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RV NonCompaction

 


Right Ventricular Noncompaction Cardiomyopathy (RV-NCC)


Understanding a rare and often under-recognized myocardial disorder


Right ventricular noncompaction (RV-NCC) is an uncommon structural cardiomyopathy characterized by a spongy, trabeculated right ventricular myocardium with deep intertrabecular recesses. While most literature focuses on left ventricular noncompaction (LVNC), isolated or predominant RV involvement is increasingly recognized with modern imaging.



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What Is RV Noncompaction?


RV-NCC occurs when the normal embryological compaction of the ventricular myocardium fails, resulting in:


Excessive trabeculations


Deep recesses that communicate with the RV cavity


A thin compacted epicardial layer


Potential impairment in RV systolic function



It may exist as:


Isolated RV noncompaction (extremely rare)


Biventricular noncompaction (more common)


RV-predominant variant of LVNC




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Etiology & Pathophysiology


During embryogenesis, the ventricles start as a loose mesh of fibers that progressively compact. When compaction is incomplete, noncompacted segments remain.

In RV-NCC:


Compaction failure is usually partial and localized


Genetic contribution is likely, especially in biventricular forms


Associated cardiomyopathies (dilated, arrhythmogenic, LVNC) may coexist




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Clinical Presentation


Symptoms vary widely depending on the degree of RV dysfunction and associated abnormalities:


Dyspnea, fatigue, or reduced exercise tolerance


Arrhythmias, especially PVCs, NSVT, or atrial arrhythmias


Right-sided heart failure in advanced disease


Syncope or palpitations


Thromboembolic events (rare but possible)



Some patients remain asymptomatic and are diagnosed incidentally on imaging.



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Imaging: How Is RV-NCC Diagnosed?


1. Echocardiography


Echo is usually the first test but RV noncompaction can be challenging to visualize due to complex RV geometry.


Typical findings include:


Prominent trabeculations along RV apex and free wall


Deep recesses filled with color Doppler flow


Reduced RV free-wall or global RV function


RV dilation in advanced cases



No universally accepted echo criteria exist for isolated RV-NCC, so diagnosis is often supportive.



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2. Cardiac MRI (CMR) — Gold Standard


CMR provides high-resolution visualization of RV trabeculations.


MRI features include:


Non-compacted/compacted (NC/C) ratio >2.3 in end-diastole (extrapolated from LV criteria)


Excessive trabecular mass


Deep recesses with delayed enhancement if fibrosis is present


RV dilation or systolic dysfunction



CMR is critical when echo is inconclusive.



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Differential Diagnosis


RV-NCC can mimic other conditions with prominent trabeculations:


Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC)


Dilated cardiomyopathy with RV involvement


Post-athletic remodeling


Hypertrabeculation in anemia, pregnancy, or athletes



A careful clinical and imaging correlation is essential.



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Complications


Ventricular arrhythmias


Progressive RV systolic dysfunction


Biventricular failure in combined disease


Rarely, thromboembolism



Risk stratification is crucial, especially for arrhythmic events.



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Management


There is no disease-specific therapy; treatment focuses on:


1. Heart Failure Management


Diuretics for volume overload


Guideline-directed therapy if LV dysfunction coexists


Oxygen therapy or PH management if secondary pulmonary hypertension develops



2. Arrhythmia Control


Beta-blockers or antiarrhythmics


Electrophysiology evaluation for recurrent VT


ICD in high-risk patients (especially with biventricular involvement)



3. Anticoagulation


Considered if:


Severe RV dilation


Depressed RV function


History of thromboembolism


Concomitant LVNC or LV dysfunction



4. Lifestyle


Avoid competitive sports in arrhythmogenic phenotype


Family screening if a genetic form is suspected




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Prognosis


Prognosis varies:


Asymptomatic patients may remain stable for years


Others progress to RV or biventricular failure


Arrhythmias can be life-threatening without proper monitoring


Outcomes depend heavily on associated LV involvement and fibrosis on CMR




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Key Takeaways


RV-NCC is rare and often misdiagnosed.


CMR is the most accurate imaging tool for diagnosis.


Management focuses on heart failure therapy, arrhythmia prevention, and risk stratification.


Early recognition is important, especially in patients with unexplained RV dysfunction or ventricular arrhythmias.

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