Disparities in the prenatal detection of critical congenital heart disease.

OBJECTIVES: Prenatal diagnosis of critical congenital heart disease, which requires surgical or catheter intervention in the first 30 days of life, allows for delivery at a specialized center and can reduce preoperative morbidity and mortality. We sought to identify the risk factors for a missed prenatal diagnosis of critical congenital heart disease. METHODS: Patients presenting to the Children's Hospital of Wisconsin with critical congenital heart disease from 2007 to 2013 were included. Those with a prenatal diagnosis were compared with those with a postnatal diagnosis. RESULTS: The cohort included 535 patients with prenatal diagnosis made in 326 (61%). The prenatal diagnostic rate improved from 44% in 2007 to 69% in 2013. Independent factors associated with a postnatal diagnosis were a lesion that required a view other than a four chamber view to make the diagnosis (p < 0.0001), absence of another organ system anomaly (p < 0.0001), and living in a higher poverty (p = 0.02) or lower population density communities (p = 0.002). CONCLUSIONS: While the prenatal diagnostic rate for critical congenital heart disease is improving, those living in impoverished or rural communities are at highest risk of not having a diagnosis made prenatally. Interventions to improve prenatal detection of congenital heart disease should target these vulnerable areas.

Psychological distress in parents of children with severe congenital heart disease: the impact of prenatal versus postnatal diagnosis.

OBJECTIVE: The purpose of the study was to evaluate coping and psychological functioning of parents of children prenatally or postnatally diagnosed with congenital heart disease. STUDY DESIGN: Parents of 10 infants prenatally diagnosed by fetal echocardiogram and 7 infants postnatally diagnosed with severe congenital heart disease completed the Brief Symptom Inventory (BSI) at the time of diagnosis, at the time of birth and 6 months after birth. Prenatal and postnatal groups were compared to each other and to BSI norms. RESULT: Although both groups scored higher than test norms at the time of diagnosis, they did not differ significantly from each other. Six months after birth, the postnatal group scores did not differ from test norms, but the prenatal group scores were still significantly higher than test norms. The severity of the child's heart lesion at diagnosis was related to parental distress levels; parents with children with more severe lesions had higher BSI scores. CONCLUSION: Results suggest the need to provide parents with psychological support, regardless of the timing of diagnosis. Parents of children with more severe lesions may be at risk for higher levels of psychological distress, particularly over time.

Experience with a DICOM-compatible digital pediatric echocardiography laboratory.

A digital pediatric echocardiography laboratory, without videotape redundancy was established at Children's Hospital of Wisconsin in December 1998. To characterize the experience, 1198 consecutive patient studies were reviewed-50% from the first 2 months after establishing the digital protocol and 50% from the last 2 months available. Each study was stored using a protocol that was based on capture of single beat clips of relevant two-dimensional/color Doppler imaging and static frame spectral Doppler tracings. Studies were digitally compressed using a DICOM-compatible JPEG algorithm at 20:1 and edited with deletions of redundant clips to minimize archival storage needs. Study quality was uniformly excellent, and no errors were attributable to the digital protocol or compression-related loss of information. The average study required 21.5 +/- 11.4 MB of storage space with 35.4 +/- 12.3 total clips/study captured. Studies reviewed from the earlier experience were not significantly larger (23.6 +/- 14 vs 19.7 +/- 8.1 MB, 35.6 +/- 12.5 vs 35.2 +/- 12 clips) than those done recently. Studies in patients with isolated ventricular septal defect used comparable storage (23.7 +/- 8.9 MB, 42.8 +/- 11.5 clips) to that of the group as a whole. More complex congenital heart disease studies were slightly larger-tetralogy of Fallot (28.2 +/- 19.5 MB, 43.4 +/- 13.9 clips), transposition of the great arteries (30.6 +/- 17.4 MB, 40.3 +/- 16.7 clips), and single ventricle (29.7 +/- 19.6 MB, 39.9 +/- 12 clips)--although this trend was not significant. This study suggests that digital pediatric echo is feasible using a DICOM-compatible protocol with maintenance of diagnostic integrity despite compression of study size to allow rapid archival storage and retrieval.

Factors affecting longevity of homograft valves used in right ventricular outflow tract reconstruction for congenital heart disease.

BACKGROUND: Few studies have explored the long-term function of cryopreserved homograft valves used for reconstruction of the right ventricular tract (RVOT) in patients with congenital heart disease. METHODS AND RESULTS: Among 205 patients receiving cryopreserved homografts for reconstruction of the RVOT between November 1985 and April 1999, the outcome of 220 homografts in 183 operative survivors was analyzed. There were 150 pulmonary and 70 aortic homografts used. Median age at implantation was 4.4 years (mean 6.9+/-7.6 years, range 3 days to 48 years). End points included (1) patient survival, (2) homograft failure (valve explant or late death), and (3) homograft dysfunction (homograft insufficiency or homograft stenosis). Survival was 88% at 10 years. Freedom from homograft failure was 74+/-4% at 5 years and 54+/-7% at 10 years. Univariable analysis identified younger age, longer donor warm ischemic time, valve Z: value <2, and previous procedure as risk factors for homograft failure and dysfunction. Aortic homograft type and extracardiac operative technique predicted homograft failure but not dysfunction. For patients

Advances in echocardiographic diagnostic modalities for the pediatrician.

