| Combined double-chambered right ventricle with double-chambered left ventricle: a rare anomaly | |
| DOI: 10.5606/e-cvsi.2017.594 | |
| Trushar Gajjar, Nageswar Rao, Neelam Desai | |
| Sri Sathya Sai Institute of Higher Medical Sciences - Prasanthigram, CTVS Department, Prasanthigram, Andhra Pradesh, India | |
| Keywords: Angiography; double-chambered left ventricle; double-chambered ventricle; echocardiography; infundibular stenosis; subaortic stenosis | |
Double-chambered right ventricle with double-chambered left ventricle is a very rare congenital anomaly. An 18-year-old female presented
with dyspnea, chest pain, and palpitation. Diagnosis was made using transthoracic echocardiography and was confirmed by angiography.
Surgical excision of the anomalous muscle bundles in the right ventricular outflow tract along with excision of the fibrous band from
the left ventricle was performed. The postoperative course was uneventful and repeated echocardiogram before discharge showed a right
ventricular outflow tract gradient of 8 mmHg, no gradient across the left ventricular cavity, and improved biventricular functions. The
management strategy is dependent on presence of symptom, associated anomalies and type of pathology. |
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Double-chambered ventricle is a rare congenital
cardiac anomaly, where the ventricular chamber
is wholly or partially partitioned usually by
abnormal muscular ridges or fibrosis.[1-9] Unlike
double-chambered right ventricle (DCRV), division
of the left ventricle (LV) is a rare anomaly.[1-5]
Previously, the description of the double-chambered
left ventricle (DCLV) was given by Gerlis et al.[1]
and Kay et al.[2] Herein, we describe a case of DCRV and DCLV in an 18-year-old female. |
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CASE PRESANTATION
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An 18-year-old female patient presented with
dyspnea, chest pain, and palpitation. The physical
examination revealed a Grade 4/6 ejection systolic
murmur at the left upper sternal border. Chest
X-ray showed a cardiothoracic ratio of 50% with
reduced pulmonary blood flow. Electrocardiogram
showed right ventricular hypertrophy. Transthoracic
echocardiography revealed levocardia and DCRV
with a gradient of 70/58 mmHg associated with
a ledge of muscle tissue in the LV cavity below
the mitral valve (Figure 1a) with 30 mmHg
gradient across the constriction with figure of 8
appearance in apical 4 chamber view. No regional
wall motion abnormality and improved biventricular functions were observed. Cardiac catheterization
revealed a right atrial pressure of 21/0 mmHg with
a mean of 10 mmHg, a right ventricular pressure of
157/10 mmHg, a right ventricular outflow tract of
31/10 mmHg, a pulmonary artery of 33/11 mmHg, a
pulmonary artery wedge of 17/6 mmHg, a LV apex of
162/11 mmHg, a LV outflow tract of 152/76 mmHg,
an aortic pressure of 141/83 mmHg, and a femoral
artery pressure of 157/77 mmHg. The gradient
between the right ventricular body and the outflow
tract was 126 mmHg, while the gradient across the
fibrous ridge between the LV apex and outflow was
10 mmHg. Left ventricular angiography showed
a ledge of tissue in the mid-LV cavity below the
mitral valve (Figure 1b), separating the LV with
a hypertrophied distal chamber and thin-walled
proximal chamber. Right ventricular angiography
revealed the presence of anomalous septal and parietal
bands, producing DCRV with a good-sized main
pulmonary artery and confluent branch pulmonary
arteries. The surgical procedure was performed
through a conventional median sternotomy and
cardiopulmonary bypass was established by aortic and bicaval cannulation. The myocardial protection
was provided with cold blood antegrade cardioplegia.
After cross-clamping the aorta, the right and the left
atrium were opened, and the anatomy was visualized.
