Nasser J. Moiduddin, MD, FACC, FSCAI; Thomas Forbes, MD, FACC, FSCAI; William Suarez, MD, FACC ; Yousuf Muhammed-Nasser
Introduction
We present a rare complex adult congenital case of extreme distortion of the aortic valve with balloon coronary compression testing in a pulmonary homograft. We believe that this is a relative contraindication for transcatheter valve implantation despite normal coronary flow and no aortic insufficiency. A few studies have described aortic distortion with aortic insufficiency in patients having transannular patch. This patient developed severe aortic valve distortion without aortic insufficiency during homograft dilation due, in part, to the presence of a residual aortic pseudoaneurysm.
Clinical Case
The patient was a 26-year-old male with a history of Truncus Arteriosus Type I. He was status post complete repair with a ventricular septal defect patch, 12 mm pulmonary homograft in the pulmonary position, and ascending aortic arch patch repair. As expected, he underwent multiple revisions and surgeries. First, his 12 mm RV-PA pulmonary homograft was upsized to a 20 mm aortic homograft. Several years later, he developed Sick Sinus Syndrome, requiring an epicardial pacemaker that was later changed to a transvenous system. Ten years later, his truncal valve was replaced with a 27 mm freestyle prosthesis, and his RV-to-PA aortic homograft was exchanged for a 23 mm Contegra conduit. He developed increased right ventricular outflow tract gradients warranting consideration of a pulmonary valve implantation. In preparation for a potential transcatheter valve, he was noted to have a significant truncal root pseudoaneurysm, and subsequently was found to have subacute infective endocarditis with streptococcus mutans. He underwent pseudoaneurysm repair. The Contegra conduit was replaced with a 24 mm pulmonary homograft with a hemashield extension and he had removal of his pacing leads and generator. Over the years, he seemed to do well without the need for pacing. After endocarditis was ruled out, he was referred for another pulmonary valve implantation with increasing right ventricular outflow tract gradients. He developed increased right ventricular outflow tract gradients and he was referred for another pulmonary valve implantation. Given his multiple surgeries, the transcatheter approach was preferred.
FIGURE 1 AP: Shallow RAO view, Lat: Straight. There is a calcified 24 mm pulmonary homograft that is stenotic down to 11 mm. The area of narrowing extends approximately 3 cm from the subvalvar area to the main pulmonary artery. There are confluent pulmonary artery branches.
Clinically he was asymptomatic, but sedentary. He was afebrile and his cardiac exam revealed an RV lift with palpable thrill. He had a harsh 4/6 systolic ejection murmur at the left sternal border. His diastole was quiet. There was mild cardiomegaly without infiltrates on a recent chest x-ray.
Echocardiography revealed moderate to severe pulmonary artery conduit stenosis proximally at a peak 55 mm Hg gradient. His calculated RV systolic pressure was 70 to 75 mm Hg. His ascending aorta was severely dilated with a suspected aneurysm on the antero-lateral side of the neoaortic (truncal valve). Biventricular systolic function was normal.
To read the full article, please go to the December 2020 Issue of CCT.