A CASE OF COMBINED COURSE OF EBSTEIN ANOMALY AND WOLF-PARKINSON-WHITE SYNDROME

Sadykova Dinara Zeinullovna, Candidate of Medical Sciences, Associate Professor of the Department of "Internal Medicine-2"

NJSC "Astana Medical University"

(010000, Republic of Kazakhstan, Nur-Sultan, Beibitshilik St., 49a, e-mail: dinara1812@mail.ru);

Rib Elena Aleksandrovna, PhD, Assistant of the Department of "Internal Medicine-2"

NJSC "Astana Medical University"

(010000, Republic of Kazakhstan, Nur-Sultan, Beibitshilik St., 49a, e-mail: tarlan186@mail.ru);

Orazaly Sholpan Serikbergenkyzy, post-graduate student

NJSC "Astana Medical University"

(010000, Republic of Kazakhstan, Nur-Sultan, Beibitshilik St., 49a, e-mail: sholpanorazaly@gmail.com);

Mamyrov Zhasulan Telibaevich, post-graduate student

NJSC "Astana Medical University"

(010000, Republic of Kazakhstan, Nur-Sultan, Beibitshilik St., 49a, e-mail: mamyrov_87@list.ru);

Ibrayeva Gulzhazira Zeinulaevna, post-graduate student

NJSC "Astana Medical University"

(010000, Republic of Kazakhstan, Nur-Sultan, Beibitshilik St., 49a, e-mail: arizazhaver@gmail.com);

Spabekov Yessimkhan Baidibekuly, post-graduate student

NJSC "Astana Medical University"

(010000, Republic of Kazakhstan, Nur-Sultan, Beibitshilik St., 49a, e-mail: esim_11_96@mail.ru);

Turanova Assel Danyarkyzy, post-graduate student

NJSC "Astana Medical University"

(010000, Republic of Kazakhstan, Nur-Sultan, Beibitshilik St., 49a, e-mail: asel.turanova@mail.ru);

Seilkhan Aidana Asylkhankyzy, post-graduate student

NJSC "Astana Medical University"

(010000, Republic of Kazakhstan, Nur-Sultan, Beibitshilik St., 49a, e-mail: aidana.seilham@bk.ru);

Bissen Shynar Akhmetkyzy, post-graduate student

NJSC "Astana Medical University"

(010000, Republic of Kazakhstan, Nur-Sultan, Beibitshilik St., 49a, e-mail: shikon_ai@mail.ru).

The purpose of demonstrating this clinical case is to improve the diagnosis of Ebstein's anomaly in patients with arrhythmias, in particular with WPW syndrome, since timely diagnosis and correction of the combined pathology significantly increases the life expectancy of such patients.

The patient, born in 1971, was taken by an ambulance team to the city hospital of Nur-Sultan with complaints of rapid, irregular heartbeat," heart disruption", discomfort in the pericardial area, nausea, double vomiting, general weakness. Arrhythmia attacks from the age of 17 were rare, short-term and were stopped earlier independently. Not examined. During the last 2 years, arterial hypertension with a maximum of 170 mm Hg, hypotensive drugs are taken  situationally. The deterioration happened suddenly when a heart attack occurred. The patient independently took Valerian, validol - without effect. Called the ambulance team due to the remaining symptoms and deterioration of the condition. The ambulance team recorded an ECG, suspected ventricular fibrillation (Fig. 1), administered cordarone 300 mg intravenously (IV) - without effect, the patient was taken to an urgent clinic.

In the clinic, hospitalized in the cardio intensive care unit.  During physical examination: the patient's condition is severe due to arrhythmia syndrome with high heart rate. The patient is conscious, adequate, oriented. The color of the skin and visible mucous membranes is normal.  No peripheral edema. The skin turgor is preserved. Subcutaneous fat is moderately developed.  With percussion of the lungs, a clear pulmonary sound is heard. Breathing in the lungs is vesicular, weakened in the lower parts, no wheezing is heard.  Breathing 20/min.The heart area is not visually changed. The limits of relative stupidity are normal. Heart activity is irregular, heart rate 240 / min. Blood pressure 110/70 mm Hg. The abdomen is soft and painless. Liver at the edge of the costal arch.  The symptom of pounding is negative. On an ECG atrial fibrillation with a heart rate of  200/min. Wolf-Parkinson-White syndrome (WPW) is suspected (Fig. 2).

