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

| S-EPMC9588453 | biostudies-literature

Isolated Double-Chambered Right Ventricle - A Rare Entity.

Project description:A double-chambered right ventricle (DCRV) is a rare congenital heart disease and an uncommon cause of congestive heart failure. An anomalous muscle band divides the right ventricle into two cavities: the proximal high-pressure chamber and the distal low-pressure chamber. Most cases are diagnosed and treated during childhood. Furthermore, there is a tendency for progression, if not treated early. Echocardiography is considered useful for the diagnosis of this ailment. Most of the patients have associated congenital anomalies, such as ventricular septal defect, pulmonary stenosis, and subaortic stenosis. Isolated DCRV is a rare entity. Hence, we report a case of an isolated DCRV in an adult patient.

| S-EPMC7799069 | biostudies-literature

Double-Chambered Right Ventricle in Adulthood: A Case Series.

Project description: Not available

| S-EPMC10954581 | biostudies-literature

'Matrioska left ventricle': a case of double-chambered left ventricle with double mitral valve apparatus.

Project description: Not available

| S-EPMC9303002 | biostudies-literature

Correction of Double-Chambered Right Ventricle 40 Years after Pulmonary Valvuloplasty.

Project description:Background Double-chambered right ventricle is a partial separation of the right ventricle by pathological tissue. This anomaly is described in infants and children but can also be diagnosed in young adults. Case Description A 47-year-old patient presented with chest pain and exercise intolerance. Further evaluation revealed a double-chambered right ventricle. During the surgical procedure, fibrotic tissue was transected, and a pulmonary valve replacement including enlargement of the main pulmonary artery was performed. Conclusion Subpulmonary obstruction of the right ventricle can be related to double-chambered right ventricle even in adulthood. Treatment options include surgical resection with pulmonary valve replacement.

| S-EPMC4670311 | biostudies-other

A rare case of double-chambered right ventricle associated with ventricular septal defect and congenital absence of the pulmonary valve.

Project description:Double-chambered right ventricle (DCRV) is a rare congenital heart disorder involving 2 different right ventricle (RV) pressure compartments that is often associated with ventricular septal defect (VSD). Usually, the obstruction is caused by an anomalous muscle bundle crossing the RV from the interventricular septum to the RV free wall. We are reporting a case of double-chambered right ventricle associated with ventricular septal defect and congenital absence of the pulmonary valve, a rare form of congenital infundibular pulmonary stenosis. In addition to ventricular septal defect, our patient had congenital absence of the pulmonary valve, which is very unusual and has never been reported to our knowledge.

| S-EPMC3966724 | biostudies-literature

Double-chambered right ventricle complicated by hypertrophic obstructive cardiomyopathy diagnosed as Noonan syndrome.

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.

| S-EPMC7160468 | biostudies-literature

Unusual double-chambered right ventricle induced by ruptured sinus of Valsalva aneurysm: A case report.

Project description:A 54-year-old male with a history of unrepaired ventricular septal defect (VSD) suffered from easy fatigability on exertion. A Levine grade V/VI continuous murmur was auscultated. Transthoracic echocardiogram showed a ruptured sinus of Valsalva aneurysm (SVA) and a significant left-to-right shunting from the ascending aorta to the right ventricle (RV). In addition, a 36 mmHg of pressure gradient was observed between the inflow and outflow tract in the RV, suggesting double-chambered RV (DCRV). Cardiac catheterization also revealed 33 mmHg of the pressure gradient in the mid-potion of the RV, which was coincident with DCRV. A calculated pulmonary-to-systemic flow ratio was 3.0. Therefore, the patient was offered surgical repair of the ruptured SVA and VSD, which was successfully performed. During the surgery, an anomalous muscle band, which is usually the cause of DCRV, was not found, instead, a thickened RV free-wall due to the exposure of the left-to-right shunt flow, so-named jet lesion, was found. Therefore, surgical resection of the anomalous muscle band was not required. The protruded SVA toward the RV, the jet lesion, and the increased RV stroke volume, which could induce relative stenosis, were the causes of the unusual DCRV. <Learning objective: A mechanism of an unusual double-chambered right ventricle induced by ruptured sinus of Valsalva aneurysm (SVA) is as follows. One is a morphological stenosis in the right ventricle (RV) due to a protruded SVA toward the RV. The second is a jet lesion; a thickened RV wall induced by the exposure of the shunt flow from the ascending aorta. The third is a relative stenosis due to the increased stroke volume of the RV.>.

| S-EPMC7588484 | biostudies-literature

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