HISTORY, CURRENT ILLNESS AND PHYSICAL EXAMINATION

History
No known allergies. Several episodes of rheumatic fever.

Present illness
A 15-year-old male patient from Malawi presented with moderate exertional dyspnoea for years, associated with asthenia, orthopnoea and some episodes of paroxysmal nocturnal dyspnoea, with progressive worsening of his functional class, up to dyspnoea on slight exertion. He was assessed by doctors in his home country, where he was diagnosed with severe mitral stenosis, mild mitral insufficiency, severe tricuspid insufficiency and severe pulmonary hypertension. Due to clinical worsening and the impossibility of adequate treatment in his place of origin, he was transferred to Spain by an NGO for the corresponding surgical treatment. After the transfer, the patient presented deterioration of his usual dyspnoea with dyspnoea at rest, concomitant abdominal pain in both hypochondria, dyspepsia and several episodes of emesis after food intake, so it was decided to admit him to the Paediatrics Department to optimise depletive treatment prior to surgical treatment.

Physical examination
Weight: 27 kg BP: 90/60 mmHg, HR: 90 bpm, SO2: 100 % with O2. Neck: jugular ingurgitation. Cardiac auscultation: R1 and R2 rhythmic and regular. Intense systolic-diastolic murmur in mitral focus radiating towards the axillary region. Pulmonary auscultation: bibasal hypoventilation, more marked in the right base. Abdomen: hepatomegaly 4 traveses, slightly painful on palpation in the right and left hypochondrium. Splenomegaly. Extremities: symmetrical, without oedema.

COMPLEMENTARY TESTS
CBC: haemogram: leucocytes 11,600 /ul. Neutrophils 82.4 %. Monocytes 9.8 %. Lymphocytes 7.2 %. Hb 11 g/dl. Ht 33.5 %. Platelets 106.000 /ul. Biochemistry: glucose 120 mg/dl, urea 34 mg/dl, creatinine 0.5 mg/dl, Na 135 mEq/L, K 3.9 mEq/L. ECG: RS at 92 bpm, p wave of biatrial growth, incomplete BRD + HBPII, signs of hypertrophy and growth of right cavities, Axis deviated to the right, ST descent of 1 mm in V3-V5. Chest X-ray: cardiomegaly at the expense of the right chambers, fluid in cystole.

Transthoracic echocardiogram: situs solidus, levocardia with levoápex. LVEF 65 %. Right chambers severely dilated. RV with preserved contractility. Severe TR due to lack of coaptation of the leaflets by annular dilatation, AD-VD gradient of 60 mmHg. PAPs 70-75mmHg. Mitral valve: thickened anterior leaflet, domed opening and anterior displacement of the posterior leaflet, fused commissures, mean transmitral gradient of 16 mmHg, area by planimetry of 4 mm and by THP of 0.75 cm2. Mild MI. Dilated IVC. Slight pericardial effusion.

EVOLUTION
After stabilisation of congestive heart failure, with adequate response to depletive treatment, given contraindication to treatment with balloon valvuloplasty due to great valve deconstruction, surgical treatment was performed with replacement of the mitral valve with a biological prosthesis, plus tricuspid annuloplasty. In the initial postoperative phase, he required vasoactive drugs and pulmonary vasodilators due to a drop in BP and oliguria, with haemodynamic stabilisation after 24 hours. He was transferred to the paediatric ward from where he was discharged on the eighth day. Postoperative echocardiographic control showed a normofunctioning bioprosthesis, with mean gradient of 3 mmHg, maximum E wave of 15 mmHg, normofunctioning LV, no pericardial effusion, mild TR.

DIAGNOSIS
Rheumatic severe mitral stenosis, mild mitral regurgitation. Severe tricuspid regurgitation. Severe pulmonary hypertension. Congestive heart failure.