Two-dimensional Doppler echocardiography has become the primary diagnostic tool in the assessment of infants and children with congenital and acquired heart disease. Over the past 10 years, specialized echocardiographic techniques have also become critical components in the evaluation and treatment of these patients. Using fetal echocardiography enables us to image the heart early in gestation and have begun to understand those lesions that can develop and progress in utero. Transesophageal echocardiography has allowed you to image the patient with congenital heart disease during repair in the operating room and in the cardiac catheterization laboratory so that adequacy of the repair can be assess and any residual lesions addressed immediately. Both of these specialized techniques are discussed in detail, with a brief overview at the three-dimensional future of echocardiography in the pediatric patient.

Anomalous origin of the right coronary artery from the left sinus of valsalva: transthoracic echocardiographic diagnosis.

Anomalous origin of the right coronary artery from the left sinus of Valsalva is a rare congenital defect that can be difficult to diagnose by echocardiography. We describe an infant with a ventricular septal defect that was diagnosed prospectively by transthoracic echocardiography as an anomalous origin of the right coronary artery from the left sinus of Valsalva. Subcostal imaging and Doppler color flow mapping were instrumental in the echocardiographic diagnosis of this unusual coronary abnormality.

Advances in the care of children with heart disease.

As we enter the next millennium, we are encouraged by the progress that has been made in the care of neonates, infants, and children with heart disease. Surgical repair can be offered at an earlier age with excellent results. Diseases that were uniformly fatal in the past have improved outcomes. Research continues in the area of interventional devices such that surgical repair might be eliminated or delayed. We continue to look forward to advances in the next several years that will allow for future improvement in outcome, better quality-of-life and better long-term results.

Aprotinin improves outcome of single-ventricle palliation.

BACKGROUND: Elevation of pulmonary vascular resistance as a consequence of cardiopulmonary bypass may lead to failure of single-ventricle palliation. We reviewed our experience with aprotinin, a nonspecific serine protease inhibitor, to determine whether it could ameliorate the inflammatory effects of cardiopulmonary bypass and improve outcome of single-ventricle palliation. METHODS: Forty-six consecutive patients undergoing single-ventricle palliation using cardiopulmonary bypass were reviewed retrospectively. Aprotinin was used in 8 of 30 bidirectional cavopulmonary shunt and 10 of 16 Fontan procedures. RESULTS: Aprotinin use was associated with a decrease in the early postoperative transpulmonary gradient among patients undergoing Fontan and bidirectional cavopulmonary shunt procedures. The bidirectional cavopulmonary shunt aprotinin group had a higher oxygen saturation and a decrease in quantity and duration of thoracic drainage. Among patients receiving aprotinin there were no episodes of mediastinitis, thrombus formation, or renal failure. CONCLUSIONS: Aprotinin use in single-ventricle palliation was associated with decreased transpulmonary gradient and increased oxygen saturation consistent with decreased pulmonary vascular resistance. This retrospective study suggests that aprotinin has a favorable impact on the early postoperative course of single-ventricle palliation.

Twenty-year experience with repair of complete atrioventricular septal defects.

BACKGROUND: To determine factors predicting mortality and morbidity after repair of complete atrioventricular septal defect, we retrospectively analyzed preoperative, operative, and postrepair factors on the outcome of 115 consecutive complete atrioventricular septal defect repairs at The Children's Hospital of Wisconsin between January 1974 and December 1993. METHODS: For the entire experience the operative mortality was 13.9% (16 patients). During the most recent era, January 1988 to December 1993, operative mortality was 3.6% (2 of 55 patients). This was significantly improved from the two previous eras, January 1974 to December 1980, 28% (7 of 25) and January 1981 to December 1987, 20% (7 of 35 patients) (p = 0.02). There were seven late deaths; 10-year actuarial survival, including operative mortality was 81%. Age at complete repair decreased; before 1982 all patients were more than 12 months of age, whereas after 1982 64% (56 of 88 patients) were 12 months of age or less. RESULTS: Moderate or severe preoperative left atrioventricular valve regurgitation was not a risk factor for operative mortality. For operative survivors with moderate to severe preoperative left atrioventricular valve regurgitation (n = 17), late postoperative left atrioventricular valve regurgitation (follow-up data available on 15 patients) was significantly reduced (severe = 1, moderate = 5, mild = 9; p = 0.007). CONCLUSIONS: Early mortality was predicted by the era of surgical repair. Conversion to routine repair during infancy was achieved with a simultaneous decrease in operative mortality. For patients with moderate to severe preoperative left atrioventricular valve regurgitation, significant improvement in the degree of left atrioventricular valve regurgitation can be expected without an increase in operative or late mortality or morbidity.

Does an additional source of pulmonary blood flow alter outcome after a bidirectional cavopulmonary shunt?

