Double Chambered Right Ventricle with Ventricular Septal Defect in Adults: Case Series and Review of the Literature

Double-chambered right ventricle (DCRV) is an uncommon congenital anomaly in which anomalous muscle bands divide the right ventricle into two chambers; a proximal high-pressure and distal low-pressure chamber. It may be associated with mid right ventricular obstruction. It is commonly associated with other congenital anomalies, most frequently perimembranous ventricular septal defect (PM-VSD). We herein present 5 adult patients with concomitant DCRV and PM-VSD who varied in their symptomatic presentations and the ways of management.

Double-Chambered Right Ventricle with Transient 2:1 Atrioventricular Block: A Rare Presentation.

We report herein a case of 35-year-old male with a history of complex congenital heart disease, consisting of double-chambered right ventricle (DCRV) with ventricular septal defect and presenting with transient 2:1 atrioventricular (AV) block on electrocardiogram. This unique presentation has not yet been described in literature, hence it was worth describing this rare complication of DCRV.

Forma de presentación y evolución del ventrículo derecho bicameral / Presentation form and evolution of double-chambered right ventricle

Introducción: El ventrículo derecho bicameral es una enfermedad poco frecuente, que constituye el 0,5% de las cardiopatías congénitas. Objetivo: Describir el comportamiento clínico y las características ecocardiográficas de una serie de 11 pacientes. Métodos: Se estudiaron 11 pacientes con diagnóstico de ventrículo derecho bicameral. Se les realizó historia clínica completa, radiografía de tórax, electrocardiograma y ecocardiograma transtorácico. Resultados: La mediana de la edad de los pacientes fue de 10 años y el 55% perteneció al sexo masculino; 4 no presentaron síntomas y el diagnóstico fue por ecocardiografía. El ecocardiograma demostró la presencia de un ventrículo derecho dividido en 2 porciones por una banda fibromuscular. Las lesiones asociadas más frecuentes fueron: comunicación interventricular, foramen oval permeable, estenosis subvalvular aórtica y tetralogía de Fallot; 7 pacientes fueron a tratamiento quirúrgico y actualmente 6 de ellos se encuentran en clase funcional NYHA I. Conclusiones: Este trabajo reviste especial importancia, porque es la serie de casos más grande en México y los resultados obtenidos son comparables con los estudios reportados en la literatura médica. El síntoma clínico predominante fue el deterioro de la clase funcional NYHA. Las manifestaciones clínicas se presentan desde la lactancia o más tardíamente hasta la vida adulta, por lo que consideramos que el grado de obstrucción puede tener un comportamiento progresivo. El ecocardiograma es el método diagnóstico de elección en estos casos. El 91% de los pacientes que recibieron tratamiento quirúrgico con ventrículo derecho bicameral tienen resultados hemodinámicos y funcionales excelentes en el seguimiento a mediano plazo.

Introduction: The double-chambered right ventricle (DCRV) is an uncommon congenital heart disease; it represents the 0,5% of these congenital cardiopathies. Objective: To describe the clinical and echocardiographic characteristics of a series of 11 patients. Methods: Eleven patients with DCRV were studied. A complete medical history, chest radiograph, electrocardiogram, and transthoracic echocardiography were performed. Results: The median age of studied patients was 10 years. The 36.45% of cases were asymptomatic and the diagnosis was made by echocardiography. Other cases had weakness during food in the lactancy stage and worsening of NYHA functional class in older children. The echocar-diogram showed double-chambered right by a muscular band in all studied patients. The most frequent associated anomalies were: ventricular septal defect, patent foramen ovale, subvalvular aortic stenosis, and Fallot's Tetralogy. Seven patients went to surgical treatment and now 6 of them are in NYHA functional class I. Conclusions: This paper has a special importance, because is the biggest series of patients with double-chambered right ventricle in Mexico and the results are comparable to the studies reported in the literature. The worsening of the NYHA functional class was the predominate symptom. The clinical manifestations can be presented from the lactancy to adulthood and the degree of obstruction could have a progressive character. The echocardiography is the method of choice in the diagnosis of these patients. The 91% of patients with DCRV who went to surgical treatment had excellent hemodynamic and functional results in the mid-follow-up.

Spontaneously Healed Membranous Type Ventricular Septal Defect with Malaligned Interventricular Septal Wall and Double-Chambered Right Ventricle in a 56-Year-Old Patient

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.

Two Cases of Double-Chambered Right Ventricle without Other Congenital Cardiac Anomalies

Double-chambered right ventricle (DCRV) is a congenital cardiac anomaly in which the right ventricle is divided into two chambers by anomalous hypertrophied muscle bundles that cross the right ventricular cavity. DCRV may be associated with other congenital cardiac anomalies, most commonly with ventricular septal defect. We report two cases of DCRV without other congenital cardiac anomalies with review of literatures. We performed echocardiography, cardiac catheterization and right ventricular angiogram to confirm the diagnosis.