The intraoperative findings showed a muscular band
in the LV cavity below the mitral valve (Figure 2a),
and there was a tight fibrous ring with anomalous
septal and parietal bands in the right ventricular
cavity. The ledge of the muscular tissue in the
LV cavity was accessed and excised through a left
atrial-mitral valve route, and the fibrous ring and
muscle bundles in the right ventricle was accessed
and excised through the right atrial-tricuspid valve
route. Weaning from the cardiopulmonary bypass was performed in a usual fashion. Intraoperative
transesophageal echocardiography showed no
gradient across the mid LV cavity, trivial mitral
regurgitation, and an-8-mmHg gradient across
the right ventricular outflow tract with improved
biventricular functions. The postoperative course was
uneventful, and transthoracic echocardiography before
discharge revealed a widely opened right ventricular
outflow tract and mid-LV cavity (Figure 2b) with
a right ventricular outflow gradient of 8 mmHg
and no gradient in the mid-cavity of the LV with
improved biventricular functions. The histopathology
examination of the resected tissue from the LV cavity
showed a fibrous tissue with normal myocardium. |
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Double-chambered left ventricle is a very rare
congenital cardiac anomaly.[1] This pathology is rarer
than DCRV, as described by Kay et al.[2] In addition,
the association of DCLV with DCRV is extremely
rare. Although the etiology of DCLV is less known, it
is thought to be congenital and non-progressive.[1,2,4,5]
Various theories for DCLV have been suggested,
including post-inflammatory pathologies, defects
or hypoplasia of the myocardial wall, and division
by a viable myocardium with fibrous opening and
the endocardial fibroelastosis.[1,2,4,5] Embryologically,
DCLV is caused by the failure of regression of the
fetal trabeculations.[4] Gerlis et al.[1] classified DCLV
into three types (Figure 3): Type 1, where the LV
cavity is divided into two chambers and both chambers
communicate through multiple orifices between
the smooth-walled chamber and the left ventricle
proper. In type 2, the LV cavity is divided by the
hypertrophied muscular wall with an apical portion,
having prominent and enlarged branching sinusoids
lined by thickened endocardium, more commonly
seen with endocardial fibroelastosis. In type 3, the
LV is completely divided into two portions and both
chambers lie side by side. Double-chambered left ventricle can be differentiated from the relatively more common congenital aneurysm and diverticula.[1] It is best differentiated from LV aneurysms and pseudoaneurysms, by the fact that the double-chambered ventricles exhibit normal contractile motions during systole.[1] Ventricular aneurysms also lack complete layering of the ventricular wall, thereby, expanding slightly due to the increased pressure during systole.[1] In general, DCLV is incidentally detected in the course of an evaluation for other cardiovascular abnormalities. The differentiation between DCRV and DCLV is evident, as both have a distinct pathophysiology. The former is more common and often presents with murmur and exertional dyspnea. Several studies have found that DCRV is associated with septal defects, tetralogy of Fallot, and transposition of the great arteries.[5-7] Conversely, the latter one is commonly asymptomatic. In addition, DCRV is often caused by a progressive thickening of the right ventricular septum due to the presence of anomalous muscle bundles, which results in a pressure gradient and formation of two chambers in series. In contrast, the chambers of a DCLV are in parallel and present less of a pressure gradient, as both contract synchronously.[4,6,7] Similarly, in our case, echocardiographic gradient was 30 mmHg; however, catheterization gradient was only 10 mmHg. The DCLV etiology is less well-known, although the anomaly is thought to be congenital and nonprogressive. As this is an extremely rare finding, no definite data regarding the prognosis, outcomes, and potential complications, such as risk of an embolism, in the DCLV are available. It is often believed that DCLV poses little risks to the patient, and treatment is usually guided by the presence of other associated abnormalities.[2,5] Furthermore, the transthoracic and transesophageal echocardiography and computed tomography can aid in the detection of the double-chambered ventricles.[2-5] Transthoracic echocardiography in short-axis inverted views and apical four-chamber views show a transverse muscle band traversing the LV dividing the LV into two halves, producing a typical figure-of-eight appearance, indicating a double-chambered.[8] However, cardiac magnetic resonance imaging allows an improved delineation of this condition, thanks to its higher spatial resolution and the ability for tissue characterization, particularly regarding the differentiation between fibrosis and normal myocardium, which would not be easily achievable with echocardiography[8] or computed tomography.[3] In the literature, several treatment strategies have been described.[2,4,5] In asymptomatic patients without any associated anomaly or non-significant gradient across the DCLV, clinical observation and follow-up are recommended. However, in symptomatic cases with or without gradient across the DCLV or with an associated lesion, surgical excision is indicated. There are several surgical techniques in the literature.[2,4,5] For type 1 and type 2 cases, excision of the obstructive shelf is recommended, while accessory chamber exclusion is indicated for type 3 disease. Due to the rarity of DCLV, on the other hand, few data exist on the treatment approaches and outcomes. Surgical excision of the accessory chamber with interposition patch reconstruction and cardiac transplant are two valid options.[4,5] Although our case did not fit to any of the types described by Gerlis et al.,1 it had a muscular self-like projections producing DCLV which was associated with DCRV. As it was associated with DCRV, presentation was delayed and required excision of an abnormal muscle bridge in the LV cavity along with coring out of the right ventricular cavity. The histopathological examination of the resected tissue showed a fibrous tissue with normal myocardium and no evidence of endocardial fibroelastosis. The etiology probably might be abnormal muscular hypertrophy or poor regression of the fetal trabeculations. In conclusion, double-chambered right ventricle is a rare congenital anomaly; however, the association of this pathology with double-chambered left ventricle is extremely rare. As in our case, both anomalies are responsible for the clinical presentation. The diagnosis can be achieved by transthoracic echocardiography and can be confirmed by angiography and cardiac magnetic resonance imaging. The treatment modalities vary according to symptoms, associated lesions, and type of the pathology.
Declaration of conflicting interests
Funding |
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