Aortico-left ventricular tunnel is a rare congenital anomaly that presents as aortic regurgitation and congestive heart failure in childhood. Its association with a ventricular septal defect is even more rare. Because of the distortion of the normal anatomy around the aortic valve and the rarity of this combination of defects, the diagnosis of aortico-left ventricular tunnel with ventricular septal defect may be difficult. The two-dimensional and Doppler echocardiographic findings of aortico-left ventricular tunnel are described.
Three infants developed greatly accelerated junctional ectopic tachycardia with a heart rate greater than 200 beats/min after open heart surgery. When the heart rate exceeded 200 beats/min for 5 hours, all the infants had congestive heart failure and clinical signs of low cardiac output. Conventional therapy (cardioversion, lidocaine, verapamil, digoxin and ice to face) has been shown in the past to be unsuccessful in controlling the heart rate. Because hypothermia is known to decrease automaticity of the heart, these patients were treated with induced hypothermia. The goal was to arbitrarily decrease the junctional ectopic rate to less than 180 beats/min to increase cardiac filling time. The duration of the junctional ectopic tachycardia greater than 180 beats/min ranged from 0.5 to 17 hours after cooling began. The duration of the hypothermia ranged from 4 to 24 hours. Spontaneous reversion to sinus rhythm occurred either during the hypothermia or shortly thereafter in all three patients. The blood pressure and urinary output remained stable during hypothermia. Hypothermia is an effective means of controlling the rate of greatly accelerated junctional ectopic tachycardia after open heart surgery in infants. Although hypothermia does not convert junctional ectopic tachycardia to sinus rhythm, it slows the rate to a more acceptable level, allowing the infants' survival and eventual recovery of sinus rhythm.
Two-dimensional echocardiography can diagnose hypoplastic left heart syndrome. However, with the advent of the possibility of palliative open heart surgery, complete anatomic diagnosis is necessary. The anatomic findings of 15 neonates with hypoplastic left heart syndrome (age 1 to 10 days, mean 4.1) who had two-dimensional Doppler echocardiographic studies were compared with the results obtained by angiography (6 cases), surgery (11 cases) and autopsy (8 cases). Complete two-dimensional echocardiographic examination of the aortic arch, pulmonary and systemic venous return, atrial septum, ductus arteriosus and proximal coronary arteries was possible in all 15 neonates and correctly diagnosed hypoplastic left heart syndrome in each. Anatomic two-dimensional echocardiographic assessment was accurate in 13 (86%) of the 15 neonates and there were no false positive results. Undiagnosed associated abnormalities were hypoplasia of a left pulmonary artery in one patient and left superior vena cava in another. Accurate quantitation of the size of the tricuspid valve anulus, ascending aorta, pulmonary anulus and right and left pulmonary arteries was possible. Doppler examination was performed in seven patients and confirmed retrograde aortic arch flow and right to left systolic shunting in the patent ductus arteriosus. In selected neonates, surgical palliation can be attempted without angiography.
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