Complications of endomyocardial biopsy in children.

OBJECTIVES: To evaluate the incidence of, and risk factors for, complications of endomyocardial biopsy in children. BACKGROUND: Endomyocardial biopsy (EMB) is a low risk procedure in adults, but there is a paucity of data with regard to performing this procedure in children. METHODS: Retrospective review of the morbidity and mortality of 1,000 consecutive EMB procedures. RESULTS: One thousand EMB procedures (right ventricle 986, left ventricle 14) were performed on 194 patients from July 1987 through March 1996. Indications for EMB included heart transplant rejection surveillance (846) and the evaluation of cardiomyopathy or arrhythmia for possible myocarditis (154). Thirty-seven (4%) procedures were performed on patients receiving intravenous inotropic support. There was one biopsy related death, secondary to cardiac perforation, in a two-week-old infant with dilated cardiomyopathy. There were nine perforations of the right ventricle, eight occurring in patients with dilated cardiomyopathy and one in a transplant recipient. The transplant patient did not require immediate intervention; two patients required pericardiocentesis alone, and six underwent pericardiocentesis and surgical intervention. All nine perforations were from the femoral venous approach (p < 0.01). Multivariate analysis demonstrated that the greatest risk of perforation occurred in children being evaluated for possible myocarditis (p = 0.01) and in those requiring inotropic support (p < 0.01). Other complications included arrhythmia (5) and single cases of coronary-cardiac fistula, flail tricuspid leaflet, pneumothorax, hemothorax, endocardial stripping and seizure. CONCLUSIONS: Risk of endomyocardial biopsy is highest in sick children with suspected myocarditis on inotropic support. However, EMB can be performed safely with very low morbidity in pediatric heart transplant recipients.

Cost-effective use of echocardiography in children with Kawasaki disease.

OBJECTIVE: To determine whether a third echocardiogram, performed 6 months to 1 year after the onset of Kawasaki disease (KD), as recommended by current American Heart Association guidelines, identified any case of coronary artery abnormalities when previous echocardiograms were normal. METHODS: Children diagnosed with KD were identified by searching our institution's database. Cases were included in the study if diagnosed between June 1988 and December 1996 and if at least two echocardiograms were documented, including at least one study between 2 weeks and 2 months from the onset of KD and another in follow-up. The patients' charts were reviewed and videotapes of the echocardiograms were reviewed if reports were unclear or contradictory. McNemar's test for discordant pairs was used for statistical analysis. Additionally, a complete review was performed in all other cases of KD in the database in which a coronary artery abnormality had been identified. RESULTS: There were 203 patients diagnosed during the study period who had 2 or more echocardiograms performed, and 67 had the requisite studies in the subacute period and later follow-up. The median age at onset of KD was 3.0 years (range: 0.2-16), the median duration of follow-up was 12.5 months (range: 1.7-100), and the median number of echocardiograms performed was 3 (range: 2-8). Intravenous immunoglobulin was given in 62 cases, and high-dose aspirin was given in 63. There were 35 children with no echocardiographic abnormalities at any point, and 15 other children had early abnormalities (including coronary ectasia, perivascular brightness, pericardial effusion, and ventricular dysfunction) but had a normal echocardiogram between 2 weeks and 2 months. Of these 50 children, none were noted to have abnormalities on later studies. Three children had effusion and/or perivascular brightness after 2 weeks; follow-up studies were normal in each. Six children had coronary ectasia after 2 weeks; it persisted on follow-up in 1 child and had resolved in 5 children. Eight children had coronary aneurysms on studies after 2 weeks; in 3 children, the aneurysm resolved on later follow-up. No coronary abnormalities were demonstrated on a late follow-up echocardiogram in any child with normal coronaries between 2 weeks and 2 months. CONCLUSIONS: All children with KD should have an echocardiogram at the time of diagnosis with a follow-up study 4 to 6 weeks after the onset of fever. In the current environment of cost-containment, additional echocardiographic studies are justified only if abnormalities are present at 4 to 6 weeks. Kawasaki disease, coronary artery aneurysm, echocardiography, coronary ectasia.

Interventricular communication in complete atrioventricular septal defect.

