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A case of ventricular tachycardia
 
Published on 2015-03-11 by Bernard Abi Saleh and Rashoin Rashoin
 
 
History and Findings
 

This case was diagnosed and managed by Drs. Rashwan Rashwan and Bernard Abi Saleh.

A 42 year old lady with HTN presented to the ER after experiencing severe abdominal pain for more than two hours. On arrival she became obtunded and barley arousable. Her vitals showed that her BP is 60by palpation and her heart rate was up to 230 bpm.

The ECG in the Emergency room (fig. 1) showed that the patient was in ventricular tachycardia with a left superior axis and negative concordance. A D/C cardioversion was done in the ER to revert her back to sinus rhythm.

Her surface ECG in sinus rhythm (fig. 2) showed negative T waves in all precordial leads and a clear Epsilon wave in V2, V3 and V4.

She denied any history of syncope, palpitations or family history of sudden cardiac death.

Echocardiography showed significant right ventricular enlargement with hypokinesia. Of note in the parasternal long axis view is the presence of a small aneurysmal area in the right ventricular free wall that is dyskinetic.

Diagnosis: Arrhythmogenic right ventricular dysplasia

 
 
 
1 parasternal long axis with aneurysm of RV free wall | 2 abnormal RV apex | 3 short axis view
 
 
   
 
 
Discussion
 

Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is characterized by progressive fibrofatty replacement of the myocardium that predisposes to ventricular tachycardia and sudden death in young individuals and athletes. It primarily affects the right ventricle; with time, it may also involve the left ventricle. The presentation of disease is highly variable even within families, and some affected individuals may not meet established clinical criteria. The mean age at diagnosis is 31 years (±13; range: 4-64 years). ARVD/C is typically inherited in an autosomal dominant manner. A proband with autosomal dominant ARVD/C may have the disorder as a result of a de novo mutation. The proportion of cases caused by de novo mutations is unknown. Each child of an individual with autosomal dominant ARVD/C has a 50% chance of inheriting the mutation. ARVD/C may also be inherited in a digenic manner (i.e., one ARVD/C-causing mutation in each of two genes (ref. 3).

.

Definite diagnosis of ARVD/C:

•   Two major criteria or

•   One major and two minor criteria or

•   Four minor criteria from different categories

Borderline diagnosis of ARVD/C:

•   One major criteria and one minor or

•   Three minor criteria from different categories

Possible diagnosis of ARVD/C:

•   One major criteria or

Two minor criteria from different categories

Major

•   By 2D echo

                        Regional RV akinesia, dyskinesia, or aneurysm

AND

                        ONE of the following (end diastole):

                                          PLAX (parasternal long axis) RVOT (right ventricular outflow tract) ≥32 mm (corrected for body surface area [PLAX/BSA] ≥19 mm/m2)

                                          PSAX (parasternal short axis) RVOT ≥36 mm (corrected for body surface area [PSAX/BSA] ≥21 mm/m2)

                                          Fractional area change ≤33%

•   By MRI

                        Regional RV akinesia or dyskinesia or dyssynchronous RV contraction

AND

                        ONE of the following:

                                          Ratio of RV end-diastolic volume to BSA ≥110mL/m2 (male) or ≥100 mL/m2 (female)

                                          RV ejection fraction ≤40%

•   By right ventricular angiography. Regional RV akinesia, dyskinesia or aneurysm

Minor

•   By 2D echo

                        Regional right ventricular akinesia or dyskinesia

AND

                        ONE of the following (end diastole):

                                          PLAX RVOT ≥29 to <32 mm (corrected for body surface area [PLAX/BSA] ≥16 to <19 mm/m2)

                                          PSAX RVOT ≥32 to <36 mm (corrected for body surface area [PSAX/BSA] ≥18 to <21 mm/m2)

                                          Fractional area change >33 to ≤40%

•   By MRI

                        Regional RV akinesia or dyskinesia or dyssynchronous RV contraction

AND

                        ONE of the following:

                                          Ratio of RV end-diastolic volume to BSA ≥100 to <110 mL/m2 (male) or ≥90 to <100 mL/m2 (female)

RV ejection fraction >40% to ≤45%

 

Repolarization Abnormalities

Major. Inverted T waves in right precordial leads (V1, V2, and V3) or beyond in individuals age >14 years (in the absence of complete right bundle branch block QRS ≥120ms)

Minor

•   Inverted T waves in leads V1 and V2 in individuals >14 years of age (in absence of complete right bundle branch block) or in V4, V5, or V6.

