Biventricular arrhythmogenic cardiomyopathy

Article written and reviewed by Dr Luca Moretti

Biventricular arrhythmogenic cardiomyopathy is the phenotype in which the scarring process and electrical vulnerability clinically demonstrably involve both the right and the left ventricle. It is not sufficient for both to show any abnormality: the findings must be established as belonging to the same myocardial process rather than representing nonspecific hemodynamic consequences. The core of the diagnosis is therefore independent ventricular involvement.

The international literature often uses the acronym ABVC, for arrhythmogenic biventricular cardiomyopathy, or BIV. The phenotype may be balanced, with similar severity in the two ventricles, or may show right- or left-sided predominance while retaining evidence of disease in the other ventricle. These configurations are not immutable categories, because the relationship between the ventricles may change during follow-up. Phenotypic geometry is a temporal description.

The biventricular form does not necessarily coincide with the end stage of arrhythmogenic right ventricular cardiomyopathy. Some patients do indeed progress from the right ventricle to the left, but in others the damage is bilateral from onset or arises from a genotype prone to affecting both sides. Always regarding it as advanced ARVC would obscure forms caused by DSP, DES or other genes with early balanced presentations. Disease history matters as much as the current image.

Similarly, severe dilated cardiomyopathy with secondary right ventricular dysfunction does not thereby become biventricular arrhythmogenic cardiomyopathy. The right ventricle may fail because of pulmonary hypertension, tricuspid regurgitation and ventricular interdependence without having a characteristic scar of its own. A diagnosis of ABVC requires coherent right-sided structural or morphofunctional abnormalities together with left-sided criteria. Causality of the finding prevents overdiagnosis based on end-stage heart failure.

The clinical relevance stems from the combination of two problems. Scar tissue in both ventricles can generate tachycardias with different circuits and morphologies, while loss of contractile mass simultaneously reduces the reserve of the pulmonary and systemic circulations. Arrhythmia and heart failure may potentiate each other, but do not always progress in parallel. An electrical-hemodynamic disease therefore requires separate assessments and therapeutic goals.

The true frequency remains uncertain. Historical series based on the 2010 criteria mainly recognized the right-sided component, others classified cases with evident left ventricular dysfunction as DCM, and many patients did not undergo contrast-enhanced CMR. The Padua criteria and the subsequent European consensus made visible a spectrum that had previously been fragmented. Epidemiologic data are nevertheless influenced by center selection and genetic composition.

Pathology, pathophysiology and progression

In the right ventricle, fibrous or fibrofatty replacement tends to begin in the epicardium and may become transmural with progression. The free wall, inflow tract, outflow tract and apex may be involved in a heterogeneous distribution. Thinned areas alternate with residual myocardium and may develop aneurysms or dyskinesia. Tissue discontinuity creates the substrate for re-entry circuits.

In the left ventricle, injury frequently affects the subepicardial or midmyocardial layer, especially in the inferolateral wall, and may extend across multiple segments to a ring-like configuration. Scar may be extensive in a nondilated chamber with still relatively preserved global function. This discordance makes an approach centered on volumes insufficient. The left ventricular substrate must be sought with tissue characterization.

The two distributions reflect different thickness, stress and architecture, but share cardiomyocyte loss and scar repair. Islands of viable tissue traversed slowly by the impulse favor monomorphic tachycardias; more diffuse dispersion of conduction and refractoriness may facilitate ventricular fibrillation. Adrenergic activity further reduces stability. Arrhythmogenic fibrosis is therefore not simply a passive end result.

Right ventricular impairment reduces left ventricular filling and raises venous pressures, whereas left ventricular impairment increases pulmonary pressure and right ventricular afterload. The septum, pericardium and pulmonary circulation thus transfer injury from one chamber to the other. Functional tricuspid and mitral regurgitation amplify congestion. Ventricular interdependence makes the clinical picture more severe than the sum of two ejection fractions.

