Article
"Development of the European Network in Orphan Cardiovascular Diseases"
„Rozszerzenie Europejskiej Sieci Współpracy ds. Sierocych Chorób Kardiologicznych”
Title: Treatment of aortic aneurysms in patients with congenital
heart disease
Author: Jakub Podolec MD, PhD
Affiliation: Interventional Cardiology Department, Cardiology Institute, Collegium
Medicum, Jagiellonian University at the John Paul II Hospital, Krakow, Poland
Introduction and literature review
Aortic diseases enclose wide spectrum of arterial diseases and include aortic aneurysms, acute
aortic syndromes (AAS) including aortic dissection (AD), intramural haematoma (IMH),
penetrating atherosclerotic ulcer (PAU) and traumatic aortic injury (TAI), pseudoaneurysm,
aortic rupture, atherosclerotic and inflammatory affections, as well as genetic diseases (e.g.
Marfan syndrome) and congenital abnormalities including the coarctation of the aorta (CoA).
Mortality rate from aortic aneurysms and AD increased from 2.49 per 100 000 to 2.78 per 100
000 inhabitants between 1990 and 2010, with higher rates for men. The incidence of
abdominal aortic aneurysms have been reduced in the recent years [1, 2]. Aortic diameter over
6.0 cm have a fivefold increased risk of rupture, with a yearly risk of rupture of 14.1% [3]
Bobylev D et al. reviewed 51 patients with thoracic aneurysms. For ascending aortic
aneurysms, conduit replacement is the method of choice. The David procedure provides good
results in selected patients. For descending aortic aneurysms, graft replacement is the
John Paul II Hospital in Kraków
Jagiellonian University, Institute of Cardiology
80 Prądnicka Str., 31-202 Kraków;
tel. +48 (12) 614 33 99; 614 34 88; fax. +48 (12) 614 34 88
e-mail: rarediseases@szpitaljp2.krakow.pl
www.crcd.eu
preferred procedure [4]. Complications include endoleaks, aortic aneurysm evolutions, stent
graft infections, proximal and distal dissections after stent graft implantations. Reports of
strokes after stent graft placement vary from 0 to 18% and the complications are very often
severe [5].
Diagnosis
Imaging techniques including Chest X-ray, Transthoracic and transesophageal echocardiography,
PET, MRI and aortography play an important role in the diagnostic evaluation, but computed
tomography plays a central role in the diagnosis, risk stratification, and management of aortic
diseases. It is recommended that diameters be measured at pre-specified anatomical landmarks,
perpendicular to the longitudinal axis. In the case of repetitive imaging of the aorta over time, to assess
change in diameter, it is recommended that the same imaging modality with the lowest iatrogenic risk
be used [6].
Treatment
Thoracic endovascular repair (TEVAR) is performed by retrograde transarterial advancement
of delivery device (up to 24 F) carrying the collapsed self-
expandable stent-graft.
According the ESC guidelines from 2014 the endovascular abdominal aortic aneurysm
repair might be performed if the proximal aortic neck (defined as the normal aortic segment
between the lowest renal artery and the most cephalad extent of the aneurysm) should have a
length of at least 10 – 15 mm and should not exceed 32 mm in diameter. The iliofemoral axis
has to be evaluated by CT, since large delivery devices (14 – 24 F) are being used.
Aneurysmal disease of the iliac arteries needs extension of the stent graft to the external iliac
artery [6].
Conclusions
Thoracic aortic aneurysms in patients with congenital heart disease is highly associated with
bicuspid aortic valve and aortic coarctation. The close follow up especially in patients with
coarctation of the aorta and bicuspid aortic valve is of great importance. Akin I et al. suggest
that rather than replacing conventional surgical treatment, endovascular repair more likely
John Paul II Hospital in Kraków
Jagiellonian University, Institute of Cardiology
80 Prądnicka Str., 31-202 Kraków;
tel. +48 (12) 614 33 99; 614 34 88; fax. +48 (12) 614 34 88
e-mail: rarediseases@szpitaljp2.krakow.pl
www.crcd.eu
will play a complementary and less invasive option in the treatment aortic aneurysms. Risk
estimation in patients qualified for surgical treatment is mostly based on the clinical
presentation and co-morbidities of a patient. The main limitations for andovascular treatment
are mostly based on anatomical criteria. The treatment should be performend in well qualified
centers with both interventional cardiology and surgery departments on site. Qualification of
those patients should be based on multidisciplinary approach with close follow up of all
patients.
References
1. Sampson UKA, Norman PE, Fowkes GR, Aboyans V, Song Y, Harrell FE,
Forouzanfar MH, Naghavi M, Denenberg JO, McDermott MM, Criqui MH, Mensah
GA, Ezzati M, Murray C. Global and regional burden of aortic dissection and
aneurysms. Global Heart 2014;8:171–180. 2. Sampson UKA, Norman PE, Fowkes GR, Aboyans V, Song Y, Harrell FE,
Forouzanfar MH, Naghavi M, Denenberg JO, McDermott MM, Criqui MH, Mensah
GA, Ezzati M, Murray C. Estimation of global and regional incidence and prevalence
of abdominal aortic aneurysms 1990 to 2010. Global Heart 2014;8: 159 – 170. 3. Davies RR, Goldstein LJ, Coady MA, et al. Yearly rupture or dissection rates for
thoracic aortic aneurysms: simple prediction based on size. Ann Thorac Surg
2002;73:17–27. 4. Bobylev D, Boethig D, Breymann T, Mathoni A, Horke A, Ono M. Surgical
Treatment of Thoracic Aortic Aneurysms in Patients with Congenital Heart Disease.
Thorac Cardiovasc Surg. 2014 Jul 4. [Epub ahead of print]
5. Akin I, Nienaber CA. Interventional treatment strategies of thoracic aortic pathologies.
Eur Rev Med Pharmacol Sci. 2014;18(17):2562-74.
6. Erbel R, Aboyans V, Boileau C, Bossone E, Bartolomeo RD, Eggebrecht H,
Evangelista A, Falk V, Frank H, Gaemperli O, Grabenwöger M, Haverich A, Iung B,
Manolis AJ, Meijboom F, Nienaber CA, Roffi M, Rousseau H, Sechtem U, Sirnes PA,
Allmen RS, Vrints CJ; ESC Committee for Practice Guidelines. 2014 ESC Guidelines
John Paul II Hospital in Kraków
Jagiellonian University, Institute of Cardiology
80 Prądnicka Str., 31-202 Kraków;
tel. +48 (12) 614 33 99; 614 34 88; fax. +48 (12) 614 34 88
e-mail: rarediseases@szpitaljp2.krakow.pl
www.crcd.eu
on the diagnosis and treatment of aortic diseases: Document covering acute and
chronic aortic diseases of the thoracic and abdominal aorta of the adult. The Task
Force for the Diagnosis and Treatment of Aortic Diseases of the European Society of
Cardiology (ESC). Eur Heart J. 2014 Nov 1;35(41):2873-926.
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John Paul II Hospital in Kraków
Jagiellonian University, Institute of Cardiology
80 Prądnicka Str., 31-202 Kraków;
tel. +48 (12) 614 33 99; 614 34 88; fax. +48 (12) 614 34 88
e-mail: rarediseases@szpitaljp2.krakow.pl
www.crcd.eu