NIETYPOWY PRZEBIEG ZAWAŁU SERCA U PACJENTA Z NIESCALENIEM LEWEJ KOMORY

Natalia Y. Osovska, Oleksandr I. Datsyuk, Natalia V. Kuzminova, Yevhen V. Shaprynskyi, Hennadii V. Bevz,
Yaroslav M. Pashynskyy, Oleksandr D. Bondarchuk, Olena V. Sergiichuk, Yaroslav V. Karyi, Galina
M. Mazur

National Pirogov Memorial Medical University, Vinnytsya, Ukraine

ABSTRACT

Non-compacted left ventricle in adults is a rare occurrence, though it is diagnosed even more rarely. As a rule in patients with non-compacted left ventricle (LVNC) other pathologic condition is diagnosed, notably hypertrophic or dilated cardiomyopathy. The majority of LVNC cases are diagnosed in early infancy but currently there are asymptomatic cases detected by means of echocardiographic examination. Real prevalence of LVNC is unknown. According to many authors LVNC occurs in 9.2-9.5% of children with diagnosed cardiomyopathies. The majority of such children do not survive till adulthood because of progressive severe heart failure, fatal arrhythmias and thromboembolisms. This value ranges from 0.014 to 0.05% in adult population. The article presents a clinical case illustrating the stages in establishing the diagnosis of non-compacted left ventricle in a young patient with myocardial infarction and congestive heart failure. Common characteristics of non-compacted left ventricle and connective tissue dysplasia syndrome in the patient suggested etiopathogenetic relationship between these two pathologic states. The basic common characteristic feature of both non-compacted left ventricle and connective tissue dysplasia syndrome proved to be multiple abnormal chords of the left ventricle. The patient was supposed to have some coronary circulation abnormality inherited together with non-compacted left ventricle and connective tissue dysplasia syndrome. Adverse prognosis and high mortality in non-compacted left ventricle require its early recognition and differentiated approach to treatment depending on the severity of the disease and using all modern methods of treatment both conservative and surgical.

Wiad Lek 2017, 70, 5, 998-1004

INTRODUCTION

During the last decade many reports have appeared in home and foreign literature concerning the cases of non-compacted left ventricle, a special structure of left ventricular myocardium, in young men with progressive chronic heart failure, diagnosed by ultrasound examination of the heart. This is a serious hereditary heart disease, its diagnostic criteria being formulated and introduced rather recently. For this reason many of NCLV cases were considered to be other severe cardio-vascular diseases (hypertrophic and dilated cardiomyopathy, myocarditis, rheumocarditis etc.) (Lofiego C. et. al. 2007).

The basic characteristic feature of non-compacted myocardium is the presence of numerous bridges and trabeculae in the left ventricle (LV) and intratrabecular recesses between them lined with endocardium and connected with the left ventricular cavity (Christian Lilje et. al. 2006). Multiple tubules-bridges together with trabeculae form a wide spongy layer of heart muscle, while the layer of homogeneous true myocardium capable of contraction remains thin and functionally weak. This determines the main clinical manifestation of the disease – progressive severe chronic heart failure by the time of its diagnostics.

Severe arrhythmia is another significant evidence of LVNC. By modern hypothesis it results from both primary myocardial pathologic structure and progressive hemodynamic remodeling of LV and left atrium and the defect in connective tissue framework of the heart and its conductive system. Fatal thromboembolism resulting from pathologic myocardial structure and severe arrhythmia are associated with LVNC both in children and adults.

According to modern concepts, left ventricular non-compaction is the result of disturbances in embryogenesis on early stages of embryo development, when the heart is composed of loose meshwork of tubular muscular fibrils (spongy myocardium) which gradually “intergrow” in the process of cardiac muscle development. In normal embryonic development large cavities with a network of trabecular tubules become smaller, flat and form coronary flow resulting in smoothing of endocardium surface. If the development process is disturbed, the connections between left ventricular cavity and intratrabecular cavities continue to exist. This forms the basis of isolated myocardial non-compaction.

Sometimes myocardial non-compaction is diagnosed together with other malformations causing an increase of pressure in heart cavities. In such cases deep trabeculae communicate not only with LV cavity but also with abnormal coronary arteries leading to severe infarction of cardiac muscle in newborns and infants. Such abnormalities occur mostly in infants of the first days-months-a year of life with genetic diseases (Barth syndrome, Emery-Dreifuss muscular dystrophy) leading to extremely severe heart failure, fatal arrhythmia and eventually to death (Pignatelli R. et. al. 2003, Monserrat L. et al., 2007).