Desmosomes withstand intercellular forces and organize signaling complexes. When a variant impairs their components, mechanical stress may promote cardiomyocyte detachment, death and replacement; gap junctions and ion-channel distribution also change, explaining early electrical abnormalities. Pathogenesis therefore extends beyond a purely structural model. Early uncoupling may become apparent before macroscopic scar.

In non-desmosomal genes, abnormalities of intermediate filaments, the cytoskeleton, calcium homeostasis or the nuclear envelope converge on cellular fragility and death. DES is particularly instructive because some variants produce characteristic biventricular involvement with conduction disorders; FLNC, PLN and LMNA can generate overlapping arrhythmogenic-dilated phenotypes. The molecular mechanism modifies the trajectory despite a shared final phenotype.

Inflammatory foci are documented in a proportion of affected hearts and may be more frequent in forms with extensive involvement. Episodes of pain and elevated troponin, particularly associated with DSP, may precede an increase in scar. In each case it has not yet been established whether inflammation initiates the injury or responds to necrosis. The inflammatory component requires caution before prescribing immunosuppression.

Intense exercise increases right ventricular volume load, wall stress and catecholamines. In desmosomal variant carriers, endurance exposure is associated with higher penetrance, arrhythmias and heart failure; reducing activity may decrease events without eliminating them. The evidence does not allow a universally safe dose to be defined for every genotype. Exercise modulation is translated into an individualized prescription.

Progression may be continuous, episodic or almost quiescent. A patient who is initially right-dominant may accumulate left ventricular scar; another with ALVC may develop right ventricular dyskinesia and dilatation, whereas a balanced form may deteriorate in parallel. A change in label does not represent a new disease, but evolution in its distribution. Serial remodeling is part of diagnosis and prognosis.

Genetics and familial spectrum

Autosomal dominant forms are the most frequent in clinical families, with a 50% probability of transmission in each pregnancy and incomplete penetrance. The same variant may produce ARVC, ABVC or ALVC in different relatives, showing that the gene alone does not determine the chamber affected. Age, sex, exercise and modifiers contribute to expression. Intrafamilial variability must be made explicit in counseling.

DSP is strongly associated with left-sided and biventricular involvement, extensive scar, arrhythmias and phases of myocardial injury. Dominant loss-of-function variants may have exclusively cardiac manifestations, whereas biallelic conditions may add woolly hair, palmoplantar keratoderma or severe cardiocutaneous phenotypes. The absence of skin signs therefore does not substantially reduce the likelihood of desmoplakin cardiomyopathy.

DSG2 and DSC2 may cause right-sided or biventricular involvement. Some biallelic DSC2 variants have been associated with early onset and bilateral disease, even without an evident cutaneous phenotype. PKP2 remains predominantly linked to the right-sided form, but left ventricular progression is possible. Frequencies depend on the population and founder effects. Allelic dose may influence severity without constituting an absolute rule.

Recessive syndromes caused by JUP or DSP illustrate the importance of examining the skin, teeth and hair. In Naxos disease and Carvajal syndrome, extracardiac signs may precede cardiomyopathy and allow surveillance during childhood. However, nomenclature and ventricular distribution vary with the gene and variant. The cardiocutaneous phenotype should lead to multidisciplinary care.

DES links Z-discs, myofibrils, the nucleus and organelles. Specific variants, such as p.Leu115Ile described in some families, have been associated with an arrhythmogenic biventricular form with scar and conduction disorders. Other variants produce DCM, restrictive cardiomyopathy or skeletal myopathy. A DES finding requires neuromuscular assessment and variant-specific interpretation. Cardiac desminopathy is not phenotypically uniform.

FLNC, PLN and LMNA enter the spectrum with different combinations of scar, arrhythmias, conduction block and dysfunction. Truncating FLNC variants are linked to fibrosis and arrhythmic risk, PLN p.Arg14del has specific data in populations with a founder effect, and LMNA often presents with early conduction disease. Classifying all of them as arrhythmogenic genes is clinically useful but biologically incomplete. Gene-specific stratification preserves details that the phenotype alone loses.