BACKGROUND: The bidirectional cavopulmonary shunt has become an important intermediate step in the treatment of pediatric patients with single ventricle physiology who are ultimately destined for palliative surgery. We wanted to know whether there would be risks or benefits if an additional source of pulmonary blood flow was left after a bidirectional cavopulmonary shunt. METHODS AND RESULTS: We retrospectively reviewed the medical and surgical records of all patients who underwent a bidirectional cavopulmonary shunt at the Children's Hospital of Wisconsin between January 1991 and December 1993. A total of 43 patients were identified. Anatomic diagnoses included double inlet left ventricle (14 patients), tricuspid atresia (8 patients), pulmonary atresia with intact septum (6 patients), single right ventricle (5 patients), hypoplastic left heart (3 patients), unbalanced atrioventricular septal defect (3 patients), and other complex lesions (4 patients). We then divided the patients into two groups for purposes of analysis. Group 1 had only the cavopulmonary shunt as a source of pulmonary flow (22 patients); group 2 had an additional source of pulmonary flow (21 patients). Patient age at the time of cavopulmonary shunt ranged from 6 months to 12 years, with group 1 patients being younger (31 versus 45 months, P = .05). Group 2 patients had higher postoperative central venous pressures (17.8 versus 14.1 mm Hg, P < .001) and oxygen saturations (86% versus 81%, P < .001) than did group 1 patients. There was no statistical difference between groups in the number of chest tube days or hospital days. There was 1 early death in group 1 related to severe ventricular dysfunction and 1 late death in group 2 related to sepsis. Five patients in group 2 were readmitted to the hospital for drainage of a large chylothorax compared with none in group 1 (P < .02). CONCLUSIONS: We conclude that patients with an additional source of pulmonary blood flow after bidirectional cavopulmonary shunt have higher postoperative central venous pressures, have higher oxygen saturations, and are at risk for the late development of a chylothorax.

Echocardiographic assessment of subvalvular aortic stenosis before and after operation.

The development of two-dimensional and Doppler echocardiography has provided a noninvasive technique for the diagnosis and serial assessment of patients with subvalvular aortic stenosis. The clinical records and echocardiographic data were reviewed of all patients with subaortic stenosis diagnosed between 1983 and 1991. Of the 77 patients identified (45 male and 32 female), 28 had isolated subaortic stenosis and 49 had associated cardiac lesions. The most frequently encountered associated lesions were ventricular septal defect (n = 19) and coarctation of the aorta/interrupted aortic arch (n = 14). Serial echocardiographic studies, performed in 38 of the 77 patients, documented significant progression of the left ventricular outflow tract gradient in 25 patients (66%) and development of aortic regurgitation in 25 patients (66%). Surgical resection was performed in 36 patients. The preoperative outflow tract peak gradient was 62.9 +/- 31 mm Hg (range 0 to 153), whereas the immediate postoperative gradient was 14.4 +/- 14 mm Hg (range 0 to 67). The two patients with a significant residual gradient (37 and 67 mm Hg, respectively) in the immediate postoperative period had severe subaortic stenosis preoperatively with marked left ventricular hypertrophy and intracavitary gradient. The immediate postoperative echocardiograms demonstrated no worsening of aortic regurgitation in any patient and regression of regurgitation in one patient from mild to none. Intermediate-term follow-up studies were available for review in 13 postoperative patients at a mean of 4 years postoperatively. In 2(15%) of these 13 patients, subaortic stenosis recurred; however, the degree of aortic regurgitation did not increase in any patient.(ABSTRACT TRUNCATED AT 250 WORDS)

Echocardiographic indexes of allograft rejection in pediatric cardiac transplant recipients.

To determine the usefulness of echocardiographic indexes of left ventricular (LV) function as possible predictors of cardiac rejection, 12 transplant recipients (ages 3 to 17 years) underwent a total of 52 serial echocardiographic examinations and cardiac biopsies. The results were compared to those of 12 normal children (ages 2 to 17 years). Biopsies were graded as no rejection (n = 23), mild rejection (cellular infiltrate, n = 13), and moderate rejection (myocyte necrosis, n = 16). LV dimensions, percent shortening fraction, indexed LV mass, and ejection fraction were measured from M-mode and two-dimensional echocardiography. From the mitral valve Doppler tracing, the following measurements were made: isovolumic relaxation time, peak E and peak A velocities, and the fraction of filling under the E and A waves as well as in the first third of diastole. Compared with normal subjects, transplant recipients with no rejection had higher heart rates (95 +/- 15 vs 80 +/- 17 beats/min), longer isovolumic relaxation time (68.8 +/- 11.2 vs 51.5 +/- 13.6 msec), decreased first third area fraction (0.48 +/- 0.10 vs 0.57 +/- 0.10), and similar shortening fraction, LV mass, and peak E and A velocities (p less than 0.03). Compared with transplant recipients with no rejection, patients in whom mild rejection developed also had decreased shortening fraction (31% +/- 10% vs 37% +/- 8%) and decreased peak E velocity (0.68 +/- 0.19 vs 0.88 +/- 0.15 m/s) (p less than 0.03). From mild to moderate rejection, no further changes were noted in any echocardiographic indexes measured.(ABSTRACT TRUNCATED AT 250 WORDS)