A 30-year-old female, with no prior history of heart disease sought medical treatment for increasing shortness of breath, oedema in the lower limbs, abdominal enlargement from seven days.
She had was adopted, was a smoker, no use of illicit drugs.
Her heart rate was 90 per minute, she had bilateral pitting oedema to mid-shin, her blood pressure was 100/70mm Hg, there were no signs of jugular venous hypertension, the pulmonary and cardiac auscultations were normal. She had ascites and her liver was palpable 3cm below the right costal margin.
Her ECG had shown sinus rhythm, heart rate of 92bm, PR interval of 160ms, left bundle branch block (QRS duration of 130ms).
Her echocardiogram showed severe global left ventricular dysfunction with left ventricular (LV) ejection fraction (EF) 18% using the apical biplane Simpson’s rule, GLS WAS -4.3%, her right ventricle was dilated and impaired (TAPSE 1,6cm).
Her BNP was 2393,90pg/ml.
To exclude coronary artery disease, especially congenital coronary artery disease a coronary CT angiography (CTA) was performed. The coronary CTA scan showed normal findings.
Cardiac MRI showed severe impairment of both left and right ventricular systolic function with a pattern of gadolinium enhancement negative for necrosis or fibrosis.
The patient, after being initially treated with large doses of diuretics and levosimendan, was promptly treated with sacubitril valsartan, empaglifozin, bisoprolol, spironolactone plus vericiguat with rapid titration to maximal tolerated dose. About 1 week later her symptoms began to subside and the patient was discharged in a stable condition.
She was referred for consideration of cardiac transplantation and cardiac resynchronisation therapy defibrillator; blood samples for genetic pathology research were taken. A program of serial echocardiograms was introduced, every 30 days, and was observed after 2 months.
At follow up our patient had an impressive improvement in terms of improved quality of life, NYHA functional class (from III to I), and performance capacity.
A new echocardiographic assessment had shown an LVEF of42% with GLS of-12%.
Conclusions: Four pillars of heart failure therapy and vericiguat addiction with rapid titration represents a safe and effective strategy for the treatment of cardiac decompensation with reduced FE.
Basal / 60 day
EF % 18 /42
GLS % -4.3 /-12.0
E/A 5 /2,1
BNP pg/ml 2393.90 / 889.90
TAPSE cm/s 1.6 / 2.1