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ABSTRACT: From 1946 to March 1989, 92 patients (33 women and 59 men) were seen with ventricular septal defect (VSD) and audible aortic regurgitation (AR). The VSD was subcristal in 62 patients, subpulmonary in 21 and unknown in the remaining 9. The median age of onset of AR was 5.3 years. The risk of developing AR was 2.5 times greater in those with a subpulmonary VSD. The aortic valve was tricuspid in 90% and bicuspid in 10%. Prolapse was seen in 90% of those with subcristal VSD and in all with subpulmonary VSD. Pulmonary stenosis was seen in 46% of the patients with gradients ranging from 10 to 55 mm Hg. The incidence of infective endocarditis was 15 episodes/1,000 patient years. Among 20 patients followed medically, for 297 patient years, 1 died (1959) and most have been stable, including 2 followed for greater than 30 years. In the 72 patients operated on, there were 15 perioperative and 5 late deaths. Operations consisted of VSD closure alone in 7, VSD closure and valvuloplasty in 50 and VSD closure and aortic valve replacement in the other 15. Valvuloplasty was more effective in those operated on under age 10 compared to those older than 15 years (46 vs 14%). The durability of the valvuloplasty was 76% at 12 years and 51% at 18 years.
The American Journal of Cardiology 09/1990; 66(3):340-5. · 3.37 Impact Factor
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ABSTRACT: The clinical course of 59 patients who underwent valvotomy for aortic stenosis before 1968 was reviewed. All were older than 1 year at the time of operation. Mean follow-up period was 17.7 years. Forty-six patients are alive; 26 (57%) are 30 to 40 years and 6 (13%) are older. Actuarial analysis indicated that the probability of survival was 94% at 5 years and 77% at 22 years. Thirteen patients died, 7 suddenly. Among the latter, significant obstruction or regurgitation was present in the 4 who underwent catheterization 0.9 to 7.2 years before death, 2 of whom were symptomatic and 2 with progression of a strain pattern on electrocardiogram. Surgery was recommended but declined by the latter 2 patients. Reoperation was carried out in 21 patients (36%), 3 (12%) of whom died. Actuarial analysis revealed the probability of reoperation to increase from 2% at 5 years to 44% at 22 years. Bacterial endocarditis occurred on 4 occasions in 3 patients, 1 of whom died suddenly during treatment. The incidence of endocarditis was 3.8 episodes/1,000 patient-years. Actuarial analysis of serious events, defined as death, reoperation and endocarditis, with the most serious of these and each patient being represented only once, indicated the probability of being free of such an episode to be 92% at 5 years, decreasing to 39% at 22 years. These data emphasize the palliative nature of valvotomy and the meticulous follow-up so necessary in these patients.
The American Journal of Cardiology 09/1986; 58(3):338-41. · 3.37 Impact Factor
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ABSTRACT: Between January 1976 and July 1983, 217 patients with atrial septal defect underwent surgical repair at Children's Hospital. Thirty with a primum atrial septal defect and 26 who underwent cardiac catheterization elsewhere before being seen were excluded from analysis. Of the 161 remaining patients, 52 (31%) underwent preoperative cardiac catheterization, 38 because the physical examination was considered atypical for a secundum atrial septal defect and 14 because of a preexisting routine indication. One hundred nine (69%) underwent surgery without catheterization, with the attending cardiologist relying on clinical examination alone in 5, additional technetium radionuclide angiocardiography in 5, M-mode echocardiography in 13 and two-dimensional echocardiography in 43; both M-mode echocardiography and radionuclide angiography were performed in 24 and two-dimensional echocardiography and radionuclide angiography in 19. Since 1976, there has been a trend toward a reduction in the use of catheterization and use of one rather than two noninvasive or semiinvasive techniques for the detection of atrial defects. Of the 52 patients who underwent catheterization, the correct anatomic diagnosis was made before catheterization in 47 (90%). Two patients with a sinus venosus defect and one each with a sinus venosus defect plus partial anomalous pulmonary venous connection, partial anomalous pulmonary venous connection without an atrial septal defect and a sinoseptal defect were missed. Of 109 patients without catheterization, a correct morphologic diagnosis was made before surgery in 92 (84%). Nine patients with a sinus venosus defect, three with sinus venous defect and partial anomolous pulmonary venous connection, four with partial anomalous pulmonary venous return without an atrial septal defect and one with a secundum defect were incorrectly diagnosed.(ABSTRACT TRUNCATED AT 250 WORDS)
Journal of the American College of Cardiology 09/1984; 4(2):333-6. · 14.16 Impact Factor
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ABSTRACT: Eighty-three patients aged 11 months to 25 years were followed up a median of 6.1 years (range 8 days to 24 years) after diagnosis of fixed subaortic stenosis (SAS). Fourteen (17%) had significant noncardiac defects and 47 (57%) had additional cardiac malformations. The left ventricular (LV) outflow gradient increased in 25 of 26 patients catheterized more than once before surgery. Of 15 patients less than 12 years old with gradients less than or equal to 40 mm Hg, 10 ultimately underwent operation after developing severe obstruction; another has progressed to a gradient of 45 mm Hg at 6 years of age. Before surgery (at a median age of 12 years), 55% had aortic regurgitation (AR), which was usually mild. Infective endocarditis occurred in 12% of the group, with a frequency of 14.3 cases per 1,000 patient-years. Seventy-four patients were operated on, with 6 early (8%) and 7 late (9%) deaths. Twelve underwent reoperation to relieve residual obstruction. Surgery reduced gradients in patients with discrete SAS from 83 +/- 33 to 29 +/- 30 mm Hg, but in 6 patients with tunnel SAS the reduction was less satisfactory. AR was absent or mild in most patients postoperatively. When the gradient was reduced to less than 80 mm Hg, infective endocarditis did not occur unless there were other residual lesions. These data suggest that it is reasonable to resect discrete SAS in children less than 10 to 12 years old with LV outflow gradients greater than or equal to 30 mm Hg.
