A population-based prospective evaluation of risk of sudden cardiac death after operation for common congenital heart defects.

OBJECTIVES: This study sought to define 1) the risk of sudden death after operation for common congenital heart defects; and 2) factors associated with an increased risk of sudden death. BACKGROUND: Although the prognosis for patients with congenital heart defects is improved by surgical treatment, they remain at a well recognized but poorly defined risk of late sudden death. METHODS: This population-based study evaluated all patients < 19 years old undergoing surgical treatment of common forms of congenital heart disease in the state of Oregon between 1958 and 1996. Patients were identified retrospectively through 1958, with prospective biannual follow-up beginning in 1982. The incidence and cause of late sudden death were evaluated for 3,589 patients surviving operation for the following defects: atrial, ventricular and atrioventricular septal defects; patent ductus arteriosus; pulmonary stenosis; aortic stenosis; coarctation of the aorta; tetralogy of Fallot; and D-transposition of the great arteries. RESULTS: There were 41 unexpected late sudden deaths during 45,857 patient-years of follow-up, an overall event rate of 1/1,118 patient-years. Thirty-seven of the 41 late sudden deaths occurred in patients with aortic stenosis, coarctation, transposition of the great arteries or tetralogy of Fallot, an event rate of 1/454 patients-years. In contrast, only four sudden deaths occurred among the other defects, an event rate of 1/7,154 patient-years (p < 0.01). The risk of late sudden death increased incrementally 20 years after operation for tetralogy of Fallot, aortic stenosis and coarctation. However, risk was not dependent on patient age at operation or surgical era. The causes of sudden death were arrhythmia in 30 patients, circulatory (embolic or aneurysm rupture) in 7 and acute heart failure in 4. CONCLUSIONS: The risk of late sudden death for patients surviving operation for common congenital heart defects is 25 to 100 times greater than an age-matched control population. This increased risk is primarily represented by patients with cyanotic or left heart obstructive lesions. The risk of sudden death appears to be time dependent, increasing primarily after the second postoperative decade.

Thirty-year incidence of infective endocarditis after surgery for congenital heart defect.

CONTEXT: The incidence of infective endocarditis after surgical repair of congenital heart defects is unknown. OBJECTIVE: To determine the long-term incidence of endocarditis after repair of any of 12 congenital heart defects in childhood. DESIGN: Population-based registry started in 1982. SETTING: State of Oregon. PARTICIPANTS: All Oregon residents who underwent surgical repair for 1 of 12 major congenital defects at the age of 18 years or younger from 1958 to the present. MAIN OUTCOME MEASURE: Diagnosis of infective endocarditis confirmed by hospital or autopsy records. RESULTS: Follow-up data were obtained from 88% of this cohort of 3860 individuals through 1993. At 25 years after surgery, the cumulative incidence of infective endocarditis was 1.3% for tetralogy of Fallot, 2.7% for isolated ventricular septal defect, 3.5% for coarctation of the aorta, 13.3% for valvular aortic stenosis, and 2.8% for primum atrial septal defect. In the cohorts with shorter follow-up, at 20 years after surgery the cumulative incidence was 4.0% for dextrotransposition of the great arteries; at 10 years, the cumulative incidence was 1.1% for complete atrioventricular septal defect, 5.3% for pulmonary atresia with an intact ventricular septum, and 6.4% for pulmonary atresia with ventricular septal defect. No children with secundum atrial septal defect, patent ductus arteriosus, or pulmonic stenosis have had infective endocarditis after surgery. CONCLUSION: The continuing incidence of endocarditis after surgery for congenital heart defect, particularly valvular aortic stenosis, merits education about endocarditis prophylaxis for children and adults with repaired congenital heart defects.

Cardiac surgery for grown-up congenital heart patients: survey of 307 consecutive operations from 1991 to 1994.