BACKGROUND: Little attention has been paid to whether the interventricular communication in complete atrioventricular septal defect is different beneath the superior and inferior bridging leaflets, a feature of obvious surgical significance. METHODS: We searched for a defect under the bridging leaflets and examined the valve morphologies in 98 autopsied and 86 surgical patients. Of the overall specimens, 27 were associated with Fallot's tetralogy, and a further 20 had subaortic stenosis, aortic coarctation, or both. In the autopsied specimens, we also measured the degree of deficiency of the ventricular septum. RESULTS: No communication was found under the inferior bridging leaflet in 30% (29 of 98) of the specimens. All 29 hearts except two without such communications showed an undivided inferior leaflet. In contrast, all patients undergoing operation except 1 had a communication beneath both bridging leaflets (p < 0.001). The absence of a communication beneath the inferior leaflet was observed more in hearts with Fallot's tetralogy (seven of 14) or those with subaortic stenosis, aortic coarctation, or both (eight of 18) than in those without associated anomalies (14 of 66; p < 0.01). Those with a communication under the inferior leaflet showed a greater deficiency of the inlet ventricular septum than did those without it (p < 0.001). CONCLUSIONS: In a certain percentage of patients with complete atrioventricular septal defect, there will be no communication under the inferior bridging leaflet. Surgeons should be aware of this possibility, particularly when confronted with a patient with obstruction in either ventricular outlet.

Morphometric analysis of atrioventricular septal defect with common valve orifice.

OBJECTIVES: We sought to analyze morphometric features of atrioventricular septal defect (AVSD) in autopsy specimens and to consider the developmental implications of obstruction in either ventricular outflow tract. BACKGROUND: Left ventricular outlet obstruction (LVO) is more prevalent in patients with Rastelli type A morphology. When tetralogy of Fallot (ToF) complicates this malformation, there is usually a free-floating superior bridging leaflet. The reasons for these associations are uncertain. METHODS: In 133 hearts with AVSD and common atrioventricular (AV) valve orifice, we measured the degrees of horizontal and anterior deviation of the great arteries from the AV valve, the diameters of the ventricular outlets and the great arteries and the degree of deficiency of the ventricular septum. RESULTS: In Rastelli type A morphology, the great arteries were deviated more leftward than in type C morphology (p < 0.01). Type A hearts also had a relatively small aorta, with a long and narrow subaortic tract. The presence of obstruction in either ventricular outlet was associated with a more oblique arrangement of the great arteries, with the pulmonary trunk being more leftward than in hearts without LVO (p < 0.01). In combination with ToF, the aorta was dextroposed and the pulmonary trunk was located more posteriorly (p < 0.01). No heart with type A morphology showed ToF (p < 0.01). CONCLUSIONS: The geometric arrangement of the great arteries correlated significantly with obstruction in either ventricular outflow tract and with the Rastelli subtypes. Malrotation of the developing outlet septum may be an embryologic factor producing obstruction, with horizontal deviation of the outlets also influencing the morphology of the superior bridging leaflet.

Intrapericardial left atrial aneurysm: noninvasive diagnosis.

Intrapericardial aneurysm of the left atrium is a rare cardiac anomaly. We present a 10-year-old girl in whom the diagnosis was made following referral for an abnormal configuration of the left heart border seen on chest radiography. Diagnosis was made by echocardiography, but magnetic resonance imaging defined the exact morphology and the relation to adjacent structures. Surgical removal of this aneurysm is recommended because of potentially serious complications.

Infective endocarditis: 35 years of experience at a children's hospital.

We review the predisposing conditions, the presenting signs and symptoms, as well as the risk factors and bacterial etiologies in children with infective endocarditis, focusing on hospital course and outcome. We conducted a retrospective analysis of 76 cases of endocarditis in 73 patients occurring at Children's Hospital of Pittsburgh from January 1958 through December 1992. The median age of the patients was 9 years (range, 1 month to 18 years). Predisposing conditions included congenital heart disease (62 patients) and rheumatic heart disease (four patients). Seventy-seven percent of the children with congenital heart disease had undergone cardiac surgery. After therapy with appropriate antibiotics was started, blood cultures for 67 patients (70 episodes of infective endocarditis) remained positive for a mean (+/-SD) of 0.7 +/- 1.41 days, and all patients who presented with fever (75 episodes in 72 patients) remained febrile for a mean (+/-SD) of 4.28 +/- 6.21 days. Secondary fever occurred in 39% of the children. Thirty (41%) of the 73 patients survived without any complications and 13 (18%) died. Fifteen children with complications required surgery. Children with endocarditis caused by Staphylococcus aureus were more likely than those with infection caused by viridans streptococci to have prolonged fever, secondary fever, and/or complications as well to require surgery.

