ABC | Volume 113, Nº3, September 2019

Case Report Immobile Tricuspid Valve: Incidental Finding in a Case of Terminal Cardiomyopathy Due to Thalassemia Major Erman Cils al Adana City Education and Research Hospital, Adana – Turkey Mailing Address: Erman Cilsal • Adana City Education and Research Hospital - Kisla Mh Mithat ÖZsan Bulv, Adana - Turquia E-mail: ermancilsal@gmail.com Manuscript received May 03, 2018, revised manuscript December 05, 2018, accepted December 05, 2018 Keywords Cardiomyopathies; beta-Thalassemia/genetics; delta- Thalassemia/genetics; Arritmias Cardíaca; Tricuspid Valve/ abnormalities; Echocardiography/methods. DOI: 10.5935/abc.20190195 Table 1 – Echocardiographic measurements of the patient Data Values M-Mode Measurements LVID, cm 4.9 Ejection Fraction 48 Fractional shortening, % 24 RVID, cm 4.8 Doppler Measurements Tricuspid E, cm/s 81 Tricuspid A, cm/s 25 Tricuspid E/A 3.2 Tissue Doppler Measurements (RV) E’ cm/s 12.1 A’ cm/s 7.8 E’/A’ 1.55 E/E’ 6.7 S’ 11.4 IVCT, ms 65 IVRT, ms 78 RV MPI 62 A: peak late diastolic velocity;A’: late diastolic velocity; E: peak early diastolic velocity; E’: early diastolic velocity; ET: ejection time; IVCT: isovolumic contraction time; IVRT: isovolumic relation time; LVIDd: Left ventricular internal diastolic diameter; MPI: myocardial performance index; RV: right ventricle; RVID: right ventricular internal diameter; S’: systolic velocity; Tissue Doppler imaging of the tricuspid valve. Introduction Thalassemia Major is an inherited disorder caused by impaired synthesis of the B globin chain and characterized by ineffective erythropoiesis that requires regular, lifelong transfusion therapy, which creates a state of iron overload. 1 Once reticuloendothelial stores saturate, iron deposition increases in myocardium such as other parenchymal tissues. 2 Cardiac complications due to this deposition are the leading cause of death. After a silent first decade, iron deposits in the cardiac tissue lead to arrhythmias, systolic and diastolic dysfunction, and congestive heart failure in the second or third decade. 3 In this case report, we present an adolescent girl who did not receive regular iron chelation therapy and had cardiomyopathy, arrhythmia and immobile tricuspid valve secondary to thalassemia major. Case presentation A 14-year-old Syrian girl with Thalassemia Major presented to the emergency roomwith a three-month history of increasing fatigue, dyspnea, and abdominal distension. Her medical history revealed that she had been diagnosed with Thalassemia Major at the age of one year old, and she received irregular erythrocyte transfusion and iron chelation therapy in her country. It was learned that the compliance for previous blood transfusion and chelation therapy was very poor. On general examination, she was undernourished with short stature (body weight < 25 p, height < 3p) and the physical examination revealed dyspnea with a typical facial thalassemic feature without cyanosis. Chest x-ray showed areas of consolidation on both sides of the lungs and increased cardiothoracic ratio (Figure 1). The electrocardiogram showed sinus rhythm with 70/min heart rate and prolongation of QTc value with 0.46 seconds (Figure 2-A). Transthoracic echocardiography revealed both ventricle systolic and diastolic ventricular dysfunction, left ventricle ejection fraction was 48% and fractional shortening was 24% were calculated with a mild left ventricle dilatation (Table 1). Mild-moderate mitral regurgitation and trivial pericardial effusion were also observed. Right ventricular inflow view in systole showing thickened, immobile leaflets of tricuspid valve in a fixed open position, causing mal-coaptation and severe regurgitation without stenosis (see Figure 3 and Video 1). Apical four-chamber view in diastole showed immobile leaflets of tricuspid valve in a fixed open position, as showed by the color Doppler (Video 2) (See additional files Video 3, 4 and 5). Right atrial, right ventricle dilatation and minimal pulmonary regurgitation with mild pulmonary hypertension were also observed. After hospitalization in the intensive care unit, inotropes, diuretics and iron chelation treatment (Dopamine, Dobutamine, Furosemide infusion, Propranolol, Enalapril, Aldactone and Deferoxamine, Deferiprone therapy) started as soon as possible. Cardiac enzymes were sent to screen possible myocarditis, and D-dimer was sent to detect pulmonary thromboembolism. Results were found to be negative. On the seventh day of hospitalization, the electrocardiogram showed 438