HISTORY, CURRENT ILLNESS AND PHYSICAL EXAMINATION

PERSONAL HISTORY
Father died of acute myocardial infarction (AMI) at the age of 54.
Sporadic smoker for 5 years. Very occasional alcohol consumption.
No hypertension, diabetes mellitus or dyslipidaemia.
No history of chest trauma or traffic accidents.
No previously known cardiopathies. She consulted the emergency department 4 years ago due to sudden chest pain radiating to the jaw. Chest X-ray, electrocardiogram and cardiac enzyme serum tests were normal.
Non-specific low back pain.
No usual medical treatment.

CURRENT ILLNESS
A 32-year-old man consulted the cardiology department due to episodes of central thoracic pain radiating to the left upper limb and cervical region, of years of evolution. At the beginning, the pain occurred with heavy exertion, but in recent weeks it increased in frequency and the exercise threshold for its onset was reduced. The pain does not change with postural changes and does not subside after taking paracetamol or non-steroidal anti-inflammatory drugs. She denies associated dyspnoea, no syncope or presyncope. His primary care doctor detected a panfocal murmur on auscultation and referred him to our clinic for further investigation.

PHYSICAL EXAMINATION
Normoconstituted. Blood pressure 130/70. Heart rate 90 bpm. O2 saturation 99% breathing room air.
Mild jugular engorgement. Jumping heart tones, aortic murmur systolic III/VI and diastolic III/IV.
Good bilateral vesicular murmur.
Peripheral pulses preserved and symmetrical.
No congestive data.

COMPLEMENTARY TESTS
Chest X-ray 4 years earlier: normal cardiothoracic index. No abnormalities in the cardiac silhouette.
Chest X-ray in the emergency department: postero-anterior projection, with marked mediastinal widening.
Electrocardiogram (ECG) in consultation: sinus rhythm 80 bpm, left axis, signs of LV enlargement with sharp T waves in precordial leads. Q wave in V5-V6.
Transthoracic echocardiogram in consultation: LV globular, hypertrabeculated and dilated with LVEDDVI 67 mm, mild LV hypertrophy of 13-14 mm, slightly depressed systolic function (LVEF 45%). LA normal. Normal right chambers. Large aortic aneurysm, with a maximum diameter of 10 cm in the ascending section, rest of the vessel not visible. Trivalve aortic valve with severe functional aortic insufficiency due to loss of morphology of the sinotubular junction. Rest of valves normal. Pericardium without effusion.
Urgent laboratory tests: no pathological values. Serial troponin T hs negative (< 0.014 μg/l).
D-dimer 0.5 μg/ml.
Thoracic-abdominal CT angiography (urgent) (images 5-10): ascending aortic aneurysm and AoAD type A with an entry portal in the anterior wall of the aortic root, extending into the abdominal aorta. The aneurysm has a maximum diameter of 11 cm. In the ascending aorta, the existence of an intimal flap in the anterior margin of the aortic root is observed above the valvular ring, in relation to type A aortic dissection. The origin of the right coronary artery arises from the lumen of smaller calibre, which is the true lumen. As for the coronary tree, a normal origin is seen with the right coronary artery (RCA) arising just at the limit of the beginning of the dilatation of the aorta. As for the supra-aortic trunks, involvement of the left common carotid artery and left subclavian artery. In the study of the abdominal aorta, the left main renal artery and the inferior mesenteric artery arise from the small lumen, the true lumen. The dissection ends before the aortic bifurcation.
Angio-CT chest at 3 weeks: dilated cardiomyopathy with significant increase in the cross-sectional diameter of the left ventricular cavity. No notable alterations were observed in the valved tube; there were no gross alterations in the coronary arteries or signs of periprosthetic leakage. Likewise, no significant alterations were identified in the stent placed in the true lumen of the proximal segment of the descending aorta.
No collapse or thrombosis was observed. Images of dissection persist below the stent and in the segment adjacent to the stent the false lumen is only partially filled with contrast, either due to partial thrombosis or because it has not had time to fill with contrast during image acquisition. There is no evidence of dissection in the supra-aortic trunks, although there are some linear images, especially in the left common carotid artery, which in principle seem to be artefacts of the high concentration of contrast in the innominate vein. There is a significant bilateral pleural effusion that does not average high density to think it is a haemothorax.
Conclusions: Postoperative changes as described in the commentary. Significant bilateral pleural effusion.
Post-surgical echocardiogram (3 months after surgery): very globular, hypertrabeculated and dilated LV with LVEDD 77 mm, (even more than before surgery) severely depressed LVEF estimated at 20% with overall hypocontractility. Valved aortic tube with criteria of prosthetic normofunctioning and low gradients (max 16, mean 10 mmHg). Signs of low LVOT output (IVT < 10 cm). Mild-moderate functional mitral regurgitation. Rest of valves normal. Pericardium without effusion.
Angio-CT of thoracoabdominal aorta post-surgery (10 months): ascending valved tube and trifurcated in patent arch, without images of periprosthetic leaks or other notable alterations. Aortic valve prosthesis. The endovascular prosthesis in the descending aorta is adequately distended, patent, and does not present images suggestive of kinking, migration, endoleaks or other complications. Chronic AoD in the descending thoracic aorta distal to the prosthesis and in the abdominal aorta, with both lumens patent. Maximum diameter (including both lumens) of 50 mm. Rest unchanged. Known dilated cardiomyopathy. No pleural or pericardial effusion identified. Pacemaker in left anterosuperior chest wall with catheter ends in right cavities. Conclusion: AoD treated surgically and endovascularly, with no signs of complications or changes with respect to the previous study.
Follow-up echocardiogram (16 months after surgery): LV not dilated, with DTD of 42 mm, with moderate concentric hypertrophy of its walls of 15 mm overall thickness. Preserved LVEF, calculated at 66% by biplane Simpson with no alterations in segmental contractility. Mitral filling with altered relaxation pattern without diastolic dysfunction criteria. Left atrium (LA) of 39 mm transverse diameter. Right chambers of normal dimensions. Right ventricular (RV) systolic function preserved. Ascending aorta tube 24 mm. 40 mm abdominal aorta. Mechanical aortic prosthesis in aortic position with normal appearance, slightly elevated antegrade gradients with Vmax of 3.2 m/s and mean gradient of 22 mmHg, but with a V1/V2 of 0.35 (IVT in LVOT of 25 cm). A very mild periprosthetic insufficiency jet is seen at 1 hour in parasternal short axis. Structural appearance and normal function of the mitral valve. No tricuspid insufficiency, low probability of pulmonary hypertension.
Genetic test: no pathogenic mutations found.