Surgical anatomy of the coronary circulation in hearts with discordant atrioventricular connections.

We examined the arrangement of the coronary arterial and cardiac venous systems in 46 specimens with discordant atrioventricular connections so as to identify any structural abnormalities and to consider their surgical implications in terms of anatomical biventricular repair. Grossly abnormal arterial courses were seen in 11 hearts (24%). A substantial branch supplying the morphologically right ventricular outflow tract, which could restrict a ventriculotomy, was found in 61% of cases. The coronary sinus received all the morphologically right ventricular veins, as well as the posterior interventricular vein, in 40 hearts, this pattern being in contrast to the pattern in the normal heart. The morphologically left ventricular and anterior interventricular veins, all of which drain via the coronary sinus in the normal heart, were frequently connected independently to the morphologically right atrium in the specimens with discordant connections, the drainage occurring through the spaces between the pectinate muscles. These direct drainages are at risk of potential damage either by extensive intra-atrial maneuvers or by postoperative intraatrial thrombosis. It is predicted, therefore, that surgical results can be improved still further when account is taken of this vascular anatomy of the heart itself.

Pathologic elucidation of the echocardiographic features of Ebstein's malformation of the morphologically tricuspid valve in discordant atrioventricular connections.

We defined the morphology of the left atrioventricular valve in Ebstein's malformation associated with congenitally corrected transposition to elucidate the approach to diagnosis by echocardiography. We found 14 unequivocal cases out of a total of 3,720 specimens. We noted the atrial arrangement, displacement, and nature of the tricuspid leaflets, and axis of the plane of the tricuspid orifice. We constructed ratios of volume of the atrialized to the functional right ventricle, and of the right to left ventricle, as well as distances of the septal attachments of the atrioventricular junctions to the respective ventricular apices. Three specimens had abnormalities of cardiac position, and 8 had ventricular septal defect. The tricuspid valve plane was rotated 47 +/- 21 degrees from its usual position into the ventricle. The tricuspid valvar tissue was variably attached to the underlying myocardium, with the most severely affected lesion being the mural leaflet followed by the septal leaflet, and the anterior leaflet attachment the least affected. Abnormalities of the tendinous cords and the effective valvar orifice occurred in 3 specimens. The ratio of the atrialized to the functional right ventricular volume was 0.74 +/- 0.49; the ratio of the fetal right to left ventricular volume was 1.18:1 +/- 0.70:1. These data suggest that plastic repairs of the right ventricle would leave a small functional right ventricle, but that valve replacement could restore the volume of the ventricle. Thus, the plane of displacement of the valve in corrected transposition appears less amenable to 4-chamber echocardiography than other forms of Ebstein's malformation. Changes in the echocardiographic planes should display the morphology and also provide some hemodynamic information.

Identification of cases of acute rheumatic fever managed on an outpatient basis.

The incidence of acute rheumatic fever has varied over the past several decades. The usual source of case ascertainment has been medical record discharge diagnosis coding. By reviewing both the Children's Hospital of Pittsburgh medical records discharge data and the Division of Cardiology computer database for the years 1982 to 1993, 173 cases of acute rheumatic fever were identified including 63 patients who were managed exclusively as outpatients. Seventy-three percent of patients diagnosed before 1990 were managed as inpatients, compared with 48% of patients diagnosed 1990 and later. The incidence of acute rheumatic fever may be underestimated if patients managed without hospitalization are not identified.

Left ventricular structures in atrioventricular septal defect associated with isomerism of atrial appendages compared with similar features with usual atrial arrangement.

