A clinical case of right ventricular myocardial infarction in a patient with diabetes mellitus and Fahr’s disease

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Introduction

Cardiovascular diseases (CVD) remain the leading cause of morbidity and mortality in developed countries. CVDs claim more lives each year than malignant tumors, chronic lower respiratory diseases, and accidents combined [1]. Coronary heart disease accounts for approximately half of CVD deaths.

In contrast to the long-standing historical interest in left ventricular MI (LVMI), right ventricular MI (RVMI) has received little clinical attention due to its low prevalence (the incidence of isolated RVMI is less than 3 % of all infarctions [2]) and low hemodynamic significance, as demonstrated by early animal studies [3]. The first clinical observation of a patient with RV MI was given by A. Sanders [4] in 1931, and the clinical and hemodynamic features of RV MI were presented in the original article by J. Cohn et al. [5] in 1974. The table shows some diagnostic criteria for RV MI based on clinical feachers and instrumental research methods.

Fahr's disease is an idiopathic calcification of the basal ganglia and vascular structures of the brain, accompanied by non-specific neurological symptoms and difficult to diagnose clinically; most often it is detected during computed tomography of the brain. A feature of the deposits in the brain substance, compared to many other calcifications, is the presence of a large number of iron ions; for this reason, Fahr's disease is also called ferrocalcinosis of the brain. Ischemic infarctions of the brain in Fahr's disease are rare, as a rule, this occurs with severe vascular damage [13; 14].

 

Features of diagnostics of myocardial infarction of the right ventricle of the heart

Methods of diagnostics	Results
Clinical	Hemodynamic triad: absence of wheezing in the lungs + hypotension + swelling of the jugular veins (sensitivity 25 %, specificity 96 %) [6]. Systolic murmur of relative tricuspid insufficiency and pathological third sound at the fourth auscultation point; paradoxical pulse; Kussmaul's sign [7]. These factors taken together highly predict the development of acute RVMI (sensitivity 88 %; specificity 100 %) [8]
Electrocardiography	ST segment elevation in the right chest leads (V3R–V6R) > 1 mm (sensitivity 100 %, specificity 87 %, positive predictive value 92 % [9]); is a transient phenomenon and may be absent 10–12 hours after the onset of pain [10]. ST segment depression in lead I + aVL > 2 mm (sensitivity 94 %, specificity 90 %) [11]. ST segment elevation in lead III > II (has low specificity and is often seen in patients without RVMI) [12]
Echocardiography	Pathology of right ventricular wall contractility. Dilation of the right heart chambers and inferior vena cava. Tricuspid regurgitation. Paradoxical motion of the interventricular septum. Blood shunting

 

On the one hand, angiopathy in diabetes mellitus manifests itself in the form of dystrophic changes in small vessels, which can lead to local circulatory disorders (for example, lacunar strokes) and organ restructuring (for example, diabetic nephropathy); on the other hand, one cannot fail to note the potentiation of atherosclerosis in diabetes mellitus, which manifests itself in acute vascular catastrophes: infarctions of the brain, myocardium and other organs [15].

Clinical Case

Patient P., 68 years old, was delivered by an ambulance team to the State Budgetary Healthcare Institution Samara City Clinical Hospital No. 1 named after N.I. Pirogov on 04.12.2023 with a diagnosis of acute cerebrovascular accident (ACVA) – ischemic stroke. The patient complained of weakness in the right limbs, nausea, and vomiting at the time of the initial examination in the department. Speech impairment was determined clinically by the type of sensory and motor aphasia. It is known from the anamnesis that the above complaints began to bother the patient on the morning of 04.12.2023. Since 2019, he has suffered from type 2 diabetes mellitus. In March 2023, he suffered from a new coronavirus infection in the form of moderate pneumonia.

Signs of dyscirculatory encephalopathy were determined during multispiral computed tomography of the brain (to exclude acute cerebrovascular accident). Cerebral atherosclerosis. Periventricular leukoaraiosis. Atrophic process in the cerebral cortex.

