HISTORY, CURRENT ILLNESS AND PHYSICAL EXAMINATION
56-year-old male, with no known drug allergies, with a history of arterial hypertension being treated with two drugs and dyslipidaemia; with no personal or family history of heart disease, heart failure or sudden death.
Three days ago she reported an episode of oppressive chest pain radiating to the jaw, left upper limb and back, after intense exertion (carrying a weight), of several hours' duration, accompanied by vegetatism. She did not consult for this episode.
On the day she sought medical attention, she presented a new episode of chest pain similar to the previous one, while making intense effort, and so she called the Emergency Medical Service. On initial assessment: BP 123/99 mmHg, HR 105 bpm and SatO2 100% with nasal goggles at 2 L/min. Initial physical examination revealed a pansystolic murmur in the mesocardium. An electrocardiogram was performed with pain, showing sinus rhythm, AV block Mobitz I. Narrow QRS. Pathological Q waves in inferior face and inferior ST elevation with DIII>DII maximum of 4 mm in DII and specular decrease in DI and aVL of up to 5 mm. ST elevation V3-V6 with negative anterior T waves. The AMI code was activated and the patient was transferred to our centre. Acetylsalicylic acid 250 mg, clopidogrel 600 mg, enoxaparin 30 mg and fentanyl 50 mcg were administered. During the transfer, hypotension with BP 73/42 mmHg.
He was transferred to the haemodynamics ward for urgent coronary angiography.
During the procedure, dobutamine and noradrenaline were started, requiring dose increases up to 20 mcg/kg/min and 20 ml/h respectively. Coronary angiography revealed 3-vessel coronary artery disease: Two severe lesions (90 %) in tandem in the anterior descending (AD) at the level of the middle third (pre- and post-exit of the second diagonal) with involvement of the ostial-proximal segment of the second diagonal (80 %); first marginal occlusion at the middle level (100 %) with no distal bed visualisation and proximal occlusion of the right coronary artery (RCA) (100 %). PCI was performed to the proximal-medial DC with implantation of a 3 x 36 mm non-drug-eluting stent at 20 atm, with good results. Urgent transthoracic cardiac ultrasound was performed during the procedure, which showed an anfractuous ventricular septal defect (VSD) located in the basal septum, about 30 mm in diameter, with a hypocontractile RV; therefore, intra-aortic balloon counterpulsation (IABP) was implanted.
After the procedure, he was admitted to the Coronary Unit for stabilisation. The case was discussed with the Coronary Unit of our reference centre and he was transferred for surgery.
Physical examination: conscious and oriented in all three spheres. Signs of poor distal perfusion. Cardiac auscultation: rhythmic heart tones, pansystolic murmur in mesocardium III/VI not radiated. Jugular ingurgitation +++. Respiratory auscultation: physiological vesicular murmur without added pathological sounds. No declining oedema.

COMPLEMENTARY TESTS
ECG: RS, BAV Mobitz I, narrow QRS, inferior Q waves and inferior ST elevation with III > II of maximum 4 mm in III and specular decrease in I and aVL of up to 5 mm; also ST elevation in V3-V6 with negative anterior T wave.
CBC: glucose 290 mg/dl, urea 65 mg/dl, creatinine 2.05 mg/dl, CK 1199 U/L, troponin I 37.73 ng/ml, sodium 136 mmol/l, potassium 4.62 mmol/l. Leukocytes 13430, haemoglobin 14.2 g/dl, haematocrit 41.5%, platelets 95,000.
Emergent coronary angiography: right dominant coronary arteries: significant stenosis. Truncus description: good calibre, no significant lesions.
Description of the anterior descending artery: vessel of good calibre and extension. Two severe tandem lesions at the level of the middle third (pre- and post-exit of the second diagonal) with involvement of the ostial-proximal segment of the second diagonal. Rest of the vessel with non-significant diffuse irregularities. Good distal bed, TIMI 3 flow.
Circumflex description: occluded at the level of the first middle marginal with no distal bed visible. Intermediate branch developed, no lesions. Right coronary description: occluded proximally. Procedure: cannulation of the DC ostium with AR2 catheter, passing a guide wire distally from the posterior descending artery. Thrombus extract is passed, which is productive and TIMI 2 flow appears, and a 3 x 36 mm non-drug-eluting stent is implanted at 20 atm, with good results. Conclusions: right dominance. Three-vessel disease. PCI to proximal-medial DC. Wide VSD
Transthoracic cardiac ultrasound: ventricular septal defect with an anfractuous course located in the basal septum, about 30 mm in diameter, RV hypocontractile.
Transthoracic cardiac echocardiography prior to hospital discharge: non-hypertrophic left ventricle, slightly dilated, with severely depressed systolic function (EF 27%) due to extensive septal and inferoposterior akinesia, patch at the level of the interventricular septum, with no flow between the two ventricles; mild aortic insufficiency and moderate-severe functional mitral insufficiency. Right ventricle slightly dilated. Depressed right ventricular dynamics (TAPSE 11). Mild tricuspid insufficiency that allows estimating normal PAPs.