In patients with isomeric atrial appendages, regurgitation of atrioventricular valves is recognized clinically as one of the risk factors that militate against successful achievement of definitive repairs. To determine whether this reflected anatomic features, we investigated 91 specimens with atrioventricular septal defect that had a common atrioventricular valve and biventricular atrioventricular connections. Of these specimens, 35 had isomeric right appendages, 23 showed isomeric left appendages, and 33 had usual atrial arrangement. We measured either the size or location of the supporting papillary muscles and the circumference of the mural leaflet within the morphologically systemic ventricle, as well as the length of outlet, inlet, and so-called scooped dimensions of the muscular ventricular septum. Presence of a solitary papillary muscle, or deviation of the attachments of the papillary muscles, was more frequent in hearts with isomeric right appendages. Values for the diameter and lengths of the papillary muscles were significantly smaller in hearts with isomeric right appendages compared with those with usual atrial arrangement (p < 0.001), as were the distances between the papillary muscles (p < 0.002) and the circumference of the mural leaflet (p < 0.001). The proportional length of ventricular outlet was longer in the setting of isomeric right appendages than in the other groups (p < 0.001), whereas the extent of septal scooping showed no differences among these three groups. We conclude that these structural features could be factors in the known insufficiency of the common atrioventricular valve and the ventricular dysfunction in patients with isomeric right appendages.

Feasibility of exercise stress echocardiography for the follow-up of children with coronary involvement secondary to Kawasaki disease.

BACKGROUND: The development of coronary aneurysms as sequelae of Kawasaki disease can result in myocardial ischemia, infarction, and sudden death. Traditionally, these patients have undergone coronary angiography and nuclear stress imaging for risk stratification and follow-up. However, angiography is invasive, and both modalities expose the patient to repeated radiation, which is an important issue in children. The purpose of this study was to determine the feasibility of performing exercise stress echocardiography in children diagnosed with coronary abnormalities secondary to Kawasaki disease. METHODS AND RESULTS: Treadmill exercise stress echocardiographic studies were performed in 28 children ages 6 to 16 years. All had acute Kawasaki disease 1 to 10 years before study, and coronary artery abnormalities were identified during previous echocardiographic imaging. Patients were exercised using a standard Bruce protocol. Transthoracic echocardiographic images, obtained in the parasternal long, short, apical two- and four-chamber views immediately before and after exercise, were digitized for review and analysis. In baseline studies before exercise, wall motion abnormalities were identified in 2 patients; these segments became normal with exercise. Two patients developed new exercise-induced wall motion abnormalities that corresponded to angiographically defined critical stenosis of the left anterior descending coronary artery. No patients had resting or exercise-induced ECG evidence of ischemia. There were no adverse reactions, and 26 of 28 patients had normal exercise tolerance. CONCLUSIONS: Among patients with coronary artery involvement resulting from Kawasaki disease, exercise stress echocardiography is a safe, noninvasive procedure and may identify children with myocardial ischemia that was not detected with ECG stress test alone.

The neurologic complications of congenital heart disease.

In recent years, an aggressive approach to the medical and surgical management of patients with complex cardiac malformations has led to an increased survival rate in this group of patients. Some of these patients often have significant residual cardiac defects and, at times, an unstable hemodynamic status. The cumulative risk for neurologic complications in this group of patients is expected to be substantial. Because the overall population of patients with congenital heart disease encompasses an increasing proportion of these high-risk patients, careful surveillance for potential neurologic complications is indicated.

Long-term results of survivors of surgical valvotomy for severe aortic stenosis in early infancy.

Long-term morbidity and mortality were evaluated in the 21 survivors of a cohort of 51 consecutive infants with severe aortic valve stenosis who underwent surgical treatment in the first 3 months of life during the period from 1958 to 1988. The 21 early survivors have been followed up from 3 to 27 years (median 7.5 years). There have been two late deaths: one at age 13 year from bacterial endocarditis and the other at age 14 years after dislodgment of a prosthetic valve. The calculated 10-year actuarial survival for this group is 100%, with a 15-year actuarial survival of 75% (standard error 15%). Seven repeat operations have been performed in six patients: Three had persistent stenosis and a repeat valvotomy was performed in two of them, aged 2 years and 15 years. The other underwent placement of a conduit from the left ventricle to the descending aorta at 2 years of age. Replacement of the aortic valve has been performed in four patients because of severe valvular insufficiency 13 to 27 years after the initial operation. One of these had required a repeat valvotomy at the age of 15 years. The calculated actuarial freedom from reoperation at 10 years is 90% (standard error 6%) and at 15 years, 67% (standard error 15%). Aortic insufficiency was progressive throughout the period of follow-up. No patient had more than moderate aortic insufficiency 3 to 5 years after the initial valvotomy, whereas aortic insufficiency was severe in five of the eight patients followed up for 11 or more years. Progression of aortic insufficiency and the need for reoperation were not related to the age at initial valvotomy. Survivors of surgical aortic valvotomy in early infancy have a relatively good long-term prognosis and a high freedom from reoperation in the period leading to adolescence. Aortic insufficiency in these patients is progressive, and valve replacement eventually may be required.