The presence of two pathogenic variants, either biallelic or in different genes, may be associated with early or severe disease in some cohorts. However, early studies tended to include as a second mutation variants that were later reclassified, creating a risk of overestimating digenic inheritance and compound heterozygosity. Only rigorously classified variants and coherent segregation justify such conclusions. Genetic complexity must not become an automatic explanation.

The diagnostic panel is selected after counseling and includes genes with robust associations, copy-number variant analysis and adequate coverage. A variant of uncertain significance does not confirm the diagnosis, does not predict which ventricle will become affected and must not be used for irreversible interventions. Periodic re-evaluation and study of affected relatives may add evidence. Interpretive discipline protects the entire family from erroneous labels.

When testing in the proband is negative, a genetic origin is not excluded and relatives remain under surveillance if the family pattern is credible. When a causal variant is present, cascade testing identifies those who require longitudinal follow-up. Non-carriers can generally leave the specific pathway if that variant explains the family. The family result has a different meaning from a negative panel in an isolated case.

Clinical manifestations and initial evaluation

Arrhythmias may originate from either ventricle. Premature beats or tachycardias with left bundle branch block morphology suggest a right ventricular origin, whereas right bundle branch block morphology points toward the left ventricle; axis and transition further localize the site. In the biventricular form, both may coexist in the same patient or appear at different times. Morphologic plurality reflects a distributed substrate.

Palpitations, presyncope and syncope are common but nonspecific. Rhythm documentation during symptoms is ideal; however, sudden unexplained syncope in the presence of biventricular scar should be considered potentially arrhythmic. Vasovagal, orthostatic and neurologic causes remain possible. Arrhythmic probability arises from integration, not from the isolated description of the episode.

Cardiac arrest may occur before advanced heart failure, especially during adrenergic activity. Conversely, some patients initially present with congestion and only later develop sustained arrhythmias. This diversity precludes using functional class as a surrogate for the risk of sudden death. The electrical axis and the hemodynamic axis must be stratified separately at every visit.

Right ventricular impairment causes edema, jugular venous distention, hepatomegaly, ascites, early satiety and reduced renal perfusion. These signs may dominate even when left ventricular dysfunction is present because the venous system poorly tolerates chronic pressure elevation. Troponin may be normal, whereas natriuretic peptides reflect stress without identifying the chamber involved. Systemic congestion requires serial clinical examination.

Left ventricular involvement causes dyspnea, orthopnea, reduced exercise tolerance and pulmonary congestion. If both chambers are impaired, low output may limit pulmonary signs despite a severe prognosis. Low blood pressure, cold extremities, worsening renal function and confusion are late signs. Effective cardiac output must be assessed beyond the absence of crackles.

Atrial fibrillation and flutter may reflect atrial dilatation and chronic pressure elevation and may also worsen filling. In stiff ventricles or low-output states, loss of atrial contraction is particularly poorly tolerated. Rate or rhythm control and anticoagulation follow individual indications. The atrial burden contributes to heart failure but does not define the cardiomyopathy.

Chest pain with increased troponin may indicate an inflammatory phase, especially in DSP genotypes, but requires exclusion of coronary emergencies and other myocarditides. CMR may show edema superimposed on chronic scar. Recurrence and pedigree increase the likelihood of genetic disease. A myocarditis-like presentation must not erase etiologic analysis.

Physical examination looks for skin signs, muscle weakness, conduction disorders, functional murmurs and systemic manifestations of phenocopies. An assessment limited to the heart would miss clues to cardiocutaneous syndromes, desminopathy, sarcoidosis or neuromuscular disease. Medications, alcohol, substances and infections are also reconstructed. The extended phenotype narrows the diagnostic pathway.

