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Asymptomatic systolic murmur unveiling a rare and complex cardiovascular anomaly

Session Clinical Case Poster session 1

Speaker Ines Silveira

Event : EuroEcho 2017

  • Topic : valvular, myocardial, pericardial, pulmonary, congenital heart disease
  • Sub-topic : Congenital Heart Disease and Pediatric Cardiology
  • Session type : Poster Session

I Silveira1 , F Oliveira1 , M Trepa1 , R Baggen Santos1 , M Oliveira1 , P Rodrigues1 , A Luz1 , S Cabral1 , S Torres1 , 1Hospital Center of Porto, Cardiology - Porto - Portugal ,

European Heart Journal Supplements ( 2017 ) 18 ( Supplement 3 ), iii37

Coronary fistulas are rare cardiac anomalies, present in 0.002% of the general population. Most patients remain asymptomatic but due to increasing age and shunt ratio they can develop cardiovascular symptoms, associated with significant morbidity and mortality.

We present a case of a young football player, asymptomatic and without pathological history. He was referenced to a cardiology consult at the age of 18 years old due to a systolic cardiac murmur grade II/VI identified during a sports medical evaluation. Transthoracic echocardiography revealed the presence of a bicuspid aortic valve, without significant stenosis and aortic coarctation distal to the left subclavian artery with a maximal gradient of 21 mmHg. At parasternal short axis view, in lateral-left portion of pulmonary trunk was visible a diastolic flow, not being possible to characterize its origin. At four chambers view it were also present multiple diastolic intramyocardial flows in both ventricles in different directions, raising the suspicion of vascular abnormalities. Biventricular systolic function was normal. In order to characterize these vascular anomalies and evaluate its hemodynamic consequence a coronary angiography was performed confirming the presence of multiple coronary fistulas that originated from the three main coronary arteries heading apparently into the pulmonary artery and a Qp/Qs = 1. To provide additional anatomical delineation the patient underwent a CT coronary angiogram that confirmed a non-significant aortic coarctation (15 mm) and showed the presence of a large anomalous vessel with 7 mm with a parallel path to the anterior descending artery along the interventricular sulcus, corresponding to an anomalous venous drainage to the lateral left side of the main pulmonary artery. Exercise SPECT test was negative for ischemia, with a good functional capacity. The case was discussed in Heart Team and it was decided conduct conservative management. Patient was advised to leave the practice of competition sport and stayed under medical treatment. In the last 6 years of follow up he remained asymptomatic, without hypertension or changes in echocardiography evaluations.

These case stands out for the unusual association, presenting three simultaneous congenital abnormalities: bicuspid aortic valve, aortic coarctation and multiple coronary fistulas drainaging through a large anomalous vessel into pulmonary artery, in an asymptomatic sportsman. This highlights the important value of imaging multimodality in the characterization of vascular abnormalities. Comprehensive imaging evaluation is necessary to precisely define the anatomy and pathophysiologic aspects of the anomalies in order to determine the treatment options for a specific patient. Currently, it is widely accepted that all symptomatic patients with coronary fistulas should be treated, however recommendations for asymptomatic patients are scarce and there is no clear consensus in the literature. This discussion becomes even more relevant in young patients practicing sports.

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