Abstract Background The double-chambered right ventricle (DCRV) is increasingly recognized as a distinct obstruction entity. The nature of the obstruction is not well defined. Methods Patients with DCRV were prospectively studied during the last 4 years according to the following criteria: 1) pressure gradient by echo Doppler and cardiac catheterization within the right ventricle; 2) angiographic demonstration, and 3) surgical confirmation. Results From March 1997 to March 2001, 10 new cases were included. Age ranged from 2 to 14 years (mean 9.5 ± 4.4 years), weight ranged from 9.9 to 75 kg (mean 23 ± 13.6 kg), and height from 0.85 to 1.48 m (mean 114 ± 19 cm). Systolic gradient by echo Doppler ranged from 20 to 135 mmHg (mean 86 ± 44 mmHg) and by cardiac catheterization, 18 to 130 mmHg (mean 78 ± 35 mmHg). In terms of angiographic findings, in six patients the right ventriculogram showed an oblique and low obstruction; in four patients the obstruction was high and horizontal. With regard to surgical findings, angiographic findings were confirmed by the surgeon except in one patient, in whom both types of obstruction were present. No mortality was observed. With follow-up 4 to 40 months after surgery (mean 24 ± 15 months), 8 of 10 patients were evaluated; all corresponded to class I NYHA. Systolic gradient by echo Doppler ranged from 0 to 11 mmHg (mean 4 ± 6 mmHg). Conclusions DCRV is produced by the following three types of muscular obstructions: low and oblique obstruction; high and horizontal obstruction, and mixed obstruction. Mid-term surgical results are satisfactory.
: A 65-year-old male was referred for to our facility for right and left heart catheterization and coronary angiography for evaluation of a ventricular septal defect and to rule out significant coronary artery disease. During the procedure, the patient was diagnosed with a double-chambered right ventricle (DCRV). Most DCRV cases are diagnosed at a young age; presentation in an adult is extremely rare.
#1Doff B. McElhinney(UCSF: University of California, San Francisco)H-Index: 77
#2Kanu Chatterjee(UCSF: University of California, San Francisco)H-Index: 70
Last. V. Mohan Reddy(UCSF: University of California, San Francisco)H-Index: 59
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Abstract Background . Double-chambered right ventricle is a form of right ventricular outflow tract obstruction that develops over time, often in patients with an abnormally short distance between the moderator band and pulmonary valve. This lesion typically presents in childhood or adolescence and is often accompanied by a ventricular septal defect. Only a handful of previous cases have been described in which double-chambered right ventricle occurred in adulthood. Methods . Since 1992, three p...
Abstract Objective: Our goal was to establish the morphologic nature of the obstructive muscular lesions in double-chambered right ventricle. Methods: We based our morphologic observations on 10 normal hearts and on the surgical findings in 26 patients, aged 0.5 to 24 years, with a mean of 6.9 years (SD 5.8 years). In the normal hearts, we measured the distance from the pulmonary valve to the apex of the right ventricle and from the takeoff of the moderator band to the ventricular apex. From ang...
Abstract The presence of anomalous muscle bundles may produce a pressure gradient between the inflow and outflow portions of the right ventricle, thus resulting in double-chambered right ventricle bearing troublesome clinically in its diagnosis. The aim of the present study was to review the diagnostic criteria. Fifty-two patients with a double-chambered right ventricle were seen during an 8-year period. They ranged in age at the catheterization from 4 months to 17 years (mean 7.5 ± 4.4 years). ...
Between May 1974 and December 1993, 37 patients (0.75%) with a double-chambered right ventricle underwent surgical repair. The patients ranged in age from 11 months to 12 years (mean 4 ± 1.1 years). Cardiac catheterization was performed in 36 patients. The proximal right chamber pressure was 118± 10 mmHg and the mean ventricular gradient pressure was 75 ± 10 mmHg. A ventricular septal defect was present in 36 cases and fixed subaortic stenosis in eight. Longitudinal right ventriculotomy, group I...
Abstract Double-chambered right ventricle (DCRV), a form of right ventricular outflow obstruction that sometimes accompanies a ventricular septal defect (VSD), is associated with superior and rightward displacement of the septal insertion of the moderator band. It was hypothesized that this superior displacement is present and identifiable by echocardiography in patients with a VSD even before right ventricular outflow tract obstruction develops. Eight patients who had a previous echocardiograph...
Double-chambered right ventricle is an uncommon congenital anomaly consisting of obstruction of the trabecular zone of the ventricle by an elevated, hypertrophied moderator band, with or without additional muscle bundles(s). During an 18 month period (August 1984 to February 1986) 12 patients underwent repair of double-chambered right ventricle. Their ages ranged from 13 months to 12 years (mean 4.4 years). Associated lesions were ventricular septal defect in 11 patients (three closed spontaneou...