The overwhelming majority of LVNC are diagnosed in early infancy, however there are asymptomatic cases detected by echocardiography. Real prevalence of LVNC is unknown. According to many authors LVNC occurs in 9.2-9.5% of children with diagnosed cardiomyopathies (Freedom R. et al., 2005, Kurosaki K. et al.,1999). This index ranges from 0.014 to 0.05% in adult population (Oechslin E. et al., 2000, Ritter M. et al., 1997).

According to WHO definition and classification of cardiomyopathies, LV non-compaction as well as fibroelastosis and mitochondrial cardiomyopathies are still referred to the category of “non-classified” cardiomyopathies (Richardson P. et al., 1996). Inclusion of LV non-compaction into this category reflects the absence of common notion of etiology and pathogenesis of the disease.

The basic method of NCLV diagnostics is echocardiography. R. Jenni and E. Oechslin (2001) suggested systematized echocardiographic criteria of LV non-compaction which include: thickening of left ventricular wall due to non-compacted layer with the ratio between non-compacted layer and true muscular layer being more than 2; b) on color flow mapping – visualization of deep trabecular sinuses with turbulent blood flow connected with left ventricular cavity; the presence of abnormal chords in the LV cavity (three or more); the absence of other heart defects except isolated LV non-compaction.

The prognosis in patients with LVNC depends on the number and volume of the segments involved, general myocardial contractility, the time of onset and the rate of heart failure symptoms increase. The patients with EF less than 35% have the most unfavourable course of the disease.

CLINICAL COURSE

Clinical course of left ventricular non-compaction in young patient
after old myocardial infarction

Patient K., a 36-year-old man, was diagnosed with acute anterior Q-wave myocardial infarction. The patient was found unconscious on the floor at home by his wife when she entered the room in 5-10 minutes after she had left it. The man had complained of back pain, so his wife went out to bring pepper plaster used by him for 2-3 weeks to relieve interscapular pain. The man was brought to consciousness and hospitalized.

Though the patient had had the signs of heart failure in the form of dyspnea and frequent palpitation attacks before he ignored them. His mother informed the boy could not run or play quick games because of rapid fatigue and dyspnea. She said he had always been in poor health since childhood but she could not tell what exactly had been wrong with her son. The patient spent most of his time in the yard performing easy work. He had never had reported employment. The patient’s wife mentioned his abuse of alcohol, and this was one of the reasons that the ECG was taken only in 2 hours after his admission to the hospital. The patient had smoked since childhood more than one pack a day. The patient’s mother had some miscarriages and an infant who had died within the first week after birth because of “turning blue” (according to mother’s words). The patient’s father died when he was nearly 40.

Clinical and instrumental findings
and treatment

At examination, in half a year after myocardial infarction, the patient had palpitation attacks, arrhythmia, pitting edema of the feet, periodical heaviness in the right hypochondrium, interscapular pain with no irradiation in moderate physical activity (going upstairs to the first floor, walking 100-200 meters). Objective assessment: height – 169 cm, weight – 65 kg, round-shouldered stature, muscle hypotrophy, very thin and limp hair evenly covering the head, wide anterior diastema, severe caries with over half teeth loss.

Left heart border – 2-3cm outward from the mammillary line. Heart sounds – moderately weakened, moderate apical systolic murmur, diastolic murmur – over the aorta. Breathing – vesicular. BP – 100/70 mmHg, heart rate – 72 bpm, rhythmical. The liver +5cm.

Plain chest radiograph showed cardiac enlargement due to the left ventricle associated with evident pulmonary emphysema. ECG: sinus rhythm with heart rate 60 bpm. The signs of left ventricular hypertrophy (LVH) (amplitude). Postinfarction cicatricial changes in the anterior septal area (Fig.1). ECG was taken after 6.25 mg of carvedilol in the morning and 20 mg of anapriline 20 minutes before ECG.