The general condition is of moderate severity according to the objective examination. The level of consciousness is stupefaction, there are no meningeal signs. Tendon reflexes D> S, atypical plantar reflex is on the right. Right-sided hemiparesis is up to 3 points. Breathing during auscultation is harsh, there are no wheezing, the respiratory rate is 16 per minute. Saturation is 98 %. Blood pressure is 150/90 mm Hg. Pulse is 78 beats per minute. Heart sounds are clear, rhythmic. On superficial palpation, the abdomen is soft and painless, the liver and spleen are not enlarged.

Taking into account the clinical symptoms, anamnesis data and multispiral computed tomography of the brain, the following preliminary diagnosis was made: acute cerebrovascular accident – ischemic stroke in the left MCA basin. Right-sided hemiplegia. Stage II cerebrovascular insufficiency, mixed genesis (hypertensive, atherosclerotic, dysmetabolic). Chronic atrophic process of the brain. COPD, severe course with pronounced clinical symptoms, outside of exacerbation; chronic cor pulmonale, subcompensation; H2A; ischemic heart disease; degenerative aortic and mitral valve disease with predominant insufficiency; H2 NYHA2; hypertension stage III, risk 4; diabetes mellitus type 2; diabetic polyneuropathy, sensorimotor form, moderately expressed (from anamnesis).

Treatment of the patient began in the neurological department. Vascular, metabolic, antidiabetic, antithrombotic and antibacterial therapy was administered. A number of laboratory and instrumental studies were performed to clarify the diagnosis.

Blood biochemistry analysis documented an increased risk of developing atherosclerosis: LDL at the upper limit of normal (3.23 mmol/l), a sharply reduced amount of HDL (0.95 mmol/l), borderline cholesterol level (5.25 mmol/l), atherogenic index of 4.54 %; hyperglycemia (glucose 10.93 mmol/l) and increased urea content (9.3 mmol/l).

The ECG revealed a regular sinus rhythm with a heart rate of 88 beats/min. Complete blockade of the right bundle branch of the His bundle. The position of the electrical axis of the heart is semi-horizontal.

The performed chest X-ray revealed signs of congestion in the pulmonary circulation. Abdominal X-ray revealed no pathology.

Ultrasound examination of the pleural cavities revealed echo signs of bilateral hydrothorax: free fluid with a volume of up to 440 ml is in the right pleural cavity; free fluid with a volume of up to 100 ml is in the left pleural region.

Against the background of the treatment, the condition remained severe, severe neurological symptoms persisted. On 07.12.2023, the patient's condition worsened, the level of consciousness is soporific state; in addition, there is smoothing of the nasolabial fold on the right, paresis of the soft palate, and no pharyngeal reflexes are determined. The patient was transferred to the intensive care unit.

On 08.12.2023 the patient's condition was serious, with negative dynamics (increasing respiratory failure), tracheal intubation was performed.

According to echocardiography, no areas of local contractility disorders were detected. Global contractility is reduced, ejection fraction is 32 % according to Teicholz.

There is dilation of all chambers. Atherosclerosis of the aorta with involvement of the aortic valve: minor stenosis, insufficiency

Stage 1. Mitral valve insufficiency of the 1st degree, tricuspid valve insufficiency of the 2nd degree. Estimated pressure in the right ventricle is 50 mm Hg.

A repeat ECG on 08.12.2023 (Figure) revealed a regular sinus rhythm. Sinus tachycardia with a heart rate of 113 beats/min. Complete right bundle branch block, altered T wave in leads V1 through V6, in leads II and aVF. Deep S wave in leads V5 and V6, deep Q wave (Q = R) in lead III. The position of the electrical axis of the heart is semi-horizontal.

 

Fig. ECG of patient P., 68 years old, 08.12.2023

 

According to the ECG data, right heart overload is suspected, and a chest CT scan is recommended to rule out pulmonary embolism. It was decided to refuse CT scanning due to the lack of an appropriate clinical picture (cyanosis of the upper half of the body, bulging jugular veins and hypoxemia) and the patient's serious condition.

