Coronary Artery Fistula Clinical Presentation

Updated: Jan 27, 2015
  • Author: Monesha Gupta, MD, MBBS, FAAP, FACC, FASE; Chief Editor: Stuart Berger, MD  more...
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Presentation

History

Most children with small coronary artery fistulae (CAF) are asymptomatic, and continuous murmur may be audible on routine examinations if the fistulae are moderate to large in size. In infants, angina may be recognized by symptoms such as irritability, diaphoresis, pallor, tachypnea, and tachycardia. Most infants present at age 2-3 months after the pulmonary vascular resistance has decreased with heart failure symptoms, such as tiredness during feeding, tachypnea and excessive diaphoresis during feeds, wheezing, episodic pallor, and failure to thrive. Thus, in infancy, they can present with signs of low-output congestive heart failure.

Older patients may present with signs of low-output congestive heart failure, arrhythmias, syncope, chest pain, and, rarely, endocarditis. Patients with large fistulae may present with high-output congestive heart failure, although rarely. In older patients, symptoms may include the following:

  • Dyspnea on exertion

  • Angina

  • Fatigue

  • Palpitations

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Physical

Most patients are asymptomatic, especially when the fistulas are small. Note the following:

  • A coronary artery fistula is suspected following detection of a continuous murmur upon routine examination. Upon clinical examination, the murmur is suggestive of patent ductus arteriosus but is heard lower on the sternal border than usual; thus, the location is often atypical for a patent ductus arteriosus. In addition, the murmur may have an unusual diastolic accentuation, and the continuous murmur of a coronary artery fistula often peaks in mid-to-late diastole, which is uncharacteristic of the systolic accentuation in a patient with patent ductus arteriosus.

  • If the fistula connects to the left ventricle, only an early diastolic murmur may be heard, as little coronary flow is evident during the period of systole.

  • Some patients with fistulae with a large shunt may present with signs of congestive heart failure and angina.

  • Wide pulse pressure and collapsing pulse may be noted.

  • The apex beat is diffuse with a palpable or audible third heart sound (S3) gallop in a large fistula. Heart sounds are often reduced in intensity.

  • A holosystolic murmur of mitral valve insufficiency is audible at the apex.

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Causes

Most coronary artery fistulae are congenital and may be found in patients with structurally normal hearts. A specific variant form of coronary artery fistula (coronary-sinusoidal connection) that occur in association with congenital heart disease arise most often in association with severe right or left ventricular outflow obstruction, such as pulmonary atresia with intact ventricular septum or aortic atresia with hypoplastic left heart syndrome. In outflow obstructions, the fistula serve to decompresses the ventricle in a retrograde flow fashion.

Rarely, acquired forms of coronary artery fistula may occur as a result of septal myectomy in association with hypertrophic cardiomyopathy, muscle bundle resection in operative repair of tetralogy of Fallot, as a complication of radiofrequency ablation of accessory pathways, penetrating or nonpenetrating trauma, endomyocardial biopsy, permanent pacemaker implantation, or as a complication of coronary arterial procedures.

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