EchoCG examination, difficult because of emphysema, found severe eccentric LVH – predominantly cardiodiosis: left ventricular muscle mass index (LVMMI) – 173 mg/cm2 (LVMM – 340 mg), end-dyastolic size (EDS) on the level of basal segments – 62 mm, end-dyastolic volume (EDV) – 194 ml, left atrium (LA) size – 49 mm, thickness of basal segments of interventricular septum (IVS) -10 mm, left ventricular posterior wall (LVPW) thickness – 13 mm. EF, defined in both M- and B-regimes – 30-33% (Fig.2). Diffuse decrease of left ventricular contractility. Bicuspid aortic valve (Fig.3) with moderate aortic regurgitation (Fig.4). Aortic root diameter – 39 cm, aneurismal dilation of ascending aorta – 44.6 mm (Fig.5). Moderate mitral valve regurgitation. Moderate tricuspid valve regurgitation. Mean pressure in pulmonary artery – 43-46 mmHg. In parasternal short-axis view (Fig.6) – considerable thickening with the signs of myocardial non-compaction marked as deep endomyocardial trabeculae with intertrabecular cavities in apical and medial LV segments.

24-hour Holter ECG Monitoring was performed to the patient because of rather specific pain syndrome in the back. Conclusion: sinus rhythm with average daily heart beat (HB) 82bpm. Maximal increase of HB to 130 bpm. Inadequate night HB decrease – circadian index 1.1. Rigid rhythm. Signs of non-compensated hypersympathicotony (tachycardia without adequate night HB decrease in beta-blocker administration ). Episodes of AF (atrial fibrillation) with HB to 130 bpm with various graphic presentation of QRS complex, presumably connected with the transient left bundle-branch block. Substantial decrease of T wave amplitude recorded steadily during 24 hours with the episodes of its inversion on the third canal (lateral wall) (Fig.7).

To choose further treatment policy coronary angiography was done to the patient. This investigation demonstrated virtually intact coronary vessels (Fig. 8).

Varicosity of both lower extremities associated with venous valve insufficiency and 2 degree chronic venous insufficiency were also found in the patient. Ultrasound examination of abdominal cavity and retroperitoneal space revealed moderate symmetrical renal ptosis, gallbladder deformity with twofold decrease of its size and the signs of its evident dysfunction.

The diagnosis was made to the patient: Ischemic heart disease. Post-infarction cardiosclerosis (anteroseptal Q-wave MI – ECG of September 12, 2014). Left ventricular non-compaction. HF 2a. FC II-III. Paroxysmal AF. Transient left bundle-branch block. Ptosis of both kidneys, deformity and dysfunction of gallbladder. Varicosity of lower extremities. Second degree chronic venous insufficiency.

Recommended treatment

The patient is treated by carvedilol – 6.25 mg twice a day, torasemide – 10 mg/day, verospiron – 100 mg/day, ramipril – 5 mg/day, rivaroxaban – 20 mg/day, cordarone – 200 mg twice a day.

At present the patient’s state is satisfactory. Private conversation was held with the patient concerning possible transplantation of the heart in future but this seems unlikely because of his social status.

DISCUSSION

Because of the absence of distinct diagnostic criteria, genetic markers as well as nonspecific clinical picture of LVNC most its cases remain undiagnosed, detected rather late or referred to other heart pathology. Quite often various cardiomyopathies are diagnosed in patients with LVNC. In this case the first established diagnosis of Q-wave MI agreed completely with its clinical picture and gave no rise to doubts as to its correctness. Considering rather low total LV contractility it would be reasonable to make the diagnosis of ischemic cardiomyopathy despite nearly “clean” coronary vessels.

LVNC was just a diagnostic finding in this patient but nevertheless it can explain both the degree of CHF and some aspects of IM development despite intact coronary vessels in young man.

EchoCG is the basis for diagnostics of any structural changes in the heart. In our case hypokinesia of anteroseptal LV segments, demonstrated on the ECG as well, confirmed the presence of cicatrical changes in the myocardium. However, there was hypokinesis of the posterior and lateral wall as well comparable with that in anteroseptal area. That is, systolic myocardial dysfunction developed in the setting of total diffuse hypokinesis of all LV segments. And those changes developed despite unaffected coronary vessels. Thus, clinical and instrumental diagnose of severe dynamically progressive ischemic cardiomyopathy in the absence of its evident cause – atherosclerosis – was made. The suggestion that vasospasm is the cause of Q-wave MI seems unlikely because of stable dynamic total myocardial involvement.

