Tetralogy of Fallot with aortopulmonary window

105

References 1 Vijayaragbavan G, Cherian G, Krishnaswami S, Sukumar IP. Left ventricular endomyocardial fibrosis in India. Br Heart J 1977;39:563-568. 2 Vijayaraghavan G, Davies J, Sadanandan S. Spry GJF, Gibson DG, Goodwin JF. Echocardiographic features of tropical endomyocardial disease in South India. Br Heart J 1983;50:450-459. 3 Eid Fawzy M, Ziady G, Halim M, Guindy R. Mercer EN. Feteih N. Endomyocardial fibrosis: report of eight cases. J Am Co11 Cardiol 1985:5:983-988. 4 Leak AM, Millar-Craig MW. Ansell BM. Aortic regurgitation in seropositive juvenile arthritis. Ann Rheum Dis 1981;40:229-234. 5 Doyle DR. McCurley TL, Sergent JS. Fatal polymyositis in D-penicillamine-treated rheumatoid arthritis. Ann Intern Med 1983;98:327-330.

International Elsevier

Journal

of Cardiology,

18 (1988) 105-108

IJC 06424

Tetralogy of Fallot with aortopulmonary

window

Shyam Sunder Kothari, Meera Rajani and Savitri Shrivastava Departments

of Cardiology and Radiology, AN India Institute (Received

29 December

1986; revision

of Medical Sciences, New Delhi, India

accepted

3 September

1987)

A patient with tetralogy of Fallot associated with aortopulmonary window has been documented. The association was found in one case out of 350 consecutive cases of Fallot’s tetralogy evaluated cineangiographically. Since tetralogy of Fallot may be completely masked by the aortopulmonary window it is suggested that a right ventricular angiogram should be obtained in all patients with an aortopulmonary window. Key

words:

Tetralogy

of Fallot;

Aortopulmonary

window

Introduction Aortopulmonary

window

between

the ascending

coexists

with tetralogy

Correspondence

0167-5273/88/$03.50

is an uncommon

cardiac

defect

consisting

of a communication

and the main pulmonary artery. Aortopulmonary window rarely of Fallot. We report a patient with this rare combination of lesions.

aorta

to: Dr. S. Shrivastava,

Dept. of Cardiology,

0 1988 Elsevier Science Publishers

AIIMS.

New Delhi-110029,

B.V. (Biomedical

Division)

India

106

Case Report A six-year-old male child was referred to our institute because of exertional dyspnoea. He was the product of a full-term normal delivery and was reported to have rapid breathing, prominent precordial pulsations and repeated chest infections since early infancy. There was no history of cyanosis. Examination revealed a 12 kg acyanotic child without congestive heart failure. There was mild cardiac enlargement. The first sound was normal and the second heart sound was narrowly split with a loud pulmonary component. A grade IV/VI long ejection

Fig. 1. Aortic root angiogram

in left anterior

oblique

view showing

aortopulmonary

window.

107 TABLE

1

Haemodynamic

data. Saturation

Pressure

(%I)

(mm Hg)

Fig. 2. Right

ventricular

73 13 85 93 92

a-3, v-l (0) 110/5-7 110/70 (84) 110/12 110/70 (84)

Right atrium Right ventricle Pulmonary trunk Left ventricle Aorta

angiogram

in right

stenosis

anterior

and ventricular

oblique

view showing

septal defect.

infundibular

pulmc

Jnary

108

systolic murmur along the left sternal border and a short apical mid diastolic murmur were present. Evidence of mild cardiomegaly, increased pulmonary vascularity. a prominent ascending aorta and pulmonary trunk were present in the thoracic roentgenogram. The aortic arch was left sided. A mean frontal plane QRS axis of + 110 O; left atria1 overload and biventricular hypertrophy were seen in the electrocardiogram. The clinical diagnosis was that of an aortopulmonary communication with left-to-right shunt and hyperkinetic pulmonary arterial hypertension. At cardiac catheterisation. the right heart catheter could not be negotiated into the pulmonary arteries. The left heart catheter, however, entered the pulmonary trunk from the ascending aorta. Oximetry revealed a 12% step-up from the right ventricle to pulmonary arteries. The systolic pressures in the right ventricle, pulmonary trunk, left ventricle and aorta were identical (Table 1). The aortic angiogram in left anterior oblique view (Fig. 1) identified the aortopulmonary window while the left ventricular angiogram in left anterior oblique showed a large perimembranous ventricular septal defect. As there was difficulty in entering the pulmonary trunk antegradely, a right ventricular angiogram in the right anterior oblique view was performed and outlined the underlying infundibular stenosis (Fig. 2). The parents did not agree to operative repair. Comments The rare association of aortopulmonary window with tetralogy of Fallot serves as a natural palliative shunt. The large left-to-right shunt through the aortopulmonary window masks the effects of pulmonary stenosis, as in our case. This fact was also commented upon by Tandon et al. [l] while describing the autopsy findings of a similar case. We have encountered this combination only once amongst the 350 cases of tetralogy of Fallot and six cases of aortopulmonary window studied. Castaneda and Kirklin [2] emphasized the rarity of this association while reporting the surgical repair of two cases. Clarke and Richardson [3], in their surgical experience of seven patients with aortopulmonary window, found two patients with tetralogy of Fallot, both with a right aortic arch. Blieden and Moller [4], reporting their experience with 17 patients with aortopulmonary window, indicated the association with tetralogy of FaIlot in one case. The patients have severe pulmonary arterial hypertension in spite of the association with tetralogy of Fallot. As such, even though they have a natural palliation, early corrective surgery is mandatory to prevent the development of pulmonary vascular obstructive disease. There is no means clinically to suspect the association of tetralogy of Fallot in a patient with findings of aortopulmonary shunt. Even at cardiac catheterisation, the haemodynamic data alone do not identify the right ventricular outflow obstruction. The pressures in the right ventricle, aorta and pulmonary trunk may be identical and the high aortic saturation masks the right-to-left shunt. A right ventricular angiogram, however, identifies the co-existence of tetralogy of Fallot. In view of this association, right ventricular angiography should be performed in all patients with aortopulmonary window.

References 1 Tandon R, D’Silva,

CL, Moller JH, Edwards JE. Aorticopulmonary septal defect coexisting with ventricular septal defect. Circulation 1974;50:188-191. 2 Castaneda AR, Kirklin JW. Tetralogy of Fallot with aorticopulmonary window: Report of two surgical cases. J Thorac Cardiovasc Surg 1977;74:467-478. 3 Clarke CP. Richardson JP. The management of aortopulmonary window. Advantages of transaortic closure with a dacron patch. J Thorac Cardiovasc Surg 1976;72:48-51. 4 Blieden LC, Moller JH. Aortopulmonary septal defect. Br Heart J 1974:36:630-635.