Percutaneous Treatment of Aortic Coarctation in Young Adult Supported by Novel Fusion Imaging Technology

Introduction: Aortic coarctation is one of the most frequent misdiagnosed congenital heart diseases and the most commonly missed fetal congenital heart In adulthood the treatment of this pathology is primarily percutaneous. present a case of a 37-year-old male with recent onset of heart failure symptoms due to misdiagnosed congenital aortic coarctation. The execution of a diagnostic multimodality imaging allowed to detect aortic coarctation, which after the multidisciplinary “heart team” discussion, received indication to percutaneous correction of the aortic defect. In the cath-lab, the use of novel digital techniques, integrating the angiographic images with previously acquired angio-CT, allowed a safe stent delivery with minimum amount of contrast injection. Conclusion: Percutaneous treatment of aortic coarctation in young adult is safe and effective. Fusion imaging tools could be very useful to optimize procedural results and sparing contrast dye, suggesting their possible implementation in different percutaneous procedures. Abbreviations: CHD: Congenital Heart Disease; CMR: Cardiac Magnetic Resonance; CoA: Coarctation of the Aorta; CT: Computer Tomography; ED: End Diastolic; EF: Ejection Fraction; ICU: Intensive Cardiologic Unit; LV: Left Ventricle; LVEDd: Left Ventricular End Diastolic Diameter; LVEDv: Left Ventricular End Diastolic Volume


Introduction
Coarctation of the aorta (CoA) accounts for 6%-8% of all congenital heart disease (CHD), but it is the most commonly missed fetal CHD diagnosis, withless than one-third of the cases been detected at prenatal screening. We describe a case of CoA in an adult patient we managed percutaneously.

History of Presentation
We present the case of a 37 years-old, who suffered from recent onset exertional dyspnoea and paroxysmal nocturnal dyspnoea, other than not previously reported lower limb claudicatio. At the out patient visit, elevated values of arterial pressure associated with severe left ventricular dysfunction (EF 0.30) were found, and

ARTICLE INFO ABSTRACT
Introduction: Aortic coarctation is one of the most frequent misdiagnosed congenital heart diseases and the most commonly missed fetal congenital heart disease. In adulthood the treatment of this pathology is primarily percutaneous.

Case Presentation:
We present a case of a 37-year-old male with recent onset of heart failure symptoms due to misdiagnosed congenital aortic coarctation. The execution of a diagnostic multimodality imaging allowed to detect aortic coarctation, which after the multidisciplinary "heart team" discussion, received indication to percutaneous correction of the aortic defect. In the cath-lab, the use of novel digital techniques, integrating the angiographic images with previously acquired angio-CT, allowed a safe stent delivery with minimum amount of contrast injection.
Conclusion: Percutaneous treatment of aortic coarctation in young adult is safe and effective. Fusion imaging tools could be very useful to optimize procedural results and sparing contrast dye, suggesting their possible implementation in different percutaneous procedures.

Management
After multidisciplinary "heart team" discussion, the patient received an indication to percutaneous correction of the aortic defect. The procedure was guided by multimodality imaging obtained by the fusion of cath-lab angiographic images with angio-CT images. A 6F right radial access and a 14F echo-guided right femoral access were obtained. Coronary angiography confirmed the absence of coronary artery disease. Before proceeding with stent deployment, two 5F pigtail were positioned on both sides of the coarctation in order to obtain a measurement of hemodynamic gradient between aortic root and abdominal aorta detecting a gradient of 46 mmHg. Successively, we started with crossing the aortic coarctation with a stiff hydrophilic guidewire and then advancing a multipurpose catheter in order to put in place an ultrastiff guide in ascending aorta. After that, we advance the covered stent delivery system and, guided by reference of

Discussion
Coarctation of the aorta (CoA) accounts for 6%-8% of all CHD, and it is the fifth most frequent one [1], but it is the most commonly missed fetal CHD diagnosis too, withless than one-third of the cases been detected at prenatal screening [2]. Coarctation of the aorta in adults accounts for 0.2% of all hypertension cases in adults [3]. CoA can be considered as a manifestation of a systemic arteriopathy [4]. It can present as a localized stenosis, or as a long, hypoplastic tract of the aorta, other than as an aortic arch interruption [4,5]. Typically, CoA is located near the ductal remnant and left subclavian artery [6]; unusually it occurs at the ascending aorta or abdominal aorta [7]. When the lesion is a discrete stenosis, it consists of a shelf in the posterolateral aortic wall opposite the (remnant of the) ductus arteriosus [5]. It is often associated with other cardiovascular lesions; the most frequent defect is bicuspid aortic valve (up to 85%), ascending aortic aneurysm, sub/supraaortic stenosis, mitral valve disease or other CHD [4]. Extra-cardiac vascular anomalies have been reported in CoA patients and the most relevant are intracerebral aneurysms (2.5% -10%) [8]. Our report is a case of aortic coarctation presenting in young male adult.
CoA presenting during adult life could be a missed case of native coarctation or more frequently a case of re-coarctation, following previous typically surgical treatment. CoA clinical presentation is variable, and depends on several factors, such as the site of coarctation or the presence of others associated lesions.
It can be asymptomatic or present with heart failure or endocarditis manifestations. Older patients may show symptoms such as headache, dizziness, tinnitus, abdominal angina, and exertional leg fatigue. The most common presenting finding is systemic hypertension [7]. In adult patients, differential diagnosis includes other causes of secondary arterial hypertension, possibly leading to hypertensive cardiomyopathy. CoA patients have a life expectancy of 35 years without treatment [7,9], but treated ones have a shorter life expectancy too. Nowadays, patients who reach adolescence can survive up to 40 years after the initial repair if an adult CHD specialized center follow them up [10]. Close lifelong follow-up is mandatory, because of the important cardiovascular morbidity related to re-coarctation, descending aortic aneurysm, hypertension, and the other associated CHD [7,8,10]. First surgical repair happened in 1944 [11], then in 1983, Lock et al. performed the first percutaneous balloon angioplasty [12]. In 1991, a stent apposition repair was described, and finally, the first Covered Stent was used in 1999 [13]. Nowadays, the development of multimodality diagnostic imaging has allowed a careful pre-procedural planning of complex percutaneous structural interventions.
Furthermore, dedicated software could provide additional information to angiographic views, integrating pre-and procedural diagnostic images. Thus, this upgraded technology allows a higher intra-procedural accuracy, shorter procedural time with radiation sparing and lower amount of dye injection. This management could translate in better procedural outcomes. In our case, a multidisciplinary "heart team" decision was consistent with the last ESC guideline for the management of adult congenital heart disease, which indicates a percutaneous repair of aortic coarctation or recoarctation in adult patients when technically feasible [4]. The use of covered stent instead of uncovered one was preferred in order to reduce the possibility of complication after stent deployment.

Conclusion
Percutaneous treatment of aortic coarctation is a quite rare procedure, but it is associated with good safety and procedural success rate. In our procedure, the support of novel imaging technique played a key role for the deployment of the covered stent. The vessel navigator system allowed us to perform the procedure with a lower risk of left subclavian artery obstructing. Furthermore, the quality of imaging allowed a very low volume of contrast dye use.