Introduction
The incidence of aortic right coronary cusp (RCC) prolapse in outlet ventricular septal defect (VSD) is reported at 5%- 16%. 1-3 Detection of RCC prolapse is critical in patients with outlet VSD because this complication may cause permanent aortic regurgitation. Aortic regurgitation occurs due to a poorly supported RCC combined with the venturi effect due to the VSD jet resulting in cusp prolapse. 4 This is an indication for VSD closure even if VSD is small and restrictive. 5,6
Case
An 8 month old baby presented to us with poor weight gain and recurrent episodes of fast breathing. On examination a grade 4 pan-systolic murmur was present at the lower left sternal area with a grade 3 diastolic murmur at the second right intercostal space. Echocardiogram showed a restrictive outlet VSD with RCC prolapse resulting in significant aortic regurgitation (figures 1-3). The child was immediately sent for VSD closure.
Figure 1.
Apical 4 chamber view showing subaortic restrictive Ventricular septal defect (L-R shunt).
Figure 2.
Parasternal Long axis view showing significant RCC prolapse.
Figure 3.
Parasternal Long axis view showing Aortic Regurgitation through venturi effect created by RCC prolapse through VSD.
References
- 1. Brauner R, Birk E, Blieden L, Sahar G, Vidne BA. Surgical management of ventricular septal defect with aortic valve prolapse: clinical considerations and results. Eur J Cardiothoracic Surg 1995; 9:315-9. [DOI] [PubMed] [Google Scholar]
- 2. Lue HC, Sung TC, Hou SH, Wu MH, Cheng SJ, Chu SH, et al. Ventricular septal defect in Chinese with aortic valve prolapse and aortic regurgitation. Heart Vessels 1981; 2:111-6. [DOI] [PubMed] [Google Scholar]
- 3. Ando M, Takao A. Pathological anatomy of ventricular septal defect associated with aortic valve prolapse and regurgitation. Heart Vessels 1986; 2:117-26. [DOI] [PubMed] [Google Scholar]
- 4. Tomita H, Arakaki Y, Ono Y, Yamada O, Yagihara T, Echigo S. Imbalance of cusp width and aortic regurgitation associated with aortic cusp prolapse in ventricular septal defect. Jpn Circ J 2001; 65: 500-4. [DOI] [PubMed] [Google Scholar]
- 5. Hideshi Tomita, Yoshio Arakaki, Yasuo Ono, Osamu Yamada, Toshikatsu Yagihara, Shigeyuki Echigo. Severity Indices of Right Coronary Cusp Prolapse and Aortic Regurgitation Complicating Ventricular Septal Defect in the Outlet Septum Which Defect Should Be Closed. Circ J 2004; 68: 139 –143. [DOI] [PubMed] [Google Scholar]
- 6. Chiu SN, Wang JK, Lin MT, Wu ET, Lu FL, Chang CI, et al. Aortic valve prolapse associated with outlet-type ventricular septal defect. Ann Thorac Surg 2005; 79: 1366-71. [DOI] [PubMed] [Google Scholar]
