Congenital heart defects (CHD) are very common in patients with trisomy 18 (T18) and trisomy 13 (T13). The surgical indication of CHD remains controversial since the natural history of these trisomies is documented to be poor. To investigate the outcome of CHD in patients with T18 and T13, we collected and evaluated clinical data from 134 patients with T18 and 27 patients with T13 through nationwide network of Japanese Society of Pediatric Cardiology and Cardiac Surgery. In patients with T18, 23 (17%) of 134 were alive at this survey. One hundred twenty-six (94%) of 134 patients had CHDs. The most common CHD was ventricular septal defect (VSD, 59%). Sixty-five (52%) of 126 patients with CHD developed pulmonary hypertension (PH). Thirty-two (25%) of 126 patients with CHD underwent cardiac surgery and 18 patients (56%) have survived beyond postoperative period. While palliative surgery was performed in most patients, six cases (19%) underwent intracardiac repair for VSD. Operated patients survived longer than those who did not have surgery (P < 0.01). In patients with T13, 5 (19%) of 27 patients were alive during study period. Twenty-three (85%) of 27 patients had CHD and 13 (57%) of 27 patients had PH. Atrial septal defect was the most common form of CHD (22%). Cardiac surgery was done in 6 (26%) of 23 patients. In this study, approximately a quarter of patients underwent surgery for CHD in both trisomies. Cardiac surgery may improve survival in selected patients with T18.
Background:The effect of carvedilol on heart failure (HF) in patients with a functionally univentricular heart (UVH) remains unclear.
Methods and Results:Carvedilol was used to treat HF in 51 patients with a UVH, classified into 3 groups: after the Fontan operation (F), after the bidirectional Glenn operation (G), and patients who had not undergone Fontan or Glenn operation (NF). Carvedilol therapy was started at a mean age of 10±12 years (range: 1 month to 34 years). The initial and maximum doses of carvedilol were 0.04±0.03 and 0.42±0.29 mg · kg -1 · day -1 , respectively. After a mean follow-up of 11 months, the cardiothoracic ratio improved from 60±8 to 58±8% (P<0.01), and the dosage of furosemide was reduced from 1.4±0.9 to 0.7±0.7 mg · kg -1 · day -1 (P<0.01). The ejection fraction also improved from 35±12 to 40±11% (P<0.05), and this improvement was prominent in the F group (from 35±15 to 45±9%; P<0.05). Clinical signs, symptoms, and New York Heart Association functional class also improved.
Conclusions:Carvedilol may play an important role in treating HF associated with a UVH. (Circ J 2011; 75: 1394 - 1399
A subcutaneous figure of eight suture hemostasis strategy can provide a safe and efficient method for large caliber venous hemostasis in a pediatric practice with improved hemostasis times and no additional morbidity.
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