Pulmonary artery growth fails to match the increase in body surface area after the Fontan operation.

OBJECTIVE: To evaluate the growth of the pulmonary arteries after a Fontan procedure. DESIGN: Retrospective review. SETTING: Two paediatric cardiology tertiary care centres. PATIENTS: 61 children who underwent a modified Fontan operation and had angiography suitable for assessment of pulmonary artery size before the Fontan procedure and during long term follow up. An atriopulmonary connection (APC) was present in 23 patients (37.7%) and a total cavopulmonary connection (TCPC) was present in 38 (62.3%). Postoperative angiograms were performed 0.5-121 months (median 19 months) after the Fontan operation. MAIN OUTCOME MEASURE: Growth of each pulmonary artery measured just before the first branching point. The diameter was expressed as a z score with established nomograms used to standardise for body surface area. RESULTS: The mean change in the preoperative to postoperative z scores of the right pulmonary artery was -1.06 (p = 0.004). The mean change in the preoperative to postoperative z scores of the left pulmonary artery was -0.88 (p = 0.003). Changes in the preoperative to postoperative z scores were more pronounced in the patients undergoing APC than TCPC, especially for the right pulmonary artery. CONCLUSION: After the Fontan operation, growth of the pulmonary arteries often fails to match the increase in body surface area.

Growth of the aorta in children with Williams syndrome: does surgery make a difference?

Supravalve aortic stenosis (SAS) and arch hypoplasia are features of Williams syndrome. The effect of aortoplasty on growth of the aorta is not established. We hypothesize that growth of the aorta remains deficient whether or not aortoplasty has been performed. Review of the Children's Hospital of Pittsburgh database revealed 18 patients with Williams syndrome and SAS. Fourteen had sufficient data for inclusion. Patients were divided into two groups based on whether or not they had undergone aortoplasty (groups 1 and 2, respectively). Echocardiographic velocity estimates of the aorta were made at two time points in all patients and one additional time point postoperatively for group 1. Measurements were converted to zeta scores and compared. Peak pulsed echo Doppler velocity (m/sec) in the ascending aorta was higher in patients who underwent aortoplasty. This decreased significantly after surgery. Preoperatively, there was no difference between the groups' annulus, ascending aorta, transverse aorta, and isthmus measurements. At a mean of 43 months postoperatively, there was no significant change in size of the ascending aorta, transverse aorta, and isthmus. Children with Williams syndrome have hypoplasia of the aortic arch that remains constant. Aortoplasty decreases the aortic gradient but has no effect on the size of the ascending aorta, transverse aorta, and aortic isthmus over the short-term.

Changes in coil morphology following transcatheter coil occlusion of the patent ductus arteriosus using a modified snare technique.

We observed a decrease in length of the Gianturco coils following transcatheter occlusion of the patent ductus arteriosus (PDA). Coil length was measured on chest radiograph within 24 hr of coil placement and compared to the length at the time of follow-up. Echocardiograms were also reviewed for evidence of duct recanalization. Twenty-seven patients met inclusion criteria. The median time to follow-up was 5 months (1--12 months). Twenty-four out of 27 (89%) patients had a decrease in coil length by 1.9 +/- 1.1 mm (P < 0.01). This was an average decrease of 16%. One patient had an increase in length and two patients had no change in coil length. Nineteen out of 27 patients had echocardiograms. Despite the change in coil length, there was no evidence of flow acceleration in the pulmonary artery or descending aorta. No patients had evidence of duct recanalization.

Use of terbutaline in the treatment of complete heart block in the fetus.

Between 1989 and 2000, 21 fetuses were diagnosed with complete atrioventricular block. Seven women with fetal ventricular rates of less than 60 were given oral terbutaline, and 6 of these had an initial increase in the fetal ventricular rate. Four fetuses (57%) maintained an increased average rate of 60 beats per minute and survived. Two fetuses returned to rates below 55 and died. The final fetus, with hypertrophic cardiomyopathy, was unresponsive. Terbutaline, therefore, is initially effective in raising the fetal ventricular rate, but this effect may be transient.

Management of retroperitoneal arterial injury after heart catheterization in children.

With the expansion of interventional cardiology into the pediatric population, vascular complications related to cardiac catheterization can be expected to occur. Cardiac surgeons must be prepared to treat these life-threatening injuries. We present a case and detail the technique of the surgical management of retroperitoneal arterial injury after interventional cardiac catheterization in a 6-month-old boy.

