Clinical vignette

Biventricular non-compaction cardiomyopathy: Rare disease and far rarer case

Izabela WarchołMarcin KsiążczykAgnieszka Dębska-KozłowskaMichał Plewka
Department of Interventional Cardiology and Cardiac Arrhythmias, Military Medical Academy, Memorial Teaching Hospital of the Medical University of Lodz, Łódź, Poland

Correspondence to:

Izabela Warchoł, MD,

Department of Interventional Cardiology and Cardiac Arrhythmias,

Military Medical Academy, Memorial Teaching Hospital of the Medical University of Lodz,

Regional Integrated Treatment Center,

Żeromskiego 113, 90–549 Łódź, Poland

phone: +48 794 98 23 91,

e-mail: izabelaritawarchol@gmail.com

Copyright by the Author(s), 2022

DOI: 10.33963/KP.a2022.0208

Received: June 14, 2022

Accepted: August 22, 2022

Early publication date: September 7, 2022

Isolated ventricular non-compaction (IVNC) is congenital cardiomyopathy defined by the appearance of prominent ventricular trabeculae and deep intertrabecular recesses on cardiac imaging [1]. Although biventricular non-compaction (BiVNC) has been reported rarely [2], its strict diagnostic criteria remain unclear. In the study by Lutokhina et al. [3], a combination of IVNC with arrhythmogenic right ventricular cardiomyopathy (ARVC) was found in 14.8% of the patient subset. The current report presents a challenging case of the biventricular hypertrabeculation phenotype and its unforeseen consequences.

A 36-year-old Caucasian man without prior medical history presented with dyspnea at rest and, on admission, angiographically documented intermediate-high risk pulmonary embolism (PE) complicated by pulmonary and renal infarction and pneumonia. Reperfusion treatment was not considered due to the patient’s hemodynamic stability. Laboratory examination revealed C-reactive protein level 369 mg/l (normal range [NR] <6 mg/l); D-dimer 4.5 mg/l (NR <0.5 mg/l); creatinine 0.97 mg/dl (NR, 0.61.6 mg/dl); N-terminal pro-B-type natriuretic peptide 8649 pg/ml (NR <125 ng/l); high-sensitivity cardiac troponin T 203 ng/l (NR <14 ng/l). An electrocardiogram on admission showed a right bundle branch block with epsilon wave (Supplementary material, Figure S1).

Transthoracic echocardiogram on admission showed right ventricular (RV) systolic function at a lower range limit (Tricuspid Annular Plane Systolic Excursion 17 mm, S’ 10 cm/s), lack of tricuspid regurgitation, severely reduced left ventricular ejection fraction (LVEF) by as much as 15%, increased left ventricular (LV) trabeculation, and a thrombus 22 × 17 mm in the LV apex (Figure 1A, Supplementary material, Video S1). Cardiac magnetic resonance confirmed LV non-compaction with a non-compacted/compacted (NC/C) ratio of 4.2 and revealed right ventricular non-compaction with an NC/C ratio of 4.2, left and right ventricular EF impairment (14% and 32%, respectively), and the presence of a thrombus in the LV apex 20 × 12 mm (Figure 1BC, Supplementary material, Video S2). Moreover, biventricular circumferential subendocardial distribution of late gadolinium enhancement, less common than mid-myocardial [4], was observed (Figure 1D). The late gadolinium enhancement pattern may result from connective tissue diseases but is also highly specific for coexisting dilated cardiomyopathy. A complete package of rheumatological tests was performed, and connective tissue diseases were excluded. The course of BiVNC was complicated with prehospital thromboembolic events diagnosed during hospitalization deriving both from RV (PE) and LV (pulmonary infarction, clinically silent renal infarction) (Figure 1EF).

Figure 1. A. Transthoracic echocardiography in apical 4-chamber view showing LV hypertrabeculation and thrombus in the LV apex (the arrow). B, C. CMR cinematographic sequences in short axis (B) and 4-chamber (C) views showing the non-compacted to compacted myocardium ratio for both ventricles and a thrombus in the LV apex (the arrow). D. CMR short tau inversion recovery sequence showing biventricular endocardial circumferential late gadolinium enhancement (the arrowheads). E. High-resolution computed tomography viewed in lung window with mixed lesions in the right lower lobe typical of infarction and pneumonia coexistence (the asterisk). F. Contrast-enhanced computed tomography shows a hypodense lesion in the right kidney’s lower pole related to the infarction area (the star)
Abbreviations: CMR, cardiac magnetic resonance; LV, left ventricle

Initial treatment included β-blocker, mineralocorticoid receptor antagonist, diuretics, ivabradine, broad-spectrum antibiotic therapy, anticoagulation primarily with low molecular weight heparin, and then vitamin K antagonist, resulting in the resolution of the LV apex thrombus. Sacubitril/valsartan and a sodium-glucose co-transporter 2 inhibitor were initiated early after acute decompensation of heart failure, resulting in LVEF improvement of up to 25% and concomitant symptom reduction (New York Heart Association class IV to II) within the next 3 months. Chronic anticoagulation with dabigatran was subsequently introduced. The patient was registered as a potential heart transplant recipient and electively implanted with a cardiac resynchronization therapy defibrillator.

Reported complications in IVNC are heterogeneous and poorly understood [5]. The present case study shows that IVNC may include both ventricles and present as thromboembolic events deriving from both sides of the heart. This unusual case of BiVNC shows the borderline features of ARVC [3] (regional RV akinesia, RVEF ≤40%, and epsilon wave), which should be considered while managing the patient.

Supplementary material

Supplementary material is available at https://journals.viamedica.pl/kardiologia_polska.

Article information

Conflict of interest: None declared.

Funding: None.

Open access: This article is available in open access under Creative Common Attribution-Non-Commercial-No Derivatives 4.0 International (CC BY-NC-ND 4.0) license, allowing to download articles and share them with others as long as they credit the authors and the publisher, but without permission to change them in any way or use them commercially. For commercial use, please contact the journal office at kardiologiapolska@ptkardio.pl.

REFERENCES

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  2. Pignatelli RH, McMahon CJ, Dreyer WJ, et al. Clinical characterization of left ventricular noncompaction in children: a relatively common form of cardiomyopathy. Circulation. 2003; 108(21): 26722678, doi: 10.1161/01.CIR.0000100664.10777.B8, indexed in Pubmed: 14623814.
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