary-aortic conduit. The patient has a mild coarctation of the aorta and remains asymptomatic at 2 years of age.

Aortic Valve↗