The treatment of paroxysmal supraventricular tachycardia (PSVT) in infancy with digitalis, adenosine triphosphate (ATP) and verapamil is reported. Treatment was successful in about 90% of the patients treated with ATP and verapamil and in 61--71% of the patients treated with digitalis (Lanatoside C). Verapamil terminated the tachycardia within 2 minutes of administration in most instances and ATP in less than 1 minute. Digitalis, however, took as long as 2 hours; it was therefore excluded as the drug of first choice in emergencies, and is better suited for treating patients with poor hemodynamics. Side effects with ATP are common but short-lived. With verapamil, side effects are rare, but may be serious if certain contraindications are not taken into account. Digitalis in the dose used in this trial rarely produced side effects. We conclude that ATP or verapamil is the drug of first choice for quick termination of PSVT in infancy.
A case of criss-cross heart is presented in which there was situs solitus with atrioventricular (AV) discordance and single outlet, the aorta arising from the right ventricle. Associated lesions were pulmonary atresia and ventricular septal defect. The apparently normal AV relation in the presence of a discordant AV connection can be explained by the hypothesis that an embryonic l-bulboventricular loop developed and was followed by marked counter-clockwise rotation of the ventricles.
SUMMARY A case of isolated atrioventricular discordance in situs solitus is described.The importance of a well defined situs and of the presence of both ventricles in making this diagnosis is underlined and previous published reports reviewed.It is suggested that the haemodynamic concept of isolated atrioventricular discordance cannot be described only by the spatial relation between the ventricles (isolated ventricular inversion), but it should be indicted by the pattern of the connection between various flows, as shown by hearts with a normal relation between the ventricles and crossed atrioventricular connection.We consider that "A-V discordance with ventriculoarterial concordance and normally related great arteries"' is the best way to describe the anatomical and the haemodynamic pattern in so called isolated ventricular inversion.In this paper we report a new example of this rare anomaly which we call "isolated atrioventricular discordance". To our knowledge, this is the first reported case in which a straddling left atrioventricular valve was present.
Case reportA 2-month-old female infant (birthweight 2-5 kg) was admitted with dyspnoea and cyanosis which had been present from birth.A xiphoid impulse was present and a grade 3/6 harsh early and mid-systolic murmur was heard at the left lower sternum; the second heart sound was normal. The peripheral pulses were diminished and the liver was much enlarged. Chest x-ray film showed a left cardiac apex, cardiomegaly, and increased pulmonary flow. The electrocardiogram showed normal sinus rhythm, left atrial enlargement, and a QRS axis at + 1400 in the frontal plane. An Laevocardia and solitus visceroatrial situs were present, with normal atrial morphology and the normal relation between the atrial chambers. Systemic and pulmonary connections were normal. The atrioventricular connection was discordant with two separated atrioventricular valves, the left of which was straddling. The right atrium was connected to a right sided and morphologically left ventricle through a bicuspid valve (Fig. IA). The subvalvular apparatus was normal. A normal-sized ventricle was connected to a right sided and posterior aorta (Fig. 1B). Fibrous continuity between the anteromedial leaflet of the right atrioventricular valve and the aortic cusps was documented. A fibromuscular membrane obstructing the aortic outflow tract was present (Fig. IB). The ventricular septum was abnormally orientated, so that its posterior aspect was to the left of its anterior aspect; a perimembranous ventricular septal defect was present (Fig. IB). Some chordae from the left atrioventricular valve had their insertion on the base of the septal defect on its right side. The left atrium was connected to a left sided, morphologically right ventricle through a tricuspid valve (Fig. 2A). The normal sized atrioventricular valve annulus was shifted medially and to the right but did not override the ventricular septum. The anterior leaflet of the left tricuspid atrioventricular valve had its insertion on the anter...
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