Is routine preoperative cardiac catheterization necessary before repair of secundum and sinus venosus atrial septal defects?

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)

Task Force 1: The underserved.

The ACC has affirmed its commitment to universal access to health care. Underserved populations exist in urban and rural centers. Common to each is a paucity of personnel trained in cardiovascular care and a lack of access to preventive and highly technologic services. These factors contribute to a poor health outcome (75). Part of the rural problem can be corrected by the transfer of information to local providers by the use of new information systems. Included would be real-time electronic consultation, on-site subspecialty visits and the appropriate use of nonphysician providers (15). The urban problem requires changes in priorities and responsibilities of the academic health centers toward the communities they serve. Curricula changes of cardiovascular specialists, internists, generalists and nonphysician health care personnel must include diversity in training, physician training of ethnically matched providers in addition to technical excellence and research into methods of patient education and motivation for a healthier life-style (51). Reimbursement must appropriately reward those caring for underserved patients and those providing evaluation and management services (43,52).

Valve replacement in children less than 5 years of age.

Between 1966 and 1984, there were 63 children less than 5 years of age who underwent 70 valve replacements (49 mitral [5 repeat], 6 aortic, 11 tricuspid [systemic ventricle; 2 repeat]), 2 tricuspid [pulmonary ventricle] and 1 multiple [mitral-aortic]) at Children's Hospital. Tissue valves were used in 20%. Since 1980, only Björk-Shiley and St. Jude valves have been used. The most common indication for valve replacement was mitral regurgitation after repair of atrioventricular (AV) canal (34%). Mortality dropped considerably over time: 76% before 1979, 33% from 1979 to 1982 and 22% since 1982. More than two-thirds of fatalities were operative deaths, usually within 3 days of surgery. Actuarial survival curves for those operated on since 1980 predict 1 and 5 year survival of 73 and 51%, respectively. For the 46 operative survivors 1 and 5 year valve survival was 97 and 70%, respectively. Postoperative hemodynamics were significantly improved on elective postoperative catheterizations. All but one patient with non-tissue valves received anticoagulant therapy. Postoperative complications included thromboembolism (1.6/100 patients-years) and hemorrhage (0.8/100 patient-years). The frequency of intravascular hemolysis and endocarditis was 1.6%, comparable with adult experience. Complete heart block requiring a pacemaker developed in nine patients (14.1%), in all after AV valve replacement. Valve replacement in young children generally results in considerable hemodynamic improvement. The mortality rate remains above that observed in adults but has declined considerably for those operated on after 1980.

Primary surgical closure of ventricular septal defect in the first year of life: results in 128 infants.

Between January 1973 and July 1981, 128 patients less than 1 year of age with failure to thrive, congestive heart failure or pulmonary artery hypertension underwent primary repair of a ventricular septal defect. The hospital mortality rate was 7.8% (10 of 128), and the late mortality rate was 2.3% (3 of 128). Mortality was highest among younger infants with preexisting respiratory problems or a hemodynamically significant residual lesion postoperatively. Complications included a large residual shunt in eight (6.2%), transient neurologic problems in five (3.9%) and persistent complete heart block in three (2.3%). Lung biopsy specimens obtained from 49 patients showed pulmonary vascular abnormalities in all. Complete right bundle branch block developed in 74 (64%) and bifascicular block appeared in 11 (9%). Recatheterization in 70 patients (55%) showed normal pulmonary artery pressures in all but 2 patients with a large residual shunt. Complete closure of the defect had been achieved in 49 (70%), and a hemodynamically insignificant shunt remained in 19 (27%). Patients without significant hemodynamic residua were asymptomatic and tended to accelerate in growth after surgery.

Reoperation for isthmic coarctation of the aorta: follow-up of 26 patients.

