|Year : 2021 | Volume
| Issue : 2 | Page : 145-147
Left ventricular noncompaction cardiomyopathy with apical septal ventricular tachycardia
Debasish Das, Debasis Acharya, Tutan Das, Jogendra Singh, Sashikant Singh, Subhas Pramanik
Department of Cardiology, All India Institute of Medical Sciences, Bhubaneswar, Odisha, India
|Date of Submission||22-Mar-2021|
|Date of Decision||19-Apr-2021|
|Date of Acceptance||30-Apr-2021|
|Date of Web Publication||25-Aug-2021|
Dr. Debasish Das
Associate Professor and HOD, Department of Cardiology, All India Institute of Medical Sciences (AIIMS), Bhubaneswar, Odisha
Source of Support: None, Conflict of Interest: None
We present a rare case of left ventricular noncompaction (LVNC) in a 23-year-old female with recurrent syncope with electrocardiography documentation of apical septal ventricular tachycardia (VT). Abnormal embryological myocardial maturation presenting as LVNC with electrogenic heterogenity across the noncompacted spongiform segments result in recurrent VT and mandates automated intracardiac defibrillator implantation (AICD). These subsets of patients in spite of AICD implantation require life-long broad-spectrum antiarrythmic in the form of amiodarone and beta-blocker to prevent a crisis of VT storm or sudden cardiac death.
Keywords: Defibrillator, noncompaction, ventricular tachycardia
|How to cite this article:|
Das D, Acharya D, Das T, Singh J, Singh S, Pramanik S. Left ventricular noncompaction cardiomyopathy with apical septal ventricular tachycardia. Heart India 2021;9:145-7
|How to cite this URL:|
Das D, Acharya D, Das T, Singh J, Singh S, Pramanik S. Left ventricular noncompaction cardiomyopathy with apical septal ventricular tachycardia. Heart India [serial online] 2021 [cited 2021 Dec 4];9:145-7. Available from: https://www.heartindia.net/text.asp?2021/9/2/145/324611
| Introduction|| |
Left ventricular noncompaction (LVNC) is a rare form of congenital cardiomyopathy with diverse heterogeneity presenting across all ages starting from infancy to old age. Arrest of compaction of loose myocardial mesh work during fetal ontogenesis results in more prominence of noncompacted (NC) layer as compared to the compacted (c) layer of the myocardium which becomes well evident in echocardiography with prominent left ventricular apical trabeculations. This NC layer weakens with the passage of time resulting in progressive left ventricular systolic dysfunction with high risk of thromboembolism, and this structural heterogeneity is also associated with electrical heterogeneity across the myocardium resulting in malignant ventricular arrhythmia in the form of ventricular tachycardia (VT), ventricular fibrillation, and sudden cardiac death (SCD). We present the rare case of LVNC cardiomyopathy in a young female presenting with recurrent hemodynamically unstable VT with syncope which was successfully managed with automated intracardiac defibrillator (AICD) implantation.
| Case Report|| |
A 23-year-old female presented with a history of recurrent syncope, i.e., three episodes in last 6 months with electrocardiography (ECG) documentation of monomorphic VT of left ventricular free wall interventricular septum (IVS) junction [Figure 1]. VT was having relatively narrow QRS with negative concordance across precordial leads V1–V5 and late transition in V6, suggestive of origin from left ventricular free wall IVS junction. The first episode of VT was reverted with amiodarone bolus, and the second and third episodes were direct cardioversion verted. Echocardiography showed spongiform left ventricular myocardium with prominent trabeculae [Figure 2] and [Figure 3] and recesses with blood flow into those recesses [Figure 4] and mild left ventricular systolic dysfunction and Grade I diastolic dysfunction without any mitral regurgitation or pulmonary arterial hypertension. Interesting echocardiography also revealed the presence of false tendon in the left ventricle (LV) [Figure 4] and [Figure 5]. Patient's serum electrolytes (K+, Ca++, and Mg++) were within the normal limit.
|Figure 1: Electrocardiography showing ventricular tachycardia from left ventricular free wall inter ventricular septum junction|
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|Figure 2: A4CH view showing prominent trabeculations of the left ventricle|
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|Figure 5: Left ventricular noncompaction with false tendon in the left ventricle|
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In view of structural heart disease with recurrent VT which is a Class I recommendation for implanting the AICD, we implanted an AICD and discharged the patient with oral amiodarone and metoprolol to have Class III synergistic effect of membrane stabilization to prevent breakthrough VT, VT storm, or SCD. Structural myocardial heterogeneity accompanies heterogeneity in electrical depolarization and dispersion resulting in recurrent VT which requires long-term antiarrhythmic in these cohorts of patients to cool the myocardium from abnormal excitation.
| Discussion|| |
LVNC is a very rare form of congenital cardiomyopathy due to embryonic arrest of the normal maturation process of the myocardium. It accounts for 0.05%–0.24% of all the cardiomyopathy. Predominantly, noncompaction involves the LV but biventricular involvement can be seen in up 22%–38% of cases. It is an autosomal dominant disorder with single-point mutation of beta myosin heavy gene; mutation in ZASP, dystrobrevin, and taffazin have also been reported. It is otherwise known as spongy myocardium, spongiform cardiomyopathy, persisting myocardial sinusoids, or zaspopathy. It has been known to be a part of Noonan, Barth, Roifman, and Toriello Carey Syndrome. In LVNC, endomyocardial trabeculations increase in the number with prominence in cardiac apex. It carries a high risk of malignant ventricular arrhythmia due to structural and electrical heterogeneity, progressive left ventricular dysfunction with advancement of age, and also carries a high risk of thromboembolism. Echocardiography plays an important role in the diagnosis of LVNC as follows:
Ratio of distance of trough of trabecular recess from epicardium (X) to distance of the peak of trabecular recess from epicardium (Y) <0.5. This can be well delineated in transthoracic echocardiography.
Ratio of thickness of the NC layer to compacted layer >2 which is well delineated in cardiac MRI in the short-axis view of LV in end systole.
Echocardiography plays an important role in children to rule out the presence of associated supra mitral ring and ventricular septal defect. Common differential diagnosis in echocardiography includes layered mural thrombus in the LV, intramyocardial hematoma, mycotic invasion of the heart, and Fabry's disease. Although heart failure is the most common presentation, our case presented with recurrent syncope with ECG demonstration of monomorphic VT of apical septal origin. We implanted an AICD to prevent SCD in this young female with recurrent hemodynamically unstable VT. We did not put the patient on oral anticoagulation at the time of AICD implantation in view of risk of pocket hematoma but the patient is on oral anticoagulation since the first follow-up. The prognosis of the patients with LVNC is poor, but asymptomatic cases have been reported to survive up to the seventh decade. The patient will be regularly followed up every 3 months to have a close look for progression of LV systolic dysfunction as time progresses. Rarity of this disorder with diverse presentation needs individual case to case management with a heart team approach; cardiac transplantation remains as an ultimate option in patients presenting with end-stage heart failure with expected survival more than 1 year.
| Conclusion|| |
LVNC is a rare heterogenic congenital cardiomyopathy with diversity in presentation and prognosis. We present a rare presentation of LVNC with recurrent hemodynamically unstable apical septal VT in a young female which was managed with AICD and conventional antifailure measures. In time intervention in those rare subsets of patients can achieve a favorable outcome.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient (s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initial s will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Debasish Das: Final Manuscript Editing Debasis Acharya: Manuscript Preparation Tutan Das, Jogendra Singh, Shashikant Singh: Literature Search Subhas Pramanik: Image Editing.
Institutional Ethical Committee (IEC) approval has been obtained.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]