Double trouble - dual outflow tract obstruction in congenital heart disease: a case report.

BACKGROUND: Double chambered right ventricle is a rare congenital heart disease that is characterised by the presence of an anomalous muscle bundle that divides the right ventricle into a low pressure superior (distal) chamber and a high pressure inferior (proximal) chamber. It is found in association with a ventricular septal defect in 90% cases with other associations being tetralogy of Fallot, transposition of great vessels, atrial septal defect and Ebstein's anomaly. On the other hand, subaortic membrane is a form of discrete subaortic stenosis that is characterised by a membranous diaphragm in the subvalvular location of the left ventricular outflow tract. Both of these entities are responsible for causing subvalvular outflow tract obstruction. The occurrence of double chambered right ventricle in association with subaortic membrane is an extremely rare entity with only a few case reports available in the literature. CASE REPORT: A 13-year-old male child with history of chest pain and palpitations presented to the outpatient department of a tertiary care center. Transthoracic echocardiography revealed a subaortic membrane producing a pressure gradient across the left ventricular outflow tract with dilatation of the right atrium and right ventricle which could not be fully evaluated on echocardiography. Cardiac computed tomography was then performed which additionally revealed an anomalous muscle bundle coursing across the right ventricle from the septum to the subinfundibular region creating a double chambered right ventricle. The patient was then taken up for reconstruction of right ventricular outflow tract and resection of subaortic membrane. CONCLUSION: Right and left outflow tract obstructions are rare congenital lesions which when seen in combination, become even more infrequent. Echocardiography is a robust tool that detects turbulent flow to identify such lesions. However, poor acoustic window may sometimes result in missing these lesions and computed tomography in such situations can play an important role in detection as well as complete preoperative imaging evaluation.

Early and mid-term outcomes of double-chambered right ventricle repair: An 8-year experience.

BACKGROUND/AIM: Double-chambered right ventricle is a rare and progressive condition that is characterised by obstruction of the right ventricular tract. Double-chambered right ventricle is usually associated with ventricular septal defect. Early surgical intervention is recommended in patients with these defects. Based on this background, the present study aimed to review early and midterm outcomes of primary repair after double-chambered right ventricle. METHODS: Between January 2014 and June 2021, 64 patients with a mean age of 13.42 ± 12.31 years underwent surgical repair for double-chambered right ventricle. The clinical outcomes of these patients were reviewed and assessed retrospectively. RESULTS: An associated ventricular septal defect was present in all the recruited patients; 48 (75%) patients of sub-arterial type, 15 (23.4%) of perimembranous, and 1 (1.6%) patient of muscular type. The patients were followed up for a mean period of 46.73 ± 27.37 months. During their follow-up, a significant decrease in the mean pressure gradient from 62.33 ± 5.52 mmHg preoperatively to 15.73 ± 2.94 mmHg postoperatively was observed (p < 0.001). Notably, there were no hospital deaths. CONCLUSIONS: The development of double-chambered right ventricle in association with ventricular septal defect results in an increased pressure gradient within the right ventricle. The defect needs correction in a timely manner. In our experience, the surgical correction of double-chambered right ventricle is safe and shows excellent early and mid-term results.

Anomalous right ventricular muscle bands obstructing a large apical muscular ventricular septal defect: From fetal to post-natal three-dimensional assessment.

Anomalous right ventricle muscle bands and apical ventricular septal defect are two anomalies sometimes associated. We report a fetal diagnosis of a large apical ventricular septal defect, right intraventricular obstruction caused by anomalous muscle bands; consequently, the high right intraventricular pressure resulted in a right-to-left bulging of ventricular septum and moderate tricuspid regurgitation. Postnatal echocardiogram confirmed the fetal diagnosis and defined accurately the right ventricular anatomy through the three-dimensional echocardiographic assessment.

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.

Endothelial-to-Mesenchymal Transition as Underlying Mechanism for the Formation of Double-Chambered Right Ventricle.

