Anomalous origin of left coronary artery from the pulmonary artery with ventricular septal defect.

Abstract

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 ...

Two-dimensional echocardiographic visualization of the left coronary artery in anomalous origin of the left coronary artery from the pulmonary artery. Pre- and postoperative studies.

“…'0' 11 In those even rarer patients with associated intracardiac defects, findings are masked by those of the associated lesion. '2-14 This lesion has a very high mortality in untreated cases.10' 11 13 Early diagnosis and definitive surgery before the occurrence of extensive myocardial damage appear to be the main hope for long-term survival.9' 10,12 In support of this view are reports documenting remarkable improvement in left ventricular function after successful surgery in a few infants. 9' 15, 17 Definitive diagnosis depends on aortography, which carries a significant risk in these sick infants.…”

Anomalous left coronary artery from the pulmonary artery discovered following total anomalous pulmonary venous return repair: a rare entity

“…pulmonary artery is usually a solitary cardiac lesion; however, it has been reported to occur in association with a variety of other cardiac malformations including patent ductus arteriosus, ventricular septal defect, coarctation of the aorta, and tetralogy of Fallot. [1][2][3][4][5][6] We report a case of anomalous left coronary artery from the pulmonary artery that was diagnosed by echocardiography after repair of the total anomalous pulmonary venous return.…”

Congenital Anomalies of the Coronary Arteries

“…It occurs approximately once per 300 000 live births and represents 0.5% of cases of congenital heart disease [18]. It generally occurs as an isolated anomaly but has been associated with patent ductus arteriosus, ventricular septal defects, atrioventricular canal defects, tetralogy of Fallot, coarctation of the aorta, and truncus arteriosus [19][20][21][22][23][24][25].…”