Particularities of the right cord pathology in a young patient: non-compact right ventricle, a rare clinical case

Objective: Presentation of a rare case of right heart pathology, non-compact right ventricle (VD) in a young patient, pathology about which there are only a few communications in literature.
Methods: A 35-year-old female patient, known with a history of (probably autoimmune) thrombocytopenia, is admited for constrictive precordial pain with resting relief, insertional dyspnea with progressive aggravati-on, orthopnea and continuous fatigue condition at mi-nimal efforts, started about a week ago. Transesophage-al echocardiography: interatrial septum with foramen ovale protruding to the left atrium without interatrial septal changes which is continuous with no defect, right atrium and ventricle very dilated, 2-th grade tricuspid regurgitation with RV- RA gradient=45 mmHg MRI: dilated right cavities: RA=60/56 mm (24 cm² area), and RV=52 mm transverse axis, 70 mm longitudinal axis. Non-compact right ventricle aspect is predomi-nantly in the apical and infundibular region. Global RV hypokinesis associated with a paradoxical interventri-cular septum movement. RVEF=64%, EDRV=238 ml, ESRV=86.9 ml. Tricuspid pulmonary valve is with mo-derate regurgitation. Signs of PAH are present.
Results: Diagnosis problems: Pulmonary embolic heart disease (deep vein doppler ultrasound, thora-cic angio-CT negatives), congenital heart disease, in-terseptum communication (high located ASD, other structural defects negatives by transesophageal echo-cardiography), pulmonary hypertension secondary to other pathologies (systemic autoimmune disease, not sustained by negative inflammatory and immunologi-cal analysis results, broncho-pulmonary pathology cli-nically and radiologically absent). Final diagnosis: Se-vere Pulmonary Arterial Hypertension. Non-compact Right Ventricle. Right Congestive Heart Failure, with peripheral edema and serous fluid collections. Treat-ment management was focused on sustained diuretics therapy (furosemide 80 mg iv initial, subsequently 20 mg/day oral adm., 50 mg spironolactone), K and Mg supplements 39/12 mg/day, sildenafil 2×25 mg, perin-dopril 2.5 mg/day, metoprolol succinate 25 mg/day.
Conclusions: Prognosis on short-term is relatively good (as was the case with our patient) and is related to obtaining and maintaining cardiac compensation, and prevention of complications. In the long term, the prognosis remains reserved, worsened by the evolution of pulmonary arterial hypertension. Theoretically, the patient would have a cardiac transplant indication in the future.

ISSN – online: 2734 – 6382
ISSN-L 1220-658X
ISSN – print: 1220-658X
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CODE: 379
CME Credits: 10 (Romanian College of Physicians)