A case of double-chambered right ventricle due to cap-like fibrous tissue and aberrant chordal insertion of tricuspid valve in adult.

BACKGROUND: A double-chambered right ventricle is a rare congenital heart disease where an anomalous structure divides the right ventricle into two cavities. CASE: A 78-year-old man with dyspnea was referred to our institution for a double-chambered right ventricle (DCRV) and ventricular septal defect (VSD). Echocardiography showed normal left ventricular function, right ventricular hypertrophy, and mild tricuspid regurgitation. On parasternal short-axis views, color-flow Doppler studies showed a mosaic pattern through the stenotic right ventricular outflow tract (RVOT). Cardiac catheterization showed a 122 mmHg pressure gradient between the high-pressure chamber and the low-pressure chamber of the RVOT. Computed tomography showed a cap-like structure consisting of fibrous tissue, with moderate stenosis, which divided the right ventricle into two cavities. The aberrant tendinous chords supporting the anterior leaflet of the tricuspid valve were found inserted into the anterior wall of the RV (Figure 1). Resection of the cap-like fibrous tissue and abnormal muscle (Figure 2), detachment of aberrant tendinous chords of the tricuspid valve, closure of VSD by direct suture, and tricuspid valve plasty (TVP) were performed. TVP was achieved using an artificial chordae replacement with expanded polytetrafluoroethylene (CV-5) suture and a 32-mm Physio Tricuspid annuloplasty ring (Edwards Lifeline). Postoperative echocardiography revealed no RVOT pressure gradient and a normal right ventricular pressure of 21 mmHg. DISCUSSION: In conclusion, we report a rare case of DCRV secondary to a cap-like structure fibrous tissue with anomalous chordal insertion of a tricuspid valve leaflet.

[Early Structure Valve Deterioration after Aortic Valve Replacement with the Externally Mounted Bioprosthetic Valve( Trifecta):Report of Two Cases].

Trifecta valve is made from single bovine pericardial sheet, which is externally mounted on a titanium stent. This valve design provides good hemodynamic performance and wide effective orifice area. However, there have been some reports of early structural valve dysfunction which caused acute heart failure. Case 1:An 80-year-old man who had undergone aortic valve replacement (AVR) using Trifecta 21 mm five years ago. He presented with fever and diastolic murmur. Echocardiography showed prosthetic valve regurgitation, and the patient later developed prosthetic valve endocarditis. Case 2:An 80-year-old woman who had undergone AVR using Trifecta GT 21 mm two years ago developed sudden chest pain and dyspnea, prosthetic valve regurgitation. Both patients underwent re-AVR with a new bioprosthetic valve via re-sternotomy. Large tear was found in the right coronary cusp of the Trifecta in both cases. During Trifecta accomodation, we should avoid oversizing, and check the position of coronary ostia and sinus of Valsalva. Secure late outcome of Trifecta GT remains unproven and its use still needs caution.

Repair of a unique sinus of Valsalva defect in an infant.

Sinus of Valsalva aneurysm is a rare disease characterized by the partial elevation of the aortic root. Few reports are available on the surgical treatment for infants. We report the repair of an extremely rare case of a sinus of Valsalva defect with a ventricular septal defect and right ventricular outflow tract stenosis in an infant. It was not a sinus of Valsalva aneurysm, but it exhibited abnormal partial bulging of the aortic root and forming an aneurysm-like cavity within the right ventricular myocardium. We performed direct closure of the sinus of Valsalva aneurysm-like cavities and intracardiac repair in two stages. Three years after total repair, the patient remained healthy and asymptomatic.

[Closure of Multiple Ventricular Septal Defects by the Sandwich Technique and Pulmonary Artery Plasty after Arterial Switch Operation].

A 14-year-old girl had undergone a Jatene operation for double-outlet right ventricle with multiple muscular ventricular septal defects (VSD) at 12-day-old. During follow up periods, she was diagnosed with bilateral pulmonary artery stenosis and multiple muscular VSDs by echocardiography. Cardiac catheterization revealed elevated right ventricular pressure up to 93% of left ventricle. Multiple times balloon angioplasty failed to improve pulmonary artery stenosis. She underwent closure of multiple VSDs by the sandwich technique and pulmonary artery plasty with translocation of superior vena cava. We present operative technique for the complicated lesions in these operation.

