International Journal of Cardiology, 33 (1991) 315-317 0 1991 Elsevier Science Publishers B.V. All rights reserved 0167-5273/91/$03.50 ADONIS 0167527391002435

CARD10 13581

Brief Reports

Anomalous origin of the left pulmonary artery from the ascending aorta in a patient with tetralogy

of Fallot and “absent pulmonary valve”

Anita Saxena, Savitri Shrivastava and Sanjeev Sharma Cardiothoracic Centre, All India Institute of Medical Sciences, New Delhi, India

(Received 30 April 1991; accepted 4 May 1991)

315

The rare association of tetralogy of Fallot, rudimentary formation of the leaflets of the pulmonary valve, and anomalous origin of the left pulmonary artery from the ascending aorta is described in a two-month-old infant. The diagnosis was made by cardiac catheterisation and angiography.

Key words: Congenital heart disease; Cardiac catheterization

Introduction

Tetralogy of Fallot is the commonest cyanotic con- genital malformation of the heart. The incidence of so-called “absent pulmonary valve” in association with this lesion ranges from 2.4 to 6.3% [1,2]. The associa- tion of anomalous origin of the left pulmonary artery from the ascending aorta has also been well described [3,4]. As far as we know, however, only two cases have been reported in the English literature where anoma- lous left pulmonary artery origin from the ascending aorta has existed in association with tetralogy of Fallot and “absent pulmonary valve” [4]. We describe another such patient seen by us.

Case Report

A two-month-old infant, the product of a full-term normal pregnancy, presented with difficulty in breath- ing and recurrent episodes of cough and fever since

Correspondence to: Dr A. Saxena, Cardiothoracic Centre, All India Institute Of Medical Sciences, New Delhi, 110029

India.

one week of age. Examination revealed a mildly cyan- otic, 2.5 kg child in gross congestive heart failure. All peripheral pulses were normal. The second heart sound was single and there was a long 3/6 ejection systolic murmur with a 2/6 early diastolic murmur along left sternal border. There were bilateral crepitations over the lung fields. The electrocardiogram showed a right axis deviation of + 120 o and evidence of right ventric- ular hypertrophy. A chest X-ray demonstrated mild cardiomegaly with decreased vascularity of lungs. No disparity in lung vasculature on the two sides could be appreciated. In addition, there was a shadow in the right hilum suggestive of a dilated right pulmonary artery. A provisional diagnosis of tetralogy of Fallot with absent leaflets of the pulmonary valve was made.

Cross-sectional echocardiography and Doppler study done on ATL UM9 machine confirmed the diagnosis of tetralogy of Fallot with absence of the leaflets of the pulmonary valve. The bifurcation of the pulmonary trunk, however, could not be identified. This was made more difficult by the marked dilatation of the right pulmonary artery. The patient subsequently underwent cardiac catheterisation and angiography. The right ven- triclllar systolic pressure was systemic and there was a peak systolic gradient of 45 mmHg across the right

ventricular outflow tract, on withdrawal of the catheter from the right pulmonary artery to the right ventricle.

The left pulmonary artery could not be entered. A right ventricular angiogram demonstrated features of obstruction of the right ventricular outflow tract with absence of the leaflets of the pulmonary valve. The right pulmonary artery was dilated, and there was no evidence of the origin of the left pulmonary artery from the pulmonary trunk. On left ventricular angiog- raphy, a single large perimembranous ventricular sep-

tal defect was visualised. In addition, the left pul- monary artery was seen taking origin from the ascend- ing aorta just beyond the aortic valve (Fig. I). The aortic arch was left sided. On repeat echocardiography, the suprasternal view clearly demonstrated the anoma- lous origin of the left pulmonary artery from the poste- rior aspect of the ascending aorta (Fig. 2). The patient

was subsequently submitted to surgery and the diagno- sis was confirmed.

Fig. 1. Left ventriculogram in left anterior oblique view show-

ing the anomalous origin of the left pulmonary artery (arrow)

from the ascending aorta.

Fig. 2. Cross-sectional echocardiographic suprasternal long axis view of the anomalous left pulmonary artery originating from the

ascending aorta. AA = ascending aorta: LPA = left pulmonary artery.

Discussion

Anomalous origin of the left pulmonary artery from the ascending aorta in patients with tetralogy of Fallot and absence of the leaflets of the pulmonary valve has

been described previously in only two infants, including one case where the diagnosis was established at au-

topsy [4]. According to those authors, the presence of jerky peripheral pulses in a patient with tetralogy of Fallot and an early diastolic murmur of pulmonary regurgitation should suggest this combination. Al- though the early diastolic murmur was heard, jerky peripheral pulses were absent in our patient, presum- ably due to gross congestive heart failure. Another suggestive feature is the presence of asymmetric lung

vascularity on the chest X-ray. Echocardiography is very useful in describing each

anatomic defect. Failure to recognise the bifurcation of the wlmonary trunk should lead to a search for an

anomalous origin of one pulmonary artery from the

aorta. In case the parasternal and subcostal views do

not show the pulmonary arteries clearly, a suprasternal view should be attempted. Recurrent respiratory infec- tions are a major threat to survival in these patients and warrant early surgical correction, although surgical mortality is high [3].

References

Rao BNS. Anderson RC, Edwards JE. Anatomic variations in the tetralogy of Fallot. Am Heart J lY7l:Xl:361-371.

Lev M. Eckner FAO. The pathologic anatomy of tetralogy

of Fallot and its variations. Dis Chest lYh4:45:251-%I.

Robin E. Silberg B, Ganguly S, Magnisalis K. Aortic origin

of left pulmonary artery variant of tetralogy of Fallot. Am J

Cardiol 1975:35:324-329.

Calder AL. Brandt PWT, Barratt-Boyes BG. Neutz JM.

Variants of tetralogy of Fallot with absent pulmonary valve

leaflets and origin of one pulmonary artery from the as-

cending aorta. Am J Cardiol 1 Y80:46: IOh- 116.

lnternutionai Journal of Cardiology, 33 (1991) 317-319 Cl 199 I Elsevier Science Publishers B.V. All rights reserved 0 167.5273/9 I /OOS.SO

ADONIS 0167527391002441

CARD10 13582

Fatal myocardial infarction in hypertrophic cardiomyopathy associated with non-penetrating chest trauma

M.R. Bennett, J.N.W. West and B.L. Pentecost Department of Cardiology, General Hospital, Birmingham, U.K.

(Received 15 March 1491; revision accepted 20 May 1991)

Myocardial infarction as a result of injury to the coronary arteries is a rare complication of non-penetrating chest trauma. We report a case of fatal inferior wall myocardial infarction following traumatic inhn-y to the right coronary artery, complicated by atrioventricular dissociation, in a patient with a combination of hypertrophic cardiomyopathy and non-occlusive coronary artery disease.

Key words: Myocardial infarction; Hypertrophic cardiomyopathy; Chest trauma; Atrioventricular dissociation

Correspondence to; Dr M.R. Bennett. Dept. of Cardiology, University of Wales College of Medicine. Heath Park. Cardiff CF4

4XN, IJ.K.