ECG, imaging and diagnostic criteria

The ECG may contain signs from both sides: T-wave inversion from V1 to V3 or beyond, terminal delay of right ventricular activation, low peripheral voltages and inferolateral inversion. The epsilon wave has interobserver variability and should not be overread. No electrical combination replaces structural evidence. A biventricular ECG is suggestive when it converges with imaging and arrhythmias.

Holter monitoring quantifies premature beats, nonsustained tachycardias, atrial arrhythmias and different morphologies. Repeated recording can identify an increasing burden or a new focus, whereas devices and implantable loop recorders answer selected questions. Ectopic frequency varies with medications and activity. A serial rhythm profile is more robust than a random single day.

Echocardiography measures right ventricular outflow tract diameters, area, fractional area change, TAPSE, strain and regional wall motion, together with left ventricular volumes and function. It also assesses regurgitation, atria and pulmonary pressure. The right ventricle is geometrically complex and load dependent, so a single measurement is insufficient. Integrated right ventricular function requires multiple views and parameters.

CMR provides more reproducible indexed volumes and ejection fractions and shows aneurysms, dyskinesia and areas of hypokinesia. In the right ventricle, the thin wall and epicardial fat make LGE less sensitive and more prone to artifacts; confirmation in two planes is essential. In the left ventricle, scar is visualized more reliably. Biventricular CMR combines anatomy and tissue characterization without being infallible.

The left ventricular pattern may be an inferolateral subepicardial or midmyocardial stria, multifocal or ring-like. In the right ventricle, unequivocal LGE must be distinguished from epicardial fat and the region of the tricuspid valve. T1 and T2 mapping help identify diffuse fibrosis or inflammatory activity, but do not have universal diagnostic thresholds. Expert interpretation limits false-positive and false-negative findings.

The 2010 Task Force Criteria primarily quantified ARVC morphology, function, histology, ECG findings, arrhythmias and family history. They were relatively specific for the classic right-sided phenotype but had no criteria for left ventricular scar, resulting in underdiagnosis of biventricular forms. Advances in CMR made this gap evident. The historical framework explains why older reports may require reassessment.

The 2020 Padua criteria applied six parallel categories to the two ventricles and introduced LGE as structural evidence. To classify the biventricular form, they required at least one right-sided morphofunctional or structural criterion and at least one corresponding left-sided criterion, with overall grading based on major and minor criteria from different categories. Evidence in both ventricles prevented one chamber from being inferred solely from dysfunction of the other.

The European Task Force consensus published in 2024 refined the Padua criteria. In the left ventricle, it regards a subepicardial or midmyocardial stria-pattern LGE extending to at least three segments of the bull's-eye model, contiguous in the same short-axis section with a ring-like distribution or noncontiguous, and confirmed in two orthogonal views, as a major criterion; involvement of one or two segments is instead given minor weight, excluding focal, patchy or junctional distributions. In the right ventricle, it recognizes unequivocal LGE as a minor criterion and retains a major role for histologic fibrous replacement. Orthogonal confirmation supports tissue specificity.

The updated classification continues to require that the biventricular phenotype be based on morphofunctional or structural abnormalities attributable to each ventricle. The definite category requires two major elements, or one major plus two minor elements, or four minor elements. The borderline category requires one major plus one minor or three minor elements; the possible category corresponds to a single major or two minor elements. The elements must come from different categories and, at every grade, morphofunctional or structural evidence must remain documented in both ventricles. The diagnostic hierarchy prevents duplicate findings from the same category from simply being added together.

Genetics and family history constitute one of the categories, but a variant alone does not demonstrate biventricular disease. The most recent consensus assigns greater weight to a pathogenic variant and lesser weight to a likely pathogenic variant in an associated gene, then calls for definition of etiology and distinction among genetic forms, acquired forms and phenocopies. A VUS does not contribute causally. The gene does not automatically localize the injury.

