The beneficial hemodynamic effects of selective patent vertical vein following repair of obstructed total anomalous pulmonary venous drainage in infants.

OBJECTIVES: Postoperative low cardiac output may persist after repair of total anomalous pulmonary venous drainage (TAPVD) because of a relatively small and non-compliant left atrium and left ventricle. We examined the effects of selective vertical vein patency on postoperative hemodynamics. METHODS: Thirty-four patients less than 3 months of age with TAPVD were operated from July 1993 to June 2000. The mean age at operation was 21+/-8 days (range, 3-62 days) and the mean weight was 3+/-0.2 kg (range, 2-4.1 kg). Supracardiac type drainage was found in 12 (35%), cardiac in three (9%), mixed in one (3%), and infracardiac in 18 (53%) patients. Twenty-two patients (65%) had obstructed venous drainage. All operations were performed with deep hypothermic circulatory arrest. Supracardiac, mixed and infracardiac types were repaired through a posterior approach, whereas, in the cardiac type, the coronary sinus was unroofed and the atrial septal defect was patched. The decision whether to keep the vertical vein open was made at the end of the operation and was based on the hemodynamic state of the patient. RESULTS: There were no operative deaths. The suture on the vertical vein was released in 22 patients who had obstructed pulmonary venous drainage (infracardiac type, n=18; supracardiac type, n=3; and mixed type, n=1), resulting in a significant drop in the left atrial pressure from 19+/-2 to 12+/-2 mmHg (P<0.05), and in the mean pulmonary artery pressure from 42+/-6 to 35+/-3 mmHg (P<0.05), associated with an immediate increase in the mean arterial blood pressure from a mean of 46+/-3 to 60+/-4 mmHg (P<0.05). During a mean follow-up of 38+/-6 months (range, 8-71 months), there were no late deaths. Follow-up, two-dimensional echocardiography with Doppler studies demonstrated good left ventricular function and trivial or no left to right shunt through the vertical vein in those patients in whom the snare was released. CONCLUSIONS: Maintaining the vertical vein patent in a selective group of patients with infracardiac total anomalous venous drainage contributes to a favorable outcome following surgery.

Low-dose methotrexate as a steroid-sparing agent in a child with Takayasu's arteritis.

Takayasu's arteritis (TA) is a chronic inflammatory arteriopathy affecting the large vessels and has been described predominantly in young adult women. In children it presents as an aggressive disease usually requiring chronic corticosteroid therapy. At present, low dose oral methotrexate (MTX) appears to be an effective steroid-sparing agent in adult patients with active TA. We report a 4-year-old child with Takayasu's arteritis who was initially placed on oral prednisone (2 mg/kg/day) therapy. Three months later, low-dose oral MTX (10 mg/m2/week) was added. Prednisone was successfully tapered over the following year to 0.2 mg/kg every other day. A repeat angiography following 12 months of therapy revealed a dramatic improvement of the vascular lesions. No toxicity was observed with MTX therapy. In conclusion, low-dose oral MTX appeared to be an efficient, safe and steroid-sparing agent in the treatment of a young child with TA.

Anti-Ro/SS-A and anti-La/SS-B antibodies associated with cardiac involvement in childhood systemic lupus erythematosus.

OBJECTIVES: To determine the frequency and type of cardiac manifestations in children with systemic lupus erythematosus (SLE) and investigate whether cardiac involvement of SLE in children was associated with any autoantibody pattern. METHODS: Retrospective analysis of the medical records of all children with SLE (31 patients) seen between January 1984 and January 1994 by the paediatric rheumatology service at Children's Hospital in New Orleans. All patients satisfied the American College of Rheumatology criteria for the diagnosis of SLE. Paediatric SLE patients with cardiac manifestations based on echocardiogram were identified. Autoantibody tests at diagnosis were identified retrospectively by chart review, and the correlation between autoantibodies and cardiac involvement was analysed using the two tailed Fisher's exact test. RESULTS: Thirteen (42%) of 31 SLE patients had cardiac manifestations of SLE. Seven (22%) had pericarditis without myocarditis, five (16%) had pericarditis and myocarditis, and one (3%) had myocarditis without pericarditis. Two patients (6%) with pericarditis had cardiac tamponade. Cardiac manifestations of SLE usually occurred at the time of diagnosis or within six months. Anti-Ro/SS-A antibodies were present in serum samples of nine of 11 (82%) patients with cardiac involvement and in five of 15 (33%) without cardiac involvement (p = 0.02). Anti-La/SS-B antibodies were present in serum samples of six of 10 (60%) patients with cardiac involvement and two of 15 (13%) without cardiac involvement (p = 0.03). Anti-Sm and anti-RNP antibodies showed no correlation with the presence of cardiac disease. CONCLUSIONS: Cardiac involvement in our paediatric SLE population was frequently found and correlated significantly with the presence of anti-Ro/SS-A and anti-La/SS-B antibodies.

Diagnosis of double aortic arch by cross sectional echocardiography with Doppler colour flow mapping.

A case of double aortic arch in a 15 month old boy was diagnosed by cross sectional echocardiography supplemented by Doppler colour flow mapping. These examinations showed two very large vessels communicating with the aorta; the demonstration of anterograde flow in one vessel and retrograde flow in the other enabled a definite diagnosis of Edwards type IA double aortic arch to be made.

Heart disease in a total population of children: the Bogalusa Heart Study.

A cross-sectional survey of a total semirural biracial population of children was conducted to determine the prevalence and types of cardiac disease. Of 4,074 children examined, 146 were thought to have significant cardiac murmurs. One hundred thirteen of these participated in a follow-up study in which they received a physical examination by two adult cardiologists and one pediatric cardiologist, a chest roentgenogram, and an electrocardiogram. An attempt at a consensus concerning the diagnosis was made by subsequent discussions among the physicians. The discussions were repeated in 33 cases after a phonocardiogram and/or an echocardiogram were done. In 20 children, cardiac disease was diagnosed by at least two cardiologists. Supplementing this with information from hospital records on some of the children and data from a previous study, cardiac abnormalities were diagnosed in 25 children from the total population (0.6%). A disproportionately greater number of black children and boys were affected. Thirteen children had congenital heart disease, five had mitral valve prolapse, and only two had rheumatic heart disease. The prevalence of mitral valve prolapse was probably underestimated in this study.