Twenty-six patients underwent reoperation for coarctation of the aorta (C of A) between 1972 and 1980. Most patients (73%) had undergone primary repair in infancy. The interval to reoperation was 5 months to 18 years (mean 8.2 years). Indications for reoperation included symptoms in 4, exercise systemic hypertension in 1, electrocardiographic changes of left ventricular strain in 1, arm hypertension in 21, and a C of A gradient at rest greater than 30 mm Hg in all. Surgical procedures included patch aortoplasty (16 patients), bypass graft (6 patients), left subclavian angioplasty (3 patients), and end-to-end anastomosis (1 patient). There were no operative complications or mortality. There was 1 late death from aortic valve disease. Duration of follow-up in the remaining 25 patients was 2 weeks to 7 years (mean 2.5 years). All patients are asymptomatic. C of A gradients were significantly reduced from 30 to 132 mm Hg (mean 56) preoperatively to 0 to 48 mm Hg (mean 15) postoperatively. Right arm systolic blood pressure also was significantly improved from 120 to 237 mm Hg (mean 153) preoperatively to 100 to 160 mm Hg (mean 124) postoperatively. Systemic systolic hypertension persisted in 5 of 25 (20%). It is concluded that C of A reoperation is a low risk procedure that improves symptoms and reduces C of A gradient and arm blood pressure. However, systolic hypertension does not always resolve postoperatively. Patch aortoplasty appears to be a safe and effective surgical approach.

His bundle electrogram after intracardiac repair of tetralogy of Fallot. Analysis of data in 59 patients.

His bundle electrograms were recorded in 59 patients after intracardiac repair of tetralogy of Fallot and were correlated with the postoperative electrocardiogram. Except for five patients with first degree atrioventricular block postoperatively all patients in Group A (those with either a normal electrocardiogram or solitary right bundle branch block) had a normal A-H interval (77.7 +/- 21.6 msec) (mean +/- standard deviation); all had a normal H-V interval (39.5 +/- 7.2 msec). Patients in Group B (bifascicular block) tended to have a normal A-H interval (97.2 +/- 26.2 msec) with a prolonged H-V interval (48.8 +/- 10.7 msec). Patients in Group C (trifascicular block) had prolongation of both the A-H (160.0 +/- 32.4 msec) and the H-V interval (58.8 +/- 10.6 msec) by comparison with control values. Patients in Group D (transient complete heart block) had a normal A-H interval (79.5 +/- 28.2 msec) but a prolonged H-V interval (57.8 +/- 16.4 msec), similar to that in Group C. A good hemodynamic result was associated with a normal H-V interval; a prolonged interval accompanied a poor result.

Use of prostaglandin E1 in infants with d-transposition of the great arteries and intact ventricular septum.

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.

Nonutility of cineurograms in children with congenital heart disease.

Cineurography is often performed after cineangiocardiography to look for occult congenital urinary tract disease. The accuracy of cineurography was investigated in 171 patients by comparing cineurograms with renal sonograms. One hundred fifteen cineurograms (67%) showed both kidneys well enough to allow assessment of renal structure and function and the results were confirmed in 112 by ultrasonography; 3 cineurograms yielded false-positive results. Limited or no information was obtained from cineurograms of 56 patients (33%) because of nonvisualization or poor visualization of 1 or both kidneys. Of the 11 patients (6%) with urinary tract disease, only 3 were correctly assessed by cineurography. Ultrasonography discovered all 11 renal abnormalities and produced only 1 false-positive result. These data indicate that cineurography is a poor screening test and should be abandoned. When uroradiologic screening is necessary for high-risk patients, sonography is recommended.

Normothermic caval inflow occlusion. Application to operations for congenital heart disease.

The technique of normothermic caval inflow occlusion was introduced primarily for relief of pulmonary valve stenosis and continues to be used for this purpose with excellent results. However, the technique may also be applied to a number of other lesions such as aortic valve stenosis in neonates and infants, for placement of outflow patches or valve excision for pulmonary atresia with intact ventricular septum, for atrial septectomy in children with restrictive atrial septum, and for other conditions wherein a period of up to 2 minutes of intracardiac exposure is sufficient. This report examines the early and long-term results in 140 children who underwent normothermic caval inflow occlusion at The Children's Hospital, Boston, Massachusetts, over the past 11 years. Ninety-four children underwent pulmonary valvotomy (early mortality 0%), 21 had aortic valvotomy (19%), 10 with pulmonary atresia and intact ventricular septum underwent various procedures (50%), 11 had atrial septectomy (9%), and there were four miscellaneous procedures (50%). Apart from avoiding many of the potential complications of cardiopulmonary bypass, inflow occlusion has provided as good or better short-term and long-term results as those obtained with cardiopulmonary bypass, particularly in infants and neonates, and also has proved more cost effective.

