(I-1B.6) 33 year-old patient with coarctation of the aorta and tetralogy of Fallot Lidia Tomkiewicz-Pająk, Tomasz Pawelec

Background
Tetralogy of Fallot (TOF) is one of the most common congenital heart development disorders (CHDs). This condition is classified as cyanotic heart disorder. It consists of right ventricular (RV) outflow tract obstruction (RVOTO) (infundibular stenosis), ventricular septal defect (VSD), aorta dextroposition, and right ventricular hypertrophy. Patients with tetralogy of Fallot initially present with cyanosis shortly after birth, thereby attracting early medical attention. Surgical correction is a procedure of choice. It is usually performed in the infancy. Sometimes reoperations are required. The prognosis is favourable if the operation is done. More than 90% survive to adulthood and live active, healthy, and productive lives.
Coarctation of the aorta (CoA) is a congenital narrowing of the lumen in a section of the aorta. The narrowing is most commonly in the upper thoracic aorta but can occur in the abdominal aorta. It is present at birth and males are more often affected than females. Clinical symptoms are variable and depend on the position, degree and extent of the narrowed segment of the aorta. Traditionally the treatment requires open heart surgery. Balloon angioplasty is an alternative option but recurrence, aneurysm and aortic dissection remained disadvantages of both treatments.

Case presentation
A 35 years old male with corrected tetralogy of Fallot modo Waterstone (1997) and reoperation with total correction (1981) and surgical correction of coarctation of the aorta (CoA) with by-pass protesis implantation followed by stent implantation due to re-coarctation (1994, 1995) was admitted with a suspicion of sequent re-coarctation. Additionally, he has double aortic arch with occluded posterior arch distal to left subclavian artery branch-of, arterial hypertension (ESC class II). Hemodynamic assessment revealed significant gradient in the region of previously implanted stents to the thoracic aorta. Angio CT showed additionally, stenosis of the left pulmonary artery. Right heart catheterization did not signs of shunts or pathological gradients in the right heart or pulmonary arteries. No pulmonary stenosis was confirmed.
What is should be the most desirable approach to this patient at this point?

Expert’s comments:(Written authorization required from each expert)

1. Bogusław Kapelak, MD, PhD
Taking into considaration all aspects of the patients medical history and observed symptoms percutaneous balloon intervention should be performed in order to reduce the gradient of the coarcted aorta.

Expert’s conclusions:
Patient was qualified for percutaneous balloon angioplasty of narrowed aorta.

Authors:
Lidia Tomkiewicz-Pająk¹, Tomasz Pawelec²

Experts:
Bogusław Kapelak, MD, PhD³

¹Department of Cardiac and Vascular Diseases, Jagiellonian University College of Medicine, John Paul II Hospital, Krakow, Poland
²Department of Hemodynamics and Angiocardiography Jagiellonian University College of Medicine, John Paul II Hospital, Krakow, Poland
³Department of Cardiac, Vessels Surgery and Transplantology, Jagiellonian University College of Medicine, John Paul II Hospital, Krakow, Poland