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Abstract
We would like to present the following clinic case, as it highlights
the importance of taking good clinical history an arranging appropriate investigations in patients with adult congenital heart disease. As well as considering as demonstrating the use of multi modality imaging in these patients to allow diagnostic certainty.
A 19-year-old gentleman with a history of repaired pulmonary atresia with a ventricular septal defect (VSD) presented to the accident and emergency department with breathlessness. He had a Blalock-Taussig shunt in infancy followed by a right ventricle-to-pulmonary artery (RV-PA) conduit and VSD repair. A further RA-PA conduit replacement followed in 2013 due to conduit stenosis. His third procedure was a percutaneous valve-in-valve (V-i-V) Melody pulmonary valve implantation 18 months prior to presentation which was technically successful. Prior to presentation he was unlimited compared to peers.
He presented following a significant blow to his chest whilst playing ice hockey 24 hours prior, with chest pains and dyspnoea, without presyncope or syncope. On assessment thereafter in the accident and emergency department he was haemodynamically stable, with a grade 3–4 systolic murmur heard. Admission baseline chest X-ray, blood tests and ECG were unremarkable.
An urgent transthoracic echocardiogram (TTE) showed significant increments in the tricuspid valve regurgitant velocity to 4.6 m/s compared to his previous study. There was an abnormal melody valve contour and substantial turbulence on colour Doppler, with a Vmax of 6 m/sec (mean gradient 92 mmHg) across the
percutaneous valve (Picture D). A subsequent computed tomography pulmonary angiogram revealed marked flattening of the proximal RV-PA conduit causing significant stenosis (Picture E,).
It was felt that the patient had become symptomatic from significant conduit stenosis, likely secondary to the injury sustained from full body blunt force trauma. The gradients appreciated were an indication for cardiac surgery.
The patient subsequently underwent an urgent third sternotomy for RV-PA conduit replacement with a 7 mm Magna-Ease valve in a 30 mm Gelweave graft. He had an uneventful post-operative recovery with a short intensive care stay.
Post-operative TTE demonstrated a well-functioning conduit with good biventricular function. He was advised to cease playing ice hockey, and avoid high contact sports.
This case highlights an unusual cause of acute, critical pulmonary valve stenosis, most likely secondary to blunt force trauma to the chest.
D: TTE showing critical V-i-V stenosis. E: CTPA showing marked new proximal flattening of the proximal RV-PA conduit stent causing significant stenosis
Conflict of Interest none