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Acquired double-chambered right ventricle.

ABSTRACT:

SUBMITTER: Zielinski P

PROVIDER: S-EPMC8084404 | biostudies-literature |

REPOSITORIES: biostudies-literature

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From Double-Chambered Right Ventricle to Double-Chambered Left Ventricle : Unusual Evolution of a Ventricular Septal Defect

Project description:We present the first imaging registry of the progressive isolation of an apical chamber of the right ventricle caused by the hypertrophy of the moderator band generated from the hemodynamic effect of a ventricular septal defect, leaving the apex of the right ventricle as an accessory chamber of the left ventricle. (Level of Difficulty: Advanced.) Central Illustration

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Project description:We present a case of double-chambered right ventricle (DCRV) complicated by hypertrophic obstructive cardiomyopathy (HOCM) in KRAS mutation-associated Noonan syndrome. The diagnosis was incidental and made during diagnostic testing for an intradural extramedullary tumour. Spinal compression, if not surgically treated, may cause paralysis of the extremities. We decided to pursue pharmacological therapy to control biventricular obstructions and reduce the perioperative complication rate. We initiated treatment with cibenzoline and bisoprolol; the doses were titrated according to the response. After 2 weeks, the peak pressure gradient of the two RV chambers decreased from 101 to 68 mmHg, and the LV peak pressure gradient decreased from 109 to 14 mmHg. Class 1A antiarrhythmic drugs and β-blockers decreased the severe pressure gradients of biventricular obstructions caused by DCRV and HOCM. The patient was able to undergo surgery to remove the intradural extramedullary tumour, which was diagnosed as schwannoma.

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