HISTORY, CURRENT ILLNESS AND PHYSICAL EXAMINATION

Personal history
32-year-old woman. AF: deceased sister with tetralogy of Fallot. No known drug allergies. CVRF: smoker. Cardiological history: acute myopericarditis in 1999 after respiratory infection. In 2003, residual pericardial effusion persisted in the diaphragmatic and apical face of both ventricles. Echocardiogram showed data compatible with restrictive cardiomyopathy, confirmed by haemodynamic study in 2004. Coronary arteries without lesions. Admission in 2012 for decompensated heart failure in the context of a postoperative period, with good evolution with diuretics. New episode in 2016 that was managed on an outpatient basis with diuretics. No other personal medical history of interest. Pilonidal sinus operation in 2012. Baseline situation: CF II. Usual treatment: seguril 40 mg 3-1-0, aldactone 100 mg 1-0-0-0, hygrotona 25 mg 1-0-0-0, Boi-K 1-1-1.

Present illness
32-year-old woman presenting with progressive worsening over the last 8 months with moderate to mild exertional dyspnoea. She has had to progressively increase the dose of diuretics to control dyspnoea and abdominal distension. Occasionally DPN. Orthopnoea on 2 pillows. No oedema in the lower limbs. Feeling of tiredness and fatigue. Normal or low blood pressure. And on some occasions dizziness due to hypotension. No syncope. Occasionally palpitations of short duration, generally nocturnal. No chest or abdominal pain. Physical examination BP 105/78. HR 75 bpm. Jugular ingurgitation. AP: normal. AC: rhythmic. No extrasystoles. No murmurs or pericardial knock. Abdomen: hepatomegaly three traves. Extremities: no oedema.

COMPLEMENTARY TESTS
Laboratory tests on admission: haemogram: normal. Biochemistry: glucose 97 mg/dl. Cholesterol 138 mg/dl. HDL 46 mg/dl. LDL 80 mg/dl. Triglycerides 63 mg/dl. Creatinine 0.65 mg/dl. Urea 52 mg/dl. Uric acid 7.1 mg/dl. Na 135 mEq/L. K 3.5 mEq/L. Cl 96 mEq/ L. GOT 29 IU/L. GPT 16 IU/L. LDH 198 mg/dL. GGT 236 IU/L. Bilirubin 0,97 mg/dL. Amylase 113 IU/L. CPK 53. Protein 7.5 g/dl. NT-proBNP 1077 ng/dl. Chest X-ray: mild cardiomegaly at the expense of left atrial and right ventricular enlargement. Dilatation of the inferior vena cava. Normal lung parenchyma. Pericardial calcification. ECG: sinus rhythm at 91 bpm. PR 0.14. Biauricular growth. Negative T in V3-V6, II, III and aVF. Transthoracic echocardiogram: left ventricle 42/24 mm. Septum 7 mm. Posterior wall 8 mm. Aortic root 25 mm. Left atrium 45 mm. Shortening fraction 42 %. Left ventricle of normal size and function. Ejection fraction 70 %. Right ventricle of normal size and function. Severe dilatation of the left atrium and moderate dilatation of the right atrium. Coronary sinus dilatation. Aneurysm of the interatrial septum. Thickened myxoid mitral valve with mild insufficiency. Tricuspid valve with mild insufficiency and pulmonary pressure of 40 mmHg. Normal aortic valve. Inferior cava 34 mm without respiratory changes. Slight pericardial effusion in the acute angle of the heart. Posterior and inferior pericardial calcification. No respiratory changes in ventricular filling or hepatic vein flow. Only the interventricular septum shows small respiratory changes in the diameter of the right ventricle with inspiration suggesting constrictive pathology. Tissue Doppler shows diastolic velocities with a restrictive filling pattern in both ventricles and an E/E ratio ́: 3 Haemodynamic study: PVI 120/0-20 mmHg. PAo 120/80 mmHg. PVD 40/10-20 mmHg. Mean DBP 20 mmHg. BP 40/18 (25) mmHg. Mean PCP 20 mmHg. Equal right and left end-diastolic pressures. LV and RV diastolic pressure with dip-plateau morphology and without reciprocal respiratory changes, only with inspiration a decrease in LV pressure < 10 % is observed. Oximetry: SVC 63 %, RV 65 %, PA 63 %, LV 96 %, Ao 96 %. CO (Fick) 2.5 l/min. IC (Fick) 1.8 l/min. PVR 2 UW. Indexed PVR 2.7 UW.m2 SVR: 29 UW. Coronary angiography: normal coronary arteries. Pericardial calcification predominantly at the apico-inferior level.
Conclusion: high and equal right and left end-diastolic pressures. Dip-plateau pressure in both ventricles. No reciprocal respiratory changes, which is suggestive of predominant myocardial restriction. Ergospirometry: performed on a cycle ergometer with a power increase of 15 W/min. Duration of effort 5 ́10". Peak O2 consumption 14.7 ml/kg/min (predicted 36.2 ml/kg/min). Ventilatory CO2 equivalent 25.0. Anaerobic threshold 7.9 ml/kg/min. Functional grade III. O2 consumption is 41% of its theoretical value. Cardiac CT: Agatston score 0. Epicardial coronary arteries without lesions. Intense concentric calcification of the pericardium with severe involvement of the free wall of the right ventricle, diaphragmatic face of both ventricles and isolated foci and lateral face of the left ventricle. There are also areas of calcification in front of the root of the great vessels. The greatest calcification is at the level of the free wall of the right ventricle with thicknesses between 5 and 10 mm. Aneurysmal inferior vena cava with a diameter of 60 x 57 mm. Dilatation of suprahepatic veins. Ventricular cavities apparently of normal size. At the level of the apex of the right ventricle within the thickness of the pericardial fat there is an area of contrast extravasation of 10 x 9 mm which could be a pseudoaneurysm of the right ventricular apex. Aneurysm of the interatrial septum. Severe dilatation of the left atrium and moderate dilatation of the right atrium. Pulmonary parenchyma without significant alterations. Cardiac MRI: left ventricular ejection fraction 66%. Concentric calcified thickening of the pericardium being more important at the level of the free wall of the right ventricle. Dilatation of both atria. Aneurysm of the interatrial septum. Normal global and segmental contractility of the left ventricle. Dyskinetic movement of the interventricular septum. Good right ventricular function. Small pericardial effusion in inferior aspect. In the late enhancement sequences no pathological uptakes were observed.

