The geometric arrangement of the great arteries correlated significantly with obstruction in either ventricular outflow tract and with the Rastelli subtypes. Malrotation of the developing outlet septum may be an embryologic factor producing obstruction, with horizontal deviation of the outlets also influencing the morphology of the superior bridging leaflet.
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
Those with Rastelli type C and an undivided inferior leaflet had a lesser degree of progression of preoperative regurgitation. However, regurgitation was likely to exist even after adequate repair once regurgitation had already advanced. Therefore, early primary repair before progression of the regurgitation may be the key to maintaining better competence of the atrioventricular valve.
We report a case of a patient with severe heart failure after Fontan procedure in whom carvedilol was very effective. A 27-year-old man had intractable congestive heart failure due to severe ventricular dysfunction after Fontan operation. Central venous pressure was elevated to 29 mmHg. A right-to-left shunt was noted across a large collateral vessel between the innominate vein and the pulmonary vein. He was administered carvedilol (initial dose, 2 mg/day; maximum dose, 30 mg/day). Cardiac catheterization performed 1 year after carvedilol administration revealed a decrease in atrial pressure and improvement of ventricular function. He underwent a conversion operation to total cavopulmonary connection (TCPC) and ligation of a collateral vein communicating with the innominate and pulmonary veins. Carvedilol may be a legitimate treatment before TCPC conversion or heart transplantation for the high-risk group of patients with a failed Fontan circulation.
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