•   Inverted T waves in leads V1, V2, V3, and V4 in individuals age >14 years in the presence of complete right bundle branch block

Depolarization/Conduction Abnormalities

Major. Epsilon waves (reproducible low-amplitude signals between end of QRS complex to onset of the T wave) in the right precordial leads (V1 to V3)

Minor

•   Late potential by signal-averaged ECG (SAECG) in at least one of three parameters in the absence of a QRS duration of ≥110 ms on the standard ECG

•   Filtered QRS duration (fQRS) ≥114 ms

•   Duration of terminal QRS <40 uV (low amplitude signal duration) ≥38 ms

•   Root-mean-square voltage of terminal 40 ms ≤20 uV

•   Terminal activation duration of QRS >55 ms measured from the nadir of the S wave to the end of the QRS, including R’, in V1, V2, or V3 in the absence of complete right bundle branch block

Arrhythmias

Major. Nonsustained or sustained ventricular tachycardia of left bundle branch morphology with superior axis (negative or indeterminate QRS in leads II, III and aVF and positive in lead aVL)

Minor

•   Nonsustained or sustained ventricular tachycardia of RV outflow configuration, left bundle-branch block morphology with inferior axis (positive QRS in leads II, III and aVF and negative in lead aVL) or of unknown axis

>500 ventricular extrasystoles per 24 hours (Holter

Treatment of Manifestations

Most affected individuals live a normal lifestyle.

Management of individuals with ARVD/C is complicated by incomplete information on the natural history of the disease as well as variable expressivity of the disease. Management of patients should be individualized and based on the specific results of detailed investigation.

Management is focused on prevention of syncope, cardiac arrest, and sudden death. Recent studies suggest that individuals who present with clinical signs of right heart failure and/or left ventricular dysfunction and have a history of ventricular tachycardia are at high risk and should be treated aggressively.

Education regarding sudden death risk to affected adults and parents of affected children is an important aspect of management.

Heart transplantation is considered when ARVD/C has progressed to right or left ventricular heart failure. Severe diffuse biventricular involvement simulating dilated cardiomyopathy and requiring heart transplantation seems to be rare.

Prevention of Primary Manifestations

Antiarrhythmia medications

•   Beta-blockers

•   Amiodarone

•   Sotalol

Note: In a study of 95 patients with ARVD/C by Marcus et al (ref 2.), neither beta blocker therapy nor sotalol were protective against ventricular arrhythmias. However, amiodarone was associated with lower risk for any clinically relevant arrhythmias. Larger studies are needed to confirm this finding.

Implantable cardioverter-defibrillators (ICDs). ICD placement should be considered in anyone with a clinical diagnosis of ARVD/C.

The ACC/AHA/ESC (American College of Cardiology/American Heart Association Task Force/European Society of Cardiology) guidelines, which are based on experience and previously published reports, recommend as a Class I indication (i.e., procedure/treatment SHOULD be performed) ICD implantation for prevention of sudden cardiac death in individuals with documented sustained ventricular tachycardia or ventricular fibrillation who are receiving chronic optimal medical therapy and who have reasonable expectation of survival with a good functional status for more than one year. Class II indications (i.e., it is REASONABLE to perform procedure/treatment) for ICD implantation include extensive disease (e.g., left ventricular involvement) or family members with sudden death, or undiagnosed syncope when ventricular fibrillation or ventricular tachycardia cannot be excluded as cause of syncope while the patient was on optimal medical therapy (ref.4).

 

References:

1.Marcus FI, McKenna WJ, Sherrill D, Basso C, Bauce B, Bluemke DA, Calkins H, Corrado D, Cox MGPJ, Daubert JP, Fontaine G, Gear K, Hauer R, Nava A, Picard MH, Protonotarios N, Safitz JE, YoegerSanborn DM, Steinberg JS, Tandri H, Theine G, Towbin JA, Tsatsopoulou A, Wichter T, Zareba W. Diagnosis of arrhythmogenic right ventricular cardiomyopathy/dysplasia: proposed modification of the task force criteria. Circulation. 2010;121:1533–41

2. Marcus GM, Glidden DV, Polonsky B, Zareba W, Smith LM, Cannom DS, Estes NAM, Marcus F, Scheinman MM. Efficacy of antiarrhythmic drugs in arrhythmogenic right ventricular cardiomyopathy. J Am Coll Cardiol. 2009;54:609–615

3. Hershberger RE, Lindenfeld J, Mestroni L, Seidman CE, Taylor MRG, Towbin JA. Genetic evaluation of cardiomyopathy-A heart failure society of America practice guideline. J Cardiac Fail. 2009;15:83–97.

4. Guidelines for Management of Patients with Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death Developed in collaboration with the European Heart Rhythm Association and the Heart Rhythm Society. Eur Heart J. 2006; 27:2099–140. 

 
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