The presence of “spongy” (non-compact, trabecular) myocardial structure in apical and partially (apical and medial segments) lateral LV wall was the explanation of such echocardiographic pattern. It is known that in LVNC not only non-compact myocardial segments but also visually “normal” ones are hypoakinetic. Echocardiographic pattern was supplemented by multiple abnormal chords at LV apex reflecting pathologically increased LV trabeculation and being one of LVNC criteria.

Multiple abnormal left ventricular chords (LVAC) take an intermediate place: on the one hand, it is a criterion of left ventricular non-compaction, and on the other – minor structural heart abnormality (MSHA) in connective tissue dysplasia syndrome (CTDS) (Jenni R. et. al., 2001, Martinov A. et. al., 1996). Left ventricular abnormal chords, particularly isolated ones, denote mostly dysplastic myocardium and have no hemodynamic significance except the doctor’s concern because of acoustical phenomenon – soft systolic murmur. But in this case multiple LVAC may be considered as a sign of both pathologic processes – LVNC and CTDS. The patient presented had certain non-classic, mostly internal, phenotypical signs of connective tissue dysplasia: varicosity of lower extremity veins with CVI, kidney ptosis, ptosis, deformation and decrease in size (hypoplasticity) of the gallbladder, early diffuse alopecia and wide anterior diastema. However, there were no basic external signs of CTDS, evident to the doctor: high stature, asymmetric chest, pronounced scoliosis, “hypermobility” of rather long arms,that is “marfanoid habitus”. Quite the contrary, the patient was not tall, had low weight and marked muscular hypotrophy as the result of life style and progressive CHF but not an evidence of CTDS.

In our patient bicuspid aortic valve (AV) with moderate aortic regurgitation and rather substantial (for a patient of such weight and height) dilation of ascending aorta to 45 mm was diagnosed. Bicuspid aortic valve is not a minor structural abnormality, this pathology is referred to congenital heart diseases, but connective tissue defect of embryogenesis is an underlying cause of this pathology as well. Probably this is the reason why bicuspid AV is recorded in young patients together with MMVP (myxomatous mitral valve prolapse), LVAC etc. denoting some relationship between the pathogenesis of bicuspid AV and MSHA, particularly LVAC [12]. Dilation of ascending aorta in young adults is also often diagnosed together with bicuspid AV and MSHA (Osovska N., 2014). Thus, besides already established assosiation LVAC (MSHA) – LVNC, it is likely to suggest some other ones: MSHA (LVAC) – bicuspid AV – aneurism of ascending aorta; MSHA – LVNC – coronary pathology.

Postinfarction changes on ECG in unaffected coronary vessels may indicate the involvement of coronary vessels into some other pathologic process common with myocardial non-compaction. In modern literature such cases which occur in early infancy are considered to be caused by general defect of embryogenesis (Pignatelli R. et. al. 2003, Monserrat L. et al., 2007). Coronary insufficiency in our patient is likely to be the result of undiagnosed coronary vessel abnormality associated with LVNC.

At present arrhythmia diagnosed by Holter ECG monitoring indicates severe prognostically unfavourable state of myocardium and most probably is caused by LVNC and post-infarction state, but it may be the sign of cardiac connective tissue dysplasia syndrome as well.

This review is a highly probable suggestion based on literature data, personal observational results and taking into account specific clinical findings and anamnesis as well as instrumental diagnostic findings of one concrete patient. However, the discussion on this problem continues for many years, achieving no evidence grade because of insufficient number of reported instances of this disease in adults. Therefore, description and detailed analysis of even single cases is relevant and well-timed.

Unfavourable prognosis and high mortality in LVNC determines the necessity of its recognition at early stages as well as specific approach to treatment depending on the patient’s state and using modern methods of conservative and operative treatment. Etiologic treatment of LVNC is unknown at the moment and is likely to be impossible considering the level of modern medicine development. Cardiac transplantation is a radical method of treatment. Treatment of congestive heart failure and rhythm disturbances seems to be reasonable according to the guidelines for treatment and prevention of these syndromes as well as adequate prevention of thromboembolic complications.

References

1. Lofiego C., Biagini E., Pasquale F., Ferlito M., Rocchi G., Perugini E., Bacchi-Reggiani L., Boriani G., Leone O., Caliskan K.,  Cate F.J., Picchio F.M., Branzi A.,.Rapezzi C. (2007) Wide spectrum of presentation and variable outcomes of isolated left ventricular non-compaction. Heart. 93, 65–69.

