Asian Cardiovasc Thorac Ann 2007;15:335-338
© 2007 Asia Publishing EXchange Ltd
Pulmonary Atresia, VSD in Association with Coronary-Pulmonary Artery Fistula
Hani K Najm, MD,
Neerod K Jha, MD,
Michael Godman, MD1,
Mansour Al Mutairi, MD1,
Ahmed I Rezk, MD,
Tarek Momenah, MD2
Department of Cardiac Surgery
1 Department of Pediatric Cardiology, King Fahad Hospital
2 Department of Cardiology, Prince Sultan Cardiac Center, Riyadh, Saudi Arabia
For reprint information contact: Neerod K Jha, MD Tel: 966 1 237 9259 Fax: 966 1 252 0088 ext. 6700 Email: nk_jha{at}hotmail.com, Department of Cardiac Surgery, Mail Code 1413, King Fahad National Guard Hospital, PO Box 22490, Riyadh, Saudi Arabia.
 |
ABSTRACT
|
|---|
Congenital coronary-pulmonary artery fistula is rare in patients with pulmonary atresia and ventricular septal defect. The nomenclature, physiological, clinical, and surgical implications of these fistulas are yet to be defined. We report a one-year-old child with pulmonary atresia, ventricular septal defect, and a right coronary-pulmonary artery fistula who also had a diminutive, disconnected left pulmonary artery in addition to aortopulmonary collaterals. The patient underwent corrective surgery. However, the fate of diminutive pulmonary arteries is unknown. The literature was reviewed to explore the clinical or surgical implications of such fistulas for improved understanding and management in the future.
|
INTRODUCTION
|
|---|
Coronary-pulmonary artery fistula (CPAF) is a rare source of pulmonary blood in patients with pulmonary atresia (PA) and ventricular septal defect (VSD). Pulmonary blood supply in these patients is highly variable and usually occurs through a patent ductus arteriosus or major aortopulmonary collaterals (MAPCA). We report a patient who presented with PA, VSD, and right CPAF in addition to disconnected and diminutive left pulmonary artery (LPA) and MAPCA to the right lung.
|
CASE REPORT
|
|---|
A one-year-old boy, product of full term delivery, was presented to us with a history of mild cyanosis since birth, especially after crying. On physical examination, there was a grade III systolic murmur at the left lower sternal border. The oxygen saturation was 80% in room air. Two-dimensional echocardiography revealed situs solitus, levocardia, and malaligned ventricular septal defect with 50% aortic overriding, pulmonary atresia, and right aortic arch. In addition, transesophageal echocardiography showed a fistulous tract between the ascending aorta and the main pulmonary artery (Figure 1
). Angiocardiography including aortography confirmed the presence of a right coronary-pulmonary artery fistula, opening into the proximal main pulmonary artery (MPA) (Figure 2). The MPA continued as the right pulmonary artery, however, the LPA could not be demonstrated by routine angiography. Pulmonary venous angiography performed through the left inferior pulmonary vein revealed a vestigial disconnected left pulmonary artery that filled retrogradly (Figure 3). In addition, MAPCA’s to the right lung were evident.
View larger version (78K):
[in this window]
[in a new window]
|
Figure 1. Transesophageal echocardiogram showing the coronary-pulmonary artery fistula. Ao = aorta, CPAF = coronary-pulmonary artery fistula, PA = pulmonary artery.
|
|
View larger version (131K):
[in this window]
[in a new window]
|
Figure 2. Aortogram: the arrow indicates the coronary-pulmonary artery fistula opening into the proximal main pulmonary artery. Ao = aorta, CPAF = coronary-pulmonary artery fistula, PA = pulmonary artery, RCA = right coronary artery.
|
|
View larger version (144K):
[in this window]
[in a new window]
|
Figure 3. Selective injection into the left inferior pulmonary vein (PV) delineating retrograde filling of the diminutive, non-communicating left pulmonary artery (LPA).
|
|
At operation, resection of the right ventricular muscle bands, patch closure of the VSD, and transannular patching was undertaken using bovine pericardium. The CPAF was identified and ligated (Figure 4). The fistulous tract was about 4 cm long, superior and to the right of the transannular patch. The vestigial left pulmonary artery was unifocalized by anastomosing it to the main PA. Postoperatively, the patient developed right chylothorax and was managed conservatively. The patient was discharged from hospital and was doing well, 5 months after surgery. The MAPCA’s were left and did not pose any problems postoperatively. However, fate of the LPA anastomosis will be assessed at a more extended follow-up.
View larger version (117K):
[in this window]
[in a new window]
|
Figure 4. Operative photograph showing the origin of the right coronary artery and a fistulous tract to the proximal main pulmonary artery. Ao = aorta, CPAF = coronary-pulmonary artery fistula, PA = pulmonary artery, RCA = right coronary artery.
|
|
|
DISCUSSION
|
|---|
Congenital coronary artery fistula occurs in approximately 1 in 50,000 patients with congenital heart disease.1 In the literature to date only 32 patients have been reported with CPAF in association with PA and VSD and the majority of them had a fistula originating from the left coronary artery (Table 1).2,3,4 Additionally, 58% of these patients had MAPCA.2,4 However, the true prevalence of CAPF cannot be estimated.2,4
Coronary-pulmonary artery fistula may provide sole or partial blood supply to the pulmonary arterial system and join the PA in an antegrade fashion similar to ductus arteriosus. However, CPAF are unlike systemic-pulmonary artery collaterals which usually make connections within the lung parenchyma or at the hilum as they are remnants of a splanchnic vascular system that normally regresses.4 Therefore, the nomenclature, embryological basis, physiological, clinical, and surgical implications of CPAF have not been well defined.