EVOLUTION
A 32-year-old woman with a diagnosis of restrictive cardiomyopathy was admitted due to worsening heart failure despite outpatient intensification of diuretics. During admission, a new echocardiogram was performed, which showed data compatible with myocardial restriction, although with signs suggestive of a constrictive component. To better characterise the restrictive and constrictive components, a CT scan and cardiac MRI were performed, which showed intense calcification of the pericardium, especially at the RV level with myocardial infiltration at that level, and preserved biventricular function without pathological enhancements. The diagnosis of constrictive pericarditis was therefore confirmed, probably in relation to the history of acute myopericarditis in adolescence. She improved clinically and was discharged with the following treatment: seguril 2-1-0, aldactone 25 mg 1-2-0; boi-k 1-1-1.

Subsequently, an outpatient haemodynamic study was also performed, with epicardial coronary arteries without significant lesions, and right catheterisation showing: increased and equalised right and left end-diastolic pressures, with no reciprocal respiratory changes. The catheterisation data suggested a predominance of myocardial restriction, but given the findings in the imaging tests, it was decided to present the patient for a medical-surgical session to consider pericardiectomy, which was finally rejected due to intense intramyocardial calcification, anatomically limiting the possibilities of a complete pericardial resection surgery. Given the poor clinical evolution with the need for increased diuretics and the impossibility of pericardiectomy, it was decided to refer the patient to a heart transplant centre. She had given up smoking and was being treated with furosemide 40 mg 4 tablets a day, aldactone 100 mg a day, hygrotona 25 mg a day and potassium 2 tablets every 8 hours. She had an episode of atrial fibrillation, and was started on anticoagulation with acenocoumarol and frequency control with bisoprolol 5 mg per day. After completing the pre-transplant study, she has no contraindications and is currently on the waiting list.

DIAGNOSIS
Constrictive pericarditis with myocardial restriction component due to myocardial infiltration. Heart failure in functional class iii, on the heart transplant list. Persistent atrial fibrillation, with heart rate control.