The American Journal of Cardiology 11/1983; 52(7):830-5. · 3.37 Impact Factor
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Journal of the American College of Cardiology 03/1983; 1(2 Pt 1):476-83. · 14.16 Impact Factor
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Circulation 09/1980; 62(2 Pt 2):I168. · 14.74 Impact Factor
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ABSTRACT: Prostaglandin E1 was used to treat five infants with d-transposition of the great arteries and intact ventricular septum who had persistent severe hypoxemia after the creation of an interatrial communication. Three infants had a dramatic improvement in systemic arterial oxygen saturation associated with dilation of the ductus arteriosus; in two of the three cases urgent surgery was avoided. Two infants had no clinical evidence of increased ductal shunting and no improvement in oxygen saturation. A trial of prostaglandin E1 is recommended for treatment of severe hypoxemia in infants with d-transposition of the great arteries with intact ventricular septum if the presence of a large atrial septal defect is established.
The American Journal of Cardiology 08/1979; 44(1):76-81. · 3.37 Impact Factor
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The American Journal of Cardiology 03/1979; 43(2):253-8. · 3.37 Impact Factor
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American Heart Journal 11/1978; 96(4):556-8. · 4.65 Impact Factor
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ABSTRACT: Left ventricular function was assessed by angiographic methods in 40 patients (ages 3-27), 1-12 years following repair of tetralogy of Fallot. Twenty of the 40 patients (group A) had a satisfactory repair with a pulmonary-systemic flow ratio (Qp/Qs) less then or equal to 1.5 and a right ventricular systolic pressure of less than or equal to 60% of the left ventricular value. The other 20 patients (group B) had a Qp/Qs greater than 1.5 and/or a right ventricular systolic pressure greater than 60% of the left ventricular value. Group A patients had a normal left ventricular end-diastolic volume (LVEDV), end-diastolic pressure (LVEDP), ejection fraction (LVEF), and mass (LVM), while the group B patients had significantly elevated LVEDV, LVEDP, LVM, and a significantly depressed LVEF. These findings indicate that patients with a satisfactory hemodynamic repair of tetralogy of Fallot have normal left ventricular function while patients with postoperative unsatisfactory hemodynamics have significantly impaired left ventricular function.
Circulation 05/1978; 57(4):798-802. · 14.74 Impact Factor
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ABSTRACT: The effect of the Mustard procedure on growth was assesed in 45 patients with simple D-transposition of the great arteries (DTGA) surviving for at least one year after operation. Growth failure (below the third percentile for height, weight, or both) was found in 25 of the 45 patients preoperatively and in 8 patients postoperatively. The principal factors associated with poor growth before repair were advancing age, increased pulmonary and systemic flow, and subpulmonic stenosis. In those patients without postoperative growth failure, growth had returned to the normal range within two years. All patients wit retarded growth after the Mustard procedure had had preoperative growth failure as well. In addition, all 8 patients with postoperative growth failure had one or more amjor residual hemodynamic abnormalities, whereas residual lesions were present in only 10 of 37 patients with normal postoperative growth.