Biopsy is reserved for scenarios in which the result may change treatment, such as suspected sarcoidosis, rapidly progressive myocarditis or infiltrative disease. The subepicardial and patchy distribution creates false negatives; a right ventricular sample does not necessarily represent the left ventricle. Imaging or electroanatomic mapping may guide the site. The biopsy strategy must balance diagnostic yield and procedural risk.

Right heart catheterization does not diagnose ABVC, but clarifies pressures, cardiac index, pulmonary vascular resistance and the contribution of both ventricles in advanced heart failure. It is useful when symptoms and imaging are discordant or before transplantation and mechanical support. Results depend on volume status and therapy at the time of examination. Invasive hemodynamics addresses functional severity, not the etiology of the scar.

Differential diagnosis

DCM with secondary right-sided failure is the main pitfall. Midmyocardial septal scar, marked left ventricular dilatation and right ventricular impairment proportional to pulmonary pressure and regurgitation favor DCM, whereas right ventricular dyskinesia, extensive left ventricular subepicardial scar and arrhythmias arising from both chambers support ABVC. Genetic overlap precludes absolute rules. Hemodynamic proportionality helps distinguish cause from consequence.

Sarcoidosis can cause multifocal scars, atrioventricular block, tachycardias and dysfunction of both ventricles. Basal septal LGE, focal PET uptake, extracardiac involvement and biopsy provide direction, but sensitivity and specificity are imperfect. Because immunosuppression may modify sarcoid activity, exclusion is not academic. Granulomatous disease should be sought particularly in sporadic cases with early conduction disease.

Myocarditis and inflammatory cardiomyopathy may produce bilateral injury, arrhythmias and LGE. An infectious trigger, acute edema and recovery favor an acquired form, whereas recurrence, progression, pedigree and a causal variant support a genetic substrate. The two conditions may also coexist, because inflammation and DSP cardiomyopathy are not mutually exclusive. Etiologic follow-up avoids premature conclusions.

Pulmonary hypertension, chronic embolic disease, shunts, Ebstein anomaly and absence of the pericardium may dilate or deform the right ventricle. Concomitant left-sided heart disease may simulate involvement of both chambers. Echocardiography, CT, catheterization and congenital history resolve most cases. Load-related disease must be proportional to the observed abnormality before ABVC is invoked.

The athlete's heart develops harmonious ventricular dilatation, occasionally borderline resting function and increased right ventricular volumes. An appropriate contractile response, absence of significant scar and complex arrhythmias, and partial regression with detraining favor adaptation. Sport does not explain aneurysms or extensive stria-pattern LGE. Physiologic remodeling is assessed with sport-specific nomograms.

Ischemic heart disease, Chagas disease, muscular dystrophies, laminopathies and toxic injury may involve both ventricles. Coronary patterns, geographic origin, neurologic signs, exposures and conduction disorders direct targeted testing. The biventricular label must not interrupt etiologic investigation. A descriptive diagnosis acquires clinical value only when linked to the most plausible cause.

Identification of fat in the right or left ventricle is not sufficient. Epicardial fat, aging, obesity and artifacts can mimic infiltration, whereas fibrosis without evident adipose tissue is common in arrhythmogenic cardiomyopathies. LGE is also not etiologically specific. Multiparametric convergence protects against diagnoses based on a striking but isolated image.

Arrhythmic risk, ICD and ablation

Secondary prevention is indicated after ventricular fibrillation, resuscitated cardiac arrest or hemodynamically poorly tolerated sustained ventricular tachycardia when there is no completely reversible cause. A well-tolerated sustained tachycardia on a biventricular substrate still requires individualized assessment for ICD, considering recurrence, scar and progression rather than applying an automatic rule. A preventive ICD terminates arrhythmias but, over time, exposes patients to infection, inappropriate shocks and lead problems.