The first examples of muscular division of the morphologically right ventricle were described by Peacock [15] more than 100 years ago. Keith [13] subsequently described cases with a muscular shelf extending into the apical part of the ventricle. More recently, Brock [5] described cases of Fallot's tetralogy with a muscular diaphragm across the base of the pulmonary infundibulum. Tsifutis et al. [18] and Lucas et al. [14] subsequently reported cases of two-chambered right ventricle due to an anom...
Last. Richard D. Rowe(U of T: University of Toronto)H-Index: 57
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Abstract Right ventricular outflow tract obstruction can complicate the natural history of patients with isolated ventricular septal defect. Although numerous pathologic mechanisms may be responsible for this phenomenon, it is less clear what the frequency is of each of these mechanisms in any cohort of patients studied in a single institution. We identified 20 patients with isolated ventricular septal defect from 1970 to 1981 who acquired a right ventricular outflow tract gradient of 25 mm Hg o...
Abstract Background Data on the features of double-chambered right ventricular (DCRV) in adults are limited. It is also unclear how invasive and Doppler-derived assessments of right ventricular (RV) obstruction in DCRV correlate. Methods Retrospective review of 17 adults (age ≥18 years) diagnosed with DCRV who underwent right heart catheterization at Mayo Clinic, MN between 1990 and 2021. Clinical, echocardiographic, and invasive hemodynamics were abstracted from the medical charts. Results Mean...
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 anato...
Double-chambered right ventricle (DCRV) is a rare condition. Stenosis of DCRV is progressive, and early surgical intervention is recommended for patients whose symptoms and/or pressure overload of right ventricular (RV) inflow are progressive. However, there are few data regarding the postoperative course of DCRV, and the surgical indications for asymptomatic patients remain to be determined. We retrospectively investigated 38 consecutive patients who were diagnosed with DCRV and underwent surgi...
Abstract A 20-month-old male alpaca was referred for evaluation of a cardiac murmur evident since birth. Echocardiography identified a ventricular septal defect (VSD) and a fibro-muscular band causing a stenosis of the right ventricular outflow tract. Right ventricular catheterization and selective angiography confirmed the diagnosis of VSD and double-chambered right ventricle with bidirectional shunting.
Abstract Background Double-chambered right ventricle (DCRV) is a rare form of congenital heart disease. Little is known about the outcome during adult life. Here we report the combined experience of two tertiary Adult Congenital Heart Disease Centres and systematically review the published literature. Results 50 patients (32 female, median age 39years [IQR: 27;53]) with DCRV under follow-up could be identified. A retrospective review of medical records was performed. Almost all patients (96%) ha...
El ventriculo derecho bicameral es una enfermedad poco frecuente, que constituye el 0,5% de las cardiopatias congenitas. Objetivo: Describir el comportamiento clinico y las caracteristicas ecocardiograficas de una serie de 11 pacientes. Metodos: Se estudiaron 11 pacientes con diagnostico de ventriculo derecho bicameral. Se les realizo historia clinica completa, radiografia de torax, electrocardiograma y ecocardiograma transtoracico. Resultados: La mediana de la edad de los pacientes fue de 10 an...
Three eight-week-old golden retriever puppy littermates were evaluated because of left basilar systolic murmurs and were diagnosed with primary infundibular stenosis. Pedigree analysis in this line was also performed to identify a mode of inheritance. All dogs were asymptomatic at the time of diagnosis; two of the three had congenital lesions in addition to primary infundibular stenosis. Two additional affected dogs were identified in the line, and pedigree analysis suggested an autosomal recess...
We present the case of a 67-year-old woman presenting with symptoms of predominantly right heart failure caused by cardiac tumor infiltration. Echocardiography showed almost complete obstruction, particularly of the right ventricular outflow tract. After complete diagnostic workup, treatment with only one course of bendamustine and rituximab resulted in complete resolution of the obstruction. Right ventricular outflow tract restriction due to infiltrative large B-cell lymphoma is a rare finding....
Objective— To report successful surgical treatment of double chambered right ventricle (DCRV) in a cat. Study Design— Clinical report. Animals— Cat with DCRV. Methods— DCRV was diagnosed in a 5-month-old male Maine Coon cat using echocardiography and angiocardiography. At 2 years, despite medical therapy, chylothorax developed. A section of the right ventricular free wall that spanned the fibromuscular obstruction was excised under total venous inflow occlusion (TVIO) using the incised pericardi...
OBJECTIVE: By means of retrospective analysis of our institutional experience, we reviewed the clinical manifestation and outcomes of patients subsequent to surgical repair of double-chambered right ventricle. METHODS: Between 1988 and 2005, we performed surgical repair in 35 of 37 patients diagnosed with double-chambered right ventricle. The patients ranged in age from 4 to 69 years, with a mean of 21.3 years. Most presented in infancy, with initial manifestation of a short systolic murmur in 3...