In the hospital, the patient underwent general clinical analyzes, chest x-ray, ultrasound of the carotid arteries, arteries and veins of the lower extremities - without pathology. A blood test was performed for the content of thyroid hormones to exclude the thyroid etiology of arrhythmia (T3 free 6.76 pmol / L, T4 free 38.34 pmol / L, TSH 0.23 μMU / ml), ultrasound of the thyroid gland (diffuse changes in the parenchyma of thyroid glands). Also, Holter ECG monitoring (HM ECG) was performed during therapy with beta-blockers and cordarone and echocardiography (EchoCG). With HM ECG, the main rhythm atrial fibrillation (AF), tachycardia during the day. During the day, the pattern is incomplete with the transition to complete blockade of the right leg of the bundle His (BRLBH), repeatedly throughout the day - transient conduction to the ventricles through the additional path of the atrioventricular connection (AVC) with a duration of QRS of 176 ms and delta waves in the left channels (Fig. 3). Also recorded single monomorphic ventricular extrasystoles (177 per day) and one episode of paired monomorphic ventricular extrasystoles. No ST segment changes detected.

When conducting echocardiography (the study was conducted on the background of arrhythmia), pathological changes from the left heart were not detected, the contractility of the left ventricle (LV) was normal (ejection fraction 55%). Noteworthy to dilation of the right ventricle (RV) to 34 mm, a decrease in its contractile function (12 mm TAPSE), dilatation of the fibrous ring of the tricuspid valve with tricuspid regurgitation of the 1st degree and pulmonary hypertension of the 1st degree (RSDLA 31 mmHg). The sinus rhythm was restored on the third day of stay in the department medically (received beta-blockers and cordaron according to the scheme). Against the background of the restoration of the sinus rhythm, an HM ECG was again performed, where recorded the main sinus rhythm, shortening of the PQ interval, negative T wave in the left channels, signs of incomplete BPNPG, an episode of ventricular tachycardia from 5 complexes, single and paired ventricular extrasystoles.

The patient was consulted by an arrhythmologist, and it was recommended that routine intracardiac electrophysiological studies (IC EPS) and radiofrequency ablation of an additional route be performed. Discharged with positive dynamics. Then she entered the planned order according to the quota to the City Hospital No. 2 of Nur-Sultan for conducting IC EPS and RFA. At the clinic, upon admission, general clinical tests of blood and urine were performed (without pathology), blood tests for hepatitis B and C, HIV (negative), repeated echocardiography.

Echocardiography diagnosed with congenital heart disease - Ebstein's anomaly with tricuspid regurgitation of 4 degree, significant dilatation of the right atrium (apically 5.8 x 4.7 cm) and the right ventricle (apically 6.4 x 5.0 x 4.6 cm), pulmonary hypertension 2 degree (RSDLA 54 mm Hg), the contractility of the right ventricle is preserved (TAPSE 2.2 cm). Left departments without features (Fig. 4,5).

After detecting signs of an Ebstein anomaly on the Echocardiography, the patient was consulted by a cardiac surgeon and diagnosed with an «Ebstein Anomaly. Tricuspid insufficiency of 4 degrees. Pulmonary hypertension of 2 degrees. CHF FC (by NYHA)». Given the high frequency of development of tachyarrhythmias that were not eliminated before the operation on the tricuspid valve, which in most cases lead to death, it was decided to conduct the first stage of IC EPS and RAH AVC. Tricuspid valve surgery is recommended as planned. On the third day of their stay in the clinic, VSEFI (latent WPW syndrome. Paroxysmal orthodromic orientation tachycardia of the right ventricular posterior paraseptal localization) and effective RAH AVC were performed (20 minutes after RAH tachycardia was not induced by program and accelerating stimulation).

After a comprehensive examination and RAH, the final diagnosis was made: "Hidden WPW syndrome. AVC of posterior-paraseptal localization. Heart rhythm disturbance: paroxysmal form of atrial fibrillation. Condition after effective RAH AVC. Background disease: CHD". Postoperative period was uneventful. Rhythm disturbances was observed (including with the control НM ECG). It was written out with positive dynamics with recommendations:

1. planned plastic tricuspid valve (hospitalization as planned),

2. НM ECG in a month,

3. perindopril 4 mg to correct concomitant arterial hypertension,

4. cardiomagnyl 75 mg / day.

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2.     Danielson, G. K. Ebstein’s anomaly. Editorial, commentaries and personal observations / G. K. Danielson // Ann. Thorac.Surg. – 1992. – Vol. 34, № 4. – P. 396–400.

3.     L. A. Bokeria, O. L. Bokeria, B. N. Sabirov, M. L. Aleksandrova. Long-term results and quality of life in combined operations in patients with Ebstein's anomaly and Wolf-Parkinson-White syndrome // Annals of Arrhythmology. 2010. No. 4. P. 49-58.