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Table of Contents
Year : 2014  |  Volume : 17  |  Issue : 2  |  Page : 139-140
Ectatic coronary arteries in a patient with Noonan syndrome on transoesophageal echocardiography

1 Department of Cardiac Anaesthesiology, M. S. Ramaiah Narayana Hrudayalaya Heart Center, Bengaluru, Karnataka, India
2 Department of Cardiothoracic Surgery, M. S. Ramaiah Narayana Hrudayalaya Heart Center, Bengaluru, Karnataka, India

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Date of Web Publication1-Apr-2014

How to cite this article:
Ramaiah AH, Das JK, Shetty K R. Ectatic coronary arteries in a patient with Noonan syndrome on transoesophageal echocardiography. Ann Card Anaesth 2014;17:139-40

How to cite this URL:
Ramaiah AH, Das JK, Shetty K R. Ectatic coronary arteries in a patient with Noonan syndrome on transoesophageal echocardiography. Ann Card Anaesth [serial online] 2014 [cited 2020 Aug 9];17:139-40. Available from:

Noonan Syndrome (NS) is the second most common syndrome, after Down's, to be associated with congenital heart disease with an incidence of 0.05 to 0.1%. [1] A rather uncommon feature associated with NS is coronary ectasia (CE), which is associated with an increased risk of myocardial ischemia. The cause for ectasia in these patients is usually due to a vasculitic process that superimposes on the connective tissue abnormalities associated with NS. [2] We describe a female patient aged 45 years with NS who was scheduled to undergo atrial septal defect (ASD) closure. On pre-operative coronary angiogram she was found to have Markis Type I CE where there is diffuse ectasia of two or three vessels. [3] The ectatic coronary arteries had sluggish flow (TIMI Grade 2) and no stenosis. Intra-operatively, on Transoesophageal Echocardiography (TEE) we noticed right ventricular hypertrophy, dysplastic pulmonary valve with severe pulmonary stenosis with peak gradient of 75 mmHg and ASD shunting left to right [Figure 1]. We could also visualize left anterior descending and circumflex arteries using a technique described by Ender et al., and the calibers measured during TEE closely corresponded with that of the angiogram [Figure 2]a, b and [Figure 3]. [4] After completion of uneventful surgical procedure, she was transferred to intensive care unit with stable hemodynamics. Six hours later, patient had a sudden run of ventricular tachycardia followed by bradycardia and asystole. She was successfully resuscitated and the total cardiac arrest time was about 8 min. Interestingly the hemodynamic parameters, arterial blood gases and the electrocardiogram just prior to the cardiac arrest were all within the normal limits except for slightly low central venous pressure (3 mmHg). We believe CE could have been one of the precipitating factors for the cardiac arrest.
Figure 1: Midesophageal four chamber view shows right ventricular hypertrophy, septal thickening and atrial septal defect shunting left to right

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Figure 2: (a) Coronary angiogram in right anterior oblique view shows Left Circumflex (LCX) and Left Anterior Descending arteries (LAD). (b) Coronary angiogram in left anterior oblique view shows Right Coronary Artery (RCA). (Compare with 6 Fr catheter which has a diameter of 2 mm)

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Figure 3: Dilated Left Circumflex (LCX) and Left Anterior Descending (LAD) arteries visualized on transoesophageal echocardiography

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Ectatic coronary arteries may predispose to thrombus formation, clinically significant arterial spasm, spontaneous dissection, angina pectoris, myocardial infarction and possibly sudden cardiac arrest (SCA). [5] Furthermore, NS accounts for 30% of cases of hypertrophic cardiomyopathy (HCM) and one-third of these cases reveal left ventricular outflow tract (LVOT) obstruction. [6] HCM is one of the common causes of SCA in healthy young adults and both HCM and CE are seen to co-exist in some cases. [7] However, in our patient there was no LVOT obstruction. We believe the probable cause could be a combination of ischemia precipitated by sluggish flow due to CE and hypertrophied right ventricles.

As per the ACC/AHA guidelines, pre-operative coronary angiogram in patients undergoing non-coronary artery surgery for congenital heart disease is indicated only in patients with chest discomfort or non-invasive evidence suggestive of coronary artery disease and in patients with known congenital anomalies of coronary arteries. [8] The guidelines, however, are not very clear if the patient is asymptomatic. In our institution, we do a coronary angiogram only in those aged more than 40 years if they are scheduled for non-coronary artery surgery.

The multiplicity of cardiovascular and other systemic anomalies seen in NS, presents many challenges to the cardiac surgical team during the perioperative period. Rare associations of the syndrome like CE can increase the risk of complications like SCA. Echocardiographer should try to image coronary arteries routinely in these cases. It is also crucial to maintain high coronary perfusion pressure during the peri-operative period and to consider anticoagulation. TEE can easily identify patients with ectatic coronary arteries and effectively guide appropriate management.

   References Top

1.Burch M, Sharland M, Shinebourne E, Smith G, Patton M, McKenna W. Cardiologic abnormalities in Noonan syndrome: Phenotypic diagnosis and echocardiographic assessment of 118 patients. J Am Coll Cardiol 1993; 22:1189-92.  Back to cited text no. 1
2.Uçar T, Atalay S, Tekin M, Tutar E. Bilateral coronary artery dilatation and supravalvular pulmonary stenosis in a child with noonan syndrome. Pediatr Cardiol 2005;26:848-50.  Back to cited text no. 2
3.Markis JE, Joffe CD, Cohn PF, Feen DJ, Herman MV, Gorlin R. Clinical significance of coronary arterial ectasia. Am J Cardiol 1976;37:217-22.  Back to cited text no. 3
4.Ender J, Selbach M, Borger MA, Krohmer E, Falk V, Kaisers UX, et al. Echocardiographic identification of iatrogenic injury of the circumflex artery during minimally invasive mitral valve repair. Ann Thorac Surg 2010;89:1866-72.  Back to cited text no. 4
5.Sorrell VL, Davis MJ, Bove AA. Current knowledge and significance of coronary artery ectasia: A chronologic review of the literature, recommendations for treatment, possible etiologies, and future considerations. Clin Cardiol 1998;21:157-60.  Back to cited text no. 5
6.Lake CL, Booker PD. Pediatric Cardiac Anesthesia, 4 th ed. Philadelphia, PY: Lippincott Williams; 2005. p. 531.  Back to cited text no. 6
7.Zografos T, Kokladi M, Katritsis D. Coronary artery ectasia and systolic flow cessation in a patient with hypertrophic cardiomyopathy: A case report. Int J Cardiol 2010;145:e114-5.  Back to cited text no. 7
8.Scanlon PJ, Faxon DP, Audet AM, Carabello B, Dehmer GJ, Eagle KA, et al. ACC/AHA guidelines for coronary angiography: Executive summary and recommendations. A report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Committee on Coronary Angiography) developed in collaboration with the Society for Cardiac Angiography and Interventions. Circulation 1999;99:2345-57.  Back to cited text no. 8

Correspondence Address:
Anil Kumar H Ramaiah
M. S. Ramaiah Narayana Hrudayalaya Heart Center, M. S. Ramaiah Memorial Hospital, New BEL Road, Bengaluru 560 054, Karnataka
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0971-9784.129859

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  [Figure 1], [Figure 2], [Figure 3]

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