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Case Reports
. 2011 Sep;19(3):148-51.
doi: 10.4250/jcu.2011.19.3.148. Epub 2011 Sep 30.

Spontaneously healed membranous type ventricular septal defect with malaligned interventricular septal wall and double-chambered right ventricle in a 56-year-old patient

Jung Sun Cho  1 , Ho-Joong Youn, Sung-Ho Her, Soe Hee Ahn, Mahn-Won Park, Min Suk Choi, Jae Bum Lee, Jeong U Baeg, Chan Seok Park, Mi-Jeong Kim
Affiliations
Case Reports

Spontaneously healed membranous type ventricular septal defect with malaligned interventricular septal wall and double-chambered right ventricle in a 56-year-old patient

Jung Sun Cho et al. J Cardiovasc Ultrasound. 2011 Sep.

Abstract

A 56-year-old male presented with resting dyspnea and chest discomfort for several years. During transthoracic and transesophageal echocardiography, a spontaneously healed membranous type ventricular septal defect (VSD) with malaligned interventricular septal wall, aneurysmal changes, a subaortic ridge and a double-chambered right ventricle (DCRV) was observed. When combined with DCRV, VSD with malalignment between the outlet and trabecular septa was associated with tetralogy of Fallot. The subaortic ridge was due to turbulent flow caused by the malalignment-type VSD. The VSD with malaligned interventricular septal wall can be developed after aneurismal changes of a perimembranous VSD. We report here in the unusual case of a 56-year-old patient who had a pathology complex comprising DCRV, subaortic ridge, spontaneously healed membranous type VSD with malaligned interventricular septal wall, and survived with surgical treatment.

Keywords: Double-chambered right ventricle; Malaligned septal wall; Membranous type ventricular septal defect; Subaortic ridge.

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Figures

Fig. 1
Fig. 1
Preoperative transthoracic and transesophageal echocardiography. (A) Transthoracic echocardiography reveals a high-velocity systolic jet arising in the mid right ventricular outflow tract. (B) Right ventricular hypertrophy is seen on the parasternal long axis view. Transesophageal echocardiography reveals a subaortic ridge (arrowhead) (C) and aneurysmal changes (arrowhead) of the ventricular septal defect with anterior deviated interventricular septum (D). AV: aortic valve, RV: right ventricle, LV: left ventricle, IVS: interventricular septum.
Fig. 2
Fig. 2
Preoperative right and left ventriculography. Right ventriculography at end-systole reveals an anomalous muscle bundle dividing the cavity into 2 chambers (A), and left ventriculography shows spontaneous closed malalignmented ventricular septal defect with aneurysmal changes (B). DCRV: double-chambered right ventricle.
Fig. 3
Fig. 3
Preoperative cardiac catheterization demonstrates a right ventricle-to-pulmonary artery gradient of 89 mmHg. There is no significant step-up of O2 saturation in the right ventricular sample that is higher than the highest right atrial sample because of spontaneously closed malalignmented ventricular septal defect. Systolic pressure/diastolic pressure is expressed as a mean pressure and O2 saturation as a percentage. SVC: superior vena cava, RA: right atrium, RV: right ventricle, MPA: main pulmonary artery, RPA: right pulmonary artery, PCWP: pulmonary capillary wedge pressure, LV: left ventricle.
Fig. 4
Fig. 4
Postoperative transthoracic and transesophageal echocardiography. A: Transthoracic echocardiography reveals that a high-velocity systolic jet arising in the mid right ventricular outflow tract disappeared after surgery. B: Transesophageal echocardiography reveals much improved right ventricular outflow tract diameter after surgery. AV: aortic valve, PA: pulmonary artery.
See this image and copyright information in PMC

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