Balloon angioplasty of right ventricular outflow tract conduits.

Palliation of complex congenital heart disease, requiring reconstruction of the right ventricular outflow tract (RVOT), is standard practice. Survival of the homograft is a limiting factor. We examined the role of balloon angioplasty (BAP) in prolonging conduit life. Twelve patients underwent 15 BAP procedures between February 1989 and October 1997. The median age at conduit insertion was 28 months with detection of a significant echo gradient 42 months later. Calcification of homografts, with attendant obstruction and valve dysfunction, was present in all patients. BAP was performed within 1 month of echocardiography and reduced the gradient from a median of 57 to 38 mmHg (p < 0.0005). Echocardiographic follow-up showed persistent gradients (median 68 mmHg) and 11/12 patients went on to conduit replacement after BAP. Only one patient had replacement deferred as a result of BAP. Complications requiring intervention occurred in 20% of the procedures and included bleeding and an unusual balloon fracture. Although BAP can reduce the pressure gradient across the RVOT conduit, the effect is transient and the delay of surgery is not due to improved hemodynamic function. Approximately 10% of cases will benefit from BAP alone, but given the high rate of complications, we do not recommend this procedure as a means of prolonging conduit life.

Postpericardiotomy syndrome: no evidence for a viral etiology.

BACKGROUND: Postpericardiotomy syndrome has been considered a disorder induced by viral infection. This conclusion is based on serologic criterions, but these may be unreliable following either cardiopulmonary bypass or transfusion therapy. Previous studies have not verified the proposed etiology either by isolation of viruses, or by detection of their genome. We sought, therefore, to clarify the role, if any, of viruses in this syndrome. METHODS AND RESULTS: We studied prospectively 149 children aged from 6 months to 16 years who were undergoing open heart surgery. Blood samples were collected from all prior to operation, and again 7 to 10 days post-operatively, and 47 were sampled at the time of development of symptoms of pericardial involvement. Serums were analyzed for the presence of IgM and IgG antibodies to cytomegalovirus, herpes simplex virus, and Epstein-Barr virus. The polymerase chain reaction was used for amplification when assessing the genome of the enteroviruses. Cultures for viruses were established on samples of stool, urine, and throat swabs collected 7 days post-operatively, and at the time of postpericardial symptoms. Pericardial fluid obtained from 5 patients with the syndrome was cultured for viruses, and tested for enterovirus genome. On the basis of clinical and echocardiographic findings, 34 children were determined to have definite evidence of the syndrome, 13 were considered to have possible evidence, and the results from these patients were compared to those from patients with no pericardial symptoms, the latter being matched for age and transfusion status. We isolated viruses from one or more sites in five patients with definite evidence (16%), from one (9%) of those with possible evidence, and from seven (19%) of the controls. All serums and pericardial samples were negative for enterovirus genome. IgM antibodies were found in only 5 patients, three with symptoms of pericardial involvement and two without. Rates of seroconversion to IgG for the viruses were lower in the patients with symptoms of pericardial involvement compared to controls, but were strongly influenced by transfusion status. CONCLUSION: Our study has provided no evidence to support a viral etiology for the postpericardiotomy syndrome.

Kawasaki disease in the older child.

OBJECTIVES: To determine the prevalence of Kawasaki disease in older children and to evaluate its clinical presentation, time to diagnosis, and outcome in comparison with younger patients with the disease. METHODOLOGY: A retrospective analysis of all patients discharged with a diagnosis of Kawasaki disease at a pediatric tertiary care hospital over a 12-year period. RESULTS: A total of 133 patients were included in this study; 7.5% were 9 years of age or older at the time of illness. Patients were grouped by age: infants included children age 1 to 8 years of age and children 9 years of age or older. Older children had a higher frequency of abnormal cardiovascular physical examination (50%) versus children (6%) and infants (10%). The older age group and the infants had a higher prevalence of coronary artery abnormalities and poor left ventricular function than did the 1- to 8-year-olds. Eighty percent of the older children had coronary arteries that were either dilated or aneurysmal, and 30% demonstrated left ventricular dysfunction on initial echocardiography. The number of days to diagnosis after meeting the diagnostic criteria was 5.8 +/- 2.3 for infants, 5.2 +/- 1.5 for older children, and 1.9 +/- 0.3 for children. Older children had a complicated course of Kawasaki disease compared with younger patients. CONCLUSION: We found a higher prevalence of older children with Kawasaki disease at our center than has previously been reported. Older patients, as well as infants, had a higher rate of coronary artery abnormalities than did the children between 1 and 8 years of age. Older age at the time of illness or a delay in treatment may be important factors in determining cardiac involvement in Kawasaki disease.

Role of transesophageal echocardiography in the management of aortic-left ventricular tunnel.