Against the background of intensive therapy, cardiac arrest occurred, resuscitation measures were unsuccessful. Biological death was confirmed at 15:00.

Final clinical diagnosis. Underlying disease: diabetes mellitus type 2. Diabetic polyneuropathy. Diabetic hepatosis. Diabetic nephropathy.

Complications of the underlying disease: acute cerebrovascular accident – ischemic stroke in the left middle cerebral artery. Dyscirculatory encephalopathy stage II, mixed genesis (hypertensive, atherosclerotic, dysmetabolic). Chronic atrophic process of the brain. Cerebral edema. Brain herniation.

Associated diseases: coronary heart disease. Myocardial infarction (unknown duration). Complete blockade of the right branch of His bundle. Stable cardiac angina, 2nd functional class. Hypertension stage III, risk 4. HIIa. 3rd functional class according to NYHA. Degenerative aortic valve disease. COPD, moderate severity. DN II. The patient suffered community-acquired bilateral polysegmental pneumonia from IX-2023. Chronic latent pyelonephritis. Reducible umbilical hernia.

Pathological autopsy and histological examination. When examining the cranial cavity, the dura mater is not tense, smooth, grayish, the pia mater is moderately full-blooded, edematous. The sulci of the brain are deepened, the convolutions are smoothed out. The vessels of the base of the brain are tortuous, with atherosclerotic plaques on the intima, stenotic up to 40 %. An extensive area of flabby brain tissue, mushy consistency, measuring 5×6 cm with secondary hemorrhagic transformation is determined in the parietal-occipital region of the right hemisphere of the brain. The brain tissue is moist, shiny, sticks to the knife when cut. There are strangulation grooves – traces of pressure from the edges of the foramen magnum on the cerebellar hemispheres.

Microscopic examination revealed moderate blood filling, mild perivascular and pericellular edema, isolated granular balls, mainly under the ependyma of the ventricles and in the perivascular spaces. Individual preparations – zones of brain tissue necrosis and hemorrhage into the brain substance, with perifocal infiltration of lymphocytes and macrophages. Small and medium-sized arteries with calcium deposits are mainly in the muscular wall. A positive reaction was obtained when staining according to Perls, von Kossa and alysrin red C during the histochemical study. The presence of trivalent iron ions in the deposits allows us to conclude that there is ferrocalcinosis, which is more consistent with Fahr's disease [13].

There is a small amount of transparent fluid in the pericardial cavity, the pericardial layers are fused along the lateral and posterior surfaces. The heart weighs 450 g. The heart cavities are sharply dilated. There is myocardium of the left ventricle is 1.6 cm thick, brownish in color with small whitish layers on the section. The myocardium of the right ventricle is 0.3 cm thick, in all sections with the transition to the interventricular septum, variegated, with alternating pale areas and reddish areas. The valve cusps are thickened and shortened; they are milky white. The coronary arteries in all sections with the presence of an abundant amount of stable atherosclerotic plaques.

Microscopic examination revealed weak blood filling, mild interstitial edema, and severe hypertrophy of the left ventricular muscle fibers. The wall of the right ventricle and the interventricular septum contained areas of cardiomyocyte necrosis with perifocal inflammatory infiltration, mainly of polymorphonuclear leukocytes, and severe stromal edema.

The tissue of the lower lobes of the lungs is moderately full-blooded, in the upper parts of the lungs it is predominantly airy, of a doughy consistency. Thickened, dense walls of the bronchi protrude from the surface of the lung cut, a moderate amount of pink foamy fluid flows down.

Microscopic examination revealed lung tissue with moderate fibrosis and scattered lymphoid infiltration along the interalveolar septa, fibrosis of the bronchial walls and lymphoid infiltration with the formation of lymphoid follicles.

Final anatomical pathology diagnosis. Underlying disease: type 2 diabetes mellitus with multiple complications: diabetic macroangiopathy: ischemic infarction of the right parietal-occipital region of the brain (6 cm in diameter) with secondary hemorrhagic transformation; myocardial infarction of the right ventricle and interventricular septum type 2 (from 6 to 12 hours ago), diabetic microangiopathy: diabetic nephropathy.

