Background
Coarctation of the aorta accounts for 5–8% of all congenital heart defects. The male-to-female ratio is 2:1. It is defined as a narrowing of the aorta, typically located in the area where the ductus arteriosus inserts.Coarctation of the aorta may occur as an isolated form or in association with various other cardiac and non-cardiac lesions. Signs and symptoms depend on the severity of CoA. Patients with serious CoA exhibit signs and symptoms early in life,
while particularly mild cases may not become evident until adulthood.
Case presentation
A 24-year-old caucasian, obese women was admitted to the Clinic due to high blood preasure and esay fatigue. The coarctation of the aorta was diagnosed two years ago. In August 2012 she underwent miscarrige caused by uncontrolled arterial hypertension.
On admission, she was hemodynamically stable with no signs of pulmonary oedema or peripheral tissue swelling. Her blood pressure was 160/100 mmHg. We noticed pressure difference between upper and lower limbs: 30 mmHg. Furthermore, systolic murmur was heard.
The ECG at rest showed sinus rhythm 70 bpm, normal heart axis, left ventricular hypertrophy. No abnormalities in blood tests were detected.
The transthoracic echocardiogram (TTE) showed normal left ventricle size and function, bicuspid aortic valve, significant narrowing of the artic isthmus and abnormal flow in descending aorta.
Computed tomography revealed significant narrowing of aortc isthmus. Distal from the occlusion, the aorta was supported by many collaterals originating from the left mammary artery and the intercostals arteries. The patient was treated by: perindopril/indapamid, bisoprolol and amlodypin.
Current guidelines
2010 ESC Guidelines for the management of grown-up congenital heart disease:
→ Indications for intervention in coarctation of the aorta:
All patients with a non-invasive pressure difference >20 mmHg between upper and
lower limbs, regardless of symptoms but with upper limb hypertension (>140/90 mmHg in
adults), pathological blood pressure response during exercise, or significant LVH should have
intervention.
Class I C.
References
1. ESC Guidelines for the management of grown-up congenital heart disease (new version 2010), European Heart Journal (2010) 31, 2931-2933.
2. Rudziński A, Kordon Z, Król-Jawień W. Zwężenie cieśni aorty. przezskórna angioplastyka cieśni aorty. In: Podolec P, Tracz W, Hoffman P, editors. Echokardiografia praktyczna tom III. Kraków, Medycyna Praktyczna; 2005. p.257-270.
Expert’s comments:(Written authorization required from each expert)
1. Prof. Andrzej Rudziński MD, PhD
The patient with significant coarctation of the aorta, bicuspid aortic valve and with arterial hypertension. The percutaneus balloon angioplasty for native coarctation of the aorta with stent implantation is possible.
2.Prof. Lesław Szydłowski MD, PhD
The percutaneus balloon angioplasty for native coarctation of the aorta with stent implantation should be performed with surgeon support.
3. Jacek Pająk MD, PhD
All young patients with arterial hypertension should be carefully examined specially to take a feel patients pulse on femoral artery.
4. Grzegorz Kopeć MD, PhD
Coarctation of aorta should be operated as soon as possible after the diagnosis. The best age for operation is between 2 and 5 years. In older patients the risk of operation is higher and the risk of persistance of arterial hypertension is 25-50%. Besides older age and ageing associated degenerative changes of the aortic wall, the surgical risk factors include a coexisting bicuspid aortic valve, mitral valve abnormalities, coronary artery disease, and end organ damage from systemic arterial hypertension. Surgical or interventional treatment can be considered. The late complications include arterial hypertension, recoarctation or residual stenosis, distension or aneurysm of the ascending aorta.
5. Jakub Podolec MD, PhD
In the last years, stent implantation has become an alternative treatment method to surgery in adult patients with coarctation of the aorta. The success rate is very high (ca. 90%). The aorta diameter increase and decrease in the systolic gradient between extremities result in better control of hypertension. Except for hypertension and probably also due to this factor it was proven that patients with coarctation of the aorta are at higher risk of developing intracranial aneurysms occurring at earlier age than in regular population. In comparison to three groups of patients: after surgery, stent implantation and ballon angioplasty, patients after stent implantation had significantly lower acute complications rate comparing to surgery or ballon angioplasty patients, although they more often required reintervention. During follow up of these patients, stent and surgical treatment resulted in better hemodynamic and integrated aortic arch imaging outcomes comparing to ballon angioplasty. Roifman et al. haven’t found any correlation between coarctation of the aorta and coronary artery disease. Therefore it is reasonable to perform less traumatic and safe procedure with stent implantation.
Expert’s conclusions:
The patient was qualify for percutaneus balloon angioplasty of the coarctation of the aorta.
Authors:
Lidia Tomkiewicz-Pająk MD, PhD1, Justyna Błaut-Jurkowska MD1 , Maria Olszowska MD, PhD1, Prof. Piotr Podolec MD, PhD1, Prof. Tadeusz Przewłocki MD, PhD1, Jakub Stpniewski MD, PhD1
Expert’s conclusions:
Prof. Andrzej Rudziński MD, PhD2, Prof. Lesław Szydłowski MD, PhD3, Jacek Pajak MD, PhD4, Grzegorz Kopeć MD, PhD1, Jakub Podolec MD5
1Department of Cardiac and Vascular Disease in John Paul II Hospital, Institute of Cardiology, Faculty of Medicine, Jagiellonian University, Krakow, Poland.
2Department of Pediatric Cardiology, Polish-American Children’s Hospital, Jagiellonian Uniwersity, Krakow, Poland
3Department of Pediatric Cardiology, Medical University of Silesia, Katowice, Poland
4Independent Public Clinical Hospital No.6 Medical University of Silesia in Katowice, Upper Silesia Center of the children’s Health. John Paul II, Pediatric Heart Surgery Department Poland
5Department of Hemodynamics and Angiocardiography, Cardiology Institute, Collegium Medicum, Jagiellonian University, Krakow, Poland; Center for Interventional Cardiac and Vascular Diseases, John Paul II Hospital, Kraków, Poland