For primary prevention, suspected arrhythmic syncope, nonsustained tachycardia, frequency and complexity of premature ventricular beats, right and left ventricular function, scar extent, genotype, age and family history are considered. Biventricular disease may combine markers of electrical and heart-failure risk, but there is no single algorithm validated for every variant. Periodic reassessment is necessary because risk changes with the phenotype.

The ARVC risk calculator was developed predominantly in the right-sided phenotype and is not automatically applicable to ABVC. In particular, the weight of scar and left ventricular function varies among DSP, PKP2 and other genotypes. Using an unvalidated number can distort both the decision to implant and the decision not to implant. The derivation population must always be compared with the actual patient.

A left ventricular ejection fraction above 35% does not guarantee low risk when extensive scar, arrhythmias or a vulnerable genotype are present. Conversely, reduced function does not automatically mandate an ICD when life expectancy, comorbidities or end-stage heart failure make benefit unlikely. The decision integrates arrhythmic prevention and competing mortality. Risk beyond ejection fraction is particularly important in the arrhythmogenic spectrum.

Beta-blockers are used to reduce adrenergic stimulation and to treat arrhythmias or heart failure. Sotalol and amiodarone may reduce tachycardias and shocks, but they do not eliminate the substrate and require monitoring of QT interval, renal function, thyroid, liver and lung according to the drug. Choice also depends on ventricular function. An antiarrhythmic drug complements the device and ablation.

Ablation is indicated for recurrent tachycardias, electrical storm or shocks despite therapy. The right-sided substrate is often epicardial and the left-sided substrate may be inferolateral subepicardial; combined endocardial and epicardial approaches are therefore common in expert centers. Pericardial adhesions or previous procedures alter the strategy. Bilateral mapping searches for multiple circuits without assuming a single chamber.

Acute success of ablation does not allow ICD removal when the indication persists. New scars or conduction channels may generate recurrences with a different morphology. Monitoring and exercise restriction continue. Substrate progression distinguishes this procedure from definitive elimination of a tachycardia in a structurally normal heart.

Device programming balances timely detection against prevention of unnecessary therapies. Antitachycardia pacing can terminate many monomorphic tachycardias, whereas zones set too low increase the risk of interventions for self-terminating or supraventricular arrhythmias. Pacing and resynchronization needs influence the type of system. Individualized programming requires knowledge of previously documented tachycardias.

Heart failure, exercise and advanced therapies

Congestion is treated with diuretics titrated according to weight, edema, blood pressure, renal function and electrolytes. In right-sided failure, excessive volume removal may reduce left ventricular preload and cardiac output, whereas an insufficient dose maintains hepatic and renal congestion. The optimal point changes over time. Volume balance requires close clinical follow-up during unstable phases.

When left ventricular function is reduced, an ARNI or renin-angiotensin system inhibition, beta-blocker, mineralocorticoid receptor antagonist and SGLT2 inhibitor are used according to guidelines if tolerated. Evidence derives from heart failure in general rather than from large ABVC trials, but biologic and clinical benefit supports their application. Foundational therapy should not be omitted because the phenotype is rare.

Right ventricular dysfunction limits titration through hypotension, renal impairment and low output. No pharmacologic therapy can specifically reverse fibrofatty replacement; pulmonary vasodilators are indicated only in appropriate forms of pulmonary hypertension, not for passive pressure elevation caused by left-sided heart disease. Right ventricular physiology guides drugs and doses.

Tricuspid and mitral regurgitation are often functional. Their isolated correction in advanced cardiomyopathy must be assessed carefully because the valve may be a marker of remodeling rather than the primary cause. Imaging, pulmonary pressure, right ventricular function and the transplantation perspective guide any intervention. Secondary valvular disease is treated in the context of the entire ventricle.

Cardiopulmonary exercise testing quantifies oxygen consumption, ventilatory efficiency, blood pressure response and chronotropic limitation. A patient may report few symptoms because activities have progressively been reduced; objective measurement reveals the true reserve. Atrial fibrillation, beta-blockade, anemia and deconditioning affect the result. Functional capacity contributes to the timing of advanced therapies.

