Introduction
A wide spectrum of cardiac defects and abnormalities develops by birth. These distinct categories of cardiac diseases are categorized as congenital heart diseases. Congenital heart disease arises due to trouble that occurs during the intrauterine (within the womb) stage, and these abnormalities or disorders can be functional deviation, structural deviation, or both. Certain congenital heart conditions remain dormant without flagging obvious manifestations, while certain conditions are highly critical and entail treatment in the newborn stage. These congenital cardiac conditions are diverse and distinct except for sharing the same period of origin.
What Is a Double Chambered Right Ventricle?
The double-chambered right ventricle is quite a rare inborn condition in which one of the heart’s chambers develops a separation within the chamber, giving rise to two smaller compartments. The condition was first demonstrated and identified in the 1850s. It is reported to have an incidence rate of less than two percent among inborn cardiac defects.
A normal healthy heart encloses four distinct compartments called chambers, two on the right side and the other two on the left side of the heart. On each side, there will be one upper and one lower chamber. The upper ones are technically coined atrium, while the lower ones are denoted as ventricles. While pointing a chamber, to avoid confusion, they are often represented along with their respective side (for example, right atrium, left ventricle).
In the double-chambered right ventricle (DCRV) condition, just as the name implies, the right ventricle, which is usually a single chamber, elicits atypical anatomical deviation. Within the right ventricle, specific muscular blockages partition the single right ventricular cavity into two smaller compartments. Studies have identified that these cavitary obstructions are created by abnormal and atypical muscle fibers, which project an abnormal trajectory in the right ventricle.
DCRV is often seen in alliance with other heart conditions like:
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Ventricular septal defect (defect in the wall separating the two lower chambers).
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Atrial septal defect (defect in the wall separating the two upper chambers).
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Valvar pulmonary stenosis (narrowing down of the pulmonary valve).
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Tetralogy of Fallot (an inborn condition that impairs the heart’s patent blood flow).
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Transposition of the great arteries (positional interchanging of the pulmonary artery and aorta).
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Aortic valve regurgitation (impaired valve function allowing seeping back of blood from the aorta).
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Tricuspid valve regurgitation (defective valve).
What Leads to the Development of a Double Chambered Right Ventricle?
In a double-chambered right ventricle, the right ventricle gets compartmentalized by the enhanced growth of certain muscle fibers. The exact etiology behind the growth of these atypical muscle bundles is still under discussion. Certain hypothesis suggests that this muscle fiber growth is triggered by enhanced blood flow, while some other point out the anatomical displacement of the septal fibers (fibers in the chamber walls) as the etiology that creates a hindrance within the right ventricle giving rise to a double-chambered right ventricle. It compartmentalizes the right ventricle into a low-pressure and high-pressure zone.
What Are the Symptoms Associated With a Double Chambered Right Ventricle?
The double-chambered right ventricle does not flag appreciable manifestation in the milder forms and often remains undetected. However, in severe DCRV, the patient may exhibit obvious symptoms. Some of the critical manifestations associated with double-chambered right ventricle include the following:
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Right Ventricular Angina: The person may develop pain in the chest region, which is often accompanied by tightness and discomfort in the area.
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Dyspnea: The person becomes unable to take deep breaths. The breaths become shallow and short, which gets pronounced during physical excretion.
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Exertional Fatigue: The patient notices intolerance to physical activities and gets easily exhausted and worn up during exertion.
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Right Ventricular Failure: In severe cases, due to the existence of two pressure gradient zones within the right ventricle, it cannot drive blood completely into the pulmonary artery, which in turn impacts the left ventricular loading. Collapse in the right ventricular function leads to right ventricular failure.
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Collapsed Right Ventricular Cardiac Output: As the high-pressure component of the right ventricle is at the proximal portion, the subvalvular region (lower) is subjected to remarkably lower pressure. It directly impacts the heart beating frequency and blood volume driven out from the right ventricle.
How Is the Double Chambered Right Ventricle Diagnosed?
The double-chambered right ventricle is often diagnosed with the assistance of cardiac diagnostic interventions. Echocardiography, electrocardiogram, and cardiac magnetic resonance imaging techniques are employed to establish the diagnosis of a double-chambered right ventricle.
Echocardiography provides a clear picture of the path of blood flow. However, it is least preferred compared to other diagnostic interventions. Electrocardiography detects abnormalities in impulse conduction and helps in identifying right ventricular enlargement.
Cardiac catheterization and cardiac magnetic resonance imaging techniques (MRI) detect the pressure contrast within the right ventricle. Cardiac MRI offers additional information regarding the intensity of pressure gradient. The information gathered from these diagnostic procedures often guides the choice of treatment.
What Are the Treatment Modalities Available for a Double Chambered Right Ventricle?
The treatment for a double-chambered right ventricle is primarily surgical, where the muscle bundle that obstructs or partitions the right ventricle will be resected out. However, not all patients detected with a double-chambered right ventricle are advised to undergo surgical correction. Doctors will consider each case independently and assess the need for surgical correction.
Routinely surgical correction is emphasized more in patients with considerable pressure gradient and potential symptomatic expressions. In mild cases, medicinal therapies can effectively deal with the condition. Pharmacological therapy using beta blockers enhances the tolerability of exercises and physical activities. It also aids in calming down minor symptoms.
In cases where the double-chambered right ventricle is associated with other cardiac anomalies, the treatment plan will be modified accordingly. For patients who develop an associated ventricular arrhythmia (arrhythmic heart-beating), Amiodarone is advised to maintain the rhythm under control.
Conclusion
The double-chambered right ventricle is an anomaly associated with the heart’s right ventricle, where certain muscle fibers split the single right ventricular cavity into two partially connected compartments. This interposed muscle layer acts as a barrier and hinders normal function in the right ventricle. Because of an abnormal bundle of muscle fibers, the pressure in the apical portion of the right ventricle cannot traverse to the inferior zone, thus creating a pressure gradient across the two compartments. Surgical elimination of the obstruction is an effective treatment modality to tackle the problems associated with the double-chambered right ventricle. Symptomatic relief can be obtained with medicinal therapy using beta blockers and antiarrhythmic drugs.