The cardiac surgery performed from 1991 to 1994 in a unit dedicated specifically for grown-up congenital heart (GUCH) patients was reviewed to determine the frequency of various procedures, incidence of first and reoperations, early mortality, and its determinants. The 295 patients, aged 16 to 77 years (31 +/- 13), had 307 operations. First operations (n = 128, 42%) were most commonly for closure of atrial septal defect (n = 40), aortic valve replacement (n = 31) or repair of aortic coarctation (n = 14). Reoperations were more frequent (n = 179, 58%) and divided among first corrective repair (n = 49), reoperation after corrective repair (n = 115), and further palliation (n = 15). First corrective surgery was mainly for aortic valve disease (n = 17), Fallot (n = 7), and lesions needing a Fontan procedure (n = 5). Reoperations after corrective repair were needed for aortic valve disease (n = 43), right-sided conduit (n = 30), or recoarctation (n = 11). Early mortality was influenced by presence of central cyanosis (9 of 49, 18% in cyanotic patients; 12 of 258, 5% in acyanotic; p <0.001), increased number of previous operations (0 = 4%, 1 = 7%, 2 = 11%, >2 = 13%; p = 0.003), and increasing age of patients. Cyanotic patients had more serious postoperative complications: pleural and pericardial effusions, severe bleeding, renal insufficiency, and sepsis, and their hospital stay was longer compared with acyanotic patients (20 +/- 17 vs 11 +/- 8 days; p <0.001). In GUCH patients, reoperations cause the largest demand on cardiac surgical services. Increased survival of patients with complex cardiovascular malformations brings difficult challenges not only to cardiologists but also to cardiovascular surgeons. There is a need to provide continued highly specialized care. Resources, patients, and funding should be concentrated in a few designated centers.

Predicting hospital charge and length of stay for congenital heart disease surgery.

Three hundred twenty-two consecutive operations between December 1985 and December 1989 for 10 types of low-risk congenital cardiac malformations were reviewed to determine the hospital charge and postoperative length of stay. Multiple regression analysis of variance was used to predict the influence of the primary diagnosis and various preoperative parameters. The average hospital charge was $27,262 +/- $20,644 and the postoperative length of stay was 9.3 +/- 8.3 days. Age at operation alone did not influence the dependent variables. The diagnosis of atrial septal defect (p = 0.002) or coarctation of the aorta (p = 0.002) decreased the mean charge, whereas the 8 other primary diagnoses did not significantly influence the mean charge. Other preoperative factors found to be predictive of increased hospital charge were: the date of operation (p < 0.001), cyanosis (p = 0.008), previous thoracic surgery (p = 0.02), failure to thrive (p < 0.001), associated major extra cardiac anomalies (p < 0.001), oxygen requirement (p = 0.02), and distance > 100 miles from home to hospital (p = 0.05). A primary diagnosis of atrial septal defect decreased the mean postoperative length of stay by 3.1 days (p < 0.001). Other preoperative conditions increased the mean postoperative length of stay: major extracardiac malformation (p < 0.001), failure to thrive (p < 0.001), and oxygen requirement (p = 0.003). Charge and length of stay equations were generated which may assist in the prediction of resource utilization in this patient population.(ABSTRACT TRUNCATED AT 250 WORDS)

Total anomalous pulmonary venous connection in neonates and young infants: repair in the current era.

Total anomalous pulmonary venous connection has been one of the more challenging congenital heart defects in newborns and young infants despite improvements in surgical technique, cardiac anesthesia, neonatal myocardial preservation, and postoperative care. Since 1981, 30 patients with total anomalous pulmonary venous connection have undergone primary total correction. Mean age at operation was 28 +/- 6 days and mean weight, 3.3 +/- 0.7 kg. Essential features of the surgical approach in these small patients included early surgical intervention, profound hypothermia with total circulatory arrest, cardioplegic myocardial preservation, and a wide posterior anastomosis. Operative mortality was 13% +/- 6%. All four deaths were in patients having emergency operation within 24 hours of the surgical consult and requiring ventilator support preoperatively. The mean follow-up is 47 +/- 7 months. There have been two late deaths, and the 7-year survival rate is 79% +/- 8%. There have been two reoperations, and 91% +/- 6% of the patients are reoperation free at 7 years. Only 1 of the 24 surviving patients is symptomatic. Growth in survivors is closely monitored. The height growth percentile is less than 5% in 15% +/- 8% of survivors and the weight growth percentile, less than 5% in 17 +/- 8%. During the past decade, with a consistent surgical approach to neonates and infants with total anomalous pulmonary venous connection, it has been possible to achieve low early mortality, low attrition, and excellent late results.

