J Cardiovasc Ultrasound.  2011 Dec;19(4):216-220. 10.4250/jcu.2011.19.4.216.

Right Coronary Cusp Prolapse Resembling Subpulmonic Stenosis in an Old Adult Patient with Ventricular Septal Defect

• Kim MG
• Chung WJ
• Choi CH
• Moon J
• Shin MS
• Han SH
• Shin EK

• KMID: 1980387
• DOI: http://doi.org/10.4250/jcu.2011.19.4.216

Abstract

Ventricular septal defect (VSD) can be associated with various complications such as aortic regurgitation (AR). AR in VSD come from a deficiency or hypoplasia of the conal septum which leads to abnormal apposition in diastole and prolapse of the poorly supported noncoronary or right coronary cusp through the VSD into the right ventricle resembling subpulmonic stenosis and subsequently results in distortion of the aortic valve and progressive AR. AR often increases in severity with age and it indicates a worse prognosis. Therefore, appropriate timing of surgical repair in progressive AR in VSD might be important. Until now, many earlier experiences about surgical repair of AR complicating VSD were on adolescents or young adults. We reported a case of AR in 48-year-old male patient with right coronary cusp prolapse complicating the subarterial type of VSD which was properly assessed by echocardiography and was successfully treated with surgical repair. Right coronary cusp or noncoronary cusp prolapse should be suspected in AR complicating VSD through proper echocardiographic assessment and the surgical repair on VSD and distorted aortic valve should be considered in the old patient, as well as the young.

Keyword

Ventricular septal defect; Aortic valve; Aortic regurgitation; Prolapse

MeSH Terms

Adolescent
Adult
Aortic Valve
Aortic Valve Insufficiency
Constriction, Pathologic
Diastole
Echocardiography
Heart Septal Defects, Ventricular
Heart Ventricles
Humans
Male
Middle Aged
Prognosis
Prolapse
Young Adult

Figure

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  Fig. 1 Transthoracic echocardiography with the short-axis view at the aortic level (A) with continuous wave Doppler at the right ventricular outflow tract (RVOT) (B). It showed mild RVOT obstruction resembling subpulmonic stenosis (arrowhead) with increased peak and mean pressure gradients (32 mm Hg and 19 mm Hg, respectively).

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  Fig. 2 The transthoracic parasternal long axis views on admission with illustrations. A and B: There were a discontinuity on interventricular septum showing subarterial type ventricular septal defect (VSD, arrow) and a finger-like projection of sinus of Valsalva (arrowhead) resulting from longstanding high velocity jet through VSD. C: Severe aortic regurgitant jet was identified with color Doppler. D: A section of the aortic root illustration showed the direction of blood flow (arrows). LV: left ventricle, LA: left atrium, IVS: interventricular septum.

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  Fig. 3 The transthoracic short-axis view at the aortic level on admission (A) and illustration (B). The right coronary cusp and sinus of Valsalva are driven into the right ventricle (RV) due to longstanding high velocity jet produced by the left-to-right shunt (arrow). As the result, the right ventricular outflow tract obstruction occurred (arrowhead). RCC: right coronary cusp, NCC: noncoronary cusp, LCC: left coronary cusp, RVOT: right ventricular outflow tract.

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  Fig. 4 Surgical findings of the aortic valve via aortotomy (A) and illustration (B). It showed a retracted right coronary cusp (RCC) (arrowhead) and the commissural fusion and calcification between the RCC and the noncoronary cusp (star).

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