PhD Scientific Days 2022

Clinical Medicine V. (Poster discussion will take place in the Aula during the Coffee Break)

A ‘general’ case of a complex congenital heart patient

Text of the abstract

A 57-year-old male patient with reconstructed double-outlet right ventricle was referred to our institute with a severe left ventricle dysfunction (HFrEF) and severe aortic stenosis for evaluation for operation.
He had the clinical signs of severe heart failure (NYHA III-IV). Non-invasive examinations (ECG, stress echocardiography, cardiac CT) showed a true severe low flow-low gradient aortic stenosis, with appropriate contractile reserve and subaortic ventricular septal defect (VSD). Surprisingly, there was not even moderate pulmonary valve disease or any signs for pulmonary hypertension. Preoperative coronarography showed a severe stenosis in the left anterior descending (LAD) coronary artery.
After multidisciplinary adult cardiology evaluation, percutaneous coronary intervention of the LAD artery and transcatheter aortic valve implantation (TAVI) was advised.
During the coronary intervention a moderate sinus valsalva ectasia was seen, which contraindicated TAVI. Further evaluation by a congenital team showed that the left outflow tract was too narrow, and the VSD was communicating with a pocket surrendered by right ventricle (RV) trabeculae and the shunt could not communicate with the RV. Based on these data our patient was referred for heart surgery.
An adult- and a congenital heart surgeon performed an aortic bioprosthesis implantation and resected the aberrant tissue from the left outflow tract, VSD remained untouched.
During a short uneventful postoperative time, optimal medical heart failure treatment started (beta-blocker, angiotensin receptor blocker, aldosterone antagonist).
After an event-free rehabilitation period, at the 3-month-visit, the patient had no dyspnoe (NYHA I). Echocardiography showed a mildly reduced ejection fraction (HFmrEF), and the aortic bioprosthesis was functioning well.

Overall this is a special case with unusual VSD, isolated calcificated aortic stenosis and coronary disease, which are not common in this population, but operated successfully.