Download A case of Tetralogy of Fallot with left coronary artery to pulmonary

The official Journal of Cardiological Society of India, Kerala Chapter
URL: http://keralaheartjournal.in/ojs/index.php/KHJ
Case Report
A case of Tetralogy of Fallot with left coronary
artery to pulmonary artery fistula
Sajeer KT, Rajesh G*, Fazil Bishara, Rakesh Jain
Govt. Medical College, Kozhikode, Kerala India
*Address for Correspondence: Dr. Sajeer KT, MD, DM, Senior Resident, Dept.of Cardiology, Govt. Medical College,
Kozhikode, Kerala India. Email: [email protected]
URL: http://keralaheartjournal.in/ojs/index.php/KHJ/article/view/58
Abstract:
Key words :
The congenital coronary artery to pulmonary artery fistula
is a rare anomaly. It is often reported in patients with ventricular
septal defect and pulmonary atresia. Herein we report a case of left
anterior descending coronary artery to pulmonary artery fistula in
a patient of tetralogy of Fallot.
Tetralogy of Fallot, fistula, coronary artery, pulmonary artery, pulmonary atresia
Case report:
ventricular septal defect patch closure, infundibular resection, pulmonary valvotomy and right ventricle outflow
tract augmentation with patch. Fistulous communication
between left coronary artery and pulmonary artery was
ligated. Post-operative period was uneventful and discharged on day 7.
An 8-year old girl presented with cyanosis since 1
year of age and dyspnea on exertion NYHA functional
class II. She had history of cyanotic spells during infancy.
On examination her pulse rate was 86 per minute with
blood pressure of 100/70 mmHg. There was cyanosis, pan
digital clubbing and polycythemia (hematocrit of 68%).
Cardiovascular system examination revealed normal first
heart sound, single second sound and continuous murmur
best heard in left 3rd intercostal space in the parasternal
area. Chest X-ray showed decreased pulmonary blood flow
with right ventricular type of apex, concave pulmonary
bay and a prominent aortic knuckle. Electrocardiogram
revealed sinus rhythm with features of right ventricular
hypertrophy and right axis deviation. The transthoracic echocardiography showed large sub aortic ventricular
septal defect with 60 % aortic override, right ventricular
hypertrophy with severe infundibular and pulmonary
valve stenosis. Cardiac catheterization and angiography
study confirmed the diagnosis of tetralogy of Fallot with
large ventricular septal defect, right ventricular hypertrophy and aortic override. There was only faint filling of
pulmonary artery on right ventricular injection (Figure
1A). The aortic root injection showed presence of large
communication from left coronary system to pulmonary
trunk. Selective left coronary injection confirmed presence of fistulous communication arising from left anterior
descending coronary artery to the main pulmonary artery
(Figure 1B). Patient underwent intra-cardiac repair with
Kerala Heart Journal
Volume - 5
Issue - 2
A
Minimal pacification of pulmonary artery (PA)
37
The official Journal of Cardiological Society of India, Kerala Chapter
fistulous communication, to ensure unhindered coronary
arterial flow, to close the ventricular septal defect with relief of right ventricular outflow obstruction, and to establish unobstructed continuity between the right ventricle
and the intra-pericardial pulmonary arteries.4
B
Conclusion:
The surgical importance of coronary artery to pulmonary artery fistula lies in the fact that the communication needs to be identified and ligated before commencing
cardiopulmonary bypass to prevent loss of volume to pulmonary vascular bed. Failure to close such communications prior to application of aortic cross clamp results in
loss of cardioplegic solution to pulmonary circulation and
may result in incomplete myocardial protection during
administration of cardioplegia.5
References:
1.
Fistula between left anterior descending coronary artery
to pulmonary artery. Ao -aorta, PA-pulmonary artery,
LAD -left anterior descending artery
Lowe JE, Louis JD, Sabiston DC. Congenital lesions of
the coronary circulation. In: Sabiston DC Jr, Lyerly HK.
Textbook of surgery: The Biological Basis of Modern Surgical practice. 15th ed. Philadelphia, PA: Saunders; 1997. p.
2124‑35.
2.
Discussion:
Amin Z, McEllinney DB, Reddy VM, Moore P, Henley FL,
Teitel DF. Coronary to pulmonary artery collateral in patients with pulmonary atresia and ventricular septal defect.
Ann Thorac Surg 2000; 70:119‑23.
3.
Dabizzi RP, Caprioli G, Aiazzi L, Castelli C, Baldrighi G,
Parenzan L, et al. Distribution and anomalies of coronary
arteries in tetralogy of Fallot. Circ 1980;61:95‑102.
4.
Sachin Talwar, Robert H Anderson, Vikas Kumar Keshri,
Shiv Kumar Choudhary, Gurpreet Singh Gulati, Balram
Airan. Coronary-pulmonary artery fistula in tetralogy of
Fallot with pulmonary atresia. Asian Cardiovasc Thorac
Ann 2009; 17:304‑6.
5.
Chander Mohan Mittal, Bishav Mohan, Rajiv Kumar, Sheetal Garg. A case of tetralogy of Fallot associated with left
anterior descending coronary artery to pulmonary artery
fistula. Ann Pediatr Cardiol. 2011; 4(2): 202-203.Fistula
between left anterior descending coronary artery to pulmonary artery. Ao-aorta, PA-pulmonary artery, LAD -left
anterior descending artery.
The congenital coronary artery to pulmonary artery
fistula is a rare anomaly, reported to occur commonly in
patients with ventricular septal defect and pulmonary
atresia with an incidence of 10%.1,2 Failure of obliteration
of the intramyocardial trabecular sinusoids with anomalous development of the intra-trabecular spaces, through
which blood is supplied to the myocardium during intrauterine life results in congenital coronary to pulmonary
artery fistula. Reports of large coronary artery to pulmonary artery communication are very rare in tetralogy
of Fallot.3 Physiologically coronary artery to pulmonary
communications in tetralogy of Fallot helps in increasing
pulmonary blood flow and reduction in degree of cyanosis.
The essential goals of surgical correction are to close the
Please cite this article as: Sajeer KT, et al., A case of Tetralogy of Fallot with left coronary artery to pulmonary artery fistula.
Kerala Heart J 2015; 5(2):36-37.
Kerala Heart Journal
Volume - 5
Issue - 2