CLINICAL EVOLUTION
The ultrasound findings in consultation were more suggestive of a chronic course of an aneurysm than of AoD as the initial problem. However, the clinical manifestations of chest pain made it necessary to rule out this pathology. He was referred to the emergency department to be assessed by angio-CT to rule out dissection and, if there was no dissection, to schedule cardiac surgery as a priority, given that with such dimensions the risk of aortic rupture was high.
On arrival at the emergency department, a chest X-ray was performed showing mediastinal widening not present in the previous X-ray performed in 2010. An angio-CT scan confirmed the dilatation of the ascending aorta and showed the presence of Stanford type A A AoD with an entry point in the anterior wall of the aortic root. The patient was referred to the cardiovascular surgery referral centre for urgent surgery and the aortic root was replaced with a valved tube using the Bono-Bentall technique with coronary artery reimplantation and stenting of the descending aorta. Postoperatively, left recurrential paralysis with secondary aphonia was observed.
Three days after discharge, the patient was admitted to the emergency department for mechanical chest pain and self-limited loss of consciousness. A thoraco-abdominal CT angiography was performed and, apart from the surgical changes considered to be within the expected range, a significant bilateral pleural effusion was described which was considered to be secondary to the operation and which resolved with the passing of days. A cranial CAT scan was also performed, which was normal.
Post-surgical echocardiogram showed a dilated and globular LV with severe systolic dysfunction. After 3 months, with optimal medical treatment, severe dysfunction with New York Heart Association (NHYA) functional class II-III was maintained, so it was decided to implant an implantable cardioverter defibrillator (ICD) for primary prevention. During subsequent follow-up while maintaining optimal medical treatment, at 16 months he presented a reduction in LV volumes and LVEF recovery to normal ranges. The patient is able to perform moderate exertion and only suffers from back discomfort that forces him to stand intermittently. A striking fact was that in the subsequent follow-up his sister presented with a very extensive type B aortic dissection, complicated and requiring urgent surgical intervention. A genetic study was requested and no pathogenic mutations associated with aortic pathology or connective tissue disease were detected. Simultaneous follow-up by cardiovascular surgery, periodic monitoring of the descending thoracic aorta by angio-CT was requested (stable size of the descending thoracic aorta of 5 cm with a patent false lumen).
Current treatment: aldocumar, omeprazole 20 mg/24 hours, ramipril 5 mg at breakfast and 2.5 mg at dinner, bisoprolol 2.5 mg/24 hours, spironolactone 25 mg/24 hours, lorazepam 1 mg/24 hours.

DIAGNOSIS
11 cm dissecting aortic aneurysm with Stanford type A dissection.
Emergent surgical intervention by aortic replacement and valve replacement.
Severe postoperative LV dilatation and dysfunction, which reversed after optimal medical treatment.
ICD carrier in primary prevention.