2. Christian Lilje, Vit Rázek, JamesJ. Joyce, Thomas Rau, Barbara F. Finckh, Florian Weiss, Christian R. Habermann, Janet C. Rice, Jochen Weil. (2006) Complications of non-compaction of the left ventricular myocardium in a paediatric population: a prospective study. European Heart Journal. 27, 1855–1860.

3. Pignatelli R., McMahon C. (2003) Clinical characterization of left ventricular non-compaction in children: a relatively common form of cardiomyopathy. Circulation. 108, 2672–2678.

4. Monserrat L., Hermida-Prieto M., Fernandez X., Rodríguez I., Dumont C., Cazón L., Cuesta M.G., Gonzalez-Juanatey C., Peteiro J., Alvarez N., Penas-Lado M.,Castro-Beiras A. (2007) Mutationin the alpha-cardiac acting gene associated with apical hypertrophic cardiomyopathy,left ventricular non-compaction, and septal defects.Eur. Heart J. 28, 1953–1961.

5. Freedom R., Yoo S., Perrin D. (2005) The morphological spectrum of ventricular non-compaction. CardiolYoung. 15, 345–364.

6. Kurosaki K., Ikeda U., Hojo Y., Fujikawa H., Katsuki T., Shimada K. (1999) Familial isolated noncompaction of the left ventricular myocardium. Cardiology. 91, 69-72.

7. Oechslin E.N., Attenhofer Jost C.H., Rojas J.R., Kaufmann P.A., Jenni R. (2000) Long-term follow-up of 34 adults with isolated left ventricular non-compaction: a distinct cardiomyopathy with poor prognosis. J. Am. Coll. Cardiol. 36, 493–500.

8. Ritter M., Oechslin E., Sütsch G., Attenhofer C., Schneider J., Jenni R. (1997) Isolated non-compaction of the myocardium in adults. Mayo Clin. Proc.72, 26–31.

9. Richardson P., McKenna W., Bristow M., Maisch B., Mautner B., O’Connell J., Olsen E., Thiene G., Goodwin J., Gyarfas I., Martin I., Nordet P. (1996) Report of the 1995 World Health Organization/International Society and Federation of Cardiology. Task Force on the Definition and Classification of cardiomyopathies. Circulation. 93, 841–842.

10. Jenni R., Oechslin E., Schneider J., Attenhofer Jost C., Kaufmann P.A. (2001) Echocardiographic and pathoanatomical characteristics of isolated left ventricular non-compaction: a step towards classification as a distinct cardiomyopathy. Heart. 86, 666–671.

11. Martinov A., Stepura O., Ostroumova O. (1996) Markers of cardiac connective tissue dysplasia syndrome in patients with idiopathic mitral valve prolapse and abnormally located chords. [Article in Russian]. Ter. Arch. 2, 332-337.

12. Kavatsyuk O., Osovska N. (2014) Clinical and anamnestic characteristics in patients of different age with ascending aorta aneurysm. [Article in Ukrainian]. Bulletin of Morphology. 20, 77- 81.

13. Osovska N. (2014) Relationship between ascending aorta aneurysm and connective tissue dysplasia syndrome. [Article in Ukrainian]. Medical prospects. 4, 73-83.

ADDRESS FOR CORRESPONDENCE

Natalia Y. Osovska

tel. +380679531836

e-mail. osovskayanatalia@gmail.com

Received: 20.06.2017

Accepted: 25.10.2017

Fig.1. ECG of the patient with anterior septal cicatricial changes.

Fig.2. Hypokinesia of heart basal areas (extremely bad visualization because of severe lung emphysema)

Fig.4. Moderate biocuspid valve regurgitation (extremely indistinct image because of severe lung emphysema)

Fig.6. Signs of LV non-compaction in parasternal short-axis view (extremely indistinct image because of severe lung emphysema)

Fig.3. Bicuspid aortic valve

Fig.5. Dilation of ascending aorta to 45 mm

Fig.7. AF episodes and specific T wave in the patient (Holter ECG monitoring)

Lipid profile: total cholesterol – 4.1 mmol/l, Triglycerides (TG) – 1.5 mmol/l,

LDL cholesterol – 2 mmol/l.

Fig.8. Scheme of coronary angiography findings