Anomalous origin of left coronary artery from pulmonary artery associated with an aorto-pulmonary septal defect

Abstract

Aorto-pulmonary septal defects and anomalous origins of left coronary artery from pulmonary artery are briefly discussed. The difficulties of diagnosis and surgical correction are put forward in a situation when the left coronary artery arises from a site of high pressure and saturation, such as the pulmonary artery in the presence of an aorto-pulmonary septal defect, as in the case described.This report describes the clinical, haemodynamic, angiographic, and necropsy findings in an infant with anomalous origin of the left coronary artery from the pulmonary artery, small ventricular septal defect, aorto-pulmonary septal defect, right aortic arch, and left brachiocephalic trunk. We have been unable to find a report of a similar combination of defects, though the brief details are recorded of a haemodynamically comparable patient with aorto-pulmonary septal defect, right aortic arch, and anomalous origin of the right coronary from the pulmonary artery which was corrected successfully by surgery (Morrow, Greenfield, and Braunwald, I962). Case reportThe patient, the only child of healthy parents, was a spontaneous normal hospital delivery at 42 weeks' gestation following an uneventful pregnancy, birthweight 3,2oo kg. There was no difficulty in onset of respiration. He was noted to be tachypnoeic from birth and fed poorly, though there was no other evidence of heart failure, and no heart murmur was heard. He was admitted to another hospital at the age of 2 months with probable cyanosis, a grade 3/6 systolic murmur, and congestive heart failure. The latter responded slowly to medical treatment, and he was discharged two months later on no maintenance therapy. He failed to thrive, developed a persistent cough, and was referred to Brompton Hospital at the age of 6 months. On examination he was an ill infant with mild central cyanosis on crying. The length and weight were both below the third percentile. He had grunting respiration (rate 6o/ min.), with conspicuous indrawing of both sides of the chest, right more than left, and suprasternal retraction. The peripheral pulses were 'waterhammer' in type, blood pressure go/50 mm.Hg. The heart was overactive and the second sound was single. There was a grade 3/6 long ejection murmur best heard at the upper left sternal border and no diastolic murmur. The lungs were normal on auscultation. The liver edge was felt 2-3 cm. below the right costal margin. No other abnormality was detected on routine clinical examination, except for a mild degree of hypospadias and shovel pinnae. Relevant investigations were: femoral arterial oxygen saturation 88 per cent; chest x-ray showed cardiomegaly with pulmonary plethora; electrocardiogram showed biventricular and biatrial hypertrophy with a mean frontal plan QRS axis of + 700 with clockwise rotation.Cardiac catheterization via the right axillary route was performed under local anaesthesia (see Table). The right ventricular pressure was at systemic level. There was a small left-to-right shunt at

Fatal outcome of congenital aortopulmonary window with patent ductus arteriosus complicating pregnancy

“…3,9 Other associated anomalies include patent ductus arteriosus, coronary artery anomalies, ventricular septal defect, atrial septal defect, tetralogy of Fallot, and transposition of great arteries. [10][11][12] In a series of 42 pediatric cases of APW, 16 cases (38%) had associated patent ductus arteriosus. 11 APW with PDA first presenting in adult has been rarely reported in only one case report, to the best of our knowledge.…”

Diagnosis, Treatment and Outcomes of Patients with Aortopulmonary Window

“…It is reported that coronary artery anomalies accompany 5-10% of cases (14,15). Diagnosis of these patients in the preoperative period may be challenging.…”

Aortopulmonary window. Clinical and surgical assessment of 18 cases

“…Anomalous origin of the coronary arteries can occur in 5 to 10% of the cases of APW 30,31 . Pre-operative diagnosis is usually difficult due to the absence of clinical manifestations of ischemia, since a high perfusion pressure secondary to the APW leads to a good coronary artery flow.…”

“…In the presence of associated lesions, its accuracy decreases 41 . APW should always be considered when the left atrium is very dilated or there is severe pulmonary hypertension 30 . The diagnosis by 2DE should be made by visualizing the aortopulmonary septum in two or more different planes.…”