Enlarged coronary sinus thrombosis after repair of Ebstein's anomaly.

A 58-year-old woman was referred to our hospital with progressively increasing breathlessness. She reported a history of bioprosthetic valve implantation for tricuspid valve replacement and direct closure of an atrial septal defect for Ebstein's anomaly, 31 years before presentation. Transthoracic echocardiography revealed prosthetic valve failure, an enlarged coronary sinus, and severe mitral regurgitation. Computed tomography revealed a giant coronary sinus with thrombosis and persistent left superior vena cava. She underwent successful mitral and tricuspid valve replacement; however, severe hemodynamic deterioration necessitated mechanical ventilatory support with extracorporeal membrane oxygenation.

[Aortic Regurgitation and Stenosis Associated with Ventricular Septal Defect in the Elderly;Report of a Case].

A 63-year-old man had ventricular septal defect (VSD) and had been followed up without heart failure. Recently, he had palpitation caused by atrial fibrillation and the echocardiography revealed moderate aortic valve regurgitation and stenosis with right coronary cusp prolapse due to subpulmonary ventricular septal defect. He underwent patch closure of VSD, aortic valve replacement with mechanical valve, and maze procedure. In recent years, advanced case like this patient is rare because most of patients with subpulmonary VSD and right coronary cusp prolapse are operated in childhood.

Acute valve malfunction with thrombosed bioprosthetic valve after surgical aortic valve replacement.

Acute valve thrombosis after bioprosthetic aortic valve replacement even under anticoagulation therapy is extremely rare. Cardiac computed tomography is a powerful imaging tool to detect valve thrombosis, and surgery is necessary in case of unstable hemodynamics.

Tetralogy of Fallot with severe ascending aortic enlargement in early childhood.

The Rastelli procedure is indicated for repair of tetralogy of Fallot (TOF) with pulmonary atresia. However, currently, there are no criteria available for concomitant surgical intervention for an enlarged ascending aorta in infancy. We report a rare case of TOF with pulmonary atresia complicated by severe enlargement of the ascending aorta in a girl aged 2 years and 5 months. We performed the Rastelli procedure with ascending aorta replacement. Pathological findings of the ascending aorta included fragmentation of elastic fibers and loss of smooth muscle cells. Postoperatively, the patient remained healthy and asymptomatic. This case indicates that ascending aortic enlargement in conotruncal anomalies may be associated with pathological lesion even in early childhood. Replacement of the diseased aorta could be considered as a concomitant procedure with definitive repair for conotruncal anomalies presenting with severe ascending aortic enlargement.

Endovascular treatment after the fenestrated frozen elephant trunk technique.

Recently, several centers have performed total arch replacement using the fenestrated frozen elephant trunk technique for acute Stanford type-A aortic dissection. However, the long-term results and need for additional treatment following this procedure are unclear. We report a case of a 54-year-old man who underwent endovascular therapy for endoleaks after total arch replacement using the fenestrated frozen elephant trunk technique for acute type-A aortic dissection with an isolated left vertebral artery. After the surgery, the endoleak was resolved, and the patient was asymptomatic with no neurological deficits. This strategy might be effective in similar cases.

Incidentally detected atrioventricular septal defect in an adult.

A 34-year-old woman, a liver transplantation donor for her son, was referred to our hospital for preoperative evaluation. She was diagnosed with an incomplete atrioventricular septal defect (AVSD). She underwent double valve repair and patch closure of the defect. Incidental discovery of an asymptomatic AVSD in an adult is rare.

Late Period Rastelli Take-Down and Arterial Switch Operation After Rastelli Operation.

The Rastelli operation is a useful technique for treating the transposition of the great arteries. However, conduit stenosis of the right ventricular outflow tract is a late complication of the procedure. We report the case of a 35-year-old man for whom an arterial switch operation was performed to treat conduit stenosis and improve ventricular arterial alignment 28 years after he underwent a Rastelli operation to treat transposition of the great arteries with a ventricular septal defect. In the 8 years that followed the operation, he was asymptomatic and was treated with warfarin.