Competitive sports and high-intensity exercise are generally contraindicated in manifest disease. Moderate activity is not indiscriminately abolished: it is prescribed after control of arrhythmias and heart failure, with intensity and duration compatible with the individual profile. A defibrillator protects against some arrhythmias but not against load-promoted myocardial injury. Exercise reduction is a disease-modifying measure, not a punishment.

Repeated hospitalizations, hyponatremia, worsening renal or hepatic function, hypotension, increasing diuretic requirements and intolerance to therapy indicate advanced heart failure. Refractory electrical storm may constitute an indication for transplantation even without extreme congestion. Waiting for shock or irreversible organ damage reduces options. Early referral to an advanced center preserves candidacy.

An isolated left ventricular assist device may be unsuitable when the right ventricle is severely impaired. Pressures, right ventricular function, tricuspid regurgitation, liver and kidney status help estimate the risk of failure after LVAD; some patients require temporary or durable biventricular support, whereas others are primarily transplant candidates. Support selection depends on physiology, not the label.

Transplantation simultaneously treats pump failure and the cardiac arrhythmogenic substrate, but introduces immunosuppression, rejection, infection and limitations of organ availability. Evaluation includes adherence, social support, comorbidities and pulmonary vascular resistance in addition to the heart. Relatives remain at genetic risk even after a successful transplant in the recipient. Replacement therapy ends one clinical phase, not the family's responsibility.

Family surveillance and longitudinal care

First-degree relatives are assessed with medical history, ECG, rhythm monitoring and imaging directed at both ventricles. A single normal echocardiogram does not exclude an early phase, especially in families with left ventricular scar or early electrical abnormalities. The frequency and starting age of surveillance depend on the youngest age at onset, the gene and family history. Family screening anticipates potentially preventable events.

Cascade testing is offered when a causal variant has been identified in the proband. Carriers without manifestations are followed over time and receive exercise advice, whereas non-carriers can generally be discharged from the specific program if the variant explains the family. A VUS does not allow this separation. Clinical segregation must always accompany the molecular finding.

In children, assessment is adapted to age, genotype and family manifestations. Recessive cardiocutaneous forms or variants associated with early onset require earlier surveillance; parental consent and the child's assent are managed according to maturity. Transition to adult services prevents interruptions. Pediatric continuity is particularly important during growth and sports participation.

Pregnancy is planned by assessing function of both ventricles, arrhythmias, medications and the device. Many stable female carriers can complete pregnancy with an expert team, whereas advanced heart failure or uncontrolled arrhythmias confer high risk. Maternal risk differs from transmission of the variant to the child. Preconception counseling allows therapeutic changes before fetal exposure.

Follow-up of a patient with manifest disease includes symptoms, signs of congestion, ECG, arrhythmic burden, renal and hepatic function, and serial imaging. Intervals are shortened after new arrhythmias, an inflammatory phase, pregnancy or functional deterioration. Repeating tests without a clinical question does not automatically improve care. Adaptive surveillance links each test to a possible decision.

Psychological support addresses fear of sudden death, shocks, sports restrictions and the responsibility of communicating risk to relatives. Clear family letters and genetic counseling reduce misunderstandings while respecting confidentiality and autonomy. Rehabilitation and work also require proportionate recommendations. Quality of life is a clinical outcome alongside survival and hospitalizations.

Biventricular arrhythmogenic cardiomyopathy ultimately requires a dynamic assessment of both ventricles. Neither nonspecific end-stage dysfunction, isolated fat nor LGE without context is sufficient; diagnosis arises from bilateral structural or morphofunctional evidence, electrical signs, genetics and exclusion of alternatives. Rigorous phenotypic precision makes it possible to protect against arrhythmic risk without losing valuable time for treating heart failure.