Complications of the underlying disease: pulmonary edema. Cerebral edema with dislocation syndrome. Acute ulcer of the duodenal bulb (symptomatic). Right-sided hydrothorax (up to 500 ml).

Associated diseases: Fahr's disease (idiopathic ferrocalcinosis of the cerebral arteries). Hypertension: concentric myocardial hypertrophy (heart weight 450 g, myocardial thickness of the left ventricle 1.6 cm, right ventricle – 0.5 cm). Chronic pyelonephritis, outside of exacerbation. Reducible umbilical hernia.

Conclusions

RVMI in the demonstrated clinical case is type 2 MI pathogenetically, i.e. secondary to ischemic imbalance and it is not associated with coronary artery disease. This is confirmed by the autopsy results, where the patient showed no signs of coronary artery disease.

One of the leading factors producing the development of such myocardial damage can be considered the development of cerebrocardial syndrome, which occurs in patients with strokes in the form of a number of metabolic disorders, including excessive activation of the hypothalamic-pituitary system with excessive secretion of catecholamines [16], which, in turn, contributes to the disruption of calcium channels, leads to the development of arrhythmias and activation of lipid peroxidation. Despite the fact that morphologically, with an excessive catecholamine response, the development of periaxonal necrosis in the myocardium and the formation of cardiomyopathy, for example, takotsubo [17], are characteristic, the authors of this observation believe that the systemic action of catecholamines cannot be neglected. The patient had normal and high blood pressure, which additionally created a load on the myocardium during hospitalization.

An additional difficulty in diagnosing RVMI in this case was the presence of Chronic Obstructive Pulmonary Disease with chronic pulmonary heart disease in the patient, which contributed to a high load on the right ventricle and directly contributed to the development of necrosis. High calculated pressure in the RV was interpreted as a manifestation of pulmonary hypertension and possible pulmonary embolism.

The diagnosis of RVMI is challenging; 12-lead ECG with additional right chest leads remains the main diagnostic tool (gold standard) in the physician's arsenal, but in case of untimely diagnosis, no changes on the ECG may be present at all. For accurate diagnosis and evaluation of RVMI, a high clinical index of suspicion is required by combining physical, laboratory and instrumental methods of examination to avoid any adverse complications associated with RVMI.

The complexity of pathological and anatomical diagnostics in this clinical case is due to the interrelation of the patient's existing diseases: diabetes mellitus, Fahr's disease, arterial hypertension – each of these factors individually could be the cause of ischemic cerebral infarction. At the same time, hypertension is not the main disease in ischemic stroke;

Far's disease is detected only microscopically, which indicates a low severity of the process. Diabetes mellitus remains the leading cause of both ischemic cerebral infarction and type 2 MI.

The demonstrated clinical case describes the possibility of developing RVMI in patients with diabetes mellitus, when the main role in the pathogenesis of infarction is played not so much by damage to the coronary arteries [18; 19], but by ischemic imbalance against the background of metabolic disorders and, in this case, the severe condition of the patient with acute cerebrovascular accident against the background of diabetes mellitus.

About the authors

S. S. Yashin

Samara State Medical University

Author for correspondence.
Email: s.s.yashin@samsmu.ru
ORCID iD: 0000-0002-0783-8709

Senior Lecturer

Russian Federation, Samara

N. V. Isakova

Samara State Medical University

Email: s.s.yashin@samsmu.ru
ORCID iD: 0000-0001-8764-7955

PhD (Medicine), Associate Professor of the Department of General and Clinical Pathology

Russian Federation, Samara

S. O. Anisimov

Samara State Medical University

Email: s.s.yashin@samsmu.ru
ORCID iD: 0009-0007-7282-2416

Student of the Institute of Clinical Medicine

Russian Federation, Samara

A. S. Yevgrafova

Samara State Medical University

Email: s.s.yashin@samsmu.ru
ORCID iD: 0009-0005-7412-5062

Student of the Institute of Clinical Medicine

Russian Federation, Samara

References

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