Double-chambered right ventricle (DCRV) is a progressive division of the right ventricular outflow tract (RVOT) often associated with a subaortic ventricular defect (VSD). The septation is caused by a mixture of hypertrophied muscle bundles and fibrous tissue, whereof the latter is of unclear pathogenesis. Our group has previously reported that flow disturbances lead to formation of fibroelastic tissue through a process called endothelial-to-mesenchymal transition (EndMT) but it is unclear whether the same mechanism exists in the RV. Tissue from patients undergoing repair of DCRV was examined to identify the histomorphological substrate of this tissue. Demographic and pre-/post-operative echocardiographic data were collected from nine patients undergoing surgery for DCRV. RVOTO tissue samples were histologically analyzed for myocardial hypertrophy, fibrosis, elastin content, and active EndMT (immunohistochemical double-staining for endothelial and mesenchymal markers and transcription factors Slug/Snail) and compared to four healthy controls. Indication for surgery were symptoms and progressive RVOT gradients. A highly turbulent flow jet through the RVOTO and VSD was observed in all patients with a preoperative median RVOT peak gradient of 77 mmHg (IQR 55.0-91.5), improved to 6 mmHg (IQR 4.5-17) postoperatively. Histological analysis revealed muscle and thick infiltratively growing fibroelastic tissue. EndMT was confirmed as underlying patho-mechanism of this fibroelastic tissue but the degree of myocardial hypertrophy was not different compared to controls (P = 0.08). This study shows for the first time that an invasive fibroelastic remodeling processes of the endocardium into the underlying myocardium through activation of EndMT contributes to the septation of the RVOT.

Double-chambered right ventricle masquerading as bipartite right ventricle.

A 10-day-old neonate with pulmonary consolidation was referred for echocardiography to rule out CHD. At first glance, the morphology appeared to be a bipartite right ventricle with normal tricuspid and pulmonary valves. In-depth analysis, however, of the images showed a double-chambered right ventricle, in which the inlet and outlet portions of the right ventricle were isolated from the apical component of the right ventricle, which itself communicated with the left ventricle through a ventricular septal defect. There was a normal pulmonary valve and tricuspid annulus.

An extremely rare association of coarctation of aorta with double chambered right ventricle: double-trouble causing bi-ventricular failure in a child.

Double chambered right ventricle is a rare cardiac defect characterised by an obstructive hypertrophied muscle bundle in the right ventricle. The common associated lesions are ventricular septal defect followed by sub-aortic membrane. We report a child who had coarctation of aorta in association with double chambered right ventricle. This case is being reported for its rarity and challenges in management.

Congenital, acquired, or both? The only two congenitally based, acquired heart diseases.

Discrete subaortic stenosis (DSS) is a type of left ventricular outflow tract obstruction whereas double-chambered right ventricle is a form of right ventricular outflow tract obstruction. Both of these cardiac malformations share lots of similar characteristics which classify them as acquired developmental heart diseases despite their congenital anatomical substrate. Both of them are frequently associated to ventricular septal defects. The initial stimulus in their pathogenetic process is anatomical abnormalities or variations. Subsequently, a hemodynamic process is triggered finally leading to an abnormal subaortic fibroproliferative process with regard to DSS or to hypertrophy of ectopic muscles as far as double-chambered right ventricle is concerned. In many cases, these pathologies are developed secondarily to surgical management of other congenital or acquired heart defects. Moreover, high recurrence rates after initial successful surgical therapy, particularly regarding DSS, have been described. Finally, an interesting coexistence of DSS and double-chambered aortic ventricle has also been reported in some cases.

Early and late outcomes of surgical repair of double-chambered right ventricle: a single-centre experience.

OBJECTIVE: Double-chambered right ventricle is characterised by division of the outlet portion of the right ventricle by hypertrophy of the septoparietal trabeculations into two parts. We aim to report our experiences regarding the presenting symptoms of double-chambered right ventricle, long-term prognosis, including the recurrence rate and incidence of arrhythmias after surgery. METHODS: We retrospectively investigated 89 consecutive patients who were diagnosed to have double-chambered right ventricle and underwent a surgical intervention from 1995 to 2016. The data obtained by echocardiography, cardiac catheterisation, and surgical findings as well as post-operative follow-up, surgical approaches, post-operative morbidity, mortality, and cardiac events were evaluated. RESULTS: Median age at the time of diagnosis was 2 months and mean age at the time of operation was 5.3 years. Concomitant cardiac anomalies were as follows: perimembranous ventricular septal defect (78 patients), atrial septal defect (9 patients), discrete subaortic membrane (32 patients), right aortic arch (3 patients), aortic valve prolapse and/or mild aortic regurgitation (14 patients), and left superior caval vein (2 patients). The mean follow-up period was 4.86 ± 4.6 years. In these patients, mean systolic pressure gradient in the right ventricle by echocardiography before, immediately, and long-term after surgical intervention was 66.3, 11.8, and 10.4 mmHg, respectively. There were no deaths during the long-term follow-up period. Surgical reinterventions were performed for residual ventricular septal defect (2), residual pulmonary stenosis (1), and severe tricuspid insufficiency (1). CONCLUSION: The surgical outcomes and prognosis of double-chambered right ventricle are favourable, recurrence and fatal arrhythmias are unlikely in long-term follow-up.