25-year mortality after surgical repair of congenital heart defect in childhood. A population-based cohort study.

OBJECTIVE: To determine long-term survival and the cause of death after repair of one of eight congenital heart defects in childhood. DESIGN: Cohort study. SETTING: General community. PARTICIPANTS: All Oregon residents with one of eight congenital heart defects, which was repaired surgically between 1958 and 1989 when the patient was aged 18 years or younger, including (1) tetralogy of Fallot; (2) isolated ventricular septal defect; (3) isolated atrial septal defect; (4) coarctation of the aorta; (5) aortic valvular stenosis; (6) pulmonary valvular stenosis; (7) transposition of the great arteries; and (8) patent ductus arteriosus. Follow-up of this cohort of 2701 individuals was obtained from 94%. MAIN OUTCOME MEASURE: Mortality from cardiac and noncardiac causes. RESULTS: Age at surgery and operative mortality have decreased significantly over the last 30 years. Late cardiac mortality at 25 years after surgery was 5% for tetralogy of Fallot and isolated ventricular septal defect, 10% for coarctation of the aorta, 17% for aortic stenosis, 5% for pulmonic stenosis, and less than 1% for patent ductus arteriosus; there were no late cardiac deaths after atrial septal defect repair. For transposition, late cardiac mortality was 15% at 15 years after the Mustard operation and was 2% at 10 years after the Senning operation. CONCLUSION: Surgical repair of most congenital heart defects is associated with lingering cardiac mortality, particularly for aortic stenosis, coarctation, and transposition.

Community cholesterol screening: medical follow-up in subjects identified with high plasma cholesterol levels.

Population screening or plasma cholesterol is an effective method of detecting hypercholesterolemia; however, follow-up and treatment are essential components of such a program. After a city-wide screening in 1987 of more than 19,872 persons, using a mailed survey with a response rate of 48%, we evaluated subsequent actions of 3,078 individuals with high plasma cholesterol levels. Slightly more than half the population was aware of high blood cholesterol levels prior to the time of screening and apparently used the program for follow-up. Overall, after the screening, 65% consulted a physician within 5 months of screening and blood cholesterol levels were remeasured in 80% of the sample. Procrastination and expense were cited as the primary reasons for failing to consult a physician. If screening is to be effectively utilized as a means of reducing the prevalence of high plasma cholesterol levels, diligent follow-up must be made of all individuals identified to be at increased risk on the basis of their initial values.

Hypoplastic left heart syndrome: natural history in a geographically defined population.

Advances in surgical treatment of hypoplastic left heart syndrome with the Norwood procedure and cardiac transplantation have made essential the understanding of the natural history of hypoplastic left heart syndrome. In a geographically defined population, we ascertained the prevalence of hypoplastic left heart syndrome in children born in Oregon from 1971 through 1986. Clinical and anatomic data were extracted from the charts of the 98 affected children and the survival rate was calculated. Hypoplastic left heart syndrome occurred in 0.162 per 1000 live births in Oregon during this period. No syndrome complex was prevalent and 84% were free of other congenital malformations. However, there was an increased occurrence of congenital heart defects in first-degree relatives of probands with hypoplastic left heart syndrome. Of the affected children 15 +/- 4% died on the first day of life, 70 +/- 5% died within the first week, and 91 +/- 3% died within 30 days. No secular change in survival occurred during the study. Palliation with the Norwood procedure was performed in 20 children. Although survival was significantly improved with this surgery (P = .01), the effect was observed principally through 30 days of life and only one of these children remains alive. Hypoplastic left heart syndrome is a lethal congenital heart defect in children and poses management and ethical dilemmas.

Cosegregation of elastin-associated microfibrillar abnormalities with the Marfan phenotype in families.