The Annals of Thoracic Surgery 04/1978; 25(3):225-30. · 3.74 Impact Factor
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ABSTRACT: A subnormal birth weight distribution was noted in a population of infants seen during the first year of life for major congenital heart disease (CHD). When arranged according to cardiac diagnostic categories, 17 of the 21 major cardiac lesions were associated with subnormal birth weight distribution. Major extracardiac anomalies present in 19.9% of all infants appeared not to influence the birth weight distributions of either the total population of 19 of 21 cardiac lesion categories. An increased incidence (6.1%) of small-for-dates infants was observed in the study population. Extra-cardiac anomalies occurred in 28% of the small-for-dates infants. Survival to 1 year of age was 42.8% for the total population with CHD and 49.2% in the small-for-dates group.
American journal of diseases of children (1960) 04/1978; 132(3):249-54.
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ABSTRACT: The clinical course of 38 patients with congenital mitral stenosis (MS) is reviewed. Associated cardiac defects were present in 28 patients, including tetralogy of Fallot in five. In all but one of the eight patients with supravalvar mitral ring (SVR), there were concomitant abnormalities of the mitral valve. Delay in the diagnosis of MS was common. Serial cardiac catheterizations and pulmonary pathologic examination indicated that pulmonary vascular obstructive disease develops during childhood. Mitral valve surgery was performed in 19 of 38 patients: valvotomy alone in eight, excision of SVR in five (two ofwhom also had valvotomy) and mitral valve replacemtnt in seven. Additional non-mitral cardiac surgery was performed in 18 patients. Overall surgical mortality was 49%; mortality for surgery on the mitral valve was 26%. Only patients having mitral valve replacement or with isolated SVR which was then resected became asymptomatic and had normal hemodynamics on postoperative catheterization.
Circulation 01/1978; 56(6):1039-47. · 14.74 Impact Factor
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Pediatrics 12/1977; 60(5):740-2. · 5.44 Impact Factor
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ABSTRACT: Infusion of prostaglandin E1 into the main pulmonary artery of an infant with interruption of the aortic arch and a closing ductus arteriosus resulted in dilation of the ductus arteriosus and improved systemic perfusion. Treatment with prostaglandin E1 is recommended for infants with interruption of the aortic arch, critical coarctation of the aorta, and other lesions in which systemic perfusion is limited by a restrictive ductus arteriosus.
Journal of Pediatrics 12/1977; 91(5):805-7. · 4.11 Impact Factor
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Circulation 09/1977; 56(1 Suppl):I38-47. · 14.74 Impact Factor
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Circulation 09/1977; 56(1 Suppl):I47-56. · 14.74 Impact Factor
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Circulation 09/1977; 56(1 Suppl):I72-7. · 14.74 Impact Factor
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ABSTRACT: The etiology of chronic congestive heart failure (CHF) after repair of tetralogy of Fallot was determined in 102 patients consecutively catheterized 1 to 12 years postoperatively. Chronic CHF was observed in 36/102 patients. The most prevalent abnormality leading to congestive failure (31/36) was a large residual ventricular septal defect alone or in combination with other lesions. All postoperative patients with pulmonary to systemic flow ratios greater than 2:1 (25/102) had congestive failure and evidence of biventricular dysfunction. Significant tricuspid regurgitation (N = 11) and persistent systemic to pulmonary artery shunts (N = 6) contributed to volume overload and congestive failure in the patients with large residual ventricular septal defect. Isolated severe residual right ventricular outflow tract obstruction was a common cause of chronic CHF. Pulmonary artery hypertension was present in 20/36 patients with CHF. The increased pulmonary pressure was not wholly due to an increased pulmonary flow since 7/20 patients had pulmonary vascular resistance greater than 3 mm Hg/L/min/m2. Our findings indicate that persistent or chronic congestive heart failure in postoperative tetralogy of Fallot patients requires bilateral cardiac catherterization since an identifiable and surgically correctable lesion is nearly always present.
Circulation 09/1977; 56(2):305-10. · 14.74 Impact Factor
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ABSTRACT: The course and prognosis of 208 patients with an ascending aorta to pulmonary artery anastomosis is reviewed. Mortality rate during, or within one month, of surgery was 24 per cent (50/208) and late mortality rate, prior to repair, was 10 per cent (21/208). An additional 5 per cent (10/208) died during subsequent intracardiac repair. Congestive heart failure developed in 25 per cent (53/208), pulmonary artery hypertension in 17 per cent (12/72), and pulmonary vascular obstruction in 6 per cent (4/72). An increase in orifice size of the stoma with time was documented in eight patients. Additional subsequent palliative surgery was required in 22 per cent (45/208). Mortality rate was directly related to age at operation and was highest in neonates less than one week of age. In infants with tetralogy of Fallot, a preliminary comparison of mortality rate between palliative surgery and primary repair clearly suggests that the latter is the preferred method of treatment.
American Heart Journal 08/1977; 94(1):14-20. · 4.65 Impact Factor