A 10-year-old patient has been followed for 10 years after repair of an aortic-left ventricular tunnel (ALVT) for residual aortic regurgitation and significant recurrent leak through the ALVT. Transesophageal echocardiography (TEE) was done prior to surgery and intraoperatively to (1) clarify the precise anatomy of the ALVT, (2) evaluate the degree of aortic regurgitation, and (3) assess the repair. Delineation of the anatomy of the ALVT and assessment of the residual aortic insufficiency helped with the intraoperative decision-making. We describe the TEE findings of a residual ALVT in this patient and discuss the role of TEE in managing the lesion.

Double-blind placebo-controlled trial of corticosteroids in children with postpericardiotomy syndrome.

The objective of this study was to assess the efficacy of corticosteroids in hastening the recovery of children with postpericardiotomy syndrome, using a randomized double-blind placebo-controlled trial in a tertiary care referral center for pediatric cardiology and cardiac surgery. Twenty-one children, 6 months of age or older (mean age 3.9 years) with postpericardiotomy syndrome following open or closed heart surgery were administered either prednisone 2 mg/kg/day reducing to zero over 14 days (n = 12) or placebo (n = 9). Progress was monitored by daily clinical assessment and alternate day cross-sectional echocardiograms. The primary measures of efficacy were the number of patients in remission at 72 h and at 1 week. No difference in remission rates were found at 72 h, but at 1 week significantly more children treated with prednisone were in remission (placebo 3/9; prednisone 10/12, p = 0.03). A trend to faster resolution of all symptoms and signs was seen in the prednisone-treated group but this was not associated with earlier hospital discharge. Enlargement of pericardial effusion was seen in two children treated with steroids. No complications of treatment were encountered. Prednisone hastens the recovery of children with postopericardiotomy syndrome. Pericardial effusions may increase in size despite the use of corticosteroids.

Systolic and diastolic function in tricuspid valve atresia before the Fontan operation.

Systolic and diastolic function were assessed at preoperative cardiac catheterization in 6 patients with classic tricuspid atresia and were compared with those in control subjects. All patients had systemic-to-pulmonary shunts. Left ventricular end-systolic and end-diastolic volumes and masses were significantly larger in the patient group; stroke volume and the ratio of volume to mass were also higher, but not significantly. Mean end-diastolic, peak systolic and end-systolic pressures were similar. Mean stress at end-systole and peak systole were significantly higher in the patient group. Operant chamber stiffness tended to be lower than normal, but was not statistically significant, and myocardial stiffness was normal for the patient group also. Indexes of systolic function were compared. The rate-corrected mean velocity of fiber shortening in the patient group was lower and its relation with stress at end-systole was variable with 4 abnormal patients. The ratio of stress at end-systole/end-systolic volume was significantly lower in patients. The duration of hospital stay did not correlate with end-diastolic volume or any parameter. Thus, this study found abnormalities of load-dependent and independent indexes of systolic function with normal diastolic function in young preoperative patients with tricuspid atresia.

Pattern of cardiac malformation in oculoauriculovertebral spectrum.

The reported prevalence of congenital heart defects (CHD) in the oculoauriculovertebral "spectrum" (OAVS) is 5-58%. This variability is mainly due to differences in the diagnostic criteria used, and to ascertainment bias introduced by selection of patients from different specialty databases. Two-thirds of the cardiac anomalies reported are either tetralogy of Fallot (TOF) or ventricular septal defect (VSD). We found a CHD frequency of 19% in a series of 32 patients identified either through a genetic or autopsy database. The cardiac lesions were much more varied and complex than previously reported and included asplenia syndrome, ventricular inversion associated with double outlet right ventricle, pulmonary atresia with VSD, double outlet right ventricle, and infradiaphragmatic total anomalous pulmonary venous connection. Pulmonary and renal abnormalities were more common in OAVS patients with CHD than in those where the heart was normal.

Immunogenicity of Haemophilus influenzae type b conjugate vaccine in children with congenital asplenia.

The immunogenicity of Haemophilus influenzae type b polysaccharide-protein conjugate vaccines in congenitally asplenic children is unknown. The short-term immunogenicity of the H. influenzae type b polysaccharide-diphtheria toxoid conjugate vaccine was therefore assessed in 10 children with congenital asplenia by measuring antipolyribosyl-ribitol phosphate antibody titers. An excellent antibody response was seen in 9 children (mean geometric titer in responders after immunization 44.7 micrograms/mL; range, 2.59-402). The remaining child responded to a booster dose. Further studies are required to assess whether H. influenzae type b conjugate vaccines are immunogenic in infancy in the presence of congenital asplenia.

Pulmonary vascular disease in neonates with transposition of the great arteries and intact ventricular septum.