The Marfan syndrome is a serious heritable connective-tissue disorder characterized primarily by ocular, cardiovascular, and musculoskeletal abnormalities but also involving multiple other tissues and organs of the body. Inherited as an autosomal dominant disorder, the etiology and pathogenesis of the Marfan syndrome are presently unknown. We have documented consistent apparent deficient content of elastin-associated microfibrillar fibers by indirect immunofluorescent (IF) studies of Marfan skin, as well as deficient accumulation of related fibrous materials in cultures of Marfan fibroblasts as compared with normal controls and patients with other heritable disorders of connective tissue. These data have suggested that abnormalities in the microfibrillar component of elastic-fiber systems may have a role in the etiology and pathogenesis of the Marfan syndrome. In the present study, we have analyzed the IF staining patterns of skin and fibroblast cultures from Marfan syndrome patients and normal first-degree relatives in nine Marfan kindreds. Three of these families had at least one affected individual in each of 2 generations, permitting intergenerational comparison of IF patterns. Six kindreds had one or more affected individuals in a single generation, making comparisons between siblings and/or parent-child possible. In all cases, IF abnormalities cosegregated with the Marfan phenotype and all nonaffected family members were normal. Within family groups containing more than one affected individual, the IF staining patterns were similar between affected patients. These data provide further confirmation of consistent and relatively specific deficiency of microfibrillar fibers in Marfan syndrome.

Late complications after femoral artery catheterization in children less than five years of age.

Fifty-eight children who underwent diagnostic femoral artery catheterization before 5 years of age, from 5 to 14 years before the study, were randomly selected from approximately 300 surviving patients undergoing diagnostic femoral artery catheterization at our institution during the interval. Each patient underwent vascular laboratory segmental pressure and waveform examination and arterial duplex scanning, as well as lower extremity bone length radiographs, which were considered positive if the catheterized leg was greater than or equal to 1.5 cm shorter than the opposite leg. Thirteen children who had only venous catheterization served as controls. No arterial abnormalities were present in the control patients (mean ankle/brachial index, 1.01). Arterial occlusion was present in both limbs of five patients who had bilateral diagnostic femoral artery catheterization and in 14 limbs of 51 patients who had unilateral diagnostic femoral artery catheterization. Thus arterial occlusion was present in 33% of patients (19 of 58) and in 37% of limbs (24 of 65). The mean ankle/brachial index in the catheterized limbs was 0.79. Leg growth retardation was present in four limbs (8%) of 51 children undergoing unilateral diagnostic femoral artery catheterization and in one (8%) control patient. The inverse relationship between ankle/brachial index and leg growth retardation was significant (R = 0.47, p less than 0.0005). Only one patient had symptoms of arterial occlusion (claudication), and one patient had symptoms of leg growth retardation (gait disturbance). We conclude that arterial occlusion is common after diagnostic femoral artery catheterization in children less than 5 years of age, but that excellent collateral supply prevents leg growth retardation and/or symptomatic arterial insufficiency in most children.(ABSTRACT TRUNCATED AT 250 WORDS)

Percutaneous cardiac catheterization and atrial balloon septostomy in pediatrics.

Between November, 1971, and July, 1975, 688 patients ranging in age from six hours to 21 years have undergone percutaneous transfemoral venous and/or arterial catheterization at the University of Oregon Health Sciences Center. This represents 93% of all venous catheterizations of the right side of the heart and 97% of all arterial catheterizations of the left side of the heart in the pediatric age range. One hundred and ninety-five patients (29% of the group) weighed 5 kg or less and 133 (20% of the group) were neonates. There were virtually no complications following the venous studies. The complication rate following percutaneous arterial studies is lower than is the reported experience with cut-down arteriotomies. Long-term survival following percutaneous atrial balloon septostomy in D-transposition of the great arteries was comparable to that following cut-down balloon septostomy. The ease of accomplishment, significantly lower complication rate, and successful accomplishment of atrial balloon septostomy prompt us to advocate that the percutaneous technique of cardiac catheterization be extended to include the neonate and young infant who weighsless than 5 kg.

Late results of surgery for left ventricular outflow tract obstruction in children.