EVOLUTION
On arrival at the reference centre, in a situation of cardiogenic shock refractory to pharmacological treatment and IABP due to large post-infarction VSD; urgent transthoracic cardiac ultrasound was performed which showed a globally normal contractile left ventricle, midbasal inferoposterior akinesia, dilated right ventricle, slightly hypocontractile; and a wide ventricular septal defect of up to 4 cm in anteroposterior diameter and 3 cm in apicobasal diameter.
An ECMO (extracorporeal membrane oxygenation) peripheral venous-arterial system was implanted as a bridge to corrective surgery for VSD. Subsequently, there was a tendency towards stability, allowing a progressive decrease in vasoactive amine support with adequate ECMO functioning. On the fourth day of admission, VSD closure of the basal and middle posterior septum was performed with a bovine pericardial patch and bypass of the internal mammary artery to the anterior descending artery. Echocardiogram was performed after surgery, decreasing support with ECMO, in which stable left ventricular function, hyperdynamic segments with preserved mobility. Non-dilated right ventricle, with severe dysfunction. Eccentric tricuspid insufficiency directed to the low interatrial septum. No ventricular septal defect.
It was decided to continue ECMO support and treatment was associated with levosimendan.
The patient continued to evolve well, decreasing ECMO litres and tolerating IABP 1:4. In view of the favourable evolution, it was decided to withdraw ECMO with 1:1 IABP support and dobutamine at 8 mcg/kg/min. Subsequent good evolution, allowing withdrawal of IABC and inotropes, and introduction of oral treatment with beta-blockers, ACE inhibitors and aldosterone receptor antagonists.
Complications during admission included: paroxysmal atrial fibrillation, prolonged respiratory weaning, requiring percutaneous tracheostomy for prolonged orotracheal intubation; due to respiratory failure secondary to alveolar haemorrhage and acute respiratory distress syndrome; nosocomial pneumonia in the right lower lobe (Enterobacter cloacae and Citrobacter koseri) and urinary tract infection by multidrug-resistant Serratia marcescens.
The patient progressed favourably, tolerating neurohormonal treatment, with negative water balance and low-dose diuretics. A control ultrasound was performed prior to hospital discharge, showing a slightly dilated left ventricle, with severely depressed systolic function (EF 27%) due to extensive septal and inferoposterior akinesia; patch at the level of the interventricular septum, with no flow between the two ventricles; mild aortic insufficiency and moderate-severe functional mitral insufficiency. Slightly dilated right ventricle. Right ventricular dysfunction.

DIAGNOSIS
Acute myocardial infarction with inferior ST elevation Killip Kimball IV.
Proximal DC thrombotic occlusion.
PPCI to proximal-medial DC. 3-vessel coronary artery disease (middle DA, 1st OM and CD) wide septal septal septal defect. Closure with bovine pericardial patch. Internal mammary artery bypass to LAD.
Ischaemic cardiomyopathy with severe LV systolic dysfunction. Moderate-severe functional mitral insufficiency.