Balloon dilation of miscellaneous lesions: results of Valvuloplasty and Angioplasty of Congenital Anomalies Registry.

Data on 111 lesions, in addition to pulmonary valve, aortic valve, branch pulmonary stenosis, native coarctation and recoarctation, were submitted to the Valvuloplasty and Angioplasty of Congenital Anomalies Registry. Because there was a very heterogenous group of lesions both in types of lesions and techniques of dilation, only minimal data can be offered concerning the efficacy of the dilation of any or all of these lesions. The registry data do, however, demonstrate the safety of these dilations compared to alternative surgical therapy.

The value of angiography in the follow-up of coronary involvement in mucocutaneous lymph node syndrome (Kawasaki disease).

From September 1976 to August 1988, 205 patients with mucocutaneous lymph node syndrome (Kawasaki disease) were evaluated; 29 were suspected of having coronary artery abnormalities because of either abnormalities on echocardiography (n = 21), congestive heart failure (n = 3), prolonged fever (n = 3) or cardiac arrest (n = 2). All 29 underwent cardiac catheterization and 22 were found to have coronary artery abnormalities. An additional 4 patients were diagnosed solely by autopsy; therefore, 26 patients (12%) in this series had coronary artery abnormalities. The clearance of contrast medium from the coronary arteries after aortography was significantly prolonged in patients with coronary artery aneurysms as compared with that in a control group (p less than 0.001). All patients exhibited delayed clearance of contrast medium on the initial aortogram as compared with that on follow-up studies (p less than 0.01). Of the 22 patients with angiographically documented coronary artery abnormalities, 19 had right and 20 had left coronary artery involvement; the majority had diffuse involvement of both vessels. In 15 patients who underwent follow-up catheterization 6 months to 7 years after initial study, complete resolution was observed in only 37% of affected coronary artery segments. In four patients, three of whom were asymptomatic, severely stenotic or occluded coronary artery segments were found on late catheterization. Coronary artery bypass surgery was performed in two of these patients. Echocardiography did not detect any of these stenoses. Although echocardiography is important for initial diagnosis and serial evaluation, angiography is essential to fully define the nature and extent of coronary artery lesions, particularly if stenosis is present.

Corrected transposition presenting as aortic stenosis in an infant.

Associated intracardiac lesions have been well documented in congenitally corrected transposition. Most often these include ventricular septal defects, dysplasia of the morphologically tricuspid valve and subpulmonic stenosis. Aortic stenosis as the only isolated intracardiac defect in association with corrected transposition has not been described previously. We present such a patient and review the clinical features of corrected transposition.

Experience with heart transplantation in children.

Between March 1981 and March 1986, 200 orthotopic heart transplantations were performed at the University of Pittsburgh. Fourteen of those procedures were carried out in children 2 to 16 years of age. Two children received combined liver and heart transplants; one because of familial hypercholesterolemia with associated ischemic heart disease, and the other because of dilated cardiomyopathy associated with intrahepatic biliary atresia. Eight patients had dilated cardiomyopathy, and two had myocarditis. Two had heart transplantations for congenital heart disease: one had multiple muscular ventricular septal defects repaired in infancy and had an associated cardiomyopathy, and the other developed a cardiomyopathic ventricle from a congenital right coronary artery to right atrial fistula. Chronic immune suppression consisted 0.2 to 0.5 mg/kg/d of prednisone and 5 to 50 mg/kg/d cyclosporine, with the addition of antithymocyte globulin for unresolved moderate or severe acute rejection. There were three early postoperative deaths: one from intracranial bleeding, one from Pseudomonas mediastinitis, and one from ischemic injury to transplanted organs. Early postoperative complications included reversible renal failure, hypertension, and seizures. Late problems were related to allograft rejection and side effects of cyclosporine and corticosteroids. Significant rejection episodes occurred in all patients surviving longer than 2 weeks, with seven requiring antithymocyte globulin. Two patients died 8 months following transplantation of severe acute and chronic rejection; another patient required retransplantation for ischemic cardiomyopathy resulting from chronic rejection but subsequently died of recurring rejection 3 months after the second transplantation.(ABSTRACT TRUNCATED AT 250 WORDS)