BACKGROUND: Progressive pulmonary vascular disease in surgically unrepaired transposition of the great arteries with or without ventricular septal defect had been frequently described in the past. Occurrence of progressive pulmonary vascular disease has been reported even after atrial switch procedure done at three months of age. With the advent of neonatal surgical repair, this problem is virtually non-existent. There is a small subgroup of infants with transposition of the great arteries who show pulmonary vascular disease in the neonatal period that can adversely affect the surgical outcome. The clinico-pathological correlation in this group of patients was studied. OBSERVATIONS: Three patients, with transposition of the great arteries and intact ventricular septum, who showed histological evidence of pulmonary vascular disease in the neonatal period or early infancy are described. Two of these patients, continued to have poor systemic oxygenation despite adequate atrial communication. One patient had a close ductus arteriosus within the first two hours of birth while on prostaglandin E1 infusion. CONCLUSIONS: In the absence of left ventricular outflow tract obstruction, a poor response to atrial septostomy suggests pulmonary hypertension and pulmonary vascular disease. Antenatal constriction of the ductus arteriosus may contribute to such changes in pulmonary vasculature.

Autoantibody production after cardiopulmonary bypass with special reference to postpericardiotomy syndrome.

A prospective study of children undergoing open heart surgery with cardiopulmonary bypass showed that many of them produced autoantibodies. No association was found between these antibodies, including anticardiolipin antibodies, and the occurrence of postpericardiotomy syndrome.

Doppler echocardiographic observations of diastolic trans-septal flow through small ventricular septal defects.

Turbulent left-to-right trans-septal diastolic blood flow with a peak velocity greater than 0.5 m.s-1 was detected by pulsed Doppler echocardiography in 10 out of 204 (4.9%) children with isolated small ventricular septal defects. For children older than 10 years, it was observed in over a quarter of cases (27%). Peak diastolic velocities ranged from 0.6-1.9 m.s-1 (mean 1.2 m.s-1) and in all patients the duration of diastolic flow exceeded that of the systolic trans-septal jet. Three different patterns of diastolic flow were observed and in eight cases pre-systolic acceleration was present. Turbulent diastolic flow across small ventricular septal defects is a common finding in older children and should not be confused with other causes of disturbed diastolic flow within the right ventricle.

Pulsed wave and color Doppler findings in congenital pulmonary vein stenosis.

A premature infant presented at 8 weeks of age with respiratory failure and pulmonary hypertension. Two-dimensional echocardiography was not diagnostic but color flow imaging and pulsed Doppler examination revealed turbulent and continuous pulmonary venous flow. Differing patterns of disturbed flow suggested varying severity of obstruction in individual pulmonary veins. The Doppler findings are compared to the angiographic and autopsy data.

Cardiac anomalies complicating congenital contractural arachnodactyly.

A newborn boy with congenital contractural arachnodactyly (CCA) was found to have an atrial septal defect, ventricular septal defect, patent ductus arteriosus, and aortic arch anomalies. These resulted in congestive failure but subsequent progressive dilatation of both great arteries and development of aortic regurgitation were associated with eventual cardiorespiratory failure and death at 11.5 months. Others have noted that cardiac anomalies in CCA may be comparable with those of infantile Marfan's syndrome. Our case further extends the spectrum of cardiac abnormalities in CCA and substantiates that there is overlap with infantile Marfan's syndrome. This fact and our literature review suggest that early and complete cardiac assessment is necessary for appropriate management and evaluation of prognosis in any newborn infant with findings suggestive of either condition.

Balloon dilatation of restrictive interatrial communications in congenital heart disease.

An adequate interatrial communication is necessary for survival in several forms of congenital heart disease. Three children are presented in whom blade atrial septostomy proved either technically impossible or failed to achieve adequate interatrial communication. In all three an adequate communication was obtained by a balloon dilatation technique similar to that used for pulmonary valve dilatation. In one critically ill infant with mitral atresia the procedure was life saving.

Assessment of bedside umbilical vein balloon septostomy using two-dimensional echocardiographic guidance in transposition of great arteries.