In earlier reported cases of PA, VSD, and CPAF the age at presentation ranged from infancy to adulthood. Similarly, variable clinical presentation in this group of patients has been reported; from asymptomatic presentation to severe cyanosis.3,5 Pahl et al described a patient with pulmonary hypertension at 7 years of age in association with PA, VSD, and CPAF secondary to excessive blood flow through the fistula.6 Similarly, congestive heart failure may occur in these patients as a result of excessive flow.3
In patients with isolated CPAF without pulmonary atresia, the clinical significance and consequences are mainly dependent on the severity of the left-to-right shunt, coronary steal phenomenon or occurrence of complications such as bacterial endocarditis, dissection, embolization, or myocardial infarction.1,7 However, cases with CPAF in association with pulmonary atresia may have a favorable prognosis due to the fistula being an additional source of pulmonary blood flow that could allow growth of collateral pulmonary arteries. Also, a patent CPAF may reduce the severity of cyanosis in these patients clinically.5
Anatomically, in our case, there was a common trunk arising from the right coronary sinus of the aorta that was branching into the right coronary artery and the fistulous tract. The pulmonary valve was severely atretic and the LPA was vestigial, disconnected from the MPA and terminating as a blind vestigial sac. Therefore, the lung blood flow was mainly dependent on the right pulmonary artery through the CPAF and MAPCA’s. The presence of CPAF allowed growth of the PA despite the atretic valve and also kept the level of cyanosis mild.
In previously reported patients, the diagnosis was made by echocardiography, angiocardiography, magnetic resonance imaging, during surgery, and at necropsy.2,3,8 However, the most significant clinical implication of CPAF is the need for detailed and accurate recognition before surgery. Therefore, preoperative cardiac catheterization is critical for successful repair.3,7 In our case, during initial angiogram the left pulmonary artery could not be delineated. However, injection of dye into the left pulmonary vein revealed a small diminutive left pulmonary artery without any connection with the main PA or aorta. During surgery, we could unifocalize the vestigial left PA by anastomosing it with the main PA hoping that it might grow in the future, however the fate of such arteries is still unknown.
From an operative point of view, early surgery is indicated to avoid complications associated with CPAF.2,7 This fistula may be ligated or divided so that redundant fistulous tissue can be used for right ventricular outflow tract reconstruction.2,3,4,7
Coronary-pulmonary artery fistula should, therefore, be considered as a possible source of pulmonary blood supply in patients with PA, VSD and should be diligently searched for by echocardiography. Their presence should be confirmed or excluded by detailed angiography even when other sources of pulmonary flow are present. The course of the CPAF has to be clearly delineated prior to surgery.
|
ACKNOWLEDGMENTS
|
|---|
The authors appreciate the contribution of Mr. Joe Franke from the research wing of the Cardiac Science Section for his valuable contribution in the editing and preparation of high quality images.
|
REFERENCES
|
|---|
- Lowe JE, St Louis JD, Sabiston DC,. Congenital lesions of the coronary circulation. In: Sabiston DC Jr, Lyerly HK, eds. Text book of surgery: the biological basis of modern surgical practice, 15th ed. Philadelphia: Saunders, 1997:2124–35.
- Kaneko Y, Okabe H, Nagata N, Kobayashi, J, Murakami A, Takamoto S. Pulmonary atresia, ventricular septal defect, and coronary-pulmonary artery fistula. Ann Thorac Surg 2001;71:355–6.[Abstract/Free Full Text]
- Solowiejczyk DE, Cooper MM, Barst RJ, Quaegebeur JM, Gersony WM. Pulmonary atresia and ventricular septal defect with coronary artery to pulmonary artery fistula: case report and review of the literature. Pediatr Cardiol 1995;16:90–4.[Medline]
- Amin Z, McElhinney DB, Reddy VM, Moore P, Hanley FL, Teitel DF. Coronary to pulmonary artery collaterals in patients with pulmonary atresia and ventricular septal defect. Ann Thorac Surg 2000;70:119–23.[Abstract/Free Full Text]
- Dark JH, Pollock JC. Coronary artery-pulmonary artery fistula in tetralogy of Fallot with pulmonary atresia. Eur Heart J 1985;6:714–6.[Abstract/Free Full Text]
- Pahl E, Fong L, Anderson RH, Park SC, Zuberbuhler JR. Fistulous communications between a solitary coronary artery and the pulmonary arteries as the primary source of pulmonary blood supply in tetralogy of Fallot with pulmonary valve atresia. Am J Cardiol 1989;63:140–3.[Medline]
- Balanescu S, Sangiorgi G, Castelvecchio S, Medda M, Inglese L. Coronary artery fistulas: clinical consequences and methods of closure. A literature review. Ital Heart J 2001;2:669–76.[Medline]
- Puvaneswary M, Warner G, Pressley L, Hawker R. Coronary artery fistula in a patient with pulmonary atresia and tricuspid atresia clinical and MRI findings. Heart Lung Circ 2004;13:317–21.[Medline]
TOP
ABSTRACT
INTRODUCTION