Atrial septal defect and an unusual anatomical variant of double-chambered right ventricle presenting with cyanosis.

We treated two patients with unexplained cyanosis, an atrial septal defect and an unusual form of non-obstructive double-chambered right ventricle, with device closure of the atrial septal defect.

Double-Chambered Right Ventricle with Ventricular Septal Defect and Subaortic Membrane- Three-Dimensional Echocardiographic Evaluation.

Double-chambered right ventricle (DCRV) is a rare congenital anomaly in which the right ventricle is divided into two compartments with varying pressures due to an anomalous muscle bundle. Here, we describe a case of an adolescent male with DCRV with associated ventricular septal defect and subaortic membrane. Two-dimensional and three-dimensional transthoracic echocardiography with color flow clearly outlined all the three cardiac anomalies as well as their relationship with each other. The diagnosis was confirmed by cardiac catheterization. The patient underwent successful surgical resection of the anomalous muscle bundle along with repair of the associated anomalies.

A Case Report of Double-Chambered Right Ventricle Associated with Subaterial Ventricular Septal Defect and Rupture of Right Coronary Sinus Aneurysm.

Double-chambered right ventricle (DCRV) is a rare congenital heart disease in which the right ventricle (RV) is divided into two chambers by anomalous muscle bundles. Here, we report a case of DCRV associated with subarterial ventricular septal defect (VSD) and rupture of right coronary sinus aneurysm (RCSA); the patient was diagnosed by echocardiography and later confirmed by surgical operation.

An adult patient of double-chambered right ventricle with ventricular septal defect incidentally diagnosed after presentation with acute myocardial infarction.

The double-chambered right ventricle (DCRV) is a rare congenital heart disease caused by hypertrophic anomalous muscle bands that divide the ventricular cavity, resulting in the formation of a high-pressure proximal chamber and other low-pressure distal one. Because of its evolving nature, its diagnosis is usually made during childhood/adolescence and very rarely during adulthood. It accounts for approximately 0.5-1% of patients with congenital heart disease. We report a case of a 50-year-old man who presented to us with acute inferior wall myocardial infarction (MI). Echocardiography revealed the features of DCRV and ventricular septal defect. Coronary angiogram revealed significant stenosis in right coronary artery. This is the first case where an adult patient with DCRV incidentally diagnosed after presentation with acute MI and had undergone successful coronary angioplasty.

A case of asymptomatic isolated double-chambered right ventricle in an adult man.

Double-chambered right ventricle is a rare congenital heart disease. An anomalous muscle band divides the right ventricle into two cavities, causing a variable degree of obstruction. Most cases of double-chambered right ventricle are diagnosed and treated during childhood, whereas cases of initial presentation during adulthood are very rare. We report a case of asymptomatic isolated double-chambered right ventricle incidentally found in an adult man.

An elderly case of aortic stenosis associated with double-chambered right ventricle.

Double-chambered right ventricle (DCRV) caused by right ventricular outflow tract obstruction, is a developmental cardiac anomaly in which the anomalous muscle bundles divide the right ventricular cavity into two chambers. Few cases with DCRV coexisting with severe aortic stenosis (AS) have been reported. Moreover, adult cases are extremely uncommon.We report an elderly case of a heavy DCRV with severe AS detected by transthoracic echocardiography and catheterization study. An 85-year-old woman with dyspnea on effort and right-sided heart failure was diagnosed with DCRV and severe AS by echocardiography. She underwent a resection of the anomalous muscle of the right ventricle and aortic valve replacement. Her symptoms disappeared postoperatively and was discharged home. At 2 years postoperatively, she was generally well without recurrence of DCRV. In conclusion, the case of DCRV with AS is rare and surgery is useful to relieve the heart failure symptoms and improve the prognosis of both young and adult patients. Learning objective: Double-chambered right ventricle (DCRV) is uncommon in the older population; however, clinicians should consider DCRV in patients with right-sided heart failure as a differential diagnosis. The case of DCRV with aortic stenosis is rare, surgical treatment is particularly useful for these patients to relieve the heart failure symptoms and improve the prognosis in young and adult cases.