Intermediate results of the arterial switch repair. A 20-institution study.

A total of 466 neonates with transposition of the great arteries, all less than 15 days of age, have been entered into a 20-institution study between Jan. 1, 1985, and June 1, 1987. Seventy-three percent were less than 48 hours old when entered into the study. Two hundred twelve of these underwent an arterial switch repair, and the 1-week, 1-year, and 2 1/2-year survival rates were 82%, 79%, and 78%, respectively. The usual coronary anatomy was present in 67% of the patients. The arterial switch repair was performed in 16 of the institutions. Six among these were shown to be "low-risk" institutions, with the prevalence of the demographic and surgical variables seeming to be the same in these as in the other institutions. The 1-week, 1-year, and 2 1/2-year survival rates after the arterial switch repair in low-risk institutions for simple transposition were 96%, 91%, and 90%, respectively; for transposition with ventricular septal defect they were 84%, 83%, and 83%. Only older age at operation (over 14 days of age), in the case of simple transposition, and transposition with ventricular septal defect were risk factors for death in these six institutions. Among the patients as a whole, freedom from reoperation for pulmonary outflow obstruction at 1 week and 1 year was 99% and 89%, respectively. A previous pulmonary artery banding and, possibly, one institution were identified as risk factors for reoperation. Inferences: In low-risk institutions, the arterial switch repair can be accomplished with good early results, which suggests the possibility that the late results will be better than after the atrial switch repair. Since young age was not a risk factor for the arterial switch repair of transposition and ventricular septal defect, this type of repair for this anomaly, as well as for simple transposition, may be more advantageously performed early in life than at 2 to 3 months of age.

Early and late results with the Mustard operation in infancy.

Eighty-one patients, ranging in age from 36 hours to 24 months and in weight from 2.5 to 12 kg had a Mustard operation for D-transposition of the great arteries (D-TGA) (20 with complex D-TGA) using either deep hypothermic circulatory arrest (68 patients) or conventional cardiopulmonary bypass (13 patients). A Dacron patch was used for the intraatrial baffle and pericardium for augmentation of the pulmonary atrium. Ten patients died following operation. Thirty-two patients had cardiac catheterization 1 year after operation. Of 24 patients with D-TGA and intact ventricular septum, 23 had normal pulmonary artery pressures. In 20 patients left ventricular outflow tract gradients decreased from a mean of 32 mm Hg to a mean of 18 mm Hg after operation. Five patients who had D-TGA and ventricular septal defect and systemic pressures in the left ventricle before operation, had a notable decrease in left ventricular pressures after the procedure. Seven patients required reoperation for baffle obstruction. Mortality following Mustard repair was primarily related to the complexity of the lesion, maturity of the infant, and degree of pulmonary vascular changes. Caval obstruction was related to the configuration of the baffle used in the early part of this series.

Early and late results of closure of ventricular septal defect in infancy.

Fifty infants ranging in age from 13 days to 18 months (mean age 6 months) and weighing from 1.7 to 8.2 kg (mean weight 4.5 kg) underwent patch closure of a ventricular septal defect (VSD) with use of deep hypothermic circulatory arrest. Seventeen infants were under 3 months of age. The principal indication for operation was intractable chronic congestive heart failure; All infants were below the third percentile for weight preoperatively; Three patients (6%) died postoperatively within the second month of life. There was no late mortality. Seven infants (14%) had seizures; these were associated with a low output state in 2 infants, with hypoxic episodes in 4 infants, and occurred postoperatively in 1 infant. Postoperatively, 8 (17%) of the surviving infants developed right bundle-branch block and left anterior hemiblock, and 16 (32%) developed right bundle-branch block alone. One year postoperatively, catheterization studies in 24 children revealed normal pulmonary artery pressure and pulmonary vascular resistance in all; there were no significant residual ventricular septal defects. Because of these results we continue to be enthusiastic about primary closure of VSD irrespective of age or weightk0

Experience with valved conduits for repair of congenital cardiac lesions.