Forty-four patients, with a mean age at surgery of 10 years, were followed for 5 to 16 years (mean 9.7 years) after relief of left ventricular outflow tract obstruction. There were no early deaths, but 5 late deaths occurred, 3 following reoperation. Twenty-five patients were recatheterized from 1 to 16 years later (mean 6.6 years). In 21 of 32 patients (66 per cent), a new diastolic murmur followed relief of valvular stenosis; 25 (78 per cent) of these patients had a postoperative diastolic murmur. Seventeen of these 25 (68 per cent) were recatheterized, and 11 of the 17 (65 per cent) had moderate-to-severe aortic incompetence on angiography. Eight patients (18 per cent) have undergone reoperation and 9 more (20 per cent) will have to be reoperated upon soon. Although the aortic valve gradient and left ventricular stroke pressure were reduced in all obstructive types after surgery, left ventricular end-diastolic pressure significantly increased and cardiac index decreased after valvotomy. Cardiomegaly and electrocardiographic (ECG) abnormalities were present in 45 and 66 per cent, respectively, of all postoperative patients. Although 93 per cent of patients may be expected to survive and 82 per cent be reoperation free at 10 years, further surgery thereafter becomes increasingly common. Timely relief of obstruction prevents sudden death and produces excellent symptomatic improvement, but the operation is only palliative. Development of a reliable pediatric valve and ventriculo-aortic conduit may encourage earlier and more aggressive therapy.

Aortic root dilatation and mitral valve prolapse in Marfan's syndrome: an ECHOCARDIOgraphic study.

Echocardiographic and phonocardiographic findings in 35 patients with Marfan's Syndrome and ten patients without Marfan's or other clinically apparent connective tissue disorders but with angiographic and echocardiographic evidence of mitral prolapse are reported and compared. Echocardiography revealed aortic root dilatation and/or mitral valve prolapse in 97% of the patients with Marfan's Syndrome. Aortic root dilatation was found in 60% of this group (74% of males, 33% of females) while mitral valve prolapse was found in 91% (87% of males, 100% of females). The incidence of aortic dilatation and mitral prolapse in patients with Marfan's syndrome was essentially equal in children and adults of the same sex. None of the nine adults or one child with mitral prolapse but without evidence of Marfan's Syndrome or other clinically apparent connective tissue disorder had aortic root enlargement. Ausculatory examination and phnocardiography revealed abnormalities in 54% of the patients with Marfan's Syndrome. Aortic regurgitation was found in 23% of this group (35% of males, 0% of females) while mitral regurgitation and/or mitral clicks were found in 46% (39% of males, 58% of females). Aortic regurgitation was much more frequent in adult males with Marfan's Syndrome (7/14, 50%) than male children (1/9, 11%), while the incidence of abnormal mitral sounds was essentially the same in adults (33% of males, 60% of females) and children (43% of males, 57% of females) of the same sex with Marfan's Syndrome. Abnormal mitral sounds were more frequent in patients without Marfan's who had mitral prolapse (90%) than in those with Marfan's (46%). It appears that cardiac abnormalities are a consistent manifestation of Marfan's Syndrome and that ultrasound is a more sensitive indicator of these abnormalities in such patients than ausculation or phonocardiography.

Postoperative hemodynamic and electrophysiologic evaluation of the interatrial baffle procedure.

Hemodynamic and electrophysiologic studies were performed in 11 children with dextrotransposition of the great arteries an average of 26 months after the interatrial baffle procedure and, in 2 patients, additional closure of a ventricular septal defect. All children are clinically well. Right to left shunts ranging from 28 to 63 percent of systemic blood flow were found at the superior vena caval-baffle junction in four children. The superior vena caval-baffle gradient averaged 7 mm Hg (range 0 to 22). Right ventricular stroke work index averaged 39 g-m/beat per m2 and right ventricular end-diastolic pressure 9 mm Hg. These values were not significantly different from the values for the systemic left ventricle in a comparable group of normal children (average left ventricular stroke work index 45 g-m/beat per m2 and average left ventricular end-diastolic pressure 8 mm Hg). Cardiac index, heart rate and arteriovenous oxygen difference were also normal. No child has complete heart block. His bundle recording demonstrated normal H-V intervals (range 27 to 40 msec); 4 of the 11 had a prolonged A-H interval. Left ventricular systolic pressure was less than 40 mm Hg in all but two children who had significant subpulmonary stenosis. Pulmonary vascular resistance averaged 1.9 units and was decreased in all children. We conclude that up to 37 months postoperatively, despite some residual abnormalities, the clinical and hemodynamic condition of these children is excellent.