Ann Thorac Surg 1995;60:706-708
© 1995 The Society of Thoracic Surgeons
Case Report
Origin of Both Coronary Arteries From the Pulmonary Artery and Aortic Coarctation
Giuseppe Santoro, MD,
Duccio di Carlo, MD,
Adriano Carotti, MD,
Roberto Formigari, MD,
Renata Boldrini, MD,
Cesare Bosman, MD,
Luigi Ballerini, MD
Departments of Pediatric Cardiology, Cardiac Surgery, and Pathology, Ospedale Bambino Gesù, Rome, Italy
Accepted for publication March 8, 1995.
 |
Abstract
|
|---|
Single trunk anomalous origin of both coronary arteries from the pulmonary artery is a rare congenital cardiac anomaly. We report on 2 cases of its association with aortic coarctation, the diagnosis of which in living patients is very difficult. We think that the possibility of this anatomic arrangement should always be considered in patients with isolated aortic coarctation whose clinical condition seems impaired rather than improved after an apparently successful coarctectomy.
|
Introduction
|
|---|
Single trunk anomalous origin of both coronary arteries from the pulmonary artery is a rare malformation [1, 2] in which the entire myocardial blood supply derives from the pulmonary artery. This anomaly is incompatible with life except in the presence of high pulmonary arterial oxygen saturation or pressure to provide adequate myocardial perfusion. Diagnosis has been usually made on autopsy, and only a few cases have been identified during life because of associated malformations [3].
In this article we report on 2 cases of association of anomalous origin of both coronary arteries by a single trunk from the pulmonary artery and isolated aortic coarctation.
|
Case Reports
|
|---|
Patient 1
A 1-month-old infant was referred for cardiologic examination because of failure to thrive and congestive heart failure. Electrocardiography showed sinus tachycardia but did not show ST-T ischemic changes suggestive of anomalous coronary artery origin. Echocardiography showed a severe aortic coarctation and mild global hypokinesia of the left ventricle; the patient underwent urgent coarctation repair by subclavian flap operation. The postoperative course was complicated by difficulty of weaning from mechanical ventilation. Repeat echocardiography showed deteriorated left ventricular function, and eventually the infant died of intractable ventricular failure. On autopsy an anomalous origin of both coronary arteries from pulmonary artery via a single trunk was diagnosed.
Patient 2
A 2-month-old infant was admitted in congestive heart failure after a coarctation repair performed in another institution 15 days before. Clinical examination revealed hepatomegaly and bilateral pulmonary rales. Electrocardiography showed sinus tachycardia (180 beats/min), and diffuse ischemic ST-T changes. On echocardiography an extremely poorly functioning left ventricle was seen, without evidence of residual obstruction at the site of previous coarctation repair. Cardiac catheterization was undertaken, which showed an anomalous origin of both coronary arteries from the right pulmonary artery via a single trunk (Fig 1
). Surgical correction with reimplantation of the coronary arteries and right pulmonary artery pericardial patch reconstruction was performed, but the infant could not be weaned from cardiopulmonary bypass and died in the operating room. Autopsy confirmed the angiocardiographic diagnosis and showed fibroelastosis and severe ischemic changes of the myocardium. The coronary ostium was patent at the reimplantation site.
View larger version (267K):
[in this window]
[in a new window]
|
Fig 1. . Pulmonary angiography in anteroposterior (A) and lateral (B) views, showing the anomalous origin of the single trunk coronary artery (arrow).
|
|
|
Comment
|
|---|
Single trunk anomalous origin of both coronary arteries from the pulmonary artery is a rare cardiac malformation, often diagnosed only on autopsy. It may occur as an isolated anomaly or in association with other cardiac malformations, which may influence the time of onset of symptoms and the clinical course. The prognosis is better in patients with associated cardiac malformations with increased pulmonary artery oxygen saturation or pressure [3, 5, 8] than in those with isolated anomaly. In case of associated malformations, the diagnosis is difficult because the clinical and electrocardiographic pictures are dominated by the associated lesions. Several cardiac anomalies have been described in association with the anomalous origin of both coronary arteries from the pulmonary artery [4–9]. In the association with isolated aortic coarctation, the coronary artery perfusion strictly depends on the degree of pulmonary hypertension which, in turn, is correlated with aortic coarctation in the neonatal age. In fact, in our patients, a sudden drop of left ventricular afterload after coarctectomy must have resulted in a lower pulmonary artery pressure and reduced coronary artery perfusion.
In our institution, cardiac catheterization and angiography are not routinely performed in the preoperative evaluation of patients with isolated aortic coarctation, even in the presence of impaired left ventricular function. In our experience, a mild-to-moderate left ventricular hypokinesia is not unusual in simple coarctation, and it is found to improve after relief of the aortic obstruction. The postoperative course of our patients, conversely, was characterized by clinical and instrumental evidence of deterioration of the myocardial function. After the first case, which remained undiagnosed until autopsy, the correct diagnosis was suspected in the second patient and cardiac catheterization confirmed it.
In conclusion, the possibility of coronary artery anomaly should be considered in patients with isolated aortic coarctation whose clinical condition seems aggravated, rather than improved, by an apparently successful coartectomy. This course of events in the neonatal period or early months of life should suggest the anomalous origin of both coronary arteries. Early diagnosis is essential to avoid the occurrence of ventricular fibroelastosis and irreversible myocardial failure, which preclude surgical repair.
|
Footnotes
|
|---|
Address reprint requests to Dr di Carlo, Department of Cardiac Surgery, Ospedale Bambino Gesù, P.zza S. Onofrio, 4, 00165 Rome, Italy.
|
References
|
|---|
- Roberts WO. Anomalous origin of both coronary arteries from the pulmonary artery. Am J Cardiol 1962;10:595–9.[Medline]
- Heifetz S, Rabinowitz M, Mueller K, Virmani R. Total anomalous origin of the coronary arteries from the pulmonary artery. Pediatr Cardiol 1986;7:11–6.[Medline]
- Goldblatt E, Adam APS, Ross IK, Savage JP, Morris LL. Single trunk anomalous origin of both coronary arteries from the pulmonary artery: diagnosis and surgical management. J Thorac Cardiovasc Surg 1984;87:59–65.[Abstract]
- Colmes RA, Sideris CI. Anomalous origin of both coronary arteries from the pulmonary trunk. Myocardial infarction in otherwise normal heart. Am J Cardiol 1963;12:263–9.[Medline]
- Feldt RH, Ongley PA, Titus JL. Total coronary arterial circulation from pulmonary artery with survival to age seven. Report of a case. Mayo Clin Proc 1965;40:539–43.[Medline]
- Ogden JA. Origin of a single coronary artery from the pulmonary artery. Am Heart J 1969;78:251–3.[Medline]
- Monselise MB, Vlodaver Z, Neufeld HW. Single coronary artery. Origin from the pulmonary trunk in association with ventricular septal defect. Chest 1970;58:613–6.[Abstract/Free Full Text]
- D'Alessandro LC, Di Lorenzo M. Coronaria unica con origine dall'arteria polmonare sinistra in un uomo di 39 anni ancora vivente, affetto da ``truncus arteriosus communis.'' G Ital Cardiol 1976;6:939–45.
- Sennari E, Sato Y, Matsuoko Y, Yamamoto K, Okishima T, Hayakawa K. A case report of anomalous origin of a single coronary artery from the pulmonary artery associated with multiple cardiac malformations. Jpn Circ J 1982;46:329–33.[Medline]
Top
Abstract
Introduction