Noonan Syndrome with Double-Chambered Right Ventricle and Atrial Septal Defect: 1 Case Report / 대한흉부외과학회지

Noonan syndrome is characterized by typical facies, congenital heart defect, and some clinical features similar to Turner syndrome, but with normal chromosomes. The most commonly associated cardiac defects are pulmonary valvular stenosis and strial septal defect. We experienced a case of Nonan syndrome associated with pulmonay valve stenosis with double-chambered right ventricle and atrial septal defect and cryptorchidism. Pulmonary valvotomy was done through transannular incision. Hypertrophied muscle bundles were excised. Atrial septal defect was closed directly. RVOT was reconstructed with pericardial transannular patch. Orchiopexy was performed simultaneously without any problem.

A Case of Double Chambered Right Ventricle with Congenital Right Ventricular True Diverticulum

Congenital diverticula of the cardiac ventricle have been reported as arising either from the left ventricule or, rarely from both ventricules. A diverticulum arising from the right ventricle alone is very rare. Double chambered right ventricle(DCRV) was first described in 1962 by Lucas et al, and account for 1.5% of congenital heart disease. Right ventricular diverticulum are associated with right ventricle outlet obstructive disease, tetralogy of fallot, double outlet right ventricle, pulmonic stenosis, ventricular septal defect, left ventricule-right atrium communication, persistant truncus arteriosus. DCRV are freqently associated with ventricular septal defect(80%), pulmonic stenosis(33%), renal anormaly(43%). Treatment consist of suture of diverticulum and resection of septum. We report a case of double chambered right ventricle with congenital right ventricular true diverticulum in 62 years of female patients admitted due to anterior chest pain and mild exertional dyspnea.

DOUBLE-CHAMBERED RIGHT VENTRICLE: ITS PATHOANATOMIC CHARACTERISTICS AND TREATMENT PROBLEMS / 解放军医学杂志

In the past ten years, 52 patients with double-chambered right ventricle (DCRV) were operated on in Xijing Hospital. They constituted 2.2% (52/1881) of all patients with congenital heart diseases. Of them, there were 32 males and 20 females, whose ages ranged from 4.5 to 30 years old. In one patient there was pure DCRV, and the remaining 51 patients DCRV was associated with other congenital cardiac anomalies, in which VSD was the most common (50), followed in order by pulmonary stenosis (6), aortic valve prolapse (4), atrial septal defect (2), and subaortic stenosis (1). The repair of DCRV and other intracardiac defects was done through the right ventricolotomy in 47 cases and through the right ventricolotomy plus atriotomy in 2 cases. In another 2 cases the operation was done via the right atriotomy and 1 case pulmonary arteriotomy, and only VSDs were closed, but DCRVs were missed. The major postoperative complications included cardiac arrhythmia (11) and low output syndrome (8). Three patients died, with the operative mortality of 5.8%. In this series, according to characteristics of the anomalous muscle bundle and its resulting obstruction, we divided 52 case DCRVs into two types: fibromuscular diaphragmatic type (24 cases, 44.7%) and muscular bundle-gap type (28 cases, 55.7%). In both types, there were not only hypertrophied anmalous muscle bundles on the septal side, but also hypertro-phied ventricoinfundibulum fold on the parietal side. In a few cases, the ventricoinfundibulum fold was more hypertrophic than the anomalous muscle bundle on the septal side.

Surgical treatment of double-chambered right ventricle: a report of 95 cases / 解放军医学杂志

Objective To summarize the experience in diagnosis and surgical treatment of double-chambered right ventricle (DCRV). Methods From 1990 to 2001, 95 patients with DCRV received surgical correction, including 56 males and 39 females, with an age ranging from 1 to 48. 82 cases had other cardiac abnormalities, 56 of whom had ventricular septal defect (VSD). Right atrium incision was made in 8 patients, right ventricular infundibular incision in 30, and both right atriotomy and ventriculotomy in 57. Results Muscular ring was found in 61 patients, and muscular shelf in 34. No death occurred. Preoperatively, 15 patients and 3 patients were misdiagnosed by echocardiography as VSD and pulmonary stenosis, respectively. The diagnosis was corrected during operation. Conclusions DCRV was often found to be complicated with other cardiac disorders. Echocardiography was the main diagnotic method, but the misdiagnosis was not uncommon. So it was very important to make surgical exploration, especially on tricuspid valve and pulmonary valve during intraventricular operations. Right ventricular infundibular incision was convenient and dependable.