Serial cardiorespiratory exercise testing in patients with congenital heart disease.

Aerobic capacity of patients with different forms of congenital heart disease was serially evaluated in 79 patients and the evolution was correlated with the lesion and the level of daily activity. The patients were divided into six groups: patients with a small ventricular septal defect (VSD) with mini shunt (n = 14), mild pulmonary valve stenosis with gradient < 40 mm Hg (PS) (n = 12), mild to moderate aortic valve stenosis (gradient 36 +/- 17 mmHg) (AS) (n = 12), patients 4.7 +/- 2.

Reliability of subjective estimates of exercise capacity after total repair of tetralogy of Fallot.

Sixty-nine patients (age 10 +/- 3.5 years, median 9.7 years) operated on for tetralogy of Fallot, underwent exercise testing 5.

Cardiorespiratory exercise performance after Senning operation for transposition of the great arteries.

Objective: To assess the cardiorespiratory response to graded exercise in patients after the Senning operation for transposition of the great arteries.

Design: Cross sectional study. Exercise performance was assessed by determination of the ventilatory anaerobic threshold.

Apparent Greig cephalopolysyndactyly and sinus node disease.

We present the clinical findings and follow-up data from birth to 10.5 years in a boy with Greig cephalopolysyndactyly who, in addition, presents sinus node disease ("sick sinus syndrome"). The significance of the concurrence of Greig cephalopolysyndactyly syndrome, an autosomal dominant condition mapped at 7p13, and sinus node disease is discussed.

Abolishment of chronic volume overload. Implications for diastolic function of the systemic ventricle immediately after Fontan repair.

Background: In patients with a univentricular heart, the chronic ventricular volume overload is acutely abolished by the creation of a Fontan circuit. This results in an immediate reduction of the ventricular cavity size but also in an inappropriate degree of ventricular wall thickness, at least in the early postoperative period. The implications for the diastolic properties of the ventricle are poorly understood.