Balloon aortic valvotomy through a carotid cutdown in infants with severe aortic stenosis: results of the multi-centric registry.

OBJECTIVES: The purpose of this study was to evaluate the short and intermediate term results of infants who have undergone balloon aortic valvotomy from the carotid arterial approach, and to identify risk factors in those infants who had a poor outcome. METHODS: Between 1988 and 1999, balloon aortic valvotomy was attempted at four centres in 95 infants with severe aortic stenosis. Echocardiographic and hemodynamic data, and outcome, were analysed retrospectively. RESULTS: Valvotomy was accomplished in 92 of the 95 infants, with a median age of 5 days, a range from 0 to 191 days, and weighing 3.4 kg, with a range from 1.0 to 6.5 kg. Major procedural complications occurred in 10 infants. Post-procedural aortic regurgitation was severe in 5 patients. There were 13 early deaths, and 4 late deaths. The period of mean follow-up has been 2.1 years, with a range from 0 to 9.3 years. The actuarial survival at 3 years was 76 +/- 6%. Further interventions were needed in 19 patients, giving a 3-year freedom from reintervention of 67 +/- 6%. The 51 infants who were duct-dependent were further analyzed, and found to have a higher mortality (38%) compared to those infants not dependent on the arterial duct (5%). Risk factors for a poor outcome in the duct-dependent infants were mitral stenosis (p<0.005), a left ventricle which did not form the cardiac apex (p<0.005), and an aortic valve with a diameter of less than 6 mm (p<0.05). CONCLUSIONS: This multi-centric registry shows good results in the intermediate term for treating infants with severe aortic valvar stenosis with balloon valvotomy through a carotid arterial cutdown. Infants dependent on prostaglandin had a worse outcome, especially if they had any of the identified risk factors.

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.

Rapid enlargement of neoaortic root after the Ross procedure in children.

Serial echocardiographic studies from 11 patients who underwent the Ross procedure were reviewed, and the rate of neoaortic annulus size increase was compared with that in a normal population. The rate of growth of the neoaortic annulus after the Ross procedure was significantly greater than that in the normal population.

Technique for repair of single-ventricle hearts with transposition of the great arteries and aortic arch hypoplasia.

We describe a modified technique of aortopulmonary anastomosis for palliative repair of hearts with a single left ventricle, a rudimentary right ventricle, transposition of the great vessels, and a hypoplastic aortic arch. This procedure creates an unobstructed left ventricular outflow tract and avoids the problems of spiral patch grafting and multiple patch enlargements of the ascending aorta and arch.

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.

Are chest radiographs and electrocardiograms still valuable in evaluating new pediatric patients with heart murmurs or chest pain?

OBJECTIVES: To determine the usefulness of electrocardiography (ECG) and chest radiography (CXR) in evaluation of patients referred to the pediatric cardiologist for the evaluation of heart murmur or chest pain. DESIGN: In this prospective study, 106 consecutive outpatients were categorized with no heart disease, possible heart disease, or definite heart disease based on history and physical examination; they then underwent ECG and CXR. Studies were reviewed and the examining cardiologist could change the diagnosis and order an echocardiogram. SETTING: Academic pediatric cardiology practice. RESULTS: In patients thought to have no heart disease, the diagnosis was changed to definite heart disease in four solely on the basis of abnormal CXR or ECG. In 25 patients thought to have possible heart disease, the diagnosis was changed to no heart disease (7) or definite heart disease (5) after review of the CXR and ECG. All 25 patients diagnosed with definite heart disease had this confirmed by abnormal CXR (2), ECG (3), both abnormal CXR and ECG, or echocardiogram (18). CONCLUSIONS: ECG and CXR helped diagnose heart disease in four patients thought to have no heart disease, helped to rule out lesions in seven patients with possible heart disease, helped diagnose heart disease in five patients thought to have possible heart disease, and helped confirm heart disease in nine patients. In these days of cost containment, routine ECG and CXR continue to be valuable tools for the pediatric cardiologist in evaluation of patients with heart murmurs or chest pain.

Influence of stenotic valve geometry on measured pressure gradients and ventricular work: the relationship between morphology and flow.