The Patient With Right Atrial Thrombus With a Previous History of Double-Chambered Right Ventricular Surgery and Atrial Fibrillation: A Rare Association.

Right atrial thrombosis, which occurs alone, is rare in clinical practice. Its incidence and mechanism are unclear, but susceptibility factors are usually present at its occurrence: ischemic heart disease, heart failure, atrial fibrillation, and chronic kidney disease. Complete isolated right atrial thrombosis rarely occurs. We report here a 47-year-old male patient with a right atrial mass on cardiac ultrasound and chest computed tomography (CT) and a history of previous right heart surgery with type 2 diabetes mellitus and atrial fibrillation, complaining of "chest tightness and shortness of breath after activity for half a month." The patient was admitted to the hospital and underwent right atrial mass resection, and the postoperative pathology showed "right atrial thrombus." As right atrial thrombus is very rare and can be a serious threat to life when it occurs in the heart, the prevention and treatment of right atrial thrombus are very important. Based on the analysis of this case, we believe that for patients with special medical history such as "post right heart surgery and atrial fibrillation," we need to be vigilant for atrial thrombosis.

A case series of double-chambered left ventricle detected by cardiovascular magnetic resonance.

Background: Double-chambered left ventricle (DCLV) is a rare congenital condition, and few case reports are mentioned in literature. Entity, clinical course, and prognosis remain unclear. Cardiovascular magnetic resonance (CMR) is often used for characterization of various congenital heart diseases and can be particularly useful for imaging rare phenomena. Case summary: Three cases of DCLV were detected by CMR within 2 years in our CMR centre with and without associated congenital heart disease or hypertrabecularization. The patients did not suffer from cardiac symptoms despite the presence of premature ventricular complexes in one patient. Diagnosis of DCLV was made based on a first CMR study that was performed in adulthood, although some anatomical suspicion was already raised by previous echocardiography. Discussion: Double-chambered left ventricle, synonymous with the terminus 'cor triventriculare sinistrum', has been previously perceived as a rare phenomenon compared with double-chambered right ventricle. It has to be distinguished from ventricular aneurysm or cardiac diverticulum and is characterized by an additional contractile septum with normal wall structure that divides the LV cavum into two (rather) same-sized chambers. The prognosis seems to be benign, since there is no restriction in functionality and no increased thrombogenicity until adulthood. Consequently, there is (presumably) no need for a tailored therapy-at least in the cases present here. Accordingly, we recommend follow-up CMR examinations for progress monitoring and recognize CMR's significant role for diagnosis and follow-up of cardiac abnormalities in orphan diseases. Due to its broader availability, we expect further cases of DLVC in the future.

Transcatheter Treatment for Double-Chambered Right Ventricle in Patient Presenting Severe Chronic Heart Failure.

We report a case involving a 9-year-old girl with severe biventricular dysfunction due to anomalous right ventricle muscle bands. After a thorough discussion, she underwent a transcatheter stenting procedure for the double-chambered right ventricle. During her follow-up, she regained biventricular function, while remaining asymptomatic, resulting in complete recovery.

Importance of the Multimodality Evaluation of a Double-chambered Right Ventricle for Surgical Indications on Admission for Acute Myocardial Infarction.

We treated a female patient known to have a double-chambered right ventricle (DCRV) who presented with symptoms of an acute myocardial infarction (AMI). Emergent coronary artery catheterization revealed acute right coronary artery (RCA) occlusion and proximal left anterior descending (LAD) stenosis. We performed percutaneous coronary intervention (PCI) for the RCA occlusion. Right heart catheterization revealed a pressure gradient across the mid-RV of 58 mmHg. Computed tomography and magnetic resonance imaging revealed no other congenital cardiac abnormalities. She underwent surgical repair of the RV stenosis and coronary artery bypass surgery for LAD stenosis.