No abstract

We report two female patients, one aged four years and the other a newborn, referred for evaluation of cyanosis with otherwise normal cardiovascular findings, who proved to have the right superior caval vein draining into the morphologically left atrium. In both patients, the diagnosis was made by color flow mapping. The older child underwent catheterization and subsequent successful surgical correction. A right superior caval vein draining into the left atrium, although very rare, should always be considered a diagnostic possibility in the presence of cyanosis and normal clinical findings. Color flow mapping is an excellent method with which to make the diagnosis.

Hypertrophic cardiomyopathy in the young is characterized by referral for murmur or heart failure; frequent secondary forms; the obstructive variant being as common as the non-obstructive form; a mortality rate similar to that for adults attending tertiary centres; and less frequent familial forms than in older populations.

BACKGROUND: Permanent dual-chambered pacing (DDD) is an alternative to surgical treatment in patients with severe hypertrophic obstructive cardiomyopathy (HOCM) who do not have a satisfactory response to medical treatment. METHODS: Five children with severe HOCM still symptomatic despite medical treatment underwent permanent DDD pacing and were followed for 21 +/- 9.7 months. RESULTS: All patients improved their functional class. Doppler echocardiographic studies showed an early reduction of the left ventricular outflow tract gradient from 66 +/- 40 to 40 +/- 20 mmHg (P < 0.05) and to 30 +/- 11 mmHg (P < 0.05 and NS for comparison with the baseline and the early post-DDD pacing gradients, respectively) at mid-term follow-up. There was no evidence of left ventricular systolic dysfunction, and the results of left ventricular filling studies ruled out deleterious effects on diastolic function. Doppler echocardiography played a key role in the initial and subsequent assessment of these patients. CONCLUSIONS: Permanent DDD pacing is a reasonable alternative to surgery in children with HOCM who are still symptomatic despite medical therapy.