No abstract

Patients with Down syndrome tend to show prolonged recovery after TCPC. However, as opposed to previous reports, the mortality rate of patients undergoing TCPC is lower with no significant difference from that of children without Down syndrome.

Down's syndrome is a risk factor in patients with functionally single ventricle due to persistent pulmonary hypertension and airway obstruction. These results show that single ventricle repair in patients with Down's syndrome is accompanied with difficulties, and patient selection for the Fontan procedure should be done carefully.

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

We report two autopsy proven cases of unguarded mitral orifice associated with mirror-imaged atrial arrangement, discordant atrioventricular connections, double outlet right ventricle, pulmonary valvar stenosis or atresia, and atrialisation of the morphologically left ventricle. The morphologically left atrioventricular junction was devoid of valvar leaflets, and there was no tension apparatus within the ventricle. To the best of our knowledge, this is the first description of this rare cardiac malformation.