CMR report:
The CMR report revealed mildly enlarged LV size without LV hypertrophy with moderately reduced LV systolic function LVEF =35% and thin and aneurysmal LV apex –Normal RV size with mildly reduced RV function (RVEF=42%).
LGE findings: In all inferolateral and septal segments, there was widespread patchy subepicardial to mid wall fibrosis, as well as sub endocardial fibrosis in the left ventricular apex.
Recent myocarditis possibly due to viral infection, as well as an autoimmune or sarcoidosis disease, should be evaluated, according to CMR tissue characterization criteria.
(Figure 1)
On the first visit in our clinic on January 2021, she complained of a dyspnea on exertion, NYHA-FC II and a mild fatigue. Her vital signs were stable with a blood pressure (BP) of 115/75 mmHg a heart rate (HR) of 90 beats per minute (bpm), she did not have fever and systemic oxygen saturation was 96%.
Her physical examination was unremarkable except for bi-basilar fine crackles. She did not have any skin lesions in terms of her LP. Her new echocardiogram revealed no new changes compared to her last exam that was mentioned earlier. On laboratory tests, there was no leukocytosis, hemoglobin was 12.1 g/dl, the renal and liver function tests were within normal limits and N terminal –pro natriuretic peptide (NT-pro BNP) was 885 pg/ml. The thyroid function test was within normal limits with a higher than normal anti TPO.
The standard anti failure therapies was already started and the heart failure (HF) guideline directed medical therapies with lisinopril, bisoprolol, eplerenone, furosemide as well as levothyroxine were continued for her. A few days later, her HF symptoms were aggravated and she developed progressive dyspnea, orthopnea, paroxysmal nocturnal dyspnea, abdominal pain and nausea. She was admitted with the above mentioned symptoms and rapidly progressed to a pre-shock state. She had a BP of 85/60, an HR of 120 bpm, a distended jugular vein, bi basilar lung crackles, hepatomegaly, mild ascites and 2+ pretibial pitting edema. The creatinine was slightly increased (1.7-1.8 mg/dl). The alanine aminotransferase (ALT), aspartate aminotransferase (AST) and total bilirubin levels were 46 U/L,40 U/L,1.5 mg/dl, respectively, NT-Pro BNP was more than 18000 pg/ml and cardiac troponin –I (CTnI) was elevated. Her ECG showed a sinus tachycardia with low voltage QRSs and Q wave in leads of I, III, aVF, V1-V6, ST segment elevations and T wave inversions/flattening in V1-V6. (Figure 2)
New echocardiography showed mild LV enlargement with severe systolic dysfunction, LVEF 15-20%, global hypokinesia and apical akinesia mild RV enlargement with severe systolic dysfunction [Tricuspid annular plane systolic excursion( TAPSE)=10 mm, RV peak systolic myocardial velocity by tissue Doppler (RV Sm) =6 cm/sec], moderate to severe mitral (MR) and tricuspid regurgitation(TR), a tricuspid regurgitation gradient (TRG) of 25 mmHg and a plethoric inferior vena cava (IVC) with an IVC size of 23 millimeter.
Considering her clinical condition, we had to start intravenous (IV) inotrope and diuretic. After starting intravenous (IV) inotrope (Milrinone) and furosemide infusion, she was scheduled for emergent endomyocardial biopsy (EMBX) due to deterioration in hemodynamic and clinical course. The right heart catheterization data at the time of EMBX has been shown in table 1;
Table 1: Hemodynamic findings of patient