SUMMARY Only two cases have been reported previously of the association of ventricular septal defect (VSD) with anomalous origin of the left coronary artery (ALCA) arising from the pulmonary artery. The purpose of this paper is to present two additional cases, to describe the pathophysiology, and to emphasize how the clinical course of this combination of defects differs from that of isolated ALCA.Patients with both of these anomalies present in infancy with manifestations only of a large left-right ventricular shunt and SINCE ANOMALOUS ORIGIN OF THE LEFT CORONARY ARTERY (ALCA) from the pulmonary artery was described by Abbot in 19081 and the natural course further characterized by Bland, White and Garland in 1933,2 numerous reports have been published describing symptoms and modes of therapy.3 Associated congenital cardiac anomalies are extremely rare. Since many cardiac centers are repairing ventricular septal defects in infants when medical management is not satisfactory, then it becomes important to properly identify associated defects. To the best of our knowledge, there are only two published cases concerning the association of ALCA with ventricular septal defect (VSD).78It is the purpose of this paper to report two additional cases of ALCA associated with VSD, to describe the hemodynamics, and to emphasize the difference in the clinical course of ALCA, both with, and without additional congenital cardiac defects. Patient MaterialCase I T.S. was brought to the emergency room at 11 weeks of age because of poor feeding, tachycardia, and tachypnea. On examination she was underdeveloped, irritable, and acyanotic. The respiratory rate was 50 per minute and the heart rate was 140 beats per minute. The first heart sound was normal but the second sound was single and of increased intensity. There was a plateau, III/VI holosystolic murmur at the left lower sternal border with radiation to the base and to the posterior chest wall bilaterally. There was a short, low-frequency mid-diastolic murmur at the apex. The liver was enlarged 3 cm below the right costal margin. The chest radiograph demonstrated cardiomegaly with increased pulmonary arterial markings. The electrocardiogram demonstrated biventricular hypertrophy ( fig. la). There were no electrocardiographic changes suggestive of ALCA. Digitalis and diuretics were administered with prompt improvement. The clinical impression was VSD. Cardiac catheterization six days later revealed a VSD. There was a significant increase in oxygen saturation in the right ventricle, with no further increase in the pulmonary artery. Other hemodynamic data are listed in table 1.During the next six months, the patient had numerous episodes of unexplained irritability, crying and tachycardia. However, the systolic murmur decreased in intensity and the diastolic murmur disappeared. Thus, despite auscultatory evidence of decrease in size of the VSD, the patient continued to have symptoms of distress and cardiomegaly by chest radiograph. There was diminution of the R wave in the anterior ...
Cardiovascular malformations were produced in 17% of A/J and C57BL/6 mice using isogeneic antiheart antibody (RAMHS) and in 22% of C57BL/6 mice using allogeneic RAMHS by injecting on four consecutive days of gestation (days 8–11 or 12–15). The antiheart antibody was raised in rabbits and adsorbed against mouse kidney and liver to yield an antiserum with high antiheart activity and low activity against other organs, demonstrably kidney. Cardiovascular maldevelopment occurred in 9% of mice injected with antikidney antibody (RAMKS), 7% receiving normal rabbit serum, and 1% receiving normal saline. A highly significant (P < .001) difference between all of the RAMHS and the normal saline groups demonstrated that antiheart antibody is teratogenic to the developing heart; and a significant difference (P < .05) between allogenic RAMHS and RAMKS antibody in the frequency of induction of cardiovascular anomalies suggests that there is organ‐specific activity to antiheart antibody. The type of cardiovascular anomalies caused in the mice studied were the same (VSD in C57BL and ASD in A/J) as those that occur spontaneously or are produced by other teratogens such as dextroamphetamine. Antiheart antibody thus did not appear to produce a specific type of maldevelopment but rather made manifest the malformation to which there appears to be a hereditary predisposition (genetic‐environmental interaction).
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