Dacron valved conduits or aortic allografts were placed between the right heart and the pulmonary artery for repair of various complex congenital cardiac anomalies in 56 patients (aged 15 days to 33 years; median, 11 years). Forty-four patients had a total of 56 previous palliative procedures, which contributed to postoperative morbidity and mortality. Six patients had a total of seven episodes of early or late sepsis involving the conduit. One patient, treated for early sepsis, again developed infection in the Hancock graft 1 year postoperatively and died. Three other patients, 2 with calcified allografts, developed infections 4 months to 7 years following repair and required graft replacement. Hemodynamic data 1 month to 5 years (mean, 1.6 years) following repair revealed mild to moderate obstruction (less than 45 mm Hg gradient) at the Hancock conduit valve ring in 13 of 19 patients, while 5 had large pressure gradients (greater than 75 mm Hg). All aortic allografts had severe obstruction and calcification necessitating graft replacement. It is anticipated that improved technique and appropriate timing of palliative and corrective operations will substantially reduce or eliminate these problems.

Transposition of the great arteries and ventricular septal defect: results with the Senning operation and closure of the ventricular septal defect in infants.

From May, 1978, to July, 1982, 46 infants ranging in age from 12 days to 12 months and in weight from 2.1 to 8.4 kg underwent repair of dextrotransposition of the great arteries (D-TGA) and ventricular septal defect (VSD) using a Senning repair and closure of the VSD. Ventricular septal defects were classified as membranous (47.8%), malaligned (28.3%), atrioventricular (AV) canal type (13.0%), subarterial (2.2%), muscular (2.2%), and multiple (6.5%). Hospital mortality was 15.2% and late mortality, 5.1%. Postoperative complications included tricuspid regurgitation (mild in 3 and severe, requiring tricuspid valve replacement, in 3), residual VSD (pulmonary/systemic flow ratio of greater than 2:1) in 3 patients (2, AV canal type and 1, multiple VSDs), pulmonary venous obstruction in 3 patients, and permanent complete heart block in 4 patients (2, AV canal type of VSD also requiring tricuspid valve replacement). Lung biopsy studies showed reversible Heath-Edwards and morphometric changes. No patient was seen with Heath-Edwards III or greater changes. In 10 patients, right ventricular end-diastolic pressures and pulmonary artery pressures at rest were within normal limits one year after operation. As the operative mortality of atrial inversion and arterial switch operations for D-TGA with VSD tends to become comparable, more extensive follow-up data, including cardiac catheterization and coronary arteriography in a large number of patients, will be necessary to establish the superiority of one approach over the other.

Infective endocarditis in the adult with congenital heart disease.

Advances in the diagnosis and therapy of congenital heart disease in the past few decades have allowed the survival of young adults who are a fertile substrate for developing bacterial endocarditis. In addition to valvular involvement, young adults with congenital disease may develop infection on the ventricular septum, or in a vascular shunt, a patent ductus arteriosus, or coarctation of the aorta. The incidence of endocarditis in children and adults with congenital heart disease is higher than with rheumatic heart disease or mitral valve prolapse. Infecting organisms, diagnosis, and therapy are similar to acquired heart disease, but the physiologic variations and patient population often add a unique flavor to the management of this group.

Recreational and sports recommendations for the child with heart disease.

Although there is widespread acceptance that some children with congenital or acquired heart disease need exercise proscription, there is little data on which to base recommendations. In this article, the author, after reviewing the hemodynamic burdens of the common cardiac anomalies in children, gives his personal recommendations for recreational and sports activities.

Clinical practice guidelines for quality patient outcomes.

In response to environmental changes brought about by the debate over health-care reform, hospitals are seeking means to decrease costs while improving quality. By examining the process of health-care delivery, problems in the system can be highlighted for further investigation. Development of clinical practice guidelines by an interdisciplinary team can be an effective approach to decrease undesirable practice variation, standardize appropriate resource use, and measure the effectiveness of care through defined expected-patient outcomes. By ensuring the appropriate, efficient, and effective delivery of health care, savings in cost can be realized by the elimination of duplication, rework, and waste.