The physiologic impact of aortic valve stenosis is most directly reflected by an increased workload placed on the ventricle. In the pediatric population the morphology of aortic stenosis varies considerably. Fluid dynamic principles suggest that different morphologies may require the ventricle to accelerate blood to different maximal velocities for constant cardiac outputs and valve areas, resulting in different ventricular workloads. This study examined this important concept in in vitro models designed to isolate the effect of valve geometry on distal velocity, pressure gradients, and proximal work. Four stenotic valve morphologies were examined using a variable-voltage pump system. For constant orifice areas and flows, markedly different workloads were required by the pump, and this difference was reflected in direct measurements of pressure gradient and Doppler predictions of gradient. These fundamental fluid dynamic studies isolate the relationship between flow, work, and stenotic valve morphology. Different orifice geometries affect the value of the coefficient of contraction, which is reflected in different maximum velocity values for stenosis with constant anatomic areas and flows. The proximal pumping chamber must generate different levels of force to achieve these different velocities, and this variability is reflected in the clinically measured pressure gradient.

Echocardiographic-morphologic correlations in tricuspid atresia.

OBJECTIVES: Our aim was to clarify the anatomic substrate in hearts diagnosed as having tricuspid atresia by studying autopsy specimens and comparing the findings with those in two-dimensional echocardiograms. BACKGROUND: Traditionally, tricuspid atresia was thought, and is still believed by some, to be due to an imperforate valvular membrane interposed between the floor of the blind-ending right atrium and the hypoplastic right ventricle. Others argued that the dimple, when present, pointed to the outflow tract of the left ventricle rather than to the right ventricle, making the lesion more akin to double-inlet left ventricle. METHODS: We examined 39 autopsy specimens catalogued as having tricuspid atresia. We then studied 24 two-dimensional echocardiograms from patients with a primary diagnosis of tricuspid atresia. RESULTS: Of the 39 specimens, 37 had a completely muscular floor to the right atrium (absent right atrioventricular [AV] connection). The dimple, when identified, was (except in one case) directed to the left ventricular outflow tract. Only two hearts had an imperforate tricuspid valve. Two-dimensional echocardiograms in all cases showed an echo-dense band, produced by the fibrofatty tissue of the AV groove and representing absence of the right AV connection, between the muscular floor of the morphologically right atrium and the ventricular mass. CONCLUSIONS: Tricuspid atresia is usually, but not always, due to morphologic absence of one AV connection. In most cases, the ventricular mass then comprises a dominant left ventricle together with a rudimentary and incomplete right ventricle.

Is a cleft in the anterior leaflet of an otherwise normal mitral valve an atrioventricular canal malformation?

OBJECTIVES: This study sought to ascertain the surgical anatomy of a cleft in the left atrioventricular (AV) valve. BACKGROUND: Important morphologic differences exist between hearts with a cleft in the anterior leaflet of an otherwise normal mitral valve and those with a so-called cleft in the left AV valve when there is an AV septal defect, but it has been customary to link the lesions together on developmental grounds. METHODS: Eight autopsied specimens with a cleft in the aortic (or anterior) leaflet of the mitral valve were studied in detail, and echocardiograms from 21 patients with such a cleft were compared with the specimens and with findings typical of the so-called partial AV canal and other forms of AV septal defect. RESULTS: The structure and direction of the cleft, location of the papillary muscles within the left ventricle and AV junctional morphology of hearts with an otherwise normally structured mitral valve were significantly different from typical findings in hearts with AV septal defects. CONCLUSIONS: It is necessary to distinguish morphologically a cleft in an otherwise normally structured mitral valve in hearts with separate right and left AV junctions from the trifoliate left component of a common AV valve in hearts with an AV septal defect and a common AV junction because the disposition of the AV conduction tissues varies markedly between the lesions.

Transatrial lead placement for endocardial pacing in children.

Transvenous placement of endocardial leads in children may be difficult due to restrictions and complications of vascular access. We have placed endocardial leads from a transatrial approach in 5 children with various cardiac malformations. The usual surgical approach involved an anterolateral thoracotomy and, under fluoroscopic guidance, passage of the lead tip directly through the right atrial wall and across the tricuspid valve to the apex of the right ventricle. At a mean follow-up time of 23.2 months (range, 12.0 to 27.9 months), all patients have low thresholds for myocardial capture, and there have been no complications. We conclude that placement of endocardial leads by a transatrial approach provides an excellent alternative to an epicardial system in children destined for lifelong pacing.

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.