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Logo of thijTexas Heart Institute JournalSee also Cardiovascular Diseases Journal in PMCSubscribeSubmissionsTHI Journal Website
 
Tex Heart Inst J. 2010; 37(5): 608–609.
PMCID: PMC2953236
WEBSITE FEATURE

Herniated Aortic Valve Contributes to Obstruction in Double-Chambered Right Ventricle

Raymond F. Stainback, MD, Section Editor
Department of Adult Cardiology, Texas Heart Institute at St. Luke's Episcopal Hospital, 6624 Fannin St., Suite 2480, Houston, TX 77030

A 52-year-old woman who developed chest pain upon effort was admitted to our hospital. A grade 3/6 systolic heart murmur was heard over both sternal borders. An electrocardiogram showed sinus rhythm and right ventricular (RV) hypertrophy. Echocardiography showed a subarterial ventricular septal defect (VSD), right coronary cusp (RCC) herniation, trivial aortic valve regurgitation, and a double-chambered RV. Cardiac catheterization and angiography showed no coronary artery disease and confirmed our diagnosis. The RV was divided into 2 chambers by anomalous muscle bundles (Fig. 1A and 1B). The pressure gradient between the outlet chamber and inlet chamber of the RV was 42 mmHg, and the Qp/Qs ratio was 1.48.

figure 24FF1
Fig. 1 Preoperative (A, B) and postoperative (C, D) angiograms. A) The severely deformed right coronary cusp (RCC) prolapsed into the right ventricle, as can be seen during systole (arrows). B) The right ventricle was divided into 2 chambers during systole ...

The surgery, performed with the patient under cardiopulmonary bypass, included VSD closure and resection of the anomalous muscle bundles, but not aortic valvuloplasty. The postoperative course was uneventful. Cardiac catheterization and echocardiography at the 3-year follow-up visit showed successful resection of the muscle bundles and resolution of the RCC prolapse into the RV, without residual VSD or worsened aortic valve regurgitation (Fig. 1C and 1D).

Comment

Double-chambered RV is relatively rare and is usually diagnosed and repaired during childhood or adolescence; it is seldom seen in adults.1 Perimembranous VSD is the most common defect seen with double-chambered RV, and its presence is strongly associated with progressive ventricular obstruction.2 In this patient, the associated defect was a large subarterial VSD, usually seen in Asians, which can lead to aortic valve regurgitation secondary to RCC deformity and to prolapse of the RCC into the RV.3 Because the patient's cardiac anomalies were not diagnosed until she was 52 years of age, the large VSD had caused progressive RCC deformity and severe RCC prolapse into the RV at systole and diastole. The ventricular obstruction appeared to have been caused by the severely damaged RCC and the anomalous muscle bundles (Fig. 1B).

Although RV obstruction has several possible causes,4 double-chambered RV is caused specifically by anomalous muscle bundles that divide the RV.1,5 In our patient, the prolapsed RCC might well have contributed to the RV obstruction. Indeed, a good outcome was achieved by closing the VSD, which prevented further RCC prolapse into the RV, and by resectioning the anomalous muscle bundles.

Supplementary Material

Video for Fig. 1A:
Video for Fig. 1B:
Video for Fig. 1C:
Video for Fig. 1D:

Footnotes

Address for reprints: Haruhiko Kondoh, MD, Department of Cardiovascular Surgery, Otemae Hospital, 1-5-34, Otemae, Chuo-ku, Osaka 540-0008, Japan

E-mail: pj.en.ten-os.3aw@khurah

References

1. Lucas RV Jr, Varco RL, Lillehei CW, Adams P Jr, Anderson RC, Edwards JE. Anomalous muscle bundle of the right ventricle. Hemodynamic consequences and surgical considerations. Circulation 1962;25:443–55. [PubMed]
2. Oliver JM, Garrido A, Gonzalez A, Benito F, Mateos M, Aroca A, Sanz E. Rapid progression of midventricular obstruction in adults with double-chambered right ventricle. J Thorac Cardiovasc Surg 2003;126(3):711–7. [PubMed]
3. Cheung YF, Chiu CS, Yung TC, Chau AK. Impact of preoperative aortic cusp prolapse on long-term outcome after surgical closure of subarterial ventricular septal defect. Ann Thorac Surg 2002;73(2):622–7. [PubMed]
4. Restivo A, Cameron AH, Anderson RH, Allwork SP. Divided right ventricle: a review of its anatomical varieties. Pediatr Cardiol 1984;5(3):197–204. [PubMed]
5. Kenaan G, Kay JH, Redington JV, Mendez AM, Zubiate P, Dunne E, Roger R. Intracardiac foreign body simulating double-chamber right ventricle. Am J Cardiol 1973;31(6):781–4. [PubMed]

Articles from Texas Heart Institute Journal are provided here courtesy of Texas Heart Institute