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HERDIN Record #: PCHRD071008030748 Submitted: 09 July 2008

Double outlet right ventricle with double orifice mitral valve.

Helen Grace B. Aragones

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due to recurrent respiratory tract infections. Initial physical examination at the Outpatient Department at 4 months of age was a grade 3/6 pansystolic murmur at the left midsternal border which was thought of as a typical shunt anomaly such as that of a ventricular septal defect. However, on subsequent follow-ups, there was already note of accentuated P2, a systolic thrill, 4/6 pansystolic murmur on the left midsternal border and systolic ejection murmur on the left upper sternal border. ECG showed left atrial enlargement and left ventricular hypertrophy. Initial 2DEcho showed a ventricular septal defect with aortic insufficiency, trivial. Repeat 2DEcho 3 years after showed a VSD, subaortic in location with severe subaortic stenosis. Latest 2DEcho showed a VSD with double orifice mitral valve, subvalvar aortic stenosis and a severe tricuspid regurgitation. Intraoperatively, there was note of a double outlet right ventricle and a double orifice mitral valve. There is no available literature on this combination of congenital anomaly. Surgical management is necessary. However, the importance of a pre-operative 2DEcho still is an invaluable tool in guiding the surgeons on what to expect during the procedure. (Author)

Publication Type
Publication Sub Type
Case report
Philippine Heart Center Journal
Publication Date
April-June 2006


This case is being reported because of its rarity and to emphasize the importance of a two-dimention echocardiography (2DEcho) as the most important tool in the diagnostic assessment of these patients preoperatively.

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