The effectiveness of "bedside" balloon atrial septostomy via the umbilical vein using 2-dimensional echocardiography was compared to the traditional femoral vein approach using fluoroscopy in a series of neonates with transposition of great arteries from March, 1984 to April, 1987. There were 7 neonates who had balloon septostomy performed at the "bedside" (Group I) compared to 13 who had the procedure performed in the catheterization laboratory (Group II). Group II consisted of 7 newborns who had elective femoral vein catheterization under fluoroscopy (Group IIA) and 6 who failed "bedside" umbilical vein balloon septostomy and subsequently had the femoral vein approach under fluoroscopy (Group IIB). Results showed that adequacy of balloon septostomy was not related to the approach used, with 4 of 7 in Group I and 9 of 13 in Group II with an adequate atrial tear and clinical response. The Delay time to septostomy (i.e. time elapsed from initial assessment to commencement of balloon septostomy) and Procedure time (i.e. time taken to complete the balloon septostomy) was significantly shorter for Group I (mean time = 0.7 hours and 0.26 hours respectively) compared with Group IIA (mean time = 2.6 hours and 1.8 hours) and Group IIB (mean time = 2.4 hours and 1.4 hours). Of note, there was no significant increase in Delay time between Group IIA and IIB.(ABSTRACT TRUNCATED AT 250 WORDS)

Influence of echocardiography in pre-operative cardiac catheterization in congenital heart disease.

The influence of echocardiography on pre-operative cardiac catheterization was assessed in a retrospective analysis comparing two 12-month periods. Of 245 operations in 1983, 200 (82%) had pre-operative cardiac catheterization when two-dimensional echocardiography only was used in the pre-operative assessment compared to 162 of 238 (68%) operations in July 1985-June 1986 when pulsed Doppler echocardiography also was used (P less than 0.001). Significant reductions in pre-operative cardiac catheterization were present in both open (87/87 vs 89/102, P less than 0.001) and closed (113/158 vs 73/136, P less than 0.005) heart procedures. The group with the most significant reduction in pre-operative cardiac catheterization before open heart surgery were children with atrial septal defects; of the closed heart surgical groups, the greatest reductions were in patent ductus arteriosus, coarctation of the aorta and tetralogy of Fallot. Echocardiogram diagnostic errors occurred in 22/245 (9%) in 1983 compared to 9/238 (4%) in 1985/86 (P less than 0.05). Echocardiography has resulted in a significant reduction in pre-operative cardiac catheterization and has become more accurate in diagnosis.

Left ventricular systolic and diastolic function after two-stage anatomic correction of transposition of the great arteries.

Anatomic repair of transposition of the great arteries (TGA) has been developed because of concerns about right ventricular function after atrial repair by the Mustard or the Senning technique. This study assessed left ventricular systolic and diastolic function in three patients after two-stage anatomic repair. Two patients had a ventricular septal defect (one with coarctation), and the third patient had right ventricular dysfunction precluding atrial repair. All had pulmonary artery banding. The mean ages at the time of repair and catheterization were 2.75 and 4.9 years, respectively. The control group included 10 patients with insignificant or no cardiac disease. At cardiac catheterization the group with TGA had a higher mean end-diastolic volume index (110.9 +/- 4.74 ml/m2) compared to normal subjects (79.1 +/- 14.55; p less than 0.001), mean end-systolic volume index (37.3 +/- 3.69 vs 22.7 +/- 4.42; p less than 0.001), mass index (101.0 +/- 16.9 vs 68.2 +/- 12.34; p = 0.038), and stroke volume index (73.6 +/- 3.52 vs 56.5 +/- 12.1; p = 0.0027). The ejection fractions, end-diastolic and peak systolic pressures, and stresses were not different. There was no difference in the relationship between the mean rate-corrected velocity of circumferential fiber shortening and end-systolic stress for the group with TGA, but myocardial stiffness was markedly elevated (29.5 +/- 1.84 vs 10.8 +/- 2.20; p less than 0.001). Thus, this study found abnormalities of left ventricular size after two-stage anatomic repair of TGA in this group of patients with TGA.(ABSTRACT TRUNCATED AT 250 WORDS)

Total anomalous pulmonary venous return with left heart hypoplasia.

A term baby had a total anomalous pulmonary venous return to the inferior vena cava. The infant underwent complete repair, but died 30 hours after operation. The postmortem examination revealed severe endocardial fibroelastosis. This uncommon association is discussed.

Aortic valve insufficiency and coronary artery narrowing in a child with polyarticular juvenile rheumatoid arthritis.

Involvement of the cardiac valves in juvenile rheumatoid arthritis (JRA) is a rare but life threatening complication. We report a 9 1/2-year-old girl with rheumatoid factor positive polyarticular JRA who developed aortic insufficiency that required valve replacement. Six months later she developed angina due to narrowing of the left coronary artery, probably secondary to aortitis.

Two-dimensional echocardiographic diagnosis of arrhythmogenic right ventricular dysplasia presenting as frequent ventricular extrasystoles in a child.

Two-dimensional echocardiographic investigation of a child with frequent isolated ventricular extrasystoles revealed an area of right ventricular dysplasia. This finding may signify a less favorable prognosis and justifies careful echocardiography in the evaluation of asymptomatic ventricular extrasystoles of left bundle branch block morphology in children.